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Sample records for acute sensorineural hearing

  1. Acute sensorineural hearing loss and severe otalgia due to scrub typhus

    Directory of Open Access Journals (Sweden)

    Kim Dong-Min

    2009-10-01

    Full Text Available Abstract Background Scrub typhus is an acute febrile illness caused by Orientia tsutsugamushi. Case presentations We encountered a patient with sensorineural hearing loss complicating scrub typhus, and three patients with scrub typhus who complained of otalgia, which was sudden onset, severe, paroxysmal, intermittent yet persistent pain lasting for several seconds, appeared within 1 week after the onset of fever and rash. The acute sensorineural hearing loss and otalgia were resolved after antibiotic administration. Conclusion When patients in endemic areas present with fever and rash and have sensorineural hearing loss or otalgia without otoscopic abnormalities, clinicians should suspect scrub typhus and consider empirical antibiotic therapy.

  2. Sensorineural hearing loss and acute vestibulopathy in HELLP syndrome: A case report

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    Edwin Halliday

    2017-03-01

    Full Text Available HELLP syndrome is a complication of pregnancy characterised by haemolysis (H, elevated liver enzymes (EL and low platelets (LP. Patients present with non-specific symptoms; delivery of the baby is the only treatment. We present the case of a woman who developed sudden sensorineural hearing loss during her HELLP syndrome episode. Poorly perfused placental tissue may release factors into the systemic circulation causing endothelial cell injury and a dysfunctional cascade of coagulation, vasoconstriction and intravascular fluid redistribution. There may be subsequent damage to the endothelium of the vasa nervorum of the vestibulocochlear nerve, resulting in sensorineural hearing loss and acute vestibulopathy.

  3. Congenital sensorineural hearing loss

    International Nuclear Information System (INIS)

    Mafee, M.F.; Selis, J.E.; Yannias, D.A.; Valvassori, G.E.; Pruzansky, S.; Applebaum, E.L.; Capek, V.

    1984-01-01

    The ears of 47 selected patients with congenital sensorineural hearing loss were examined with complex-motion tomography. The patients were divided into 3 general categories: those with a recognized syndrome, those with sensorineural hearing loss unrelated to any known syndrome, and those with microtia. A great variety of inner ear anomalies was detected, but rarely were these characteristic of a particular clinical entity. The most common finding was the Mondini malformation or one of its variants. Isolated dysplasia of the internal auditory canal or the vestibular aqueduct may be responsible for sensorineural hearing loss in some patients. Patients with microtia may also have severe inner ear abnormalities despite the fact that the outer and inner ears develop embryologically from completely separate systems

  4. Congenital sensorineural hearing loss

    Energy Technology Data Exchange (ETDEWEB)

    Mafee, M.F.; Selis, J.E.; Yannias, D.A.; Valvassori, G.E.; Pruzansky, S.; Applebaum, E.L.; Capek, V.

    1984-02-01

    The ears of 47 selected patients with congenital sensorineural hearing loss were examined with complex-motion tomography. The patients were divided into 3 general categories: those with a recognized syndrome, those with sensorineural hearing loss unrelated to any known syndrome, and those with microtia. A great variety of inner ear anomalies was detected, but rarely were these characteristic of a particular clinical entity. The most common finding was the Mondini malformation or one of its variants. Isolated dysplasia of the internal auditory canal or the vestibular aqueduct may be responsible for sensorineural hearing loss in some patients. Patients with microtia may also have severe inner ear abnormalities despite the fact that the outer and inner ears develop embryologically from completely separate systems.

  5. Sudden Sensorineural Hearing Loss

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    Kuhn, Maggie; Heman-Ackah, Selena E.; Shaikh, Jamil A.

    2011-01-01

    Sudden sensorineural hearing loss (SSNHL) is commonly encountered in audiologic and otolaryngologic practice. SSNHL is most commonly defined as sensorineural hearing loss of 30dB or greater over at least three contiguous audiometric frequencies occurring within a 72-hr period. Although the differential for SSNHL is vast, for the majority of patients an etiologic factor is not identified. Treatment for SSNHL of known etiology is directed toward that agent, with poor hearing outcomes characteristic for discoverable etiologies that cause inner ear hair cell loss. Steroid therapy is the current mainstay of treatment of idiopathic SSNHL in the United States. The prognosis for hearing recovery for idiopathic SSNHL is dependent on a number of factors including the severity of hearing loss, age, presence of vertigo, and shape of the audiogram. PMID:21606048

  6. Idiopathic sudden sensorineural hearing loss and acute low-tone sensorineural hearing loss: a comparison of the results of a nationwide epidemiological survey in Japan.

    Science.gov (United States)

    Yoshida, Tadao; Sone, Michihiko; Kitoh, Ryosuke; Nishio, Shin-Ya; Ogawa, Kaoru; Kanzaki, Sho; Hato, Naohito; Fukuda, Satoshi; Hara, Akira; Ikezono, Tetsuo; Ishikawa, Kotaro; Iwasaki, Satoshi; Kaga, Kimitaka; Kakehata, Seiji; Matsubara, Atsushi; Matsunaga, Tatsuo; Murata, Takaaki; Naito, Yasushi; Nakagawa, Takashi; Nishizaki, Kazunori; Noguchi, Yoshihiro; Sano, Hajime; Sato, Hiroaki; Suzuki, Mikio; Shojaku, Hideo; Takahashi, Haruo; Takeda, Hidehiko; Tono, Testuya; Yamashita, Hiroshi; Yamasoba, Tatsuya; Usami, Shin-Ichi

    2017-01-01

    The aim of this study was to investigate the differences between idiopathic sudden sensorineural hearing loss (SSNHL), and acute low-tone sensorineural hearing loss (ALHL) using the results of a nationwide survey database in Japan and to analyze the variables associated with their clinical features and the severity of hearing impairment, treatment, and prognosis. Participants were patients registered between April 2014 and March 2016 in a multicenter epidemiological survey database involving 30 university hospitals and medical centers across Japan. Statistical analysis was performed to clarify the factors associated with their clinical characteristics and the severity of hearing impairment, treatment, and prognosis. Idiopathic SSNHL and ALHL differed significantly in terms of male-to-female ratio, age distribution, and time from onset to start of treatment. The treatment methods and hearing prognosis also differed markedly between the two diseases. A majority (92%) of idiopathic SSNHL patients were administered some type of corticosteroid, while half of the ALHL patients received corticosteroids and a diuretic agent. The results suggested that idiopathic SSNHL and ALHL belonged to different categories of inner ear disease.

  7. AICA syndrome with facial palsy following vertigo and acute sensorineural hearing loss.

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    Ikegami-Takada, Tomoko; Izumikawa, Masahiko; Doi, Tadashi; Takada, Yohei; Tomoda, Koichi

    2012-04-01

    We report a case of infarction of the anterior inferior cerebellar artery (AICA) with peripheral facial palsy following vertigo and acute sensorineural hearing loss. A 39-year-old female presented with vertigo and sudden hearing loss, tinnitus, and aural fullness of the right ear. An audiogram revealed a severe hearing loss at all tested frequencies in the right ear. Spontaneous nystagmus toward the left side was also observed. Otoneurological examinations showed sensorineural hearing loss of the right ear and horizontal and rotatory gaze nystagmus toward the left side, and a caloric reflex test demonstrated canal paresis. Initially, we diagnosed the patient for sudden deafness with vertigo. However, right peripheral facial palsy appeared 2 days later. An eye tracking test (ETT) and optokinetic pattern test (OKP) showed centralis abnormality. The patient's brain was examined by magnetic resonance imaging (MRI) and magnetic resonance angioglaphy (MRA) and showed an infarction localized in the pons and cerebellum. MRI and MRA revealed infarction of the right cerebellar hemisphere indicating occlusion of the AICA. Consequently, the patient was diagnosed with AICA syndrome but demonstrated regression following steroid and edaravone treatment. We suggest that performing MRI and MRA in the early stage of AICA syndrome is important for distinguishing cerebellar infarction resulting from vestibular disease. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

  8. Acute unilateral sensorineural hearing loss associated with anabolic steroids and polycythaemia: case report.

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    Tikka, T; Mistry, N; Janjua, A

    2016-03-01

    Unilateral sudden sensorineural hearing loss due to an infarct in the vertebrobasilar system has been widely reported. Most patients have a background of traditional coronary risk factors related to these cerebrovascular episodes. A 32-year-old male, a regular user of anabolic steroids, presented to the emergency department with unilateral sensorineural hearing loss and symptoms suggestive of an infarct of the anterior inferior cerebellar artery but in the absence of risk factors for ischaemic stroke. Magnetic resonance imaging confirmed the presence of infarction in the region supplied by the anterior inferior cerebellar artery. Polycythaemia was found on haematological analysis, which we believe was secondary to the use of anabolic steroids. The patient was commenced on aspirin as per the stroke management protocol. There was resolution of neurological symptomatology six weeks after the episode, but no improvement in hearing. To our knowledge, this is the first case report of unilateral sensorineural hearing loss secondary to the use of anabolic steroids causing polycythaemia. This cause should be considered in the differential diagnosis of patients presenting with sensorineural hearing loss, especially in young males, when no other risk factors can be identified.

  9. Pediatric Sudden Sensorineural Hearing Loss.

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    Kizilay, Ahmet; Koca, Çiğdem Firat

    2016-06-01

    Sudden sensorineural hearing loss is defined as sudden unilateral or bilateral sensorineural hearing loss with at least 30 dB decrease in threshold in 3 contiguous test frequencies occurring over 72 hours or less. It is rare among children. The mechanism of the process and prognosis of the disorder remains unclear. The current incidence of sudden sensorineural hearing loss among pediatric population is unknown. The authors carried out a retrospective chart analysis of patients under 15 years of age from 2004 to 2015, who consulted to the Otolaryngology Head and Neck Surgery Department of Inonu University Medical Faculty. Age, sex, number of affected ear and side, audiometric evaluations, medical follow-up, treatment method, duration of treatment recovery, associated complaints; tinnitus and/or vertigo, presence of mumps disease were recorded for each patient. A 4-frequency pure-tone average (500, 1000, 2000, and 4000 Hz) was calculated for each ear. Complete recovery, defined as some hearing level compared with the nonaffected ear, was observed in 3 patients (21.4 %) and there was no partial hearing recovery. The hearing loss of 11 patient remained unchanged after prednisolone treatment. Two of the 11 patients had bilaterally total sensorineural hearing loss and evaluated as appropriate for cochlear implantation. Sex of patient and laterality of hearing loss were not correlated with hearing recovery. Sensorineural hearing loss among pediatrics has been the issue of otolaryngologists. The incidence, etiology, and treatment methods should be more studied.

  10. Sensorineural hearing loss in hemorrhagic dengue?

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    Ribeiro, Bruna Natália Freire; Guimarães, Alexandre Caixeta; Yazawa, Felipe; Takara, Tammy Fumiko Messias; de Carvalho, Guilherme Machado; Zappelini, Carlos Eduardo Monteiro

    2015-01-01

    Dengue is an acute febrile infectious disease, with high fever followed by symptoms flu-like. Dengue hemorrhagic fever (DHF) is a vascular leak syndrome and could present spontaneous bleeding and worsening of symptoms after some days. Dengue could have some ENT manifestations, however hearing loss is not one of them. Sudden hearing loss is considered as sensorineural or perceptual hearing loss with a sudden onset in a person without other prior otological history. The relation between infectious diseases and sudden hearing are been investigated, some viruses were already linked, but the relation between dengue virus and sudden hearing still remains unknown. This article has the goal of presenting a case of DHF that evolved with SSHL in his hospitalization process. We report a 60 years-male patient of with DHF who developed bilateral secretory otitis media and sensorineural hearing loss after the fifth day of onset of symptoms. His hearing loss remained even after 7 months and the patient was referred for hearing aid fitting. This is the first case report that brings together DHF and sudden hearing loss. In the development of this case no other cause to sudden hearing loss was found and the correlation between dengue and hearing loss was questioned. In the literature review was found that some viruses, as mumps virus, varicella-zoster virus and HSV-1 and HSV-2 are related to sudden hearing loss, all of them fit in the viral theory. Besides the viral theory of sudden hearing loss, there is the vascular theory that is the occlusion of the end artery that supplies the cochlea. DHF has a vascular commitment, and the hypothesis of a vascular cause could be elicited in this case. Many studies in this area are needed and this article has the objective of elicit the discussion about the subject. Could dengue be associated with sensorineural hearing loss? Copyright © 2015. Published by Elsevier Ltd.

  11. Sensorineural hearing loss in children.

    LENUS (Irish Health Repository)

    Wormald, R

    2010-02-01

    The objective of the study was to examine the aetiology of sensorineural hearing loss (SNHL) in a paediatric population presenting to the National Centre of Medical Genetics. A retrospective chart review from 1998 to 2006. One hundred and twenty nine children were investigated for SNHL. The average age of diagnosis of hearing loss was 36 months. The degree of hearing loss was mild in 8 children, moderate in 33 children, severe in 31 children and profound in 57 children. Eighty-five children (66%) were diagnosed with a hereditary hearing loss, 11 (8%) children had an acquired hearing loss and no cause found in 33 (26%) children. This is the first report of the causes of hearing loss in Irish children. The mean age of diagnosis in our cohort is high and emphasises the need for a neonatal screening programme. There remains a number of children for whom the cause of hearing loss remains unknown.

  12. MR Imaging in Sudden Sensorineural Hearing Loss. Time to Talk.

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    Conte, G; Di Berardino, F; Sina, C; Zanetti, D; Scola, E; Gavagna, C; Gaini, L; Palumbo, G; Capaccio, P; Triulzi, F

    2017-08-01

    Sudden sensorineural hearing loss is defined as acute hearing loss of the sensorineural type of at least 30 dB over 3 contiguous frequencies that occurs within a 72-hour period. Although many different causative factors have been proposed, sudden sensorineural hearing loss is still considered "idiopathic" in 71%-85% of cases, and treatments are empiric, not based on etiology. MR imaging implemented with a 3D FLAIR sequence has provided new insights into the etiology of sudden sensorineural hearing loss. Herein, we review the current management trends for patients with sudden sensorineural hearing loss, from the initial clinical diagnosis to therapeutic strategies and diagnostic work-up. We focused primarily on MR imaging assessment and discuss the relevance that MR imaging findings might have for patient management, pointing out different perspectives for future clinical research. © 2017 by American Journal of Neuroradiology.

  13. Methadone Induced Sensorineural Hearing Loss

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    Chadi Saifan

    2013-01-01

    Full Text Available Background. Sudden sensorineural hearing loss (SSHL caused by opiate abuse or overuse has been well documented in the medical literature. Most documented case reports have involved either heroin or hydrocodone/acetaminophen. Recently, case reposts of methadone induced SSHL have been published. Case Report. We present the case of a 31-year-old man who developed SSHL after a methadone overdose induced stupor. He was subsequently restarted on methadone at his regular dose. On follow-up audiometry exams, he displayed persistent moderately severe sensorineural hearing loss bilaterally. Discussion. This case is notable because unlike all but one previously reported case, the patient—who was restated on methadone—did not make a complete recovery. Conclusion. Methadone overuse in rare cases causes SSHL.

  14. Hearing Disorders and Sensorineural Aging

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    Alessandra Fioretti

    2014-01-01

    Full Text Available The physiological age-related hearing loss is defined as presbycusis and it is characterized by reduced hearing sensitivity and problems in understanding spoken language especially in a noisy environment. In elderly the reduced speech recognition is generally caused by a reduction of the cochlear cells in the organ of Corti and degeneration of the central auditory pathways. In order to have a complete management strategy of central and peripheral presbycusis the diagnostic evaluation should include clinical ENT examination, standard audiological tests, and tests of central auditory function. Treatment should include not only the appropriate instruments for peripheral compensation but also auditory rehabilitative training and counseling to prevent social isolation and loss of autonomy. Other common hearing disorders in elderly are tinnitus and hyperacusis which are often undervalued. Tinnitus is characterized by the perception of a “phantom” sound due to abnormal auditory perception. Hyperacusis is defined as a reduced tolerance to ordinary environmental sounds. Furthermore auditory, visual, nociceptive, and proprioceptive systems may be involved together in a possible context of “sensorineural aging.” The aim of this review is to underline the presence of hearing disorders like tinnitus and hyperacusis which in many cases coexist with hearing loss in elderly.

  15. Bilateral sudden sensorineural hearing loss: review.

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    Sara, S A; Teh, B M; Friedland, P

    2014-01-01

    Unilateral and bilateral sudden sensorineural hearing loss represent different disease entities. The unilateral condition is more common and predominantly idiopathic, and up to 65 per cent of patients spontaneously recover hearing. Conversely, the bilateral condition is rare, mostly associated with serious systemic conditions, and has a higher prevalence of morbidity and mortality. A literature search using the PubMed database was conducted using the MeSH terms 'sudden', 'bilateral' and 'sensorineural hearing loss'. One hundred and three reported cases of bilateral sudden sensorineural hearing loss were identified. The condition is most often associated with toxic, autoimmune, neoplastic and vascular conditions. A younger age of onset, with a bimodal age distribution, was seen for bilateral sudden sensorineural hearing loss, compared with the unilateral condition. Patients with the bilateral condition had more profound hearing loss, with poorer recovery and a 35 per cent mortality rate. Vestibular symptoms were also less common than in the unilateral condition. The presentation of bilateral sudden onset sensorineural hearing loss is a medical emergency requiring thorough and urgent investigation to exclude life-threatening and reversible conditions.

  16. Sensorineural hearing loss after magnetic resonance imaging

    DEFF Research Database (Denmark)

    Mollasadeghi, Abolfazl; Mehrparvar, Amir Houshang; Atighechi, Saeid

    2013-01-01

    Magnetic resonance imaging (MRI) devices produce noise, which may affect patient's or operators' hearing. Some cases of hearing impairment after MRI procedure have been reported with different patterns (temporary or permanent, unilateral or bilateral, with or without other symptoms like tinnitus......). In this report, a case of bilateral sensorineural hearing loss in an otherwise healthy patient underwent brain MRI was described. The patient's hearing loss was accompanied with tinnitus and was not improved after 3 months of followup....

  17. Disrupted functional brain connectome in unilateral sudden sensorineural hearing loss.

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    Xu, Haibo; Fan, Wenliang; Zhao, Xueyan; Li, Jing; Zhang, Wenjuan; Lei, Ping; Liu, Yuan; Wang, Haha; Cheng, Huamao; Shi, Hong

    2016-05-01

    Sudden sensorineural hearing loss (SSNHL) is generally defined as sensorineural hearing loss of 30 dB or greater over at least three contiguous audiometric frequencies and within a three-day period. This hearing loss is usually unilateral and can be associated with tinnitus and vertigo. The pathogenesis of unilateral sudden sensorineural hearing loss is still unknown, and the alterations in the functional connectivity are suspected to involve one possible pathogenesis. Despite scarce findings with respect to alterations in brain functional networks in unilateral sudden sensorineural hearing loss, the alterations of the whole brain functional connectome and whether these alterations were already in existence in the acute period remains unknown. The aim of this study was to investigate the alterations of brain functional connectome in two large samples of unilateral sudden sensorineural hearing loss patients and to investigate the correlation between unilateral sudden sensorineural hearing loss characteristics and changes in the functional network properties. Pure tone audiometry was performed to assess hearing ability. Abnormal changes in the peripheral auditory system were examined using conventional magnetic resonance imaging. The graph theoretical network analysis method was used to detect brain connectome alterations in unilateral sudden sensorineural hearing loss. Compared with the control groups, both groups of unilateral SSNHL patients exhibited a significantly increased clustering coefficient, global efficiency, and local efficiency but a significantly decreased characteristic path length. In addition, the primary increased nodal strength (e.g., nodal betweenness, hubs) was observed in several regions primarily, including the limbic and paralimbic systems, and in the auditory network brain areas. These findings suggest that the alteration of network organization already exists in unilateral sudden sensorineural hearing loss patients within the acute period

  18. Sudden (reversible) sensorineural hearing loss in pregnancy.

    LENUS (Irish Health Repository)

    Kenny, R

    2011-03-01

    Sudden hearing loss directly associated with pregnancy or birth is a little known and rare occurrence. The temporary, unilateral, low-frequency sensorineural hearing loss in this case was reported after the birth of the patient\\'s first child, and again during the third trimester of her second pregnancy.

  19. Sudden Sensorineural Hearing Loss; Prognostic Factors

    OpenAIRE

    Arjun, Dass; Neha, Goel; Surinder K, Singhal; Ravi, Kapoor

    2015-01-01

    Introduction: Sudden sensorineural hearing loss (SSNHL) is a frightening and frustrating symptom for the patient as well as the physician. Prognosis is affected by multiple factors including duration of hearing loss, presence of associated vertigo and tinnitus, and co-morbidities such as hypertension and diabetes.   Materials and Methods: Forty subjects presenting to our department with features of sudden hearing loss were included in the study. Detailed otological history and examination, se...

  20. Gd enhanced MRI in sensorineural hearing loss

    Energy Technology Data Exchange (ETDEWEB)

    Takenaka, Mika; Tono, Tetsuya; Toyama, Katsuhiro; Kano, Kiyo; Morimitsu, Tamotsu [Miyazaki Medical Coll., Kiyotake (Japan)

    1996-09-01

    The enhanced MRI hearing findings of the inner ear in 124 patients with sensorineural hearing loss were evaluated. MR images were obtained before and after the intravenous administration of gadolinium (0.1 mmol/kg). In three out of seventy-nine patients with unilateral healing loss, cochlear and/or the vestibular enhancement was noted on the symptomatic side. The positive cases included those with Ramsay-Hunt syndrome, mumps and so-called sudden deafness. Forty-five patients with bilateral hearing loss showed no enhancement within the inner ear. Although positive gadolinium enhancement of the inner ear may detect inflammatory lesions due to a viral infection, its incidence in sensorineural hearing loss, including cases of sudden deafness. seems to be extremely rare. (author)

  1. Sensorineural Hearing Loss after Magnetic Resonance Imaging

    Directory of Open Access Journals (Sweden)

    Abolfazl Mollasadeghi

    2013-01-01

    Full Text Available Magnetic resonance imaging (MRI devices produce noise, which may affect patient’s or operators’ hearing. Some cases of hearing impairment after MRI procedure have been reported with different patterns (temporary or permanent, unilateral or bilateral, with or without other symptoms like tinnitus. In this report, a case of bilateral sensorineural hearing loss in an otherwise healthy patient underwent brain MRI was described. The patient’s hearing loss was accompanied with tinnitus and was not improved after 3 months of followup.

  2. Hyperbaric oxygen therapy for sudden sensorineural hearing loss in divers.

    Science.gov (United States)

    Van Der Wal, A W; Van Ooij, P J A M; De Ru, J A

    2016-11-01

    Sudden sensorineural hearing loss in divers may be caused by either inner-ear barotrauma or inner-ear decompression sickness. There is no consensus on the best treatment option. This study aimed to evaluate the therapeutic value of hyperbaric oxygen therapy for sudden sensorineural hearing loss in divers. A literature review and three cases of divers with sudden sensorineural hearing loss treated with hyperbaric oxygen therapy are presented. Hyperbaric oxygen therapy resulted in hearing improvement in 80 per cent of patients: 39 per cent had hearing improvement and 41 per cent had full recovery. Hyperbaric oxygen therapy improved hearing in divers with sudden sensorineural hearing loss.

  3. [Analyses prognostic factors relevant to sudden sensorineural hearing loss].

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    Wang, Jun; Xiao, Shuifang; Zeng, Zhengang; Zhen, Zhen; Zhang, Xuexi; Lin, Feng; Dong, Mingmin; Lu, Wei; Qin, Zhaobing; Zuo, Bin; Bai, Xianfeng

    2015-06-01

    To investigate the prognostic factors relevant to sudden sensorineural hearing loss. The internationally accepted standardized clinical research methods, unified design, and unified program were adopted to conduct the prospective clinical multi-center study. The sudden deafness patients between 18 to 65 years old, with the course of this disorder less than two weeks, and without any medical treatments were collected, and then, divided into four types according to the hearing curve: type A, acute sensorineural hearing loss in low tone frequencies; type B, acute sensorineural hearing loss in high tone frequencies; type C, acute sensorineural hearing loss in all frequencies; and type D, total deafness. The factors, in terms of age, gender, type of initial audiogram, time delay before the first visit, and severity of hearing loss, were included in the analyses. A total of 1 024 cases with single side sudden deafness were collected in the study from 33 hospitals in China from August 2007 to October 2011, inclusive of for 492 males (48.05%) and 532 females (51.95%). The average age was (41.2 ± 12.8) years old. There were 553 cases (54.00%) in left ear, and 471 cases (46.00%) in right ear. The curative effects of different types were shown as follows: the type in low tone frequencies had the highest rate of 90.73%, the type in all frequencies was 82.59%; the type of total deafness was 70.29%; and the type in high tone frequencies had the lowest rate of 65.96%. It had significant difference of the effective rate between different types (χ(2) = 231.58, P = 0.000). Age, time delay before first visit, and severity of initial hearing loss were significantly correlated with hearing improvement. Initial audiogram of SSNHL might predict hearing recovery. The young in age and a short time delay before starting treatment are positive prognostic factors for hearing recovery in SSNHL. The initial severity of hearing loss is negative prognostic factor of hearing recovery.

  4. Sensorineural hearing loss in hemorrhagic dengue?

    Directory of Open Access Journals (Sweden)

    Bruna Natália Freire Ribeiro

    2015-01-01

    Discussion and conclusion: This is the first case report that brings together DHF and sudden hearing loss. In the development of this case no other cause to sudden hearing loss was found and the correlation between dengue and hearing loss was questioned. In the literature review was found that some viruses, as mumps virus, varicella-zoster virus and HSV-1 and HSV-2 are related to sudden hearing loss, all of them fit in the viral theory. Besides the viral theory of sudden hearing loss, there is the vascular theory that is the occlusion of the end artery that supplies the cochlea. DHF has a vascular commitment, and the hypothesis of a vascular cause could be elicited in this case. Many studies in this area are needed and this article has the objective of elicit the discussion about the subject. Could dengue be associated with sensorineural hearing loss?

  5. Recovery of Idiopathic Sudden Sensorineural Hearing Loss.

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    Edizer, Deniz Tuna; Çelebi, Özlem; Hamit, Bahtiyar; Baki, Ahmet; Yiğit, Özgür

    2015-08-01

    The objective was to identify and evaluate factors that may influence the recovery rate in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). A retrospective analysis was performed for patients with sudden sensorineural hearing loss between 2009 and 2013. Those with an identified etiology were excluded. The patients were divided into four treatment groups: (i) systemic corticosteroids (SC) only, (ii) SC+low-molecular-weight heparin (LMWH), (iii) SC+hyperbaric oxygen (HBO), and (iv) SC+LMWH+HBO. Recovery was evaluated according to Siegel's criteria. Age, initial hearing level, onset, treatment and audiogram types, comorbidities, and associated tinnitus and vestibular symptoms were investigated for their impact on prognosis. Two hundred five patients with ISSNHL were included. Recovery was seen in 59% of the patients. The complete recovery rate was significantly lower in patients older than 60 years and in patients presenting with profound hearing loss. Different audiogram curves had no significant effect on recovery. Sudden hearing loss was accompanied by tinnitus in 107 (52.1%) patients and vestibular symptoms in 55 (26.8%); however, neither was noted to affect prognosis. Different treatment combinations did not significantly affect prognosis. However, hypertension and a delay in treatment by more than 10 days from the onset of hearing loss were associated with a worse prognosis. Profound hearing loss, older than 60 years, a delay in treatment by more than 10 days, and hypertension were negative prognostic factors in this study, whereas, the type of audiogram curve and addition of HBO to SC did not affect prognosis.

  6. Complex Treatment of Sensorineural Hearing Loss

    OpenAIRE

    Aleksandruk, N. V.

    2014-01-01

    Recent data on use of Ginkgo Biloba extract in otorhinolaryngological practice were presented. The mechanism of the curative action of Ginkgo Biloba extract (vasoprotective, antioxidative, rheological, and edematous) was described. Effectiveness of Ginkgo Biloba as a part of complex treatment of sensorineural hearing loss in children was elucidated. Results of the research proved effectiveness of treatment with Ginkgo Biloba and showed perspectives of Ginkgo Biloba use in treatment programs f...

  7. Altered Contralateral Auditory Cortical Morphology in Unilateral Sudden Sensorineural Hearing Loss.

    Science.gov (United States)

    Fan, Wenliang; Zhang, Wenjuan; Li, Jing; Zhao, Xueyan; Mella, Grace; Lei, Ping; Liu, Yuan; Wang, Haha; Cheng, Huamao; Shi, Hong; Xu, Haibo

    2015-12-01

    To investigate the cerebral gray matter volume alterations in unilateral sudden sensorineural hearing loss patients within the acute period by the voxel-based morphometry method, and to determine if hearing impairment is associated with regional gray matter alterations in unilateral sudden sensorineural hearing loss patients. Prospective case study. Tertiary class A teaching hospital. Thirty-nine patients with left-side unilateral sudden sensorineural hearing loss and 47 patients with right-side unilateral sudden sensorineural hearing loss. Diagnostic. To compare the regional gray matter of unilateral sudden sensorineural hearing loss patients and healthy control participants. Compared with control groups, patients with left side unilateral sudden sensorineural hearing loss had significant gray matter reductions in the right middle temporal gyrus and right superior temporal gyrus, whereas patients with right side unilateral sudden sensorineural hearing loss showed gray matter decreases in the left superior temporal gyrus and left middle temporal gyrus. A significant negative correlation with the duration of the sudden sensorineural hearing loss (R = -0.427, p = 0.012 for left-side unilateral SSNHL and R = -0.412, p = 0.013 for right-side unilateral SSNHL) was also found in these brain areas. There was no region with increased gray matter found in both groups of unilateral sudden sensorineural hearing loss patients. This study confirms that detectable decreased contralateral auditory cortical morphological changes have occurred in unilateral SSNHL patients within the acute period by voxel-based morphometry methods. The gray matter volumes of these brain areas also perform a negative correlation with the duration of the disease, which suggests a gradual brain structural impairment after the progression of the disease.

  8. Mobile phone induced sensorineural hearing loss

    International Nuclear Information System (INIS)

    Al-Dousary, Surayie H.

    2007-01-01

    The increased use of mobile phones worldwide has focused interest on the biological effects and possible health outcomes of exposure to radiofrequency fields from mobile phones, and their base stations. Various reports suggest that mobile phone use can cause health problems like fatigue, headache, dizziness, tension and sleep disturbances, however, only limited research data is available in medical literature regarding interaction between electromagnetic fields emitted by mobile phones and auditory function and the possible impact on hearing. We report a case of sensorineural hearing loss due to Global System for Mobile Communication mobile phone use in a 42-year-old male. (author)

  9. Macrocytosis and sudden sensorineural hearing loss: a case report.

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    McMurran, A E L; Adair, R A

    2015-11-01

    Although other blood dyscrasias are known to cause sudden sensorineural hearing loss, macrocytosis has not previously been implicated in the absence of another causative agent. We present a case of bilateral sequential sudden sensorineural hearing loss in a patient with significant macrocytosis (mean corpuscular volume at presentation 124 fl) secondary to alcohol-induced liver dysfunction. A possible pathophysiological mechanism linking macrocytosis and sudden sensorineural hearing loss was identified, suggesting areas for further investigation.

  10. Sudden Sensorineural Hearing Loss; Prognostic Factors

    Directory of Open Access Journals (Sweden)

    Arjun Dass

    2015-09-01

    Full Text Available Introduction: Sudden sensorineural hearing loss (SSNHL is a frightening and frustrating symptom for the patient as well as the physician. Prognosis is affected by multiple factors including duration of hearing loss, presence of associated vertigo and tinnitus, and co-morbidities such as hypertension and diabetes.   Materials and Methods: Forty subjects presenting to our department with features of sudden hearing loss were included in the study. Detailed otological history and examination, serial audiometric findings and course of disease were studied.   Results: Subjects presenting late (in older age, having associated vertigo, hypertension and diabetes had a significantly lower rate of recovery.   Conclusion:  Only 60–65% of patients experiencing SSNHL recover within a period of 1 month; this rate is further affected by presence of multiple prognostic indicators.

  11. A honey bee can threat ear: Sudden sensorineural hearing loss.

    Science.gov (United States)

    Düzenli, Ufuk; Bozan, Nazım; Ayral, Abdurrahman; Yalınkılıç, Abdülaziz; Kıroğlu, Ahmet Faruk

    2017-11-01

    Sudden sensorineural hearing loss is an otologic emergency. Many etiological factors can lead to this pathology. Honey bee (Apis mellifera) sting may lead to local and systemic reactions due to sensitization of the patient. In this paper we described a sudden sensorineural hearing loss occurred after honey bee sting. Copyright © 2017 Elsevier Inc. All rights reserved.

  12. Mobile phone usage does not affect sudden sensorineural hearing loss.

    Science.gov (United States)

    Sagiv, D; Migirov, L; Madgar, O; Nakache, G; Wolf, M; Shapira, Y

    2018-01-01

    Recent studies found that mobile phone users had a significantly greater risk of having elevated thresholds in speech frequencies. This study investigated the correlation between the laterality of sudden sensorineural hearing loss, handedness and the preferred ear for mobile phone use. The study included all patients who presented with sudden sensorineural hearing loss to the Department of Otolaryngology - Head and Neck Surgery in our tertiary referral medical centre between 2014 and 2016. Patients were asked to indicate their dominant hand and preferred ear for mobile phone use. The study comprised 160 patients. No correlation was found between the dominant hand or preferred ear for mobile phone use and the side of sudden sensorineural hearing loss. There was no correlation between the side of the sudden sensorineural hearing loss (preferable or non-preferable for mobile phone use) and audiometric characteristics. No correlation was found between the laterality of ears used for mobile phone and sudden sensorineural hearing loss.

  13. Sensorineural hearing loss in Kawasaki disease.

    Science.gov (United States)

    Aggarwal, Varun; Etinger, Veronica; Orjuela, Andres F

    2016-01-01

    Kawasaki disease is a common nonspecific vasculitis seen in childhood. The most significant long-term sequela is coronary artery aneurysm. However, the spectrum of complications involves not only the heart, but also other organs such as the eyes, skin, kidneys, gallbladder, liver, and central nervous system. Sensorineural hearing loss (SNHL) is a relatively unrecognized complication of the disease. Although most of the complications (except coronary artery aneurysm) are self-limiting, SNHL can be persistent. It is, especially important in infants and young children who might not be able to report the hearing deficits and are most likely to have cognitive and speech delays if this hearing loss is not addressed in a timely manner. We report a child with Kawasaki disease who had SNHL during the 2(nd) week of the illness. The aim of this article is to briefly review the pathophysiology behind this hearing loss and strongly emphasize the importance of universal hearing evaluation in all children diagnosed with Kawasaki disease. This screening in children with Kawasaki disease may provide some timely intervention if needed. Since most Kawasaki disease patients will be seen by cardiologists, we hope to create more awareness about this complication to the cardiology community as well.

  14. Studying VEMP in Sudden Sensorineural Hearing Loss

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    Mohsen Rajati

    2011-03-01

    Full Text Available Introduction: Sudden sensorineural hearing loss (SSNHL has a prevalence of 10 in 100,000. Viral infections, vascular obstruction and rupture of the intracochlear membranes are supposed to be its most common etiologies. About 40% of patients experience vertigo or imbalance. The VEMP (vestibular evoked myogenic potentials test is a known approach for detailed study of the labyrinth. The advantage of this test in comparison to other tests is the selected study of sacculus and sacculocochlear pathways. Materials and Methods:  In this cross sectional study all patients with SSNHL diagnosis were admitted and underwent routine standard treatments and diagnostic tests. Clinical symptoms and paraclinic findings were recorded in especially designed forms and VEMP test was performed on admission. Results: Among the 43 cases with sudden sensorineural hearing loss, 14 (32.6% had vertigo. Thirteen patients (39.2% out of 43 had a negative (abnormal VEMP, 6 of which (42.9% had vertigo, while in the 30 VEMP positive (normal cases, vertigo was detected in 8 (26.6%. Discussion: Saccular dysfunction seems to be an important finding in SSNHL. Although it is more prevalent in the patients with vertigo, it can be found in the non-dizzy cases. VEMP disturbance in SSNHL shows more extensive pathological involvement. Conclusion: In SSNHL the pathology isn’t limited to the cochlea and even in patients with no vestibular symptoms sacculus might be involved.

  15. Prognostic Factors in Sudden Sensorineural Hearing Loss

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    Gamze Atay

    2016-02-01

    Full Text Available Background: Sudden sensorineural hearing loss (SSNHL is still a complex and challenging process which requires clinical evidence regarding its etiology, treatment and prognostic factors. Therefore, determination of prognostic factors might aid in the selection of proper treatment modality. Aims: The aim of this study is to analyze whether there is correlation between SSNHL outcomes and (1 systemic steroid therapy, (2 time gap between onset of symptoms and initiation of therapy and (3 audiological pattern of hearing loss. Study Design: Retrospective chart review. Methods: Patients diagnosed at our clinic with SSNHL between May 2005 and December 2011were reviewed. A detailed history of demographic features, side of hearing loss, previous SSNHL and/or ear surgery, recent upper respiratory tract infection, season of admission, duration of symptoms before admission and the presence of co-morbid diseases was obtained. Radiological and audiological evaluations were recorded and treatment protocol was assessed to determine whether systemic steroids were administered or not. Treatment started ≤5 days was regarded as “early” and >5 days as “delayed”. Initial audiological configurations were grouped as “upward sloping”, “downward sloping”, “flat” and “profound” hearing loss. Significant recovery was defined as thresholds improved to the same level with the unaffected ear or improved ≥30 dB on average. Slight recovery was hearing improvement between 10-30dB on average. Hearing recovery less than 10 dB was accepted as unchanged. Results: Among the 181 patients who met the inclusion criteria, systemic steroid was administered to 122 patients (67.4%, whereas 59 (32.6% patients did not have steroids. It was found that steroid administration did not have any statistically significant effect in either recovered or unchanged hearing groups. Early treatment was achieved in 105 patients (58% and 76 patients (42% had delayed treatment

  16. Constraint-induced sound therapy for sudden sensorineural hearing loss--behavioral and neurophysiological outcomes.

    Science.gov (United States)

    Okamoto, Hidehiko; Fukushima, Munehisa; Teismann, Henning; Lagemann, Lothar; Kitahara, Tadashi; Inohara, Hidenori; Kakigi, Ryusuke; Pantev, Christo

    2014-01-29

    Sudden sensorineural hearing loss is characterized by acute, idiopathic hearing deterioration. We report here the development and evaluation of "constraint-induced sound therapy", which is based on a well-established neuro-rehabilitation approach, and which is characterized by the plugging of the intact ear ("constraint") and the simultaneous, extensive stimulation of the affected ear with music. The sudden sensorineural hearing loss patients who received the constraint-induced sound therapy in addition to the standard corticosteroid therapy showed significantly better recovery of hearing function compared to those who had only received corticosteroid treatments. Additionally, the brain activity obtained in a subgroup of patients suggested that the constraint-induced sound therapy could have prevented maladaptive auditory cortex reorganization. Constraint-induced sound therapy thus appears to be an effective, practical, and safe treatment option for sudden sensorineural hearing loss.

  17. Constraint-induced sound therapy for sudden sensorineural hearing loss – behavioral and neurophysiological outcomes

    Science.gov (United States)

    Okamoto, Hidehiko; Fukushima, Munehisa; Teismann, Henning; Lagemann, Lothar; Kitahara, Tadashi; Inohara, Hidenori; Kakigi, Ryusuke; Pantev, Christo

    2014-01-01

    Sudden sensorineural hearing loss is characterized by acute, idiopathic hearing deterioration. We report here the development and evaluation of “constraint-induced sound therapy”, which is based on a well-established neuro-rehabilitation approach, and which is characterized by the plugging of the intact ear (“constraint”) and the simultaneous, extensive stimulation of the affected ear with music. The sudden sensorineural hearing loss patients who received the constraint-induced sound therapy in addition to the standard corticosteroid therapy showed significantly better recovery of hearing function compared to those who had only received corticosteroid treatments. Additionally, the brain activity obtained in a subgroup of patients suggested that the constraint-induced sound therapy could have prevented maladaptive auditory cortex reorganization. Constraint-induced sound therapy thus appears to be an effective, practical, and safe treatment option for sudden sensorineural hearing loss. PMID:24473277

  18. Management of sudden sensorineural hearing loss.

    Science.gov (United States)

    Metrailer, Aaron M; Babu, Seilesh C

    2016-10-01

    Sudden sensorineural hearing loss (SNHL) is an otologic emergency and should be managed quickly and effectively. This review focuses on the management of sudden SNHL, primarily idiopathic sudden SNHL as it is the most common cause. Management options include observation, oral steroids, intratympanic steroids, or combined oral/intratympanic steroids. One-third to two-thirds of patients will achieve spontaneous recovery, most likely within the first 2 weeks. Despite the lack of randomized controlled trials on steroid therapy efficacy, all patients should be offered steroid treatment given low risk and possible significant benefits. All patients should undergo MRI with gadolinium to rule out retrocochlear disorder. Bilateral sudden hearing loss should alert the clinician to possible systemic disease. Sudden hearing loss is an otologic emergency. Appropriate counseling of patients is necessary to allow physician and patient to make a joint, educated decision. It is paramount to rule out retrocochlear disorder and to follow patients closely for improvement or need for future auditory rehabilitation.

  19. Relation between Glaucoma and Sensorineural Hearing Loss

    Directory of Open Access Journals (Sweden)

    A Mollasadeghi

    2008-01-01

    Full Text Available Introduction: Glaucoma is one of the leading causes of blindness throughout the world. Some studies have suggested a relationship between glaucoma and sensorineural hearing loss, while others have found no evidence of an association. We performed a study to determine whether there is a significant difference in hearing of patients with glaucoma and a match control population. Methods: In this cross-sectional study, from February, 2005 till April, 2006, 44 patients with glaucoma were studied. The age range was between 15 to 60 years. After taking a complete medical history, those suffering from presbycusis, history of exposure to ototoxic drugs and substances and history of ear surgery were excluded from the study. All of the patients were cases of open-angle glaucoma, and were surveyed separately for normal-pressure glaucoma. Then complete audiometric tests (PTA, SDS, SRT, Impedance were conducted for all of them, and the results compared with a control group. Results: There was no statistically significant difference between the case group and control group in PTA, SDS, and SRT, except for Normal Tension Glaucoma (NTG. There wasn't any statistically significant difference between two groups with respect to age, gender, and history of diseases. In the NTG group, significant difference was seen only in high frequencies. Conclusion: As mentioned, there was a statistically significant difference between NTG group and control group. It is therefore recommended to conduct complete audiometric tests and histopathologic examinations in this group for early detection of hearing loss and application of rehabilitative measures.

  20. Sensorineural and conductive hearing loss in infants diagnosed in the program of universal newborn hearing screening.

    Science.gov (United States)

    Wroblewska-Seniuk, Katarzyna; Dabrowski, Piotr; Greczka, Grazyna; Szabatowska, Katarzyna; Glowacka, Agata; Szyfter, Witold; Mazela, Jan

    2018-02-01

    The aim of this study was to analyze infants diagnosed with sensorineural or conductive hearing deficit and to identify risk factors associated with these defects. A retrospective analysis of infants diagnosed with hearing deficit based on the database of the universal newborn hearing screening program and medical records of the patients. 27 935 infants were covered by the universal neonatal hearing screening program. 109 (0.39%) were diagnosed with hearing deficit and referred for treatment and rehabilitation. 56 (51.4%) children were diagnosed with conductive, 38 (34.9%) with sensorineural and 15 (13.8%) with mixed type of hearing deficit. Children with sensorineural hearing deficit more frequently suffered from hyperbilirubinemia (p conductive hearing loss were more frequently diagnosed with isolated craniofacial anomalies (p hearing deficit occurred almost 3 times more often bilaterally than unilaterally (p hearing deficit, the difference was not significant. In children with conductive and mixed type of hearing loss the impairment was mainly mild while among those with sensorineural hearing deficit in almost 45% it was severe and profound (p hearing screening test by means of otoacoustic emissions and the final diagnosis of hearing deficit we found that the highest agreement rate was observed in children with sensorineural hearing loss (p hearing deficit was similar in children with sensorineural, conductive and mixed type of hearing loss, only hyperbilirubinemia seemed to predispose to sensorineural hearing deficit and isolated craniofacial malformations seemed to be associated with conductive hearing loss. Sensorineural hearing deficit usually occurred bilaterally and was severe or profound, while conductive and mixed type of hearing deficit were most often of mild degree. Most children with the final diagnosis of sensorineural hearing deficit had positive result of hearing screening by means of otoacoustic emissions. Copyright © 2017 Elsevier B.V. All

  1. Sensorineural Hearing Loss and Celiac Disease: A Coincidental Finding

    Directory of Open Access Journals (Sweden)

    Umberto Volta

    2009-01-01

    Full Text Available BACKGROUND: Celiac disease (CD can be associated with a variety of extraintestinal manifestations, including neurological diseases. A new neurological correlation has been found between CD and sensorineural hearing loss (SNHL.

  2. Sensorineural hearing loss in patients with Kawasaki disease.

    Science.gov (United States)

    Park, Sun Young; Kim, Young Hyun; Kim, Yeo Hyang; Hyun, Myung Chul; Lee, Young Hwan

    2015-11-01

    Kawasaki disease involves acute febrile systemic vasculitis that can cause a variety of symptoms by affecting various organs. Here, we aimed to evaluate the prevalence, causes, and prognosis of sensorineural hearing loss (SNHL) occurring in children with Kawasaki disease. Patients who were diagnosed with Kawasaki disease and received inpatient treatment in the Pediatrics Department at one of three university hospitals in Daegu city from February 2012 to September 2012 were enrolled in the study. The clinical features, hematological results, echocardiography results, audiometry results, and aspirin and salicylic acid serum levels of the patients were evaluated. Of the 59 children enrolled in the study, three showed mild bilateral SNHL on audiometry tests conducted after 48 hours of defervescence; these patients demonstrated normal patterns of recovery on follow-up tests 8 weeks later. Aspirin serum levels were significantly higher in the SNHL group after 48 hours of afebrile condition with high dose aspirin intake (P=0.034). However, no significant differences were found in other laboratory tests or for fever duration (P>0.05). Upon echocardiography, coronary artery abnormality was observed in 9 cases, but none of these patients showed hearing loss. The results indicate that SNHL in children with Kawasaki disease might occur during treatment of the acute phase; this SNHL usually involves mild bilateral hearing loss and recovers naturally. However, this study suggests that determination of the causes and clinical implications of hearing loss in Kawasaki disease requires long-term follow-up studies with more cases.

  3. Disacusia neurossensorial imunomediada Immunomediated sensorineural hearing loss

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    Norma de Oliveira Penido

    2002-10-01

    Full Text Available A disacusia neurossensorial imunomediada (DNSI é caracterizada geralmente por uma disacusia neurossensorial bilateral, progressiva e assimétrica, acompanhada ou não por outros sintomas da orelha interna. Três pacientes com DNSI cujo quadro clínico e audiométrico eram sugestivos de doença auto-imune, e apresentaram resposta positiva à terapia imunossupressora ou pesquisa positiva de anticorpo anti hsp-70 68kD, foram estudados com relação às características clínicas, testes diagnósticos, alternativas terapêuticas e evolução da doença. Dois pacientes apresentaram quadro de disacusia neurossensorial rapidamente progressiva, associado a quadro vestibular, e outro, quadro de surdez súbita unilateral. Nenhum paciente apresentou positividade às provas reumatológicas, e apenas um paciente apresentou aumento na velocidade de hemossedimentação. Nenhum paciente obteve resposta adequada sustentada à corticoterapia, mas dois deles melhoraram com outras terapias imunossupressoras. O diagnóstico da DNSI é clínico e baseado na resposta positiva ao teste terapêutico com imunossupressores. A pesquisa de anticorpo anti-hsp70 de 68 kD pelo Western Blot é o único exame laboratorial específico para seu diagnóstico, possuindo sensibilidade de 42% e especificidade de 90%. Apenas 1 paciente apresentou positividade para este teste e não respondeu à terapia imunossupressora. Os dois pacientes com teste negativo responderam satisfatoriamente ao tratamento. A baixa sensibilidade do Western Blot e seu alto custo dificultam sua difusa utilização em nosso meio. A introdução precoce do tratamento é de suma importância por auxiliar no diagnóstico e por proporcionar um melhor prognóstico auditivo.The immunomediated sensorineural hearing loss (ISHL is characterized as an asymmetric and progressive sensorineural hearing loss. Tree patients with ISHL were studied, regarding clinical aspects, diagnostic tests, treatment options and disease

  4. Genetic and audiologic study in elderly with sensorineural hearing loss.

    Science.gov (United States)

    Martins, Kelly; Fontenele, Marília; Câmara, Silva; Sartorato, Edi Lúcia

    2013-01-01

    This study aimed to correlate probable predisposing factors for sensorineural hearing loss in elderly by investigating the audiologic characteristics and frequency of mutations in genes considered responsible for non-syndromic hearing loss. Sixty elderly patients were separated into two groups: the Case Group, composed of 30 individuals, 21 females and nine males, all 60 years old or older and presenting diagnoses of sensorineural hearing loss, and the Control Group, composed of 30 elderly individuals matched to the experimental group by age and gender, presenting normal hearing. The patients underwent anamnesis and pure tone audiometry in frequencies of 250, 500, 1000, 2000, 3000, 4000 and 6000 Hz. Blood samples were collected from each patient for analysis of mutations in nuclear and mitochondrial genes related to non-syndromic sensorineural hearing loss. It was observed a greater tendency to noise exposure and consumption of alcohol in the Case Group. The statistically significant symptoms between the groups were tinnitus and hearing difficulty in several situations as: silent environment, telephone, television, sound location and in church. All the individuals of Case Group presented sensorineural and bilateral hearing loss. The symmetry and progression of the hearing impairment were also statistically significant between the groups. No genetic mutations were identified. The most reported symptoms were communication difficulties and tinnitus. The predominant auditory characteristics included sensorineural, bilateral, progressive and symmetrical hearing loss. It was not evidenced a relationship between sensorineural hearing loss in elderly and genes considered responsible for non-syndromic hearing loss as no genetic mutation was found in this study.

  5. Hyperbaric oxygen therapy for sudden sensorineural hearing loss in large vestibular aqueduct syndrome.

    Science.gov (United States)

    Shilton, H; Hodgson, M; Burgess, G

    2014-01-01

    We report the first use in Australia of hyperbaric oxygen therapy for sudden hearing loss following head trauma in a child with large vestibular aqueduct syndrome. A 12-year-old boy with large vestibular aqueduct syndrome presented with significant hearing loss following head trauma. He was treated with steroids and hyperbaric oxygen therapy, with good improvement of hearing thresholds on audiography. This case represents the first reported use of hyperbaric oxygen therapy for this indication in Australia, following a few previous reports of patients in Japan. We review the literature on management of acute sensorineural hearing loss in large vestibular aqueduct syndrome. The reported case demonstrates a potentially beneficial therapy for a rare condition that usually results in an inevitable decline in hearing. Hyperbaric oxygen therapy can be tolerated well by children, and may represent a potential treatment for sudden sensorineural hearing loss in patients with large vestibular aqueduct syndrome.

  6. Weather conditions and sudden sensorineural hearing loss

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    Kateri Maria

    2002-07-01

    Full Text Available Abstract Background Climatic or meteorological condition changes have been implicated in the pathogenesis of Idiopathic Sudden Sensorineural Hearing Loss (ISSHL. We investigated the seasonal distribution of ISSHL and evaluated the influence of meteorological parameters (such as temperature, humidity, and atmospheric pressure, their variation and covariation on the incidence of the disease. Methods A total of 82 cases of ISSHL, admitted to our department over a five-year period, were enrolled in the study. Seasonal distribution of the disease was investigated by dividing the year in four seasons. Meteorological data included daily values of 13 distinct parameters recorded at the meteorological station of the University of Ioannina during this period. A relationship between each meteorological variable and the incidence of ISSHL was investigated by applying (χ2 test on data from 13 contingency tables as well as by using logistic regression and t-test approaches. In addition, the influence of different weather types on the incidence of ISSHL was investigated using Cluster Analysis in order to create eight clusters (weather types characteristic for the prefecture of Ioannina. Results The results of the study could not indicate any seasonal distribution of the disease. The incidence of ISSHL could not be significantly correlated either to any distinct meteorological parameter or to any specific weather type. Conclusions Meteorological conditions, such as those dominating in the Northwestern Greece, and/or their changes, have no proven effect on the incidence of ISSHL.

  7. Resolution of Sudden Sensorineural Hearing Loss Following a Roller Coaster Ride

    OpenAIRE

    Kumar, Aman; Sinha, Amrita; Al-Waa, Ahmad M.

    2011-01-01

    We report a case of sudden unilateral sensorineural hearing loss of sudden onset during an aeroplane flight, which completely resolved during a roller coaster ride at Alton Towers theme park. A review of the literature concerning sudden idiopathic sensorineural hearing loss and spontaneous resolution are discussed. Initially, pure-tone audiometry showed a profound sensorineural hearing loss in the right ear and mild sensorineural hearing loss in the left ear (of note, the hearing was normal p...

  8. Idiopathic sudden sensorineural hearing loss: vascular or viral?

    Science.gov (United States)

    Linthicum, Fred H; Doherty, Joni; Berliner, Karen I

    2013-12-01

    To demonstrate that sudden sensorineural hearing loss is possibly of viral origin rather than vascular. The histopathologic morphology in 7 temporal bones with known vascular impairment due to surgical interventions was compared with that of 11 bones with a history of idiopathic sudden sensorineural hearing loss (ISSNHL). Attention was paid to the spiral ligament, stria vascularis, organ of Corti hair cells, tectorial membrane, ganglion cell population, and degree of perilymph fibrosis and the auditory nerve. A temporal bone laboratory that has been in operation for more than 50 years and includes a database consisting of clinical and histopathological information that facilitates quantitative and qualitative analysis. Eight hundred forty-nine individuals who pledged their temporal bones for scientific study, of which 18 were selected for this study by means of the database criteria of sudden sensorineural hearing loss and postmiddle fossa and retro sigmoid sinus tumor removal or vestibular nerve section. Sudden sensorineural hearing loss bones exhibited no perilymph fibrosis compared with 6 of 7 vascular cases with fibrosis (P ≤ .001), exhibited less loss of ganglion cells (P ≤ .026), exhibited greater survival of spiral ligament (P ≤ .029), and averaged twice the survival of hair cells and more widespread tectorial membrane abnormalities. Analysis of human temporal bones from patients with a sudden sensorineural hearing loss does not support a vascular insufficiency but is more suggestive of a viral etiology.

  9. Sensorineural Hearing Loss due to Air Bag Deployment

    Directory of Open Access Journals (Sweden)

    Masafumi Ohki

    2012-01-01

    Full Text Available Deployment of the air bag in a passenger vehicle accident rarely causes otologic injuries. However, sensorineural hearing loss induced by air bag deployment is extremely rare, with only a few cases reported in the English literature. A 38-year-old man involved in a traffic accident while driving his car at 40 km/hour presented with right sensorineural hearing loss and tinnitus, without associated vertigo. Pure-tone audiometry demonstrated elevated thresholds of 30 dB and 25 dB at 4 kHz and 8 kHz, respectively, on the right side. Air bag deployment in car accidents is associated with the risk of development of sensorineural hearing loss.

  10. [Prognostic factors of sudden sensorineural hearing loss in children].

    Science.gov (United States)

    Li, Fengjiao; Xue, Xijun; Wang, Li; Yang, Fengbo; Wang, Hongyang; Guan, Jing; Du, Wan; Xiong, Wenping; Wu, Kaiwen; Wu, Mukun; Yin, Zifang; Lan, Lan; Wang, Dayong; Wang, Qiuju

    2015-11-01

    The aim of this retrospective study was to analyze the recovery rate of sudden sensorineural hearing loss in children, and explore the prognostic factors in order to guide the clinical diagnosis and treatment. A retrospective review was conducted for the prognosis of children with sudden sensorineural hearing loss during the past 5 years (from November 2010 to May 2015) in Chinese PLA General Hospital. This paper have a complete clinical data of 101 patients (113 ears)with sudden hearing loss, ranging from 0 to 18 years old Patients were divided into four groups according to hearing recovery and eight putative prognostic factors were analyzed. Among 101 patients (113 ears), the ratio of male and female was 60:53. Treatment was initiated from 1 to 183 days after disease onset, with an average of (18.5 ± 22.1) d. Bilateral and unilateral hearing loss were 24 ears and 89 ears, respectively. The proportion of mild hearing loss, moderate hearing loss, severe hearing loss and profound hearing loss were 7.1%, 6.2%, 23.9% and 62.8%, respectively. Vertigo and tinnitus occurred in 54.9% and 77.9% of the patients, respectively. After the treatment, the complete recovery rate was 9.7% and the overall recovery rate was 36.3%. The degree of hearing loss, earlier treatment onset, sex and bilateral involvement were significantly associated with hearing recovery (P Sudden sensorineural hearing loss in children was generally identified as severe and profound hearing loss, but after positive and timely treatment, it can be improved or even cured. The mild hearing loss, earlier treatment onset, unilateral hearing loss and female were positive prognostic factors. The concurrence of tinnitus or vertigo, the results of ABR and DPOAE had no significant influence on prognosis.

  11. Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss

    Science.gov (United States)

    Sekiya, Kenichi; Fukushima, Munehisa; Teismann, Henning; Lagemann, Lothar; Kakigi, Ryusuke; Pantev, Christo; Okamoto, Hidehiko

    2016-01-01

    Sudden sensorineural hearing loss (SSHL) is characterized by acute, idiopathic hearing loss. The estimated incidence rate is 5-30 cases per 100,000 people per year. The causes of SSHL and the mechanisms underlying SSHL currently remain unknown. Based on several hypotheses such as a circulatory disturbance to the cochlea, viral infection, and autoimmune disease, pharmaco-therapeutic approaches have been applied to treat SSHL patients; however, the efficacy of the standard treatment, corticosteroid therapy, is still under debate. Exposure to intense sounds has been shown to cause permanent damage to the auditory system; however, exposure to a moderate level enriched acoustic environment after noise trauma may reduce hearing impairments. Several neuroimaging studies recently suggested that the onset of SSHL induced maladaptive cortical reorganization in the human auditory cortex, and that the degree of cortical reorganization in the acute SSHL phase negatively correlated with the recovery rate from hearing loss. This article reports the development of a novel neuro-rehabilitation approach for SSHL, "constraint-induced sound therapy (CIST)". The aim of the CIST protocol is to prevent or reduce maladaptive cortical reorganization by using an enriched acoustic environment. The canal of the intact ear of SSHL patients is plugged in order to motivate them to actively use the affected ear and thereby prevent progress of maladaptive cortical reorganization. The affected ear is also exposed to music via a headphone for 6 hr per day during hospitalization. The CIST protocol appears to be a safe, easy, inexpensive, and effective treatment for SSHL. PMID:26863274

  12. Pediatric unilateral sensorineural hearing loss: implications and management.

    Science.gov (United States)

    Dornhoffer, James R; Dornhoffer, John L

    2016-12-01

    The purpose of this review is to summarize current studies detailing the impact of unilateral sensorineural hearing loss in children and the most current modalities of treatment used in its management. Current studies corroborate historic views on the impact of unilateral sensorineural hearing loss on patient wellbeing and academic success and stress the use of additional surveillance and studies to diagnose those patients that may pass standard screening practices and suffer from lack of prompt and proper care. With respect to management, notable findings include the continuous development of improved conventional and contralateral routing of signal amplification devices that may act to provide alternatives to percutaneous bone-anchored hearing aid implantation. These include improvements in more conventional hearing aid technology, so as to bridge the performance gap with the classical bone-anchored hearing aid implant, and the development of partially implanted transcutaneous bone conduction hearing devices. Due to dissatisfaction with sound localization, a new and significant development is the burgeoning accumulation of research on cochlear implantation for the treatment of unilateral sensorineural hearing loss in children. With advances in technology in historic modalities of treatment, and the advent of new modalities such as cochlear implantation, the clinician has a wide armamentarium by which to provide treatment to patients based on clinical circumstances and patient desires.

  13. Sudden sensorineural hearing loss after non-otologic surgery.

    Science.gov (United States)

    Page, Joshua Cody; Peters, Bob

    2015-01-01

    Sudden sensorineural hearing loss following non-otologic surgery is a rare event described in the medical literature. Cardiopulmonary bypass surgery is most commonly associated with this type of hearing loss. Our case report and review of the literature describe two cases with postoperative hearing loss - neither of which are cardiac surgeries - making them exceedingly rare in the medical literature. Regardless of the rarity of this unfortunate event, the possibility for permanent hearing loss is a potentially devastating unanticipated complication and one that all surgeons should be aware. Copyright © 2015 Elsevier Inc. All rights reserved.

  14. Steroid Use in Idiopathic Sudden Sensorineural Hearing Loss: What ...

    African Journals Online (AJOL)

    Idiopathic sudden sensorineural hearing loss is a disease of unknown etiology. Controversy in the literature argues whether the condition should be treated by steroid therapy. In this case study, a Medline literature search was completed to find out if there is any evidence to support its use in this condition.

  15. P300 in individuals with sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Ana Cláudia Mirandola Barbosa Reis

    2015-04-01

    Full Text Available INTRODUCTION: Behavioral and electrophysiological auditory evaluations contribute to the understanding of the auditory system and of the process of intervention.OBJECTIVE: To study P300 in subjects with severe or profound sensorineural hearing loss.METHODS: This was a descriptive cross-sectional prospective study. It included 29 individuals of both genders with severe or profound sensorineural hearing loss without other type of disorders, aged 11 to 42 years; all were assessed by behavioral audiological evaluation and auditory evoked potentials.RESULTS: A recording of the P3 wave was obtained in 17 individuals, with a mean latency of 326.97 ms and mean amplitude of 3.76 V. There were significant differences in latency in relation to age and in amplitude according to degree of hearing loss. There was a statistically significant association of the P300 results with the degrees of hearing loss (p = 0.04, with the predominant auditory communication channels (p < 0.0001, and with time of hearing loss.CONCLUSIONS: P300 can be recorded in individuals with severe and profound congenital sensorineural hearing loss; it may contribute to the understanding of cortical development and is a good predictor of the early intervention outcome.

  16. Bilateral sudden sensorineural hearing loss as a first symptom of infective endocarditis: two case reports.

    Science.gov (United States)

    Chroni, M; Prappa, E; Kokkevi, I

    2018-02-21

    Septic emboli are an unusual cause of sudden sensorineural hearing loss, for which few reports exist in the literature. This paper presents two cases of sudden sensorineural hearing loss, initially considered as idiopathic, but which were caused by septic emboli. Hearing loss in these cases was bilateral, sequential and total. The first patient had mild fever one week prior to their presentation with sudden sensorineural hearing loss; the other patient had no additional symptoms at presentation. These patients were later diagnosed with infective endocarditis, at two and seven months following the sudden sensorineural hearing loss respectively, showing that septic emboli had been the cause of sudden sensorineural hearing loss. Septic emboli should be considered as a possible cause of sudden sensorineural hearing loss in cases of total hearing loss. This form of hearing loss should prompt the otolaryngologist to further investigate for infective endocarditis.

  17. Isolated Sensorineural Hearing Loss as a Sequela after Lightning Strike

    Directory of Open Access Journals (Sweden)

    Mahfuz Turan

    2015-01-01

    Full Text Available In most of the surviving patients after a lightning strike, audiovestibular abnormalities have been reported. The most frequently reported type of abnormalities is a tympanic membrane perforation with hearing loss and external ear canal burn. However a sensor neural hearing loss and mixed type hearing loss can also occur, but these occur rarely. A nineteen-year-old female patient had, after a lightning strike, serious burns on the left ear, behind the ear, and on the chest and neck. She also had in her left ear 108 dB hearing loss with irregular central perforation and in her right ear 52 dB sensorineural hearing loss. There was no hearing loss before the strike. A hearing aid was recommended for the right ear and good care and follow-up were recommended for the left ear. A lightning strike can cause serious audiological damage. Therefore, it is necessary to make a careful audiovestibular evaluation of the patients. Although there exist rarely healed cases from sensorineural hearing loss after lightning strike in literature, in our case hearing loss occurred bilaterally and then it healed unilaterally. This condition is quite rare in literature.

  18. Adjunctive hyperbaric oxygen treatment for idiopathic sudden sensorineural hearing loss.

    Science.gov (United States)

    Chin, Chun-Shih; Lee, Tsai-Yun; Wu, Ming-Feng

    2017-01-01

    This study aims to evaluate the hearing gain efficacy from adjunctive hyperbaric oxygen (HBO₂) treatment in patients with idiopathic sudden sensorineural hearing loss (ISSHL). A retrospective analysis of chart reviews was performed on patients with ISSHL between January 2013 through December 2015. All patients were referred to us from our ENT Department for adjunctive hyperbaric oxygen treatment. The results were assessed through pure-tone audiometry (PTA) data change (hearing gain), both before and after HBO₂) treatment. Age, gender, affected ear side, HBO₂) treatment sessions, both before and after HBO₂ treatment PTA were all recorded. Ninety-three (93) patients with ISSHL were included in the study. The average hearing gain in this study was 17.9 dB (p=0.001), where a total of 46 (49.46%) patients showed an improvement (hearing gain≥dB) in response to HBO₂ treatment (p=0.002). Patients with the poorest initial severity of hearing loss who displayed a greater degree of hearing improvement after HBO₂ treatment were male and in the 40- to 59-year-old age group. This study found that adjunctive hyperbaric oxygen treatment was efficacious for patients with idiopathic sudden sensorineural hearing loss. The total average hearing gain was recorded to be 17.9 dB.

  19. Pediatric Idiopathic Intracranial Hypertension Presenting With Sensorineural Hearing Loss.

    Science.gov (United States)

    Reitsma, Sietze; Stokroos, Robert; Weber, Jacobiene W; van Tongeren, Joost

    2015-12-01

    To present the rare case of a young boy with idiopathic intracranial hypertension presenting with bilateral sensorineural hearing loss developing over several months. This was accompanied by headaches, otalgia, tinnitus, and vertigo. Furthermore, we aim to provide a concise review on this matter, as this report represents the second case in literature of pediatric idiopathic intracranial hypertension presenting with hearing loss. Workup of a 9-year-old boy with bilateral sensorineural hearing loss, including (among others) physical examination, audiometry, diagnostic imaging, and lumbar puncture. Physical examination including fundoscopy as well as imaging showed no abnormalities. At presentation, pure tone audiometry revealed bone conduction thresholds of about 30 dB HL in both ears. Two months later, this declined to about 35 dB HL in both ears. Lumbar puncture revealed an increased intracranial pressure. The boy was thus diagnosed with idiopathic intracranial hypertension. After the lumbar puncture, the otological complaints gradually resolved, and the hearing normalized (bone conduction thresholds of 0-5 dB HL). Although rare, sensorineural hearing loss in the pediatric population together with otalgia, tinnitus, and vertigo can be due to idiopathic intracranial hypertension and as such can be reversible. © The Author(s) 2015.

  20. Radiological quiz. Sudden sensorineural hearing loss due to multiple sclerosis.

    Science.gov (United States)

    Cabbarzade, Cavid; Özgen, Burçe; Sennaroglu, Levent

    2014-01-01

    A case with sudden sensorineural hearing loss (SSNHL) owing to multiple sclerosis (MS) who had clinical and dramatic radiological improvement just after medical therapy was reported in this article. Case report and review of related literature. A 22-year-old female patient with MS related SSNHL was presented in this article. Magnetic resonance imaging (MRI) revealed an MS plaque localized at pons extending from right cochlear nucleus to proximal part of the right cochlear nerve. Most dramatic recovery was present in the 5th day control MRI, where the plaque located on pons disappeared completely. On the 10th day control audiogram hearing recovery was observed and pure tone audiogram levels were almost normal. Sudden sensorineural hearing loss owing to MS is seen more common than expected. It has good prognosis. Magnetic resonance imaging is also thought to have an important role in diagnosis and treatment efficacy of SSNHL owing to MS. Published by Elsevier Urban & Partner Sp. z.o.o.

  1. Sudden sensorineural hearing loss as the first manifestation of chronic myeloid leukaemia: case report.

    Science.gov (United States)

    Diao, M; Tian, F; Sun, J

    2014-11-01

    Sudden sensorineural hearing loss rarely occurs in patients with chronic myeloid leukaemia. We present a case report of a patient who presented with sudden sensorineural hearing loss as the first manifestation of chronic myeloid leukaemia, and review the mechanisms responsible for sudden sensorineural hearing loss in leukaemic patients. A 31-year-old female presented to our clinic with unilateral sudden sensorineural hearing loss and tinnitus. Pure tone audiometry revealed profound sensorineural hearing loss in the left ear at all frequencies. During an investigation into her hearing loss, the patient was found to have chronic myeloid leukaemia. Every case of sudden sensorineural hearing loss must be carefully evaluated, and haematological disorders must be considered in the differential diagnosis of sudden hearing loss.

  2. The relationship between neonatal hyperbilirubinemia and sensorineural hearing loss.

    Science.gov (United States)

    Corujo-Santana, Cándido; Falcón-González, Juan Carlos; Borkoski-Barreiro, Silvia Andrea; Pérez-Plasencia, Daniel; Ramos-Macías, Ángel

    2015-01-01

    Severe jaundice that requires exchange transfusion has become a relatively rare situation today. About 60% of full term neonates and 80% of premature ones will suffer from jaundice within the first week of life. Hyperbilirubinemia at birth is a risk factor associated with hearing loss that is usually further linked to other factors that might have an effect on hearing synergistically. This study aimed to identify the relationship between hyperbilirubinemia at birth as a risk factor for sensorineural hearing loss in children born at Complejo Hospitalario Universitario Insular Materno-Infantil de Gran Canaria, in the 2007-2011 period. This was a retrospective study of 796 newborns that had hyperbilirubinemia at birth, using transient evoked otoacoustic emissions and evoked auditory brainstem response. Hundred eighty-five newborns (23.24%) were referred for evoked auditory brainstem response. Hearing loss was diagnosed for 35 (4.39%): 18 neonates (51.43%) with conductive hearing loss and 17 (48.57%) with sensorineural hearing loss, 3 of which were diagnosed as bilateral profound hearing loss. Half of the children had other risk factors associated, the most frequent being exposure to ototoxic medications. The percentage of children diagnosed with sensorineural hearing loss that suffered hyperbilirubinemia at birth is higher than for the general population. Of those diagnosed, none had levels of indirect bilirubin≥20mg/dl, only 47% had hyperbilirubinemia at birth as a risk factor and 53% had another auditory risk factor associated. Copyright © 2014 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

  3. [Subclinical sensorineural hearing loss in female patients with rheumatoid arthritis].

    Science.gov (United States)

    Treviño-González, José Luis; Villegas-González, Mario Jesús; Muñoz-Maldonado, Gerardo Enrique; Montero-Cantu, Carlos Alberto; Nava-Zavala, Arnulfo Hernán; Garza-Elizondo, Mario Alberto

    2015-01-01

    The rheumatoid arthritis is a clinical entity capable to cause hearing impairment that can be diagnosed promptly with high frequencies audiometry. To detect subclinical sensorineural hearing loss in patients with rheumatoid arthritis. Cross-sectional study on patients with rheumatoid arthritis performing high frequency audiometry 125Hz to 16,000Hz and tympanometry. The results were correlated with markers of disease activity and response to therapy. High frequency audiometry was performed in 117 female patients aged from 19 to 65 years. Sensorineural hearing loss was observed at a sensitivity of pure tones from 125 to 8,000 Hz in 43.59%, a tone threshold of 10,000 to 16,000Hz in 94.02% patients in the right ear and in 95.73% in the left ear. Hearing was normal in 8 (6.84%) patients. Hearing loss was observed in 109 (93.16%), and was asymmetric in 36 (30.77%), symmetric in 73 (62.37%), bilateral in 107 (91.45%), unilateral in 2 (1.71%), and no conduction and/or mixed hearing loss was encountered. Eight (6.83%) patients presented vertigo, 24 (20.51%) tinnitus. Tympanogram type A presented in 88.90% in the right ear and 91.46% in the left ear, with 5.98 to 10.25% type As. Stapedius reflex was present in 75.3 to 85.2%. Speech discrimination in the left ear was significantly different (p = 0.02)in the group older than 50 years. No association was found regarding markers of disease activity, but there was an association with the onset of rheumatoid arthritis disease. Patients with rheumatoid arthritis had a high prevalence of sensorineural hearing loss for high and very high frequencies. Copyright © 2015 Academia Mexicana de Cirugía A.C. Published by Masson Doyma México S.A. All rights reserved.

  4. Sensorineural hearing loss in insulin-dependent diabetic patients

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    Koosha A.

    2007-11-01

    Full Text Available Background: Among patients who have sensorineural hearing loss of unknown etiology, diabetes is one of the diseases to be routinely investigated. The relationship between diabetes mellitus and hearing loss is still controversial. The purpose of this study was to examine the prevalence of sensorineural hearing loss in patients with insulin-dependent diabetes mellitus (IDDM compared to control group.Methods: In a cross-sectional study pure tone audiometry (PTA and speech audiometry was performed in 62 patients with insulin-dependent diabetes mellitus (IDDM, aged under 40 years, and in 62 randomly selected age-matched non-diabetic control subjects. Subjects with otological and other metabolic diseases were excluded from the study. We applied the SPSS.10 statistical analysis software Chi-square and student's test. Results: Statistical analysis showed that the hearing of the diabetic patients were significantly worsen than the control subjects. The hearing level tended to be worsen in the diabetic patients than that in control subjects, but the differences were statistically significant only at frequencies of 250,500, 4000 and 8000 Hz p>0.05(. There wasn't statistical significant difference between sex in two study groups p>0.05(. The mean duration of diabetes was no statistically significant with hearing loss p>0.05(. The frequency of complications such as retinopathy, nephropathy, and neuropathy in the diabetic groups had no correlation with speech threshold (p>0.05(.‏ There were no significant differences between speech reception threshold, speech discrimination score and acoustic reflex in two groups.Conclusions: We conclude that type I diabetes mellitus can cause sensorineural hearing loss.

  5. Sudden Bilateral Sensorineural Hearing Loss Secondary to Cerebral Venous Thrombosis

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    Muhammad-Nu’aim Ishak

    2018-03-01

    Full Text Available Introduction: Sudden sensorineural hearing loss (SSNHL is an important otological emergency. Up to 90% of the cases are idiopathic. Cerebral venous thrombosis (CVT is an extremely rare identifiable cause as it only represents 0.5% of all strokes.   Case Report: In this paper, an unusual case of bilateral SSNHL secondary to bilateral CVT with rapid and complete recovery is reported. The patient presented with sudden bilateral hearing loss associated with some neurological symptoms. Initial computed tomography (CT venography revealed a CVT of bilateral transverse sinuses. The patient was started on an anticoagulant and imaging was repeated after five days, revealing the absence of the thrombosis. Serial pure tone audiometry (PTA showed complete recovery of bilateral hearing within 10 days.   Conclusion: Early detection and intervention may fasten hearing recovery and improve the quality of life. The immediate restoration of venous blood flow and intracranial pressure may lead to the complete recovery of bilateral hearing loss.

  6. Sudden onset unilateral sensorineural hearing loss after rabies vaccination.

    Science.gov (United States)

    Okhovat, Saleh; Fox, Richard; Magill, Jennifer; Narula, Antony

    2015-12-15

    A 33-year-old man developed profound sudden onset right-sided hearing loss with tinnitus and vertigo, within 24 h of pretravel rabies vaccination. There was no history of upper respiratory tract infection, systemic illness, ototoxic medication or trauma, and normal otoscopic examination. Pure tone audiograms (PTA) demonstrated right-sided sensorineural hearing loss (thresholds 90-100 dB) and normal left-sided hearing. MRI internal acoustic meatus, viral serology (hepatitis B, C, HIV and cytomegalovirus) and syphilis screen were normal. Positive Epstein-Barr virus IgG, viral capsid IgG and anticochlear antibodies (anti-HSP-70) were noted. Initial treatment involved a course of high-dose oral prednisolone and acyclovir. Repeat PTAs after 12 days of treatment showed a small improvement in hearing thresholds. Salvage intratympanic steroid injections were attempted but failed to improve hearing further. Sudden onset sensorineural hearing loss (SSNHL) is an uncommon but frightening experience for patients. This is the first report of SSNHL following rabies immunisation in an adult. 2015 BMJ Publishing Group Ltd.

  7. Sudden Sensorineural Hearing Loss in the Only Hearing Ear: Large Vestibular Aqueduct Syndrome

    Directory of Open Access Journals (Sweden)

    Kemal Koray Bal

    2016-01-01

    Full Text Available Sudden hearing loss in the only hearing ear cases are rarely published in the English literature; most of the cases are idiopathic. It is an otologic emergency needing urgent treatment. Delayed diagnosis can interfere with patient’s social life with interrupting the verbal communication. In this case report we presented a 33-year-old female patient having sudden sensorineural hearing loss in the only hearing ear diagnosed as bilateral large vestibular aqueduct syndrome.

  8. A Clinical Analysis of Sudden Sensorineural Hearing Loss Cases

    Science.gov (United States)

    Lee, Hyun Soo; Lee, You Jae; Kang, Bo Sung; Lee, Ji Sung

    2014-01-01

    Background and Objectives High-dose systemic steroid therapy is the mainstay treatment for sudden sensorineural hearing loss (SSNHL). Recovery rates from SSNHL range are about 47-63% and are influenced by various prognostic factors. To evaluate the prognostic value of specific clinical parameters, we reviewed 289 cases by clinical and statistical analysis. Subjects and Methods This study included 289 patients with SSNHL who visited the Department of Otolaryngology at Soonchunhyang University Hospital from January 2005 to December 2012. The cases were reviewed retrospectively based on clinical charts. Hearing improvement was evaluated in relation to pure-tone audiogram results, duration between SSNHL onset and time of initial treatment, seasonal incidence, dizziness, patient age, degree of hearing loss, patterns of initial pure-tone audiogram and presence of underlying disease. Results Hearing improvement was observed in 196 of 289 (67.8%) patients; such improvement began within 7 days in most patients, followed by rapid hearing recovery. Cases that failed to show improvement within 14 days were unlikely to achieve hearing recovery. The more severe the hearing loss during the early stage, the lower the hearing recovery rates. Patients aged less than 60 years appear to have better prognosis of hearing improvement compared to those who are over 60 years. Conclusions Important prognostic factors for recovery in patients with SSNHL include the time of initiating treatment after symptom onset, the degree of early-stage hearing loss, and the age of the affected patient. PMID:25279228

  9. Identification of evidence for autoimmune pathology of bilateral sudden sensorineural hearing loss using proteomic analysis.

    Science.gov (United States)

    Lee, Jeon Mi; Kim, Jin Young; Bok, Jinwoong; Kim, Kyu-Sung; Choi, Jae Young; Kim, Sung Huhn

    2017-10-01

    Sudden sensorineural hearing loss (S-SNHL) is an inner ear disorder with an abrupt hearing loss occurring sudden sensorineural hearing loss; LC-MS: liquid chromatography-mass spectrometry; MS: mass spectrometry; autoAb: autoantibody; 1-DE: one-dimensional electrophoresis. Copyright © 2017 Elsevier Inc. All rights reserved.

  10. Cochlear implantation for severe sensorineural hearing loss caused by lightning.

    Science.gov (United States)

    Myung, Nam-Suk; Lee, Il-Woo; Goh, Eui-Kyung; Kong, Soo-Keun

    2012-01-01

    Lightning strike can produce an array of clinical symptoms and injuries. It may damage multiple organs and cause auditory injuries ranging from transient hearing loss and vertigo to complete disruption of the auditory system. Tympanic-membrane rupture is relatively common in patients with lightning injury. The exact pathogenetic mechanisms of auditory lesions in lightning survivors have not been fully elucidated. We report the case of a 45-year-old woman with bilateral profound sensorineural hearing loss caused by a lightning strike, who was successfully rehabilitated after a cochlear implantation. Copyright © 2012 Elsevier Inc. All rights reserved.

  11. Risk factors for sensorineural hearing loss in NICU infants compared to normal hearing NICU controls

    NARCIS (Netherlands)

    Coenraad, S.; Goedegebure, A.; van Goudoever, J. B.; Hoeve, L. J.

    2010-01-01

    To evaluate independent etiologic factors associated with sensorineural hearing loss in infants who have been admitted to the neonatal intensive care unit compared to normal hearing controls. Between 2004 and 2009, 3366 infants were admitted to the neonatal intensive care unit of Sophia Children's

  12. Sensorineural Hearing Loss in Selective Immunglobulin A Deficiency.

    Science.gov (United States)

    Eşki, Erkan; Usta, Belgin Emine; Asilsoy, Suna; Yılmaz, İsmail

    2017-03-01

    To assess hearing functions in pediatric patients with selective immunoglobulin A (IgA) deficiency (SIGAD). Pure-tone audiometry, acoustic impedance, otoacoustic emission, and brainstem audiometric measurements were taken during a non-infectious period in 28 patients with SIGAD and 28 healthy children with normal otoscopic examination. The results of the hearing tests were compared between the two groups. Two male patients and one female patient in the SIGAD group were found to have sensorineural hearing loss (SNHL). However, a comparison of the average pure tone cut-off values at 0.5, 1, 2, and 4 kHz did not reveal any statistically significant difference between the groups (p>0.05). Pediatric patients with SIGAD may exhibit SNHL at certain frequencies and require follow-up for the potential development of hearing loss.

  13. Thiol/disulphide homeostasis as a novel indicator of oxidative stress in sudden sensorineural hearing loss.

    Science.gov (United States)

    Dinc, M E; Ulusoy, S; Is, A; Ayan, N N; Avincsal, M O; Bicer, C; Erel, O

    2016-05-01

    To investigate a novel oxidative stress marker, thiol/disulphide literature homeostasis, in patients with idiopathic sudden sensorineural hearing loss, and to compare the results with healthy controls for the first time. Thirty-two patients with idiopathic sudden sensorineural hearing loss and 30 healthy individuals were included in the study. Serum native thiol, total thiol and disulphide levels were measured, and disulphide/native thiol and disulphide/total thiol ratios were determined in all subjects. Serum native thiol and total thiol levels were significantly lower in patients with sudden sensorineural hearing loss compared with controls (p sudden sensorineural hearing loss in those patients.

  14. Dose passive smoking induce sensorineural hearing loss in children?

    Science.gov (United States)

    Talaat, Hossam Sanyelbhaa; Metwaly, Mohamed Akram; Khafagy, Ahmed Hafez; Abdelraouf, Hatem Ragaa

    2014-01-01

    Smoking plays major role in development of vascular and respiratory serious diseases. It has been reported that negative smoker children are prone for conductive hearing impairment due to repeated attacks of Eustachian tube dysfunction and middle ear effusion. This study aims to identify negative smoking as potential risk factor for development of sensorineural hearing loss. This study was done between January 2010 and November 2012. 411 children aged 5-11 years (8.2 ± 1.5) participated in this study; they were children attending the Ear, Nose, and Throat clinic of a tertiary care hospital and their siblings. The inclusion criteria were: (i) normal speech and language, (ii) absence of any disease or condition that may cause sensorineural hearing loss, and (iii) normal middle ear function on the day of hearing assessment. They were divided into three groups according to the exposure to second-hand smoke at home; group of "no exposure" whereas no smoker in the family (131 children), group of 'mild exposure" whereas the father was the only smoking parent and smoking was prohibited at home (155 children), and group of "heavy exposure", whereas the mother was smoking, or the father was freely smoking at home and in the presence of his children (125 children). Audiological evaluation in the form of pure tone and speech audiometry and immitancemetry was done for the study group. Audiological evaluation revealed that the prevalence of hearing loss was 3.8%, 4.5% and 12% in the "no exposure", "mild exposure", and "heavy exposure" groups, respectively. Significant difference was only detected between the high exposure group and the other two groups. All children had minimal sensorineural hearing loss, i.e. threshold of frequencies showing hearing loss was 20 or 25 dB HL. The risk ratios (95% confidence interval) for hearing loss in the study subgroups were 1.18 (0.38, 3.64) for mild exposure group (p>0.05), 3.14 (1.18, 8.3) for heavy exposure group (phearing loss, and it is

  15. Risk of sudden sensorineural hearing loss in patients with common preexisting sensorineural hearing impairment: a population-based study in Taiwan.

    Directory of Open Access Journals (Sweden)

    Malcolm Koo

    Full Text Available The role of preexisting sensorineural hearing impairment on the risk for sudden sensorineural hearing loss (SSHL is still unclear. In this study, we aimed to assess the risk of SSHL in patients with common preexisting sensorineural hearing impairment using population-based data.A population-based case-control study design was used to analyze claims data between January 2001 and December 2011 obtained from the Taiwan National Health Insurance Research Database. The cases consisted of 514 patients with SSHL and the controls were frequency matched to 2,570 cases by sex, 10-year age group, and year of index date. Common sensorineural hearing impairments were retrospectively assessed in the cases and controls. Associations between sensorineural hearing impairment and risk of SSHL were evaluated using unconditional univariate and multivariate logistic regression analyses.The mean age for the 3,084 study subjects was 53.1 years (standard deviation, S.D. = 15.6. Of the 514 cases, 49 (9.5% had sensorineural hearing impairment while only 44 (1.7% of the 2,570 controls had the same condition. Univariate logistic regression analyses indicated that preexisting sensorineural hearing impairment was significantly associated with SSHL (odds ratio, OR = 6.05, p < 0.001. Other comorbidities including hypertension, diabetes mellitus, and hyperlipidemia also showed significant associations with SSHL. Similar results were obtained when the association between SSHL and sensorineural hearing impairment was adjusted with either all the covariates (adjusted OR = 6.22, p < 0.001 or with only those selected using a backward elimination procedure (adjusted OR = 6.20, p < 0.001.Results from this population-based case-control study revealed that common sensorineural hearing impairment might be a novel risk factor for SSHL.

  16. Sensorineural deafness

    Science.gov (United States)

    Nerve deafness; Hearing loss - sensorineural; Acquired hearing loss; SNHL; Noise-induced hearing loss; NIHL; Presbycusis ... that carries the signals to the brain. Sensorineural deafness that is present at birth (congenital) is most ...

  17. Sensorineural Hearing Loss in Pseudoexfoliation Syndrome

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    Shahin Yazdani

    2008-12-01

    Full Text Available

    PURPOSE: To determine hearing thresholds at sound frequencies important for speech comprehension in subjects with ocular pseudoexfoliation (PXF and to compare them with that of controls without PXF. METHODS: Eighty-three subjects with ocular PXF and 83 age and sex matched controls without PXF were enrolled in this case-control study. Pure tone audiometry (bone conduction was performed at 1, 2 and 3 kilohertz (KHz in all subjects. Thresholds were compared to an age and sex stratified standard (ISO7029 and between study groups. Hearing loss was defined as sum of tested hearing thresholds (HTL-1,2,3 lower than the ISO7029 standard median. RESULTS: The study included 60 male and 23 female subjects in each group. Hearing loss was present in 147 of 166 (88.6% of examined ears in the case group vs 89 of 166 (53.6% in the control group (P < 0.001; odds ratio [OR] = 6.69; 95% confidence interval [CI], 3.49-11.79. Overall 78 subjects (94.0% in the case group vs 58 subjects (69.9% in the control group had hearing loss in one or both ears (P < 0.001; OR=6.72; 95%CI, 2.42-18.62. Hearing thresholds at each of the examined frequencies and the HTL-1,2,3 were also significantly higher in individuals with PXF. Although glaucoma was significantly more common in subjects with PXF (51.8% vs 22.9%, P < 0.001, it was not associated with hearing

  18. Oral steroid treatment for idiopathic sudden sensorineural hearing loss

    Science.gov (United States)

    Chen, Wei T.; Lee, Jui W.; Yuan, Chien H.; Chen, Rong F.

    2015-01-01

    Objectives: To describe the efficacy of long-term oral steroids in idiopathic sudden sensorineural hearing loss (ISSHL), and to explore potential prognosis factors, the relationship of hearing recovery outcome, and the recovery time-course in ISSHL. Methods: In this retrospective study, we analyzed 215 cases diagnosed with idiopathic unilateral sudden deafness between January 2003 and December 2012 at a regional hospital in southern Taiwan. All of them received oral steroid therapy and were followed for at least 3 months. Results: Young age, the presence of no other disease (diabetes, hypertension, or cardiovascular disease), treatment within 7 days of the onset of ISSHL, mild hearing loss, and audiograms with ascending curves had a statistically significant better hearing recovery. The average pure-tone audiometry (PTA) was 67.8±23.9 dB initially, and was improved between one month (51.6±28.7 dB) and 2 months post treatment (49.7±28.6 dB). The average last-visit PTA was 49.9±29.2 dB. Conclusion: Approximately one third of patients had full recovery in hearing, one third had partial recovery, and approximately one third did not recover from hearing loss. In addition, the hearing level remained relatively stable following 2 months of treatment. More than 2 months of oral steroid therapy for ISSHL is not recommended because no additional benefits were evidenced. PMID:25737170

  19. Prognostic predictors of sudden sensorineural hearing loss in defibrinogenation therapy.

    Science.gov (United States)

    Oya, Ryohei; Horii, Arata; Akazawa, Hitoshi; Osaki, Yasuhiro; Inohara, Hidenori

    2016-01-01

    Defibrinogenation therapy rather than corticosteroids therapy should be chosen for patients specifically with profound hearing loss and with initial high fibrinogen. Corticosteroids therapy is the standard treatment for sudden sensorineural hearing loss (SSNHL) and prognostic factors by this therapy were reported. Defibrinogenation therapy is one of the treatment options for SSNHL. Aims of this study were to identify prognostic factors and correlative markers with hearing improvement in treating SSNHL by defibrinogenation therapy. During the early phase of the study, consecutive 61 patients were treated by defibrinogenation therapy with batroxobin (50 units), whereas corticosteroids (500 mg/day of hydrocortisone tapered by 9 days) were used for consecutive 64 patients during the late phase. Blood data that could predict a complete recovery were identified. Coagulation/fibrinolysis markers correlated with hearing improvement by defibrinogenation therapy were investigated. Although there were no overall differences in hearing improvement between the two therapies, recovery rate in profound hearing loss patients was better in defibrinogenation therapy. In patients who showed complete recovery, serum fibrinogen level before treatment was significantly higher in the defibrinogenation group than the corticosteroid group. Responses of several fibrinolysis markers to defibrinogenation therapy evaluated by post-/pre-values were negatively correlated with hearing improvement.

  20. Epileptiform electroencephalogram abnormality in children with congenital sensorineural hearing loss.

    Science.gov (United States)

    El-Badry, Mohamed Mohamed; Hamdy, Nermin Aly; Sobhy, Sayed; Gamal, Reham

    2014-04-01

    This work was designed to study electroencephalogram findings in children with congenital sensorineural hearing loss and correlate these findings with the SNHL parameters as duration, etiology, severity, and type. Ninety children with bilateral congenital sensorineural hearing loss served as the study group. They were free from any neurological disorders or symptoms that are commonly associated with abnormal electroencephalogram as convulsions or loss of consciousness. Twenty children having normal hearing with no history of otological or neurological disorders served as the control group. All children participating in the study were subjected to full medical and audiological history, otological examination, neurological examination, audiological evaluation and electroencephalogram recording. Mean age of the children in the control group was 3.56 ± 2.1 years and mean age of the children in the study group was 3.8 ± 2.2 years. While none of the control children had abnormal electroencephalogram, 38 (42.2%) of children with congenital SNHL had epileptiform electroencephalogram abnormality. The epileptiform abnormality was generalized in 14 children (36.8%), focal temporal in 17 children (44.7%) and focal other than temporal in 7 children (18.4%). According to the hemispheric side affected, the abnormality was right in 14 children (36.8%), left in 10 children (26.3%) and bilateral in 14 children (36.8%). No statistically significant predominance of specific site or side of the epileptiform abnormality was found. Similarly, no statistical significant prevalent of the epileptiform abnormality was found in relation to the age or sex of children, duration of hearing loss or etiology of hearing loss (i.e., genetic vs. neonatal insults). On the other hand, the epileptiform abnormality was statistically prevalent in children with moderate degree of hearing loss, and in children with auditory neuropathy spectrum disorder. The epileptiform electroencephalogram abnormality is

  1. Evaluation of cardiovascular risks and recovery of idiopathic sudden sensorineural hearing loss in hospitalised patients: comparison between complete and partial sudden sensorineural hearing loss.

    Science.gov (United States)

    Haremza, C; Klopp-Dutote, N; Strunski, V; Page, C

    2017-10-01

    To evaluate the presence of cardiovascular risk factors and recovery of idiopathic sudden sensorineural hearing loss in hospitalised patients. A single-centre retrospective study of 80 patients hospitalised for idiopathic sudden sensorineural hearing loss was conducted over a 6-year period. Mean pure tone hearing thresholds were assessed by pure tone audiometry. Twenty-three of 80 patients (28.75 per cent) initially had no cardiovascular risk factors. Forty-five patients had hyperlipidaemia, 22 patients had hypertension, 7 patients had diabetes mellitus and 7 patients were obese. No statistically significant difference was observed between patients with complete versus partial sudden sensorineural hearing loss (p = 0.0708) concerning the cardiovascular risk factors. At long-term follow up, the hearing recovery rate was not significantly different between the two groups of patients (p = 0.7541). The lack of a clear relationship between idiopathic sudden sensorineural hearing loss and cardiovascular risk factors suggests that sudden sensorineural hearing loss has a predominantly multifactorial disease profile regardless of hearing impairment severity.

  2. Prediction of hearing outcomes by multiple regression analysis in patients with idiopathic sudden sensorineural hearing loss.

    Science.gov (United States)

    Suzuki, Hideaki; Tabata, Takahisa; Koizumi, Hiroki; Hohchi, Nobusuke; Takeuchi, Shoko; Kitamura, Takuro; Fujino, Yoshihisa; Ohbuchi, Toyoaki

    2014-12-01

    This study aimed to create a multiple regression model for predicting hearing outcomes of idiopathic sudden sensorineural hearing loss (ISSNHL). The participants were 205 consecutive patients (205 ears) with ISSNHL (hearing level ≥ 40 dB, interval between onset and treatment ≤ 30 days). They received systemic steroid administration combined with intratympanic steroid injection. Data were examined by simple and multiple regression analyses. Three hearing indices (percentage hearing improvement, hearing gain, and posttreatment hearing level [HLpost]) and 7 prognostic factors (age, days from onset to treatment, initial hearing level, initial hearing level at low frequencies, initial hearing level at high frequencies, presence of vertigo, and contralateral hearing level) were included in the multiple regression analysis as dependent and explanatory variables, respectively. In the simple regression analysis, the percentage hearing improvement, hearing gain, and HLpost showed significant correlation with 2, 5, and 6 of the 7 prognostic factors, respectively. The multiple correlation coefficients were 0.396, 0.503, and 0.714 for the percentage hearing improvement, hearing gain, and HLpost, respectively. Predicted values of HLpost calculated by the multiple regression equation were reliable with 70% probability with a 40-dB-width prediction interval. Prediction of HLpost by the multiple regression model may be useful to estimate the hearing prognosis of ISSNHL. © The Author(s) 2014.

  3. Sensorineural hearing loss and celiac disease: a coincidental finding.

    Science.gov (United States)

    Volta, Umberto; Ferri, Gian Gaetano; De Giorgio, Roberto; Fabbri, Angela; Parisi, Claudia; Sciajno, Laura; Castellari, Alessandra; Fiorini, Erica; Piscaglia, Maria; Barbara, Giovanni; Granito, Alessandro; Pirodda, Antonio

    2009-08-01

    Celiac disease (CD) can be associated with a variety of extraintestinal manifestations, including neurological diseases. A new neurological correlation has been found between CD and sensorineural hearing loss (SNHL). To verify the association between SNHL and CD, and to establish whether the neurological hearing impairment in CD is related to nonorgan-specific and antineuronal antibodies, as well as the presence of autoimmune disorders. A sample of 59 consecutive biopsy- and serologically proven CD patients were studied. Among CD patients, 11 were newly diagnosed and 48 were on a gluten-free diet. Hearing function was assessed by audiometric analysis in all CD patients as well as in 59 age- and sex-matched controls. Patients were tested for a panel of immune markers including nonorgan-specific autoantibodies and antineuronal antibodies. SNHL was detected in five CD patients (8.5%) and in two controls (3.4%). In one patient, the SNHL was bilateral, whereas the remaining four had a monolateral impairment. The prevalence of SNHL was not significantly different between CD patients and controls. At least one of the antibodies tested for was positive in two of the five CD patients with SNHL and in 12 of the 54 CD patients without SNHL. Antineuronal antibodies to central nervous system antigens were consistently negative in the five CD patients with SNHL. Only one of the five CD patients with SNHL had Hashimoto thyroiditis. SNHL and CD occur coincidentally. Hearing function should be assessed only in CD patients with clinical signs of hearing deficiency.

  4. Gait performance of children and adolescents with sensorineural hearing loss.

    Science.gov (United States)

    Melo, Renato de Souza

    2017-09-01

    Several studies have demonstrated that children with sensorineural hearing loss (SNHL) may exhibit balance disorders, which can compromise the gait performance of this population. Compare the gait performance of normal hearing (NH) children and those with SNHL, considering the sex and age range of the sample, and analyze gait performance according to degrees of hearing loss and etiological factors in the latter group. This is a cross-sectional study that assessed 96 students, 48 NH and 48 with SNHL, aged between 7 and 18 years. The Brazilian version of the Dynamic Gait Index (DGI) was used to analyze gait and the Mann-Whitney test for statistical analysis. The group with SNHL obtained lower average gait performance compared to NH subjects (p=0.000). This was also observed when the children were grouped by sex female and male (p=0.000). The same difference occurred when the children were stratified by age group: 7-18 years (p=0.000). The group with severe and profound hearing loss exhibited worse gait performance than those with mild and moderate loss (p=0.048) and children with prematurity as an etiological factor demonstrated the worst gait performance. The children with SNHL showed worse gait performance compared to NH of the same sex and age group. Those with severe and profound hearing loss and prematurity as an etiological factor demonstrated the worst gait performances. Copyright © 2017 Elsevier B.V. All rights reserved.

  5. Glucocorticoid influence on prognosis of idiopathic sudden sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Eduardo Amaro Bogaz

    2014-06-01

    Full Text Available INTRODUCTION: Idiopathic Sudden Sensorineural Hearing Loss (ISSHL is defined when a loss of at least 30 dB occurs in over 3 continuous frequencies, in up to 72 hours, of which etiology is not established, despite adequate investigation. Different types of treatment regimens have been proposed, but only glucocorticoids have shown some evidence of benefit in the literature. OBJECTIVE: To analyze whether the type of treatment or time of treatment with glucocorticoids have any influence on hearing recovery in ISSHL. METHODS: Observational retrospective cohort study. One hundred twenty-seven patients with ISSHL, treated at outpatient clinics between the years 2000 and 2010, were studied. We evaluated the prognostic correlation of the type of treatment and time to treatment with glucocorticoids and ISSHL. RESULTS: The absolute hearing gain and the relative hearing gain was as follows: 23.6 dB and 37.2%. Complete recovery was observed in 15.7% of patients, significant recovery in 27.6% and recovery in 57.5%. CONCLUSION: In this study, there was no difference between the use and nonuse of glucocorticoids in hearing improvement. However, when started within seven days after onset, the use of glucocorticoids was a factor of better prognosis.

  6. Comparison between Bilateral and Unilateral Sudden Sensorineural Hearing Loss

    Science.gov (United States)

    Bing, Dan; Wang, Da-Yong; Lan, Lan; Zhao, Li-Dong; Yin, Zi-Fang; Yu, Lan; Chen, Guo-Hui; Guan, Jing; Wang, Qiu-Ju

    2018-01-01

    Background: Bilateral sudden sensorineural hearing loss (BSSHL) is rare and assumed to be a different clinical entity compared to unilateral SSHL (USSHL). This study examined the differences between the idiopathic BSSHL and USSHL. Methods: Forty-six sequential BSSHL patients (Se-BSSHL) and 68 simultaneous BSSHL (Si-BSSHL) were consecutively admitted between June 2008 and December 2015. Two sets of patients served as control groups: (1) USSHL patients with healthy contralateral ear and (2) USSHL patients with contralateral preexisting hearing loss (USSHLwCHL). We retrospectively analyzed differences among four cohorts using analysis of variance, Kruskal-Wallis test, Welch's t-test, and Chi-square test as appropriate before and after propensity score matching (PSM) based on age, gender, and body mass index (BMI). Results: The prevalence of idiopathic BSSHL was 8.6% (114/1329) among the total SSHL patients. In the total cohort, USSHL patients tended to be younger, female, and tended to have lower BMI, renal parameters, and total cholesterol in addition to higher high-density lipoprotein compared to the other three groups. Most routine blood indicators, some coagulation markers, and immunoglobulin M (H = 13.4, P = 0.004) were significantly different among the study groups. After PSM, the major significant differences were found in audiometric characteristics. Si-BSSHL and Se-BSSHL patients demonstrated similar hearing thresholds as USSHL but were significantly better than the USSHLwCHL patients across most frequencies before and after treatment (H = 30.0, P hearing and H = 12.0, P = 0.007 for final hearing). Moreover, the BSSHL patients showed different hearing loss distribution patterns (more descending type, χ2 = 33.8, P = 0.001) with less hearing gain (H = 17.5, P hearing outcome rather than being classified as a completely different disease entity compared to USSHL. PMID:29363646

  7. Endothelial Dysfunction in Idiopathic Sudden Sensorineural Hearing Loss: A Review

    Science.gov (United States)

    Quaranta, Nicola; De Ceglie, Vincenzo; D’Elia, Alessandra

    2016-01-01

    An endothelial dysfunction has been described in idiopathic sudden sensorineural hearing loss (ISSHL) patients. The purpose of our review was to: i) identify, evaluate and review recent research about cardiovascular risk factors involvement and signs of endothelial dysfunction in ISSHL; ii) implication of these discovering in clinical practice and future research. A Medline literature search was conducted to identify any study on the involvement of endothelial dysfunction in ISSHL, published in the English language in the last decade. The following MEDLINE search terms were used: sudden sensorineural hearing loss (SSHL) and endothelial dysfunction (text words). Additional studies were identified by hand searching the references of original articles and review articles. Studies were not excluded on the basis of the qualitative or quantitative definitions of SSHL, treatment regimens, or outcome measures. Data were extracted from included papers by a reviewer. Information on the patients, investigations, methods, interventions, and outcomes were systematically analyzed. Characteristics and results of all included studies were reviewed systematically. High levels of adhesion molecules, hyperhomocysteinemia and lower folate levels, unbalanced oxidative status, a lower value of flow-mediated dilatation of brachial artery and a reduced percentage of circulating endothelial progenitor cells in patients affected by ISSHL support the hypothesis that this syndrome should be considered as a microcirculation disorder based on endothelial dysfunction and drive clinicians to implement all the traditional strategies used for preventing cardiovascular events, to also reduce the likelihood of ISSHL occurrence. PMID:27588164

  8. [Iatrogenic and non-iatrogenic factors as causes of progressive sensorineural hearing loss].

    Science.gov (United States)

    De Capua, B; Barbieri, M T; Tozzi, A; Passàli, D

    1998-08-01

    The aim of our study was to analyze factors such as noise, chemical drugs, industrial solvents and radiotherapy, which can cause cochlear lesions with progressive sensorineural hearing loss. Although an acute overstimulation by acoustic energy may induce an irreversible hearing loss, in most cases the noise-induced deafness is related to the duration of the exposure and to the level of the acoustic stimulation. A permanent hearing deficit occurs when the acoustic level exceeds 85 dBs. Also several classes of drugs are described as having ototoxic potential: aminoglycoside antibiotics, loop diuretics, antimalarial drugs such as quinine, salicylates, some chemotherapeutic antineoplastic agents. Their potential ototoxic effect seems to be related not only to the molecule, but also to individual predisposition, dose and route of administration. Regarding the benzene derivatives, there is a relationship between their ototoxicity and factors such as duration of exposure and concentration in the local environment. Finally, radiotherapy to areas near the temporal bone may produce a degenerative insult to the vascular stria and the hair cell causing a progressive sensorineural hearing loss.

  9. Possible mechanisms for sensorineural hearing loss and deafness in patients with propionic acidemia.

    Science.gov (United States)

    Grünert, S C; Bodi, I; Odening, K E

    2017-02-13

    Propionic acidemia is an inborn error of metabolism caused by deficiency of the mitochondrial enzyme propionyl-CoA carboxylase. Sensorineural deafness and severe hearing loss have been described as long-term complications of this disease, however, the mechanism has not yet been elucidated. We have recently shown by patch clamping experiments and Western blots that acute and chronic effects of accumulating metabolites such as propionic acid, propionylcarnitine and methylcitrate on the KvLQT1/KCNE1 channel complex cause long QT syndrome in patients with propionic acidemia by inhibition of K + flow via this channel. The same KvLQT1/KCNE1 channel complex is expressed in the inner ear and essential for luminal potassium secretion into the endolymphatic space. A disruption of this K + flow results in sensorineural hearing loss or deafness. It can be assumed that acute and chronic effects of accumulating metabolites on the KvLQT1/KCNE1 channel protein may similarly cause the hearing impairment of patients with propionic acidemia.

  10. Clinical Study on 136 Children with Sudden Sensorineural Hearing Loss.

    Science.gov (United States)

    Li, Feng-Jiao; Wang, Da-Yong; Wang, Hong-Yang; Wang, Li; Yang, Feng-Bo; Lan, Lan; Guan, Jing; Yin, Zi-Fang; Rosenhall, Ulf; Yu, Lan; Hellstrom, Sten; Xue, Xi-Jun; Duan, Mao-Li; Wang, Qiu-Ju

    2016-04-20

    The prevalence of sudden sensorineural hearing loss in children (CSSNHL) is consistently increasing. However, the pathology and prognosis of CSSNHL are still poorly understood. This retrospective study evaluated clinical characteristics and possible associated factors of CSSNHL. One hundred and thirty-six CSSNHL patients treated in Department of Otolaryngology-Head and Neck Surgery and Institute of Otolaryngology at Chinese PLA General Hospital between July 2008 and August 2015 were included in this study. These patients were analyzed for clinical characteristics, audiological characteristics, laboratory examinations, and prognostic factors. Among the 136 patients (151 ears), 121 patients (121 ears, 80.1%) were diagnosed with unilaterally CSSNHL, and 15 patients (30 ears, 19.9%) with bilateral CSSNHL. The complete recovery rate of CSSNHL was 9.3%, and the overall recovery rate was 37.7%. We found that initial degree of hearing loss, onset of treatment, tinnitus, the ascending type audiogram, gender, side of hearing loss, the recorded auditory brainstem response (ABR), and distortion product otoacoustic emissions (DPOAEs) had prognostic significance. Age, ear fullness, and vertigo had no significant correlation with recovery. Furthermore, the relevant blood tests showed 30.8% of the children had abnormal white blood cell (WBC) counts, 22.1% had elevated homocysteine levels, 65.8% had high alkaline phosphatase (ALP), 33.8% had high IgE antibody levels, and 86.1% had positive cytomegalovirus (CMV) IgG antibodies. CSSNHL commonly occurs unilaterally and results in severe hearing loss. Initial severe hearing loss and bilateral hearing loss are negative prognostic factors for hearing recovery, while positive prognostic factors include tinnitus, gender, the ascending type audiogram, early treatment, identifiable ABR waves, and DPOAEs. Age, vertigo, and ear fullness are not correlated with the recovery. Some serologic indicators, including the level of WBC, platelet

  11. Clinical Study on 136 Children with Sudden Sensorineural Hearing Loss

    Science.gov (United States)

    Li, Feng-Jiao; Wang, Da-Yong; Wang, Hong-Yang; Wang, Li; Yang, Feng-Bo; Lan, Lan; Guan, Jing; Yin, Zi-Fang; Rosenhall, Ulf; Yu, Lan; Hellstrom, Sten; Xue, Xi-Jun; Duan, Mao-Li; Wang, Qiu-Ju

    2016-01-01

    Background: The prevalence of sudden sensorineural hearing loss in children (CSSNHL) is consistently increasing. However, the pathology and prognosis of CSSNHL are still poorly understood. This retrospective study evaluated clinical characteristics and possible associated factors of CSSNHL. Methods: One hundred and thirty-six CSSNHL patients treated in Department of Otolaryngology-Head and Neck Surgery and Institute of Otolaryngology at Chinese PLA General Hospital between July 2008 and August 2015 were included in this study. These patients were analyzed for clinical characteristics, audiological characteristics, laboratory examinations, and prognostic factors. Results: Among the 136 patients (151 ears), 121 patients (121 ears, 80.1%) were diagnosed with unilaterally CSSNHL, and 15 patients (30 ears, 19.9%) with bilateral CSSNHL. The complete recovery rate of CSSNHL was 9.3%, and the overall recovery rate was 37.7%. We found that initial degree of hearing loss, onset of treatment, tinnitus, the ascending type audiogram, gender, side of hearing loss, the recorded auditory brainstem response (ABR), and distortion product otoacoustic emissions (DPOAEs) had prognostic significance. Age, ear fullness, and vertigo had no significant correlation with recovery. Furthermore, the relevant blood tests showed 30.8% of the children had abnormal white blood cell (WBC) counts, 22.1% had elevated homocysteine levels, 65.8% had high alkaline phosphatase (ALP), 33.8% had high IgE antibody levels, and 86.1% had positive cytomegalovirus (CMV) IgG antibodies. Conclusions: CSSNHL commonly occurs unilaterally and results in severe hearing loss. Initial severe hearing loss and bilateral hearing loss are negative prognostic factors for hearing recovery, while positive prognostic factors include tinnitus, gender, the ascending type audiogram, early treatment, identifiable ABR waves, and DPOAEs. Age, vertigo, and ear fullness are not correlated with the recovery. Some serologic indicators

  12. Otolithic organ function in patients with profound sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Yujuan Zhou

    2016-06-01

    Full Text Available Profound sensorineural hearing loss (PSHL is not uncommonly encountered in otology. In clinics, there is a high incidence of otolithic damage in patients with PSHL, but relevant reports are few. Sharing a continuous membranous structure and similar receptor cell ultrastructures, the cochlea and vestibule may be susceptible to the same harmful factors. Disorders of the inner ear may result in a variety of manifestations, including vertigo, spatial disorientation, blurred vision, impaired articulation, and hearing impairment. Considering the diversity of clinical symptoms associated with PSHL with otolithic dysfunction, it may be frequently misdiagnosed, and objective means of testing the function of otolithic organs should be recommended for hearing-impaired patients. Vestibular-evoked myogenic potentials (VEMPs via air-conducted sound are of great importance for the diagnosis of otolithic function. Hearing devices such as cochlear implants are commonly accepted treatments for PSHL, and early identification and treatment of vestibular disorders may increase the success rate of cochlear implantation. Therefore, it is necessary to increase awareness of otolithic functional states in patients with PSHL.

  13. Bone-anchored hearing aids and unilateral sensorineural hearing loss: why do patients reject them?

    Science.gov (United States)

    Siau, D; Dhillon, B; Andrews, R; Green, K M J

    2015-04-01

    This study aimed to report the bone-anchored hearing aid uptake and the reasons for their rejection by unilateral sensorineural deafness patients. A retrospective review of 90 consecutive unilateral sensorineural deafness patients referred to the Greater Manchester Bone-Anchored Hearing Aid Programme between September 2008 and August 2011 was performed. In all, 79 (87.8 per cent) were deemed audiologically suitable: 24 (30.3 per cent) eventually had a bone-anchored hearing aid implanted and 55 (69.6 per cent) patients declined. Of those who declined, 26 (47.3 per cent) cited perceived limited benefits, 18 (32.7 per cent) cited reservations regarding surgery, 13 (23.6 per cent) preferred a wireless contralateral routing of sound device and 12 (21.8 per cent) cited cosmetic reasons. In all, 32 (40.5 per cent) suitable patients eventually chose the wireless contralateral routing of sound device. The uptake rate was 30 per cent for audiologically suitable patients. Almost half of suitable patients did not perceive a sufficient benefit to proceed to device implantation and a significant proportion rejected it. It is therefore important that clinicians do not to rush to implant all unilateral sensorineural hearing loss patients with a bone-anchored hearing aid.

  14. Anisomorphic cortical reorganization in asymmetric sensorineural hearing loss.

    Science.gov (United States)

    Cheung, Steven W; Atencio, Craig A; Levy, Eliott R J; Froemke, Robert C; Schreiner, Christoph E

    2017-08-01

    Acoustic trauma or inner ear disease may predominantly injure one ear, causing asymmetric sensorineural hearing loss (SNHL). While characteristic frequency (CF) map plasticity of primary auditory cortex (AI) contralateral to the injured ear has been detailed, there is no study that also evaluates ipsilateral AI to compare cortical reorganization across both hemispheres. We assess whether the normal isomorphic mirror-image relationship between the two hemispheres is maintained or disrupted in mild-to-moderate asymmetric SNHL of adult squirrel monkeys. At week 24 after induction of acoustic injury to the right ear, functional organization of the two hemispheres differs in direction and magnitude of interaural CF difference, percentage of recording sites with spectrally nonoverlapping binaural activation, and the concurrence of peripheral and central activation thresholds. The emergence of this anisomorphic cortical reorganization of the two hemispheres is replicated by simulation based on spike timing-dependent plasticity, where 1 ) AI input from the contralateral ear is dominant, 2 ) reestablishment of relatively shorter contralateral ear input timing drives reorganization, and 3 ) only AI contralateral to the injured ear undergoes major realignment of interaural frequency maps that evolve over months. Asymmetric SNHL disrupts isomorphic organization between the two hemispheres and results in relative local hemispheric autonomy, potentially impairing performance of tasks that require binaural input alignment or interhemispheric processing. NEW & NOTEWORTHY Mild-to-moderate hearing loss in one ear and essentially normal hearing in the other triggers cortical reorganization that is different in the two hemispheres. Asymmetry of cochlea sensitivities does not simply propagate to the two auditory cortices in mirror-image fashion. The resulting anisomorphic cortical reorganization may be a neurophysiological basis of clinical deficits in asymmetric hearing loss, such as

  15. Motor proficiency and dynamic visual acuity in children with bilateral sensorineural hearing loss.

    Science.gov (United States)

    Martin, Willemien; Jelsma, Jennifer; Rogers, Christine

    2012-10-01

    Due to the close relationship between the cochlea and the peripheral vestibular system, the function of the vestibular system may be impaired in children with sensorineural hearing loss. The aims of this study were to determine the prevalence of impairments of motor performance and dynamic visual acuity, and the nature and extent of interaction between these in children with sensorineural hearing loss between the ages of 4 and 14 years. This research utilized a correlational, cross-sectional, descriptive design. Thirty-two children with sensorineural hearing loss were matched according to age and gender with children with no hearing impairment. Motor performance was evaluated by means of the Movement Assessment Battery for Children-2 and dynamic visual acuity was evaluated with the dynamic visual acuity test. The performances of the two groups on the different tests were then compared. The one-sided chi-square test or Fisher's exact test was used to determine whether there was any association between sensorineural hearing loss, impaired motor performance and poor dynamic visual acuity. The Mann-Whitney U-test was used to determine the difference between children with sensorineural hearing loss and those with normal hearing on the Movement Assessment Battery for Children-2. Forward stepwise regression was used to establish the predictors of the Movement Assessment Battery for Children-2 total standard score. The Kruskal-Wallis test was used to compare scores of children with normal hearing and those with a mild to moderate sensorineural hearing loss on the Movement Assessment Battery for Children-2. Reduced dynamic visual acuity is associated with sensorineural hearing loss (p=0.026). Motor performance is dependent on dynamic visual acuity and severity of sensorineural hearing loss (r(2)=0.41, p=0.001). The results of this study indicate that in children with sensorineural hearing loss, the prevalence of reduced dynamic visual acuity is 15.6% and of motor impairment

  16. Characteristics and Spontaneous Recovery of Tinnitus Related to Idiopathic Sudden Sensorineural Hearing Loss

    Science.gov (United States)

    Mühlmeier, Guido; Baguley, David; Cox, Tony; Suckfüll, Markus; Meyer, Thomas

    2016-01-01

    Objective: To evaluate the characteristics and spontaneous recovery of tinnitus related to idiopathic sudden sensorineural hearing loss (ISSNHL). Study Design: Retrospective analysis from two randomized placebo-controlled clinical trials for treatment of ISSNHL within 48 hours from onset (Study A), or of tinnitus related to ISSNHL within 3 months from onset (Study B). Setting: Forty-eight European sites (academic tertiary referral centers, private ENT practices). Patients: One hundred thirteen adult patients of which 65 with hearing loss ≥30 dB (Study A) and 48 with persistent acute tinnitus (Study B) at baseline. Interventions: Intratympanic (i.t.) injection of placebo gel in single dose or in triple dose during 3 consecutive days. Main Outcome Measures: Frequency of tinnitus, subjective tinnitus loudness, rates of complete tinnitus remission, and complete hearing recovery during 3 months follow-up. Results: In acute ISSNHL, tinnitus loudness decreased rapidly in cases of mild-moderate hearing loss, and tinnitus had completely resolved in two-thirds of patients after 3 months. Hearing recovery preceded tinnitus resolution. When associated with severe-profound hearing loss, tinnitus improved significantly less. Complete hearing recovery and full tinnitus remission were both about three times more frequent in mild-moderate hearing loss patients than in severe-profound cases. Improvement in tinnitus loudness over time can be approximated by a negative exponential function. Conclusions: Prognosis for ISSNHL-related tinnitus is relatively poor in case of severe-profound hearing loss and the longer it has persisted. Alleviation or management of tinnitus should be a key therapeutic objective especially in pronounced ISSNHL cases. PMID:27228021

  17. Quality of Life and Hearing Eight Years After Sudden Sensorineural Hearing Loss.

    Science.gov (United States)

    Härkönen, Kati; Kivekäs, Ilkka; Rautiainen, Markus; Kotti, Voitto; Vasama, Juha-Pekka

    2017-04-01

    To explore long-term hearing results, quality of life (QoL), quality of hearing (QoH), work-related stress, tinnitus, and balance problems after idiopathic sudden sensorineural hearing loss (ISSNHL). Cross-sectional study. We reviewed the audiograms of 680 patients with unilateral ISSNHL on average 8 years after the hearing impairment, and then divided the patients into two study groups based on whether their ISSNHL had recovered to normal (pure tone average [PTA] ≤ 30 dB) or not (PTA > 30 dB). The inclusion criteria were a hearing threshold decrease of 30 dB or more in at least three contiguous frequencies occurring within 72 hours in the affected ear and normal hearing in the contralateral ear. Audiograms of 217 patients fulfilled the criteria. We reviewed their medical records; measured present QoL, QoH, and work-related stress with specific questionnaires; and updated the hearing status. Poor hearing outcome after ISSNHL was correlated with age, severity of hearing loss, and vertigo together with ISSNHL. Quality of life and QoH were statistically significantly better in patients with recovered hearing, and the patients had statistically significantly less tinnitus and balance problems. During the 8-year follow-up, the PTA of the affected ear deteriorated on average 7 dB, and healthy ear deteriorated 6 dB. Idiopathic sudden sensorineural hearing loss that failed to recover had a negative impact on long-term QoL and QoH. The hearing deteriorated as a function of age similarly both in the affected and the healthy ear, and there were no differences between the groups. The cumulative recurrence rate for ISSNHL was 3.5%. 4 Laryngoscope, 127:927-931, 2017. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

  18. Sudden sensorineural hearing loss in children: Etiology, management, and outcome.

    Science.gov (United States)

    Pitaro, Jacob; Bechor-Fellner, Avital; Gavriel, Haim; Marom, Tal; Eviatar, Ephraim

    2016-03-01

    Pediatric sudden sensorineural hearing loss (SSNHL) is uncommon, and the current guidelines for its management refer to adults. Our objective was to review cases of SSNHL in children and examine their etiologies, management, and outcome. We performed a retrospective chart review of all children under the age of 18 years treated for SSNHL between January 2003 and September 2014. Data recorded included age, gender, symptoms, onset of hearing loss, audiometric results, diagnostic studies, treatment, and outcome. Nineteen children were included. Mean age was 14 years (range 7-18 years). Male: female ratio was 9:10. Degree of hearing loss varied from mild to profound across the tested frequencies. Most common accompanying symptom was tinnitus. Serologic tests demonstrated recent Epstein-Barr virus infection in one patient and previous cytomegalovirus infection in six patients. Imaging studies included computed tomography scan (n=3) and/or magnetic resonance imaging (n=12). All imaging studies did not demonstrate any pathology. Treatment included systemic steroids in 19 (100%) children and intratympanic steroids in eight (42%). Hearing completely improved in three (16%) children, partially improved in nine (47%), and there was no improvement in six (32%). One child was lost to follow-up. Viral infection was a common finding in children with SSNHL and no pathological changes were demonstrated on imaging studies. In most patients (63%), hearing improvement was observed. Intratympanic steroid injection can benefit these children. Further studies are required to investigate the etiologies and establish guidelines for the management of SSNHL in children. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  19. Molecular Investigation of Pediatric Portuguese Patients with Sensorineural Hearing Loss

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    Célia Nogueira

    2011-01-01

    Full Text Available The understanding of the molecular genetics in sensorineural hearing loss (SNHL has advanced rapidly during the last decade, but the molecular etiology of hearing impairment in the Portuguese population has not been investigated thoroughly. To provide appropriate genetic testing and counseling to families, we analyzed the whole mitochondrial genome in 95 unrelated children with SNHL (53 nonsyndromic and 42 syndromic and searched for variations in two frequent genes, GJB2 and GJB6, in the non-syndromic patients. Mutations in mtDNA were detected in 4.2% of the cases, including a hitherto undescribed change in the mtDNA-tRNATrp gene (namely, m.5558A>G. We also identified mono- or biallelic GJB2 mutations in 20 of 53 non-syndromic cases and also detected two novel mutations (p.P70R and p.R127QfsX84. Our data further reinforce the notion that genetic heterogeneity is paramount in children with SNHL.

  20. Association between Family History and Idiopathic Sudden Sensorineural Hearing Loss.

    Science.gov (United States)

    Binnetoğlu, Adem; Yumuşakhuylu, Ali Cemal; Demir, Berat; Bağlam, Tekin; Derinsu, Ufuk; Sarı, Murat

    2015-04-01

    Sudden sensorineural hearing loss (SSNHL) is defined as hearing loss of at least 30 dB occurring within three days over at least three contiguous frequencies. The etiology of SSNHL cannot always be precisely determined; in such cases, this condition is termed idiopathic SSNHL (ISSNHL). This unique study investigates the relationship between ISSNHL and positive family history for ISSNHL. In total, 125 patients diagnosed with ISSNHL were retrospectively reviewed. The presence of ISSNHL in the family medical history and degree of kinship of family members diagnosed with ISSNHL were determined. For univariate analysis, a chi-squared test and/or Fisher's exact test was used for between-group comparisons of qualitative variables; a t-test was used for quantitative variables. Significant variables in the univariate analysis were introduced into stepwise logistic regression for multivariate analysis. Pfamily medical history of ISSNHL, whereas 83 (66.4%) did not. A statistically significant association between the development of ISSNHL and a family history of ISSNHL was observed (p<0.05). Our study supports an association between ISSNHL and genetic predisposition. Proving genetic susceptibility to ISSNHL will lead to improvements in the prediction, early diagnosis, and treatment of this disease.

  1. Sensorineural hearing loss after concurrent chemoradiotherapy in nasopharyngeal cancer patients

    International Nuclear Information System (INIS)

    Petsuksiri, Janjira; Sermsree, Achariyaporn; Thephamongkhol, Kullathorn; Keskool, Phawin; Thongyai, Kanthong; Chansilpa, Yaowalak; Pattaranutaporn, Pittayapoom

    2011-01-01

    Sensorineural hearing loss (SNHL) is one of the major long term side effects from radiation therapy (RT) in nasopharyngeal cancer (NPC) patients. This study aims to review the incidences of SNHL when treating with different radiation techniques. The additional objective is to determine the relationship of the SNHL with the radiation doses delivered to the inner ear. A retrospective cohort study of 134 individual ears from 68 NPC patients, treated with conventional RT and IMRT in combination with chemotherapy from 2004-2008 was performed. Dosimetric data of the cochlea were analyzed. Significant SNHL was defined as > 15 dB increase in bone conduction threshold at 4 kHz and PTA (pure tone average of 0.5, 1, 2 kHz). Relative risk (RR) was used to determine the associated factors with the hearing threshold changes at 4 kHz and PTA. Median audiological follow up time was 14 months. The incidence of high frequency (4 kHz) SNHL was 44% for the whole group (48.75% in the conventional RT, 37% with IMRT). Internal auditory canal mean dose of > 50 Gy had shown a trend to increase the risk of high frequency SNHL (RR 2.02 with 95% CI 1.01-4.03, p = 0.047). IMRT and radiation dose limitation to the inner ear appeared to decrease SNHL

  2. Sensorineural hearing loss and Mondini dysplasia caused by a deletion at locus DFN3.

    Science.gov (United States)

    Arellano, B; Ramírez Camacho, R; García Berrocal, J R; Villamar, M; del Castillo, I; Moreno, F

    2000-09-01

    To study a family with inner ear malformations and sensorineural hearing loss. Clinical, radiological, and genetic study of the members of a family with different degrees of sensorineural hearing loss. The males in the family manifested profound congenital hearing loss with severe inner ear malformations, while the only affected female had progressive hearing loss that had begun during puberty. Computed tomography showed inner ear malformations in both males, with enlarged internal auditory meatus and Mondini dysplasia. Genetic analysis disclosed a microdeletion at the locus DFN3 on chromosome X. A familial Mondini dysplasia is associated to a microdeletion at the deafness locus DFN3.

  3. STUDY OF HEARING OUTCOMES IN SUDDEN SENSORINEURAL HEARING LOSS TREATED WITH TISSUE PLASMINOGEN ACTIVATOR (TPA

    Directory of Open Access Journals (Sweden)

    Rama Krishna

    2015-09-01

    Full Text Available Sudden Sensorineural Hearing Loss (SSHNL is a clinical condition that requires immediate management. There are many treatment options, which may not always revert the hearing to normal. Not only recording the degree of hearing loss, but also establishing the concurrent dysfunction of saccule by VEMP has facilitated a new approach to treatment strategy. Recombinant tissue Plasminogen Activator ((rtPA proved its efficacy in stroke and subsequently considered an option in the management of ISSNHL. The curren t study, conducted at different centres, on 15 patients utilized rtPA. The results showed a promising trend when saccular pathology is also evident by VEMP in association with Hearing loss. We recommend use of rtPA as primary modality in cases of ISSNHL wi th Saccular involvement.

  4. Bilateral sudden sensorineural hearing loss as a presenting feature of systemic lupus erythematosus

    Science.gov (United States)

    Chawki, Sylvain; Aouizerate, Jessie; Trad, Selim; Prinseau, Jacques; Hanslik, Thomas

    2016-01-01

    Abstract Introduction: Sudden sensorineural hearing loss is an unusual presenting clinical feature of systemic lupus erythematosus. Case report: We report the case of a young woman who was admitted to hospital for sudden sensorineural hearing loss and hemophagocytic syndrome which was attributed to systemic lupus erythematosus on the basis of specific renal involvement, thrombocytopenia, and consistent autoantibodies. Favorable outcome was obtained on high-dose corticosteroids, and the hearing fully recovered. Discussion: Sudden sensorineural hearing loss in systemic lupus erythematosus is seemingly more frequently associated with severe systemic involvement and antiphospholipid antibodies may be present. Although management remains empirical, the high risk of permanent hearing impairment seems to justify emergency treatment with high-dose corticosteroids. When the clinical and laboratory criteria of antiphospholipid syndrome are met, antiplatelets agents or anticoagulation therapy shall be considered. PMID:27603334

  5. Prognostic factors for idiopathic sudden sensorineural hearing loss treated with hyperbaric oxygen therapy and intravenous steroids.

    Science.gov (United States)

    Hosokawa, S; Sugiyama, K; Takahashi, G; Takebayashi, S; Mineta, H

    2017-01-01

    This study evaluated the prognosis of idiopathic sudden sensorineural hearing loss when treated with hyperbaric oxygen therapy and intravenous steroids. The clinical data for 334 patients with idiopathic sudden sensorineural hearing loss treated by hyperbaric oxygen therapy and intravenous steroids at our hospital were retrospectively reviewed. These data included the initial averaged five-frequency hearing level, patient age, interval between onset of symptoms and treatment, vertigo as a complication, and co-existence of diabetes mellitus. The overall improvement rate was 69.2 per cent, including better improvement (25.5 per cent), good improvement (21.0 per cent) and fair improvement (22.7 per cent). Hyperbaric oxygen therapy appears to confer a significant additional therapeutic benefit when used in combination with steroid therapy for idiopathic sudden sensorineural hearing loss. If performed early, hyperbaric oxygen therapy may bring about hearing improvement in many patients who are unresponsive to initial therapy.

  6. Impact of mean platelet volume on the occurrence and severity of sudden sensorineural hearing loss.

    Science.gov (United States)

    Sagit, M; Kavugudurmaz, M; Guler, S; Somdas, M A

    2013-10-01

    We aimed to determine: (1) whether mean platelet volume was elevated in patients with sudden sensorineural hearing loss, compared with healthy controls; and (2) whether mean platelet volume level was related to hearing loss severity. The study included 31 patients with sudden sensorineural hearing loss and 31 age- and sex-matched, healthy controls. Peripheral venous blood samples were taken from subjects and mean platelet volume and levels of glucose, total cholesterol, high-density lipoprotein, low-density lipoprotein and triglyceride were measured. Mean platelet volume was significantly greater in the sudden sensorineural hearing loss group compared with the control group. However, there was no significant correlation between mean platelet volume level and hearing loss severity. Mean platelet volume, a determinant of platelet activation, is elevated in patients with sudden sensorineural hearing loss. To our knowledge, this is the first report investigating mean platelet volume levels in such patients. Our findings indirectly support the hypothesis of vascular impairment as a pathogenetic factor in sudden sensorineural hearing loss.

  7. [The treatment effects analysis of 164 patients with sudden sensorineural hearing loss].

    Science.gov (United States)

    Zhang, Wei; Xie, Wen; Xu, Hong; Liu, Yuehui

    2015-05-01

    To explore the effective treatment of sudden sensorineural hearing loss and factors affecting its prognosis. The clinical data and follow-up results of 164 patients with sudden sensorineural hearing loss were analyzed retrospectively. All the 164 patients were given intravenous vasodilator, neurotrophic drugs treatment, oral prednisone treatment, and intratympanic dexamethasone injection. All patients were divided into low frequency hearing loss type,intermediate frequency hearing loss, high frequency hearing loss, all frequency hearing loss and total deafness group. Pure tone hearing threshold test were performed before and 3 months after treatment. All patients and different groups were compared before and after treatment damage frequency of average air conduction and various frequency air conduction hearing. Analysis of gender, age, process and hearing curve type, frequency hearing of impaired before treatment, the symptoms with or without vertigo. All the patients' hearing improved after treatment. The treatment efficiency was 46.3%, and low frequency hearing improvements were better than the high frequency hearing. Including age, process, frequency hearing of impaired before treatment, with or without vertigo isindependent factors influencing its prognosis. Based on the regular treatment,oral and intratympanic injection glucocorticoid therapy are safe and effective for sudden hearing loss,The prognosis and age, course, impaired hearing before curve type, treatment frequency hearing level is closely related, with or without vertigo.

  8. Positive family history of idiopathic sudden sensorineural hearing loss.

    Science.gov (United States)

    Gäckler, A; Eickelmann, A K; Brors, D; Dazert, S; Epplen, J T; Kunstmann, E

    2010-12-01

    Idiopathic sudden sensorineural hearing loss (ISSNHL) is a heterogenic disease. Multiple factors influencing aetiology and prognosis are discussed. A retrospective clinical characterisation and analysis of family history of ISSNHL patients was performed to investigate influences on the disease. 186 inpatients diagnosed with ISSNHL were characterised by health records and a standardised questionnaire. Audiograms were observed. 75 controls that had never experienced an event of ISSNHL were questioned about family members being affected by ISSNHL. 63.4% of all patients could be assigned to at least one group with similar causes of ISSNHL (noise exposure, positive family history, infectious diseases, hypothyroidism and fibromuscular dysplasia). A positive family history for ISSNHL has not been reported so far. Therefore, we accentuated the characterisation of patients with positive family history. 21.4% affirmed a positive family history. In ten families, at least two family members were reported as ISSNHL patients. In comparison with patients with negative family history, they tend to be younger, experience more events of ISSNHL and show less improvement of hearing abilities under therapeutic treatment (non-significant). Differences intensified between smokers with positive family history and non-smokers with negative family history. Differences concerning average age were statistically significant (p = 0.001). Within 75 controls 11 families were reported with one member being affected by ISSNHL. In the control group we did not detect any family with more than one ISSNHL patient. The results indicated that patients with positive family history tend to have an aggravated course of ISSNHL. Further studies should help to confirm these results and to identify environmental or genetic factors leading to ISSNHL. This might support a better understanding of the aetiology of ISSNHL and offer new possibilities for prevention and therapy.

  9. The significance of routine laboratory parameters in patients with sudden sensorineural hearing loss.

    Science.gov (United States)

    Yasan, Hasan; Tüz, Mustafa; Yariktaş, Murat; Aynali, Giray; Tomruk, Onder; Akkuş, Omer

    2013-12-01

    There are several factors (viral infections, metabolic and ototoxic disorders etc.) accused for the development of sudden sensorineural hearing loss. Some prognostic factors (late onset of treatment etc.) had been evaluated in the literature. There is no sufficient data on the effect of routine laboratory parameters on the development and/or prognosis of sudden sensorineural hearing loss. The aim of this study is to investigate the effects of routine blood chemistry and hematological parameters on the development and prognosis of disease in patients with idiopathic sudden sensorineural hearing loss. One hundred and forty-seven patients with the diagnosis of idiopathic sudden sensorineural hearing loss followed up during the periods of 2000-2010 years were included in this study. One hundred and three septoplasty patients with no otologic complaints were enrolled as control group. Following the clinical and demographic evaluations, patients with idiopathic sudden sensorineural hearing loss and control groups, and patients treated successfully and patients with poor outcome were compared with each other. Data were analyzed by T test. All hematological and biochemical parameters were compared. Hemoglobin, hematocrit, white blood cell count, total and direct bilirubin, fasting blood glucose level and aspartate aminotransferase were significantly different between idiopathic sudden sensorineural hearing loss and control groups. There was no significantly different parameter between patients treated successfully and patients with poor outcome. Hemoglobin, hematocrit, white blood cell count, total and direct bilirubin, fasting blood glucose level and AST all can be risk factors for SHL, or they can be the result of undetermined pathology, because these parameters have no effect on the prognosis. Other routine parameters seem to have no effect on the development and/or prognosis of idiopathic sudden sensorineural hearing loss.

  10. Acute otitis media associated bilateral sudden hearing loss: case report and literature review.

    Science.gov (United States)

    Smith, A; Gutteridge, I; Elliott, D; Cronin, M

    2017-07-01

    Sudden sensorineural hearing loss is a rare otological condition with potential for dire outcomes including permanent hearing loss. Although the majority of cases are deemed idiopathic, bilateral sudden sensorineural hearing loss represents a rare subset typically related to systemic conditions, with higher morbidity and mortality. A controversial association with acute otitis media has been reported, with few bilateral cases published in the literature. A very rare case of bilateral sudden sensorineural hearing loss associated with acute otitis media is described, with a review of the literature. The limited evidence available suggests that acute otitis media with tinnitus and/or bacterial pathology may have an increased risk of sudden sensorineural hearing loss, which is consistent with the case described. Although there is no sufficiently powered published evidence to provide definitive treatment guidelines, the literature reviewed suggests that early myringotomy and antibiotics may greatly improve treatment outcomes.

  11. Antiviral treatment of idiopathic sudden sensorineural hearing loss : A prospective, randomized, double-blind clinical trial

    NARCIS (Netherlands)

    Stokroos, RJ; Albers, FWJ; Tenvergert, EM

    A subclinical viral labyrinthitis has been postulated in the literature to elicit Idiopathic Sudden Sensorineural Hearing Loss. An etiological role for the herpes virus family is assumed. Corticosteroids possess a limited beneficial effect on hearing recovery in ISSHL. In this study, the therapeutic

  12. Profile and Stability of Sensorineural Hearing Loss in Persistent Pulmonary Hypertension of the Newborn.

    Science.gov (United States)

    Walton, Joseph P.; Hendricks-Munoz, Karen

    1991-01-01

    This study found that 19 of 51 infants with persistent pulmonary hypertension of the newborn were diagnosed with sensorineural hearing loss, an incidence 25 times greater than that of intensive care unit infants in general. Treatment durations with mechanical ventilation were significantly longer for the hearing-impaired group compared to the…

  13. Bilateral sudden sensorineural hearing loss as an initial presentation of myelodysplastic syndrome.

    Science.gov (United States)

    Lee, Eun Jung; Yoon, Yong Joo

    2012-01-01

    This study reports an unusual case in which myelodysplastic syndrome presented bilateral sudden sensorineural hearing loss as the first symptom of the disease. The aural symptoms and signs such as tinnitus, dizziness, and hearing impairment of a hematologic disease are common. However, sudden hearing loss as the first manifestation of a hematologic disease is extremely rare. A 76-year-old woman presented with bilateral sudden hearing loss. The patient was found to have myelodysplastic syndrome during a workup for her hearing loss. Unfortunately, the patient's hearing loss did not improve after the medical treatment. Copyright © 2012 Elsevier Inc. All rights reserved.

  14. [A clinical analysis for sudden sensorineural hearing loss with acoustic neurinoma].

    Science.gov (United States)

    Gong, Qi-lin; Zhou, Ai-dong; Lin, Chang

    2013-04-01

    Retrospectively analyzed the clinical data of sudden sensorineural hearing loss with acoustic neuroma. The clinical data of 467 cases with sudden sensorineural hearing loss were collected between Jan, 2008 and Aug, 2012. Discussed the clinical data which were diagnosed as acoustic neuroma. In 467 cases of sudden sensorineural hearing loss, nine cases were diagnosed as acoustic neuromas (9 ears, 1.93%), two males and seven females, with a age range of 28 to 57 years. Among them, seven cases accompanied with tinnitus, seven cases with vertigo. The hearing results in nine cases, two cases were found to be mild, two were moderate, four were severe, and one was profound hearling loss respectively. Hearing was classified into five types according to audiogram shape (1 of up-sloping, 1 of down-sloping, 2 of mid-frequency, 1 of profound loss, 4 of flat audiogram). Eight cases had abnormal ABR, nine cases with ear ipsilateral stapedius reflex were completely not elicited, seven cases with health ear contralateral stapedius reflex were completely not elicited. Tumors were graded by Koos Grades according to size (7 of grade I, 1 of grade II, 1 of grade IV). Seven small acoustic neuroma was taken waiting strategies. Meanwhile, we use glucocorticoid and improve the microcirculation of the inner ear medication short-termly for these patients. Four patients' hearing were improved. The initial symptoms of some acoustic neuroma are sudden hearing loss, especially the small tumors in internal auditory canal. In order to prevent misdiagnosis, MRI and ABR should be performed as a routine test for sudden sensorineural hearing loss. It is necessary to give appropriate treatment to protecting hearing for the small acoustic neuroma patients whose first symptoms are diagnosed as sudden sensorineural hearing loss.

  15. High fibrinogen in peripheral blood correlates with poorer hearing recovery in idiopathic sudden sensorineural hearing loss.

    Directory of Open Access Journals (Sweden)

    Sho Kanzaki

    Full Text Available OBJECTIVES: We used hearing tests and peripheral blood sample analyses to characterize the pathology of idiopathic sudden sensorineural hearing loss (ISSNHL and to identify possible prognostic factors for predicting recovery of hearing loss. STUDY DESIGN: A retrospective, multicenter trial was conducted. METHODS: Two hundred three patients examined within 7 days after the onset of ISSNHL received prednisone with lipo-prostaglandin E1. Pure-tone auditory tests were performed before and after treatment with these drugs. Blood tests were performed on blood samples collected during the patients' initial visit to our clinic. RESULTS: In all patients, elevated white blood cell (WBC counts, fasting blood sugar levels, HgbA1c, and erythrocyte sedimentation rate (ESR significantly correlated with high hearing threshold measurements obtained on the initial visit. High fibrinogen levels, WBC counts, ESR, and low concentrations of fibrinogen degradation products (FDP were associated with lower hearing recovery rates. Additionally, different audiogram shapes correlated with different blood test factors, indicating that different pathologies were involved. CONCLUSIONS: High fibrinogen levels measured within seven days after ISSNHL onset correlated with poorer hearing recovery. This may be a consequence of ischemia or infections in the inner ear. The high WBC counts also observed may therefore reflect an immune response to inner ear damage induced by ischemic changes or infections. Our data indicate that therapeutic strategies should be selected based on the timing of initial treatment relative to ISSNHL onset.

  16. Phosphodiesterase 4D gene polymorphisms in sudden sensorineural hearing loss.

    Science.gov (United States)

    Chien, Chen-Yu; Tai, Shu-Yu; Wang, Ling-Feng; Hsi, Edward; Chang, Ning-Chia; Wang, Hsun-Mo; Wu, Ming-Tsang; Ho, Kuen-Yao

    2016-09-01

    The phosphodiesterase 4D (PDE4D) gene has been reported as a risk gene for ischemic stroke. The vascular factors are between the hypothesized etiologies of sudden sensorineural hearing loss (SSNHL), and this genetic effect might be attributed for its role in SSNHL. We hypothesized that genetic variants of the PDE4D gene are associated with susceptibility to SSNHL. We conducted a case-control study with 362 SSNHL cases and 209 controls. Three single nucleotide polymorphisms (SNPs) were selected. The genotypes were determined using TaqMan technology. Hardy-Weinberg equilibrium (HWE) was tested for each SNP, and genetic effects were evaluated according to three inheritance modes. We carried out sex-specific analysis to analyze the overall data. All three SNPs were in HWE. When subjects were stratified by sex, the genetic effect was only evident in females but not in males. The TT genotype of rs702553 exhibited an adjusted odds ratio (OR) of 3.83 (95 % confidence interval = 1.46-11.18) (p = 0.006) in female SSNHL. The TT genotype of SNP rs702553 was associated with female SSNHL under the recessive model (p = 0.004, OR 3.70). In multivariate logistic regression analysis, TT genotype of rs702553 was significantly associated with female SSNHL (p = 0.0043, OR 3.70). These results suggest that PDE4D gene polymorphisms influence the susceptibility for the development of SSNHL in the southern Taiwanese female population.

  17. Endothelial dysfunction in patients with sudden sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Nezamoddin Berjis

    2016-01-01

    Full Text Available Background: Endothelial dysfunction probably has a role in the etiology of sudden sensorineural hearing loss (SSNHL. The aim of this study was determining of the relationship between endothelial dysfunction and SSNHL. Materials and Methods: In a case-control study, 30 patients with SSNHL and 30 otherwise healthy age and sex-matched controls were studied. Demographic data gathered included age, gender, family history of SSNHL, and history of smoking, cardiovascular disease, hypertension, diabetes, and dyslipidemia. Laboratory data included measurement of hemoglobin, fasting blood sugar (FBS and lipid profile. Endothelial function was assessed by measuring flow-mediated dilation (FMD. Results: The two groups were the same in age (47.9 ± 9.3 and 48.1 ± 9.6 years, P = 0.946 with female/male ratio of 1:1 in both groups. Diabetes and dyslipidemia were more frequent in patients than controls (20% vs. 0%, P = 0.024. Brachial artery diameter was greater in patients than controls before (4.24 ± 0.39 vs. 3.84 ± 0.23 mm, P < 0.001 and after ischemia (4.51 ± 0.43 vs. 4.28 ± 0.27 mm, P = 0.020, but FMD was lower in patients than controls (6.21 ± 3.0 vs. 11.52 ± 2.30%, P < 0.001. Binary logistic regression analysis showed that FMD was associated with SSNHL independent from FBS and lipid profile (odds ratio [95% confidence interval] =0.439 [0.260-0.740], P = 0.002. Conclusion: Endothelial dysfunction, among other cardiovascular risk factors, is associated with SSNHL. This association is independent from other cardiovascular risk factors including diabetes and dyslipidemia.

  18. The clinical characteristics and treatment for sudden sensorineural hearing loss with vestibular schwannoma.

    Science.gov (United States)

    Lin, Chang; Gong, Qilin; Zuo, Wenjing; Zhang, Rong; Zhou, Aidong

    2015-04-01

    The aim of this study is to analyze the clinical characteristics and treatment of sudden sensorineural hearing loss (SSNHL) patients with vestibular schwannoma (VS). The clinical features of the VS patients were explored by retrospectively analyzing the clinical data from 542 cases of SSNHL patients between January 2008 and March 2013. There were 10 cases (10 ears) diagnosed with VS in 542 cases of SSNHL patients (10 ears, 1.85 %), 3 males, 7 females, with a range of 28-57 years. Among all the cases, eight patients with abnormal ABR, ten with ear ipsilateral stapedius reflexes which were completely not elicited and seven patients with healthy ear contralateral stapedius reflexes which were completely not elicited. Neuromas were classified by Koos grades according to size (8 of grade I, 1 of grade II, 1 of grade IV). Eight small VS  patients were taken waiting and MRI therapy strategies. Meanwhile, we used glucocorticoid treatment and timely and short-term medication to improve the microcirculation of the inner ear for these patients. And four cases' hearing was improved. Some vestibular schwannomas have SSNHL as initial symptoms, especially the small ones in internal auditory canal. To prevent misdiagnosis or leak-diagnosis, MRI should be performed as a routine test for SSNHL, and ABR is sometimes necessary for SSNHL patients. It is also necessary to give appropriate treatment to protect hearing of the small vestibular schwannoma patients whose first symptoms are diagnosed as SSNHL in acute phase.

  19. The clinical analysis of bilateral successive sudden sensorineural hearing loss.

    Science.gov (United States)

    Wang, Yaowen; Zhang, Litao; Zhang, Jianhua; Zhang, Xuqun; Zhang, Weimin; Chen, Xing; Tang, Shixiong

    2016-11-01

    The objective of this study is to investigate the clinical characteristics of successive bilateral sudden sensorineural hearing loss (SSNHL) with an interval of more than 1 year to aid the evaluation and management of successive bilateral SSNHL (BSSNHL). 14 successive BSSNHL patients and 118 unilateral SSNHL patients with severe and profound hearing loss were reviewed retrospectively. Information about successive BSSNHL was collected included demographics, the intervals between the attacks of bilateral ears, the past medical history, inducing factors, accompanying symptoms, pure-tone tests, blood tests, b-ultrasound examinations of vertebral artery and carotid artery, and medical interventions. And the comparison of improvement rate was made between successive BSSNHL and unilateral SSNHL. SPSS 15.0 was used to analyze the data. In successive BSSNHL, there were six males and eight females; the average aged was 49.86 ± 15.45 years (20-73 years). The interval of the two attacks was 11.43 ± 12.07 years (1-50 years) on average. The onset of treatment was 18.86 ± 12.71 days. Tinnitus was seen in 100 % of the patients, followed by vertigo in 42.85 %, and ear fullness in 21.43 %. 3 of 14 patients described obvious inducing factor: tiredness. Five patients (35.71 %) had hypertension histories, three (21.43 %) had diabetes histories, two (14.29 %) had surgery histories, one (7.14 %) was with depression history, one (7.14 %) was with coronary heart disease history. 30 % (3/10) patients were with atherosclerotic plaque in carotid artery. 4 (28.57 %) patients were with high blood sugar. 8 patients (57.14 %) were with high blood lipids. Thyroid function tests were positive in 27.27 % (3/11) patients. No abnormality was found in antinuclear antibodies titer. The percentage of profound and severe hearing loss were 71.43 and 78.57 % in the recently affected ear and contralateral ear separately. The PTAs of the recently affected ear were 77.14 ± 27.12

  20. Ampleness of head movements of children and adolescents with sensorineural hearing loss.

    Science.gov (United States)

    de Souza Melo, Renato

    2017-02-01

    Head movements are controlled by the vestibular system. Children with sensorineural hearing loss can present restrictions in ampleness of head movements due to damage in the vestibule-cochlear systems, resulting from injury in the inner ear. To evaluate the ampleness of head movements of children with normal hearing and children with sensorineural hearing loss and compare data between groups. Cross-sectional study that evaluated the ampleness of head movements of 96 students, being 48 with normal hearing and 48 with sensorineural hearing loss, of both sexes, with aged between 7 and 18 years old. The performance of ampleness of head movements was analyzed by a manual goniometric evaluation, according the references proposed by Marques. To the statistical analysis we used the t-Student test in case of normality of the data or the Mann-Whitney test when did not applied the suppositions of normality. Hearing loss children showed less mean in ampleness of all movements of head compared to normal hearing children, pointing difference to movements of flexion (p = 0,001), lateral inclination to the right (p = 0,025) and lateral rotation to the left (p = 0,021). Hearing loss children showed reduction in the ampleness of these head movements: flexion, lateral inclination to the right and lateral rotation to the left compared to normal hearing children. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  1. Predictors of hearing recovery in patients with severe sudden sensorineural hearing loss.

    Science.gov (United States)

    Weiss, Daniel; Böcker, Armin Julius; Koopmann, Mario; Savvas, Eleftherios; Borowski, Matthias; Rudack, Claudia

    2017-04-04

    Sudden sensorineural hearing loss (SSHL) is a disease, which severely affects the patient's social and relational life. The underlying pathomechanisms have not been finally clarified yet and outcome is not predictable. We conducted a retrospective study in order to identify parameters that influence hearing recovery. The data base contains results of basic otoneurological tests and clinical parameters of 198 patients with idiopathic SSHL of at least 60 dB in at least four frequencies, diagnosed and treated at the University Hospital of Münster, Germany, between 1999 and 2015. Hearing recovery was measured by pure tone audiometry. Multivariate linear and logistic regression analyses indicate that the chance as well as the magnitude of hearing recovery is higher for patients with normal caloric testing than for patients with pathological caloric testing. However, for the subgroup of patients who attained a hearing recovery, the caloric testing result was not found to influence the magnitude. Instead, the magnitude was noticeably lower for patients within this subgroup who had a previous hearing loss. Furthermore, we found indications that the magnitude is higher for men than for women and that receiving a high-dose steroid therapy is associated with a higher chance and magnitude of a hearing recovery. We conclude that SSHL associated with disorders of the vestibular system or previous hearing loss represent special sub-entities of SSHL that may be caused by unique pathophysiological mechanisms and are associated with worse outcome. Furthermore, our data support the importance of elevated dosage of steroids in SSHL therapy.

  2. Secondary superficial siderosis of the central nervous system in a patient presenting with sensorineural hearing loss

    International Nuclear Information System (INIS)

    Lemmerling, M.; De Praeter, G.; Mollet, P.; Mortele, K.; Kunnen, M.; Mastenbroek, G.

    1998-01-01

    We present a 50-year-old man who was investigated for sensorineural hearing loss. On MRI of the brain superficial siderosis of the central nervous system was seen, while MRI of the spine revealed an ependymoma of the cauda equina. This case illustrates the importance of performing T2-weighted imaging of the brain and posterior fossa when sensorineural hearing loss is present. Spine imaging is mandatory when superficial siderosis of the brain is diagnosed without identification of a bleeding source in the brain. (orig.)

  3. Sudden sensorineural hearing loss: a review of diagnosis, treatment, and prognosis.

    Science.gov (United States)

    Kuhn, Maggie; Heman-Ackah, Selena E; Shaikh, Jamil A; Roehm, Pamela C

    2011-09-01

    Sudden sensorineural hearing loss (SSNHL) is commonly encountered in audiologic and otolaryngologic practice. SSNHL is most commonly defined as sensorineural hearing loss of 30 dB or greater over at least three contiguous audiometric frequencies occurring within a 72-hr period. Although the differential for SSNHL is vast, for the majority of patients an etiologic factor is not identified. Treatment for SSNHL of known etiology is directed toward that agent, with poor hearing outcomes characteristic for discoverable etiologies that cause inner ear hair cell loss. Steroid therapy is the current mainstay of treatment of idiopathic SSNHL in the United States. The prognosis for hearing recovery for idiopathic SSNHL is dependent on a number of factors including the severity of hearing loss, age, presence of vertigo, and shape of the audiogram.

  4. Metabolic Syndrome Increases the Risk of Sudden Sensorineural Hearing Loss in Taiwan: A Case-Control Study.

    Science.gov (United States)

    Chien, Chen-Yu; Tai, Shu-Yu; Wang, Ling-Feng; Hsi, Edward; Chang, Ning-Chia; Wu, Ming-Tsang; Ho, Kuen-Yao

    2015-07-01

    Sudden sensorineural hearing loss has been reported to be associated with diabetes mellitus, hypertension, and hyperlipidemia in previous studies. The aim of this study was to examine whether metabolic syndrome increases the risk of sudden sensorineural hearing loss in Taiwan. A case-control study. Tertiary university hospital. We retrospectively investigated 181 cases of sudden sensorineural hearing loss and 181 controls from the Department of Otorhinolaryngology, Kaohsiung Medical University Hospital, in southern Taiwan from 2010 to 2012, comparing their clinical variables. We analyzed the relationship between metabolic syndrome and sudden sensorineural hearing loss. Metabolic syndrome was defined according to the National Cholesterol Education Program Adult Treatment Panel III with Asian modifications. The demographic and clinical characteristics, audiometry results, and outcome were reviewed. Subjects with metabolic syndrome had a 3.54-fold increased risk (95% confidence interval [CI] = 2.00-6.43, P diabetes mellitus, hypertension, and hyperlipidemia. With increases in the number of metabolic syndrome components, the risk of sudden sensorineural hearing loss increased (P for trend Vertigo was associated with a poor outcome (P = .02; 95% CI = 1.13~5.13, adjusted odds ratio = 2.39). The hearing loss pattern may influence the outcome of sudden sensorineural hearing loss (P Vertigo and total hearing loss were indicators of a poor outcome in sudden sensorineural hearing loss. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2015.

  5. The relationship between hearing loss and vestibular dysfunction in patients with sudden sensorineural hearing loss.

    Science.gov (United States)

    Niu, Xiaorong; Zhang, Yan; Zhang, Qing; Xu, Xinda; Han, Peng; Cheng, Ying; Gao, Ying; Zhang, Rui; Yang, Yintong; Chen, Zichen; Hu, Juan; Chen, Yanfei; Xu, Min

    2016-01-01

    To investigate the relationship between hearing loss and vestibular dysfunction in patients with sudden sensorineural hearing loss (SSHL). Clinical data including the symptom of vertigo of 149 SSHL patients were investigated retrospectively. Pure tone audiometry, ocular vestibular-evoked myogenic potential (oVEMP) and cervical vestibular-evoked myogenic potential (cVEMP) evoked by air-conducted sound (ACS), and caloric test were employed for cochlear and vestibular function assessment. The relationship between hearing level and vestibular dysfunction was analyzed. The pure tone averages (PTAs) (mean ± SD) of SSHL patients with and without vertigo were 88.81 ± 21.74 dB HL and 72.49 ± 21.88 dB HL (Z = -4.411, p = 0.000), respectively. The PTAs of SSHL patients with abnormal and normal caloric test were 84.71 ± 22.54 dB HL and 70.41 ± 24.07 dB HL (t = -2.665, p = 0.009), respectively. Conversely, vertigo and abnormal caloric results also happened more frequently in patients with profound hearing loss. However, no consistent tendency could be found among vestibular evoked myogenic potentials (VEMPs) responses or hearing loss. SSHL patients with vertigo or abnormal caloric test displayed worse hearing loss; and vice versa, vertigo and abnormal caloric results happened more frequently in SSHL patients with profound hearing loss.

  6. Does acute sensorineural deafness befall to urgent conditions?

    Directory of Open Access Journals (Sweden)

    Čvorović Ljiljana

    2009-01-01

    Full Text Available Background/Aim. Idiopathic sudden sensorineural hearing loss (ISSHL is one of the most controversial issues in otology. The aim of this study was to determine whether a delay in treatment has any influence on hearing recovery in ISSHL. Method. This study was designed as a retrospective evaluation of an electronic patient data base of the University Hospital Zürich from January 1995 to August 2006. Five hundred and forty one patients with a sudden hearing loss were identified. The standard treatment was carbogen inhalation (95% O2 and 5% CO2 eight times per day in the duration of 30 minutes and prednisone orally (100 mg in one morning dose for 7 days. Factor that was analyzed included the interval between the onset of symptoms and the beginning of the treatment. The initial hearing loss was described using the pure tone average (PTA in dB hearing level at 4 frequencies (0.5, 1, 2 and 4 kHz. Hearing gain was expressed either as absolute hearing gain (dB values from initial PTA minus dB values from final PTA or as relative hearing gain (absolute hearing gain divided by initial PTA minus baseline PTA × 100. Significant recovery of hearing was defined as the final PTA ≤ 30 dB (or same as PTA of the opposite ear. Results. An absolute hearing gain between the initial audiogram and the final audiogram was 15.1 dB. The mean relative hearing gain was 47%. Three hundred one (57% patients had a significant recovery of hearing, and 228 (43% had not. If the patients received treatment in the first 24 hours after onset of symptoms, then the rate of significant recovery was 56%, and no significant difference existed between this group and the patients who received the therapy after 24 hours, but within seven days (χ2 = 0.007, DF = 1, p > 0.01. Conclusion. These results suggest that it is not critical to begin the treatment of ISSHL immediately as an emergency, but within seven days.

  7. Lyme disease associated with sudden sensorineural hearing loss: case report and literature review.

    Science.gov (United States)

    Peeters, Natalie; van der Kolk, Brigitta Y M; Thijsen, Steven F T; Colnot, David R

    2013-07-01

    Is sudden sensorineural hearing loss associated with Lyme disease in adults? A retrospective case report and a systematic literature search in PubMed and Embase were performed. We describe a patient presenting with sudden sensorineural hearing loss, followed by a facial paralysis, which could be attributed to Lyme disease confirmed by positive serology and a positive immunoblot. She was successfully treated with ceftriaxone, with recovery of the facial paralysis, although no recovery of the hearing loss was observed. A systematic literature search resulted in 4 relevant and valid articles revealing that confirmed positive serology for Borrelia burgdorferi varies from 0% to 21.3%, suggesting active Lyme disease as a cause in patients with sudden sensorineural hearing loss. Two studies demonstrated a significantly higher incidence of confirmed positive serology for Borrelia burgdorferi as compared with the incidence in the general local population. Literature suggests that sudden sensorineural hearing loss may coincide with Borrelia burgdorferi infection. A higher incidence of confirmed positive serology for Borrelia burgdorferi in patients with sudden deafness seems to be depending on the country and on the tests used to confirm Lyme disease. This should be taken into account if serologic testing for Lyme disease in patients with sudden deafness is considered.

  8. Correlation between audiovestibular function tests and hearing outcomes in severe to profound sudden sensorineural hearing loss.

    Science.gov (United States)

    Wang, Chi-Te; Huang, Tsung-Wei; Kuo, Shih-Wei; Cheng, Po-Wen

    2009-02-01

    This study investigated whether audiovestibular function tests, namely auditory brain stem response (ABR) and vestibular-evoked myogenic potential (VEMP) tests were correlated to hearing outcomes after controlling the effects of other potential confounding factors in severe to profound sudden sensorineural hearing loss (SSHL). Eighty-eight patients with severe to profound SSHL were enrolled in this study. Pretreatment hearing levels, results of audiovestibular function tests, and final hearing outcomes were recorded from retrospective chart reviews. Other factors, including age, gender, delay of treatment, vertigo, diabetes mellitus, and hypertension, were collected as well. Comparative analysis between multiple variables and hearing outcomes was conducted using the cumulative logits model in overall subjects. Further, multivariate analysis of prognostic factors was conducted in the stratified groups of severe (70 dB HL 90 dB HL) SSHL. Multivariate analysis showed that pretreatment hearing levels, presence of vertigo, and results of ABR and VEMP testing were significant outcome predictors in the overall subjects. Stratification analysis demonstrated that both the presence of ABR and VEMP waveforms were significantly correlated with better hearing outcomes in the group of severe SSHL [ABR: adjusted odds ratio (aOR) = 14.7, 95% confidence interval (CI) = 1.78 to 122, p = 0.01; VEMP: aOR = 5.91, 95% CI = 1.18 to 29.5, p = 0.03], whereas the presence of vertigo was the only significant negative prognostic factor in the group of profound SSHL (aOR = 0.24, 95% CI = 0.06 to 0.95, p = 0.04). Other variables, including age, gender, diabetes mellitus, hypertension, and delay of treatment, were not significantly related to hearing outcomes in both groups (p > 0.05). A predictive hearing recovery table with the combined ABR and VEMP results was proposed for the group of severe SSHL. ABR and VEMP tests should be included in the battery of neurootological examinations in

  9. Idiopathic sudden sensorineural hearing loss: cardiovascular risk factors do not influence hearing threshold recovery.

    Science.gov (United States)

    Ciorba, A; Hatzopoulos, S; Bianchini, C; Iannini, V; Rosignoli, M; Skarzynski, H; Aimoni, C

    2015-04-01

    Previous studies have suggested that risk factors for ischaemic vascular disease, such as cigarette smoking, hypertension and hyperlipidaemia, can also be considered risk factors for the development of idiopathic sudden sensorineural hearing loss (ISSNHL). In this study, we have evaluated the hypothesis that these factors can influence hearing threshold recovery in patients affected by ISSNHL. A total of 141 subjects who suffered an episode of ISSNHL were included. All subjects were assessed with tonal audiometry, auditory brainstem responses and MRI to exclude retrocochlear pathology. Hearing tests were conducted at ISSNHL onset (t = 0) and after 30 days. Patients were divided into three classes according to the presence/absence of one or more cardiovascular risk factors including: history of smoking, total serum cholesterol/triglycerides, history of hypertension and diabetes mellitus. Values of hearing threshold recovery were estimated and comparisons were conducted across the three risk factor classes. 75% of patients affected by ISSNHL showed a threshold recovery. However, the threshold recovery was found to be class-independent (average recovery value of 18 dB HL per classes) and also independent of age and gender. Even if cardiovascular risk factors have been found to be involved in the pathogenesis of ISSNHL, the present study suggests that these factors do not have any significant influence on the threshold recovery in ISSNHL.

  10. Prognostic Value of Labyrinthine 3D-FLAIR Abnormalities in Idiopathic Sudden Sensorineural Hearing Loss.

    Science.gov (United States)

    Lee, J I; Yoon, R G; Lee, J H; Park, J W; Yoo, M H; Ahn, J H; Chung, J W; Park, H J

    2016-12-01

    According to recent research, modern MR imaging can detect the presense of abnormalities on labyrinthine. Our aim was to report the patterns and prognostic role of abnormal findings on labyrinthine imaging in patients with sudden sensorineural hearing loss. This study comprised 113 patients who were diagnosed with unilateral sudden sensorineural hearing loss and underwent 3T MR imaging, including pre-/postcontrast 3D fluid-attenuated inversion recovery and T1-weighted imaging. We analyzed abnormalities on MR imaging and correlated them with audiometric results. Thirty-one (27%) patients showed abnormal findings on labyrinthine MR imaging in the affected ear. The initial/final hearing levels of the MRI+ group (91 ± 25/73 ± 27 dB hearing loss) were significantly worse than those of the MRI- group (69 ± 30/48 ± 24 dB hearing loss). The incidence of abnormalities on labyrinthine MR imaging was significantly lower (3 of 40, 8%) in 40 patients with initial mild-to-moderate hearing loss than in those with profound hearing loss (16 of 34, 47%). Considering hearing improvement by the Siegel criteria, the rate of complete or partial recovery was significantly higher in the MRI- group (34%) than in the MRI+ group (10%). In patients with initial severe or profound hearing loss, the MRI- group showed greater hearing improvement (38 ± 21 dB) than the MRI+ group (23 ± 22 dB). Abnormalities on labyrinthine MR imaging were found in 27% of patients with sudden sensorineural hearing loss. The initial hearing loss was worse in the MRI+ group than in the MRI- group. In patients with initial severe and profound hearing loss, the presence of abnormalities on labyrinthine MR imaging indicated a poor prognosis. © 2016 by American Journal of Neuroradiology.

  11. Clinical and experimental studies on the sensorineural hearing loss caused by irradiation

    International Nuclear Information System (INIS)

    Yamamoto, Matsunori

    1979-01-01

    In our study, 43 patients whose ears were presumably irradiated, were examined periodically before and after the irradiation; 10 were found to be affected by the sensorineural hearing loss, and these 10 were classified into two types. Type I; Patients of this group had a hearing impairment during or soon after irradiation and usually revealed marked deterioration of bone conduction in high frequencies accompanied by worsening of air conduction in low frequencies. The impaired hearing of this group soon recovered to the pre-treatment level. Type II; Patients of this group developed a slow progressive sensorineural hearing loss. They noticed the deafness with tinnitus several months after irradiation. To clarify the mechanism of the sensorineural hearing loss, histopathological investigations were done using nembutal anesthetized guinea pigs which were irradiated in the confined ear region unilaterally. We also examined histopathologically one human temporal bone belonging to a patient who had been irradiated for middle ear carcinoma. The histopathology of the guinea pigs and human case revealed the following conclusions: Type I hearing loss may be caused by toxic labyrinthitis secondary to the radiation otitis media or by the aseptic labyrinthitis as the result of hyperemia and increased permeability of the irradiated blood vessels in the cochlea. Type II hearing loss may be caused by the late rediation response of the cochleal blood vessels i.e. by the vasculitis which gives rise to obliteration of the vascular lumen and affects the blood supply of the hair cells. (author)

  12. Therapeutic effect of Intra-Tympanic Dexamethasone-Hyaluronic Acid Combination in Sudden Sensorineural Hearing Loss.

    Science.gov (United States)

    Rogha, Mehrdad; Kalkoo, Amin

    2017-09-01

    Hearing loss is fairly a common disorder which is usually treated with corticosteroids via systemic administration and/or intra-tympanic injection. This study aimed to compare the effectiveness of intra-tympanic injections of dexamethasone with its combination with hyaluronic acid in patients with sudden sensorineural hearing loss. In this clinical trial, 40 patients were randomly assigned to two groups; in the first group, 20 patients received 2.4 mg intra-tympanic dexamethasone, while in the second group patients received injections of 2.4 mg of dexamethasone plus 2 mg of hyaluronic acid in combination. Patients in both groups were injected every other day to a total of three injections. The hearing status of patients was evaluated by pure tone audiometry (bone conduction threshold) before and 2 weeks after the intervention. Assessment of hearing threshold before and after treatment in the two groups showed a significant difference between hearing thresholds at frequencies of 4,000 to 8,000 Hz (Psudden sensorineural hearing loss may be more effective than dexamethasone alone. Because hyaluronic acid lacks certain side effects, and also makes it possible to reduce the steroid dose, we recommend the use of this combination in the treatment of patients with sudden sensorineural hearing loss.

  13. Chinese herbal medicine for idiopathic sudden sensorineural hearing loss: a systematic review of randomised clinical trials.

    Science.gov (United States)

    Su, C-X; Yan, L-J; Lewith, G; Liu, J-P

    2013-12-01

    Idiopathic sudden sensorineural hearing loss has great impact on quality of life. Many clinical trials using Chinese herbal medicine for idiopathic sudden sensorineural hearing loss have been conducted and reported beneficial results. However, there is no critical appraised evidence on efficacy and safety of Chinese herbal medicine for idiopathic sudden sensorineural hearing loss to inform clinical use. To assess the beneficial effect and safety of Chinese herbal medicine for idiopathic sudden sensorineural hearing loss. Systematic review of randomised clinical trials. Seven electronic databases and two trial registries were searched for all eligible trials from inception to January 2013. Two authors independently selected trials and extracted data. The Cochrane risk of bias tool was utilised to assess the methodological quality of the included trials. revman 5.2 software was applied for data analysis with effect estimate presented as risk ratio and mean difference with its 95% confidence interval. Forty-one randomised clinical trials involving 3560 participants were identified. Five kinds of Chinese herbal medicine were trialed. All trials compared conventional therapies of steroids, vasodilators, anticoagulants, nutritional supplements or hyperbaric oxygen with or without herbal medicine. No trial was identified that compared herbal medicine alone with placebo. No trial was identified that blinded the participants or the observers to their herbal medication. Only one trial adequately reported its method of randomisation. No trial reported the sample size calculated to show an effect. All trials had material other defects giving a high likelihood of bias. Because of the overall poor quality of all 41 trials, it was concluded that there was no level-one evidence to support the use of Chinese herbal medicine, alone or in addition to conventional therapies, to improve the hearing in adults with idiopathic sudden sensorineural hearing loss. Two trials reported adverse

  14. Cochlear implantation is a therapeutic option for superficial siderosis patients with sensorineural hearing loss.

    Science.gov (United States)

    Omichi, R; Kariya, S; Maeda, Y; Nishizaki, K

    2016-04-01

    Superficial siderosis is a rare disease that results from chronic bleeding in the subarachnoid space. Haemosiderin deposits throughout the subpial layers of the brain and spinal cord lead to progressive sensorineural hearing loss, which is seen in 95 per cent of patients with superficial siderosis. The impact of cochlear implantation on the quality of life of superficial siderosis patients is under debate. A 38-year-old male with superficial siderosis presented with bilateral progressive sensorineural hearing loss. The patient underwent cochlear implantation and his quality of life was improved as evaluated by the Abbreviated Profile of Hearing Aid Benefit inventory. The remarkable improvement in Abbreviated Profile of Hearing Aid Benefit scores shown in this study indicates that cochlear implantation leads to a better quality of life in superficial siderosis patients.

  15. Use of vasodilators in idiopathic sudden sensorineural hearing loss : A systematic review

    NARCIS (Netherlands)

    De Sousa, Melissa Bravenboer; Cazemier, Selma; Stegeman, Inge; Thomeer, Hans

    2017-01-01

    To compare the effect of vasodilators with that of corticosteroids in patients with idiopathic sudden sensorineural hearing loss (ISSHL). A search in PubMed, Cochrane, and Embase was conducted. Two reviewers screened the data sources to identify articles that comply with predefined inclusion

  16. Megadolicho basilar artery as a cause of asymmetrical sensorineural hearing loss - case report

    Directory of Open Access Journals (Sweden)

    Melo, Antonio Antunes

    2011-07-01

    Full Text Available Introduction: At the differentiated diagnosis of asymmetrical sensorineural hearing losses, vascular disorders are present, one of which is megadolicho basilar artery. This disease is generally asymptomatic, and when symptoms are found, they can be caused by a compression or ischemia. Clinically, sensorineural hearing loss, tinnitus, headache, facial hypoesthesia, trigeminal neuralgia, vertigo, diplopia and facial palsy, among others, are likely to occur. The image examination of choice for its diagnosis is nuclear magnetic resonance. The megadolicho basilar artery therapy can be surgical or conservative, according to the associated findings. A multidisciplinary approach, including a neurologist, neurosurgeon and an otorhinolaryngologist is recommended for a proper administration of the case. Objective: Report the case of a patient with asymmetrical sensorineural hearing loss, diagnosed of megadolicho basilar artery. Case report: JBS, 57-year-old white male with a history of asymmetrical sensorineural hearing loss and bilateral whistle-like tinnitus for several years. The otorhinolaryngologic evaluation, including otoscopy, anterior rhinoscopy and oral pharynx, was normal. Final Comments: The treatment consisted in following up with the patient, controlling the tinnitus by drugs and using an individual sound amplification apparatus on the left ear.

  17. Progression of low-frequency sensorineural hearing loss (DFNA6/14-WFS1).

    NARCIS (Netherlands)

    Pennings, R.J.E.; Bom, S.J.H.; Cryns, K.; Flothmann, K.; Huygen, P.L.M.; Kremer, J.M.J.; Camp, G. van; Cremers, C.W.R.J.

    2003-01-01

    OBJECTIVE: To assess the audiometric profile and speech recognition characteristics in affected members of 2 families with DFNA6/14 harboring heterozygous mutations in the WFS1 gene that cause an autosomal dominant nonsyndromic sensorineural hearing impairment trait. DESIGN: Family study. SETTING:

  18. Language Development and Impairment in Children with Mild to Moderate Sensorineural Hearing Loss

    Science.gov (United States)

    Halliday, Lorna F.; Tuomainen, Outi; Rosen, Stuart

    2017-01-01

    Purpose: The goal of this study was to examine language development and factors related to language impairments in children with mild to moderate sensorineural hearing loss (MMHL). Method: Ninety children, aged 8-16 years (46 children with MMHL; 44 aged-matched controls), were administered a battery of standardized language assessments, including…

  19. Sudden sensorineural hearing loss is associated with chronic rhinosinusitis: population-based study.

    Science.gov (United States)

    Hung, S H; Lin, H C; Kao, L T; Wu, C S; Chung, S D

    2016-06-01

    This study aimed to evaluate the association of chronic rhinosinusitis with sudden sensorineural hearing loss using a population-based database. Sampled subject data were obtained from the Taiwan Longitudinal Health Insurance Database 2000. A total of 3325 patients with sudden sensorineural hearing loss were identified and 9975 controls were randomly selected. A conditional logistic regression was used to calculate the odds ratio for having been previously diagnosed with chronic rhinosinusitis, for cases and controls. The adjusted odds ratio of having prior chronic rhinosinusitis among cases compared to controls was 1.36 (95 per cent confidence interval = 1.16-1.60). The significant relationship between sudden sensorineural hearing loss and chronic rhinosinusitis was most pronounced among those patients aged 44 years or less (compared to controls) (odds ratio = 2.18; 95 per cent confidence interval = 1.63-2.92). However, the significant relationship between sudden sensorineural hearing loss and prior chronic rhinosinusitis was not sustained for patients older than 60 years compared to controls.

  20. Magnetic resonance imaging of the inner ear in patients with idiopathic sudden sensorineural hearing loss

    NARCIS (Netherlands)

    Stokroos, RJ; Albers, FWJ; Krikke, AP; Casselman, JW

    1998-01-01

    Although gadolinium-enhanced magnetic resonance imaging (Gd-MRI) has been used to indicate the presence of a subclinical labyrinthitis in patients with idiopathic sudden sensorineural hearing loss (ISSHL), its sensitivity in daily clinical practice is unknown. We describe Gd-MRI findings in 27 ISSHL

  1. Sudden sensorineural hearing loss with positional vertigo: Initial findings of positional nystagmus and hearing outcomes.

    Science.gov (United States)

    Kim, Chang-Hee; Shin, Jung Eun; Yang, Young Soo; Im, Donghyuk

    2016-10-01

    To investigate the initial findings of positional nystagmus in patients with sudden sensorineural hearing loss (SSNHL) and positional vertigo, and to compare hearing improvement among patients with different types of positional nystagmus. The characteristics of positional nystagmus upon initial examination were analysed, and the initial mean pure-tone audiometry (PTA) threshold was compared with that at three months after treatment. Forty-four SSNHL patients with concomitant positional vertigo were included. Positional nystagmus was classified into five subgroups; persistent geotropic direction-changing positional nystagmus (DCPN) in head-roll test (HRT) and negative Dix-Hallpike test (DHT), persistent apogeotropic DCPN in HRT and negative DHT, positive DHT and negative HRT, persistent geotropic DCPN in HRT and positive DHT, and persistent apogeotropic DCPN in HRT and positive DHT. PTA threshold improvement was significantly greater in SSNHL patients with negative DHT than with positive DHT (p = 0.027). When geotropic DCPN was elicited by HRT, the nystagmus was persistent, which suggests that alteration of specific gravity of the endolymph, rather than the lateral canal canalolithiasis, may be a cause of this characteristic positional nystagmus. Positive DTH may be a prognostic factor for worse hearing recovery among patients with SSNHL and positional vertigo.

  2. Increased risk of sudden sensorineural hearing loss in patients with depressive disorders: population-based cohort study.

    Science.gov (United States)

    Lin, C-S; Lin, Y-S; Liu, C-F; Weng, S-F; Lin, C; Lin, B-S

    2016-01-01

    To evaluate the incidence rates and risk of sudden sensorineural hearing loss among patients with depressive disorders. Data for 27 547 patients with newly diagnosed depressive disorders and 27 547 subjects without depressive disorders between 2001 and 2008 were yielded from the Taiwan National Health Insurance Research Database. Sudden sensorineural hearing loss incidence at the end of 2011 was determined. Cumulative incidence and adjusted hazard ratio were computed. Sudden sensorineural hearing loss incidence was 1.45 times higher in the depressive disorders group compared to the non-depressive disorders group (p = 0.0041), with an adjusted hazard ratio of 1.460. A significant increased risk of developing sudden sensorineural hearing loss was noted in patients with diabetes mellitus, chronic kidney disease and hyperlipidaemia (p sudden sensorineural hearing loss in patients with depressive disorders. Co-morbidities such as diabetes mellitus, chronic kidney disease and hyperlipidaemia significantly aggravated the risk. Depressive disorders might be considered a risk factor for sudden sensorineural hearing loss. It remains to be seen whether control of depressive disorders can decrease the incidence of sudden sensorineural hearing loss in patients with depressive disorders.

  3. Intratympanic methylprednisolone perfusion as a salvage treatment for profound idiopathic sudden sensorineural hearing loss.

    Science.gov (United States)

    Dai, Y; Lu, L; Hou, J; Yang, X; Li, H; Yang, Y; She, W

    2017-05-01

    This study aimed to examine the effectiveness of intratympanic methylprednisolone perfusion as salvage treatment for profound idiopathic sudden sensorineural hearing loss. A retrospective clinical study of 97 patients with unilateral profound idiopathic sudden sensorineural hearing loss was performed. In all, 83 patients who received salvage intratympanic methylprednisolone perfusion plus conventional treatment (except for steroids) as the second-line therapy were assigned to the study group, while 14 patients who received conventional treatment alone were assigned to the comparison group. In the study group, treatments in patients with a shorter interval from disease onset to intratympanic methylprednisolone perfusion (up to 15 days) had significantly greater improvements in the overall effective rate and pure tone average compared with patients with a longer interval (over 15 days). For patients with a short interval from disease onset to intratympanic methylprednisolone perfusion, those in the study group had significantly greater improvements in the overall effective rate and pure tone average compared with those in the comparison group. In both the study and comparison groups, hearing improvements were greater at low frequencies than at medium and high frequencies. The interval from disease onset to intratympanic methylprednisolone perfusion was the major factor affecting hearing recovery. Early second-line salvage intratympanic methylprednisolone perfusion significantly improved the degree of hearing recovery in profound idiopathic sudden sensorineural hearing loss patients after failure of systemic steroid treatment.

  4. Outcomes of Hearing Aid Use by Individuals with Unilateral Sensorineural Hearing Loss (USNHL).

    Science.gov (United States)

    Bishop, Charles E; Hamadain, Elgenaid; Galster, Jason A; Johnson, Mary Frances; Spankovich, Christopher; Windmill, Ian

    Unilateral sensorineural hearing loss (USNHL) can have a negative impact on functions associated with the advantages of balanced, binaural hearing. Although single-sided deafness, which is a complete loss of audibility in one ear, has gained increased interest in the published research, there is a gap in the literature concerning hearing aid outcomes for individuals with residual, or otherwise "aidable," hearing in the affected ear. To assess hearing aid outcomes for a group of individuals with USNHL with residual, aidable function. A quasi-experimental study of hearing aid outcomes with paired comparisons made between unaided and aided test conditions. A convenience sample of twenty-two individuals with USNHL, with sufficient residual hearing in the affected ear as to receive audibility from use of a hearing aid, were recruited into the study from September 2011 to August 2012. Each participant was fit with a digital behind-the-ear hearing aid coupled to a custom ear mold. Assessments were performed at baseline (unaided) and after a three-month field trial (aided) with primary outcomes involving objective measures in sound field yielding signal-to-noise ratio loss (SNR Loss) via the Quick Speech-in-Noise Test and word recognition scores (WRS) via the Northwestern University Auditory Test, No. 6. Outcomes also involved the administration of two well-established subjective benefit questionnaires: The Abbreviated Profile of Hearing Aid Benefit (APHAB) and the 49-item Speech, Spatial, and Qualities of Hearing Scale (SSQ49). As a group, participants showed significantly improved median SNR Loss thresholds when aided in a test condition that included spatial separation of speech and noise, with speech stimuli directed toward the worse ear and noise stimuli directed toward the better ear (diff. = -4.5; p Hearing aid use had a small, though statistically significant, negative impact on median SNR Loss thresholds, when speech and noise stimuli originated from the same 0

  5. Variables with prognostic value in the onset of idiopathic sudden sensorineural hearing loss.

    Science.gov (United States)

    Bogaz, Eduardo Amaro; Maranhão, André Souza de Albuquerque; Inoue, Daniel Paganini; Suzuki, Flavia Alencar de Barros; Penido, Norma de Oliveira

    2015-01-01

    The establishment of an individualized prognostic evaluation in patients with a diagnosis of idiopathic sudden sensorineural hearing loss (ISSHL) remains a difficult and imprecise task, due mostly to the variety of etiologies. Determining which variables have prognostic value in the initial assessment of the patient would be extremely useful in clinical practice. To establish which variables identifiable at the onset of idiopathic sudden sensorineural hearing loss have prognostic value in the final hearing recovery. Prospective, longitudinal cohort study. Patients with ISSHL followed by the Department of Otology-Neurotology of a quaternary hospital were included. The following variables were evaluated and correlated with final hearing recovery: age, gender, vertigo, tinnitus, initial degree of hearing loss, contralateral ear hearing, and elapsed time to treatment. 127 patients with ISSHL were evaluated. Rates of absolute and relative recovery were 23.6dB and 37.2% respectively. Complete hearing improvement was observed in 15.7% patients; 27.6% demonstrated significant improvement and improvement was noted in 57.5%. During the onset of ISSHL, the following variables were correlated with a worse prognosis: dizziness, profound hearing loss, impaired hearing in the contralateral ear, and delay to start treatment. Tinnitus at the onset of ISSHL correlated with a better prognosis. Copyright © 2015 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  6. Prothrombotic gene mutations in patients with sudden sensorineural hearing loss and cardiovascular thrombotic disease.

    Science.gov (United States)

    Capaccio, Pasquale; Cuccarini, Valeria; Ottaviani, Francesco; Fracchiolla, Nicola Stefano; Bossi, Anna; Pignataro, Lorenzo

    2009-03-01

    Impaired cochlear perfusion seems to be an important event in sudden sensorineural hearing loss. Prothrombotic gene mutations have been related to vascular disorders and sudden hearing loss. We assessed the prothrombotic risk in 10 patients with sudden sensorineural hearing loss who had previously experienced cardiovascular events to support its vascular pathogenesis. Ten patients underwent hematologic tests (MTHFR C677T/A1298C, prothrombin G20210A, platelet GlyIIIaA1/A2, and V Leiden G1691A genotyping; fibrinogenemia; cholesterolemia: homocysteinemia; folatemia). The results were compared with those of 100 previously investigated patients with sudden hearing loss alone and those of 200 healthy controls. DNA was isolated from peripheral blood leukocytes, and the gene mutations were investigated by polymerase chain reaction and a LightCycler DNA analyzer. Two patients had 2 mutant alleles, 6 had 3, and 2 had 4. The mean homocysteine, cholesterol, and fibrinogen levels were above the upper limit of normal; the mean folate levels were slightly above the lower limit of normal. Multiple mutations were more frequent in the patient group than in the previously analyzed patients and healthy controls. The association between inherited and acquired prothrombotic factors in patients with sudden sensorineural hearing loss and thrombotic diseases in other sites suggests that a multifactorial mechanism may underlie microvascular cochlear impairment. Hematologic investigation, including MTHFR, prothrombin, platelet, and V Leiden genotyping, may help to detect patients at potential risk of recurrent hearing loss and multiple microvascular diseases, and could be usefully performed in otherwise idiopathic sudden sensorineural hearing loss.

  7. Sensorineural Hearing Impairment is a Common Feature of ...

    African Journals Online (AJOL)

    ... so, prevention is essential to reduce the incidence of genetic hearing loss. Premarital and antenatal screening should be applied whenever possible, at least for those at risk of developing genetic diseases including hearing impairement. Keywords: Consanguinity; hearing disorders; preventive medicine. Egypt. J. Hum.

  8. Hearing Aid Benefit in Patients with Mild Sensorineural Hearing Loss: A Systematic Review.

    Science.gov (United States)

    Johnson, Carole E; Danhauer, Jeffrey L; Ellis, Blakely B; Jilla, Anna Marie

    2016-04-01

    Untreated sensorineural hearing loss (SNHL) is associated with chronic health-care conditions, isolation, loneliness, and reduced quality of life. Although hearing aids can minimize the negative effects of SNHL, only about one in five persons with SNHL seeks help for communication problems. Many persons wait 10 yr or more from the time they first notice a problem before pursuing amplification. Further, little information about the benefits of amplification is available for persons with mild SNHL (MSNHL), who likely defer treatment even longer. To conduct a systematic review to weigh the evidence regarding benefits derived from the use of amplification by adults with MSNHL. Systematic review with meta-analysis. Adult hearing aid wearers with bilateral average pure-tone thresholds ≤45 dB HL at 500, 1000, 2000, and 4000 Hz. PubMed, Cumulative Index to Nursing and Allied-Health Literature, Cochrane Collaboration, and Google Scholar were searched independently by the authors during September 2013. The authors used a consensus approach to assess the quality and extract data for the meta-analysis. Of 106 articles recovered for full-text review, only 10 met inclusion criteria (at least Level IV of evidence and involved and reported separate pre-/postfitting hearing aid outcomes for patients with MSNHL). Included studies involved mainly middle-aged to elderly patients using hearing aids of various styles and circuitry. Results from all of the studies indicated positive benefits from amplification for patients with MSNHL. Data from five studies were suitable for a meta-analysis, which produced a small-to-medium effect size of 0.85 (95% confidence intervals = 0.44-1.25) after adjusting for a small publication bias. This evidence confirmed benefits from the use of amplification in adults with MSNHL. Evidence exists supporting the notion that adults with MSNHL benefit from hearing aids. This information is important and useful to audiologists, patients, and third-party payers

  9. Sudden bilateral sensorineural hearing loss as an unusual consequence of accidental ingestion of potassium hydroxide.

    Science.gov (United States)

    Ciorba, A; Bovo, R; Castiglione, A; Pirodda, A; Martini, A

    2010-01-01

    To discuss the possible etiopathogenetic mechanism of inner ear damage induced by the ingestion of potassium hydroxide (KOH). We report the case of a 37-year-old patient with sudden bilateral sensorineural hearing loss after accidental ingestion of a KOH solution. The first ear, nose and throat examination disclosed only mild edema of the upper airways. He was treated in the intensive care unit and prescribed high-dose steroids, proton pump inhibitors and sucralfate for 2 weeks. Unfortunately, there was no recovery of the hearing loss, and no audiogram changes were noticed after 12 months of follow-up. After exploring the possible etiopathogenetic mechanism involved, the authors believe that in this case, a transient severe hemodynamic imbalance can actually be considered to be the most reliable explanation for the inner ear damage and subsequent onset of permanent bilateral sensorineural hearing loss. Copyright 2010 S. Karger AG, Basel.

  10. Sensorineural Hearing Loss Associated with Occupational Noise Exposure: Effects of Age-Corrections

    Directory of Open Access Journals (Sweden)

    2009-02-01

    Full Text Available Noise-induced permanent threshold shifts (NIPTS were computed from retrospective audiometric analyses by subtracting aging effects on hearing sensitivity in sixty-eight patients with bilateral sensorineural hearing loss who reported significant occupational noise exposure histories. There were significant effects of age on NIPTS but no significant gender- or ear- differences in terms of NIPTS. The NIPTS at 2,000 Hz was found to be significantly greater than NIPTS at frequencies 500 Hz, 1,000 Hz, 4,000 Hz, and 8,000 Hz. Defined noise notches were seen in the audiograms of 38/136 (27% ears with SNHL. Results support models that suggest interactive effects of aging and noise on sensorineural hearing loss in ears with occupational noise exposure.

  11. Sensorineural hearing loss associated with occupational noise exposure: effects of age-corrections.

    Science.gov (United States)

    Krishnamurti, Sridhar

    2009-03-01

    Noise-induced permanent threshold shifts (NIPTS) were computed from retrospective audiometric analyses by subtracting aging effects on hearing sensitivity in sixty-eight patients with bilateral sensorineural hearing loss who reported significant occupational noise exposure histories. There were significant effects of age on NIPTS but no significant gender- or ear- differences in terms of NIPTS. The NIPTS at 2,000 Hz was found to be significantly greater than NIPTS at frequencies 500 Hz, 1,000 Hz, 4,000 Hz, and 8,000 Hz. Defined noise notches were seen in the audiograms of 38/136 (27%) ears with SNHL. Results support models that suggest interactive effects of aging and noise on sensorineural hearing loss in ears with occupational noise exposure.

  12. Ethics of Placebo Control in Trials for Idiopathic Sudden Sensorineural Hearing Loss.

    Science.gov (United States)

    Maldonado Fernández, Miguel; Kornetsky, Susan; Rubio Rodriguez, Laura

    2016-07-01

    Idiopathic sudden sensorineural hearing loss (ISSHL) involves sudden loss of hearing from cochlear or retrocochlear origin of unknown cause. Systemic corticosteroids may be considered in the management of ISSNHL. However, an updated Cochrane systematic review concludes that "the value of steroids in the treatment of idiopathic sudden sensorineural hearing loss remains unclear since the evidence obtained from randomized controlled trials is contradictory in outcome." Therefore, a new clinical trial that addresses this question is mandatory. A first step in its design is to determine if placebo control would be ethically acceptable. We conclude that there is equipoise (uncertainty) about the use of corticosteroids for ISSHL. A new trial is justified-but with the inclusion of interim analyses to detect early imbalances on efficacy or safety and with the ability to stop the trial if needed. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2016.

  13. GJB2 sequencing in deaf and profound sensorineural hearing loss children.

    Science.gov (United States)

    Mielczarek, Marzena; Zakrzewska, Anna; Olszewski, Jurek

    2016-06-30

    GJB2 mutations are the most frequent reason of genetic congenital hearing loss. The aim of the study was to assess the prevalence of GJB2 mutations in the deaf and profound hearing loss children. The material of the study was a group of 61 patients divided into two groups. Group I - 35 deaf or with profound sensorineural hearing loss children (the pupils of the deaf and hard of hearing school), aged 5-17 years (average 9.2 years), 14 males, 21 females, II - control group comprised 26 normal hearing patients, aged 5-16 years (average 10.4years), 14 males, 12 females (patients of Department of Pediatric Otolaryngology, Audiology and Phoniatrics, Medical University of Lodz). In both groups, exon 2 sequencing of GJB2 gene was performed. In group I in 6 patients (17%) 35delG in GJB2 gene was found. The patients were homozygotes, with negative family history of hearing loss. No other mutations in GJB2 gene were found. In group II no mutations in GJB2 were observed. The most frequent cause of hearing impairment in the deaf and profound sensorineural hearing loss children was 35delG mutation in GJB2 gene. No other mutations in GJB2 gene were detected.

  14. Sensorineural hearing loss enhances auditory sensitivity and temporal integration for amplitude modulation.

    OpenAIRE

    Wallaert, Nicolas; Moore, Brian Cecil; Ewert, Stephan D; Lorenzi, Christian

    2017-01-01

    Amplitude-modulation detection thresholds (AMDTs) were measured at 40 dB sensation level for listeners with mild-to-moderate sensorineural hearing loss (age: 50-64 yr) for a carrier frequency of 500 Hz and rates of 2 and 20 Hz. The number of modulation cycles, N, varied between two and nine. The data were compared with AMDTs measured for young and older normal-hearing listeners [Wallaert, Moore, and Lorenzi (2016). J. Acoust. Soc. Am. 139, 3088-3096]. As for normal-hearing listeners, AMDTs we...

  15. The etiology of idiopathic sudden sensorineural hearing loss - Experimental herpes simplex virus infections of the inner ear

    NARCIS (Netherlands)

    Stokroos, RJ; Albers, FWJ; Schirm, J

    Hypothesis: Experimentally induced herpes simplex virus type 1 (HSV-1) labyrinthitis provides a suitable model for idiopathic sudden sensorineural hearing loss (ISSHL). Background: Viral labyrinthitis has been postulated to play a role in the pathophysiology of ISSHL. Circumstantial evidence is

  16. 3-DIMENSIONAL MAGNETIC-RESONANCE-IMAGING OF THE INNER-EAR IN IDIOPATHIC SUDDEN SENSORINEURAL HEARING-LOSS

    NARCIS (Netherlands)

    ALBERS, FWJ; DEMUYNCK, KMNP; CASSELMAN, JW

    1994-01-01

    Five patients with idiopathic sudden sensorineural hearing loss (ISSHL) were examined by a combination of high-resolution computed tomography and special magnetic resonance imaging techniques. By three-dimensional constructive interference in steady state magnetic resonance imaging excellent

  17. The risk ratio for development of hereditary sensorineural hearing loss in consanguineous marriage offspring.

    Science.gov (United States)

    Sanyelbhaa, Hossam; Kabel, Abdelmagied; Abo El-Naga, Heba Abd El-Rehem; Sanyelbhaa, Ahmed; Salem, Hatem

    2017-10-01

    This study aims to define the relative risk of development of hearing loss in offspring of consanguineous marriages. This is a retrospective case-control study conducted in a tertiary referral center in Jeddah, KSA. The study group included 1600 probands (848 males, 752 females), with age range 0.5-12 years (6.6 ± 3.6). The study group comprised of two equal, age and sex matched subgroups; Hearing Loss (HL) group and Normal Hearing (NH) group. The children included in the HL group should have idiopathic or non syndromic genetic sensorineural hearing loss. The HL Group comprised 800 children with variable degrees of sensorineural hearing loss. Profound and severe degrees of hearing loss were the most prevalent degrees (P marriage offspring in the NH group was 42.5%, while in the HL group it was 68.9% (P  0.05). The relative risk and 95% confidence interval (RR, 95% CI) for development of hearing loss in offspring of consanguineous marriage was 1.76 (95% CI 1.57-1.97, P marriage progeny to develop SNHL when compared to non consanguineous progeny. Copyright © 2017. Published by Elsevier B.V.

  18. Assessment of Hematological Factors Involved in Development and Prognosis of Idiopathic Sudden Sensorineural Hearing Loss.

    Science.gov (United States)

    Durmuş, Kasim; Terzi, Hatice; Karataş, Tuba Doğan; Doğan, Mansur; Uysal, Ismail Önder; Şencan, Mehmet; Altuntaş, Emine Elif

    2016-01-01

    The aim of this study was to investigate the possible effects of routine hematological parameters on the development and prognosis of idiopathic sudden sensorineural hearing loss in patients applying to our clinic. A retrospective clinical study. One academic health center from 2008 to 2014. One hundred forty patients with sudden hearing loss and 132 healthy controls were included in the present study. Patients having idiopathic sudden sensorineural hearing loss were divided into 2 subgroups based on whether they recovered (complete, partial, and slight recovery) (Group 1; n = 83, 59.3%) or not (Group 2; n = 57, 40.7%) during the follow-up term. Group 1, Group 2, and the controls differed statistically significantly in terms of neutrophil-to-lymphocyte ratio (P = 0.001), platelet-to-lymphocyte ratio (P = 0.001), lymphocytes % (P = 0.001), mean corpuscular hemoglobin (P = 0.019), mean corpuscular hemoglobin concentration (P = 0.015), platelet (P  = 0.001), mean platelet volume (P = 0.001), platelet distribution width (P = 0.009), and glucose (P = 0.001). The study groups and the controls did not have any significant difference in terms of other laboratory parameters affecting the prognosis of Idiopathic sudden sensorineural hearing loss. The results the authors obtained showed that laboratory parameters such as lymphocyte, lymphocyte%, platelet, mean platelet volume, platelet distribution width, neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio, mean corpuscular hemoglobin, and mean corpuscular hemoglobin concentration may be indicative for prognosis and treatment success in groups of patients suffering idiopathic sudden sensorineural hearing loss in whose etiology many factors play a role.

  19. A new nonsyndromic X-linked sensorineural hearing impairment linked to Xp21.2

    Energy Technology Data Exchange (ETDEWEB)

    Lalwani, A.K.; Brister, J.R.; Fex, J.; Grundfast, K.M.; Pikus, A.T.; Ploplis, B.; San Agustin, T.; Skarka, H.; Wilcox, E.R. [National Institutes of Health, Bethesda, MD (United States)

    1994-10-01

    X-linked deafness is a rare cause of hereditary hearing impairment. We have identified a family with X-linked dominant sensorineural hearing impairment, characterized by incomplete penetrance and variable expressivity in carrier females, that is linked to the Xp21.2, which contains the Duchenne muscular dystrophy (DMD) locus. The auditory impairment in affected males was congenital, bilateral, profound, sensorineural, affecting all frequencies, and without evidence of radiographic abnormality of the temporal bone. Adult carrier females manifested bilateral, mild-to-moderate high-frequency sensorineural hearing impairment of delayed onset during adulthood. Eighteen commercially available polymorphic markers from the X chromosome, generating a 10-15-cM map, were initially used for identification of a candidate region. DXS997, located within the DMD gene, generated a two-point LOD score of 2.91 at {theta} = 0, with every carrier mother heterozygous at this locus. Recombination events at DXS992 (located within the DMD locus, 3{prime} to exon 50 of the dystrophin gene) and at DXS1068 (5{prime} to the brain promoter of the dystrophin gene) were observed. No recombination events were noted with the following markers within the DMD locus: 5{prime}DYS II, intron 44, DXS997, and intron 50. There was no clinical evidence of Duchenne or Becker muscular dystrophy in any family member. It is likely that this family represents a new locus on the X chromosome, which when mutated results in nonsyndromic sensorineural hearing loss and is distinct from the heterogeneous group of X-linked hearing losses that have been previously described. 57 refs., 6 figs., 1 tab.

  20. The relationship between hearing aid frequency response and acceptable noise level in patients with sensorineural hearing loss.

    Science.gov (United States)

    Jalilvand, Hamid; Pourbakht, Akram; Jalaee, Shohreh

    2015-01-01

    When fitting hearing aid as a compensatory device for an impaired cochlea in a patient with sensorineural hearing loss (HL), it is needed to the effective and efficient frequency response would be selected regarding providing the patient's perfect speech perception. There is not any research about the effects of frequency modifications on speech perception in patients with HL regarding the cochlear desensitization. The effect (s) of modifications in frequency response of hearing aid amplification on the results of acceptable noise level (ANL) test is the main aim of this study. The amounts of ANL in two conditions of linear amplification (high frequency emphasis [HFE] and mid frequency emphasis [MFE]) were measured. Thirty-two male subjects who participated in this study had the moderate to severe sensorineural HL. There was not any significant difference between ANL in linear amplification of hearing aid with HFE frequency response and ANL in linear amplification of hearing aid with MFE frequency response. The gain modification of frequency response not only does not affect the patient's performance of speech intelligibility in ANL test. This indicates that we need to note to the cochlear desensitization phenomenon when fitting hearing aid as a compensatory device for an impaired cochlea in a patient. The cochlear desensitization has not been considered properly in hearing aid fitting formula which is needed to be explored more about the bio-mechanisms of impaired cochlea.

  1. Allan-Herndon-Dudley syndrome with unusual profound sensorineural hearing loss.

    Science.gov (United States)

    Gagliardi, Lucia; Nataren, Nathalie; Feng, Jinghua; Schreiber, Andreas W; Hahn, Christopher N; Conwell, Louise S; Coman, David; Scott, Hamish S

    2015-08-01

    The Allan-Herndon-Dudley syndrome is caused by mutations in the thyroid hormone transporter, Monocarboxylate transporter 8 (MCT8). It is characterized by profound intellectual disability and abnormal thyroid function. We report on a patient with Allan-Herndon-Dudley syndrome (AHDS) with profound sensorineural hearing loss which is not usually a feature of AHDS and which may have been due to a coexisting nonsense mutation in Microphthalmia-associated transcription factor (MITF). © 2015 Wiley Periodicals, Inc.

  2. [Vibrant Sound Bridge System. A new kind hearing prosthesis for patients with sensorineural hearing loss. 2. Audiological results].

    Science.gov (United States)

    Lenarz, T; Weber, B P; Issing, P R; Gnadeberg, D; Ambjørnsen, K; Mack, K F; Winter, M

    2001-07-01

    Implantable hearing aids present a new treatment modality for patients suffering from sensorineural hearing loss. The functional gain obtained with the partially implantable Symphonix soundbridge system was evaluated in a clinical study. The audiological results achieved with n = 34 patients over a period of up to three years are presented in this second part of the publication. 34 patients have received the Symphonix Vibrant soundbridge system since February 1997. The average age at implantation was 47.2 years (minimum: 18.9 years; maximum: 80.3 years). All patients have had several years of experience with hearing aids, which, however, provided insufficient functional gain or could not be fitted with a conventional hearing aid for medical reasons (such as auditory ear canal problems). All patients fulfilled the audiological selection criteria as they had bilateral moderate to severe sensorineural hearing loss. As a rule, the ear with poorer performance was implanted. All patients were fitted with the audio processor eight weeks after the implantation. The pure tone thresholds, the functional gain, the monosyllable and sentence understanding (Göttinger Sentence Test in quiet and noise) were preoperatively and postoperatively assessed. Standardized self-assessment questionnaires were used to evaluate the subjective benefit (PHAB) and the quality of hearing (HDSS) as compared to the preoperative situation. Further hearing tests were performed after four weeks, three, six, nine, twelve, eighteen, twenty-four and thirty-six months postoperatively. During the observation period of up to three years the audioprocessor was updated several times, most recently with the fully digital three-channel-system Vibrant D. The results obtained were documented. Postoperatively, the pure tone threshold with the soundbridge system switched off did not change significantly in the implanted ear. All patients had a functional gain that was either comparable to the gain achieved with

  3. Hearing outcome does not depend on the interval of intratympanic steroid administration in idiopathic sudden sensorineural hearing loss.

    Science.gov (United States)

    Suzuki, Hideaki; Koizumi, Hiroki; Ohkubo, Jun-Ichi; Hohchi, Nobusuke; Ikezaki, Shoji; Kitamura, Takuro

    2016-10-01

    We studied the effect of intratympanic steroid administration with different intervals on hearing outcomes in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). The subjects were 197 consecutive patients (197 ears) with ISSNHL (hearing level ≥40 dB, interval between onset and treatment ≤30 days). They received systemic administration of prednisolone (100 mg followed by tapered doses) combined with intratympanic injection of dexamethasone (4 mg/ml). Intratympanic injection was performed once a week for 4 weeks in 105 patients (long-interval group), or 4 times in 1 week in 92 patients (short-interval group). The hearing outcomes were evaluated at two points of time: 1 week from the start of treatment, and 1-2 months after the completion of treatment when the hearing level reached a plateau. There was no significant difference in the cure rate, marked-recovery rate, recovery rate, hearing gain, hearing level, or percent hearing improvement between the long- and short-interval groups at either point of time. Multiple regression analysis also showed that the final hearing level did not depend on the interval of intratympanic steroid injection. These results indicate that the hearing outcome of ISSNHL does not improve even if the interval of intratympanic injection is shortened. This implies that a lower total number of intratympanic steroid injections may be as effective as the present protocol.

  4. Characterizing auditory processing and perception in individual listeners with sensorineural hearing loss

    DEFF Research Database (Denmark)

    Jepsen, Morten Løve; Dau, Torsten

    2011-01-01

    –438 (2008)] was used as a framework. The parameters of the cochlear processing stage of the model were adjusted to account for behaviorally estimated individual basilar-membrane inputoutput functions and the audiogram, from which the amounts of inner hair-cell and outer hair-cell losses were estimated......This study considered consequences of sensorineural hearing loss in ten listeners. The characterization of individual hearing loss was based on psychoacoustic data addressing audiometric pure-tone sensitivity, cochlear compression, frequency selectivity, temporal resolution, and intensity...

  5. Cochlear synaptopathy in acquired sensorineural hearing loss: Manifestations and mechanisms.

    Science.gov (United States)

    Liberman, M Charles; Kujawa, Sharon G

    2017-06-01

    Common causes of hearing loss in humans - exposure to loud noise or ototoxic drugs and aging - often damage sensory hair cells, reflected as elevated thresholds on the clinical audiogram. Recent studies in animal models suggest, however, that well before this overt hearing loss can be seen, a more insidious, but likely more common, process is taking place that permanently interrupts synaptic communication between sensory inner hair cells and subsets of cochlear nerve fibers. The silencing of affected neurons alters auditory information processing, whether accompanied by threshold elevations or not, and is a likely contributor to a variety of perceptual abnormalities, including speech-in-noise difficulties, tinnitus and hyperacusis. Work described here will review structural and functional manifestations of this cochlear synaptopathy and will consider possible mechanisms underlying its appearance and progression in ears with and without traditional 'hearing loss' arising from several common causes in humans. Copyright © 2017 Elsevier B.V. All rights reserved.

  6. Bilateral sudden sensorineural hearing loss in Staphylococcus aureus endocarditis.

    Science.gov (United States)

    Lau, Joanne Wai Ling; Ceranic, Borka; Harris, Robert; Timehin, Elwina

    2015-09-14

    This case highlights the diagnostic challenges in patients presenting with bilateral sudden sensorinueral hearing loss (SNHL). The aetiology of bilateral sudden SNHL may span several medical disciplines. Therefore, clinicians should be mindful of such presentations, and consider aetiologies beyond otological and neurological causes. We present a case of a previously healthy 51-year-old woman who presented with coryzal symptoms and sudden audiovestibular failure. Examination revealed fever, tachycardia, bilateral profound hearing loss and nystagmus. Following investigations, an initial working diagnosis of vasculitis was made. Later, blood cultures revealed methicillin-sensitive Staphylococcus aureus (MSSA) and a transoesophageal echocardiogram confirmed endocarditis. The patient made a good recovery, but the hearing loss was permanent and managed with a cochlear implant. 2015 BMJ Publishing Group Ltd.

  7. [Clinical features of sudden sensorineural hearing loss accompanied with inner ear hemorrhage].

    Science.gov (United States)

    Chen, Xihang; Lin, Chang; Fang, Zheming; Chen, Xi; Ye, Shengnan; Cheng, Jinmei; Zhang, Rong

    2015-12-01

    To investigate the clinical features, diagnosis and prognosis of sudden sensorineural hearing loss accompanied with inner ear hemorrhage. Eleven cases of sudden sensorineural hearing loss accompanied with inner ear hemorrhage were retrospectively analyzed, including clinical manifestation, hematological and audiological examinations, as well as characteristics of inner ear three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging (3D-FLAIR MRI). Eleven cases of sudden hearing loss with inner ear hemorrhage were accompanied by varying degrees of vertigo, lasting from several minutes to several hours, nine of whom had persistent tinnitus. The audiometry curves of the 11 cases included total deafness or flat descending type. The inner ear 3D-FLAIR MRI studies revealed abnormal high signals at different positions of the inner ear, one case with endolymphatic hydrops. After the treatment, seven cases were invalid, two cases had notable effect, and two cases were effective. Abnormal high signals in the inner ear were sensitively detected by 3D-FLAIR MRI sequence, which was applied for the diagnosis of the sudden hearing loss accompanied with inner ear hemorrhage. The effect of conventional treatment was not good and the appropriate treatments for this type of sudden hearing loss need further investigation.

  8. Sensori-neural hearing loss in patients treated with irradiation for nasopharyngeal carcinoma

    International Nuclear Information System (INIS)

    Grau, C.; Moller, K.; Overgaard, M.; Overgaard, J.; Elbrond, O.

    1991-01-01

    The present investigation has been carried out to evaluate the sensitivity of the inner ear to irradiation. Cochlear function was tested in a cohort of 22 patients before and 7-84 months after receiving external irradiation for nasopharyngeal carcinoma. The pre-irradiation sensori-neural hearing threshold at 500, 1000, 2000, and 4000 Hz was used as a baseline for the individual patient, and the observed sensori-neural hearing loss (SNHL) was calculated as the difference between pre- and post-irradiation values. The pre-irradiation hearing level or patient age was not correlated with the actual SNHL. In contrast, there was a significant correlation between the total radiation dose to the inner ear and the observed hearing impairment. SNHL was most pronounced in the high frequencies, with values up to 35 dB (4000 Hz) and 25 dB (2000 Hz) in some patients. The latent period for the complication appeared to be 12 months or more. The deleterious effect of irradiation on the hearing should be kept in mind both in treatment planning and in the follow-up after radiotherapy

  9. Challenges in management of childhood sensorineural hearing loss in sub-Saharan Africa, Nigeria.

    Science.gov (United States)

    Lasisi, O A; Ayodele, J K; Ijaduola, G T A

    2006-04-01

    The evaluation of a hearing-impaired child attempts to determine the aetiology, the degree of hearing loss and intervention to aid speech and language. This remains a challenge to practising otolaryngologists, especially in the developing countries as 85-90% of causes of hearing losses were never discovered leading to delayed intervention and irreversible effects. In a review of children presenting at the otolaryngology outpatient in the University College Hospital, Ibadan, Nigeria, sensorineural hearing loss was found in 103, giving a hospital prevalence rate of 14%, we still perceived this figure to be unrepresentatively and low, probably due to poor access to medicare, poverty and other factors. Genetic factor accounted for 25%, followed by measles infections 13% and meningitis 8%. About 60% of them had educationally significant hearing loss at presentation. Access to hearing aid was poor as only 12.5% of the patients could afford it and the rest were managed by deaf training. We conclude by suggesting an audiologic programme which has the comprehensive function of neonatal and infant hearing screening, subsidized hearing aid services and hearing rehabilitation surgery.

  10. Phenotypic characterization of DFNA24: prelingual progressive sensorineural hearing impairment.

    NARCIS (Netherlands)

    Santos, R.L.; Hafner, F.M.; Huygen, P.L.M.; Linder, T.E.; Schinzel, A.; Spillmann, T.; Leal, S.M.

    2006-01-01

    This article describes the hearing impairment (HI) phenotype which segregates in a large multi-generation Swiss-German family with autosomal dominant nonsyndromic HI. The locus segregating within this pedigree is located on chromosome 4q35-qter and is designated as DFNA24. For this pedigree,

  11. Sudden Sensorineural Hearing Loss: The Question of Perilymph Fistula.

    Science.gov (United States)

    Backous, Douglas D.; Niparko, John K.

    1997-01-01

    Perilymph fistula (PLF) is an abnormal communication between the fluid-containing spaces of the inner ear and the air-containing spaces of the temporal bone that can cause hearing loss, tinnitus, aural fullness, vertigo, and postural instability. Diagnosis of PLF and management of those with presumed PLF are discussed. (Contains extensive…

  12. Effect of antioxidant supplementation on the auditory threshold in sensorineural hearing loss: a meta-analysis.

    Science.gov (United States)

    Souza, Maria Eduarda Di Cavalcanti Alves de; Costa, Klinger Vagner Teixeira da; Vitorino, Paulo Augusto; Bueno, Nassib Bezerra; Menezes, Pedro de Lemos

    2017-08-26

    Hearing loss is conceptualized as any impairment of the ability to hear and/or detect speech or environment sounds, regardless of cause, type, or degree. It may occur at different stages of life; during pregnancy or childbirth, in childhood, adulthood or old age. It should be noted that aging is the most common cause of sensorineural hearing loss followed by noise-induced hearing loss, and both are closely related to the formation of reactive oxygen species. Dietary antioxidant supplementation has been employed as a therapeutic strategy to prevent and/or delay the risks of major human diseases. To assess randomized clinical trials to determine the effect of antioxidant supplementation on the auditory thresholds in patients of different age groups with sensorineural hearing loss. This systematic review consisted of a search in the following databases: MEDLINE, CENTRAL, ScienceDirect, Scopus, Web of Science, LILACS, SciELO and ClinicalTrials.gov. Additionally, the gray literature was also searched. The search strategy included terms related to the intervention (antioxidant supplementation), primary outcome (sensorineural hearing loss), as well as terms related to randomized clinical trials to improve search sensitivity. Based on 977 potentially relevant records identified through the search in the databases, ten full-text publications were retrieved for further evaluation. The increase in threshold at the 4kHz frequency was statistically higher in the control group (1.89 [1.01-2.78], p<0.0001) when compared to the NAC group and the ginseng group, whereas at 6kHz, the threshold increase was higher in the control group (1.42 [-1.14-3.97], p=0.28), but no statistically significant differences were found between groups. Ginseng was the antioxidant agent that showed the best effect in preventing auditory threshold worsening at the frequency of 4kHz, but not at 6kHz in patients with sensorineural hearing loss caused by exposure to high sound pressure levels. There was no

  13. Coronary artery bypass grafting and sensorineural hearing loss, a cohort study

    Directory of Open Access Journals (Sweden)

    Ashraf Omer

    2005-12-01

    Full Text Available Abstract Background Sudden sensorineural hearing loss is routinely encountered by the otologist. The etiology is varied and often identifiable. One of the relatively less frequent causes is surgery. Apart from being an established entity with otological surgeries, sensorineural hearing loss has also been known to occur after non-otological procedures under general anesthesia. Commonest amongst these procedures is cardiopulmonary bypass, an association that has long been recognized. However, despite the proposition of diverse hypotheses in the past, the pathophysiology remains unclear. Methods The study is a prospective matched cohort study that will be carried out in Aga Khan University Hospital, Karachi, Pakistan. Participants among exposed would include all those patients who would be undergoing coronary artery bypass surgery in the hospital who fall under the criteria for inclusion. Unexposed group would comprise of patients undergoing a non-bypass procedure of similar duration under the same type of anesthesia who meet the selection criteria. Both these groups will undergo audiometric testing at our hospital on three different occasions during the course of this study. Initially before the procedure to test the baseline hearing capacity; then one week after the procedure to assess any changes in hearing ability following the surgery; and finally a third audiogram at six weeks follow-up to assess further changes in any hearing deficits noted during the second phase of testing. Certain variables including the subjects' demographics and those concerning the procedure itself will be noted and used later for risk factors analysis. A detailed past medical and surgical history will also be obtained. Data analysis would include calculation of relative risk and significance of the results, by running the chi-square test. Other statistical tests like Fisher exact test may then be employed to facilitate data interpretation. Continuous scale may then be

  14. A mismatch negativity study in Mandarin-speaking children with sensorineural hearing loss.

    Science.gov (United States)

    Fu, Mingfu; Wang, Liyan; Zhang, Mengchao; Yang, Ying; Sun, Xibin

    2016-12-01

    a) To examine the effects of sensorineural hearing loss on the discriminability of linguistic and non-linguistic stimuli at the cortical level, and b) to examine whether the cortical responses differ based on the chronological age at intervention, the degree of hearing loss, or the acoustic stimulation mode in children with severe and profound hearing loss. Mismatch negativity (MMN) responses were collected from 43 children with severe and profound bilateral sensorineural hearing loss, and 20 children with normal hearing (age: 3-6 years). In the non-verbal stimulation condition, pure tones with frequencies of 1 kHz and 1.1 kHz were used as the standard and the deviant respectively. In the verbal stimulation condition, the Chinese mandarin tokens/ba2/and/ba4/were used as the standard and the deviant respectively. Latency and amplitude of the MMN responses were collected and analyzed. Overall, children with hearing loss showed longer latencies and lower amplitudes of the MMN responses to both non-verbal and verbal stimulations. The latency of the verbal/ba2/-/ba4/pair was longer than that of the nonverbal 1 kHz-1.1 kHz pair in both groups of children. Children with hearing loss, especially those who received intervention after 2 years of age, showed substantial weakness in the neural responses to lexical tones and pure tones. Thus, the chronological age when the children receive hearing intervention may have an impact on the effectiveness of discriminating between verbal and non-verbal signals. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  15. Sudden sensorineural hearing loss: Is antiviral treatment really necessary?

    Science.gov (United States)

    Övet, Gültekin; Alataş, Necat; Kocacan, Fatma Nur; Gürcüoğlu, Sermin Selver; Görgülü, Hakan; Güzelkara, Fatih; Övet, Habibe

    2015-01-01

    It was aimed to investigate the necessity of antiviral agents in the ISSHL treatment. In this study, the patients, diagnosed with sudden hearing loss and admitted in the first 7 days of hearing loss were divided into two groups; a combination therapy was administered to one of the groups, and famciclovir was administered to the other group as an antiviral treatment in addition to the combined therapy. Both groups were compared in terms of levels of recovery. No statistically significant difference was found in the recovery rates between the two groups (p=0.7). In this study, the additional antiviral treatment was found to have no effect on the remission rates in patients with ISSHL treated with combined therapy. Copyright © 2015 Elsevier Inc. All rights reserved.

  16. Treatment of idiopathic sudden sensorineural hearing loss with antiviral therapy : A prospective, randomized, double-blind clinical trial

    NARCIS (Netherlands)

    Westerlaken, BO; Stokroos, RJ; Dhooge, IJM; Wit, HP; Albers, FWJ

    2003-01-01

    A subclinical viral labyrinthitis has been postulated in the literature to elicit idiopathic sudden sensorineural hearing loss (ISSHL). An etiologic role for the herpes family is assumed. Corticosteroids possess a limited beneficial effect on hearing recovery in ISSHL. In this study, we evaluated

  17. Mutations in the Wolfram syndrome 1 gene (WFS1) are a common cause of low frequency sensorineural hearing loss.

    NARCIS (Netherlands)

    Bespalova, I.N.; Camp, G. van; Bom, S.J.H.; Brown, D.J.; Cryns, K.; Wan, A.T. de; Erson, A.E.; Flothmann, K.; Kunst, H.P.M.; Kurnool, P.; Sivakumaran, T.A.; Cremers, C.W.R.J.; Leal, S.M.; Burmeister, M.; Lesperance, M.M.

    2001-01-01

    Non-syndromic low frequency sensorineural hearing loss (LFSNHL) affecting only 2000 Hz and below is an unusual type of hearing loss that worsens over time without progressing to profound deafness. This type of LFSNHL may be associated with mild tinnitus but is not associated with vertigo. We have

  18. Mitochondrial Sensorineural Hearing Loss: A Retrospective Study and a Description of Cochlear Implantation in a MELAS Patient

    Directory of Open Access Journals (Sweden)

    Mauro Scarpelli

    2012-01-01

    Full Text Available Hearing impairment is common in patients with mitochondrial disorders, affecting over half of all cases at some time in the course of the disease. In some patients, deafness is only part of a multisystem disorder. By contrast, there are also a number of “pure” mitochondrial deafness disorders, the most common probably being maternally inherited. We retrospectively analyzed the last 60 genetically confirmed mitochondrial disorders diagnosed in our Department: 28 had bilateral sensorineural hearing loss, whereas 32 didn't present ear's abnormalities, without difference about sex and age of onset between each single group of diseases. We reported also a case of MELAS patient with sensorineural hearing loss, in which cochlear implantation greatly contributed to the patient's quality of life. Our study suggests that sensorineural hearing loss is an important feature in mitochondrial disorders and indicated that cochlear implantation can be recommended for patients with MELAS syndrome and others mitochondrial disorders.

  19. Role of Platelet Parameters on Sudden Sensorineural Hearing Loss: A Case-Control Study in Iran

    Science.gov (United States)

    2016-01-01

    Sudden sensorineural hearing loss (SSNHL) is a common otological disorder characterized by a hearing loss greater than 30 dB over three consecutive frequencies, in less than 72 hours. It has been established that platelet parameters, such as mean platelet volume, are associated with ischemic heart events, whose clinical manifestations are similar to those of SSNHL. Hence, we aimed to determine if the platelet count, mean platelet volume and platelet distribution width are related to the occurrence and severity of sudden sensorineural hearing loss. A case-control prospective study was conducted in a teaching hospital in Iran. One hundred-eight patients with SSNHL and an equal number of healthy, age- and sex-matched controls were enrolled in the study. Peripheral venous blood samples were collected from the subjects, and the platelet count, mean platelet volume and platelet distribution width were measured with an automated blood cell counter. Analysis of the audiometry and hematological test results using SPSS22 software showed no statistical correlation between the platelet parameters and the occurrence of SSNHL, but correlation coefficients showed a significant correlation between PDW and hearing loss severity in patients group. However, further investigation is required to unequivocally establish the absence of correlation between the platelet parameters and occurrence of SSNHL. PMID:26829393

  20. Perspectives for the treatment of sensorineural hearing loss by cellular regeneration of the inner ear.

    Science.gov (United States)

    Almeida-Branco, Mario S; Cabrera, Sonia; Lopez-Escamez, Jose A

    2015-01-01

    Sensorineural hearing loss is a caused by the loss of the cochlear hair cells with the consequent deafferentation of spiral ganglion neurons. Humans do not show endogenous cellular regeneration in the inner ear and there is no exogenous therapy that allows the replacement of the damaged hair cells. Currently, treatment is based on the use of hearing aids and cochlear implants that present different outcomes, some difficulties in auditory discrimination and a limited useful life. More advanced technology is hindered by the functional capacity of the remaining spiral ganglion neurons. The latest advances with stem cell therapy and cellular reprogramming have developed several possibilities to induce endogenous regeneration or stem cell transplantation to replace damaged inner ear hair cells and restore hearing function. With further knowledge of the cellular and molecular biology of the inner ear and its embryonic development, it will be possible to use induced stem cells as in vitro models of disease and as replacement cellular therapy. Investigation in this area is focused on generating cellular therapy with clinical use for the treatment of profound sensorineural hearing loss. Copyright © 2014 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

  1. Combined Intratympanic and Systemic Steroid Therapy for Poor-Prognosis Sudden Sensorineural Hearing Loss

    Directory of Open Access Journals (Sweden)

    Shima Arastou

    2012-12-01

    Full Text Available Introduction: The aim of this study was to evaluate the efficacy of combined intratympanic and systemic steroid therapy compared with systemic steroid therapy alone in idiopathic sudden sensorineural hearing loss (ISSNHL patients with poor prognostic factors.     Materials and Methods: Seventy-seven patients with sudden sensorineural hearing loss (SSNHL who had at least one poor prognostic factor (age greater than 40 years, hearing loss more than 70 db, or greater than a 2-week delay between the onset of hearing loss and initiation of therapy were included in this study. Patients were randomized to the intervention group (combined intratympanic and systemic steroid therapy or the control group (systemic steroid therapy alone. All patients received oral treatment with systemic prednisolone (1 mg/kg/day for 10 days, acyclovir (2 g/day for 10 days, divided into four doses, triamterene H (daily, and omeprazole (daily, during steroid treatment, and were advised to follow a low salt diet. The intervention group also received intratympanic dexamethasone injections (0.4 ml of 4 mg/ml dexamethasone two times a week for two consecutive weeks (four injections in total. A significant hearing improvement was defined as at least a 15-db decrease in pure tone average (PTA.  Results: Among all participants, 44 patients (57.14% showed significant improvement in hearing evaluation. More patients showed hearing improvement in the intervention group than in the control group (27 patients (75% versus 17 patients (41.4%, respectively; P = 0.001.  Conclusion:  The combination of intratympanic dexamethasone and systemic prednisolone is more effective than systemic prednisolone alone in the treatment of poor-prognosis SSNHL.

  2. The effect of initial treatment on hearing prognosis in idiopathic sudden sensorineural hearing loss: a nationwide survey in Japan.

    Science.gov (United States)

    Okada, Masahiro; Hato, Naohito; Nishio, Shin-Ya; Kitoh, Ryosuke; Ogawa, Kaoru; Kanzaki, Sho; Sone, Michihiko; Fukuda, Satoshi; Hara, Akira; Ikezono, Tetsuo; Ishikawa, Kotaro; Iwasaki, Satoshi; Kaga, Kimitaka; Kakehata, Seiji; Matsubara, Atsushi; Matsunaga, Tatsuo; Murata, Takaaki; Naito, Yasushi; Nakagawa, Takashi; Nishizaki, Kazunori; Noguchi, Yoshihiro; Sano, Hajime; Sato, Hiroaki; Suzuki, Mikio; Shojaku, Hideo; Takahashi, Haruo; Takeda, Hidehiko; Tono, Tetsuya; Yamashita, Hiroshi; Yamasoba, Tatsuya; Usami, Shin-Ichi

    2017-01-01

    To investigate the hearing prognosis of idiopathic sudden sensorineural hearing loss (SSNHL) treated with different initial therapies. Subjects consisted of patients diagnosed with idiopathic SSNHL within 7 days from onset and showing severe hearing loss (≥60 dB), who were registered in a Japanese multicenter database between April 2014 and March 2016. Subjects were divided into four groups according to initial therapy: (1) steroids, (2) steroids + Prostaglandins (PGs), (3) intratympanic steroids (ITS), and (4) no steroids. Hearing outcomes were compared among the groups. In total, 1305 patients were enrolled. The final hearing level and hearing gain of patients treated with steroids + PGs were significantly higher than those of patients treated with steroids alone or no steroids. The ratio of good prognosis (complete recovery or marked improvement) in patients treated with steroids + PGs was higher than that in patients treated with steroids alone or no steroids. There was no difference in the prognosis of patients treated with steroids alone or no steroids. A large number of patients with idiopathic SSNHL were registered in a multicenter database. PG use in combination with steroid administration was associated with a good hearing prognosis in patients with severe hearing loss.

  3. Prevalence of mitochondrial DNA mutations in sporadic patients with nonsyndromic sensorineural hearing loss.

    Science.gov (United States)

    Jiang, Hua; Chen, Jia; Li, Ying; Lin, Peng-Fang; He, Jian-Guo; Yang, Bei-Bei

    2016-01-01

    Several mitochondrial DNA mutations have been reported to be associated with nonsyndromic hearing loss in several families. However, little is known about the prevalence of these mutations in sporadic patients with nonsyndromic sensorineural hearing loss. The purpose of our study was to investigate the incidence of these mitochondrial DNA mutations in such population. A total of 178 sporadic patients with nonsyndromic sensorineural hearing loss were enrolled in this study. Genomic DNA was extracted from the peripheral blood sample. We employed the SNaPshot(®) sequencing method to detect five mitochondrial DNA mutations, including A1555G and A827G in 12S rRNA gene and A7445G, 7472insC, and T7511C in tRNA(Ser(UCN)) gene. Meanwhile, we used polymerase chain reaction and sequenced the products to screen GJB2 gene mutations in patients carrying mitochondrial DNA mutations. We failed to detect the presence of A1555G mutation in 12S rRNA gene, and of A7445G, 7472insC, T7511C mutations in tRNA(Ser(UCN)) gene in our population. However, we found that 6 patients (3.37%) were carriers of a homozygous A827G mutation and one of them also carried homozygous GJB2 235delC mutation. Our findings in the present study indicate that even in sporadic patients with nonsyndromic sensorineural hearing loss, mitochondrial DNA mutations might also contribute to the clinical phenotype. Copyright © 2015 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  4. Acoustic reflex measurements and the loudness function in sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Sheila Uliel

    1980-11-01

    Full Text Available The suprathreshold acoustic reflex responses of forty two ears affected by sensorineural hearing loss of cochlear origin and fifty-eight ears demonstrating normal hearing, were recorded by means of an electro-acoustic impedance meter and attached X-Y recorder. The recordings were done in ascending and descending fashion,  at successively increasing and decreasing 5dB intensity levels from 90-120-90 dB HL respectively, for the individual pure-tone frequencies of 500, 1 000, 2 000 and 4 000 Hz. The contralateral mode of measurement was employed. Analysis of  these recordings indicated that the acoustic reflex  responses could be differentiated into five  characteristic patterns of  growth, which could be depicted upon a continuum of peaked, peaked-rounded, rounded, rounded-flat,  and flat  shapes. The peaked and peaked-rounded patterns were found  to predominate at all four pure-tone frequencies  in the normal ears, while the rounded-fiat  and flat  patterns were found  to predominate only at the higher pure-tone frequencies of 2 000 and 4 000 Hz in the ears affected  by sensorineural hearing loss. This latter relationship was also able to be applied to two disorders of  the loudness functio— loudness recruitment and hyperacusis. It was concluded that the flattened  acoustic reflex  patterns at the higher pure-tone frequencies  constituted a potential diagnostic cue related to the differential  diagnosis of sensorineural hearing loss, and to disorders of  the loudness function.

  5. Congenital inner ear malformations without sensorineural hearing loss.

    Science.gov (United States)

    Yukawa, Kumiko; Horiguchi, Satoshi; Suzuki, Mamoru

    2008-03-01

    It has been reported that normal hearing is rare in patients with severe inner ear vestibular malformations [Kokai H, Oohashi M, Ishikawa K, Harada K, Hiratsuka H, Ogasawara M et al. Clinical review of inner ear malformation. J Otolaryngol Jpn 2003;106(10):1038-44; Schuknecht HF. Mondini dysplasia. A clinical pathological study. Ann Otol Rhinol Laryngol 1980;89(Suppl. 65):1-23; Jackler RK, Luxford WM, House WF. Congenital malformations of the inner ear: a classification based on embryogenesis. Laryngoscope 1987;97:2-14; Phelps PD. Congenital lesions of the inner ear, demonstrated by tomography. Arch Otolaryngol 1974;100:11-8]. A 37-year-old woman had combined dysplasia of the posterior and lateral semicircular canals (PSCC, LSCC) with normal cochlear development and normal hearing in both ears. She had complained of dizziness for 8 months. High resolution computed tomography (CT) showed hypogenesis of the bony labyrinth in both ears. Bilateral PSCC and LSCC dysplasia and dilatation of the vestibule were detected. Magnetic resonant imaging (MRI) revealed that the deformity of the PSCC was more severe than the LSCC. Although the caloric test of the left ear elicited no nystagmus and there was reduced response in the right ear, the horizontal vestibulo-occular reflex (VOR) was present. Her dizzy sensation disappeared within 3 months without special treatment. The dizziness attack might have been caused by a temporary breakdown of her peripheral vestibular system.

  6. Complex-Tone Pitch Discrimination in Listeners With Sensorineural Hearing Loss

    DEFF Research Database (Denmark)

    Bianchi, Federica; Fereczkowski, Michal; Zaar, Johannes

    2016-01-01

    Physiological studies have shown that noise-induced sensorineural hearing loss (SNHL) enhances the amplitude of envelope coding in auditory-nerve fibers. As pitch coding of unresolved complex tones is assumed to rely on temporal envelope coding mechanisms, this study investigated pitch-discrimination...... performance in listeners with SNHL. Pitch-discrimination thresholds were obtained for 14 normal-hearing (NH) and 10 hearing-impaired (HI) listeners for sine-phase (SP) and random-phase (RP) complex tones. When all harmonics were unresolved, the HI listeners performed, on average, worse than NH listeners...... in the RP condition but similarly to NH listeners in the SP condition. The increase in pitch-discrimination performance for the SP relative to the RP condition (F0DL ratio) was significantly larger in the HI as compared with the NH listeners. Cochlear compression and auditory-filter bandwidths were...

  7. Relation between temporal envelope coding, pitch discrimination, and compression estimates in listeners with sensorineural hearing loss

    DEFF Research Database (Denmark)

    Bianchi, Federica; Santurette, Sébastien; Fereczkowski, Michal

    2015-01-01

    Recent physiological studies in animals showed that noise-induced sensorineural hearing loss (SNHL) increased the amplitude of envelope coding in single auditory-nerve fibers. The present study investigated whether SNHL in human listeners was associated with enhanced temporal envelope coding...... resolvability. For the unresolved conditions, all five HI listeners performed as good as or better than NH listeners with matching musical experience. Two HI listeners showed lower amplitude-modulation detection thresholds than NH listeners for low modulation rates, and one of these listeners also showed a loss......, whether this enhancement affected pitch discrimination performance, and whether loss of compression following SNHL was a potential factor in envelope coding enhancement. Envelope processing was assessed in normal-hearing (NH) and hearing-impaired (HI) listeners in a behavioral amplitude...

  8. Sensorineural hearing loss degrades behavioral and physiological measures of human spatial selective auditory attention

    Science.gov (United States)

    Dai, Lengshi; Best, Virginia; Shinn-Cunningham, Barbara G.

    2018-01-01

    Listeners with sensorineural hearing loss often have trouble understanding speech amid other voices. While poor spatial hearing is often implicated, direct evidence is weak; moreover, studies suggest that reduced audibility and degraded spectrotemporal coding may explain such problems. We hypothesized that poor spatial acuity leads to difficulty deploying selective attention, which normally filters out distracting sounds. In listeners with normal hearing, selective attention causes changes in the neural responses evoked by competing sounds, which can be used to quantify the effectiveness of attentional control. Here, we used behavior and electroencephalography to explore whether control of selective auditory attention is degraded in hearing-impaired (HI) listeners. Normal-hearing (NH) and HI listeners identified a simple melody presented simultaneously with two competing melodies, each simulated from different lateral angles. We quantified performance and attentional modulation of cortical responses evoked by these competing streams. Compared with NH listeners, HI listeners had poorer sensitivity to spatial cues, performed more poorly on the selective attention task, and showed less robust attentional modulation of cortical responses. Moreover, across NH and HI individuals, these measures were correlated. While both groups showed cortical suppression of distracting streams, this modulation was weaker in HI listeners, especially when attending to a target at midline, surrounded by competing streams. These findings suggest that hearing loss interferes with the ability to filter out sound sources based on location, contributing to communication difficulties in social situations. These findings also have implications for technologies aiming to use neural signals to guide hearing aid processing. PMID:29555752

  9. Working memory and referential communication – multimodal aspects of interaction between children with sensorineural hearing impairment and normal hearing peers

    Directory of Open Access Journals (Sweden)

    Olof eSandgren

    2015-03-01

    Full Text Available Whereas the language development of children with sensorineural hearing impairment (SNHI has repeatedly been shown to differ from that of peers with normal hearing (NH, few studies have used an experimental approach to investigate the consequences on everyday communicative interaction. This mini review gives an overview of a range of studies on children with SNHI and NH exploring intra- and inter-individual cognitive and linguistic systems during communication.Over the last decade, our research group has studied the conversational strategies of Swedish speaking children and adolescents with SNHI and NH using referential communication, an experimental analogue to problem-solving in the classroom. We have established verbal and nonverbal control and validation mechanisms, related to working memory capacity (WMC and phonological short term memory (PSTM. We present main findings and future directions relevant for the field of cognitive hearing science and for the clinical and school-based management of children and adolescents with SNHI.

  10. Cochlear Homocysteine Metabolism at the Crossroad of Nutrition and Sensorineural Hearing Loss

    Directory of Open Access Journals (Sweden)

    Isabel Varela-Nieto

    2017-04-01

    Full Text Available Hearing loss (HL is one of the most common causes of disability, affecting 360 million people according to the World Health Organization (WHO. HL is most frequently of sensorineural origin, being caused by the irreversible loss of hair cells and/or spiral ganglion neurons. The etiology of sensorineural HL (SNHL is multifactorial, with genetic and environmental factors such as noise, ototoxic substances and aging playing a role. The nutritional status is central in aging disability, but the interplay between nutrition and SNHL has only recently gained attention. Dietary supplementation could therefore constitute the first step for the prevention and potential repair of hearing damage before it reaches irreversibility. In this context, different epidemiological studies have shown correlations among the nutritional condition, increased total plasma homocysteine (tHcy and SNHL. Several human genetic rare diseases are also associated with homocysteine (Hcy metabolism and SNHL confirming this potential link. Accordingly, rodent experimental models have provided the molecular basis to understand the observed effects. Thus, increased tHcy levels and vitamin deficiencies, such as folic acid (FA, have been linked with SNHL, whereas long-term dietary supplementation with omega-3 fatty acids improved Hcy metabolism, cell survival and hearing acuity. Furthermore, pharmacological supplementations with the anti-oxidant fumaric acid that targets Hcy metabolism also improved SNHL. Overall these results strongly suggest that cochlear Hcy metabolism is a key player in the onset and progression of SNHL, opening the way for the design of prospective nutritional therapies.

  11. Cochlear Homocysteine Metabolism at the Crossroad of Nutrition and Sensorineural Hearing Loss

    Science.gov (United States)

    Partearroyo, Teresa; Vallecillo, Néstor; Pajares, María A.; Varela-Moreiras, Gregorio; Varela-Nieto, Isabel

    2017-01-01

    Hearing loss (HL) is one of the most common causes of disability, affecting 360 million people according to the World Health Organization (WHO). HL is most frequently of sensorineural origin, being caused by the irreversible loss of hair cells and/or spiral ganglion neurons. The etiology of sensorineural HL (SNHL) is multifactorial, with genetic and environmental factors such as noise, ototoxic substances and aging playing a role. The nutritional status is central in aging disability, but the interplay between nutrition and SNHL has only recently gained attention. Dietary supplementation could therefore constitute the first step for the prevention and potential repair of hearing damage before it reaches irreversibility. In this context, different epidemiological studies have shown correlations among the nutritional condition, increased total plasma homocysteine (tHcy) and SNHL. Several human genetic rare diseases are also associated with homocysteine (Hcy) metabolism and SNHL confirming this potential link. Accordingly, rodent experimental models have provided the molecular basis to understand the observed effects. Thus, increased tHcy levels and vitamin deficiencies, such as folic acid (FA), have been linked with SNHL, whereas long-term dietary supplementation with omega-3 fatty acids improved Hcy metabolism, cell survival and hearing acuity. Furthermore, pharmacological supplementations with the anti-oxidant fumaric acid that targets Hcy metabolism also improved SNHL. Overall these results strongly suggest that cochlear Hcy metabolism is a key player in the onset and progression of SNHL, opening the way for the design of prospective nutritional therapies. PMID:28487633

  12. Framingham Risk Score as a Prognostic Predictor of Sudden Sensorineural Hearing Loss: A Preliminary Study.

    Science.gov (United States)

    Chang, Young-Soo; Choi, Ji Eun; Ahn, Jungmin; Ryu, Nam-Gyu; Moon, Il Joon; Hong, Sung Hwa; Cho, Yang-Sun; Chung, Won-Ho

    2017-05-01

    Predicting the prognosis of idiopathic sudden sensorineural hearing loss (ISSHL) remains challenging. This investigation aimed to apply Framingham Risk Scores (FRS) to assess the combination of prognostic factors following ISSHL and investigate the predictive role of FRS in patients with multiple comorbidities including hypertension, diabetes, and hyperlipidemia. Retrospective study. Twenty-one patients presenting with unilateral idiopathic sudden sensorineural hearing loss and multiple comorbidities were surveyed. Framingham Risk Score was calculated, and patients were assigned into high-risk (FRS ≥20%) and low-risk (FRS hearing outcomes following established criteria were investigated. All patients were treated with the same protocol of oral methylprednisolone. Overall successful recovery rate (complete + marked recovery) was 23.81%. The mean PTA threshold of the low-risk group showed significant improvement (mean PTA ± standard error, SE: pretreatment, 73.23 ± 11.80; posttreatment, 54.89 ± 10.25, P = .002), while the high-risk group did not show significant improvement in mean PTA threshold (mean PTA ± SE: pretreatment, 71.94 ± 11.77; posttreatment, 68.89 ± 12.81, P = .73). Framingham Risk Scores may be useful in predicting outcomes for ISSHL patients with multiple comorbidities.

  13. Auditory Steady-State Response Thresholds in Adults With Conductive and Mild to Moderate Sensorineural Hearing Loss

    OpenAIRE

    Hosseinabadi, Reza; Jafarzadeh, Sadegh

    2014-01-01

    Background: The Auditory steady state response (ASSR) provides a frequency-specific and automatic assessment of hearing sensitivity and is used in infants and difficult-to-test adults. Objectives: The aim of this study was to compare the ASSR thresholds among various types (normal, conductive, and sensorineural), degree (normal, mild, and moderate), and configuration (flat and sloping) of hearing sensitivity, and measuring the cutoff point between normal condition and hearing loss for differe...

  14. Multivariate analysis of prognostic factors for idiopathic sudden sensorineural hearing loss in children.

    Science.gov (United States)

    Chung, Jae Ho; Cho, Seok Hyun; Jeong, Jin Hyeok; Park, Chul Won; Lee, Seung Hwan

    2015-09-01

    To evaluate clinical characteristics and possible associated factors of idiopathic sudden sensorineural hearing loss (ISSNHL) in children using univariate and multivariate analyses. A retrospective case series with comparisons. From January 2007 to December 2013, medical records of 37 pediatric ISSNHL patients were reviewed to assess hearing recovery rate and examine factors associated with prognosis (gender; side of hearing loss; opposite side hearing loss; treatment onset; presence of vertigo, tinnitus, and ear fullness; initial hearing threshold), using univariate and multivariate analysis, and compare them with 276 adult ISSNHL patients. Pediatric patients comprised only 6.6% of pediatric/adult cases of ISSNHL, and those below 10 years old were only 0.7%. The overall recovery rates (complete and partial) of the pediatric and adult patients were 57.4% and 47.2%, respectively. The complete recovery rate of the pediatric group (46.6%) was higher than that of the adult group (30.8%, P = .040). According to multivariate analysis, absence of tinnitus, later onset of treatment, and higher hearing threshold at initial presentation were associated with a poor prognosis in pediatric ISSNHL. The recovery rate of ISSNHL in pediatric patients is higher than in adults, and the presence of tinnitus and earlier treatment onset is associated with favorable outcomes. 4. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

  15. Nationwide epidemiological survey of idiopathic sudden sensorineural hearing loss in Japan.

    Science.gov (United States)

    Kitoh, Ryosuke; Nishio, Shin-Ya; Ogawa, Kaoru; Kanzaki, Sho; Hato, Naohito; Sone, Michihiko; Fukuda, Satoshi; Hara, Akira; Ikezono, Tetsuo; Ishikawa, Kotaro; Iwasaki, Satoshi; Kaga, Kimitaka; Kakehata, Seiji; Matsubara, Atsushi; Matsunaga, Tatsuo; Murata, Takaaki; Naito, Yasushi; Nakagawa, Takashi; Nishizaki, Kazunori; Noguchi, Yoshihiro; Sano, Hajime; Sato, Hiroaki; Suzuki, Mikio; Shojaku, Hideo; Takahashi, Haruo; Takeda, Hidehiko; Tono, Testuya; Yamashita, Hiroshi; Yamasoba, Tatsuya; Usami, Shin-Ichi

    2017-01-01

    Using a large-scale nationwide survey database, we investigated the epidemiological characteristics for idiopathic SSNHL in Japan. The subjects for this analysis were patients registered in a Japanese multicentre database between April 2014 and March 2016. A total of 3419 idiopathic SSNHL patients were registered in the database, and the clinical characteristics of the idiopathic SSNHL patients were obtained. Several factors associated with the severity of hearing impairment and prognosis were then investigated. Statistical analysis was performed to clarify the factors associated with the severity of hearing impairment and prognosis. There were significant correlations between the severity of hearing loss and diabetes mellitus, kidney disease, past history of brain infarction, heart disease, age (under 16 years/elderly), and symptoms of vertigo/dizziness. We also analyzed the prognostic factors for idiopathic SSNHL, and found that the severity of hearing loss (Grade 3 or 4), heart disease, aged 65 years or over, time from onset to treatment (over 7 days), and symptoms of vertigo/dizziness were all significantly related to poor prognosis. The present large-scale clinical survey revealed current epidemiological trends for idiopathic sudden sensorineural hearing loss (SSNHL) and various factors associated with the severity of hearing impairment and prognosis.

  16. Otoacoustic emissions in the prediction of sudden sensorineural hearing loss outcome.

    Science.gov (United States)

    Shupak, Avi; Zeidan, Reem; Shemesh, Rafael

    2014-12-01

    To evaluate the role of otoacoustic emissions (OAEs) in the prediction of idiopathic sudden sensorineural hearing loss (ISSNHL) outcome. Open-label prospective study. Tertiary referral medical center. Fifteen ISSNHL patients (age: 57.6 ± 16.2 years) were prospectively followed 7 days, 14 days, and 3 months post-presentation and the commencement of treatment. Pure-tone audiometry, TEOAEs (Transient Evoked OAEs), and DPOAEs (Distortion Product OAEs) testing. The pure-tone threshold averages of the three most affected frequencies, detectability, and the signal-to-noise ratios (SNRs) values of the TEOAEs and DPOAEs were calculated. The main outcome measures were pure-tone hearing improvement, sensitivity, and specificity of the OAEs measures towards ISSNHL outcome. Patients having detectable TEOAEs on the first follow-up evaluation had average hearing improvement of 62 ± 41% whereas those with no response improved only by 11 ± 15% (P hearing improvement, results were 71 ± 37% and 10 ± 14%, respectively (P hearing improvement reached 71% and the specificity 100%. For the DPOAEs, the corresponding values were 83% and 100%. Univariate analysis showed significant contribution for the variance in hearing improvement by both TEOAEs and DPOAEs and their interaction (P values of 0.043, 0.005, and 0.009, respectively). The results suggest potential role of TEOAEs and DPOAEs evaluation in the early stage of treatment in the prediction of ISSNHL outcome.

  17. Tonotopic organisation of the auditory cortex in sloping sensorineural hearing loss.

    Science.gov (United States)

    Wolak, Tomasz; Cieśla, Katarzyna; Lorens, Artur; Kochanek, Krzysztof; Lewandowska, Monika; Rusiniak, Mateusz; Pluta, Agnieszka; Wójcik, Joanna; Skarżyński, Henryk

    2017-11-01

    Although the tonotopic organisation of the human primary auditory cortex (PAC) has already been studied, the question how its responses are affected in sensorineural hearing loss remains open. Twenty six patients (aged 38.1 ± 9.1 years; 12 men) with symmetrical sloping sensorineural hearing loss (SNHL) and 32 age- and gender-matched controls (NH) participated in an fMRI study using a sparse protocol. The stimuli were binaural 8s complex tones with central frequencies of 400 Hz CF , 800 Hz CF , 1600 Hz CF , 3200 Hz CF , or 6400 Hz CF , presented at 80 dB(C). In NH responses to all frequency ranges were found in bilateral auditory cortices. The outcomes of a winnermap approach, showing a relative arrangement of active frequency-specific areas, was in line with the existing literature and revealed a V-shape high-frequency gradient surrounding areas that responded to low frequencies in the auditory cortex. In SNHL frequency-specific auditory cortex responses were observed only for sounds from 400 Hz CF to 1600 Hz CF , due to the severe or profound hearing loss in higher frequency ranges. Using a stringent statistical threshold (p auditory cortex when outcomes obtained in all patients were contrasted with those revealed in normal hearing individuals (although statistically significant only for the secondary auditory cortex). The outcomes of the study suggest preserved patterns of large-scale tonotopic organisation in SNHL which can be further refined following auditory experience, especially when the hearing loss occurs prelingually. SNHL can induce both enlargement and reduction of the extent of responses in the topically organized auditory cortex. Copyright © 2017 The Authors. Published by Elsevier B.V. All rights reserved.

  18. Intratympanic Methylprednisolone Injection as First Line Therapy for Idiopathic Sudden Sensorineural Hearing Loss

    Directory of Open Access Journals (Sweden)

    Mukul Patar

    2017-08-01

    Full Text Available Introduction Steroid therapy is considered to be the gold standard for sudden sensorineural hearing loss (SSNHL. Delivering steroids by intratympanic injection is more efficient than systemic injections with minimum or no side effects. The present study was aimed to evaluate the efficacy and safety of intratympanic methylprednisolone injections as initial first line therapy for unilateral idiopathic SSNHL and the ease of giving it by otoendoscopy. Materials and Methods A prospective analysis was performed for the patients diagnosed as unilateral idiopathic SSNHL from April 2014 to April 2016 and receiving intratympanic steroids injections as first line therapy. Patients with unilateral sensorineural hearing loss of at least 30 dB at 3 contiguous frequencies occurring within a period of not more than 3 days are only included. All of the intratympanic steroid (ITS injections were administered as OPD procedures. Each patient was treated by 3 injections given at 3 days interval.  Results A total of 22 patients who underwent primary intratympanic steroid (ITS injection for unilateral SSNHL during the study period were included in the study. The mean age was 42.22 years (+ 9.79 and age ranged from 27 to 68 years. Patients included in our study came within 2nd to 27th day of occurrence of deafness and the mean duration (days from onset of disease to start of ITS was 7.86 days. The average hearing gain in our study was 44.22 dB. In the present study 11 patients (50% showed complete hearing improvement and 10 cases (45.45% had partial and one (4.54% showed no hearing recovery at 3 weeks follow up period. Conclusion Minimal systemic absorption with minimum or no systemic effects and high percentage of success rate encouraged the surgeons to prefer ITS as primary therapy for idiopathic unilateral SSNHL. It is effective, cheap, well-tolerated and can be performed as OPD procedure.

  19. Cochlear implantation in autistic children with profound sensorineural hearing loss.

    Science.gov (United States)

    Lachowska, Magdalena; Pastuszka, Agnieszka; Łukaszewicz-Moszyńska, Zuzanna; Mikołajewska, Lidia; Niemczyk, Kazimierz

    2016-11-19

    Cochlear implants have become the method of choice for the treatment of severe-to-profound hearing loss in both children and adults. Its benefits are well documented in the pediatric and adult population. Also deaf children with additional needs, including autism, have been covered by this treatment. The aim of this study was to assess the benefits from cochlear implantation in deafened children with autism as the only additional disability. This study analyzes data of six children. The follow-up time was at least 43 months. The following data were analyzed: medical history, reaction to music and sound, Ling's six sounds test, onomatopoeic word test, reaction to spoken child's name, response to requests, questionnaire given to parents, sound processor fitting sessions and data. After cochlear implantation each child presented other communication skills. In some children, the symptoms of speech understanding were observed. No increased hyperactivity associated with daily use cochlear implant was observed. The study showed that in autistic children the perception is very important for a child's sense of security and makes contact with parents easier. Our study showed that oral communication is not likely to be a realistic goal in children with cochlear implants and autism. The implantation results showed benefits that varied among those children. The traditional methods of evaluating the results of cochlear implantation in children with autism are usually insufficient to fully assess the functional benefits. These benefits should be assessed in a more comprehensive manner taking into account the limitations of communication resulting from the essence of autism. It is important that we share knowledge about these complex children with cochlear implants. Copyright © 2016 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  20. Cochlear implantation in autistic children with profound sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Magdalena Lachowska

    Full Text Available Abstract Introduction Cochlear implants have become the method of choice for the treatment of severe-to-profound hearing loss in both children and adults. Its benefits are well documented in the pediatric and adult population. Also deaf children with additional needs, including autism, have been covered by this treatment. Objective The aim of this study was to assess the benefits from cochlear implantation in deafened children with autism as the only additional disability. Methods This study analyzes data of six children. The follow-up time was at least 43 months. The following data were analyzed: medical history, reaction to music and sound, Ling's six sounds test, onomatopoeic word test, reaction to spoken child's name, response to requests, questionnaire given to parents, sound processor fitting sessions and data. Results After cochlear implantation each child presented other communication skills. In some children, the symptoms of speech understanding were observed. No increased hyperactivity associated with daily use cochlear implant was observed. The study showed that in autistic children the perception is very important for a child's sense of security and makes contact with parents easier. Conclusion Our study showed that oral communication is not likely to be a realistic goal in children with cochlear implants and autism. The implantation results showed benefits that varied among those children. The traditional methods of evaluating the results of cochlear implantation in children with autism are usually insufficient to fully assess the functional benefits. These benefits should be assessed in a more comprehensive manner taking into account the limitations of communication resulting from the essence of autism. It is important that we share knowledge about these complex children with cochlear implants.

  1. A retrospective study of the clinical characteristics and post-treatment hearing outcome in idiopathic sudden sensorineural hearing loss

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    Purushothaman Ganesan

    2017-02-01

    Full Text Available The purpose of this retrospective study was to analyze the clinical characteristics and document hearing recovery in patients with idiopathic sudden sensorineural hearing loss (ISSNHL. 122 patients diagnosed with unilateral ISSNHL, from March 2009 to December 2014, were treated with oral steroids and pentoxifylline. Hearing change was evaluated by comparing pre-treatment and post-treatment pure-tone average (PTA (500, 1K, and 2K Hz, and categorized into complete, partial, and no recovery of hearing. T-test, Wilcoxon Signed Rank test and Regression analysis were employed to analyze the statistical significance. Of the 122 patients, seventy-one (58% had complete recovery and 34 (28% had partial recovery. The average pre-treatment PTA was 78.3 ± 16.9 dB whereas post-treatment average was 47.0 ± 20.8 dB, showing statistically significant improvement (t=24.89, P≤0.001. The factors such as presence of tinnitus (P=0.005 and initial milder hearing loss (P=0.005 were found to be significant predictors for hearing recovery. Conventional steroid regimes produced a recovery rate in ISSNHL, which exceeds the spontaneous recovery rate. The current study results highlight the importance of medical treatment in the management of ISSNHL.

  2. A Retrospective Study of the Clinical Characteristics and Post-treatment Hearing Outcome in Idiopathic Sudden Sensorineural Hearing Loss

    Science.gov (United States)

    Kothandaraman, Purushothaman Pavanjur; Swapna, Simham; Manchaiah, Vinaya

    2017-01-01

    The purpose of this retrospective study was to analyze the clinical characteristics and document hearing recovery in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). 122 patients diagnosed with unilateral ISSNHL, from March 2009 to December 2014, were treated with oral steroids and pentoxifylline. Hearing change was evaluated by comparing pre-treatment and post-treatment pure-tone average (PTA) (500, 1K, and 2K Hz), and categorized into complete, partial, and no recovery of hearing. T-test, Wilcoxon Signed Rank test and Regression analysis were employed to analyze the statistical significance. Of the 122 patients, seventy-one (58%) had complete recovery and 34 (28%) had partial recovery. The average pre-treatment PTA was 78.3±16.9 dB whereas post-treatment average was 47.0±20.8 dB, showing statistically significant improvement (t=24.89, P≤0.001). The factors such as presence of tinnitus (P=0.005) and initial milder hearing loss (P=0.005) were found to be significant predictors for hearing recovery. Conventional steroid regimes produced a recovery rate in ISSNHL, which exceeds the spontaneous recovery rate. The current study results highlight the importance of medical treatment in the management of ISSNHL. PMID:28286637

  3. Successful treatment of sudden sensorineural hearing loss by means of pharmacotherapy combined with early hyperbaric oxygen therapy: Case report.

    Science.gov (United States)

    Olex-Zarychta, Dorota

    2017-12-01

    According to the World Health Organization reports, adult-onset hearing loss is the 15th leading cause of burden of disease, and is projected to move up to 7th by the year 2030, especially in high-income countries. Sudden sensorineural hearing loss is considered by otologists as a true otologic emergency. The current standard treatment for sudden hearing loss is a tapered course of oral high-dose corticosteroids. The described clinical case points to the validity of undertaking early hyperbaric oxygenation (HBO) therapy together with corticosteroids for full recovery of adult onset idiopathic sudden sensorineural hearing loss. A 44-year-old woman complained of an abrupt hearing deterioration in the left ear with the sensation of aural fullness and loud tinnitus presented for 48 hours. The patient was admitted to the Department of Otolaryngology of Public Hospital for diagnosis and treatment. The patient was diagnosed with unilateral sudden idiopathic sensorineural hearing loss, assessed by measuring the tonal audiograms. The patient received treatment including oral high-dose corticosteroids combined with HBO protocol including 15 daily 1-hour exposures to 100% oxygen at 2.5 atmosphere absolute. A pharmacotherapy combined with early HBO resulted in full recovery of hearing. Early implementation of HBO to the pharmacotherapy in sudden sensorineural hearing loss may lead to full recovery of hearing. There is a need for systematic research to establish guidelines for optimal number of HBO sessions in relation to the timeframe from hearing loss symptoms onset to implementation of HBO therapy. Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

  4. [Analysis of the relevant factors for recurrent sudden sensorineural hearing loss].

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    Liang, H; Zhong, S X

    2016-09-07

    Objective: To investigate the possible factors related to recurrence and prognosis of sudden sensorineural hearing loss(SSNHL). Methods: Four hundred and ninety-five patients with unilateral sudden sensorineural hearing loss between January 2013 to April 2014 were analyzed retrospectively(34 patients lost to follow-up with a dropout rate of 6.87%). Twenty of the 495 patients were diagnosed as recurrent SSNHL and treated again in the same hospital. The data of the patients were summarized to analyze the related factors which might influence the recurrence and prognosis of SSNHL. Results: In the 20 patients with recurrent SSNHL, 19 had the second attack in same ear as the first attack, and the other one had in both ears. There were seven male patients, and thirteen female patients. Patients ranged in age from 24 to 77years, with a median age of 39.5 years. Types of hearing loss: low frequency in eight patients, high frequency in two patients, flat frequency in eight patients, total deafness in two patients, the types of the second attack in 17 patients were same as the first attack, only one patient was changed from total deafness to flat frequency, one case was changed from flat frequency to high frequency, one case changed from flat frequency to total deafness. The intervals between of the first attack time and the second attack time were 1-36 months with the median time of 3.5 months. After systemic oral and (or) transtympanic steroid treatment, recovered in three cases, effective in three cases and 14 cases invalid, the cure rate was 15%, and the total effective rate was 30%. There were statistically significant differences in the recovery rate(χ 2 =8.640, P vertigo and/or dizziness, with a history more than seven days, with hypertension or diabetes mellitus, and with a type of hearing loss except low frequency type, the treatment effect was invalid. The patients with hearing loss at low frequency had the best outcomes. The total effective rates were significant

  5. Potential Benefits of Combination Therapy as Primary Treatment for Sudden Sensorineural Hearing Loss.

    Science.gov (United States)

    Lee, Jong Bin; Choi, Seong Jun

    2016-02-01

    We analyzed the effectiveness of combination therapy (CT) for idiopathic sudden sensorineural hearing loss (ISSNHL) and the utility of intratympanic dexamethasone injection (ITDI) reapplication as salvage treatment for ISSNHL refractory to CT. Case series with chart review. Academic university hospital. We reviewed 229 patients with ISSNHL and divided these patients into 2 groups according to treatment: systemic steroid therapy (SST) and CT groups. The SST group received prednisolone therapy. The CT group also received ITDI daily. Patients who demonstrated no recovery (Hearing recovery rates were 77.8% (77/99) in the CT group and 60.8% (79/130) in the SST group. The difference was statistically significant (P = .011). Initial pure-tone audiometry and vertigo were affective factors on hearing recovery rates in the CT group. After salvage therapy, hearing improvement of 10 dB or greater was noted in 6 of the 22 (27.3%) patients in the CT group and 16 of the 51 (31.4%) patients in the SST group. The difference in efficacy of salvage therapy between the CT and SST groups was simply not significant (P = .612). Combination therapy was more effective for ISSNHL in achieving hearing gain than SST alone. Furthermore, ITDI reapplication for ISSNHL refractory to CT was as effective as salvage ITDI for ISSNHL refractory to SST. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2015.

  6. Therapeutic effect of Intra-Tympanic Dexamethasone–Hyaluronic Acid Combination in Sudden Sensorineural Hearing Loss

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    Mehrdad Rogha

    2017-09-01

    Full Text Available Introduction: Hearing loss is fairly a common disorder which is usually treated with corticosteroids via systemic administration and/or intra-tympanic injection. This study aimed to compare the effectiveness of intra-tympanic injections of dexamethasone with its combination with hyaluronic acid in patients with sudden sensorineural hearing loss.   Materials and Methods: In this clinical trial, 40 patients were randomly assigned to two groups; in the first group, 20 patients received 2.4 mg intra-tympanic dexamethasone, while in the second group patients received injections of 2.4 mg of dexamethasone plus 2 mg of hyaluronic acid in combination. Patients in both groups were injected every other day to a total of three injections. The hearing status of patients was evaluated by pure tone audiometry (bone conduction threshold before and 2 weeks after the intervention.   Results: Assessment of hearing threshold before and after treatment in the two groups showed a significant difference between hearing thresholds at frequencies of 4,000 to 8,000 Hz (P

  7. Difference in the nature of dizziness between vestibular neuritis and sudden sensorineural hearing loss with vertigo.

    Science.gov (United States)

    Jung, Jae Yun; Kim, Yun-Ho; Suh, Myung-Whan

    2012-06-01

    The objective of this study was to examine whether any clinical difference existed with respect to the characteristics of dizziness in vestibular neuritis (VN) and sudden sensorineural hearing loss with vertigo (SHLV). Retrospective review. Tertiary referral center. Included in this study were 22 SHLV patients and 32 VN patients who had been confirmed of unilateral caloric weakness. Sudden sensorineural hearing loss accompanied sudden attack of rotatory vertigo. Clinical features such as comorbidity, duration of vertigo, duration of spontaneous nystagmus, and others were compared between the 2 groups. Medical records related to the vestibular function test were also reviewed. When comparing comorbid diseases and symptoms, there was no significant difference between the 2 groups. There were also no differences in the duration of spontaneous nystagmus and vertigo symptoms. However, the proportion of patients with abnormal vestibular evoked myogenic potential response was significantly smaller in the VN group compared with the SHLV group. Patients with SHLV are much more likely to have saccule/inferior vestibular nerve involvement than patients with VN.

  8. Inner ear anomalies causing congenital sensorineural hearing loss: CT and MR imaging findings

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    Hong, Hyun Sook; Paik, Sang Hyun; Cha, Jang Gyu; Park, Seong Jin; Joh, Joon Hee; Park, Jai Soung; Kim, Dae Ho; Lee, Hae Kyung; Kim, Shi Chan [Soonchunhyang University Bucheon Hospital, Buchon(Korea, Republic of)

    2005-07-15

    Many congenital dysplasias of the osseous labyrinth have been identified, and the differential diagnosis of these dysplasias is essential for delivering proper patient management. We retrospectively reviewed the computed tomography (CT) and magnetic resonance (MR) imaging findings of 20 children who had congenital sensorineural hearing loss. The children included cases of enlarged vestibular aqueduct and endolymphatic sac (n=8), aplasia of the semicircular canal (n=4), lateral semicircular canal-vestibule dysplasia (n=3), common cavity malformations with a large vestibule (n=1), cochlear hypoplasia (n=1), Mondini's dysplasia with large vestibular aqueduct (n=1), Mondini's dysplasia with a large vestibule (n=1), and small internal auditory canal (n=1). Six cases were unilateral. Nine cases had combined deformities, and nine cased had cochlear implants. CT was performed with a 1.0-mm thickness in the direct coronal and axial sections with using bone algorithms. MR was performed with a temporal 3D T2 FSE 10-mm scan and with routine brain images. We describe here the imaging features for the anomalies of the inner ear in patients suffering from congenital sensorineural hearing loss.

  9. Inner ear anomalies causing congenital sensorineural hearing loss: CT and MR imaging findings

    International Nuclear Information System (INIS)

    Hong, Hyun Sook; Paik, Sang Hyun; Cha, Jang Gyu; Park, Seong Jin; Joh, Joon Hee; Park, Jai Soung; Kim, Dae Ho; Lee, Hae Kyung; Kim, Shi Chan

    2005-01-01

    Many congenital dysplasias of the osseous labyrinth have been identified, and the differential diagnosis of these dysplasias is essential for delivering proper patient management. We retrospectively reviewed the computed tomography (CT) and magnetic resonance (MR) imaging findings of 20 children who had congenital sensorineural hearing loss. The children included cases of enlarged vestibular aqueduct and endolymphatic sac (n=8), aplasia of the semicircular canal (n=4), lateral semicircular canal-vestibule dysplasia (n=3), common cavity malformations with a large vestibule (n=1), cochlear hypoplasia (n=1), Mondini's dysplasia with large vestibular aqueduct (n=1), Mondini's dysplasia with a large vestibule (n=1), and small internal auditory canal (n=1). Six cases were unilateral. Nine cases had combined deformities, and nine cased had cochlear implants. CT was performed with a 1.0-mm thickness in the direct coronal and axial sections with using bone algorithms. MR was performed with a temporal 3D T2 FSE 10-mm scan and with routine brain images. We describe here the imaging features for the anomalies of the inner ear in patients suffering from congenital sensorineural hearing loss

  10. The efficacy of combination therapy for idiopathic sudden sensorineural hearing loss.

    Science.gov (United States)

    Jung, Da Jung; Park, Ji Hye; Jang, Jeong Hun; Lee, Kyu-Yup

    2016-08-01

    The aim of this study was to compare the hearing results of patients with idiopathic sudden sensorineural hearing loss (ISSNHL) who initially were treated with either a combination therapy of systemic steroids (SS) and intratympanic steroid injection (IT-S) or SS only. The study followed a retrospective case-control design. One-hundred five patients who were diagnosed with ISSNHL and required treatment via admission were enrolled in this study. The control group (n = 53) was treated with SS, and the study group (n = 52) received SS as well as IT-S. We divided the patients according to pretreatment hearing levels. The outcome measured was improvement after the treatment audiology data at pretreatment were compared with those at day 10 and > 90 days after the treatment. Outcome data were analyzed using the t test, Pearson's χ(2) test, Fisher's exact test, and multivariate analyses of covariance with adjustment for pretreatment hearing levels. After more than 90 days, the average pure tone audiometry (PTA) in the control and study groups were 54.8 ± 31.4 dB and 43 ± 31.2 dB, respectively (P = 0.013). Hearing recovery rate was 57.7% in the control group and 69.8% in the study group. The hearing gain of the study group was higher than that of the control group at high frequencies within the 10 days of the treatment period. Analysis between subgroups, divided according to the severity of pretreatment hearing level, showed that the recovery rate was higher at all frequencies in the study group than in the control group in patients with mild to moderate hearing loss (pretreatment PTA hearing loss (pretreatment PTA ≥ 70 dB), the hearing outcomes of the study and control groups were not significantly different, except at a low frequency. The results of this study suggest that the treatment of ISSNHL with combination therapy results in higher hearing recovery rates when compared to treatment with SS alone, even in patients with severe hearing loss. 3b. Laryngoscope, 126

  11. Pediatric sudden sensorineural hearing loss: Etiology, diagnosis and treatment in 20 children.

    Science.gov (United States)

    Dedhia, Kavita; Chi, David H

    2016-09-01

    1. To report our experience in children with sudden-onset sensorineural hearing loss (SSNHL). 2. To describe the etiology and management of children with SSNHL. Retrospective review of 20 children with SSNHL, from 2000 to 2013 at a tertiary pediatric facility. Patients had the following inclusion criteria: history of normal hearing, hearing loss occurring in less than 3 days, and audiogram documentation. The average age of patients presenting with SSNHL is 11 years 3 months (22months-18years). Only 6 (30%) children presented prior to 2 weeks. Tinnitus (55%) was the most common associated symptoms followed by otalgia (25%), and vertigo (20%). Eight patients had bilateral hearing loss, 6 only right and 6 only left. Hearing loss severity ranged from profound (45%) being most common to mild. Etiology was unknown (30%), viral (25%), anatomic abnormality (25%), Meniere's disease (5%), autoimmune (5%), perilymphatic fistula (5%), and suppurative labyrinthitis (5%). Eight patients had initial treatment with oral steroids of which 50% had improvement on audiograms. Two patients underwent intratympanic injections, both showed improvement. Of the 12 patients with no treatment, only 1 had improved hearing. The true incidence of pediatric SSNHL is not well established in our literature. Unique aspects of pediatric SSNHL are delayed presentation and higher percent of anatomic findings. In our study 70% presented more than 2 weeks after experiencing symptoms. Anatomic abnormalities are in 40% of patients. Hearing improvement occurred in 50% of children treated with oral steroids. Intratympanic steroid treatment is another option but may have practical limitation in the pediatric population. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  12. Auditory steady-state response thresholds in adults with conductive and mild to moderate sensorineural hearing loss.

    Science.gov (United States)

    Hosseinabadi, Reza; Jafarzadeh, Sadegh

    2015-01-01

    The Auditory steady state response (ASSR) provides a frequency-specific and automatic assessment of hearing sensitivity and is used in infants and difficult-to-test adults. The aim of this study was to compare the ASSR thresholds among various types (normal, conductive, and sensorineural), degree (normal, mild, and moderate), and configuration (flat and sloping) of hearing sensitivity, and measuring the cutoff point between normal condition and hearing loss for different frequencies. This clinical trial was performed in Iran and included patients who were referred from Ear, Nose, and Throat Department. A total of 54 adults (27 with sensorineural hearing loss, 17 with conductive hearing losses, and 10 with normal hearing) were randomly chosen to participate in our study. The type and degree of hearing loss were determined through testing by otoscopy, tympanometry, acoustic reflex, and pure tone audiometry. Then the ASSR was tested at carrier frequencies of 500, 1000, 2000, and 4000 Hz. The ASSR accurately estimates the behavioral thresholds as well as flat and sloping configurations. There was no correlation between types of hearing loss and difference of behavioral and ASSR thresholds (P = 0.69). The difference between ASSR and behavioral thresholds decreased as severity of hearing loss increased. The 40, 35, 30, and 35 dB could be considered as cutoffs between normal hearing and hearing loss for 500, 1000, 2000, and 4000 Hz, respectively. The ASSR can accurately predict the degree and configuration of hearing loss and discriminate the normal hearing from mild or moderate hearing loss and mild from moderate hearing loss, except for 500 Hz. The Air-conducted ASSR could not define the type of hearing loss.

  13. Prognostic impact of gene polymorphisms in patients with idiopathic sudden sensorineural hearing loss.

    Science.gov (United States)

    Kitoh, Ryosuke; Nishio, Shin-Ya; Usami, Shin-Ichi

    2017-01-01

    To investigate whether genetic polymorphisms (single-nucleotide polymorphism [SNPs]) have a prognostic influence on hearing recovery after standardized corticosteroid therapy. A total of 192 gene samples from idiopathic sudden sensorineural hearing loss (SSNHL) patients registered in the Intractable Inner Ear Disease Gene Bank were enrolled and, as the candidate genes, 16 SNPs from 13 genes were selected for this study. Fischer's exact test was used to compare allele frequencies in each SNP between the patients with good hearing recovery and patients with poor hearing recovery. The SNPs of the GSR gene (rs2251780 and rs3779647) and NOS3 gene (rs1799983) were associated with treatment outcome (p < .05). Furthermore, there was a marginal correlation between the SNP of the NR3C1 gene (rs4912910) and treatment outcome (p = .05). The results of this study indicate that the analysis of genetic factors might make it possible to predict the treatment outcome, at least in part, in patients with idiopathic SSNHL.

  14. Sudden sensorineural hearing loss: is there a relationship between routine haematological parameters and audiogram shapes?

    Science.gov (United States)

    Salvago, Pietro; Rizzo, Serena; Bianco, Antonino; Martines, Francesco

    2017-03-01

    To investigate the relationship between haematological routine parameters and audiogram shapes in patients affected by sudden sensorineural hearing loss (SSNHL). A retrospective study. All patients were divided into four groups according to the audiometric curve and mean values of haematological parameters (haemoglobin, white blood cell, neutrophils and lymphocytes relative count, platelet count, haematocrit, prothrombin time, activated partial thromboplastin time, fibrinogen and neutrophil-to-lymphocite ratio) of each group were statistically compared. The prognostic role of blood profile and coagulation test was also examined. A cohort of 183 SSNHL patients without comorbidities. With a 48.78% of complete hearing recovery, individuals affected by upsloping hearing loss presented a better prognosis instead of flat (18.36%), downsloping (19.23%) and anacusis (2.45%) groups (p = 0.0001). The multivariate analysis of complete blood count values revealed lower mean percentage of lymphocytes (p = 0.041) and higher platelet levels (p = 0.015) in case of downsloping hearing loss; with the exception of fibrinogen (p = 0.041), none of the main haematological parameters studied resulted associated with poorer prognosis. Our work suggested a lack of association between haematological parameters and a defined audiometric picture in SSNHL patients; furthermore, only fibrinogen seems to influence the prognosis of this disease.

  15. Expression of toll-like receptor genes in leukocytes of patients with sudden sensorineural hearing loss.

    Science.gov (United States)

    Yang, Chao-Hui; Hwang, Chung-Feng; Yang, Ming-Yu; Lin, Pai-Mei; Chuang, Jiin-Haur

    2015-12-01

    Sudden sensorineural hearing loss (SNNHL) is a disease entity that could be caused by multiple etiologies in which the innate immunity status of the patients might be involved. The aim of this study is to investigate the expression of Toll-like receptor (TLR) genes in peripheral blood leukocytes of SNNHL patients. Basic research. We examined the expression of six TLR genes in the peripheral blood leukocytes of SNNHL patients and normal controls using real-time quantitative reverse transcriptase-polymerase chain reaction. We found significantly higher expression of TLR2, TLR3, TLR4, TLR7, TLR8, and TLR9 genes in SNNHL patients as compared with normal controls (P hearing loss compared with those with less severe hearing loss (P hearing thresholds of the affected ear (P < 0.05). Our study implies a role for TLRs in SNNHL. The expression of TLR2 in particular correlates with the severity of the disease. N/A. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

  16. Sudden Bilateral Sensorineural Hearing Loss Associated with HLA A1-B8-DR3 Haplotype

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    G. Psillas

    2013-01-01

    Full Text Available Sudden sensorineural hearing loss may be present as a symptom in systemic autoimmune diseases or may occur as a primary disorder without another organ involvement (autoimmune inner ear disease. The diagnosis of autoimmune inner ear disease is still predicated on clinical features, and to date specific diagnostic tests are not available. We report a case of bilateral sudden hearing loss, tinnitus, intense rotatory vertigo, and nausea in a female patient in which the clinical manifestations, in addition to raised levels of circulating immune complexes, antithyroglobulin antibodies, and the presence of the HLA A1-B8-DR3 haplotype, allowed us to hypothesize an autoimmune inner ear disease. Cyclosporine-A immunosuppressive treatment in addition to steroids helped in hearing recovery that occurred progressively with normalization of the hearing function after a five-month treatment. Cyclosporine-A could be proposed as a therapeutic option in case of autoimmune inner ear disease allowing the suspension of corticosteroids that, at high dose, expose patients to potentially serious adverse events.

  17. The treatment of idiopathic sudden sensorineural hearing loss using pulse therapy : A prospective, randomized, double-blind clinical trial

    NARCIS (Netherlands)

    Westerlaken, Boris O.; Kleine, Emile de; van der Laan, Bernard; Albers, Frans

    Objectives. The etiology and treatment of idiopathic sudden sensorineural hearing loss (ISSHL) is still unclear. The anti-inflammatory effect of corticosteroids is thought to play an important part in the recovery from ISSHL. We aimed to determine whether a more powerful anti-inflammatory technique

  18. A Comparison of Phonological Processing Skills of Children with Mild to Moderate Sensorineural Hearing Loss and Children with Dyslexia

    Science.gov (United States)

    Park, Jungjun; Lombardino, Linda J.

    2012-01-01

    Using the Comprehensive Test of Phonological Processes (Wagner, Torgesen, & Rashotte, 1999), the researchers compared strengths and weaknesses in phonological processing skills in three groups: 21 children with mild to moderate sensorineural hearing loss (MSNH group), 29 children with dyslexia, and 30 age-matched controls. The MSNH group showed…

  19. A Dutch family with progressive autosomal dominant non-syndromic sensorineural hearing impairment linked to DFNA13.

    NARCIS (Netherlands)

    Ensink, R.J.H.; Huygen, P.L.M.; Snoeckx, R.L.; Caethoven, G.; Camp, G. van; Cremers, C.W.R.J.

    2001-01-01

    We present a Dutch family with autosomal dominantly inherited mid-frequency and high-frequency sensorineural hearing impairment. Genetic linkage analysis in this family indicated linkage to DFNA13 with logarithm of the odds ratio (LOD) scores > +4. The majority of the affected persons presented

  20. A connexin 26 mutation causes a syndrome of sensorineural hearing loss and palmoplantar hyperkeratosis (MIM 148350)

    OpenAIRE

    Heathcote, K.; Syrris, P.; Carter, N.; Patton, M.

    2000-01-01

    We report a missense mutation in the connexin 26 gene (GJB2) in a family with an autosomal dominant syndrome of hearing loss and hyperkeratosis. The affected family members have high frequency, slowly progressive, bilateral, sensorineural hearing loss and palmoplantar hyperkeratosis. The mutation causes an amino acid substitution (G59A), which may disrupt a reverse turn in the first extracellular loop of connexin 26. Connexin 26 mutations have been reported in syndromes of deafness and palmop...

  1. Second Hand Smoke is Associated with Sensorineural Hearing Loss in Adolescents

    Science.gov (United States)

    Lalwani, Anil K.; Liu, Ying-Hua; Weitzman, Michael

    2014-01-01

    Background Second hand smoke (SHS) exposure, either in utero or during childhood, has been linked to low birth weight, sudden infant death syndrome, upper and lower respiratory infections, increased asthma severity, dental caries, behavioral problems, ADHD, emotional problems, and otitis media (OM).To our knowledge, no previous study has examined the possible association between SHS and sensorineural hearing loss (SNHL) in adolescent. Objectives The study objectives were to (1) exam risk factors for sensorial hearing loss in different age, gender, race, and income/poverty groups among adolescents (age 12 to 19) in the U.S. using data from most recent waves of NHANES (2005–2006); and, (2) evaluate the independent association between SHS and sensorial hearing loss among adolescents. Design Cross-sectional analysis of nationally representative data. Setting National Health and Nutrition Examination Survey 2005–2006. Participants 1533 non-institutionalized adolescents age 12–19 who underwent audiometric testing, had serum cotinine levels available, and were not actively smoking. Measurements The serum cotinine levels, presence of household smokers, and self-report of smoking were used to determine SHS exposure and active smoking. Low frequency hearing loss was defined as the average pure tone level greater than 15 dB for 500, 1000, and 2000 Hz; high frequency hearing loss was defined as the average pure tone level greater than 15 dB for 3, 4, 6, and 8 kHz. Results SHS exposure was associated with elevated pure tone hearing levels at 2, 3 and 4 kHz. and 1.8 fold increased risk of unilateral low frequency SNHL in multivariate analyses (95% C.I.: 1.08-3.46). The incidence of SNHL was directly related to level of SHS exposure as reflected in serum cotinine levels. In addition, nearly 82% of adolescents with low frequency SNHL did not report hearing difficulty. Conclusions SHS is associated with increased incidence of LFSNHL that is directly related to level of

  2. Progressive sensorineural hearing loss, subjective tinnitus and vertigo caused by elevated blood lipids.

    Science.gov (United States)

    Pulec, J L; Pulec, M B; Mendoza, I

    1997-10-01

    The otologist frequently sees patients with progressive sensorineural hearing loss, subjective aural tinnitus and vertigo with no apparent cause. Elevated blood lipids may be a cause of inner ear malfunction on a biochemical basis. To establish the true incidence of this condition, all new patients (4,251) seen during an eight-year period were evaluated; of these, 2,332 patients had complaints of inner ear disease. All had a complete neurotologic examination, appropriate audiometric and vestibular studies and imaging, and blood tests including lipid phenotype studies. Hyperlipoproteinemia was found in 120 patients (5.1%). Most patients were found to be overweight and had additional coexisting conditions such as diabetes mellitus. Treatment with vasodilators and a 500-calorie, high-protein, low-carbohydrate diet yielded improvement of symptoms in 83% of patients within five months of initiation of treatment.

  3. Immune system of the inner ear as a novel therapeutic target for sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Takayuki eOkano

    2014-09-01

    Full Text Available Sensorineural hearing loss (SNHL is a common clinical condition resulting from dysfunction in one or more parts in the auditory pathway between the inner ear and auditory cortex. Despite the prevalence of SNHL, little is known about its etiopathology, although several mechanisms have been postulated including ischemia, viral infection or reactivation, and microtrauma. Immune-mediated inner ear disease has been introduced and accepted as one SNHL pathophysiology; it responds to immunosuppressive therapy and is one of the few reversible forms of bilateral SNHL. The concept of immune-mediated inner ear disease is straightforward and comprehensible, but criteria for clinical diagnosis and the precise mechanism of hearing loss have not been determined. Moreover, the therapeutic mechanisms of corticosteroids are unclear, leading to several misconceptions by both clinicians and investigators concerning corticosteroid therapy. This review addresses our current understanding of the immune system in the inner ear and its involvement in the pathophysiology in SNHL. Treatment of SNHL, including immune-mediated inner ear disorder, will be discussed with a focus on the immune mechanism and immunocompetent cells as therapeutic targets. Finally, possible interventions modulating the immune system in the inner ear to repair the tissue organization and improve hearing in patients with SNHL will be discussed. Tissue macrophages in the inner ear appear to be a potential target for modulating the immune response in the inner ear in the pathophysiology of SNHL.

  4. [Treatment outcome of patients with idiopathic sudden sensorineural hearing loss and concomitant benign paroxysmal positional vertigo].

    Science.gov (United States)

    Zhang, Haixiong; Zhang, Qing; Xu, Dadao; Xu, Min

    2014-08-01

    To evaluate the treatment outcome of patients with idiopathic sudden sensorineural hearing loss (ISSHL) with concomitant benign paroxysmal positional vertigo (BPPV). Out of 252 ISSHL patients, 96 patients were diagnosed with complicating vertigo and examined using electronystagmography. All patients were divided into three groups, namely, ISSHL without vertigo group (n=156), ISSHL with non-BPPV vertigo group (n=70) and ISSHL with BPPV group (n=26). All patients received daily intravenous infusion of 200,000 U urokinase for 3 consecutive days and 100,000 U for 3 additional days. Concomitant medications included intravenous Ginkgo biloba leave compound and vitamin B6 and intramuscular vitamins B1 and B12 for 2 weeks. Twenty-six patients diagnosed with BPPV on electronystagmography positioning test also received canalith repositioning maneuver. Vertigo-associated symptoms completely resolved after canalith repositioning maneuver in ISSHL patients with complicating BPPV. With respect to post-treatment hearing, ISSHL patients without vertigo exhibited a similar improvement as compared to those with BPPV, while those with non-BPPV vertigo had a significantly less improvement than those without vertigo and with BPPV. ISSHL patients with complicating BPPV exhibit a relatively favorable outcome with respect to hearing and vertigo-associated symptoms after medical and repositioning treatment.

  5. Incidence and factors associated with sensorineural and conductive hearing loss among survivors of congenital diaphragmatic hernia.

    Science.gov (United States)

    Partridge, Emily A; Bridge, Christina; Donaher, Joseph G; Herkert, Lisa M; Grill, Elena; Danzer, Enrico; Gerdes, Marsha; Hoffman, Casey H; D'Agostino, Jo Ann; Bernbaum, Judy C; Rintoul, Natalie E; Peranteau, William H; Flake, Alan W; Adzick, N Scott; Hedrick, Holly L

    2014-06-01

    The reported incidence of sensorineural hearing loss (SNHL) in long-term survivors of congenital diaphragmatic hernia varies widely in the literature. Conductive hearing loss (CHL) is also known to occur in CDH patients, but has been less widely studied. We sought to characterize the incidence and risk factors associated with SNHL and CHL in a large cohort of CDH patients who underwent standardized treatment and follow-up at a single institution. We retrospectively reviewed charts of all CDH patients in our pulmonary hypoplasia program from January 2004 through December 2012. Categorical variables were analyzed by Fisher's exact test and continuous variables by Mann-Whitney t-test (p≤0.05). A total of 112 patients met study inclusion criteria, with 3 (2.7%) patients diagnosed with SNHL and 38 (34.0%) diagnosed with CHL. SNHL was significantly associated with requirement for ECMO (p=0.0130), prolonged course of hospitalization (p=0.0011), duration of mechanical ventilation (p=0.0046), requirement for tracheostomy (p=0.0013), and duration of loop diuretic (p=0.0005) and aminoglycoside therapy (p=0.0003). We have identified hearing anomalies in over 30% of long-term CDH survivors. These findings illustrate the need for routine serial audiologic evaluations throughout childhood for all survivors of CDH and stress the importance of targeted interventions to optimize long-term developmental outcomes pertaining to speech and language. Copyright © 2014 Elsevier Inc. All rights reserved.

  6. Short-term changes in tinnitus pitch related to audiometric shape in sudden sensorineural hearing loss.

    Science.gov (United States)

    Kim, Tae Su; Yoo, Myung Hoon; Lee, Hwan Seo; Yang, Chan Joo; Ahn, Joong Ho; Chung, Jong Woo; Park, Hong Ju

    2016-06-01

    Sudden sensorineural hearing loss (SSNHL) is frequently accompanied by tinnitus. This study investigated the relationships between new-onset tinnitus and audiometric parameters in SSNHL patients. Sixty-one patients with SSNHL with new-onset tinnitus were enrolled. Changes of tinnitus pitch, and their correlations with the maximum hearing loss frequency (Fmax) and the edge frequency (Fedge) at initial and 1-month follow-up (FU) were analyzed. At 1-month FU, tinnitus disappeared in 16 (26%) patients and they also showed hearing normalization. In 36 patients who still showed tonal tinnitus, the mean tinnitus pitch (2.9 kHz) at initial examination, which was close to Fedge (2.7 kHz), increased to a significantly higher frequency (4.6 kHz) at 1-month examination, which was close to Fmax (5.6 kHz). The tinnitus pitch had a more significant correlation with Fedge (r=0.46) than with Fmax (r=0.33) at initial examination and the tinnitus pitch showed a significant correlation only with Fmax (r=0.52) at 1-month examination. Hearing normalization was crucial for the disappearance of tinnitus at 1-month FU. Patients who still had tonal tinnitus at 1-month FU showed the tinnitus pitch closer to the edge frequency (Fedge) at initial examination, and this tinnitus pitch increased to the maximum hearing loss frequency (Fmax) at 1-month FU. This change in tinnitus pitch may give insight about tinnitus generation. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  7. Hearing Instruments for Unilateral Severe-to-Profound Sensorineural Hearing Loss in Adults: A Systematic Review and Meta-Analysis.

    Science.gov (United States)

    Kitterick, Pádraig Thomas; Smith, Sandra Nelson; Lucas, Laura

    2016-01-01

    A systematic review of the literature and meta-analysis was conducted to assess the nature and quality of the evidence for the use of hearing instruments in adults with a unilateral severe to profound sensorineural hearing loss. The PubMed, EMBASE, MEDLINE, Cochrane, CINAHL, and DARE databases were searched with no restrictions on language. The search included articles from the start of each database until February 11, 2015. Studies were included that (a) assessed the impact of any form of hearing instrument, including devices that reroute signals between the ears or restore aspects of hearing to a deaf ear, in adults with a sensorineural severe to profound loss in one ear and normal or near-normal hearing in the other ear; (b) compared different devices or compared a device with placebo or the unaided condition; (c) measured outcomes in terms of speech perception, spatial listening, or quality of life; (d) were prospective controlled or observational studies. Studies that met prospectively defined criteria were subjected to random effects meta-analyses. Twenty-seven studies reported in 30 articles were included. The evidence was graded as low-to-moderate quality having been obtained primarily from observational before-after comparisons. The meta-analysis identified statistically significant benefits to speech perception in noise for devices that rerouted the speech signals of interest from the worse ear to the better ear using either air or bone conduction (mean benefit, 2.5 dB). However, these devices also degraded speech understanding significantly and to a similar extent (mean deficit, 3.1 dB) when noise was rerouted to the better ear. Data on the effects of cochlear implantation on speech perception could not be pooled as the prospectively defined criteria for meta-analysis were not met. Inconsistency in the assessment of outcomes relating to sound localization also precluded the synthesis of evidence across studies. Evidence for the relative efficacy of

  8. Hyperbaric oxygen therapy as salvage treatment for sudden sensorineural hearing loss: a prospective controlled study.

    Science.gov (United States)

    Pezzoli, M; Magnano, M; Maffi, L; Pezzoli, L; Marcato, P; Orione, M; Cupi, D; Bongioannini, G

    2015-07-01

    The most commonly used treatment for sensorineural sudden hearing loss (SSHL) in clinical practice is the administration of steroids; however, a favorable result is not always obtained. We studied 58 patients who failed to recover after primary treatment with IV steroids, 44 of these met our inclusion criteria (mean age 50.7, 27 males, range 30-74). We treated 23 patients (mean age 47.3, 16 males, age range 22-74) with hyperbaric oxygen therapy (HBO) (2.5 ATA for 60 min for 15 treatments), while 21 (mean age 54.5, 11 males, age range 22-71) patients refused to be treated and served as a non-randomized control group. Patients treated with HBO had a mean improvement of 15.6 dB (SD ± 15.3), with 1 of them completely healed, 5 with a good recovery, 10 with a fair recovery and 7 unchanged. Patients who were not treated had a spontaneous mean improvement of 5.0 dB (SD ± 11.4) with 3 patients with a good recovery, 1 patient with a fair recovery and 17 patients unchanged. Mean improvement was significantly better in patients treated with HBO compared to controls (p = 0.0133). Patients with worst hearing had the greater degree of improvement whether or not they were treated in the first 10 days after the onset of the hearing loss or between 11 and 30 days. In conclusion, hyperbaric oxygen therapy can lead to significant improvement of pure tone hearing thresholds in patients with SSHL who failed primary corticosteroid treatment and are within 4 weeks of the onset of deafness.

  9. Assessment of balance and vestibular functions in patients with idiopathic sudden sensorineural hearing loss.

    Science.gov (United States)

    Liu, Jia; Zhou, Ren-Hong; Liu, Bo; Leng, Yang-Ming; Liu, Jing-Jing; Liu, Dong-Dong; Zhang, Su-Lin; Kong, Wei-Jia

    2017-04-01

    This study investigated the relationship among the severity of hearing impairment, vestibular function and balance function in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). A total of 35 ISSNHL patients (including 21 patients with vertigo) were enrolled. All of the patients underwent audiometry, sensory organization test (SOT), caloric test, cervical vestibular-evoked myogenic potential (cVEMP) test and ocular vestibular-evoked myogenic potential (oVEMP) test. Significant relationship was found between vertigo and hearing loss grade (P=0.009), and between SOT VEST grade and hearing loss grade (P=0.001). The abnormal rate of oVEMP test was the highest, followed by the abnormal rates of caloric and cVEMP tests, not only in patients with vertigo but also in those without vertigo. The vestibular end organs were more susceptible to damage in patients with vertigo (compared with patients without vertigo). Significant relationship was found between presence of vertigo and SOT VEST grade (P=0.010). We demonstrated that vestibular end organs may be impaired not only in patients with vertigo but also in patients without vertigo. The cochlear and vestibular impairment could be more serious in patients with vertigo than in those without vertigo. Vertigo does not necessarily bear a causal relationship with the impairment of the vestibular end organs. SOT VEST grade could be used to reflect the presence of vertigo state in the ISSNHL patients. Apart from audiometry, the function of peripheral vestibular end organs and balance function should be evaluated to comprehensively understand ISSNHL. Better assessment of the condition will help us in clinical diagnosis, treatment and prognosis evaluation of ISSNHL.

  10. Intratympanic dexamethasone injection vs methylprednisolone for the treatment of refractory sudden sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Nezamoddin Berjis

    2016-01-01

    Full Text Available Background: During the past years various drugs have been used for sudden sensorineural hearing loss (SSNHL treatment including steroids that are shown to be beneficial. Directed delivery of high doses of steroids into the inner ear is suggested for its potential and known as intratympanic steroids therapy (IST. Despite the use of dexamethasone and methylprednisolone as the traditional treatments, there are still debates about the optimal dosage, preferred drug, and the route of administration. Materials and Methods: We performed a randomized clinical trial study in which 50 patients suffering from SSNHL and resistant to standard therapy were employed. Each patient took 0.5 ml methylprednisolone (40 mg/mg along with bicarbonate or dexamethasone (4 mg/mL through direct intratympanic injection. This method was performed and scheduled once every 2 days for three times only for the dexamethasone receiving group. Hearing test was carried out and the results were analyzed according to a four-frequency (0.5, 1.0, 2.0, 3.0 kHz pure tone average (PTA and Siegel′s criteria. Results: According to Siegel′s criteria, three out of 25 (12% dexamethasone receiving patients were healed in 1 and 4 (16%, 9 (32% were respectively recovered in Siegel′s criteria 2, 3, and 9 (32% showed no recovery. In the group receiving methylprednisolone, recovery was found in 6 (24%, 8 (32%, 7 (28% patients in the Siegel′s criteria 1, 2, 3, respectively, and in 4 (16% patients no recovery was recorded. In methylprednisolone group, hearing was significantly improved compared to the dexamethasone group (P < 0.05. The general hearing improvement rate was 84% in methylprednisolone receiving patients showing a significantly higher improvement than 64% in the dexamethasone group. Conclusions: Topical intratympanic treatment with methylprednisolone is safe and an effective treatment approach for those SSNHL cases that are refractory to the common therapies by Dexamethasone.

  11. Our experience in the treatment of idiopathic sensorineural hearing loss (ISNHL): Effect of combination therapy with HBO2 and vasodilator infusion therapy

    Czech Academy of Sciences Publication Activity Database

    Kratochvílová, B.; Profant, Oliver; Astl, J.; Holý, R.

    2016-01-01

    Roč. 43, č. 7 (2016), s. 771-780 ISSN 1066-2936 Institutional support: RVO:68378041 Keywords : idiopathic sensorineural hearing logs * vasodilator infusion * hyperbaric oxygen therapy Subject RIV: FH - Neurology Impact factor: 0.895, year: 2016

  12. Randomised, double-blinded, placebo-controlled, clinical trial of ozone therapy as treatment of sudden sensorineural hearing loss.

    Science.gov (United States)

    Ragab, A; Shreef, E; Behiry, E; Zalat, S; Noaman, M

    2009-01-01

    To investigate the safety and efficacy of ozone therapy in adult patients with sudden sensorineural hearing loss. Prospective, randomised, double-blinded, placebo-controlled, parallel group, clinical trial. Forty-five adult patients presented with sudden sensorineural hearing loss, and were randomly allocated to receive either placebo (15 patients) or ozone therapy (auto-haemotherapy; 30 patients). For the latter treatment, 100 ml of the patient's blood was treated immediately with a 1:1 volume, gaseous mixture of oxygen and ozone (from an ozone generator) and re-injected into the patient by intravenous infusion. Treatments were administered twice weekly for 10 sessions. The following data were recorded: pre- and post-treatment mean hearing gains; air and bone pure tone averages; speech reception thresholds; speech discrimination scores; and subjective recovery rates. Significant recovery was observed in 23 patients (77 per cent) receiving ozone treatment, compared with six (40 per cent) patients receiving placebo (p ozone-treated patients compared with placebo-treated patients (p Ozone therapy is a significant modality for treatment of sudden sensorineural hearing loss; no complications were observed.

  13. Metabolomic analysis of urine with Nuclear Magnetic Resonance spectroscopy in patients with idiopathic sudden sensorineural hearing loss: A preliminary study.

    Science.gov (United States)

    Carta, Filippo; Lussu, Milena; Bandino, Fabrizio; Noto, Antonio; Peppi, Marcello; Chuchueva, Natalia; Atzori, Luigi; Fanos, Vassilios; Puxeddu, Roberto

    2017-08-01

    Idiopathic sudden sensorineural hearing loss is a frequent emergency, with unknown aetiology and usually treated with empiric therapy. Steroids represent the only validated treatment but prognosis is unpredictable and the possibility to select the patients who will not respond to steroids could avoid unnecessary treatments. Metabolomic profiling of the biofluids target the analysis of the final product of genic expression and enzymatic activity, defining the biochemical phenotype of a whole biologic system. We studied the metabolomics of the urine of a cohort of patients with idiopathic sudden sensorineural hearing loss, correlating the metabolic profiles with the clinical outcomes. Metabolomic profiling of urine samples was performed by 1 H Nuclear Magnetic Resonance spectroscopy in combination with multivariate statistical approaches. 26 patients were included in the study: 5 healthy controls, 13 patients who did not recover after treatment at 6 months while the remaining 8 patients recovered from the hearing loss. The orthogonal partial least square-discriminant analysis score plot showed a significant separation between the two groups, responders and non-responders after steroid therapy, R 2 Y of 0.83, Q 2 of 0.38 and p value sudden sensorineural hearing loss is a specific disease with unclear systemic changes, but our data suggest that there are different types of this disorder or patients predisposed to effective action of steroids allowing the recover after treatment. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  14. False air-bone gaps at 4 kHz in listeners with normal hearing and sensorineural hearing loss.

    Science.gov (United States)

    Margolis, Robert H; Eikelboom, Robert H; Johnson, Chad; Ginter, Samantha M; Swanepoel, De Wet; Moore, Brian C J

    2013-08-01

    This report presents data from four studies to examine standard bone-conduction reference equivalent threshold force levels (RETFL), especially at 4 kHz where anomalous air-bone gaps are common. Data were mined from studies that obtained air- and bone-conduction thresholds from normal-hearing and sensorineural hearing loss (SNHL) participants, using commercial audiometers and standard audiometric transducers. There were 249 normal-hearing and 188 SNHL participants. (1) Normal-hearing participants had small air-bone gaps at 0.5, 1.0, and 2.0 kHz (-1.7 to 0.3 dB) and larger air-bone gaps at 4 kHz (10.6 dB). (2) SNHL participants had small air-bone gaps at 0.5, 1.0, and 2.0 kHz (-0.7 to 1.7 dB) and a larger air-bone gap at 4 kHz (14.1 dB). (3) The 4-kHz air-bone gap grew with air-conduction threshold from 10.1 dB when the air-conduction threshold was 5-10 dB HL to 21.1 dB when the air-conduction threshold was greater than 60 dB. (4) With the 4-kHz RETFL corrected by the average SNHL air-bone gap, the relationship between RETFL and frequency is linear with a slope of - 12 dB per octave. The 4-kHz air-bone gaps for listeners with SNHL could be avoided by adjusting the 4-kHz RETFL by - 14.1 dB.

  15. Language Development and Impairment in Children With Mild to Moderate Sensorineural Hearing Loss.

    Science.gov (United States)

    Halliday, Lorna F; Tuomainen, Outi; Rosen, Stuart

    2017-06-10

    The goal of this study was to examine language development and factors related to language impairments in children with mild to moderate sensorineural hearing loss (MMHL). Ninety children, aged 8-16 years (46 children with MMHL; 44 aged-matched controls), were administered a battery of standardized language assessments, including measures of phonological processing, receptive and expressive vocabulary and grammar, word and nonword reading, and parental report of communication skills. Group differences were examined after controlling for nonverbal ability. Children with MMHL performed as well as controls on receptive vocabulary and word and nonword reading. They also performed within normal limits, albeit significantly worse than controls, on expressive vocabulary, and on receptive and expressive grammar, and worse than both controls and standardized norms on phonological processing and parental report of communication skills. However, there was considerable variation in performance, with 26% showing evidence of clinically significant oral or written language impairments. Poor performance was not linked to severity of hearing loss nor age of diagnosis. Rather, outcomes were related to nonverbal ability, maternal education, and presence/absence of family history of language problems. Clinically significant language impairments are not an inevitable consequence of MMHL. Risk factors appear to include lower maternal education and family history of language problems, whereas nonverbal ability may constitute a protective factor.

  16. Subjective visual vertical perception in patients with vestibular neuritis and sudden sensorineural hearing loss.

    Science.gov (United States)

    Ogawa, Yasuo; Otsuka, Koji; Shimizu, Shigetaka; Inagaki, Taro; Kondo, Takahito; Suzuki, Mamoru

    2012-01-01

    To determine the subjective visual vertical (SVV) perception in patients with vestibular neuritis (VN) and sudden sensorineural hearing loss (SSHL) using the SVV test and other neuro-otological examinations, namely, the vestibular evoked myogenic potential (VEMP) and caloric tests, and to clarify which vestibular nerve function is associated with an SVV shift. We performed the SVV test in 36 VN patients and 80 SSHL patients. Thereafter, we investigated directional changes in the SVV in the VN and SSHL patients, and compared the results of the SVV test with those of the VEMP and caloric tests. Abnormal SVV (> 2° was found at a rate of 69.4% in the VN patients and 26.3% in the SSHL patients. In all except 1 VN patient, the SVV tilted to the lesion side. The rate of abnormal SVV was significantly higher in patients with complete canal paresis (CP) than in patients with partial CP. There was no significant relationship between the rates of abnormal SVV and VEMP. In the SSHL patients, neither the SVV nor the VEMP affected the hearing outcome and patients with abnormal VEMP tended to show abnormal SVV. VN patients showed a higher rate of abnormal SVV than SSHL patients. From the results, it is speculated that the superior vestibular nerve function mainly affects the SVV tilt, although the inferior vestibular nerve function may also have an effect.

  17. Concomitant imaging and genetic findings in children with unilateral sensorineural hearing loss.

    Science.gov (United States)

    Gruber, M; Brown, C; Mahadevan, M; Neeff, M

    2017-08-01

    To describe the concomitant imaging and genetic findings in children diagnosed with non-syndromic unilateral sensorineural hearing loss. A retrospective cohort study was conducted of 60 children diagnosed between January 2005 and December 2015 in a tertiary-level paediatric institution. Average age at diagnosis was 4.3 years. All children were considered non-syndromic. Hearing loss was categorised as mild (17 children), moderate (17 children), severe (7 children) or profound (19 children). Imaging was performed in 43 children (71.66 per cent). Nineteen patients (44.2 per cent) had positive computed tomography or magnetic resonance imaging findings. Genetic testing was performed in 51 children (85 per cent). Sixteen children (31 per cent) tested positive to connexin 26 (GJB2); 1 patient (2 per cent) had a homozygous mutation of GJB2 and 15 were heterozygous carriers. Amongst children who tested positive as heterozygous carriers of a GJB2 mutation, there was a high rate of positive imaging findings (47 per cent compared to 37.2 per cent in the total cohort). A genetic abnormality was confirmed in 50 per cent of children with positive imaging findings who underwent genetic testing. Rates of concomitant imaging and genetic findings suggest that both investigations are of value in the study of these patients.

  18. Sudden sensorineural hearing loss: Is there a connection with inner ear electrolytic disorders? A literature review.

    Science.gov (United States)

    Ciorba, Andrea; Corazzi, Virginia; Bianchini, Chiara; Aimoni, Claudia; Skarzynski, Henryk; Skarzynski, Piotr Henryk; Hatzopoulos, Stavros

    2016-12-01

    Electrolytic disorders of the inner ear represent a model that could be implicated in partially explaining the pathogenesis of sudden sensorineural hearing loss (SSNHL). Different types of electrolytes and different inner-ear loci are involved in cochlear homeostasis physiologically, to ensure the maintenance of an ion-balanced cochlear environment allowing a normal hair cell function. It has been hypothesized that a sudden loss of endocochlear potential, due to a rapid disruption of the inner ear fluid osmolality, could be responsible for a deterioration of the hearing function caused by damaged hair cells. The aim of this paper was to review the current literature and identify sources which might validate/fortify the hypothesis that inner ear electrolytic disorders have a role in the etiopathogenesis of SSNHL. The data in the literature underline the importance of ionic homeostasis in the inner ear, but they do not support a direct link between SSNHL and electrolyte disorders/imbalances. There is marginal evidence from otoacoustic emissions research that an indirect link might be present. © The Author(s) 2016.

  19. Vitamins A, C, and E and selenium in the treatment of idiopathic sudden sensorineural hearing loss.

    Science.gov (United States)

    Kaya, Hakan; Koç, Arzu Karaman; Sayın, İbrahim; Güneş, Selçuk; Altıntaş, Ahmet; Yeğin, Yakup; Kayhan, Fatma Tülin

    2015-05-01

    This study evaluated the effectiveness of vitamins A, C, and E, with selenium, in the treatment of idiopathic sudden sensorineural hearing loss (ISSNHL). This was a prospective, controlled study performed at a tertiary teaching and research hospital. Over a 32-month period, patients were treated with either our standard ISSNHL treatment regimen plus vitamins A, C, and E and selenium (ACE+ group) or with only our standard ISSNHL treatment regimen (ACE- group). The demographics, additional symptoms, mean initial and final hearing levels, mean hearing gain, and recovery data were compared between the two groups. The ACE+ group, consisting of 70 (55.5 %) patients, received vitamin A (natural beta-carotene, 26,000 IU), vitamin C (ascorbic acid, 200 mg), vitamin E (d-alpha-tocopherol, 200 IU), and selenium (50 μg) twice daily for 30 days in addition to our ISSNHL treatment regimen: methylprednisolone at an initial dose of 1 mg/kg body weight per day, tapered over 14 days; Rheomacrodex(®) [(10 g of dextran and 0.9 g of NaCl)/100 ml] 500 ml daily for 5 days; Vastarel(®) 20-mg tablet (20 mg of trimetazidine dihydrochloride) three times daily for 30 days; and ten 60-min hyperbaric oxygen (HBO) sessions (2.5 absolute atmospheres of 100 % O2), once daily, starting the day of hospitalization. The ACE- group comprised 56 (44.4 %) patients, who received only our ISSNHL treatment regimen. The mean hearing gains were 36.2 ± 20.3 dB in the ACE+ group and 27.1 ± 20.6 dB in the ACE- group. The mean hearing gain rates were significantly higher in the ACE+ group than in the ACE- group (p = 0.014). Treatment with vitamins A, C, and E and selenium was effective in ISSNHL patients undergoing treatment with methylprednisolone, dextran, trimetazidine dihydrochloride, and HBO, and might be more effective when the initial hearing level is below 46 dB.

  20. Comparison of two different steroid treatments with hyperbaric oxygen for idiopathic sudden sensorineural hearing loss.

    Science.gov (United States)

    Sevil, Ergun; Bercin, Sami; Muderris, Togay; Gul, Fatih; Kiris, Muzaffer

    2016-09-01

    The purpose of the study was to assess the efficacy of the association of intratympanic (IT) steroid and hyperbaric oxygen (HBO) therapy in patients presenting with idiopathic sudden sensorineural hearing loss (ISSNHL), and to compare this protocol with another consisting of intravenous (IV) steroid administration and HBO therapy. A total of 80 patients diagnosed with ISSNHL were included in this prospective trial. Patients were divided into three categories: a mild-to-moderate ISSNHL group with a pure-tone average (PTA) ≤60 decibels (dB), a severe ISSNHL group with a PTA of 60-80 dB, and a profound ISSNHL group with a PTA ≥81 dB. The first protocol consisted of 20 sessions of HBO therapy together with IV methylprednisolone 1 mg/kg body weight and a 10 mg taper every 3 days for 10 days. The second protocol consisted of HBO therapy for 20 sessions, together with an IT injection of dexamethasone at a dose of 4 mg/mL, 0.5-0.7 mL once a day for 7 consecutive days, performed 3 h before the HBO therapy. In the mild-to-moderate ISSNHL patients, the mean hearing gain and successful treatment rate was 19 (0-27) dB and 78.9 %, respectively in the IT + HBO treatment group, and 18 (3-44) dB and 70.5 % in the IV + HBO therapy group. In the severe ISSNHL patients, the mean hearing gain and successful treatment rate was 33 (1-54) dB and 81.8 %, respectively in the IT + HBO treatment group and 33.5 (7-57) dB and 58.2 % in the IV + HBO group. In the profound ISSNHL patients, the mean hearing gain and successful treatment rate was 36 (4-69) dB and 40 %, respectively in the IT + HBO therapy group, and 39.5 (0-92) dB and 72.7 % in the IV + HBO treatment group. The results demonstrated that patients with severe hearing loss success rate was superior in the group submitted to IT + HBO treatment, conversely IV + HBO therapy may be benefit for patients with profound hearing loss. Nevertheless, these clinical results were not statistically significant.

  1. Beneficios económicos del implante coclear para la hipoacusia sensorineural profunda Economic benefits of the cochlear implant for treating profound sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Augusto Peñaranda

    2012-04-01

    Full Text Available OBJETIVO: Evaluar el costo-beneficio (CB, costo-utilidad (CU y costo-efectividad (CE de la implantación coclear, comparándola con el uso de audífonos en niños con hipoacusia sensorineural profunda bilateral. MÉTODOS: Se empleó la técnica no paramétrica Propensity Score Matching (PSM para realizar la evaluación de impacto económico del implante y así llevar a cabo los análisis CB, CU y CE. Se utilizó información primaria, tomada aleatoriamente a 100 pacientes: 62 intervenidos quirúrgicamente con el implante coclear (grupo de tratamiento y 38 pertenecientes al grupo de control o usuarios de audífono para tratar la hipoacusia sensorineural profunda. RESULTADOS: Se halló un diferencial de costos económicos -en beneficio del implante coclear- cercano a US$ 204 000 entre el implante y el uso de audífonos durante la esperanza de vida de los pacientes analizados. Dicha cifra indica los mayores gastos que deben cubrir los pacientes con audífono. Con este valor descontado, el indicador costo-beneficio señala que por cada dólar invertido en el implante coclear, para tratar al paciente, el retorno de la inversión es US$ 2,07. CONCLUSIONES: El implante coclear genera beneficios económicos para el paciente. También produce utilidades en salud dado que se encontró una relación positiva de CU (ganancia en decibeles y CE (ganancia en discriminación del lenguaje.OBJECTIVE: Evaluate the cost-benefit, cost-utility, and cost-effectiveness of cochlear implantation, comparing it to the use of hearing aids in children with profound bilateral sensorineural hearing loss. METHODS: The nonparametric propensity score matching method was used to carry out an economic and impact assessment of the cochlear implant and then perform cost-benefit, cost-utility, and cost-effectiveness analyses. Primary information was used, taken randomly from 100 patients: 62 who received cochlear implants (treatment group and 38 belonging to the control group who used

  2. Evaluation of the Vestibular System and Etiology in Children with Unilateral Sensorineural Hearing Loss.

    Science.gov (United States)

    Birdane, Leman; İncesulu, Armağan; Özüdoğru, Erkan; Cingi, Cemal; Caklı, Hamdi; Gürbüz, Melek Kezban; Adapınar, Baki

    2016-08-01

    The aim of this study was to evaluate the vestibular system of children with unilateral sensorineural hearing loss (USNHL), investigate the etiological factors of USNHL and analyze whether a genetic predisposition exists. Thirty-three children aged less than 18 years with USNHL, who visited the ear, nose, and throat (ENT) department between January 2004 and December 2012, were included in this study. Cases with conductive hearing loss were excluded from the study. The patients were subjected to etiologic, genetic, and ophthalmologic evaluation; radiologic imaging; electronystagmography (ENG); and vestibular evoked myogenic potential (VEMP) tests. The control group, which included 25 healthy children (13 males and 12 females), had undergone audiological assessment and were subjected to ENG and VEMP tests. All of the patients had severe-to-profound hearing loss. Mumps immunoglobulin G was positive in 22 (66.7%) of 33 patients. The 35delG mutation was not found in any of the patients. All of the patients underwent temporal computed tomography (CT) and magnetic resonance imaging (MRI). Inner ear anomaly was present in 51.5% of the patients. Overall, 21 of 31 ENG patients had canal paresis in the affected ear. The VEMP response was absent on the affected side in three patients. The n23 latency average of the patient group was longer than that of the control group. Because USNHL causes irreversible problems in children, early diagnosis and auditory rehabilitation are very important. As USNHL is accompanied by inner ear anomaly, children with USNHL should undergo temporal bone CT and MRI. To evaluate the vestibular system, ENG and VEMP are non-invasive and diagnostic tests.

  3. Loudness of brief tones in listeners with normal hearing and sensorineural hearing loss

    DEFF Research Database (Denmark)

    Buus, Søren; Florentine, Mary; Poulsen, Torben

    1997-01-01

    To investigate how hearing loss affects the loudness of brief tones, loudness matches between 5- and 200-ms tones were obtained as a function of level. Loudness functions derived from these data indicated that the gain required to restore loudness usually is the same for short and long sounds....

  4. Phonemic restoration by hearing-impaired listeners with mild to moderate sensorineural hearing loss

    NARCIS (Netherlands)

    Baskent, Deniz; Eiler, Cheryl L; Edwards, Brent

    The auditory system is capable of perceptually restoring inaudible portions of speech. This restoration may be compromised as a result of hearing impairment, particularly if it is combined with advanced age, because of degradations in the bottom-up and top-down processes. To test this hypothesis,

  5. The effect of sensorineural hearing loss and tinnitus on speech recognition over air and bone conduction military communications headsets.

    Science.gov (United States)

    Manning, Candice; Mermagen, Timothy; Scharine, Angelique

    2017-06-01

    Military personnel are at risk for hearing loss due to noise exposure during deployment (USACHPPM, 2008). Despite mandated use of hearing protection, hearing loss and tinnitus are prevalent due to reluctance to use hearing protection. Bone conduction headsets can offer good speech intelligibility for normal hearing (NH) listeners while allowing the ears to remain open in quiet environments and the use of hearing protection when needed. Those who suffer from tinnitus, the experience of perceiving a sound not produced by an external source, often show degraded speech recognition; however, it is unclear whether this is a result of decreased hearing sensitivity or increased distractibility (Moon et al., 2015). It has been suggested that the vibratory stimulation of a bone conduction headset might ameliorate the effects of tinnitus on speech perception; however, there is currently no research to support or refute this claim (Hoare et al., 2014). Speech recognition of words presented over air conduction and bone conduction headsets was measured for three groups of listeners: NH, sensorineural hearing impaired, and/or tinnitus sufferers. Three levels of speech-to-noise (SNR = 0, -6, -12 dB) were created by embedding speech items in pink noise. Better speech recognition performance was observed with the bone conduction headset regardless of hearing profile, and speech intelligibility was a function of SNR. Discussion will include study limitations and the implications of these findings for those serving in the military. Published by Elsevier B.V.

  6. Vestibular Dysfunctions in Sudden Sensorineural Hearing Loss: A Systematic Review and Meta-analysis

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    Huiqian Yu

    2018-02-01

    Full Text Available BackgroundSudden sensorineural hearing loss (SSHL not only involves cochlear function but might also be accompanied by vestibular disturbances. The assessment of vestibular function could be of great relevance in SSHL.ObjectiveTo investigate the prevalence of vestibulocochlear lesions in SSHL and the correlation of specific vestibular organs with hearing prognosis.Data sourcesA complete literature search of eligible studies in the PubMed and EMBASE databases was performed.Study selectionFor our aim, studies that focused on vestibular examination in the case of SSHL were retrieved, including caloric tests, cervical vestibular-evoked myogenic potential (cVEMP tests, or ocular vestibular-evoked myogenic potential (oVEMP tests.ResultsOf the 18 studies included, a caloric test was performed in 16 studies, cVEMP in 13 studies, and oVEMP in 5 studies, and together the studies included a total population of 1,468 subjects. The scores on the Agency for Healthcare Research and Quality (AHRQ questionnaire ranged from 6 to 11. These results indicated that the most commonly damaged vestibular organ in SSHL was the utricle and superior vestibular pathway (U + S followed by the lateral semicircular canal and superior vestibular pathway (LSC + S, the saccule and inferior vestibular pathway (S + I, and the cochlea only (C only. The meta-analysis indicated that SSHL patients with vertigo have a statistically increased risk of vestibular organ lesions compared with those without vertigo, including the LSC + S subgroup (OR = 4.89, 95% CI = 1.20–19.93, I2 = 80%, p = 0.03 and the S + I subgroup (OR = 3.58, 95% CI = 1.61–7.95, I2 = 0%, p = 0.002. The pooled possibility of hearing recovery within the LSC + S lesion group was less than half that of the non-LSC + S lesion group (OR = 0.24, 95% CI = 0.11–0.52, I2 = 68%, p = 0.0003.ConclusionThis study shows the relevance of vestibular damage

  7. Canal Paresis in Benign Paroxysmal Positional Vertigo Secondary to Sudden Sensorineural Hearing Loss.

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    Lee, Jong Bin; Choi, Seong Jun

    2015-12-01

    To review the clinical features of benign paroxysmal positional vertigo (BPPV) secondary to sudden sensorineural hearing loss (SNHL) and to analyze the correlation between canal paresis and the effectiveness of canalith repositioning procedures (CRPs) for the treatment. Study case series with chart review. Academic university hospital. We retrospectively reviewed 277 patients with BPPV. For sudden SNHL with a recent vertigo, patients underwent electronystagmography and bithermal caloric tests. We investigated the clinical characteristics including causes, type of involved semicircular canal, frequency of CRPs, treatment results, and canal paresis (CP). The diagnosis of BPPV accompanied by same-sided sudden SNHL was based on the results of head roll and Dix-Hallpike tests. Of 277 patients with BPPV, 24 (8.7%) had sudden SNHL. Multiple-canal involvement most commonly developed in post-sudden SNHL BPPV (p BPPV received a greater number of CRPs (2.95) than those with idiopathic BPPV (1.58) (p = 0.043). In patients with post-sudden SNHL BPPV, the average number of CRPs was 3.31 in the presence of CP and 2.1 sessions in the absence of CP (p = 0.030); the CP value was correlated with the frequency of CRPs (p = 0.020). In post-sudden SNHL BPPV, the worse the canal paresis becomes, the greater the number of CRPs will be needed for treatment; the simultaneous involvement of the posterior semicircular canal and horizontal semicircular canal is most common.

  8. Laboratory assessment of sudden sensorineural hearing loss: A case-control study.

    Science.gov (United States)

    Fasano, Tommaso; Pertinhez, Thelma A; Tribi, Lorenzo; Lasagni, Daniela; Pilia, Annalisa; Vecchia, Luigi; Baricchi, Roberto; Bianchin, Giovanni

    2017-10-01

    Sudden sensorineural hearing loss (SSHL) is an otologic emergency that affects five to 30 subjects per 100,000/year. The cause of SSHL remains unknown or uncertain in 70% to 90% of cases, and treatment decisions are usually made without knowing the etiology. Prospective case-control study. One hundred thirty-one idiopathic SSHL patients were recruited from January 2014 to June 2015 in concordance with the Statements of Clinical Practice Guideline and divided into groups according to the disease severity. A clinical laboratory assessment was completed on blood samples collected from SSHL patients and control subjects. Multivariable regression analysis was performed to investigate the association between laboratory data and SSHL basis. Only a few SSHL patients were positive for autoimmunity or viral infection. Statistically significant (P < .05) higher levels of blood glucose, glycated hemoglobin (HbA1C), lipoprotein (a), and factor VIII were found in SSHL patients compared to controls. Furthermore, blood glucose, HbA1C, uric acid, factor VIII, and homocysteine were significantly associated to disease severity. Gluco-metabolic, lipidic, and coagulative laboratory data support the vascular hypothesis for SSHL and its severity. 3b Laryngoscope, 127:2375-2381, 2017. © 2017 The American Laryngological, Rhinological and Otological Society, Inc.

  9. Otoacoustic Emissions in Sudden Sensorineural Hearing Loss: Changes of Measures with Treatment

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    Shadman Nemati

    2011-01-01

    Full Text Available Introduction: To identify changes in OAEs parameters in treatment course of idiopathic sudden sensorineural hearing loss (iSSNHL. Materials and Methods: In aprospective studyfromAugust 2005 to January 2009, 26 patients with iSSNHL underwent conventional audiometry/tympanometry and two types of OAEs (TEOAEs and DPOAEs before and after the completion of standard drug therapy.The changes in pre- and post- treatment parameters were compared with each other and with normal-contralateral ears. Results: In TEOAEs, the mean overall correlation (reproducibility and the mean overall strength in involved ears were 10.96±23.36 and 0.99±3.45 dB, respectively, before the treatment, which reached 22.88±36.55 and 1.85±5.3, respectively, after the treatment (P>0.05. Significant difference between “correlation score” (average of correlations at 3-4 involved frequencies before and after treatment was found: 6.52 ±18.19 vs. 21.67±37.8 (P

  10. Characteristics and application of inner ear CT in 20 cases of sensorineural hearing loss in children.

    Science.gov (United States)

    Huo, Liang; Wang, Hua

    2012-12-01

    This study shows that a number of children with sensorineural hearing loss (SNHL) have inner ear malformations demonstrated by multi-slice spiral computed tomography (MSCT). MSCT allows a comprehensive assessment of various congenital inner ear malformations through high quality multiplanar reformation (MPR) and can display the site and degree of the malformation three-dimensionally and intuitively. This is very useful for cochlear implantation. To evaluate the feasibility and usability of MSCT in pediatric SNHL with inner ear malformations. Sixty-five patients were diagnosed with SNHL by brainstem auditory evoked response (BAER). Inner ear MSCT scan and coronal MPR reconstruction were performed in all cases. This study demonstrated 20 cases (33 ears) with inner ear malformations, which included 10 ears with cochlear malformations, 7 with vestibular malformations, 5 with semicircular canal malformations, 8 with internal auditory canal (IAC) malformations, and 15 with vestibular malformations. Cochlear malformations included one ear with Michel deformity, two ears with common cavity deformity, one with cochlear aplasia, one with hypoplastic cochlea, two with incomplete partition type I, and three with incomplete partition type II (Mondini deformity).

  11. Quality-of-life outcomes after bone-anchored hearing device surgery in children with single-sided sensorineural deafness.

    Science.gov (United States)

    Doshi, Jayesh; Banga, Rupan; Child, Anne; Lawrence, Rebecca; Reid, Andrew; Proops, David; McDermott, Ann-Louise

    2013-01-01

    To report our experience in a series of children with single-sided sensorineural deafness where a bone-anchored hearing device (BAHD) was used for auditory rehabilitation. Retrospective case review. Tertiary referral centre. Eight children (4 boys and 4 girls) who had BAHD surgery for single-sided sensorineural deafness between 2007 and 2010. Bone-anchored hearing device was used for auditory rehabilitation. Glasgow Children's Benefit Inventory (GCBI), Single-sided Deafness (SSD) Questionnaire and change in health benefit scores (visual analogue scale). All but one of the children showed a positive GCBI score; the child that reported a negative score was because of low self confidence and self-esteem issues secondary to bullying at school. The results of the SSD questionnaire were generally positive with a mean satisfaction score of the BAHD as 9/10. All the children had an improvement in heath benefit. Our findings add further evidence to support patient perceived benefit of a BAHD in single sided sensorineural deafness in the paediatric population.

  12. Prevalence of TECTA mutation in patients with mid-frequency sensorineural hearing loss.

    Science.gov (United States)

    Yamamoto, Nobuko; Mutai, Hideki; Namba, Kazunori; Morita, Noriko; Masuda, Shin; Nishi, Yasuyuki; Nakano, Atsuko; Masuda, Sawako; Fujioka, Masato; Kaga, Kimitaka; Ogawa, Kaoru; Matsunaga, Tatsuo

    2017-09-25

    To date, 102 genes have been reported as responsible for non-syndromic hearing loss, some of which are associated with specific audiogram features. Four genes have been reported as causative for mid-frequency sensorineural hearing loss (MFSNHL), among which TECTA is the most frequently reported; however, the prevalence of TECTA mutations is unknown. To elucidate the prevalence of TECTA mutation in MFSNHL and clarify genotype-phenotype correlations, we analyzed the genetic and clinical features of patients with MFSNHL. Subjects with bilateral non-syndromic hearing loss were prescreened for GJB2 and m.1555A > G and m.3243A > G mitochondrial DNA mutations, and patients with inner ear malformations were excluded. We selected MFSNHL patients whose audiograms met the U-shaped criterion proposed by the GENDEAF study group, along with those with shallow U-shaped audiograms, for TECTA analysis. All TECTA exons were analyzed by Sanger sequencing. Novel missense variants were classified as possibly pathogenic, non-pathogenic, and variants of uncertain significance, based on genetic data. To evaluate novel possibly pathogenic variants, we predicted changes in protein structure by molecular modeling. Pathogenic and possibly pathogenic variants of TECTA were found in 4 (6.0%) of 67 patients with MFSNHL. In patients with U-shaped audiograms, none (0%) of 21 had pathogenic or possibly pathogenic variants. In patients with shallow U-shaped audiograms, four (8.7%) of 46 had pathogenic or possibly pathogenic variants. Two novel possibly pathogenic variants were identified and two previously reported mutations were considered as variant of unknown significance. The clinical features of patients with pathogenic and possibly pathogenic variants were consistent with those in previous studies. Pathogenic or possibly pathogenic variants were identified in 3 of 23 families (13.0%) which have the family histories compatible with autosomal dominant and 1 of 44 families (2.3%) which have

  13. Comparison of 2 and 4 Intratympanic Steroid Injections in the Treatment of Idiopathic Sudden Sensorineural Hearing Loss.

    Science.gov (United States)

    Suzuki, Hideaki; Wakasugi, Tetsuro; Kitamura, Takuro; Koizumi, Hiroki; Do, Ba Hung; Ohbuchi, Toyoaki

    2018-04-01

    We studied the effect of intratympanic steroid administration with different total injection times on hearing outcomes in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). The subjects were 191 consecutive patients (192 ears) with ISSNHL (hearing level ≥40 dB, interval between onset and treatment ≤30 days). They received systemic prednisolone (100 mg followed by tapered doses) combined with intratympanic injection of dexamethasone (4 mg/ml). Intratympanic injection was performed 4 times (days 1, 2, 4, and 7) in 92 patients (92 ears) or 2 times (days 1 and 2) in 99 patients (100 ears). The hearing outcomes were evaluated at 1 week from the start of treatment and 1 to 2 months after the completion of treatment. There was no significant difference in hearing outcomes between the 4- and 2-injection groups at either time point. Multiple regression analysis also showed that the hearing level after treatment did not depend on the total number of intratympanic steroid injections. These results indicate that a protocol using only 2 intratympanic steroid injections exerts a sufficient effect on the hearing outcomes of ISSNHL. This simplified treatment protocol would be greatly beneficial to relieve the physical and mental stress of patients.

  14. Early cortical metabolic rearrangement related to clinical data in idiopathic sudden sensorineural hearing loss.

    Science.gov (United States)

    Micarelli, Alessandro; Chiaravalloti, Agostino; Viziano, Andrea; Danieli, Roberta; Schillaci, Orazio; Alessandrini, Marco

    2017-07-01

    Results in studies concerning cortical changes in idiopathic sudden sensorineural hearing loss (ISSNHL) are not homogeneous, in particular due to the different neuroimaging techniques implemented and the diverse stages of ISSNHL studied. Considering the recent advances in state-of-the-art positron emission tomography (PET) cameras, the aim of this study was to gain more insight into the neuroanatomical differences associated with the earliest stages of unilateral ISSNHL and clinical-perceptual performance changes. After an audiological examination including the mean auditory threshold (mean AT), mean speech discrimination score (mean SDS) and Tinnitus Handicap Inventory (THI), 14 right-handed ISSNHL patients underwent brain [ 18 F]fluorodeoxyglucose (FDG)-PET within 72 h of the onset of symptoms. When compared to an homogeneous group of 35 healthy subjects by means of statistical parametric mapping, a relative increase in FDG uptake was found in the right superior and medial frontal gyrus as well as in the right anterior cingulate cortex in ISSNHL patients. Conversely, the same group showed a significant relative decrease in FDG uptake in the right middle temporal, precentral and postcentral gyrus as well as in the left posterior cingulate cortex, left lingual, superior, middle temporal and middle frontal gyrus and in the left insula. Regression analysis showed a positive correlation between mean THI and glucose consumption in the right anterior cingulate cortex and a positive correlation between mean SDS and glucose consumption in the left precentral gyrus. The relative changes in FDG uptake found in these brain regions and the positive correlation with mean SDS and THI scores in ISSNHL could possibly highlight new aspects of cerebral rearrangement, contributing to further explain changes in those functions that support speech recognition during the sudden impairment of unilateral auditory input. Copyright © 2017 Elsevier B.V. All rights reserved.

  15. Association between sudden sensorineural hearing loss and anxiety disorder: a population-based study.

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    Chung, Shiu-Dong; Hung, Shih-Han; Lin, Herng-Ching; Sheu, Jau-Jiuan

    2015-10-01

    Anxiety disorder (AD) is commonly associated with a number of physical illnesses. No previous study has investigated the association between AD and sudden sensorineural hearing loss (SSNHL). In this study, we investigated the association between prior AD and SSNHL using a population-based dataset in Taiwan. Sampled subjects of this case-control study were retrieved from the Taiwan "Longitudinal Health Insurance Database". We identified 3,522 patients who had a diagnosis of SSNHL as cases and 10,566 age- and gender-matched subjects without SSNHL as controls. A conditional logistic regression was used to calculate the odds ratio (OR) for having previously been diagnosed with AD between cases and controls. We found that of 14,088 patients, 13.4% had a prior AD diagnosis, 17.8 and 11.9% for the SSNHL group and controls, respectively. After adjusting for patient socioeconomic characteristics and comorbid medical disorders, SSNHL patients were more likely to have prior AD than the controls (OR 1.49, 95% confidence interval (CI) 1.34-1.66, p < 0.001). Furthermore, we found that the significant relationship between SSNHL and prior AD decreased with age. The relationship was the most pronounced among those aged ≤44 years, with an adjusted OR of 1.86 (95% CI 1.48-2.33, p < 0.001) for cases compared to controls. We concluded that patients with SSNHL had a higher proportion of prior AD than non-SSNHL-diagnosed controls. Further study is needed to confirm our findings and explore the underlying pathomechanisms.

  16. Jugular valve function and petrosal sinuses pressure: a computational model applied to sudden sensorineural hearing loss

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    Mirko Tessari

    2017-04-01

    Full Text Available Reports of extra-cranial venous outflow disturbances have recently been linked to sudden sensorineural hearing loss (SSNHL. Aims of the present study are: i to quantify, with mathematical model, the impact of jugular valve function on the pressure of the superior and inferior petrosal sinuses (SPS, IPS and the main auricolar veins; ii to verify the feasibility of the application of mathematical model in the clinical setting in terms of consistency respect to the usual measures of SSNHL outcome. Extra-cranial venous outflow and post analysis were respectively blindly assessed by echo colour-Doppler (ECD and a validated mathematical model for the human circulation. The pilot study was conducted on 1 healthy control and in a group of 4 patients with different outcome of SSNHL. The main finding was the significant increased pressure calculated in the SPS and IPS of patients with ipsilateral jugular obstruction due to not mobile valve leaflets (6.55 mmHg, respect to the other subjects without extracranial complete obstruction (6.01 mmHg, P=0.0006. Moreover, we demonstrated an inverted correlation between the extrapolated pressure values in the SPS/IPS and the mean flow measured in the correspondent internal jugular vein (r= –0.87773; r-squared= 0.7697; P=0.0009. The proposed mathematical model can be applied to venous extra-cranial ECD investigation in order to derive novel clinical information on the drainage of the inner ear. Such clinical information seems to provide coherent parameters potentially capable to drive the prognosis. This innovative approach was proven to be feasible by the present pilot investigation and warrants further studies with an increased sample of patients.

  17. Discovery of CDH23 as a Significant Contributor to Progressive Postlingual Sensorineural Hearing Loss in Koreans.

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    Bong Jik Kim

    Full Text Available CDH23 mutations have mostly been associated with prelingual severe-to-profound sensorineural hearing loss (SNHL in either syndromic or nonsyndromic SNHL (DFNB12. Herein, we demonstrate the contribution of CDH23 mutations to postlingual nonsyndromic SNHL (NS-SNHL. We screened 32 Korean adult probands with postlingual NS-SNHL sporadically or in autosomal recessive fashion using targeted panel or whole exome sequencing. We identified four (12.5%, 4/32 potential postlingual DFNB12 families that segregated the recessive CDH23 variants, qualifying for our criteria along with rapidly progressive SNHL. Three of the four families carried one definite pathogenic CDH23 variant previously known as the prelingual DFNB12 variant in a trans configuration with rare CDH23 variants. To determine the contribution of rare CDH23 variants to the postlingual NS-SNHL, we checked the minor allele frequency (MAF of CDH23 variants detected from our postlingual NS-SNHL cohort and prelingual NS-SNHL cohort, among the 2040 normal control chromosomes. The allele frequency of these CDH23 variants in our postlingual cohort was 12.5%, which was significantly higher than that of the 2040 control chromosomes (5.53%, confirming the contribution of these rare CDH23 variants to postlingual NS-SNHL. Furthermore, MAF of rare CDH23 variants from the postlingual NS-SNHL group was significantly higher than that from the prelingual NS-SNHL group. This study demonstrates an important contribution of CDH23 mutations to poslingual NS-SNHL and shows that the phenotypic spectrum of DFNB12 can be broadened even into the presbycusis, depending on the pathogenic potential of variants. We also propose that pathogenic potential of CDH23 variants and the clinical fate of DFNB12 may be predicted by MAF.

  18. Sensorineural hearing loss among cerebellopontine-angle tumor patients examined with pure tone audiometry and brainstem-evoked response audiometry

    Science.gov (United States)

    Rinindra, A. M.; Zizlavsky, S.; Bashiruddin, J.; Aman, R. A.; Wulani, V.; Bardosono, S.

    2017-08-01

    Tumor in the cerebellopontine angle (CPA) accurs for approximately 5-10% of all intracranial tumors, where unilateral hearing loss and tinnitus are the most frequent symptoms. This study aimed to collect data on sensorineural hearing loss in CPA tumor patients in Dr. Cipto Mangunkusumo Hospital (CMH) using pure tone audiometry and brainstem-evoked response audiometry (BERA). It also aimed to obtaine data on CPA-tumor imaging through magnetic resonance imaging (MRI). This was a descriptive, analytic, and cross-sectional study. The subjects of this study were gathered using a total sampling method from secondary data between July 2012 and November 2016. From 104 patients, 30 matched the inclusion criteria. The CPA-tumor patients in the ENT CMH outpatient clinic were mostly female, middle-aged patients (41-60 years) whose clinical presentation was mostly tinnitus and severe, asymmetric sensorineural hearing loss in 10 subjects. From 30 subjects, 29 showed ipsilaterally impaired BERA results, and 17 subjects showed contralaterally impaired BERA results. There were 24 subjects who with large-sized tumors and 19 subjects who had intracanal tumors that had spread until they were extracanal in 19 subjects.

  19. Tympanotomy with sealing of the round window as surgical salvage option in sudden idiopathic sensorineural hearing loss.

    Science.gov (United States)

    Loader, Benjamin; Atteneder, Claudia; Kaider, Alexandra; Franz, Peter

    2013-12-01

    Tympanotomy with sealing of the round window is a promising surgical alternative as a salvage strategy in younger patients with acute idiopathic sensorineural hearing loss (SNHL) and may be considered as an alternative drug delivery method to the round window. To analyze the potential benefit of the sealing of the round window with special emphasis on the age of treated patients and the influence of time elapsed between symptom onset and surgery. This was a retrospective cohort study of 25 patients (13 males,12 females) with an average age of 55 years (range 31-75 years) with sudden SNHL, who underwent an enaural tympanotomy with sealing of the round niche using triamcinolone-soaked fascia. Pre- and postoperative bone conduction thresholds were compared for each frequency and for the pure-tone average (PTA) measured at 500 Hz, 1 kHz, 2 kHz, and 3 kHZ. The time (days) between primary symptom onset and surgery as well as age was scrutinized. Hearing improvement was described as 'no improvement' (0-9 dB), 'moderate recovery' (10-29 dB), or 'marked recovery' (≥30 dB). The difference in age of patients who improved was compared to those who did not. A statistically significant improvement in PTA values (mean change 20.4 dB) was noted (p = 0.0002). In all, 13/25 (52%) patients exhibited improved bone conduction postoperatively regardless of age and time of treatment. A marked recovery (≥30 dB) could be seen in eight patients (median time to surgery, 9 days; average age, 48.5 years); recovery between 10 and 30 dB in five patients (median time to surgery, 10 days; average age, 46.6 years). Twelve significantly older patients showed no improvement (average time to surgery, 19 days; average age, 61.8 years; p = 0.004). No patient over the age of 65 years showed improvement in bone conduction.

  20. A Comparison of Thresholds in Auditory Steady - State Response with Pure Tone Audiometry in Subjects with Normal Hearing and Those with Mild and Moderate Sensorineural Hearing los

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    Sadegh Jafarzadeh

    2008-06-01

    Full Text Available Background and Aim: Among all auditory assessment tools, auditory steady state response (ASSR is a modern test. Modulation frequency for this test is usually 80 Hz. The purpose of this study, was to examined adult subjects with 40 Hz and 80 Hz ASSR and compare the results.Materials and Methods: Thirty adult (60 ears were evaluated by ASSR and PTA test, Results were divided into three groups: normal hearing, mild and moderate sensorineural hearing loss. Results: In all groups, forty hertz ASSR thresholds were relatively closer to behavioral threshold than those of 80 Hz ASSR(p<0.05. Besides, the more severe hearing loss, the lower the difference between those two thresholds. Correlation coefficients were also higher in 40 Hz ASSR(p<0.05. Conclusion: Frequency modulation thresholds with 40 Hz are more likely to be closer to the behavioral thresholds. Moreover, it has better results than the thresholds with 80 Hz.

  1. Histopathology of the Human Inner Ear in a Patient With Sensorineural Hearing Loss Caused by a Variant in DFNA5.

    Science.gov (United States)

    Nadol, Joseph B; Handzel, Ophir; Amr, Sami

    2015-12-01

    Describe the histopathology of the inner ear in a patient with hearing loss caused by a pathogenic variant of the DFNA5 gene. Variants in DFNA5 have been described as causing an autosomal dominant nonsyndromic sensorineural hearing loss. To date, there has been no description of the histopathology of the inner ear in humans with hearing loss because of pathogenic variants in DFNA5. Temporal bone histopathology by light microscopy, next-generation sequencing (NGS) of DNA obtained from blood, and Sanger sequencing of DNA obtained from formalin fixed temporal bone sections. Both the temporal bone donor and her daughter were shown to have the same pathogenic variant in the DFNA5 gene. The principal histopathologic correlates of the hearing loss were loss of the inner and outer hair cells and severe degeneration of the stria vascularis and spiral ligament throughout the cochlea. In addition, there was severe degeneration of spiral ganglion cells, particularly in the basal turn, and degeneration of vestibular neuroepithelium and neurons. The donor had undergone unilateral cochlear implantation during life. Histopathology demonstrated that the cochlear implant was inserted into the scala vestibuli with considerable new bone formation around the track of the implanted electrode. This is the first report of the histopathology of the inner ear in a patient with hearing loss caused by a pathogenic variant in the DFNA5 gene.

  2. Evaluation of the outcome of CT and MR imaging in pediatric patients with bilateral sensorineural hearing loss.

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    van Beeck Calkoen, E A; Merkus, P; Goverts, S T; van de Kamp, J M; Mulder, M F; Sanchez Aliaga, E; Hensen, E F

    2018-05-01

    To evaluate the clinically relevant abnormalities as visualized on CT and MR imaging in children with symmetric and asymmetric bilateral sensorineural hearing loss (SNHL), in relation to age and the severity of hearing loss. Retrospective cohort study. Tertiary referral otology and audiology center. From January 2006 until January 2016, a total of 207 children diagnosed with symmetric and asymmetric bilateral SNHL were included. They underwent CT and/or MR imaging for the evaluation of the etiology of their hearing loss. Radiologic abnormalities associated with SNHL. 302 scans were performed in 207 children (median age of 0.8 years old) with bilateral SNHL. The most frequently identified cause of bilateral SNHL was a malformation of the labyrinth. The combined diagnostic yield of CT and MR imaging was 32%. The diagnostic yield of MR (34%) was considerably higher than that of CT (20%). We found a higher rate of abnormalities in children with profound hearing loss (41%) compared to milder hearing loss (8-29%), and in asymmetric SNHL (52%) compared to symmetric SNHL (30%). Imaging is essential in the etiologic evaluation of children with bilateral SNHL. The highest diagnostic yield is found in children with bilateral asymmetric SNHL or profound SNHL. Based on our findings, MR is the primary imaging modality of choice in the etiological evaluation of children with bilateral SNHL because of its high diagnostic yield. Copyright © 2018. Published by Elsevier B.V.

  3. Ipsilateral distortion product otoacoustic emission (2 f1-f2) suppression in children with sensorineural hearing loss

    Science.gov (United States)

    Abdala, Carolina; Fitzgerald, Tracy S.

    2003-08-01

    Distortion product otoacoustic emission (DPOAE) ipsilateral suppression has been applied to study cochlear function and maturation in laboratory animals and humans. Although DPOAE suppression appears to be sensitive to regions of specialized cochlear function and to cochlear immaturity, it is not known whether it reflects permanent cochlear damage, i.e., sensorineural hearing loss (SNHL), in a reliable and systematic manner in humans. Eight school-aged children with mild-moderate SNHL and 20 normal-hearing children served as subjects in this study. DPOAE (2 f1-f2) suppression data were collected at four f2 frequencies (1500, 3000, 4000, and 6000 Hz) using moderate-level primary tones. Features of the DPOAE iso-suppression tuning curves and suppression growth were analyzed for both subject groups. Results show that DPOAE suppression tuning curves from hearing-impaired subjects can be reliably recorded. DPOAE suppression tuning curves were generally normal in appearance and shape for six out of eight hearing-impaired subjects but showed subtle abnormalities in at least one feature. There was not one single trend or pattern of abnormality that characterized all hearing-impaired subjects. The most prominent patterns of abnormality included: broadened tuning, elevated tip, and downward shift of tip frequency. The unique patterns of atypical DPOAE suppression in subjects with similar audiograms may suggest different patterns of underlying sensory cell damage. This speculation warrants further investigation.

  4. Comparison of steroid administration methods in patients with idiopathic sudden sensorineural hearing loss: a retrospective observational study.

    Science.gov (United States)

    Kim, S H; Jung, S Y; Kim, M G; Byun, J Y; Park, M S; Yeo, S G

    2015-06-01

    To compare the recovery rates of patients with idiopathic sudden sensorineural hearing loss (ISSHL) treated with oral systemic steroids (PO) or intratympanic steroid injection (IT) or both. A retrospective observational study. Tertiary referral centre. Eight hundred and forty-four patients diagnosed with ISSHL within 14 days of the onset of symptoms. The patients were divided into three groups by treatment modality. Threshold of pure-tone tests, age, accompanying symptoms and underlying diseases were compared. The level of final hearing recovery was evaluated by the application of the results of the pure-tone test that was performed at least 3 months after the completion of each treatment. Final hearing recovery rate differed significantly by the type of treatment (P = 0.031). Recovery rates in the PO and combined groups were significantly higher in patients with mild (85.1% and 88.6%, respectively) than with profound (52.8% and 69.0%, respectively) hearing loss (P 0.05). Combined treatment yielded significantly higher recovery rates than other treatment modalities in patients without hypertension (HTN) and diabetes mellitus (DM) (P = 0.021). In the group treated with combined therapy, better hearing improvement was obtained than in the groups treated with systemic steroid only or with intratympanic steroid injection only without complications. These findings suggest that the combination of systemic administration and intratympanic injection may improve patient prognosis. © 2014 John Wiley & Sons Ltd.

  5. Ischemia as a potential etiologic factor in idiopathic unilateral sudden sensorineural hearing loss: Analysis of posterior circulation arteries.

    Science.gov (United States)

    Kim, Chulho; Sohn, Jong-Hee; Jang, Min Uk; Hong, Sung-Kwang; Lee, Joong-Seob; Kim, Hyung-Jong; Choi, Hui-Chul; Lee, Jun Ho

    2016-01-01

    The association between idiopathic sudden sensorineural hearing loss (ISSNHL) and the radiologic characteristics of the vertebrobasilar artery is unclear. We hypothesized that the degree and direction of vertebrobasilar artery curvature in the posterior circulation contribute to the occurrence of ISSNHL. We consecutively enrolled patients diagnosed with unilateral ISSNHL in two tertiary hospitals. Magnetic resonance images were performed in all patients to exclude specific causes of ISSNHL, such as vestibular schwannoma, chronic mastoiditis, and anterior inferior cerebellar artery infarct. We measured the following parameters of posterior circulation: vertebral and basilar artery diameter, the degree of basilar artery curvature (modified smoker criteria), and vertebral artery dominance. Pure tone audiometries were performed at admission and again 1 week and 3 months later. A total of 121 ISSNHL patients (mean age, 46.0 ± 17.3 years; 48.8% male) were included in these analyses. The proportion of patients with the left side hearing loss was larger than the proportion with the right side hearing loss (left, 57.9%; right, 42.1%). The majority of patients were characterized by a left dominant vertebral artery and right-sided basilar artery curvature. The direction of the basilar artery curvature was significantly associated with hearing loss lateralization (p = 0.036). Age and sex matched multivariable analyses revealed the absence of diabetes and right-sided basilar artery curvature as significant predictors for left sided hearing loss. There was no statistical difference between atherosclerotic cardiovascular risk score (high versus low) and hearing outcomes at 3 months. In ISSNHL, the laterality of hearing loss was inversely associated with the direction of basilar artery curvature. Our results, therefore, indicate the importance of vascular assessment when evaluating ISSNHL. Copyright © 2015 Elsevier B.V. All rights reserved.

  6. Buying a Hearing Aid

    Science.gov (United States)

    ... other medical information about your ears and health. Conductive Hearing Loss: A hearing loss is conductive when there is ... be available for these and other forms of conductive hearing loss. Sensorineural Hearing Loss: A hearing loss is sensorineural ...

  7. Longitudinal development of phonology and morphology in children with late-identified mild-moderate sensorineural hearing loss.

    Science.gov (United States)

    Moeller, Mary Pat; McCleary, Elizabeth; Putman, Coille; Tyler-Krings, Amy; Hoover, Brenda; Stelmachowicz, Patricia

    2010-10-01

    Studies of language development in children with mild-moderate hearing loss are relatively rare. Longitudinal studies of children with late-identified hearing loss are relevant for determining how a period of unaided mild-moderate hearing loss impacts development. In recent years, newborn hearing screening programs have effectively reduced the ages of identification for most children with permanent hearing loss. However, some children continue to be identified late, and research is needed to guide management decisions. Furthermore, studies of this group may help to discern whether language normalizes after intervention and/or whether certain aspects of language might be vulnerable to persistent delays. The current study examines the impact of late identification and reduced audibility on speech and language outcomes via a longitudinal study of four children with mild-moderate sensorineural hearing loss. Longitudinal outcomes of four children with late-identified mild-moderate sensorineural hearing loss were studied using standardized measures and language sampling procedures from at or near the point of identification (28 to 41 mos) through 84 mos of age. The children with hearing loss were compared with 10 age-matched children with normal hearing on a majority of the measures through 60 mos of age. Spontaneous language samples were collected from mother-child interaction sessions recorded at consistent intervals in a laboratory-based play setting. Transcripts were analyzed using computer-based procedures (Systematic Analysis of Language Transcripts) and the Index of Productive Syntax. Possible influences of audibility were explored by examining the onset and productive use of a set of verb tense markers and by monitoring the children's accuracy in the use of morphological endings. Phonological samples at baseline were transcribed and analyzed using Computerized Profiling. At entry to the study, the four children with hearing loss demonstrated language delays with

  8. Intratympanic steroid injection as a first-line therapy in uremia patients with sudden sensorineural hearing loss.

    Science.gov (United States)

    Tsai, Hsun-Tien; Hsueh, Naiwei; Huang, Chih-Ming; Lin, Hung-Ching

    2015-08-01

    ITSI as a first-line therapy in uremia patients with SSNHL offers a valid and safe treatment compared with intravenous systemic steroid treatment. A specific pathophysiology caused by possible sodium pump paralysis may be explained for uremia patients with SSNHL. To compare the efficacy of intratympanic steroid injection (ITSI) with that of systemic intravenous steroids as a first-line therapy in uremia patients with sudden sensorineural hearing loss (SSNHL). A total of 23 consecutive uremia patients with SSNHL were enrolled in this study. Patients were divided into two groups: the ITSI group (n = 15) and the non-ITSI group (n = 8), in which patients received intravenous systemic steroid treatment. The two groups were homogeneous in all respects. The hearing improvement and relative gain were statistically significant between the two groups. The value of hearing gain (ΔPTA = PTA pre - PTA post) in the ITSI group and the non-ITSI group was 24.6 ± 16.4dB and 8.4 ± 19.3dB. The value of relative gain (ΔPTA/PTApre) in the ITIS group and the non-ITSI group was 31.1 ± 22% and 9.4 ± 20.5%. In the ITSI group, 11 patients (73.3%) exhibited hearing recovery (ΔPTA > 10 dB).

  9. Synaptopathy in the noise-exposed and aging cochlea: Primary neural degeneration in acquired sensorineural hearing loss.

    Science.gov (United States)

    Kujawa, Sharon G; Liberman, M Charles

    2015-12-01

    The classic view of sensorineural hearing loss (SNHL) is that the "primary" targets are hair cells, and that cochlear-nerve loss is "secondary" to hair cell degeneration. Our recent work in mouse and guinea pig has challenged that view. In noise-induced hearing loss, exposures causing only reversible threshold shifts (and no hair cell loss) nevertheless cause permanent loss of >50% of cochlear-nerve/hair-cell synapses. Similarly, in age-related hearing loss, degeneration of cochlear synapses precedes both hair cell loss and threshold elevation. This primary neural degeneration has remained hidden for three reasons: 1) the spiral ganglion cells, the cochlear neural elements commonly assessed in studies of SNHL, survive for years despite loss of synaptic connection with hair cells, 2) the synaptic terminals of cochlear nerve fibers are unmyelinated and difficult to see in the light microscope, and 3) the degeneration is selective for cochlear-nerve fibers with high thresholds. Although not required for threshold detection in quiet (e.g. threshold audiometry or auditory brainstem response threshold), these high-threshold fibers are critical for hearing in noisy environments. Our research suggests that 1) primary neural degeneration is an important contributor to the perceptual handicap in SNHL, and 2) in cases where the hair cells survive, neurotrophin therapies can elicit neurite outgrowth from spiral ganglion neurons and re-establishment of their peripheral synapses. This article is part of a Special Issue entitled . Copyright © 2015 Elsevier B.V. All rights reserved.

  10. Auditory Brainstem Responses to Bone-Conducted Brief Tones in Young Children with Conductive or Sensorineural Hearing Loss

    Directory of Open Access Journals (Sweden)

    Jennifer L. Hatton

    2012-01-01

    Full Text Available The bone-conduction (BC tone ABR has been used clinically for over 20 years. The current study formally evaluated the test performance of the BC tone-evoked ABR in infants with hearing loss. Method. By comparing BC-ABR results to follow-up behavioural results, this study addressed two questions: (i whether the BC tone ABR was successful in differentiating children with conductive versus sensorineural hearing loss (Study A; conductive: 68 ears; SNHL: 129 ears and (ii the relationship between BC ABR and behavioural hearing loss severity (Study B: 2000 Hz: 104 ears; 500 Hz: 47 ears. Results. Results demonstrate that the “normal” BC-ABR levels accurately differentiated normal versus elevated cochlear sensitivity (accuracy: 98% for 2000 Hz; 98% for 500 Hz. A subset of infants in Study A with elevated BC-ABR (i.e., no response at normal level had additional testing at higher intensities, which allowed for categorization of the degree of cochlear impairment. Study B results indicate that the BC ABR accurately categorizes the degree of cochlear hearing loss for 2000 Hz (accuracy = 95.2%. A preliminary dBnHL-to-dBHL correction factor of “0 dB” was determined for 2000 Hz BC ABR. Conclusions. These findings further support the use of BC tone ABR for diagnostic ABR testing.

  11. Auditory Brainstem Responses to Bone-Conducted Brief Tones in Young Children with Conductive or Sensorineural Hearing Loss

    Science.gov (United States)

    Hatton, Jennifer L.; Janssen, Renée M.; Stapells, David R.

    2012-01-01

    The bone-conduction (BC) tone ABR has been used clinically for over 20 years. The current study formally evaluated the test performance of the BC tone-evoked ABR in infants with hearing loss. Method. By comparing BC-ABR results to follow-up behavioural results, this study addressed two questions: (i) whether the BC tone ABR was successful in differentiating children with conductive versus sensorineural hearing loss (Study A; conductive: 68 ears; SNHL: 129 ears) and (ii) the relationship between BC ABR and behavioural hearing loss severity (Study B: 2000 Hz: 104 ears; 500 Hz: 47 ears). Results. Results demonstrate that the “normal” BC-ABR levels accurately differentiated normal versus elevated cochlear sensitivity (accuracy: 98% for 2000 Hz; 98% for 500 Hz). A subset of infants in Study A with elevated BC-ABR (i.e., no response at normal level) had additional testing at higher intensities, which allowed for categorization of the degree of cochlear impairment. Study B results indicate that the BC ABR accurately categorizes the degree of cochlear hearing loss for 2000 Hz (accuracy = 95.2%). A preliminary dBnHL-to-dBHL correction factor of “0 dB” was determined for 2000 Hz BC ABR. Conclusions. These findings further support the use of BC tone ABR for diagnostic ABR testing. PMID:22988461

  12. The presence of a widened vestibular aqueduct and progressive sensorineural hearing loss in the branchio-oto-renal syndrome. A family study.

    NARCIS (Netherlands)

    Stinckens, C.I.C.; Standaert, L.; Casselman, J.W.; Huygen, P.L.M.; Kumar, S.; Wallen, J. van de; Cremers, C.W.R.J.

    2001-01-01

    OBJECTIVE: A new large family with the BOR syndrome is reported with special reference to the presence of a widened vestibular aqueduct and a progressive sensorineural component in the mixed hearing loss. A review of the BOR literature of 184 patients is given. Setting: University Hospitals.

  13. Blood-Labyrinth Barrier Permeability in Menière Disease and Idiopathic Sudden Sensorineural Hearing Loss: Findings on Delayed Postcontrast 3D-FLAIR MRI.

    Science.gov (United States)

    Pakdaman, M N; Ishiyama, G; Ishiyama, A; Peng, K A; Kim, H J; Pope, W B; Sepahdari, A R

    2016-06-02

    Menière disease and idiopathic sudden sensorineural hearing loss can have overlapping clinical presentation and may have similar pathophysiology. Prior studies using postcontrast 3D-FLAIR MR imaging suggest abnormal blood-labyrinth barrier permeability in both conditions, but the 2 diseases have not been directly compared by using the same imaging techniques. We hypothesized that delayed postcontrast 3D-FLAIR MR imaging would show differences in blood-labyrinth barrier permeability between Menière disease and idiopathic sudden sensorineural hearing loss. Patients with unilateral Menière disease (n = 32) and unilateral idiopathic sudden sensorineural hearing loss (n = 11) imaged with delayed postcontrast 3D-FLAIR MR imaging were retrospectively studied. Signal intensities of the medulla and perilymph of the cochlear basal turns of both ears in each patient were measured in a blinded fashion. Cochlea/medulla ratios were calculated for each ear as a surrogate for blood-labyrinth barrier permeability. The ears were segregated by clinical diagnosis. Cochlea/medulla ratio was higher in symptomatic ears of patients with Menière disease (12.6 ± 7.4) than in patients with idiopathic sudden sensorineural hearing loss (5.7 ± 2.0) and asymptomatic ears of patients with Menière disease (8.0 ± 3.1), indicating increased blood-labyrinth barrier permeability in Menière disease ears. The differences in cochlea/medulla ratio between symptomatic and asymptomatic ears were significantly higher in Menière disease than in idiopathic sudden sensorineural hearing loss. Asymptomatic ears in patients with Menière disease showed higher cochlea/medulla ratio than symptomatic and asymptomatic ears in patients with idiopathic sudden sensorineural hearing loss. Increased cochlea/medulla ratio indicates increased blood-labyrinth barrier permeability in Menière disease compared with idiopathic sudden sensorineural hearing loss. Increased cochlea/medulla ratio in asymptomatic ears of

  14. Management of Children with Mild, Moderate, and Moderately Severe Sensorineural Hearing Loss.

    Science.gov (United States)

    Tharpe, Anne Marie; Gustafson, Samantha

    2015-12-01

    Any degree of hearing loss can have a negative impact on child development. The amount of impact is largely determined by the type, quality, and timeliness of intervention. Early identification and management of hearing loss is essential for minimizing the impact of hearing loss and ensuring that children can reach their cognitive, linguistic, educational, and social potential. Advances in hearing technology and broadening of candidacy for same, have resulted in improved outcomes for many children with hearing loss. Through ongoing hearing monitoring throughout childhood, children with congenital, late-onset, or progressive losses can receive timely management from interprofessional, collaborative teams. Copyright © 2015 Elsevier Inc. All rights reserved.

  15. Study of the posterior communicating arteries of the circle of willis in idiopathic sudden sensorineural hearing loss.

    Science.gov (United States)

    Van Prooyen-Keyzer, Sandrine; Sadik, Jean-Claude; Ulanovski, David; Parmantier, Monique; Ayache, Denis

    2005-05-01

    To evaluate the incidence of non-functioning posterior communicating arteries (PCoA) of the circle of Willis in patients presenting with idiopathic sudden sensorineural hearing loss (SSHL). Pathogenesis of SSHL remains unclear despite several theories. A strong association between non-functioning PCoA of the circle of Willis and SSHL has been recently suggested, supporting the theory of vascular disorders. Tertiary referral center. Ultrasonographic Doppler flow measurements of the vertebrobasilar system were performed prospectively in 27 consecutive patients with idiopathic SSHL and in 27 healthy subjects. A non-functioning PCoA was found in 4 patients with idiopathic SSHL and in 4 cases in the control group. The results of our study did not show any association between non-functioning PCoA of the circle of Willis and idiopathic SSHL, contrasting with previous data. We do not recommend adding routine transcranial Doppler ultrasonography in the test-battery for SSHL.

  16. Direct Reprogramming of Spiral Ganglion Non-neuronal Cells into Neurons: Toward Ameliorating Sensorineural Hearing Loss by Gene Therapy

    Directory of Open Access Journals (Sweden)

    Teppei Noda

    2018-02-01

    Full Text Available Primary auditory neurons (PANs play a critical role in hearing by transmitting sound information from the inner ear to the brain. Their progressive degeneration is associated with excessive noise, disease and aging. The loss of PANs leads to permanent hearing impairment since they are incapable of regenerating. Spiral ganglion non-neuronal cells (SGNNCs, comprised mainly of glia, are resident within the modiolus and continue to survive after PAN loss. These attributes make SGNNCs an excellent target for replacing damaged PANs through cellular reprogramming. We used the neurogenic pioneer transcription factor Ascl1 and the auditory neuron differentiation factor NeuroD1 to reprogram SGNNCs into induced neurons (iNs. The overexpression of both Ascl1 and NeuroD1 in vitro generated iNs at high efficiency. Transcriptome analyses revealed that iNs displayed a transcriptome profile resembling that of endogenous PANs, including expression of several key markers of neuronal identity: Tubb3, Map2, Prph, Snap25, and Prox1. Pathway analyses indicated that essential pathways in neuronal growth and maturation were activated in cells upon neuronal induction. Furthermore, iNs extended projections toward cochlear hair cells and cochlear nucleus neurons when cultured with each respective tissue. Taken together, our study demonstrates that PAN-like neurons can be generated from endogenous SGNNCs. This work suggests that gene therapy can be a viable strategy to treat sensorineural hearing loss caused by degeneration of PANs.

  17. Direct Reprogramming of Spiral Ganglion Non-neuronal Cells into Neurons: Toward Ameliorating Sensorineural Hearing Loss by Gene Therapy.

    Science.gov (United States)

    Noda, Teppei; Meas, Steven J; Nogami, Jumpei; Amemiya, Yutaka; Uchi, Ryutaro; Ohkawa, Yasuyuki; Nishimura, Koji; Dabdoub, Alain

    2018-01-01

    Primary auditory neurons (PANs) play a critical role in hearing by transmitting sound information from the inner ear to the brain. Their progressive degeneration is associated with excessive noise, disease and aging. The loss of PANs leads to permanent hearing impairment since they are incapable of regenerating. Spiral ganglion non-neuronal cells (SGNNCs), comprised mainly of glia, are resident within the modiolus and continue to survive after PAN loss. These attributes make SGNNCs an excellent target for replacing damaged PANs through cellular reprogramming. We used the neurogenic pioneer transcription factor Ascl1 and the auditory neuron differentiation factor NeuroD1 to reprogram SGNNCs into induced neurons (iNs). The overexpression of both Ascl1 and NeuroD1 in vitro generated iNs at high efficiency. Transcriptome analyses revealed that iNs displayed a transcriptome profile resembling that of endogenous PANs, including expression of several key markers of neuronal identity: Tubb3, Map2, Prph, Snap25, and Prox1. Pathway analyses indicated that essential pathways in neuronal growth and maturation were activated in cells upon neuronal induction. Furthermore, iNs extended projections toward cochlear hair cells and cochlear nucleus neurons when cultured with each respective tissue. Taken together, our study demonstrates that PAN-like neurons can be generated from endogenous SGNNCs. This work suggests that gene therapy can be a viable strategy to treat sensorineural hearing loss caused by degeneration of PANs.

  18. WFS1 variants in Finnish patients with diabetes mellitus, sensorineural hearing impairment or optic atrophy, and in suicide victims.

    Science.gov (United States)

    Kytövuori, Laura; Seppänen, Allan; Martikainen, Mika H; Moilanen, Jukka S; Kamppari, Seija; Särkioja, Terttu; Remes, Anne M; Räsänen, Pirkko; Rönnemaa, Tapani; Majamaa, Kari

    2013-08-01

    Mutations in the wolframin gene, WFS1, cause Wolfram syndrome, a rare recessive neurodegenerative disorder. The clinical features include early-onset bilateral optic atrophy (OA), diabetes mellitus (DM), diabetes insipidus, hearing impairment, urinary tract abnormalities and psychiatric illness, and, furthermore, WFS1 variants appear to be associated with non-syndromic DM and hearing impairment. Variation of WFS1 was investigated in Finnish subjects consisting 182 patients with DM, 117 patients with sensorineural hearing impairment (SNHI) and 44 patients with OA, and in 95 suicide victims. Twenty-two variants were found in the coding region of WFS1, including three novel nonsynonymous variants. The frequency of the p.[His456] allele was significantly higher in the patients with SNHI (11.5%; corrected P=0.00008), DM (6.6%; corrected P=0.036) or OA (9.1%; corrected P=0.043) than that in the 285 controls (3.3%). The frequency of the p.[His611] allele was 55.8% in the patients with DM being higher than that in the controls (47%; corrected P=0.039). The frequencies of p.[His456] and p.[His611] were similarly increased in an independent group of patients with DM (N=299). The results support previous findings that genetic variation of WFS1 contributes to the risk of DM and SNHI.

  19. Altered Brain Functional Activity in Infants with Congenital Bilateral Severe Sensorineural Hearing Loss: A Resting-State Functional MRI Study under Sedation

    Directory of Open Access Journals (Sweden)

    Shuang Xia

    2017-01-01

    Full Text Available Early hearing deprivation could affect the development of auditory, language, and vision ability. Insufficient or no stimulation of the auditory cortex during the sensitive periods of plasticity could affect the function of hearing, language, and vision development. Twenty-three infants with congenital severe sensorineural hearing loss (CSSHL and 17 age and sex matched normal hearing subjects were recruited. The amplitude of low frequency fluctuations (ALFF and regional homogeneity (ReHo of the auditory, language, and vision related brain areas were compared between deaf infants and normal subjects. Compared with normal hearing subjects, decreased ALFF and ReHo were observed in auditory and language-related cortex. Increased ALFF and ReHo were observed in vision related cortex, which suggest that hearing and language function were impaired and vision function was enhanced due to the loss of hearing. ALFF of left Brodmann area 45 (BA45 was negatively correlated with deaf duration in infants with CSSHL. ALFF of right BA39 was positively correlated with deaf duration in infants with CSSHL. In conclusion, ALFF and ReHo can reflect the abnormal brain function in language, auditory, and visual information processing in infants with CSSHL. This demonstrates that the development of auditory, language, and vision processing function has been affected by congenital severe sensorineural hearing loss before 4 years of age.

  20. Altered Brain Functional Activity in Infants with Congenital Bilateral Severe Sensorineural Hearing Loss: A Resting-State Functional MRI Study under Sedation

    Science.gov (United States)

    Che, Jing; Li, Qiang; Chai, Chao; Zheng, Meizhu; Shen, Wen

    2017-01-01

    Early hearing deprivation could affect the development of auditory, language, and vision ability. Insufficient or no stimulation of the auditory cortex during the sensitive periods of plasticity could affect the function of hearing, language, and vision development. Twenty-three infants with congenital severe sensorineural hearing loss (CSSHL) and 17 age and sex matched normal hearing subjects were recruited. The amplitude of low frequency fluctuations (ALFF) and regional homogeneity (ReHo) of the auditory, language, and vision related brain areas were compared between deaf infants and normal subjects. Compared with normal hearing subjects, decreased ALFF and ReHo were observed in auditory and language-related cortex. Increased ALFF and ReHo were observed in vision related cortex, which suggest that hearing and language function were impaired and vision function was enhanced due to the loss of hearing. ALFF of left Brodmann area 45 (BA45) was negatively correlated with deaf duration in infants with CSSHL. ALFF of right BA39 was positively correlated with deaf duration in infants with CSSHL. In conclusion, ALFF and ReHo can reflect the abnormal brain function in language, auditory, and visual information processing in infants with CSSHL. This demonstrates that the development of auditory, language, and vision processing function has been affected by congenital severe sensorineural hearing loss before 4 years of age. PMID:28255465

  1. Effects of Various Extents of High-Frequency Hearing Loss on Speech Recognition and Gap Detection at Low Frequencies in Patients with Sensorineural Hearing Loss

    Directory of Open Access Journals (Sweden)

    Bei Li

    2017-01-01

    Full Text Available This study explored whether the time-compressed speech perception varied with the degree of hearing loss in high-frequency sensorineural hearing loss (HF SNHL individuals. 65 HF SNHL individuals with different cutoff frequencies were recruited and further divided into mildly, moderately, and/or severely affected subgroups in terms of the averaged thresholds of all frequencies exhibiting hearing loss. Time-compressed speech recognition scores under both quiet and noisy conditions and gap detection thresholds within low frequencies that had normal thresholds were obtained from all patients and compared with data from 11 age-matched individuals with normal hearing threshold at all frequencies. Correlations of the time-compressed speech recognition scores with the extents of HF SNHL and with the 1 kHz gap detection thresholds were studied across all participants. We found that the time-compressed speech recognition scores were significantly affected by and correlated with the extents of HF SNHL. The time-compressed speech recognition scores also correlated with the 1 kHz gap detection thresholds except when the compression ratio of speech was 0.8 under quiet condition. Above all, the extents of HF SNHL were significantly correlated with the 1 kHz gap thresholds.

  2. Association between metabolic syndrome and sensorineural hearing loss: a cross-sectional study of 11,114 participants

    Directory of Open Access Journals (Sweden)

    Aghazadeh-Attari J

    2017-11-01

    Full Text Available Javad Aghazadeh-Attari,1 Behnam Mansorian,2 Mohammad Mirza-Aghazadeh-Attari,3 Jamal Ahmadzadeh,2 Iraj Mohebbi2 1Social Determinants of Health Research Center, Department of Neurosurgery, 2Social Determinants of Health Research Center, Occupational Medicine Center, Urmia University of Medical Sciences, Urmia, 3Medical Philosophy and History Research Center, Tabriz University of Medical Sciences, Tabriz, Iran Background/objectives: Hearing loss (HL is associated with certain diseases and affects health, resulting in a low quality of life. Some components of the metabolic syndrome (MetS coincide with the risk factors for sensorineural hearing loss (SNHL. To date, very few studies have examined the link between MetS and HL. The aim of the current study was to try to understand the potential association between MetS and HL.Methods: Using Iranian health surveys of professional drivers, we enrolled 11,114 individuals aged 20–60 years, whose main job is to operate a motor vehicle. We examined participants for the presence and absence of SNHL and the components of the MetS. Additionally, we investigated the relationship between MetS and the pure tone air conduction hearing thresholds of participants with SNHL, including low-frequency and high-frequency thresholds.Results: This cross-sectional study consisted of 11,114 participants: 3202 (28.81% diagnosed with MetS and 7911 (71.18% without and 2772 (24.94% with SNHL and 8432 (75.86% without. Participants with SNHL had a higher number of components of MetS (P<0.001 for all components.Conclusion: Our results demonstrated that an association possibly exists between different components of MetS (obesity, hypertension, hypertriglyceridemia, high fasting glucose levels, and waist circumference and SNHL in a population of West Azerbaijan drivers. Therefore, it is important to schedule periodic checkups for drivers to detect and avoid the increase in MetS components at an early stage in this population

  3. Genotype-phenotype correlation for DFNA22: characterization of non-syndromic, autosomal dominant, progressive sensorineural hearing loss due to MYO6 mutations

    DEFF Research Database (Denmark)

    Topsakal, Vedat; Hilgert, Nele; van Dinther, Joost

    2010-01-01

    Clinical and audiological examination was done in 2 Belgian families with autosomal dominant sensorineural hearing loss (SNHL) linked to DFNA22. Nineteen subjects in family 1 had mild to moderate SNHL starting in the third decade. The hearing loss was characterized by a flat audiogram affecting all...... tested frequencies with statistically significant progression. In family 2 eleven subjects were affected with mild to moderate SNHL starting in the second decade. Most of them showed a flat audiogram, but some had mid-frequency hearing loss. Significant progression of thresholds was present at 4 and 8 k...

  4. Revisiting the relationship of three-dimensional fluid attenuation inversion recovery imaging and hearing outcomes in adults with idiopathic unilateral sudden sensorineural hearing loss

    Energy Technology Data Exchange (ETDEWEB)

    Liao, Wen-Huei [School of Medicine, National Yang Ming University, Taipei, 11221, Taiwan (China); Department of Otolaryngology, Taipei Veterans General Hospital, Taipei, 11217, Taiwan (China); Wu, Hsiu-Mei [School of Medicine, National Yang Ming University, Taipei, 11221, Taiwan (China); Department of Radiology, Taipei Veterans General Hospital, Taipei, 11217, Taiwan (China); Wu, Hung-Yi [Department of Radiology, Taipei Veterans General Hospital, Taipei, 11217, Taiwan (China); Tu, Tzong-Yang; Shiao, An-Suey [School of Medicine, National Yang Ming University, Taipei, 11221, Taiwan (China); Department of Otolaryngology, Taipei Veterans General Hospital, Taipei, 11217, Taiwan (China); Castillo, Mauricio [Department of Radiology, University of North Carolina, Chapel Hill, NC, 27599-7510 (United States); Hung, Sheng-Che, E-mail: hsz829@gmail.com [School of Medicine, National Yang Ming University, Taipei, 11221, Taiwan (China); Department of Radiology, Taipei Veterans General Hospital, Taipei, 11217, Taiwan (China); Department of Biomedical Imaging and Radiological Sciences, National Yang Ming University, Taipei, 11221, Taiwan (China)

    2016-12-15

    Background and purpose: Three-dimensional fluid attenuation inversion recovery (3D FLAIR) may demonstrate high signal in the inner ears of patients with idiopathic sudden sensorineural hearing loss (ISSNHL), but the correlations of this finding with outcomes are still controversial. Here we compared 4 3D MRI sequences with the outcomes of patients with ISSNHL. Materials and methods: 77 adult patients with ISSNHL underwent MRI with pre contrast FLAIR, fast imaging employing steady-state acquisition images (FIESTA-C), post contrast T1WI and post contrast FLAIR. The extent and degree of high signal in both cochleas were evaluated in all patients, and asymmetry ratios between the affected ears and the normal ones were calculated. The relationships among MRI findings, including extent and asymmetry of abnormal cochlear high signals, degree of FLAIR enhancement, and clinical information, including age, vestibular symptoms, baseline hearing loss, and final hearing outcomes were analyzed. Results: 54 patients (28 men; age, 52.1 ± 15.5 years) were included in our study. Asymmetric cochlear signal intensities were more frequently observed in pre contrast and post contrast FLAIR (79.6% and 68.5%) than in FIESTA-C (61.1%) and T1WI (51.9%) (p < 0.001). Age, baseline hearing loss, extent of high signal and asymmetry ratios of pre contrast and post contrast FLAIR were all correlated with final hearing outcomes. In multivariate analysis, age and the extent of high signals were the most significant predictors of final hearing outcomes. Conclusion: 3D FLAIR provides a higher sensitivity in detecting the asymmetric cochlear signal abnormality. The more asymmetric FLAIR signals and presence of high signals beyond cochlea indicated a poorer prognosis.

  5. Longitudinal Development of Phonology and Morphology in Children with Late-Identified Mild-Moderate Sensorineural Hearing Loss

    Science.gov (United States)

    Moeller, Mary Pat; McCleary, Elizabeth; Putman, Coille; Tyler-Krings, Amy; Hoover, Brenda; Stelmachowicz, Patricia

    2010-01-01

    Objective Studies of language development in children with mild-moderate hearing loss are relatively rare. Longitudinal studies of children with late-identified hearing loss have not been conducted, and they are relevant for determining how a period of unaided mild-moderate hearing loss impacts development. In recent years, newborn hearing screening programs have effectively reduced the ages of identification for most children with permanent hearing loss. However, some children continue to be identified late and research is needed to guide management decisions. Further, studies of this group may help to discern if language normalizes following intervention, and/or if certain aspects of language might be vulnerable to persistent delays. The current study examines the impact of late identification and reduced audibility on speech and language outcomes via a longitudinal study of four children with mild-moderate sensorineural hearing loss. Design Longitudinal outcomes of four children with late-identified mild-moderate sensorinueral hearing loss were studied using standardized measures and language sampling procedures, from at or near the point of identification (28 – 41 months) through 84 months of age. The children with hearing loss were compared to ten age-matched children with normal hearing on a majority of the measures through 60 months of age. Spontaneous language samples were collected from mother-child interaction sessions, recorded at consistent intervals in a laboratory-based play setting. Transcripts were analyzed using computer-based procedures (Systematic Analysis of Language Transcripts) and the Index of Productive Syntax. Possible influences of audibility were explored by examining the onset and productive use of a set of verb tense markers, and by monitoring the children’s accuracy in use of morphological endings. Phonological samples at baseline were transcribed and analyzed using Computerized Profiling. Results At entry to the study, the four

  6. Effect of hyperinsulinism on sensorineural hearing impairment in Ménière's disease: a cohort study.

    Science.gov (United States)

    Lavinsky, Joel; Wolff, Michelle Lavinsky; Trasel, Andrea Ruschel; Valerio, Marcel Machado; Lavinsky, Luiz

    2014-01-01

    To compare the degree of sensorineural hearing loss in patients with Ménière's disease (MD) with and without hyperinsulinism by different methods of assessment. Historical cohort study. Ménière's Disease Care and Research Clinics of Hospital de Clinicas de Porto Alegre, a tertiary care university hospital in Southern Brazil. Patients with a definite diagnosis of MD based on the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) guidelines. Patients were assessed by glucose overload tests (5-h glucose and insulin curves) and under baseline physiological conditions (Homeostasis Model Assessment/Insulin Resistance [HOMA-IR], Quantitative Insulin Sensitivity Check Index [QUICKI], and glucose/insulin ratio). These patients underwent annual pure-tone audiometry and were analyzed using 4-tone average (FTA), that is, arithmetic mean of 500, 1,000, 2,000, and 3,000 Hz, during the third, fourth, and fifth years of disease progression. Hearing loss assessed by FTA and classified in Stages I to IV (AAO-HNS). Forty-nine (76.6%) patients were defined as hyperinsulinemic and 15 (23.4%) as normoinsulinemic. Impairment on FTA was higher in the hyperinsulinemic group (52.04 ± 17.5 versus 39.75 ± 9.20, p = 0.027) when assessed by the 5-hour insulin curve. Hyperinsulinemic subjects were 3.5 times more likely to develop hearing damage greater than 40 dB (i.e., Stages III and IV) than normoinsulinemic subjects (OR = 3.52; 95% CI, 1.05-11.76). A moderate correlation between the insulin curve and the HOMA-IR was found (r = 0.524, p = 0.001). Hyperinsulinism in MD is associated with greater clinical hearing damage.

  7. The relationship between serum lipids and sudden sensorineural hearing loss: a systematic review and meta-analysis.

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    I Jen Chang

    Full Text Available Sudden sensorineural hearing loss (SSNHL is a relatively common condition that is usually of unknown etiology. A number of individual studies have investigated the association between various serum lipids and SSNHL; however, the findings have been inconsistent. In an attempt to obtain more definitive information on the relationship between serum lipids and SSNHL, we carried out a systematic review and meta-analysis.Medline, the Cochrane Library, and EMBASE were searched using the following key words: lipid, cholesterol, triglyceride, fat, serum, blood, sudden hearing loss, hearing loss, hearing disorders. Randomized controlled trials, prospective cohort studies, and retrospective case-control studies involving patients with SSNHL and healthy controls that examined the relationship (reported as odds ratios [OR] between lipid profiles and SSNHL were included. Primary outcomes were total cholesterol and low-density lipoprotein cholesterol (LDL-C concentrations. Secondary outcomes were triglyceride, high-density lipoprotein cholesterol, and lipoprotein(a concentrations.A total of 6 case-control studies were included in this systematic review/meta-analysis. The total number of participants ranged from 30 to 250 in the case group and from 43 to 271 in the control group. Meta-analysis revealed no significant difference in total cholesterol levels between the case and control groups (pooled OR = 1.79, 95% confidence interval [CI] = 0.98 to 3.26, P = 0.057. Likewise, meta-analysis revealed no significant difference in LDL-C concentrations between the case and control groups (pooled OR = 1.15, 95% CI = 0.64 to 2.07, P = 0.639. Since there were an insufficient number of studies reporting data for the secondary outcomes, meta-analysis was not possible.Our results do not provide evidence for serum lipids being associated with SSNHL, nor do they definitively rule out such an association. Additional studies are needed to ascertain the relationship, or lack

  8. The combination of vestibular impairment and congenital sensorineural hearing loss predisposes patients to ocular anomalies, including Usher syndrome.

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    Kletke, S; Batmanabane, V; Dai, T; Vincent, A; Li, S; Gordon, K A; Papsin, B C; Cushing, S L; Héon, E

    2017-07-01

    The co-occurrence of hearing impairment and visual dysfunction is devastating. Most deaf-blind etiologies are genetically determined, the commonest being Usher syndrome (USH). While studies of the congenitally deaf population reveal a variable degree of visual problems, there are no effective ophthalmic screening guidelines. We hypothesized that children with congenital sensorineural hearing loss (SNHL) and vestibular impairment were at an increased risk of having USH. A retrospective chart review of 33 cochlear implants recipients for severe to profound SNHL and measured vestibular dysfunction was performed to determine the ocular phenotype. All the cases had undergone ocular examination and electroretinogram (ERG). Patients with an abnormal ERG underwent genetic testing for USH. We found an underlying ocular abnormality in 81.81% (27/33) of cases; of which 75% had refractive errors, and 50% of those patients showed visual improvement with refractive correction. A total of 14 cases (42.42%; 14/33) had generalized rod-cone dysfunction on ERG suggestive of Usher syndrome type 1, confirmed by mutational analysis. This work shows that adding vestibular impairment as a criterion for requesting an eye exam and adding the ERG to detect USH increases the chances of detecting ocular anomalies, when compared with previous literature focusing only on congenital SNHL. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  9. Identification of a Novel TECTA mutation in a Chinese DFNA8/12 family with prelingual progressive sensorineural hearing impairment.

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    Zhengyue Li

    Full Text Available Tectorial membrane, an extracellular matrix of the cochlea, plays a crucial role in the transmission of sound to the sensory hair cells. Alpha-tectorin is the most important noncollagenous component of the tectorial membrane and the otolith membrane in the maculae of the vestibular system. Defects in TECTA, the gene encodes alpha-tectorin, are cause of both dominant (DFNA8/12 and recessive (DFNB21 forms of deafness. Here, we report a three-generation Chinese family characterized by prelingual progressive sensorineural hearing impairment. We mapped the disease locus to chromosome 11q23-24 region, overlapping with the DFNA8/12 locus. Sequencing of candidate gene TECTA revealed a heterozygous c.5945C>A substitution in exon 19, causing amino acid substitution of Ala to Asp at a conservative position 1982. The A1982D substitution is consistent with hearing loss in this Chinese family and has not been found in 200 random control chromosomes. To our knowledge, this is the first TECTA mutation identified in Chinese population. Our data provides additional molecular and clinical information for establishing a better genotype-phenotype understanding of DFNA8/12.

  10. [Constitute, imaging and auditory characteristics of pediatric patients with congenital malformations of inner ear in sensorineural hearing loss].

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    Li, Youjin; Yang, Jun; Li, Yun

    2011-01-01

    To analyze the constitute, imaging and auditory characteristics of pediatric patients with congenital malformations of inner ear in sensorineural hearing loss(SNHL). Imaging and auditory data of 125 cases (225 ears) in 860 pediatric patients with congenital SNHL who referred to ENT department of Shanghai Children Medical Center from February 2005 to January 2010 were retrospectively studied. Congenital malformations of inner ear accounted for 14.5% in 860 pediatric patients with congenital SNHL. Bilateral ear was involved in 98 cases (78.4%), unilateral in 27 cases (21.6%). One hundred and sixty-seven ears (74.2%) were identified as profound deafness, 36 ears (16%) severe deafness and 22 ears (9.8%) moderate deafness in 225 ears, respectively. In present group, large vestibular aqueduct (75.6%) was the most common and next was vestibular deformity (32%), then cochleovestibular deformity (23.1%). Mondini deformity (55.8%) was the most common and common cavity deformity (28.9%) next in cochleovestibular deformity. Profound deafness in cochlea involved congenital malformations of inner ear was significantly more than those in no cochlea involved malformations. The results are of importance for understanding the constitute of congenital malformations of inner ear in pediatric patients with congenital SNHL in China, for etiological diagnosis of congenital SNHL, for intervention including hearing aids or cochlear implant and prognosis.

  11. Intronic variants in the NFKB1 gene may influence hearing forecast in patients with unilateral sensorineural hearing loss in Meniere's disease.

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    Sonia Cabrera

    Full Text Available Meniere's disease is an episodic vestibular syndrome associated with sensorineural hearing loss (SNHL and tinnitus. Patients with MD have an elevated prevalence of several autoimmune diseases (rheumatoid arthritis, systemic lupus erythematosus, ankylosing spondylitis and psoriasis, which suggests a shared autoimmune background. Functional variants of several genes involved in the NF-κB pathway, such as REL, TNFAIP3, NFKB1 and TNIP1, have been associated with two or more immune-mediated diseases and allelic variations in the TLR10 gene may influence bilateral affectation and clinical course in MD. We have genotyped 716 cases of MD and 1628 controls by using the ImmunoChip, a high-density genotyping array containing 186 autoimmune loci, to explore the association of immune system related-loci with sporadic MD. Although no single nucleotide polymorphism (SNP reached a genome-wide significant association (p40 dB HL (log-rank test, corrected p values were p = 0.009 for rs3774937 and p = 0.003 for rs4648011, respectively. No variants influenced hearing in bilateral MD. Our data support that the allelic variants rs3774937 and rs4648011 can modify hearing outcome in patients with MD and unilateral SNHL.

  12. High prevalence of abnormalities on CT and MR imaging in children with unilateral sensorineural hearing loss irrespective of age or degree of hearing loss.

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    van Beeck Calkoen, E A; Sanchez Aliaga, E; Merkus, P; Smit, C F; van de Kamp, J M; Mulder, M F; Goverts, S T; Hensen, E F

    2017-06-01

    Evaluation of causal abnormalities identified on CT and MR imaging in children with unilateral sensorineural hearing loss (USNHL), and the association with age and severity of hearing loss. Retrospective cohort study. Tertiary referral otology/audiology center. 102 children diagnosed with USNHL between 2006 and 2016 were included. They underwent CT and/or MR imaging for the evaluation of the etiology of their hearing loss. Radiologic abnormalities of the inner ear and brain associated with USNHL. Using CT and/or MR imaging, causal abnormalities were identified in 49%, which is higher than previously reported (25-40%). The most frequently affected site was the labyrinth (29%), followed by the cochlear nerve (9%) and brain (7%). No significant difference in the number or type of abnormalities was found for the degree of hearing loss or age categories. Imaging is essential in the etiologic analysis of USNHL because of the high prevalence of causative abnormalities that can be identified with radiology, irrespective of the patients' age or degree of hearing loss. CT and MR imaging are complementary imaging options. The ideal imaging algorithm is controversial. Based on our findings, we conclude that there is limited additional diagnostic value of simultaneous dual modality imaging over sequential diagnostics. We therefore perform a stepwise radiological workup in order to maximize the diagnostic yield while minimizing impact and costs. If the primary imaging modality does not identify a cause for USNHL, performing the alternative imaging modality should be considered. Retrospective cohort study 2b. Copyright © 2017 Elsevier B.V. All rights reserved.

  13. Hearing loss

    Science.gov (United States)

    Decreased hearing; Deafness; Loss of hearing; Conductive hearing loss; Sensorineural hearing loss; Presbycusis ... Conductive hearing loss (CHL) occurs because of a mechanical problem in the outer or middle ear. This may be ...

  14. Congenital abnormality of the inner ear and internal auditory canal in a patient with deep sensorineural hearing loss: A case report

    International Nuclear Information System (INIS)

    Rivera, Diego; Saab, Said; Cordoba, Claudia; Montes, Guillermo; Barreto, Tatiana

    2010-01-01

    CT and MRI are complementary studies that have proven to be the best radiological tools in screening of children with unilateral or bilateral sensorineural hearing loss. Only about a 20% of the patients with congenital sensor neural hearing loss have manifestations in images. Due to the fact that most of these manifestations initiate in the bone, the CT is the first line of image study. MRI is indicated in the evaluation of suspected agenesis, neuropathy, aplasia or hypoplasia of the vestibulocochlear nerve, often associated with this type of hearing loss. We present a case of a 6 year old patient with deep bilateral sensor neural hearing loss, with radiological studies to determine a potential candidate for a cochlear or auditory brainstem implant as hearing rehabilitation.

  15. Newborn Dried Blood Spot Polymerase Chain Reaction to Identify Infants with Congenital Cytomegalovirus-Associated Sensorineural Hearing Loss.

    Science.gov (United States)

    Ross, Shannon A; Ahmed, Amina; Palmer, April L; Michaels, Marian G; Sánchez, Pablo J; Stewart, Audra; Bernstein, David I; Feja, Kristina; Fowler, Karen B; Boppana, Suresh B

    2017-05-01

    To determine the utility of dried blood spot (DBS) polymerase chain reaction (PCR) in identifying infants with cytomegalovirus (CMV) infection-associated sensorineural hearing loss (SNHL). Newborns at 7 US hospitals between March 2007 and March 2012 were screened for CMV by saliva rapid culture and/or PCR. Infected infants were monitored for SNHL during the first 4 years of life to determine sensitivity, specificity, and positive and negative likelihood ratios of DBS PCR for identifying CMV-associated SNHL. DBS at birth was positive in 11 of 26 children (42%) with SNHL at age 4 years and in 72 of 270 children (27%) with normal hearing (P = .11). The sensitivity (42.3%; 95% CI, 23.4%-63.1%) and specificity (73.3%; 95% CI, 67.6%-78.5%) was low for DBS PCR in identifying children with SNHL at age 4 years. The positive and negative likelihood ratios of DBS PCR positivity to detect CMV-associated SNHL at age 4 years were 1.6 (95% CI, 0.97-2.6) and 0.8 (95% CI, 0.6-1.1), respectively. There was no difference in DBS viral loads between children with SNHL and those without SNHL. DBS PCR for CMV has low sensitivity and specificity for identifying infants with CMV-associated hearing loss. These findings, together with previous reports, demonstrate that DBS PCR does not identify either the majority of CMV-infected newborns or those with CMV-associated SNHL early in life. Copyright © 2017 Elsevier Inc. All rights reserved.

  16. Sensitivity and specificity of vestibular bed-side examination in detecting VIII cranial nerve schwannoma with sensorineural sudden unilateral hearing loss as presenting symptom.

    Science.gov (United States)

    Califano, L; Salafia, F; Melillo, M G; Mazzone, S

    2017-08-01

    The objectives of this study were to identify signs of vestibular nerve suffering through a bedside vestibular examination protocol in case of sudden sensorineural unilateral hearing loss without spontaneous signs of vestibular impairment and to propose a bed-side vestibular examination based protocol for the focused execution of gadolinium-enhanced magnetic resonance imaging (MRI) only if a vestibular schwannoma is suspected. 96 patients, 52 men, 44 women, mean age 57.73 +/- 12.85 years, suffering from sudden sensorineural unilateral hearing loss, which presented neither vertigo nor spontaneous nystagmus, were enrolled. Pure tone audiometry, tympanometry, measurement of acoustic reflexes and Anderson test to detect adaptation, bedside vestibular examination through head shaking test, vibration test, head impulse test, hyperventilation test and detection of nystagmus in supine and lateral decubitus to search for signs of vestibular impairment were performed. Patients with signs of vestibular impairment and pure tone audiometry threshold at high frequencies better than 70 dB nHL were subjected to auditory brainstem responses. Gadolinium enhanced MRI centred on internal acoustic canals was carried out in all patients with sudden sensorineural unilateral hearing loss. Main outcome measures were signs of vestibular impairment at vestibular bedside examination and presence of vestibular schwannoma on MRI. Signs of vestibular impairment were detected in 22/96 cases (22.9%); a vestibular schwannoma was detected by MRI in 5/96 cases (5.2%), always when vestibular impairment was present. In case of sudden sensorineural unilateral hearing loss, vestibular bedside examination seems to be useful to restrict the suspicion of a vestibular schwannoma to cases with signs of vestibular impairment, reducing the number of MRI exams, with considerable economic savings. © Copyright by Società Italiana di Otorinolaringologia e Chirurgia Cervico-Facciale, Rome, Italy.

  17. [Research progress of mutational spectrum and pathophysiology of WFS1 gene in Wolfram syndrome and nonsyndromic low frequency sensorineural hearing loss].

    Science.gov (United States)

    Shi, S M; Han, Y H; Wang, H B

    2016-09-07

    Compound homozygous or heterozygous mutations in WFS 1 can lead to autosomal recessive Wolfram syndrome (WS), and heterozygous mutations in WFS 1 can lead to autosomal dominant non-syndromic low frequency sensorineural hearing loss (LFSNHL). In addition, mutations in the WFS region has relationship with diabetes and psychiatric diseases. In this paper, we provide an overview of genetic research with different phenotypes, including WS and LFSNHL.

  18. Comparison of intermittent intratympanic steroid injection and near-continual transtympanic steroid perfusion as salvage treatments for sudden sensorineural hearing loss.

    Science.gov (United States)

    Chou, Yi-Fan; Chen, Peir-Rong; Kuo, Ian-Jiun; Yu, Szu-Hui; Wen, Yu-Hsuan; Wu, Hung-Pin

    2013-09-01

    The purpose of this study was to investigate whether near-continual transtympanic steroid perfusion is more effective than intermittent intratympanic steroid injection as a salvage therapy for idiopathic sudden sensorineural hearing loss. Case control study. We designed a case-control study consisting of 60 patients with sudden sensorineural hearing loss who did not respond well to systemic steroid therapy. From November 2008 to October 2010, we prospectively enrolled subjects for the transtympanic steroid perfusion therapy. We retrospectively collected data from age- and sex-matched patients who had undergone intratympanic steroid injection between January 2003 and October 2008. The audiological results of the two groups were compared. The presalvage pure tone threshold was 65.4 ± 13.5 dB in the transtympanic steroid perfusion group. After the therapy, the hearing threshold was improved by an average of 15.0 ± 9.7 dB, and 53.3% of subjects had improved by 10 dB or more. The speech discrimination score was improved from 12.6% ± 7.0% to 54.4 ± 6.4%. In the intratympanic steroid injection group, the presalvage pure tone threshold was 68.8 ± 16.0 dB. After the therapy, the hearing threshold was improved by an average of 10.7 ± 9.8 dB, and 43.3% of subjects had improved by 10 dB or more. The speech discrimination score was improved from 13.3 ± 6.0% to 46.4 ± 12%. The degree of hearing improvement was significantly greater in the transtympanic group. Both transtympanic steroid perfusion and intratympanic steroid injection can be used as salvage therapies for idiopathic sudden sensorineural hearing loss. Near-continual transtympanic steroid perfusions may provide better audiological results. Copyright © 2013 The American Laryngological, Rhinological and Otological Society, Inc.

  19. Otoacoustic emissions and auditory brainstem responses in patients with sudden sensorineural hearing loss. Do otoacoustic emissions have prognostic value?

    Directory of Open Access Journals (Sweden)

    Manoochehr Amiridavan

    2006-11-01

    Full Text Available BACKGROUND: Sudden sensorineural hearing loss (SSNHL is a perplexing condition for patients and there are many controversies about its etiology, audiologic characteristics, prognostic factors, and treatment. METHODS: In this prospective study, we performed some audiologic tests, including PTA, IA, ABR, and OAE (TEOAE before beginning treatment of 53 patients with SSNHL. We assigned the patients randomly to two treatment groups: oral steroids + acyclovir vs. intravenous urographin. Twenty-eight patients underwent Magnetic Resonance Imaging (MRI of the Brain. RESULTS: Of 53 patients (22 female and 31 male, 22 (41.5% had negative or no signal to noise ratio and overall correlation in TEOAE. Twenty-six patients (49% had positive overall correlations less than 50%, and 5 patients (4.4% had overall correlations >50%. Fifteen patients (28. 3% responded completely or well, 20 (37.7% responded partially, and 18 (33.9% had poor or no response to the treatment. The mean values for overall correlation in 3 subgroups of patients (no response, partial response, and complete response were – 3. 5% (+ 1/16%, +11% (+ 1/99%, and +36.6% (+3/07%, respectively (P = 0.01. Twenty out of 52 patients had no reproducible wave in ABR (38.5%, and waves I, III, and V were absent in 40 (77%, 31 (59.6% and 21 (40% patients, respectively. There were some limitations (false positive and false negative results in ABR use in our cases, but it may be useful in detecting site of lesion in SSNHL. Overall, according to the results of OAE, ABR, and brain MRI of these patients, 3 were affected by acoustic neurinomas, at least 1 had auditory neuropathy, and the site of lesion was cochlear in 6, and cochlear + retrocochlear in 13 patients. CONCLUSIONS: ABR has limitations for use in SSNHL and seems not to obviate the need for brain MRI, but may help in determining the site of lesions such as ischemia or neuropathy. Overall correlation (and S/N ratio in TEOAE is a valuable

  20. Long-term benefit perception, complications, and device malfunction rate of bone-anchored hearing aid implantation for profound unilateral sensorineural hearing loss.

    Science.gov (United States)

    Gluth, Michael B; Eager, Katrise M; Eikelboom, Robert H; Atlas, Marcus D

    2010-12-01

    To longitudinally evaluate short- and long-term subject satisfaction/benefit perception, device usage rates, complication rates, and external device repair rates of bone-anchored hearing aid (BAHA) implantation on a cohort of adult subjects with profound unilateral sensorineural hearing loss (PUSHL). Prospective clinical trial. Tertiary referral center. Fifty-six adults with PUSHL, 21 of which underwent BAHA implantation (followed for an average of 3.2 years after implantation; range, 0.8-4.6 yr). Short- and long-term satisfaction/benefit perception outcomes consisting of the Glasgow Hearing Aid Benefit Profile, Abbreviated Profile of Hearing Aid Benefit, and Single-Sided Deafness Questionnaire, including a comparison of results between implanted and nonimplanted subjects. Short- and long-term device usage rates, complications, and device failure issues also were carefully documented. There were statistically significant improvements in nearly all measures of benefit perception documented as well as a high rate of long-term device usage (81%). Although satisfaction and benefit perception outcomes generally tended to regress over time when compared with initial short-term outcomes, long-term scores still tended to be significantly improved nevertheless as compared with preoperative levels. Approximately 38% of implants experienced severe local skin reactions (Grade 2 and above) around the implant site at some point throughout the follow-up period, whereas only one (4.8%) required implant removal. 66.7% of subjects required repair of their external sound processor. BAHA implantation seems to provide a high level of short- and long-term perceived benefit and satisfaction in subjects with PUSHL and high rate of long-term device usage. Implant site adverse local skin reactions and repairs of the external sound processor were quite common.

  1. Vestibular (dys)function in children with sensorineural hearing loss: a systematic review.

    Science.gov (United States)

    Verbecque, Evi; Marijnissen, Tessa; De Belder, Niels; Van Rompaey, Vincent; Boudewyns, An; Van de Heyning, Paul; Vereeck, Luc; Hallemans, Ann

    2017-06-01

    The objective of this study is to provide an overview of the prevalence of vestibular dysfunction in children with SNHL classified according to the applied test and its corresponding sensitivity and specificity. Data were gathered using a systematic search query including reference screening. Pubmed, Web of Science and Embase were searched. Strategy and reporting of this review was based on the Meta-analysis of Observational Studies in Epidemiology (MOOSE) guidelines. Methodological quality was assessed with the COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) checklist. All studies, regardless the applied vestibular test, showed that vestibular function differs significantly between children with hearing loss and normal hearing (p 30).

  2. [Multi-center study on the treatment for intermediate and high-frequency sudden sensorineural hearing loss].

    Science.gov (United States)

    Wang, Ming-ming; Fan, Zhao-min; Luo, Jian-fen; Hou, Zhi-qiang; Ai, Yu; Wang, Hai-bo; Xu, Min; Zhu, Kang; Hou, Jin; Li, Wen-yan

    2013-05-01

    To analyze the therapeutic effect of treatment for intermediate and high-frequency sudden sensorineural hearing loss (SSNHL). A prospective clinical multicentre research was conducted using international standardized approach of clinical research. SSNHL Cases with intermediate and high-frequency hearing loss, that accepted no medication from onset of hearing loss within two weeks duration and ages ranged between 18 and 65, were collected. All patients were treated by one of four treatments plans chosen by unified random table. 141 patients with intermediate and high-frequency SSNHL were recruited in the research. Twenty subjects were treated with lidocaine, 21 cases with lidocaine and hormone, 40 cases with Ginaton, and 60 cases with Ginaton and hormone. 42 out of 141 (29.79%) patients were total recovery, 24 (17.02%)achieved excellent recovery, 27 (19.15%)achieved partial recovery, and 48 (34.04%) were ineffective. The total effective rate was 65.96%. In lidocaine group, the total effective rate was 55.00%, 66.67% in lidocaine and hormone group, 67.50% in Ginaton group, and 68.33% in Ginaton and hormone group. Considering the total effective rate, there was no statistical difference between four groups (P > 0.05). However, the recovery rate in Ginaton group was significant difference comparing with that in lidocaine group (P = 0.0496). 119 had concomitant symptom of tinnitus, and the tinnitus was improved in patients of 81.51%. With regard to total effective rate of tinnitus in four treatment groups, it was 57.89% (11/19) in lidocaine group, 100.00% (18/18) in lidocaine and hormone group, 88.57% (31/35) in Ginaton group, 78.72% (37/47) in Ginaton and hormone group. There was significant ascendancy in lidocaine and hormone group versus that in lidocaine group (P = 0.002) and Ginaton and hormone group (P = 0.029). And the difference between lidocaine and Ginaton groups was statistical significance (χ(2) = 6.705, P hearing has no significantly different between

  3. Symposium on sensorineural hearing loss in children: early detection and intervention. Delivery systems in the management of hearing impaired children.

    Science.gov (United States)

    Fellendorf, G W

    1975-02-01

    The management of a hearing impaired child involves the family as well as the child himself, especially when the child is very young. Delivery systems therefore must include the parents as an integral part of the delivery chain and be sensitive to the parents' need to understand the treatment or therapy and, when appropriate, to sustain it in the home. Health care services that have educational implications have been termed eduhealth, which in the case of the hearing impaired child, refers to such services as the early detection of hearing loss, the proper fitting of hearing aids, the prompt treatment of upper respiratory infections that might lead to further hearing loss, and vision screening. The otolaryngologist, audiologist, and parent all have eduhealth responsibilities. Professionals in medicine and allied fields, although well trained and experienced in their specialities, are rarely prepared to meet all the needs of the young hearing impaired child and his parents with appropriate services and in the manner most appropriate for them. Some agencies, however, have demonstrated comprehensive capabilities in this area. In referring a family to a delivery system, the otolaryngologist should establish to his own satisfaction that the requisite skills are present. Common complaints of parents of hearing impaired children are the lack of communication with the physician and clinician, which is frequently the result of the vocabulary used by the specialist and the limited time spent with the parents during visits. The result can be parental failure to follow instructions and possibly irreversible damage to the child's development of listening, language, and speech skills. The eduhealth delivery service index is a new attempt to measure the effectiveness of systems for the delivery of education and health care services to young hearing impaired children and their parents. It has been demonstrated in a limited population and may prove useful in the comparison of

  4. Efficacy comparison of oral steroid, intratympanic steroid, hyperbaric oxygen and oral steroid + hyperbaric oxygen treatments in idiopathic sudden sensorineural hearing loss cases.

    Science.gov (United States)

    Alimoglu, Yalcin; Inci, Ender; Edizer, Deniz Tuna; Ozdilek, Alper; Aslan, Mehmet

    2011-12-01

    Idiopathic sudden sensorineural hearing loss is a rare disorder of unknown pathogenesis in which hearing is lost partially or totally. About 60 treatment modalities have been described. We aimed to compare the efficacy of hyperbaric oxygen, oral steroid, intratympanic steroid therapy and their combinations in idiopathic sudden sensorineural hearing loss patients. Files of patients who were followed up between 2004 and 2010 in our clinic were examined retrospectively. Patients were divided into four groups according to the therapy received: Oral steroid, oral steroid + hyperbaric oxygen, intratympanic steroid and hyperbaric oxygen. Treatment success was assessed by Siegel criteria and mean gains using pre-treatment and post-treatment audiograms. 217 patients and 219 ears were examined. The proportion of patients responding to therapy was the highest in the oral steroid + hyperbaric oxygen group with 86.88% (53/61) followed by the oral steroid group with 63.79% (37/58), the intratympanic steroid group with 46,51% (20/43) and the hyperbaric oxygen group with 43.85% (25/57). The proportion of patients who had complete recovery was the highest in the oral steroid + hyperbaric oxygen group with 42.6% (26/61) followed by the oral steroid group with 19.0% (11/58), the hyperbaric oxygen group with 17.5% (10/57) and the intratympanic steroid group with 11.6% (5/43). The oral steroid + hyperbaric oxygen group has the highest mean hearing gain among all groups (p sudden sensorineural hearing loss patients receiving oral steroid + hyperbaric oxygen combination therapy have a higher likelihood of recovery than patients receiving oral steroids, hyperbaric oxygen or intratympanic steroids alone.

  5. Computed tomographic features of the bony canal of the cochlear nerve in pediatric patients with unilateral sensorineural hearing loss

    International Nuclear Information System (INIS)

    Kono, Tatsuo

    2008-01-01

    The aim of this study was to evaluate the diameters of the various bony canals of the inner ear in patients with sensorineural hearing loss (SNHL) and establish criteria for detecting hypoplasia of the bony canal of the cochlear nerve. Measurements obtained in 118 patients without inner ear malformations among 160 patients with unilateral SNHL were analyzed. The diameters of the internal auditory canal and the bony canals of the cochlear, vestibular, and facial nerves were measured on transverse or coronal computed tomographic images. Mean values (±standard deviation (SD)) were compared between the affected and unaffected ears, and statistical analysis was done. The diameter of the bony canal of the cochlear nerve was significantly smaller in affected ears than in unaffected ears (P<0.01). The affected ears could be divided into groups with (72 ears) and without (46 ears) bony canal stenosis. Most (60%) of the patients with unilateral SNHL showed a significant difference in the diameters of the bony canals of the cochlear nerve between the affected and unaffected sides; moreover, the mean value was significantly smaller in affected ears. The diameter of <1.7 mm on transverse images or <1.8 mm on coronal images suggests hypoplasia. (author)

  6. Presence of cytomegalovirus in the perilymphatic fluid of patients with profound sensorineural hearing loss caused by congenital cytomegalovirus infection.

    Science.gov (United States)

    Ogawa, Hiroshi; Matsui, Takamichi; Baba, Yoko; Yamada, Naoko; Suzuki, Yukie; Suzutani, Tatsuo

    2016-01-01

    Not all patients diagnosed with congenital infection using umbilical cord assay were found to be positive for CMV-DNA by perilymphatic fluid assay. In addition, a CMV-DNA-positive result was observed in one patient who had not been diagnosed with congenital infection. Sampling of perilymphatic fluid from a large population of patients with congenital SNHL caused by congenital CMV infection or of unknown etiology is required to determine the prevalence of CMV-related profound HL. Sensorineural hearing loss (SNHL) is one of the most frequent manifestations in patients with congenital cytomegalovirus (CMV) infection. Using dried umbilical cord, a PCR-based assay was recently developed for the retrospective detection of congenital CMV infection. This study analyzed the presence of CMV in the perilymphatic fluid and evaluated differences in the effect of cochlear implantation between CMV-positive and -negative groups. Perilymphatic fluid was collected from each patient at the time of cochlear implantation and analyzed for the presence of CMV using a PCR method. The perilymphatic fluid in two of the five patients suffering from congenital CMV infection and in one of the 17 patients without congenital CMV infection was found to be positive for CMV.

  7. Using Balance Function to Screen for Vestibular Impairment in Children With Sensorineural Hearing Loss and Cochlear Implants.

    Science.gov (United States)

    Oyewumi, Modupe; Wolter, Nikolaus E; Heon, Elise; Gordon, Karen A; Papsin, Blake C; Cushing, Sharon L

    2016-08-01

    1) To determine if bilateral vestibular dysfunction can be predicted by performance on standardized balance tasks in children with sensorineural hearing loss (SNHL) and cochlear implants (CI). 2) To provide clinical recommendations for screening for vestibular impairment in children with SNHL. Retrospective cohort study. Tertiary care pediatric implant center. Pediatric patients (4.8-18.6 years) with profound SNHL using CIs. Vestibular end-organ (horizontal canal and otoliths), and balance assessment. Comparison of balance skills, measured by the Bruininks Oseretsky Test of Motor Proficiency II (BOT-2), was performed between two groups of children with SNHL and CI: 1) total bilateral vestibular loss (TBVL) (n = 45), and 2) normal bilateral vestibular function (n = 20). Sensitivity, specificity, and suitability of each task as a screening tool for the detection of TBVL were assessed. Balance as measured by the BOT-2 balance subtest was significantly poorer in children with TBVL then those with normal vestibular function (p balance function using one of the BOT-2 balance subtest tasks, one-foot standing eyes closed, is able to identify children at risk of TBVL with excellent sensitivity and specificity and should be used to screen for TBVL in all children presenting with SNHL.

  8. Risk of sudden sensorineural hearing loss in adults using phosphodiesterase type 5 inhibitors: Population-based cohort study.

    Science.gov (United States)

    Liu, Wei; Antonelli, Patrick J; Dahm, Philipp; Gerhard, Tobias; Delaney, Joseph A C; Segal, Richard; Crystal, Stephen; Winterstein, Almut G

    2018-03-07

    The objective of the study was to determine the risk of sudden sensorineural hearing loss (SNHL) associated with use of phosphodiesterase type 5 (PDE5) inhibitors. We conducted a retrospective cohort study in the MarketScan Commercial Claims and Encounters Database including adult men who initiated a PDE5 inhibitor (n = 377,722) and 1,957,233 nonusers between 1998 and 2007. Periods of drug exposure were assessed on a weekly basis based on pharmacy billing records, assuming use of 1 dose per week (current use). Incident sudden SNHL was defined based on inpatient or outpatient visits with International Classification of Diseases, Ninth Revision, Clinical Modification codes 389.1x, 389.2x, or 388.2 plus ≥2 procedure codes for audiometric hearing testing within ±30 days of sudden SNHL diagnosis. We used age- and propensity score-adjusted Cox proportional hazards model to evaluate the risk of sudden SNHL during periods of current or recent use compared with that of nonuse. We conducted sensitivity analyses by varying the assumed drug utilization frequency and sudden SNHL case definition. We evaluated 1233 sudden SNHL cases, resulting in an incidence of 4.35, 5.58, and 2.38 per 10,000 person-years for current, recent, and nonuse of PDE5 inhibitors, respectively. Compared with nonuse, the adjusted hazard ratio was 1.25 (1.01-1.55) for current use with a risk difference of 1.97 (1.12-2.82) per 10,000 person-years. For recent use, the adjusted hazard ratio was 1.60 (1.33-1.94) and risk difference was 3.19 (2.24-4.14). Estimates were consistent across the sensitivity analyses. Use of PDE5 inhibitors is associated with a small but significantly increased risk of sudden SNHL. Copyright © 2018 John Wiley & Sons, Ltd.

  9. WFS1 and non-syndromic low-frequency sensorineural hearing loss: a novel mutation in a Portuguese case.

    Science.gov (United States)

    Gonçalves, A C; Matos, T D; Simões-Teixeira, H R; Pimenta Machado, M; Simão, M; Dias, O P; Andrea, M; Fialho, G; Caria, H

    2014-04-01

    Low-frequency sensorineural hearing loss (LFSNHL) is an unusual type of HL in which frequencies at 2,000 Hz and below are predominantly affected. Most of the families with LFSNHL carry missense mutations in WFS1 gene, coding for wolframin. A Portuguese patient aged 49, reporting HL since her third decade of life, and also referring tinnitus, was shown to display bilateral moderate LFSNHL after audiological evaluation. Molecular analysis led to the identification of a novel mutation, c.511G>A (p.Asp171Asn), found in heterozygosity in the exon 5 of the WFS1 gene, and changing the aspartic acid at position 171 to an asparagine, in the extracellular N-terminus domain of the wolframin protein. This novel mutation wasn't present either in 200 control chromosomes analyzed or in the hearing proband's half-brother, and it had not been reported in 1000 Genomes, Exome Variant Server, HGMD or dbSNP databases. No mutations were found in GJB2 and GJB6 genes. Multi-alignment of 27 wolframin sequences from mammalian species, against the human wolframin sequence in ConSurf, indicated a conservation score corresponding to 7 in a 1-9 color scale where 9 is conserved and 1 is variable. In addition, the mutation p.Asp171Asn was predicted to be damaging and possibly damaging by SIFT and Polyphen-2, respectively. The auditory phenotype of this patient could thus be due to the novel mutation p.Asp171Asn. Further functional characterization might enable to elucidate in which way the change in the residue 171, as other changes introduced by LFSNHL-associated mutations previously described, leads to this type of HL. Copyright © 2014 Elsevier B.V. All rights reserved.

  10. Ocular Disorders in Turkish Children with Sensorineural Hearıng Loss: A Cross-Sectional Study and Literature Review.

    Science.gov (United States)

    Ozer, Pinar Altiaylik; Kabatas, Emrah Utku; Ertugrul, Gokce Tasdemir; Kurtul, Bengi Ece; Kaygusuz, Umut; Ozgursoy, Selmin Karatayli

    2016-09-14

    To investigate types and frequencies of ocular disorders in children with sensorineural hearing loss (SNHL), and to emphasize the importance of ophthalmological examination in these children. A retrospective analysis of the examination records of children examined in our instutititon between January 2011 and September 2014 was performed. Ocular disorders of children with SHNL were selectively reviewed. Among 55340 patients, SNHL was present in 110 (0.2%). SNHL was bilateral in 104 patients (94.5%) and unilateral in 6 (5.5%). Ninety-one cases had congenital hearing loss (83%), and 19 (17%) had acquired SNHL. Forty cases (36%) had an ocular disorder, either refractive or non-refractive or both. Seventy cases (64%) had normal ocular examination. No difference was found between congenital or acquired SNHL cases in terms of possessing an ocular disorder (p=0.0962). The most common ocular abnormality was refractive error, mainly hypermetropia (21%). There was no significant difference between the prevalences of ocular abnormalities among cases with different lateralites or severities of SNHL (p=0.051, p=0.874, respectively). Twenty-six cases (23.6%) had SNHL as a component of a genetically defined syndrome. All of them had coexisting refractive or non-refractive ocular abnormalities. Some genetic, non-syndromic abnormalities, including Achondroplasia, Celiac disease, and focal segmental glomerulosclerosis, were diagnosed in four cases, among whom refractive errors and/or strabismus were detected. Due to the common coexistence of ocular problems and SNHL in children, ophthalmological screening is crucial. Families and healthcare providers should be informed about the critical role of ophthalmic assesment in these children for their future quality of life.

  11. Speech recognition in normal hearing and sensorineural hearing loss as a function of the number of spectral channels

    NARCIS (Netherlands)

    Baskent, Deniz

    2006-01-01

    Speech recognition by normal-hearing listeners improves as a function of the number of spectral channels when tested with a noiseband vocoder simulating cochlear implant signal processing. Speech recognition by the best cochlear implant users, however, saturates around eight channels and does not

  12. The paracrine effect of mesenchymal human stem cells restored hearing in β-tubulin induced autoimmune sensorineural hearing loss.

    Science.gov (United States)

    Yoo, T J; Du, Xiaoping; Zhou, Bin

    2015-12-01

    The aim of this study was to examine the activities of hASCs (Human Adipose tissue Derived Stem Cells) on experimental autoimmune hearing loss (EAHL) and how human stem cells regenerated mouse cochlea cells. We have restored hearing in 19 years old white female with autoimmune hearing loss with autologous adipose tissue derived stem cells and we wish to understand the mechanism of restoration of hearing in animal model. BALB/c mice underwent to develop EAHL; mice with EAHL were given hASCs intraperitoneally once a week for 6 consecutive weeks. ABR were examined over time. The helper type 1 autoreactive responses and T-reg cells were examined. H&E staining or immunostaining with APC conjugated anti-HLA-ABC antibody were conducted. The organ of Corti, stria vascularis, spira ligament and spiral ganglion in stem cell group are normal. In control group, without receiving stem cells, the organ of Corti is replaced by a single layer of cells, atrophy of stria vascularis. Systemic infusion of hASCs significantly improved hearing function and protected hair cells in established EAHL. The hASCs decreased the proliferation of antigen specific Th1/Th17 cells and induced the production of anti-inflammatory cytokine interleukin10 in splenocytes. They also induced the generation of antigen specific CD4(+)CD25(+)Foxp3(+)T-reg cells. The experiment showed the restoration is due to the paracrine activities of human stem cells, since there are newly regenerated mice spiral ganglion cells, not human mesenchymal stem cells derived tissue given by intraperitoneally. Copyright © 2015. Published by Elsevier B.V.

  13. Hearing Loss due to Carbon Monoxide Poisoning

    DEFF Research Database (Denmark)

    Mehrparvar, Amir Houshang; Davari, Mohammad Hossein; Mollasadeghi, Abolfazl

    2013-01-01

    Carbon monoxide poisoning is one of the rare causes of hearing loss which may cause reversible or irreversible, unilateral or bilateral hearing loss after acute or chronic exposure. In this report, we present a case of bilateral sensorineural hearing loss in a secondary smelting workshop worker...... after an acute exposure to carbon monoxide. This complication was diagnosed by pure-tone audiometry and confirmed by transient evoked otoacoustic emissions. Hearing loss has not improved after 3 months of followup....

  14. The internal acoustic canal - another review area in paediatric sensorineural hearing loss

    Energy Technology Data Exchange (ETDEWEB)

    Chetcuti, Karen [The Royal Children' s Hospital, Department of Medical Imaging, Parkville, VIC (Australia); Kumbla, Surekha [The Royal Children' s Hospital, Department of Medical Imaging, Parkville, VIC (Australia); Monash Health, Clayton, VIC (Australia)

    2016-04-15

    Morphological abnormalities of the internal acoustic canal (IAC), albeit rare, are sometimes associated with hearing loss in children. We present an illustration of the spectrum of IAC abnormalities together with a brief review of the embryology and anatomy of the IAC and the techniques used when imaging the petrous temporal bone. This review focuses on morphological abnormalities of the IAC together with their clinical implications and impact on clinical management. (orig.)

  15. The internal acoustic canal - another review area in paediatric sensorineural hearing loss

    International Nuclear Information System (INIS)

    Chetcuti, Karen; Kumbla, Surekha

    2016-01-01

    Morphological abnormalities of the internal acoustic canal (IAC), albeit rare, are sometimes associated with hearing loss in children. We present an illustration of the spectrum of IAC abnormalities together with a brief review of the embryology and anatomy of the IAC and the techniques used when imaging the petrous temporal bone. This review focuses on morphological abnormalities of the IAC together with their clinical implications and impact on clinical management. (orig.)

  16. EEG activity as an objective measure of cognitive load during effortful listening: A study on pediatric subjects with bilateral, asymmetric sensorineural hearing loss.

    Science.gov (United States)

    Marsella, Pasquale; Scorpecci, Alessandro; Cartocci, Giulia; Giannantonio, Sara; Maglione, Anton Giulio; Venuti, Isotta; Brizi, Ambra; Babiloni, Fabio

    2017-08-01

    Deaf subjects with hearing aids or cochlear implants generally find it challenging to understand speech in noisy environments where a great deal of listening effort and cognitive load are invested. In prelingually deaf children, such difficulties may have detrimental consequences on the learning process and, later in life, on academic performance. Despite the importance of such a topic, currently, there is no validated test for the assessment of cognitive load during audiological tasks. Recently, alpha and theta EEG rhythm variations in the parietal and frontal areas, respectively, have been used as indicators of cognitive load in adult subjects. The aim of the present study was to investigate, by means of EEG, the cognitive load of pediatric subjects affected by asymmetric sensorineural hearing loss as they were engaged in a speech-in-noise identification task. Seven children (4F and 3M, age range = 8-16 years) affected by asymmetric sensorineural hearing loss (i.e. profound degree on one side, mild-to-severe degree on the other side) and using a hearing aid only in their better ear, were included in the study. All of them underwent EEG recording during a speech-in-noise identification task: the experimental conditions were quiet, binaural noise, noise to the better hearing ear and noise to the poorer hearing ear. The subjects' Speech Recognition Thresholds (SRT) were also measured in each test condition. The primary outcome measures were: frontal EEG Power Spectral Density (PSD) in the theta band and parietal EEG PSD in the alpha band, as assessed before stimulus (word) onset. No statistically significant differences were noted among frontal theta power levels in the four test conditions. However, parietal alpha power levels were significantly higher in the "binaural noise" and in the "noise to worse hearing ear" conditions than in the "quiet" and "noise to better hearing ear" conditions (p children with sensorineural hearing loss with the purpose of studying

  17. " Evaluation Of Some Epidemiologic Parameters, Risk Factors, Clinical And Audiological Characteristics Of 48 Patients With Sudden Sensorineural Hearing Loss "

    Directory of Open Access Journals (Sweden)

    M. Amiridavan

    2006-05-01

    Full Text Available Background and Aim: Sudden Sensorineural Hearing Loss (SSNHL is an emergency situation, and is one of the most controversial subjects in domain of otolaryngology. In this article, we have analyzed some Epidemiologic Characteristics, clinical features, audiological Characteristics, and other findings in routine serological tests and MRI of 48 cases with SSNHL ,who came or were referred to us in the past 2 years. Study design: Cross sectional. Materials and Methods: In 48 patients with chief complaint of SSNHL, from June 2003 to Feb. 2005, who were admitted in clinic of otolaryngology- in Kashani Hospital -Isfahan- Iran, physical examination and history taking, audiological evaluation, MRI,and serological tests were performed in a similar way , and data were analyzed by SPSS software. Results: From 48 cases(M:28 ,F:20 with mean age of 40.9(+/-15.9 years, left ear was involved in 26 cases (54.1% ,and right ear in 19 cases (39.5% ,and in 3 cases (6.3%,both sides were involved. The severity of hearing loss was “subjectively” HIGH in 78% of patients, and the mean threshold of hearing had been calculated as 69 dB. The most common pattern in pure tone audiometry curves ,was ‘flat pattern’(75% ,and then ,’down sloping pattern’(16%.The most adjunctive clinical symptom was “tinnitus”(in 78.7%, and 40% of patients had “true vertigo”. 44.4% of our patients had some evidences of upper respiratory tract infections (URIs during recent 2 weeks. Positive family history, smoking, alcohol intake ,oral contraceptive and ototoxic drugs consumption were uncommon. 24% of cases (11 of 39 had increased ESR, and 100% of 39 patients had negative VDRL. Diabetes mellitus was the most common underlying disease (in 6 cases.From 20 patients ,who were succeeded to perform brain and ear MRI, 2 cases had tumor in internal auditory canal and cerebellopontine angle. Conclusion: SSNHL has some limitations in being studied histopathologically or in the form of

  18. Hyperbaric Oxygen Therapy in the Treatment of Idiopathic Sudden Sensorineural Hearing Loss

    Directory of Open Access Journals (Sweden)

    Debasish Guha

    2015-12-01

    Males comprised 62.5% (30/48 of our study population, mean age of the population was 49.3 ± 13.4 years. 45/48 patients (93.7% had unilateral ISSNHL, while 3 (6.3% had bilateral ISSNHL. Average hearing loss in the affected ear before and after treatment was 79.96dBHL and 62.27dBHL respectively. The most common secondary clinical feature was tinnitus (27/48, 56.3%, followed by vertigo (24/48, 50%, aural fullness (15/48, 31.25% and nystagmus (5/48, 10.41%. After treatment tinnitus and vertigo were showed marked improvement; (22/27, 81.48% and (18/24, 75%. 5 out of 15 (33.34% patients showed reduced aural fullness. There was no improvement in patients suffering from nystagmus.

  19. Degeneration of auditory nerve fibers in guinea pigs with severe sensorineural hearing loss.

    Science.gov (United States)

    Kroon, Steven; Ramekers, Dyan; Smeets, Emma M; Hendriksen, Ferry G J; Klis, Sjaak F L; Versnel, Huib

    2017-03-01

    Damage to and loss of the organ of Corti leads to secondary degeneration of the spiral ganglion cell (SGC) somata of the auditory nerve. Extensively examined in animal models, this degeneration process of SGC somata following deafening is well known. However, degeneration of auditory nerve axons, which conduct auditory information towards the brainstem, and its relation to SGC soma degeneration are largely unknown. The consequences of degeneration of the axons are relevant for cochlear implantation, which is applied to a deafened system but depends on the condition of the auditory nerve. We investigated the time sequence of degeneration of myelinated type I axons in deafened guinea pigs. Auditory nerves in six normal-hearing and twelve deafened animals, two, six and fourteen weeks (for each group four) after deafening were histologically analyzed. We developed a semi-automated method for axon counting, which allowed for a relatively large sample size (20% of the total cross-sectional area of the auditory nerve). We observed a substantial loss of auditory nerve area (29%), reduction in axon number (59%) and decrease in axoplasm area (41%) fourteen weeks after deafening compared to normal-hearing controls. The correlation between axonal degeneration and that of the SGC somata in the same cochleas was high, although axonal structures appeared to persist longer than the somata, suggesting a slower degeneration process. In the first two weeks after induction of deafness, the axonal cross-sectional area decreased but the axon number did not. In conclusion, the data strongly suggest that each surviving SGC possesses an axon. Copyright © 2017 Elsevier B.V. All rights reserved.

  20. Acupuncture therapy for sudden sensorineural hearing loss: a systematic review and meta-analysis of randomized controlled trials.

    Science.gov (United States)

    Zhang, Xin-chang; Xu, Xiu-ping; Xu, Wen-tao; Hou, Wen-zhen; Cheng, Ying-ying; Li, Chang-xi; Ni, Guang-xia

    2015-01-01

    Acupuncture has commonly been used in China, either alone or in combination with Western medicine, to treat sudden sensorineural hearing loss (SSHL). The purpose of this systematic review is to assess the efficacy and safety of acupuncture therapy for patients with SSHL. We searched PubMed, the Cochrane Library, Embase, China National Knowledge Internet (CNKI), Database for Chinese Technical Periodicals (VIP), and Chinese Biomedical literature service system (SinoMed) to collect randomized controlled trials of acupuncture for SSHL published before July 2014. A meta-analysis was conducted according to the Cochrane systematic review method using RevMan 5.2 software. The evidence level for each outcome was assessed using the GRADE methodology. Twelve trials involving 863 patients were included. A meta-analysis showed that the effect of manual acupuncture combined with Western medicine comprehensive treatment (WMCT) was better than WMCT alone (RR 1.33, 95%CI 1.19-1.49) and the same as the effect of electroacupuncture combined with WMCT (RR 1.33, 95%CI 1.19-1.50). One study showed a better effect of electroacupuncture than of WMCT (RR 1.34, 95%CI 1.24-1.45). For mean changes in hearing over all frequencies, the meta-analysis showed a better effect with the combination of acupuncture and WMCT than with WMCT alone (MD 10.85, 95%CI 6.84-14.86). However, the evidence levels for these interventions were low or very low due to a high risk of bias and small sample sizes in the included studies. There was not sufficient evidence showing that acupuncture therapy alone was beneficial for treating SSHL. However, interventions combining acupuncture with WMCT had more efficacious results in the treatment of SSHL than WMCT alone. Electroacupuncture alone might be a viable alternative treatment besides WMCT for SSHL. However, given that there were fewer eligible RCTs and limitations in the included trials, such as methodological drawbacks and small sample sizes, large-scale RCTs are

  1. Acupuncture therapy for sudden sensorineural hearing loss: a systematic review and meta-analysis of randomized controlled trials.

    Directory of Open Access Journals (Sweden)

    Xin-chang Zhang

    Full Text Available Acupuncture has commonly been used in China, either alone or in combination with Western medicine, to treat sudden sensorineural hearing loss (SSHL. The purpose of this systematic review is to assess the efficacy and safety of acupuncture therapy for patients with SSHL.We searched PubMed, the Cochrane Library, Embase, China National Knowledge Internet (CNKI, Database for Chinese Technical Periodicals (VIP, and Chinese Biomedical literature service system (SinoMed to collect randomized controlled trials of acupuncture for SSHL published before July 2014. A meta-analysis was conducted according to the Cochrane systematic review method using RevMan 5.2 software. The evidence level for each outcome was assessed using the GRADE methodology.Twelve trials involving 863 patients were included. A meta-analysis showed that the effect of manual acupuncture combined with Western medicine comprehensive treatment (WMCT was better than WMCT alone (RR 1.33, 95%CI 1.19-1.49 and the same as the effect of electroacupuncture combined with WMCT (RR 1.33, 95%CI 1.19-1.50. One study showed a better effect of electroacupuncture than of WMCT (RR 1.34, 95%CI 1.24-1.45. For mean changes in hearing over all frequencies, the meta-analysis showed a better effect with the combination of acupuncture and WMCT than with WMCT alone (MD 10.85, 95%CI 6.84-14.86. However, the evidence levels for these interventions were low or very low due to a high risk of bias and small sample sizes in the included studies.There was not sufficient evidence showing that acupuncture therapy alone was beneficial for treating SSHL. However, interventions combining acupuncture with WMCT had more efficacious results in the treatment of SSHL than WMCT alone. Electroacupuncture alone might be a viable alternative treatment besides WMCT for SSHL. However, given that there were fewer eligible RCTs and limitations in the included trials, such as methodological drawbacks and small sample sizes, large

  2. Rubella in Sub-Saharan Africa and sensorineural hearing loss: a case control study.

    Science.gov (United States)

    Caroça, Cristina; Vicente, Vera; Campelo, Paula; Chasqueira, Maria; Caria, Helena; Silva, Susana; Paixão, Paulo; Paço, João

    2017-02-01

    Rubella infection can affect several organs and cause birth defects that are responsible for congenital rubella syndrome (CRS). Congenital hearing loss is the most common symptom of this syndrome, occurring in approximately 60% of CRS cases. Worldwide, over 100 000 babies are born with CRS every year. There is no specific treatment for rubella, but the disease is preventable by vaccination. Since 1969, the rubella vaccine has been implemented in many countries, but in Africa, only a few countries routinely immunize against rubella. The aim of this study was to estimate the rate of infection from the wild-type rubella virus in São Tomé and Príncipe by determining rubella seroprevalence with a DBS method. The goal of this study was to reinforce the need for implementation of the rubella vaccine in this country. As secondary objectives, the validation of a DBS method was first attempted and an association between seroprevalence and hearing loss was assessed. We collected samples from individuals observed during humanitarian missions in São Tomé and Príncipe. All individuals underwent an audiometric evaluation, and a drop of blood was collected for the dried blood spot (DBS). We define two groups: the case group (individuals with unilateral or bilateral hearing loss (HL)) and the control group (individuals with two normal ears). Patients were excluded if they suffered from conductive HL, if they showed evidence of possible causes of HL, if they had developmental delay or if they refused to participate in the study. Among the 315 subjects, we found 64.1% individuals with IgG for the rubella virus, 32.1% without immunity for the rubella virus and 3.8% who were borderline. In the control group, 62.6% were positive for the rubella IgG, whereas in the case group, 72% were positive. Analyzing both groups, with ages ranging from 2 to 14 years of age and from 15 to 35 years of age, we found a seroprevalence of 50.3% to rubella in the younger group and 82.1% in the

  3. Rubella in Sub-Saharan Africa and sensorineural hearing loss: a case control study

    Directory of Open Access Journals (Sweden)

    Cristina Caroça

    2017-02-01

    Full Text Available Abstract Background Rubella infection can affect several organs and cause birth defects that are responsible for congenital rubella syndrome (CRS. Congenital hearing loss is the most common symptom of this syndrome, occurring in approximately 60% of CRS cases. Worldwide, over 100 000 babies are born with CRS every year. There is no specific treatment for rubella, but the disease is preventable by vaccination. Since 1969, the rubella vaccine has been implemented in many countries, but in Africa, only a few countries routinely immunize against rubella. The aim of this study was to estimate the rate of infection from the wild-type rubella virus in São Tomé and Príncipe by determining rubella seroprevalence with a DBS method. The goal of this study was to reinforce the need for implementation of the rubella vaccine in this country. As secondary objectives, the validation of a DBS method was first attempted and an association between seroprevalence and hearing loss was assessed. Methods We collected samples from individuals observed during humanitarian missions in São Tomé and Príncipe. All individuals underwent an audiometric evaluation, and a drop of blood was collected for the dried blood spot (DBS. We define two groups: the case group (individuals with unilateral or bilateral hearing loss (HL and the control group (individuals with two normal ears. Patients were excluded if they suffered from conductive HL, if they showed evidence of possible causes of HL, if they had developmental delay or if they refused to participate in the study. Results Among the 315 subjects, we found 64.1% individuals with IgG for the rubella virus, 32.1% without immunity for the rubella virus and 3.8% who were borderline. In the control group, 62.6% were positive for the rubella IgG, whereas in the case group, 72% were positive. Analyzing both groups, with ages ranging from 2 to 14 years of age and from 15 to 35 years of age, we found a seroprevalence of 50.3% to

  4. Neuroradiological imaging in patients with sensorineural hearing loss prior to cochlear implantation; Neuroradiologische Diagnostik bei Patienten mit sensorineuralem Hoerverlust vor Cochlea-Implantation

    Energy Technology Data Exchange (ETDEWEB)

    Biller, A.; Bartsch, A.; Solymosi, L.; Bendszus, M. [Abteilung fuer Neuroradiologie, Universitaet Wuerzburg (Germany); Knaus, C.; Mueller, J. [Klinik und Poliklinik fuer Hals-, Nasen- und Ohrenkranke, Universitaet Wuerzburg (Germany)

    2007-09-15

    Cochlear implantation (CI) is an established technique for enabling speech perception in patients suffering from severe bilateral sensorineural hearing loss (SNHL). Thorough preoperative radiological assessment is essential for correctly evaluating the indication for surgery and safely performing cochlear implantation. CT and conventional and functional MRI are available for radiological assessment. Therefore, knowledge of the most frequent congenital syndromal, non-syndromal, and acquired malformations of inner ear structures is fundamental. This article provides information about imaging techniques prior to CI and relevant malformations of the inner ear. Safety aspects for patients with a cochlear implant undergoing MR imaging are also discussed. (orig.)

  5. A novel WFS1 mutation in a family with dominant low frequency sensorineural hearing loss with normal VEMP and EcochG findings

    OpenAIRE

    Bramhall, Naomi F; Kallman, Jeremy C; Verrall, Aimee M; Street, Valerie A

    2008-01-01

    Abstract Background Low frequency sensorineural hearing loss (LFSNHL) is an uncommon clinical finding. Mutations within three different identified genes (DIAPH1, MYO7A, and WFS1) are known to cause LFSNHL. The majority of hereditary LFSNHL is associated with heterozygous mutations in the WFS1 gene (wolframin protein). The goal of this study was to use genetic analysis to determine if a small American family's hereditary LFSNHL is linked to a mutation in the WFS1 gene and to use VEMP and Ecoch...

  6. Genetic frequencies related to severe or profound sensorineural hearing loss in Inner Mongolia Autonomous Region

    Directory of Open Access Journals (Sweden)

    Yongzhi Liu

    Full Text Available Abstract The aim was to study the frequencies of common deafness-related mutations and their contribution to hearing loss in different regions of Inner Mongolia. A total of 738 deaf children were recruited from five different ethnic groups of Inner Mongolia, including Han Chinese (n=486, Mongolian (n=216, Manchurian (n=24, Hui (n=6 and Daur (n=6. Nine common mutations in four genes (GJB2, SLC26A4, GJB3 and mitochondrial MT-RNR1 gene were detected by allele-specific PCR and universal array. At least one mutated allele was detected in 282 patients. Pathogenic mutations were detected in 168 patients: 114 were homozygotes and 54 were compound heterozygotes. The 114 patients were carriers of only one mutated allele. The frequency of GJB2 variants in Han Chinese (21.0% was higher than that in Mongolians (16.7%, but not significantly different. On the other hand, the frequency of SLC26A4 variants in Han Chinese (14.8% was lower than that in Mongolians (19.4%, but also not significantly different. The frequency of patients with pathogenic mutations was different in Ulanqab (21.4%, Xilingol (40.0%, Chifeng (40.0%, Hulunbeier (30.0%, Hohhot (26.3%, and in Baotou (0%. In conclusion, the frequency of mutated alleles in deafness-related genes did not differ between Han Chinese and Mongolians. However, differences in the distribution of common deafness-related mutations were found among the investigated areas of Inner Mongolia.

  7. Perda auditiva sensorioneural em pacientes com acromegalia em tratamento Sensorineural hearing loss in acromegalic patients under treatment

    Directory of Open Access Journals (Sweden)

    Marcelo Alexandre Carvalho

    2012-08-01

    Full Text Available Acromegalia é uma doença endócrina rara. Poucos estudos avaliaram sua associação com deficiência auditiva (DA e os resultados são conflitantes. OBJETIVOS: Avaliar a prevalência e características da DA em um grupo de pacientes com acromegalia em tratamento. Analisar a transmissão auditiva central e periférica. MATERIAL E MÉTODOS: Estudo transversal. Um grupo de 34 pacientes com acromegalia submeteu-se à avaliação metabólica, audiometria tonal e potenciais evocados auditivos de tronco encefálico (PEATE. Considerou-se DA quando a média dos tons puros foi > 25 DBNA para baixas frequências (250, 500, 1000 e 2000 Hz ou altas frequências (3000, 4000, 6000 e 8000 Hz. Os pacientes foram divididos em grupo A (com DA e B (sem DA. RESULTADOS: Doze pacientes (35,3% mostraram DA sensorioneural (grupo A, sendo oito bilateral e quatro unilateral. Nenhum apresentou DA mista ou condutiva. A prevalência de diabetes/intolerância à glicose de jejum foi similar entre os grupos. As frequências de 3000, 4000, 6000 e 8000 Hz foram as mais afetadas e com padrão similar em ambos os lados. CONCLUSÃO: DA sensorioneural esteve presente em 35,3% dos casos. Não foram notadas diferenças clínicas ou metabólicas significativas entre os grupos, bem como na transmissão neural auditiva periférica e central.Acromegaly is a rare endocrine disease. Few studies have evaluated its association with hearing loss (HL and the results are conflicting. AIM: To evaluate the prevalence and features of HL in a group of patients being treated for acromegaly. To analyze peripheral and central auditory transmission. METHODS: Cross-sectional study. A group of 34 patients with acromegaly were submitted to metabolic evaluation, tonal audiometry and brainstem auditory evoked potentials. HL was considered when pure tone average was > 25 DBHL for low frequencies (250, 500, 1000 and 2000 Hz or high frequencies (3000, 4000, 6000 and 8000 Hz. The patients were divided in group

  8. Perda auditiva sensorioneural no lúpus eritematoso sistêmico: relato de três casos Sensorineural hearing loss in systemic lupus erythematosus: report of three cases

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    Suzana B. Cecatto

    2004-06-01

    Full Text Available INTRODUÇÃO: O Lúpus Eritematoso Sistêmico (LES é uma doença sistêmica do tecido conectivo, de etiologia desconhecida, provavelmente multifatorial. Acomete principalmente o sexo feminino podendo afetar múltiplos órgãos, dentre eles o sistema auditivo. A orelha interna pode ser lesada por diversos mecanismos auto-imunes, sendo a manifestação mais freqüente a disacusia sensorioneural flutuante, geralmente bilateral, rapidamente progressiva e com boa responsividade a imunossupressores. OBJETIVO: O objetivo deste trabalho é relatar três casos de disacusia de etiologia auto-imune, enfocando formas de acometimento e manifestações clínicas, bem como correlacionando o efeito ototóxico da cloroquina - droga empregada no controle do LES - com a perda auditiva. CONCLUSÃO: As perdas auditivas sensorioneurais súbitas, rapidamente progressivas ou flutuantes, podem ocorrer em pacientes com doença auto-imune e devem ser sempre lembradas nos casos de disacusia sem causa aparente.INTRODUCTION: The Systemic Lupus Erythematosus (SLE is a systemic disease of the connective tissue, with unknown etiology, probably associated to multiple events. It is a multiple organs disease that affects mainly women. The inner ear can be damaged by several immunopathogenic mechanisms, and the most common symptom is a progressive sensorineural hearing loss, generally bilateral, with good response to immunossupression. AIM: The purpose of this article is to report three cases of women suffering from SLE and hearing loss and to establish a link between the autoimmune and the vascular mechanisms of the disease, also focusing attention on the ototoxicity due to chloroquine applied during the treatment of SLE. CONCLUSION: Sudden or fluctuant sensorineural hearing loss may affect patients with autoimmune disease, so it must always be taken into account when dealing with patients suffering from hearing loss without any apparent cause.

  9. A novel mutation of EYA4 in a large Chinese family with autosomal dominant middle-frequency sensorineural hearing loss by targeted exome sequencing.

    Science.gov (United States)

    Sun, Yi; Zhang, Zhao; Cheng, Jing; Lu, Yu; Yang, Chang-Liang; Luo, Yan-Yun; Yang, Guang; Yang, Hui; Zhu, Li; Zhou, Jia; Yao, Hang-Qi

    2015-06-01

    The middle-frequency sensorineural hearing loss (MFSNHL) is rare among hereditary non-syndromic hearing loss. To date, only three genes are reported to be associated with MFSNHL, including TECTA, EYA4 and COL11A2. In this report, we analyzed and explored the clinical audiological characteristics and the causative gene of a Chinese family named HG-Z087 with non-syndromic autosomal dominant inherited MFSNHL. Clinical audiological characteristics and inheritance pattern of a family were evaluated, and pedigree was drawn based on medical history investigation. Our results showed that the Chinese family was characterized by late onset, progressive, non-sydromic autosomal dominant MFSNHL. Targeted exome sequencing, conducted using DNA samples of an affected member in this family, revealed a novel heterozygous missense mutation c.1643C>G in exon 18 of EYA4, causing amino-acid (aa) substitution Arg for Thr at a conserved position aa-548. The p.T548R mutation related to hearing loss in the selected Chinese family was validated by Sanger sequencing. However, the mutation was absent in control group containing 100 DNA samples from normal Chinese families. In conclusion, we identified the pathogenic gene and found that the novel missense mutation c.1643C>G (p.T548R) in EYA4 might have caused autosomal dominant non-syndromic hearing impairment in the selected Chinese family.

  10. Screening of Long Q-T Syndrome in Patients with Congenital Sensorineural Hearing Loss (Jervell and Lange Neilesen Syndrome: Prevention of Fatal Events

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    Farid Matin

    2001-01-01

    Full Text Available Objective:The idiopathic long Q-T syndrome is an infrequently occurring disorder in which affected individuals have an unusual electrocardiographic repolarization abnormality presenting as syncope or loss of consciousness related to ventricular tachycardia or fibrillation. Congenital long Q-T prolongation can be associated with congenital deafness in an autosomal recessive manner (Jervell and Lange-Nielsen syndrome. The purpose of this stuff was to screen this electrocardiographic abnormality in deaf-mute school children in our population, which has not been yet performed. Materials & Methods:  Of 1190 patients with hearing loss, 779 had congenital sensorineural deafness (CSD, aged 13±3.8 years (4-24, 63% female and 37% male. The family history of deafness was as follows: Cardiac axis deviation was found in 56 (7% patients. Electrical conduction abnormalities were found in 12 (15% patients, Wolff-Parkinson-White syndrome, sinus bradycardia, and sinus arrhythmia were found in 2 (0.25%, 4 (0.5%, and 3 (0.38% patients, respectively. The Q-T interval, and Q-Tc duration were 312.6±28.9 ms (200-500 ms, median 320 ms, and 383.6±29.3 ms (232-527 ms, median 413ms, respectively. Long Q-T syndrome was found in 4 (0.5% patients (3F and 1M. Results: Two of these 4 patients had total deafness and 2 had profound hearing loss. None of the patients with mild deafness had Q-T prolongation. Only one of these patients was symptomatic, and had been treated as a case of epilepsy for several years. Conclusion: This data supports the presence of long Q-T syndrome in patients with sensorineural hearing loss in our population, so routine electrocardiographic screening of anyone with congenital deafness is warranted to prevent subsequent associated cardiac arrhythmias and sudden cardiac death.

  11. [Analysis the relationship between SLC26A4 mutation and current diagnosis of inner ear malformation in children with sensorineural hearing loss].

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    Sun, Baochun; Zhou, Chengyong; Dai, Zhiyao

    2014-11-01

    Explore the relationship between the pathogenic mutations of SLC26A4 gene and inner ear malformation, and analyze the feasibility of genetic testing to help current diagnosis in part of children with sensorineural hearing loss. 2094 cases of children were detected by SLC26A4 with the method of DNA sequence. CT phenotypes of those children were classified according to the method proposed by Sennaroglu. We analyzed the relationship between the pathogenic mutations of gene and the CT phenotypes. (1) 685 cases of inner ear malformations were found in 2094 cases of children with sensorineural hearing loss by CT examination (371 cases of cochlea malformation were consisted of the follow types of malformation. Michel deformity was 6 cases, cochlea aplasia was 8 cases, common cavity deformity was 12 cases, incomplete partition type I was 27 cases, cochlea hypoplasia was 30 cases and Mondini malformation was 288 cases); Vestibular aqueduct was 265 cases; Vestibular/semicircular canal/internal auditory canal were 49 cases, normal was 1409 cases. (2) The DNA sequence results revealed that 465 cases carried pathogenic mutations (Bi-allelic mutations) of SLC26A4 gene, among which 135 cases were homozygous, 330 cases were compound heterozygous. (3) Pathogenic mutations of SLC26A4 gene detected 100% (465/465) in the group related to vestibular aqueduct malformation. The results suggest that pathogenic mutation of SLC26A4 gene is closely related to the CT phenotype of vestibular aqueduct malformation. Detecting of pathogenic mutations for hearing loss is binging the possibility to identify children with inner malformations at an early stage. As a consequence, it will improve the current diagnosis and therapeutical option.

  12. Serum immunoglobulins in 28 adults with autoimmune sensorineural hearing loss: increased prevalence of subnormal immunoglobulin G1 and immunoglobulin G3.

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    Bertoli, Luigi F; Pappas, Dennis G; Barton, J Clayborn; Barton, James C

    2014-10-22

    Our informal observations suggested that some patients with acute sensorineural hearing loss (ASNHL) have subnormal serum immunoglobulin (Ig) levels. We evaluated 28 consecutive adults (18 men, 10 women) at ASNHL diagnosis using: antibodies to 68 kD protein, 30 kD protein, and type II collagen; and serum total IgG, IgG subclasses, total IgA, and IgM. Reference ranges for Ig levels were mean ± 2 SD. We compared prevalences of subnormal IgG subclasses to those in 275 healthy European adults in previous reports. We also reviewed charts of consecutive adult index patients with primary Ig deficiency (35 common variable immunodeficiency, 406 IgG subclass deficiency) to identify other patients with probable ASHNL. Mean age was 53 ± 10 (SD) y. Six patients (21.4%) had other autoimmunity manifestations. Antibodies to 68 kD protein, 30 kD protein, and type II collagen were detected in 21.4% (6/28), 21.1% (4/19) and 18.8% (3/16), respectively. Three patients (10.7%) had subnormal IgG1, six (21.4%) had subnormal IgG3, and four (14.3%) had subnormal IgG1 and IgG3. Some had subnormal IgG2, IgG4, IgA, and IgM (n = 1, 2, 3, and 1, respectively). Prevalences of subnormal IgG1 or IgG3 were greater in ASNHL patients (25.0% and 35.7%) than 275 controls (2.1% and 3.3%), respectively (p < 0.0001, each comparison). Relative risks of subnormal IgG1 and IgG3 in ASNHL were 11.5 [95% CI: 4.1, 31.7] and 10.9 [4.8, 25.6], respectively. Hearing improved after initial therapy in 17 patients (60.7%). Multiple regressions on Ig levels revealed no significant associations with other available variables. Logistic regressions on initial therapy response revealed a positive association with men (p = 0.0392) and a negative association with IgA (p = 0.0274). Our estimated prevalence of probable ASNHL in 35 patients with common variable immunodeficiency during a follow-up interval of 8 ± 4 y was 0% [95% CI: 0, 12.3]). Prevalence of probable ASNHL in 406 patients with IgG subclass deficiency during the

  13. Sensorineural hearing loss: there is no correlation with isolated dysplasia of the lateral semi-circular canal on temporal bone CT

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    Yamashita, Koji; Yoshiura, Takashi; Hiwatashi, Akio; Tuvshinjargal, Dashjamts; Kamano, Hironori; Honda, Hiroshi (Dept. of Clinical Radiology, Graduate School of Medical Sciences, Kyushu Univ. Fukuoka (Japan)), email: tyoshiu@radiol.med.kyushu-u.ac.jp; Inoguchi, Takashi (Dept. of Otolaryngology, Kitakyushu Municipal Medical Center, Kitakyushu (Japan))

    2011-02-15

    Background: Inner ear malformations may cause sensorineural hearing loss (SNHL). However, the correlation between the small lateral semi-circular canal (LSCC) and SNHL is controversial. Purpose: To determine whether there is a correlation between the two using CT-based measurement. Material and Methods: We retrospectively reviewed the high-resolution CT images of the temporal bone obtained from consecutive patients. A total 136 ears of 68 patients (25 men and 43 women; age range 20-85 years, mean 49.8 years) were included in this study. Patients who were clinically suspected to have otosclerosis were also excluded. Two radiologists independently measured the width and cross-sectional area of the bony island of LSCC. We evaluated the correlation between LSCC bone island width or cross-sectional area and hearing level in all cases using Pearson correlation co-efficients. In addition, we compared hearing levels among the patient group with normal-sized LSCC (>=mean-SD), small LSCC (hearing level (P>0.05). No significant difference in hearing levels were found among groups of the normal-sized, small and very small LSCC (P>0.05). Conclusion: We conclude that there is no correlation between isolated small LSCC and SNHL

  14. Alteration of frequency range for binaural beats in acute low-tone hearing loss.

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    Karino, Shotaro; Yamasoba, Tatsuya; Ito, Ken; Kaga, Kimitaka

    2005-01-01

    The effect of acute low-tone sensorineural hearing loss (ALHL) on the interaural frequency difference (IFD) required for perception of binaural beats (BBs) was investigated in 12 patients with unilateral ALHL and 7 patients in whom ALHL had lessened. A continuous pure tone of 30 dB sensation level at 250 Hz was presented to the contralateral, normal-hearing ear. The presence of BBs was determined by a subjective yes-no procedure as the frequency of a loudness-balanced test tone was gradually adjusted around 250 Hz in the affected ear. The frequency range in which no BBs were perceived (FRNB) was significantly wider in the patients with ALHL than in the controls, and FRNBs became narrower in the recovered ALHL group. Specifically, detection of slow BBs with a small IFD was impaired in this limited (10 s) observation period. The significant correlation between the hearing level at 250 Hz and FRNBs suggests that FRNBs represent the degree of cochlear damage caused by ALHL.

  15. Inter-trial coherence as a marker of cortical phase synchrony in children with sensorineural hearing loss and auditory neuropathy spectrum disorder fitted with hearing aids and cochlear implants

    Science.gov (United States)

    Nash-Kille, Amy; Sharma, Anu

    2014-01-01

    Objective Although brainstem dys-synchrony is a hallmark of children with auditory neuropathy spectrum disorder (ANSD), little is known about how the lack of neural synchrony manifests at more central levels. We used time-frequency single-trial EEG analyses (i.e., inter-trial coherence; ITC), to examine cortical phase synchrony in children with normal hearing (NH), sensorineural hearing loss (SNHL) and ANSD. Methods Single trial time-frequency analyses were performed on cortical auditory evoked responses from 41 NH children, 91 children with ANSD and 50 children with SNHL. The latter two groups included children who received intervention via hearing aids and cochlear implants. ITC measures were compared between groups as a function of hearing loss, intervention type, and cortical maturational status. Results In children with SNHL, ITC decreased as severity of hearing loss increased. Children with ANSD revealed lower levels of ITC relative to children with NH or SNHL, regardless of intervention. Children with ANSD who received cochlear implants showed significant improvements in ITC with increasing experience with their implants. Conclusions Cortical phase coherence is significantly reduced as a result of both severe-to-profound SNHL and ANSD. Significance ITC provides a window into the brain oscillations underlying the averaged cortical auditory evoked response. Our results provide a first description of deficits in cortical phase synchrony in children with SNHL and ANSD. PMID:24360131

  16. Amplified hearing device use in acute care settings for patients with hearing loss: A feasibility study.

    Science.gov (United States)

    Kimball, Amber R; Roscigno, Cecelia I; Jenerette, Coretta M; Hughart, Kimberly M; Jenkins, Wendy W; Hsu, Wesley

    2017-11-10

    The objective of this study was to assess the feasibility of using amplified hearing devices (AHD) in acute care settings for patients with hearing loss. Secondary objectives include patient and nurse satisfaction, and nursing perceived productivity. Twenty-five adult hard of hearing patients and 15 nurses were evaluated. Patients with a perceived hearing handicap were identified through the Hearing Handicap for the Elderly Screening Version. Patient and staff nurse surveys were used to assess for satisfaction with using the AHD. Nurses were surveyed to evaluate whether they felt the AHD made patient communication more efficient and effective. Twenty-four patients expressed satisfaction with the AHD and would use it in future hospitalizations. Nurses also reported satisfaction, perceived improvement of patient communication and decreased time spent communicating with patients. Results demonstrate the feasibility of using an AHD in acute care inpatient settings where elderly hard of hearing patients are common. Copyright © 2017 Elsevier Inc. All rights reserved.

  17. Detection of Perinatal Cytomegalovirus Infection and Sensorineural Hearing Loss in Belgian Infants by Measurement of Automated Auditory Brainstem Response▿

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    Verbeeck, Jannick; Van Kerschaver, Erwin; Wollants, Elke; Beuselinck, Kurt; Stappaerts, Luc; Van Ranst, Marc

    2008-01-01

    Since auditory disability causes serious problems in the development of speech and in the total development of a child, it is crucial to diagnose possible hearing impairment as soon as possible after birth. This study evaluates the neonatal hearing screening program in Flanders, Belgium. The auditory ability of 118,438 babies was tested using the automated auditory brainstem response. We selected 194 babies with indicative hearing impairment and 332 matched controls to investigate the associa...

  18. Biochemical alteration in children with idiopathic nephrotic syndrome associated with an increased risk of sensorineural hearing loss; additional insights in cochlear renal relationship.

    Science.gov (United States)

    El Mashad, Ghada Mohamed; Abo El Fotoh, Wafaa Moustafa M; Zein El Abedein, Ahmed Mahmoud; Abd El Sadek, Fatma Abd El Raoof

    2017-06-01

    Children with Idiopathic Nephrotic Syndrome (INS) are at risk of hearing loss due to the adverse impact of medications and related immunological and genetic factors on both cochlea and kidney. So this work was planned to evaluate hearing status in children with INS and to clarify the possible associated risk factors by interpreting the clinical and laboratory profiles of those children. Ninety children with INS aged 5-14 years [30 patients with steroid-sensitive nephrotic syndrome (SSNS), 30 patients with steroid dependent/frequently relapsing nephrotic syndrome (SDNS/FRNS), and 30 patients with steroid-resistant nephrotic syndrome (SRNS)], and 90 age and sex matched normal controls were enrolled into this study. Laboratory measurements of serum calcium, creatinine, cholesterol, blood urea and other relevant investigations were done. Pure tone audiometry was done with the sensory-neural hearing loss (SNHL) diagnosed when the level bone conduction was >20 dB and the difference in air to the bone gap was children with INS had SNHL, mostly of mild degree HL and primarily occurred at the lower frequencies. A highly significant statistical difference between controls and various types of nephrotic syndrome regarding pure tone audiometry measurements at frequencies 250, 500, 1000 Hz, whereas insignificant difference interpreting pure tone audiometry measurements in 2000, 4000 and 8000 Hz. Children with different phenotypes of nephrotic syndrome are at risk of sensorineural hearing impairment. The hazards associated with this impairment were higher blood pressure, hypercholesterolemia, hypoalbuminemia, and hypocalcemia. Copyright © 2017 Elsevier B.V. All rights reserved.

  19. Neutrophil-to-Lymphocyte Ratio and Platelet-to-Lymphocyte Ratio: Novel Markers for Diagnosis and Prognosis in Patients with Idiopathic Sudden Sensorineural Hearing Loss

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    Young Joon Seo

    2014-01-01

    Full Text Available Background. We aim to provide useful evidence about the association of neutrophil-to-lymphocyte ratio (NLR and platelet-to-lymphocyte ratio (PLR with idiopathic sudden sensorineural hearing loss (ISSNHL and its possibility of emerging as a cheap, reliable, and independent prognostic marker of ISSNHL. Methods. 348 patients diagnosed with ISSNHL were included in our retrospective data analysis. Blood samples and the hearing assessments of the patients were carried out. Then, the patients were divided into 2 groups as “recovered” and “unrecovered” according to their response to the treatment. Results. Both mean NLR and PLR values of the ISSNHL patients were significantly higher than the control group (both P<0.001. The NLR value was 5.98±4.22 in the unrecovered group and 3.50±3.38 in the recovered group (P<0.001. After adjustment in a binary logistic regression model, only NLR value was associated with the recovery of ISSNHL (P=0.001. Discussion. We demonstrated for the first time that NLR and PLR values were significantly high in ISSNHL patients. Also the NLR level might be taken into account as a novel potential marker to predict the patients’ prognosis in terms of recovery.

  20. Exogenous IL-4-Expressing Bone Marrow Mesenchymal Stem Cells for the Treatment of Autoimmune Sensorineural Hearing Loss in a Guinea Pig Model

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    Chang-qiang Tan

    2014-01-01

    Full Text Available Bone marrow mesenchymal stem cells (BMSCs expressing recombinant IL-4 have the potential to remediate inflammatory diseases. We thus investigated whether BMSCs expressing exogenous IL-4 could alleviate autoimmune sensorineural hearing loss. BMSCs isolated from guinea pigs were transfected with recombinant lentivirus expressing IL-4. A total of 33 animals were divided into three groups. Group A received scala tympani injection of IL-4-expressing BMSCs, and Group B received control vector-expressing BMSCs, and Group C received phosphate-buffered saline. The distribution of implanted BMSCs in the inner ears was assessed by immunohistochemistry and fluorescence microscopy. Auditory brain-stem response (ABR was monitored to evaluate the auditory changes. Following BMSCs transplantation, the threshold levels of ABR wave III decreased in Groups A and B and significant differences were observed between these two groups P<0.05. Transplanted BMSCs distributed in the scala tympani and scala vestibuli. In some ears with hearing loss, there was a decrease in the number of spiral ganglion cells and varying degrees of endolymphatic hydrops or floccule. Following transplantation, the lentivirus-infected BMSCs migrated to the inner ear and produced IL-4. Our results demonstrate that, upon transplantation, BMSCs and BMSCs expressing recombinant IL-4 have the ability to remediate the inflammatory injury in autoimmune inner ear diseases.

  1. Comparison of speech discrimination in noise and directional hearing with 2 different sound processors of a bone-anchored hearing system in adults with unilateral severe or profound sensorineural hearing loss.

    Science.gov (United States)

    Wesarg, Thomas; Aschendorff, Antje; Laszig, Roland; Beck, Rainer; Schild, Christian; Hassepass, Frederike; Kroeger, Stefanie; Hocke, Thomas; Arndt, Susan

    2013-08-01

    To evaluate and compare the benefit of a bone-anchored hearing implant with 2 different sound processors in adult patients with unilateral severe to profound sensorineural hearing loss (UHL). Prospective crossover design. Tertiary referral center. Eleven adults with UHL and normal hearing in the contralateral ear were assigned to 2 groups. All subjects were unilaterally implanted with a bone-anchored hearing implant and were initially fitted with 2 different sound processors (SP-1 and SP-2). SP-1 is a multichannel device equipped with an omnidirectional microphone and relatively simple digital signal-processing technology and provides a user-adjustable overall gain and tone control with compression limiting. SP-2 is a fully channel-by-channel programmable device, which can be set with nonlinear dynamic range compression or linear amplification. In addition, SP-2 features automatic noise management, an automatic multichannel directional microphone, microphone position compensation, and an implementation of prescription rules for different types of hearing losses, one of them unilateral deafness. After at least 1-month use of the initial processor, both groups were fitted with the alternative processor. Speech discrimination in noise and localization tests were performed at baseline visit before surgery, after at least 1-month use of the initial processor, and after at least 2-week use of the alternative processor. Relative to unaided baseline, SP-2 enabled significantly better overall speech discrimination results, whereas there was no overall improvement with SP-1. There was no difference in speech discrimination between SP-1 and SP-2 in all spatial settings. Sound localization was comparably poor at baseline and with both processors but significantly better than chance level for all 3 conditions. Patients with UHL have an overall objective benefit for speech discrimination in noise using a bone-anchored hearing implant with SP-2. In contrast, there is no overall

  2. [Combined treatment supported by piracetam and/or acyclovir in idiopathic sudden sensorineural hearing loss: experience with 81 cases].

    Science.gov (United States)

    Karakurt, Süleyman Emre; Ozkul, Mehmet Doğan; Cukurova, Ibrahim; Demirhan, Erhan; Yiğitbaşi, Orhan Gazi

    2009-01-01

    To investigate the efficiency of piracetam and acyclovir in treating sudden hearing loss. Eightyone patients (44 males, 37 females; mean age 40.4 year; range 18 to 72 years) who had treatment between January 2002 and December 2006 with diagnosis of idiopathic sudden hearing loss were evaluated retrospectively. These patients were divided into four groups according to the treatment they received. The patients who had combined treatment constituted the first group; those who had combined treatment and piracetam the second; those who had combined treatment and acyclovir the third; those who had combined treatment, acyclovir, and piracetam the fourth group. For the four treatment groups, in the pre-and post-treatment (10th day) evaluation of the treatment efficiency made by calculation of the hearing thresholds in 250-8000 Hz frequencies, no significant difference between the groups was determined (p>0.05). No additional benefit was obtained with acyclovir and piracetam in treatment.

  3. Comparação entre critérios de recuperação auditiva na perda neurossensorial súbita Comparison of hearing recovery criteria in sudden sensorineural hearing loss

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    Daniel Paganini Inoue

    2012-06-01

    Full Text Available Inúmeros métodos de análise da recuperação auditiva na perda auditiva neurossensorial súbita idiopática (PANSI dificultam a comparação adequada dos diversos tratamentos encontrados na Literatura. OBJETIVO: Comparar diversos critérios de recuperação auditiva na PANSI, baseados na Literatura. MATERIAL E MÉTODO: Foi realizado um estudo clínico observacional de coorte, a partir de um protocolo prospectivo, em pacientes com PANSI, atendidos entre 2000 e 2010. Foram comparados cinco critérios de recuperação auditiva significativa e quatro critérios para recuperação completa, pela audiometria tonal, por meio de teste não paramétrico e de comparações múltiplas, ambos com um nível de significância de 5%. Após determinação do critério de recuperação auditiva mais rígido, foram adicionados parâmetros da audiometria vocal. RESULTADOS: Houve diferença significativa, entre esses critérios (p The countless methods available to analyze hearing recovery in idiopathic sudden sensorineural hearing loss (ISSHL cases hinder the comparison of the various treatments found in the literature. OBJECTIVE: This paper aims to compare the different criteria for hearing recovery in ISSHL found in the literature. MATERIALS AND METHODS: This is an observational clinical cohort study from a prospective protocol in patients with ISSHL, treated between 2000 and 2010. Five criteria were considered for significant hearing recovery and four for complete recovery by pure tone audiometry, using non-parametric tests and multiple comparisons at a significance level of 5%. After determining the stricter criteria for hearing recovery, vocal audiometry parameters were added. RESULTS: There was a significant difference between the criteria (p < 0.001 as they were analyzed together. Mild auditory recovery occurred in only 35 (27.6% patients. When speech audiometry was added, only 34 patients (26.8% showed significant improvement. CONCLUSIONS: There is a

  4. A Sound Therapy-Based Intervention to Expand the Auditory Dynamic Range for Loudness among Persons with Sensorineural Hearing Losses: A Randomized Placebo-Controlled Clinical Trial

    Science.gov (United States)

    Formby, Craig; Hawley, Monica L.; Sherlock, LaGuinn P.; Gold, Susan; Payne, JoAnne; Brooks, Rebecca; Parton, Jason M.; Juneau, Roger; Desporte, Edward J.; Siegle, Gregory R.

    2015-01-01

    The primary aim of this research was to evaluate the validity, efficacy, and generalization of principles underlying a sound therapy–based treatment for promoting expansion of the auditory dynamic range (DR) for loudness. The basic sound therapy principles, originally devised for treatment of hyperacusis among patients with tinnitus, were evaluated in this study in a target sample of unsuccessfully fit and/or problematic prospective hearing aid users with diminished DRs (owing to their elevated audiometric thresholds and reduced sound tolerance). Secondary aims included: (1) delineation of the treatment contributions from the counseling and sound therapy components to the full-treatment protocol and, in turn, the isolated treatment effects from each of these individual components to intervention success; and (2) characterization of the respective dynamics for full, partial, and control treatments. Thirty-six participants with bilateral sensorineural hearing losses and reduced DRs, which affected their actual or perceived ability to use hearing aids, were enrolled in and completed a placebo-controlled (for sound therapy) randomized clinical trial. The 2 × 2 factorial trial design was implemented with or without various assignments of counseling and sound therapy. Specifically, participants were assigned randomly to one of four treatment groups (nine participants per group), including: (1) group 1—full treatment achieved with scripted counseling plus sound therapy implemented with binaural sound generators; (2) group 2—partial treatment achieved with counseling and placebo sound generators (PSGs); (3) group 3—partial treatment achieved with binaural sound generators alone; and (4) group 4—a neutral control treatment implemented with the PSGs alone. Repeated measurements of categorical loudness judgments served as the primary outcome measure. The full-treatment categorical-loudness judgments for group 1, measured at treatment termination, were

  5. Association of the 4 g/5 g polymorphism of plasminogen activator inhibitor-1 gene with sudden sensorineural hearing loss. A case control study

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    Cho Seong

    2012-06-01

    Full Text Available Abstract Background The 5 G/5 G genotype of PAI-1 polymorphism is linked to decreased plasminogen activator inhibitor-1 (PAI-1 levels and it has been suggested that lower PAI-1 levels may provide protective effects on inflammation, local microcirculatory disturbance, and fibrotic changes, which are likely associated with development of sudden sensorineural hearing loss (SSNHL. Methods The association of the 4 G/5 G PAI-1 polymorphism with the development and clinical outcome of SSNHL is evaluated via a case control study. 103 patients with SSNHL and 113 age and sex-matched controls were enrolled at University of Ferrara, Italy and hearing loss outcome was measured at least 3 months after the onset of hearing loss. DNA was isolated from peripheral blood using the QIAamp kit and the 4 G/5 G polymorphism in the −675 promoter region was genotyped with an allele-specific PCR. Genotype distribution was tested in patients and compared to controls by chi-square and odd-ratio analysis. The codominant and recessive models were used for the multiple logistic regression analyses of the PAI-1 gene allele. Results In this population, 5 G/5 G genotype had a two-time lower frequency in SSNHL patients compared to healthy controls (15.5% vs 30.1% and was associated with decreased odds compared to 4 G/5 G genotype (OR 0.37, 95% CI 0.19-0.75, p = 0.005. In addition, the patients with 5 G/5 G genotype showed a trend of more than 2 times higher ratio of hearing recovery (> 20 dB after systemic corticosteroid treatment compared to 4 G/5 G genotype (OR 2.3, 95% CI 0.32 - 16.83, p = 0.39, suggesting a better clinical outcome. Conclusions The 5 G/5 G genotype of PAI-1 may be associated with a reduced risk of SSNHL in the Italian population.

  6. A novel WFS1 mutation in a family with dominant low frequency sensorineural hearing loss with normal VEMP and EcochG findings.

    Science.gov (United States)

    Bramhall, Naomi F; Kallman, Jeremy C; Verrall, Aimee M; Street, Valerie A

    2008-06-02

    Low frequency sensorineural hearing loss (LFSNHL) is an uncommon clinical finding. Mutations within three different identified genes (DIAPH1, MYO7A, and WFS1) are known to cause LFSNHL. The majority of hereditary LFSNHL is associated with heterozygous mutations in the WFS1 gene (wolframin protein). The goal of this study was to use genetic analysis to determine if a small American family's hereditary LFSNHL is linked to a mutation in the WFS1 gene and to use VEMP and EcochG testing to further characterize the family's audiovestibular phenotype. The clinical phenotype of the American family was characterized by audiologic testing, vestibular evoked myogenic potentials (VEMP), and electrocochleography (EcochG) evaluation. Genetic characterization was performed by microsatellite analysis and direct sequencing of WFS1 for mutation detection. Sequence analysis of the WFS1 gene revealed a novel heterozygous mutation at c.2054G>C predicting a p.R685P amino acid substitution in wolframin. The c.2054G>C mutation segregates faithfully with hearing loss in the family and is absent in 230 control chromosomes. The p.R685 residue is located within the hydrophilic C-terminus of wolframin and is conserved across species. The VEMP and EcochG findings were normal in individuals segregating the WFS1 c.2054G>C mutation. We discovered a novel heterozygous missense mutation in exon 8 of WFS1 predicting a p.R685P amino acid substitution that is likely to underlie the LFSNHL phenotype in the American family. For the first time, we describe VEMP and EcochG findings for individuals segregating a heterozygous WFS1 mutation.

  7. Identification of two novel missense WFS1 mutations, H696Y and R703H, in patients with non-syndromic low-frequency sensorineural hearing loss.

    Science.gov (United States)

    Sun, Yi; Cheng, Jing; Lu, Yanping; Li, Jianzhong; Lu, Yu; Jin, Zhanguo; Dai, Pu; Wang, Rongguang; Yuan, Huijun

    2011-02-01

    Non-syndromic low-frequency sensorineural hearing loss (LFSNHL) is an unusual type of hearing loss in which frequencies ≤2000 Hz predominantly are affected. To date, different mutations in two genes, DIAPH1 and WFS1, have been found to be associated with LFSNHL. Here, we report a five-generation Chinese family with postlingual and progressive LFSNHL. We mapped the disease locus to a 2.5 Mb region on chromosome 4p16 between markers SNP_A-2167174 and D4S431, overlapping with the DFNA6/14/38 locus. Sequencing of candidate gene revealed a heterozygous c.2086C>T substitution in exon 8 of WFS1, leading to p.H696Y substitution at the C-terminus of Wolframin (WFS1). In addition, we performed mutational screening of WFS1 in 37 sporadic patients, 7-50 years of age, with LFSNHL. We detected a heterozygous c.2108G>A substitution in exon 8 of WFS1, leading to p.R703H substitution in a patient. The H696 and R703 in WFS1 are highly conserved across species, including human, orangutan, rat, mouse, and frog (Xenopus). Sequence analysis demonstrated the absence of c.2086C>T or c.2108G>A substitutions in the WFS1 genes among 200 unrelated control subjects of Chinese background, supporting the hypothesis that they represent causative mutations, and not rare polymorphisms. Our data provide additional molecular and clinical information for establishing a better genotype-phenotype correlation for LFSNHL. Copyright © 2011. Published by Elsevier Ltd.

  8. A novel WFS1 mutation in a family with dominant low frequency sensorineural hearing loss with normal VEMP and EcochG findings

    Directory of Open Access Journals (Sweden)

    Verrall Aimee M

    2008-06-01

    Full Text Available Abstract Background Low frequency sensorineural hearing loss (LFSNHL is an uncommon clinical finding. Mutations within three different identified genes (DIAPH1, MYO7A, and WFS1 are known to cause LFSNHL. The majority of hereditary LFSNHL is associated with heterozygous mutations in the WFS1 gene (wolframin protein. The goal of this study was to use genetic analysis to determine if a small American family's hereditary LFSNHL is linked to a mutation in the WFS1 gene and to use VEMP and EcochG testing to further characterize the family's audiovestibular phenotype. Methods The clinical phenotype of the American family was characterized by audiologic testing, vestibular evoked myogenic potentials (VEMP, and electrocochleography (EcochG evaluation. Genetic characterization was performed by microsatellite analysis and direct sequencing of WFS1 for mutation detection. Results Sequence analysis of the WFS1 gene revealed a novel heterozygous mutation at c.2054G>C predicting a p.R685P amino acid substitution in wolframin. The c.2054G>C mutation segregates faithfully with hearing loss in the family and is absent in 230 control chromosomes. The p.R685 residue is located within the hydrophilic C-terminus of wolframin and is conserved across species. The VEMP and EcochG findings were normal in individuals segregating the WFS1 c.2054G>C mutation. Conclusion We discovered a novel heterozygous missense mutation in exon 8 of WFS1 predicting a p.R685P amino acid substitution that is likely to underlie the LFSNHL phenotype in the American family. For the first time, we describe VEMP and EcochG findings for individuals segregating a heterozygous WFS1 mutation.

  9. The efficacy and safety of systemic injection of Ginkgo biloba extract, EGb761, in idiopathic sudden sensorineural hearing loss: a randomized placebo-controlled clinical trial.

    Science.gov (United States)

    Koo, Ja-Won; Chang, Mun Young; Yun, Sung-Cheol; Kim, Tae Su; Kong, Soo-Keun; Chung, Jong Woo; Goh, Eui-Kyung

    2016-09-01

    Steroids are currently the most frequently accepted agents for idiopathic sudden sensorineural hearing loss (ISSNHL). However, the therapeutic effect of steroids is not always satisfactory. In this pilot study, we evaluated whether systemic treatment with Ginkgo biloba extract (EGb761) has an additive therapeutic effect in patients receiving a systemic steroid due to ISSNHL. A multicenter, randomized, double-blind clinical trial was performed. Fifty-six patients with ISSNHL were allocated to either EGb761 or placebo. In both groups, methylprednisolone was administered for 14 days. EGb761 was infused intravenously for 5 days in the EGb761 group, while the same amount of normal saline was infused in the placebo group. For the efficacy evaluation, pure-tone audiometry, speech audiometry, tinnitus handicap inventory (THI) and short form-36 health (SF-36) survey outcomes were obtained before administration and on days 3, 5, 14 and 28 of administration. Twenty-four patients in each group completed the study protocol. There was no difference in hearing loss between the two groups before treatment. At day 28, air conduction threshold values in the placebo and EGb761 groups were 34.63 ± 28.90 and 23.84 ± 25.42 dB, respectively (p = 0.082). Speech discrimination scores in the placebo and EGb761 groups were 69.17 ± 40.89 and 87.48 ± 28.65 %, respectively (p = 0.050). THI and SF-36 scores in the placebo and EGb761 groups were similar. Although a combination of steroid and EGb761 for initial treatment did not show better pure tone threshold, compared with steroid alone, speech discrimination was significantly improved in combination therapy. Further studies will be needed to know if addition of EGb761 actually improves the outcome of ISSNHL treatment.

  10. Open-Fit Domes and Children with Bilateral High-Frequency Sensorineural Hearing Loss: Benefits and Outcomes.

    Science.gov (United States)

    Johnstone, Patti M; Yeager, Kelly R; Pomeroy, Marnie L; Hawk, Nicole

    2018-04-01

    Open-fit domes (OFDs) coupled with behind-the-ear (BTE) hearing aids were designed for adult listeners with moderate-to-severe bilateral high-frequency hearing loss (BHFL) with little to no concurrent loss in the lower frequencies. Adult research shows that BHFL degrades sound localization accuracy (SLA) and that BTE hearing aids with conventional earmolds (CEs) make matters worse. In contrast, research has shown that OFDs enhance spatial hearing percepts in adults with BHFL. Although the benefits of OFDs have been studied in adults with BHFL, no published studies to date have investigated the use of OFDs in children with the same hearing loss configuration. This study seeks to use SLA measurements to assess efficacy of bilateral OFDs in children with BHFL. To measure SLA in children with BHFL to determine the extent to which hearing loss, age, duration of CE use, and OFDs affect localization accuracy. A within-participant experimental design using repeated measures was used to determine the effect of OFDs on localization accuracy in children with BHFL. A between-participant experimental design was used to compare localization accuracy between children with BHFL and age-matched controls with normal hearing (NH). Eighteen children with BHFL who used CE and 18 age-matched NH controls. Children in both groups were divided into two age groups: older children (10-16 yr) and younger children (6-9 yr). All testing was done in a sound-treated booth with a horizontal array of 15 loudspeakers (radius of 1 m). The stimulus was a spondee word, "baseball": the level averaged 60 dB SPL and randomly roved (±8 dB). Each child was asked to identify the location of a sound source. Localization error was calculated across the loudspeaker array for each listening condition. A significant interaction was found between immediate benefit from OFD and duration of CE usage. Longer CE usage was associated with degraded localization accuracy using OFDs. Regardless of chronological age

  11. Interstitial keratitis and sensorineural hearing loss as a manifestation of rheumatoid arthritis: clinical lessons from a rare complication

    OpenAIRE

    Lee, Lennard YW; Akhtar, Mohammed Majid; Kirresh, Othman; Gibson, Terence

    2012-01-01

    Cogan's syndrome or non-syphilitic interstitial keratitis with vestibule-auditory dysfunction is a serious and under-recognised complication of rheumatoid arthritis. It is an autoimmune condition characterised by inflammatory infiltrates on the cornea and extensive vestibulocochlear damage. If left untreated, patients progress to develop profound hearing loss. We present a case that was incorrectly diagnosed and treated as conjunctivitis by several emergency departments prior to being correct...

  12. Effects of hearing aids in the balance, quality of life and fear to fall in elderly people with sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Lacerda, Clara Fonseca

    2012-01-01

    Full Text Available Introduction: The aging process provokes structural modifications and functional to it greets, compromising the postural control and central processing. Studies have boarded the necessity to identify to the harmful factors of risk to aged the auditory health and security in stricken aged by auditory deficits and with alterations of balance. Objective: To evaluate the effect of auditory prosthesis in the quality of life, the balance and the fear of fall in aged with bilateral auditory loss. Method: Carried through clinical and experimental study with 56 aged ones with sensorineural auditory loss, submitted to the use of auditory prosthesis of individual sonorous amplification (AASI. The aged ones had answered to the questionnaires of quality of life Short Form Health Survey (SF-36, Falls Efficacy International Scale- (FES-I and the test of Berg Balance Scale (BBS. After 4 months, the aged ones that they adapted to the use of the AASI had been reevaluated. Results: It had 50% of adaptation of the aged ones to the AASI. It was observed that the masculine sex had greater difficulty in adapting to the auditory device and that the variable age, degree of loss, presence of humming and vertigo had not intervened with the adaptation to auditory prosthesis. It had improvement of the quality of life in the dominance of the State General Health (EGS and Functional Capacity (CF and of the humming, as well as the increase of the auto-confidence after adaptation of auditory prosthesis. Conclusion: The use of auditory prosthesis provided the improvement of the domains of the quality of life, what it reflected consequently in one better auto-confidence and in the long run in the reduction of the fear of fall in aged with sensorineural auditory loss.

  13. Effects of hearing aids in the balance, quality of life and fear to fall in elderly people with sensorineural hearing loss.

    Science.gov (United States)

    Lacerda, Clara Fonseca; Silva, Luciana Oliveira E; de Tavares Canto, Roberto Sérgio; Cheik, Nadia Carla

    2012-04-01

     The aging process provokes structural modifications and functional to it greets, compromising the postural control and central processing. Studies have boarded the necessity to identify to the harmful factors of risk to aged the auditory health and security in stricken aged by auditory deficits and with alterations of balance.  To evaluate the effect of auditory prosthesis in the quality of life, the balance and the fear of fall in aged with bilateral auditory loss.  Carried through clinical and experimental study with 56 aged ones with sensorineural auditory loss, submitted to the use of auditory prosthesis of individual sonorous amplification (AASI). The aged ones had answered to the questionnaires of quality of life Short Form Health Survey (SF-36), Falls Efficacy International Scale- (FES-I) and the test of Berg Balance Scale (BBS). After 4 months, the aged ones that they adapted to the use of the AASI had been reevaluated.  It had 50% of adaptation of the aged ones to the AASI. It was observed that the masculine sex had greater difficulty in adapting to the auditory device and that the variable age, degree of loss, presence of humming and vertigo had not intervened with the adaptation to auditory prosthesis. It had improvement of the quality of life in the dominance of the State General Health (EGS) and Functional Capacity (CF) and of the humming, as well as the increase of the auto-confidence after adaptation of auditory prosthesis.  The use of auditory prosthesis provided the improvement of the domains of the quality of life, what it reflected consequently in one better auto-confidence and in the long run in the reduction of the fear of fall in aged with sensorineural auditory loss.

  14. Idiopathic Sudden Sensorineural Hearing Loss: Average Time Elapsed Before Presentation to the Otolaryngologist and Effectiveness of Oral and/or Intratympanic Steroids in Late Presentations.

    Science.gov (United States)

    Anyah, Anwuli; Mistry, Devin; Kevern, Erin; Markiewicz, Kenneth

    2017-12-14

    Objectives To determine how long after symptom onset that the average patient with an idiopathic sudden sensorineural hearing loss (ISSNHL) presents to the otolaryngology clinic.  In late presentations, to determine the time to presentation cutoff after which intervention may not be effective.  To evaluate the effectiveness of oral steroids versus a combination of oral and intratympanic steroid therapy in late presentations of ISSNHL.  Methods and procedures Sixty-four patients met inclusion criteria after chart review of 2,037 patients seen at Metro Health Hospital from 2006 to 2016 for sensorineural hearing loss. All sixty-four patients were used to calculate the average time to presentation, but only 40 were included to evaluate treatment efficacy because 24 were lost to follow-up or declined treatment. Audiograms were analyzed for baseline status and response to treatment. Therapy was either oral steroids or intratympanic (IT) steroids. Thirty-nine of the 40 treated patients received oral steroid therapy. Eighteen of these 39 patients received both oral and IT steroids. One patient received IT steroids only.  Results For all 64 patients in the study, the average time to presentation was 55 days, ranging from one day to 240 days. Data for 32 of the 40 treated patients were analyzed. These patients were further divided into smaller groups: Group 1 (N = 11) - treatment within seven days of symptom onset, Group 2 (N = 17) - time to treatment greater than seven days but less than 90 days of symptom onset, and Group 3 (N = 4) - greater than 90 days of symptom onset. In Group 2, there was a significant improvement in pure tone average (P-value: 0.005). Forty-seven percent of patients in this group had objective treatment response utilizing Wilson's criteria. Two patients had a complete recovery and six had a partial recovery. Hearing gains ranged from 10 dB (decibels) to 23 dB. Sixty-three percent of patients with objective improvement also

  15. OI Issues: Hearing Loss

    Science.gov (United States)

    ... loss can occur at any age, including childhood. Conductive Hearing Loss : Usually results from a physical problem in the ... This type of test can determine if the hearing loss is conductive or sensorineural. Adults who have an identified hearing ...

  16. Nontumorous enlargement of the internal auditory canal. A risk factor for sensorineural hearing loss? A high resolution CT-study

    Energy Technology Data Exchange (ETDEWEB)

    Stimmer, H.; Rummeny, E.J. [Technical University Munich, Klinikum rechts der Isar (Germany). Dept. of Radiology; Niedermeyer, H.P. [Technical University Munich, Klinikum rechts der Isar (Germany). ENT-Clinic; Kehl, V. [Technical University Munich, Klinikum rechts der Isar (Germany). Inst. for Medical Statistics and Epidemiology

    2015-06-15

    First aim of the study was to define normal shape and diameter of the internal auditory canal (IAC). In the second part the clinical relevance of IAC-enlargement was analyzed, considering also lesions of the subtle structures at the fundus of the internal auditory canal. 440 high resolution CT-scans of the temporal bone were used for retrospective analysis of the internal auditory canal and its fundus region. The mean value of the IAC diameter in axial and coronal plane was determined. In 20 of 440 patients IAC enlargement was found. In the group with pronounced enlargement (3fold SD) nearly all patients suffered from hearing impairment. In some of them we found structural abnormalities near the IAC fundus in the CSF/perilymph border zone. A new CT-based definition of normal shape and diameter of the internal auditory canal is presented. There is some evidence that a pathologic transmission of CSF-pressure in case of IAC-enlargement and/or abnormal fistulous communications could play an important role in the pathophysiology of hearing loss.

  17. Vestibular Evoked Myogenic Potential Produced by Bone-Conducted Stimuli: A Study on its Basics and Clinical Applications in Patients With Conductive and Sensorineural Hearing Loss and a Group With Vestibular Schawannoma

    Directory of Open Access Journals (Sweden)

    Parvane Mahdi

    2013-06-01

    Full Text Available Introduction: Vestibular evoked myogenic potential (VEMP has recently been broadly studied in vestibular disorders. As it is evoked by loud sound stimulation, even mild conductive hearing loss may affect VEMP results. Bone-conducted (BC stimulus is an alternative stimulation for evoking this response. This study aims to assess the characteristics of BC-VEMP in different groups of patients.   Materials and Methods: We performed a cross sectional analysis on 20 healthy volunteers with normal pure-tone audiometry as a control group; and on a group of patients consisted of 20 participants with conductive hearing loss, five with bilateral sensorineural hearing loss and four with vestibular schawannoma. AC and BC-VEMP were performed in all participants.   Results: In control group the VEMP responses to both kinds of stimuli had an acceptable morphology and consisted of p13 and n23 waves. Latency value of these main components in each type of stimulus was not significantly different (P>0.05. However, the mean amplitude was larger in BC modality than AC stimulation (P=0.025. In the group with conductive hearing loss, the VEMP response was absent in fifteen (46.87% of the 32 ears using the AC method, whereas all (100% displayed positive elicitability of VEMP by BC method. Normal VEMP responses in both stimuli were evoked in all patients with sensorineural hearing loss. In patients with unilateral vestibular schwannomas (VS, 2 (50.00% had neither AC-VEMP nor BC-VEMP. Conclusion:  Auditory stimuli delivered by bone conduction can evoke VEMP response. These responses are of vestibular origin and can be used in vestibular evaluation of patients with conductive hearing loss.

  18. Vestibular Evoked Myogenic Potential Produced by Bone-Conducted Stimuli: A Study on its Basics and Clinical Applications in Patients with Conductive and Sensorineural Hearing Loss and a Group with Vestibular Schawannoma.

    Science.gov (United States)

    Mahdi, Parvane; Amali, Amin; Pourbakht, Akram; Karimi Yazdi, Alireza; Bassam, Ali

    2013-06-01

    Vestibular evoked myogenic potential (VEMP) has recently been broadly studied in vestibular disorders. As it is evoked by loud sound stimulation, even mild conductive hearing loss may affect VEMP results. Bone-conducted (BC) stimulus is an alternative stimulation for evoking this response. This study aims to assess the characteristics of BC-VEMP in different groups of patients. We performed a cross sectional analysis on 20 healthy volunteers with normal pure-tone audiometry as a control group; and on a group of patients consisted of 20 participants with conductive hearing loss, five with bilateral sensorineural hearing loss and four with vestibular schawannoma. AC and BC-VEMP were performed in all participants. In control group the VEMP responses to both kinds of stimuli had an acceptable morphology and consisted of p13 and n23 waves. Latency value of these main components in each type of stimulus was not significantly different (P>0.05). However, the mean amplitude was larger in BC modality than AC stimulation (P=0.025). In the group with conductive hearing loss, the VEMP response was absent in fifteen (46.87%) of the 32 ears using the AC method, whereas all (100%) displayed positive elicitability of VEMP by BC method. Normal VEMP responses in both stimuli were evoked in all patients with sensorineural hearing loss. In patients with unilateral vestibular schwannomas (VS), 2 (50.00%) had neither AC-VEMP nor BC-VEMP. Auditory stimuli delivered by bone conduction can evoke VEMP response. These responses are of vestibular origin and can be used in vestibular evaluation of patients with conductive hearing loss.

  19. Protection, regeneration and replacement of hair cells in the cochlea: implications for the future treatment of sensorineural hearing loss.

    Science.gov (United States)

    Bodmer, Daniel

    2008-11-29

    In the last few years progress has been made in understanding basic mechanisms involved in damage to the inner ear and various potential therapeutic approaches have been developed. It was shown that hair cell loss mediated by noise or toxic drugs may be prevented by antioxidants, inhibitors of intracellular stress pathways and neurotrophic factors/neurotransmission blockers. Moreover, there is hope that once hair cells are lost, their regeneration can be induced or that stem cells can be used to build up new hair cells. However, although tremendous progress has been made, most of the concepts discussed in this review are still in the "animal stage" and it is difficult to predict which approach will finally enter clinical practice. In my opinion it is highly probable that some concepts of hair cell protection will enter clinical practice first, while others, such as the use of stem cells to restore hearing, are still far from clinical utility.

  20. Auditory processing deficits are sometimes necessary and sometimes sufficient for language difficulties in children: Evidence from mild to moderate sensorineural hearing loss.

    Science.gov (United States)

    Halliday, Lorna F; Tuomainen, Outi; Rosen, Stuart

    2017-09-01

    There is a general consensus that many children and adults with dyslexia and/or specific language impairment display deficits in auditory processing. However, how these deficits are related to developmental disorders of language is uncertain, and at least four categories of model have been proposed: single distal cause models, risk factor models, association models, and consequence models. This study used children with mild to moderate sensorineural hearing loss (MMHL) to investigate the link between auditory processing deficits and language disorders. We examined the auditory processing and language skills of 46, 8-16year-old children with MMHL and 44 age-matched typically developing controls. Auditory processing abilities were assessed using child-friendly psychophysical techniques in order to obtain discrimination thresholds. Stimuli incorporated three different timescales (µs, ms, s) and three different levels of complexity (simple nonspeech tones, complex nonspeech sounds, speech sounds), and tasks required discrimination of frequency or amplitude cues. Language abilities were assessed using a battery of standardised assessments of phonological processing, reading, vocabulary, and grammar. We found evidence that three different auditory processing abilities showed different relationships with language: Deficits in a general auditory processing component were necessary but not sufficient for language difficulties, and were consistent with a risk factor model; Deficits in slow-rate amplitude modulation (envelope) detection were sufficient but not necessary for language difficulties, and were consistent with either a single distal cause or a consequence model; And deficits in the discrimination of a single speech contrast (/bɑ/ vs /dɑ/) were neither necessary nor sufficient for language difficulties, and were consistent with an association model. Our findings suggest that different auditory processing deficits may constitute distinct and independent routes to

  1. Cost-effectiveness analysis of a non-contrast screening MRI protocol for vestibular schwannoma in patients with asymmetric sensorineural hearing loss

    Energy Technology Data Exchange (ETDEWEB)

    Crowson, Matthew G.; Rocke, Daniel J.; Kaylie, David M. [Duke University Medical Center, Division of Otolaryngology-Head and Neck Surgery, Durham, NC (United States); Hoang, Jenny K. [Duke University Medical Center, Department of Radiology, Durham, NC (United States); Weissman, Jane L. [Oregon Health Sciences University, Professor Emerita of Diagnostic Radiology, Portland, OR (United States)

    2017-08-15

    We aimed to determine if a non-contrast screening MRI is cost-effective compared to a full MRI protocol with contrast for the evaluation of vestibular schwannomas. A decision tree was constructed to evaluate full MRI and screening MRI strategies for patients with asymmetric sensorineural hearing loss. If a patient were to have a positive screening MRI, s/he received a full MRI. Vestibular schwannoma prevalence, MRI specificity and sensitivity, and gadolinium anaphylaxis incidence were obtained through literature review. Institutional charge data were obtained using representative patient cohorts. One-way and probabilistic sensitivity analyses were completed to determine CE model threshold points for MRI performance characteristics and charges. The mean charge for a full MRI with contrast was significantly higher than a screening MRI ($4089 ± 1086 versus $2872 ± 741; p < 0.05). The screening MRI protocol was more cost-effective than a full MRI protocol with a willingness-to-pay from $0 to 20,000 USD. Sensitivity analyses determined that the screening protocol dominated when the screening MRI charge was less than $4678, and the imaging specificity exceeded 78.2%. The screening MRI protocol also dominated when vestibular schwannoma prevalence was varied between 0 and 1000 in 10,000 people. A screening MRI protocol is more cost-effective than a full MRI with contrast in the diagnostic evaluation of a vestibular schwannoma. A screening MRI likely also confers benefits of shorter exam time and no contrast use. Further investigation is needed to confirm the relative performance of screening protocols for vestibular schwannomas. (orig.)

  2. Congenital malformations of the inner ear and the vestibulocochlear nerve in children with sensorineural hearing loss: evaluation with CT and MRI.

    Science.gov (United States)

    Westerhof, J P; Rademaker, J; Weber, B P; Becker, H

    2001-01-01

    The purpose of this work was to study the diagnostic value of CT and MRI in children with sensorineural hearing loss and to analyze anatomic abnormalities of the inner ear and the vestibulocochlear nerve in this patient group. We evaluated 42 inner ears in 21 children with congenital deafness who had congenital inner ear malformations and who were candidates for cochlear implants. All patients were studied with high resolution MR and helical CT examinations. The MR study included a T2-weighted 3D fast SE sequence. We describe and tabulate the anatomic abnormalities. Special attention was given to abnormalities of the vestibulocochlear nerve. The field of view in the plane according to the length axis of the internal auditory canal (IAC) was 4 cm. Additional continuous parasagittal reformations perpendicular to the length axis of the IAC were studied with a field of view of 3 cm. CT and MRI allowed accurate identification of malformations of the inner ear in children with congenital deafness. We identified 99 malformations, with a majority of patients demonstrating multiple abnormalities. Common imaging findings were Mondini abnormality and Mondini variants (12/42) and fusion of the lateral or superior semicircular canal with the vestibule (12/42). MRI demonstrated in 9 of 21 patients a rudimentary or absent vestibulocochlear nerve in the auditory canal. CT and MRI are important modalities to analyze the inner ear in children who are candidates for cochlear implants. MRI with an extremely small field of view should be used to study possible abnormalities of the vestibulocochlear nerves. This may alter clinical care and allow cochlear implant placement in patients whose electrodiagnostic studies suggest that the implant should not be performed. The detailed analysis of abnormalities of the inner ear might establish prognostic factors.

  3. The significance of a hypoplastic bony canal for the cochlear nerve in patients with sensorineural hearing loss: CT and MRI findings

    International Nuclear Information System (INIS)

    Choi, Yoon Jung; Park, Sang Yoo; Kim, Myung Soon; Sung, Ki Jun

    2004-01-01

    The purpose of this study is to evaluate the significance of the hypoplastic canal for the cochlear nerve in patients with sensorineural hearing loss (SNHL) and the relationship between the hypoplastic bony canal and aplasia or hypoplasia of the cochlear nerve. A retrospective review of high resolution temporal CT(HRCT) and MRI findings was conducted. The narrow bony canal of the cochlear nerve and the relative size of the internal auditory canal were correlated with the cochlear nerve deficiency on MRI. The comparative size of the component nerves (facial, cochlear, superior vestibular, inferior vestibular nerve), and the relative size of the internal auditory canal and the bony canal of the cochlear nerve were measured. The clinical history and the results of the clinical examination were reviewed for each patient. High resolution MRI showed aplasia of the common vestibulocochlear nerve in one patient and a deficiency of the cochlear nerve in 9 patients. These abnormalities occurred in association with a prominent narrowing of the canal for the cochlear nerve and a stenosis of the internal auditory canal, which was observed on temporal bone CT in 9 patients with congenital SNHL. Three patients had normal IAC, despite the presence of a hypoplastic cochlear nerve on the side on which they had SNHL. In one patient, the narrowing of the canal for the cochlear nerve and internal auditory canal were not found to be associated with acquired SNHL. The hypoplastic bony canal for the cochlear nerve might be more highly indicative of congenital cochlear nerve deficiency than that of the narrow internal auditory canal, and the position of the crista falciformis should also be carefully

  4. Generation of an induced pluripotent stem cell line from a 39-year-old female patient with severe-to-profound non-syndromic sensorineural hearing loss and a A1555G mutation in the mitochondrial MTRNR1 gene

    Directory of Open Access Journals (Sweden)

    Yu-Hung Hsu

    2017-12-01

    Full Text Available Sensorineural hearing loss (SNHL is a prevalent form of deafness commonly arising from damage to the cochlear sensory hair cells and degeneration of the spiral ganglion neurons. In this study, Sendai virus was used to generate an induced pluripotent stem cell (iPSC line from a 39-year-old female patient diagnosed with severe-to-profound, non-syndromic SNHL. The patient also carries a A1555G mutation in the mitochondrial 12S ribosome RNA gene (MTRNR1. This iPSC line was verified to express pluripotent markers, possess normal karyotype, harbor the specific mutation and demonstrated the capacity to differentiate into three germ layers.

  5. Systemic Polyarteritis Nodosa as the Cause of Sudden Onset Bilateral Sensorineural Hearing Loss Following Lassa Virus Infection

    Science.gov (United States)

    2016-07-05

    011-8281-z). 2. L. M. Branco, J. N. Grove, M. L. Boisen, J. G. Shaffer, A. Goba, M. Fullah, M. Momoh, D . S. Grant, R. F. Garry, Emerging trends in...Lassa fever: redefining the role of immunoglobulin M and inflammation in diagnosing acute infection. Virology journal 8, 478 (2011)10.1186/1743-422X-8...478). 3. . pp. http://www.who.int/mediacentre/factsheets/fs179/en/index.html. 4. D . Cummins, Rats, fever and sudden deafness in Sierra Leone

  6. Hearing Loss in Children: Types of Hearing Loss

    Science.gov (United States)

    ... hearing. There are four types of hearing loss: Conductive Hearing Loss Hearing loss caused by something that stops sounds ... Hearing Loss Hearing loss that includes both a conductive and a sensorineural hearing loss. Auditory Neuropathy Spectrum Disorder Hearing loss that occurs ...

  7. [Acute hearing loss and tinnitus caused by amplified recreational music].

    Science.gov (United States)

    Metternich, F U; Brusis, T

    1999-11-01

    Hearing loss resulting from exposure to permanent or repeated amplified music in professional musicians and music consumers is described in literature. The risk of hearing loss does not exist only after prolonged exposure to music. Short-term exposure to very high sound levels, for example in concerts, can also cause hearing loss and tinnitus. The retrospective study includes 24 patients who required rheologic therapy between 1994 and 1997 due to a music related acoustic trauma. The type, intensity, and length of music exposure as well as the distance and the position to the source of noise were examined. The type of hearing damage and its development during rheological treatment was studied by pure-tone audiometry. In the majority of examined patients (67%) the hearing loss developed on the basis of one-time exposure at a rock concert or pop concert, followed by hearing loss from attending discotheques (17%) or parties (12%), and music exposure from personal cassette players (4%). The majority of patients showed a maximum hearing loss of 40-60 dB (A) in a frequency between 3 kHz and 4 kHz. Pure-tone audiometry in 58% of the patients exhibited a unilateral threshold in a frequency between 3 kHz and 4 kHz combined with ipsilateral tinnitus of the same frequency. Twenty-one percent of the patients showed a symmetric bilateral threshold and tinnitus between 3 kHz and 4 kHz. In 8% there was a unilateral tinnitus, and in 13% a bilateral tinnitus without any hearing loss. All patients improved their hearing loss during rheologic treatment. Improvement in the tinnitus was only achieved in 33% of the examined cases. The risk of permanent hearing loss resulting from short-term exposure to amplified music is low compared to the risk of continuous tinnitus. Given the lack of acceptance of personal ear protectors, the risk of acute hearing damage due to amplified music could be reduced by avoiding the immediate proximity to the speakers.

  8. Acute unilateral hearing loss as an unusual presentation of cholesteatoma

    Directory of Open Access Journals (Sweden)

    Bickerton Richard C

    2005-09-01

    Full Text Available Abstract Background Cholesteatomas are epithelial cysts that contain desquamated keratin. Patients commonly present with progressive hearing loss and a chronically discharging ear. We report an unusual presentation of the disease with an acute hearing loss suffered immediately after prolonged use of a pneumatic drill. Case presentation A 41 year old man with no previous history of ear problems presented with a sudden loss of hearing in his right ear immediately following the prolonged use of a pneumatic drill on concrete. The cause was found to be a fractured long process of incus which had been eroded by the presence of an attic cholesteatoma. A tympanomastoidectomy and ossiculoplasty was performed with good result. Conclusion Cholesteatomas may be asymptomatic and insidious in their onset. This case illustrates the point that an indolent disease such as this may present in unusual ways and the clinician must always have a high index of suspicion combined with thorough assessment and examination of every patient.

  9. A comparison of inner ear imaging features at different time points of sudden sensorineural hearing loss with three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging.

    Science.gov (United States)

    Zhu, Honglei; Ou, Yongkang; Fu, Jia; Zhang, Ya; Xiong, Hao; Xu, Yaodong

    2015-10-01

    It has been reported that about half of patients with sudden sensorineural hearing loss (SSNHL) show high signals in the affected inner ear on three-dimensional, fluid-attenuated inversion recovery magnetic resonance imaging (3D-FLAIR MRI). These signals may reflect minor hemorrhage or an increased concentration of protein in the inner ear, which has passed through blood vessels with increased permeability. Our objective was to compare the positive ratio of the high signal in affected inner ears at different time points to determine the suitable imaging time point for 3D-FLAIR MRI in SSNHL. 3D-FLAIR MRI images were taken at three times, precontrast and approximately 10 min and 4 h after intravenous injection of a single dose of gadodiamide (Gd) (0.1 mmol/kg), in 46 patients with SNHL. We compared the positive findings of the high signals in the inner ear of patients with SNHL as well as the signal intensity ratio (SIR) between the affected cochleae and unaffected cochleae at three time points. The positive ratios of the high signals in the affected inner ear at the time points of precontrast and 10 min and 4 h after the intravenous Gd injection were 26.1, 32.6, and 41.3%, respectively. The high signal intensity ratios of affected inner ears at the three time points were 1.28, 1.31, and 1.48, respectively. The difference between the positive ratios precontrast and at 10 min after the intravenous Gd injection was statistically significant (P = 0.006); the differences between the positive ratios at 4 h after the intravenous Gd injection and precontrast and between the ratios at 4 h and 10 min after the intravenous Gd injection were not statistically significant. The time effects of the median value of SIR were not significant (P = 0.064). We do not recommend 4 h after intravenous Gd injection as a time point to image the inner ear in SNHL. We believe that imaging precontrast and at 10 min after the intravenous Gd injection are suitable time points.

  10. A case of bilateral sudden hearing loss and tinnitus after salicylate intoxication.

    Science.gov (United States)

    Kim, Sang Min; Jo, Joon-Man; Baek, Moo Jin; Jung, Kyu Hwan

    2013-04-01

    Salicylate, the active ingredient of aspirin can cause sensorineural hearing loss and tinnitus when plasma concentrations reach a critical level. The ototoxic mechanisms of salicylate remain unclear but hearing and tinnitus usually recovers a few days after intoxication. There have been few reports of salicylate-induced ototoxicity in Korea, and the majority is caused by a low dose of aspirin. Herein, we report a case of sudden hearing loss and tinnitus after acute salicylate intoxication and review recent updates on salicylate ototoxicity.

  11. Avaliação do Voice Handicap Index em pacientes com perda auditiva neurossensorial bilateral a partir de grau moderado Voice Handicap Index evaluation in patients with moderate to profound bilateral sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Felipe Barbosa Madeira

    2010-02-01

    Full Text Available A produção da voz e da fala é processo que envolve mecanismos reguladores dependentes da audição. As alterações vocais em pacientes com perda auditiva ainda não foram avaliadas quanto ao grau subjetivo de incapacidade que trazem a este grupo. OBJETIVO: Comparar os resultados do Voice Handicap Index (VHI em pacientes com e sem perda auditiva neurossensorial bilateral a partir de grau moderado. FORMA DE ESTUDO: Estudo transversal controlado. MATERIAL E MÉTODO: Foram avaliados 76 pacientes adultos (38 com e 38 sem perda auditiva entre 19 e 59 anos, atendidos em serviço terciário de otorrinolaringologia mediante preenchimento do VHI. RESULTADOS: A mediana da pontuação total do VHI obtida no grupo de pacientes e controle foi de 23,5 e 4,0, respectivamente (p = 0,000. Diferenças significativas entre os dois grupos também foram conseguidas nas medianas dos subitens funcional, físico e emocional do VHI (p = 0,000. CONCLUSÃO: Os resultados obtidos permitem verificar a maior desvantagem social e econômica conforme avaliada pelo VHI em pacientes com perda auditiva neurossensorial bilateral a partir de grau moderado.Voice and speech are regulated by hearing. Vocal disorders in patients with hearing loss have not been evaluated yet as to the subjective degree of disability they cause in this group. AIM: to compare the results of the Voice Handicap Index (VHI obtained for patients with normal hearing and moderate to profound bilateral sensorineural hearing loss. STUDY DESIGN: Controlled, cross-sectional. MATERIALS AND METHODS: A total of 76 adult patients being treated on a University Otolaryngology center were enrolled (38 with and 38 without hearing loss, ages ranging between 19 and 59 years, were asked to complete the Portuguese version of the VHI. RESULTS: Total VHI score median values obtained were 23.5 and 4.0 for the study and control groups, respectively (p = 0.000. Significant differences between the two groups were found for all

  12. What's Hearing Loss?

    Science.gov (United States)

    ... Loss There are a few different types of hearing loss: conductive , sensorineural , mixed (conductive and sensory combined), neural , and central . Conductive (say: kun- duk -tiv) hearing loss. This happens when there is a problem with ...

  13. Potencial evocado auditivo de tronco encefálico por via óssea em indivíduos com perda auditiva sensorioneural Brainstem auditory evoked potential in subjects with sensorineural hearing losses

    Directory of Open Access Journals (Sweden)

    Luciana Castelo Branco Camurça Fernandes

    2012-01-01

    Full Text Available OBJETIVO: caracterizar os resultados do PEATE por via óssea em indivíduos com perda auditiva sensorioneural leve, comparando esses dados com o grupo controle, formado por indivíduos audiologicamente normais. MÉTODO: a amostra foi constituída por 40 adultos, de ambos os sexos, com faixa etária de 18 a 55 anos, distribuídos em um grupo controle de 30 indivíduos com audição normal e um grupo estudo composto de 10 indivíduos com perda auditiva sensorioneural de grau leve. O PEATE foi realizado com equipamento EP15, da marca Interacoustics. O estímulo utilizado foi o clique com taxa de apresentação de 27,7/s, em um total de 2000 estímulos, com polaridade de rarefação por VA e alternada para VO e filtro passa-banda de 50Hz e 3000Hz. RESULTADOS: em indivíduos com perda sensorioneural de grau leve, não houve diferenças estatisticamente significantes entre o limiar do PEATE por via aérea e óssea, estando esses limiares equivalentes, com GAP aéreo-ósseo menor que 10dB. A latência da onda V no limiar eletrofisiológico e a 50 dBnNA foram menores que as referidas latências observadas em indivíduos com audição normal. CONCLUSÃO: foram encontrados limiares eletrofisiológicos por via óssea equivalente aos limiares obtidos por via aérea, com presença de GAP aéreo-ósseo menor que 10dBnNA. Assim a utilização do PEATE por VO fornece dados para uma caracterização mais detalhada do tipo da perda auditiva.PURPOSE: to characterize the results of ABR via bone in subjects with mild sensorineural hearing loss, comparing these data with the control group made up by subjects with normal hearing. METHOD: the sample consisted of 40 adults of both genders, 18 - 55 year old, divided into a control group of 30 subjects with normal hearing and a study group made up of 10 subjects with mild sensorineural hearing loss. ABR was carried out with Interacoustics brand EP15. The stimulus was the click presentation rate of 27.7 / s, for a total of

  14. Whole-exome sequencing to identify the cause of congenital sensorineural hearing loss in carriers of a heterozygous GJB2 mutation

    OpenAIRE

    Parzefall, Thomas; Frohne, Alexandra; Koenighofer, Martin; Kirchnawy, Andreas; Streubel, Berthold; Schoefer, Christian; Frei, Klemens; Lucas, Trevor

    2017-01-01

    Bi-allelic variations in the gap junction protein beta-2 (GJB2) gene cause up to 50% of cases of newborn hearing loss. Heterozygous pathogenic GJB2 variations are also fivefold overrepresented in idiopathic patient groups compared to the normal-hearing population. Whether hearing loss in this group is due to unidentified additional variations within GJB2 or variations in other deafness genes is unknown in most cases. Whole-exome sequencing offers an effective approach in the search for causat...

  15. Autosomal dominant inherited non-syndromic sensorineural hearing impairment : phenotype and genotype correlations of DFNA2-DFNA13-DFNA14-DFNA21

    NARCIS (Netherlands)

    Kunst, H.P.M.

    1999-01-01

    Hearing impairment is one of the most prevalent disorders. About 10% of the population aged 55-60 years suffers from significant hearing impairment, this increases to about 50% at the age of 80 years. In this thesis the phenotype of 5 families is described extensively, each family showed about 20-40

  16. Whole-exome sequencing to identify the cause of congenital sensorineural hearing loss in carriers of a heterozygous GJB2 mutation.

    Science.gov (United States)

    Parzefall, Thomas; Frohne, Alexandra; Koenighofer, Martin; Kirchnawy, Andreas; Streubel, Berthold; Schoefer, Christian; Frei, Klemens; Lucas, Trevor

    2017-10-01

    Bi-allelic variations in the gap junction protein beta-2 (GJB2) gene cause up to 50% of cases of newborn hearing loss. Heterozygous pathogenic GJB2 variations are also fivefold overrepresented in idiopathic patient groups compared to the normal-hearing population. Whether hearing loss in this group is due to unidentified additional variations within GJB2 or variations in other deafness genes is unknown in most cases. Whole-exome sequencing offers an effective approach in the search for causative variations in patients with Mendelian diseases. In this prospective genetic cohort study, we initially investigated a family of Turkish origin suffering from congenital autosomal recessive hearing loss. An index patient and his normal-hearing father, both bearing a single heterozygous pathogenic c.262G>T (p.Ala88Ser) GJB2 transversion as well as the normal-hearing mother were investigated by means of whole-exome sequencing. Subsequently the genetic screening was extended to a hearing-impaired cohort of 24 families of Turkish origin. A homozygous missense c.5492G>T transversion (p.Gly1831Val) in the Myosin 15a gene, previously linked to deafness, was identified as causative in the index family. This very rare variant is not listed in any population in the Genome Aggregation Database. Subsequent screening of index patients from additional families of Turkish origin with recessive hearing loss identified the c.5492G>T variation in an additional family. Whole-exome sequencing may effectively identify the causes of idiopathic hearing loss in patients bearing heterozygous GJB2 variations.

  17. Characteristics of hearing-impairment among patients in Ghana ...

    African Journals Online (AJOL)

    Again, the occurrence of Sensorineural Hearing Loss was more than other types of hearing loss. Noise, Fever, Presbycusis, Sickness, Meningitis and Meniere\\'s diseases were the major causes of Sensorineural Hearing Loss. Conductive Hearing Loss was attributed in the main to Wax, Foreign Bodies, Otitis Media, and ...

  18. Mitochondrial 12S ribosomal RNA A1555G mutation associated with cardiomyopathy and hearing loss following high-dose chemotherapy and repeated aminoglycoside exposure

    DEFF Research Database (Denmark)

    Skou, Anne-Sofie; Tranebjærg, Lisbeth; Jensen, Tim

    2014-01-01

    A 19-month-old girl with the A1555G mitochondrial mutation in the 12S ribosomal RNA gene and acute myelogenous leukemia developed dilated cardiomyopathy and bilateral sensorineural hearing loss before undergoing allogeneic stem cell transplantation. She had received gentamicin during episodes...

  19. Sudden sensorineural hearing loss: a case report supporting the immunologic theory Surdez neurosensorial súbita: relato de um caso clínico corroborando a teoria auto-imune

    Directory of Open Access Journals (Sweden)

    Antonio Luiz dos Santos Werneck

    2003-12-01

    Full Text Available Sudden sensorineural hearing loss (SSNHL is one of the autoimmune diseases of the inner ear (AIED, which is characterized by a hearing loss of above 30 decibels in at least three contiguous audiometric frequencies over a time course of 72 hours or shorter. Its cause can be found in only 10% to 15% of patients. Histopathologic findings have reported retrograde neuronal degeneration and atrophy of Corti's organ and of the vascular stria. This paper describes a case of a middle-aged female patient undergoing a treatment for hyperthyroidism who developed bilateral SSNHL. The patient was treated with methylprednilisone (1mg/kg/day for three days with considerable hearing improvement. This treatment was followed by lung and kidney tuberculosis. The immune mechanism of this entity and the possibility of interconnected participation of the antigen type, of an autoimmune disease and of bacterial infection are discussed.A surdez neurosensorial aguda (SNSA é uma das doenças auto-imunes do ouvido interno caracterizada pela perda da audição acima de 30 decibéis, em pelo menos 3 freqüências audiométricas contíguas, no período máximo de 3 dias. Sua causa só é definida em 10 a 15% dos pacientes. Achados histopatológicos demonstram degeneração neuronal retrógrada e atrofia do órgão de Corti e da estria vascular. Relatamos o caso de uma paciente de média idade em tratamento de hipertireoidismo que apresentou SNSA bilateral. A paciente foi tratada com metil-prednisilona (1mg/kg/dia durante três dias evoluindo para uma melhora considerável da audição. Este tratamento foi seguido de tuberculose pulmonar e renal. Discute-se o mecanismo imunológico desta entidade, e a possibilidade de participação cruzada do tipo antígeno, doença autoimune e infecção bacteriana.

  20. Efficacy of systemic and intratympanic corticosteroid combination therapy versus intratympanic or systemic therapy in patients with idiopathic sudden sensorineural hearing loss: a randomized controlled trial.

    Science.gov (United States)

    Ashtiani, Mohammadtaghi Khorsandi; Firouzi, Farzad; Bastaninejad, Shahin; Dabiri, Sasan; Nasirmohtaram, Sevil; Saeedi, Niloufar; Ghazavi, Hossein; Sahebi, Leyla

    2018-01-01

    The present study was conducted to compare the rates of recovery from idiopathic sudden deafness after the treatment with oral and intratympanic corticosteroids in both mono and combination therapies. Triple-blind randomized clinical trial. Tertiary referral hospital. A total of 112 patients who were admitted to the ENT emergency department randomly divided into three groups: an oral corticosteroid plus intratympanic placebo (systemic corticosteroid monotherapy group); an intratympanic corticosteroid plus oral placebo group (IT monotherapy group); and a combination therapy group (IT plus systemic combination group). All patients were treated additionally with antiviral and proton pomp inhibitor. An audiometry was performed once before beginning the therapies and again at the end of the therapy. Of the total of 112 patients, 32 received intratympanic (IT) corticosteroids, 45 were receiving systemic corticosteroids, and 35 were receiving a combination of the two. A total of 74 patients (66.1%) responded positively [response to treatment was calculated as gain of at least 10 dB in 10 dB in average threshold or with the minimum improvement of 15% in speech discrimination scores (SDS)] to corticosteroid therapy. No significant differences were observed between the three groups (IT, systemic group, and combination therapy group) in their overall response to treatment (p = 0.5). Patients who suffered from concomitant tinnitus and dizziness responded less positively to the treatment (p family history of SSNHL seems to be negative prognostic factors in the response to treatment (p hearing loss (p = 0.9). This study did not find any difference in the rate of hearing improvement between systemic, intratympanic, and combined corticosteroid therapy for sudden hearing loss. 1b.

  1. Genetics Home Reference: age-related hearing loss

    Science.gov (United States)

    ... Description Age-related hearing loss (also known as presbycusis) is a decrease in hearing ability that happens ... age-related old-aged sensorineural hearing impairment presbyacusia presbycusis Related Information How are genetic conditions and genes ...

  2. Progressive Hearing Impairment in Children with Congenital Cytomegalovirus Infection.

    Science.gov (United States)

    Dahle, Arthur J.; And Others

    1979-01-01

    Audiological assessment of 86 children (mean age 38 months at last evaluation time) with congenital cytomegalovirus infection revealed progressive hearing loss in four of 12 Ss with sensorineural hearing impairments. Case descriptions documented the progression of the hearing loss. (Author)

  3. Characteristics and Progression of Hearing Loss in Children with Down Syndrome.

    Science.gov (United States)

    Kreicher, Kathryn L; Weir, Forest W; Nguyen, Shaun A; Meyer, Ted A

    2018-02-01

    To evaluate hearing impairment in children with Down syndrome, and to describe the factors that influence the severity of hearing loss or changes in hearing over time. Using the Audiological and Genetic Database (AudGenDB), audiograms of children with Down syndrome were analyzed retrospectively for type, severity, and laterality of hearing loss, as well as changes over time. Medical charts and imaging results were reviewed to identify factors influencing hearing loss. Among the 1088 patients with Down syndrome included in the study, 921 had hearing loss in at least 1 ear, 91.1% had bilateral hearing loss, and 8.9% had unilateral hearing loss (1760 total ears with hearing loss). Of the ears with hearing loss, 18.8% (n = 180) had moderate or worse hearing loss. "Undefined" hearing loss and pure conductive hearing loss (CHL) were the most common types, followed by mixed hearing loss and sensorineural hearing loss (SNHL). Three-quarters (75.4%) of the children had experienced chronic otitis media or more than 2 episodes of acute otitis media. Patients with bilateral, mixed hearing loss or a history of seizures were at risk for more severe hearing loss. CHL, absence of cholesteatoma, and placement of first ear tubes before age 2 years were associated with greater improvement in hearing over time, whereas SNHL and mixed hearing loss were associated with progressive decline. Children with Down syndrome who have bilateral, mixed hearing loss or a history of seizures are at risk for more severe hearing loss. SNHL and mixed hearing loss should not be overlooked in patients with CHL. All patients with Down syndrome will benefit from serial audiograms, especially those children with SNHL or mixed hearing loss, which is likely to worsen over time. Copyright © 2017 Elsevier Inc. All rights reserved.

  4. Rastreamento da mutação mitocondrial A1555G em pacientes com deficiência auditiva sensorioneural Screening of the mitochondrial A1555G mutation in patients with sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Luciano Pereira Maniglia

    2008-10-01

    Full Text Available A mutação mitocondrial A1555G é a principal alteração associada à surdez ocasionada pelo uso de aminoglicosídeos. OBJETIVO: Investigar a prevalência da mutação A1555G em pacientes com deficiência auditiva sensorioneural com e sem uso de antibióticos aminoglicosídeos. MATERIAL E MÉTODO: Estudo em amostras de 27 pacientes com surdez, como casos, e em 100 neonatos, com audição normal, como grupo controle. O DNA foi extraído de leucócitos de amostras de sangue e "primers" específicos foram utilizados para amplificar o gene do citocromo b e a região que abrange a mutação A1555G do DNA mitocondrial, usando as técnicas da Reação em Cadeia da Polimerase e do Polimorfismo no Comprimento de Fragmentos de Restrição. DESENHO CIENTÍFICO: Estudo de casos em corte transversal. RESULTADOS: A região do gene do citocromo b foi amplificada, sendo confirmada a presença do DNA mitocondrial em todas as 127 amostras do estudo. A mutação A1555G não foi identificada nos 27 pacientes com deficiência auditiva e no grupo controle (100 neonatos. CONCLUSÕES: Os resultados são concordantes com estudos que relatam que a mutação A1555G não é prevalente nas Américas. Há interesse na determinação da real prevalência dessa mutação e na investigação de outras mutações que possam ocasionar deficiência auditiva associada ou não ao uso de aminoglicosídeos na população brasileira.The A1555G mitochondrial mutation is the main alteration associated with aminoglycoside-induced deafness. AIM: to investigate the prevalence of the A1555G mutation in patients sensorineural hearing loss patients with and without aminoglycosides antibiotic use. MATERIAL AND METHOD: a study of 27 cases with deafness as the sample, and 100 neonates with normal hearing as the control group. DNA was extracted from blood leukocyte samples, and specific oligonucleotide primers were designed to amplify the cytochrome b gene and the region which encloses the A1555

  5. Pharmacological reversal of endothelin-1 mediated constriction of the spiral modiolar artery: a potential new treatment for sudden sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Arnold Wolfgang

    2005-11-01

    Full Text Available Abstract Background Vasospasm of the spiral modiolar artery (SMA may cause ischemic stroke of the inner ear. Endothelin-1 (ET-1 induces a strong, long-lasting constriction of the SMA by increasing contractile apparatus Ca2+ sensitivity via Rho-kinase. We therefore tested several Rho-kinase inhibitors and a cell-permeable analogue of cAMP (dbcAMP for their ability to reverse ET-1-induced constriction and Ca2+-sensitization. Methods The present study employed SMA isolated from gerbil temporal bones. Ca2+sensitivity was evaluated by correlating vascular diameter and smooth muscle cell [Ca2+]i, measured by fluo-4-microfluorometry and videomicroscopy. Results The Rho-kinase inhibitors Y-27632, fasudil, and hydroxy-fasudil reversed ET-1-induced vasoconstriction with an IC50 of 3, 15, and 111 μmol/L, respectively. DbcAMP stimulated a dose-dependent vasodilation (Ec50 = 1 mmol/L and a reduction of [Ca2+]i (EC50 = 0.3 μmol/L of ET-1-preconstricted vessels (1 nmol/L. Fasudil and dbcAMP both reversed the ET-1-induced increase in Ca2+ sensitivity. Conclusion Rho-kinase inhibition and dbcAMP reversed ET-1-induced vasoconstriction and Ca2+-sensitization. Therefore, Rho-kinase inhibitors or cAMP modulators could possess promise as pharmacological tools for the treatment of ET-1-induced constriction, ischemic stroke and sudden hearing loss.

  6. Risk factors for hearing loss in elderly

    OpenAIRE

    Kelly Vasconcelos Chaves Martins; Marília Fontenele e Silva Câmara

    2012-01-01

    Objective: To identify risk factors related to sensorineural hearing loss in elderly. Methods: The sample consisted of 60 selected elderly, divided into two groups: the Case Group, composed by 30 individuals, 21 females and 9 males, aged at least 60 years, presenting sensorineural hearing loss, and the Control Group, composed by 30 individuals matched on gender and age, with normal hearing. The patients were submitted to audiological anamnesis and tonal audiometry. The hearing impairment ...

  7. Acute streptococcal meningitis presenting as bilateral conductive hearing loss.

    Science.gov (United States)

    Dougherty, Matthew J; Smith, Austin T

    2018-04-05

    Bacterial meningitis is a relatively uncommon condition encountered in the emergency department and the constellation of symptoms varies. Hearing loss has been well documented in the literature as a complication of the disease process, but not as the presenting complaint. We describe a case of a 59-year-old female who presented to the emergency department with sudden onset bilateral hearing loss who was found to have S. pneumonia meningitis bacterial meningitis. Even with advances in therapy, bacterial meningitis still carries a significant mortality rate. Early diagnosis and treatment is critical to achieving good outcomes. Copyright © 2018 Elsevier Inc. All rights reserved.

  8. Roteiro diagnóstico e de conduta frente à perda auditiva sensorioneural genética Diagnosis routine and approach in genetic sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Fatima Regina Abreu Alves

    2007-06-01

    determine inheritance patterns. Through a high suspicion index, syndromic cases can be diagnosed or excluded, with a careful evaluation and molecular basis tests used to better determine the hearing loss. Genetic tests and mitochondrial inheritance should be considered in any family with many affected individuals, except when the hearing loss was clearly transmitted by a male. In cases of non-syndromic SNHL, GJB2 mutation analysis must be proposed.

  9. Prevalence of Hearing Loss in Patients with Acute Otitis Media ...

    African Journals Online (AJOL)

    The study was a prospective, cross-sectional type conducted at the Ear, Nose and Throat Department of Komfo Anokye Teaching Hospital (KATH) over a six month period from 1st August 2005 to 31st January 2006. Fifty one patients involving 69 ears (few with bilateral and majority with unilateral hearing loss) were ...

  10. Unusual magnetic resonance findings in two children with sudden sensorineural hearing loss Achados incomuns nas ressonâncias magnéticas de duas crianças com perda auditiva neuro-sensorial súbita

    Directory of Open Access Journals (Sweden)

    Emerson L. Gasparetto

    2005-12-01

    Full Text Available OBJECTIVE: To describe the MRI findings of two pediatric patients with sudden sensorineural hearing loss (SSHL. CASE REPORTS: Two male patients (two-year and three-months-old, and one year and four-months-old presented with sudden dumbness. Physical and neurological examinations were unremarkable besides bilateral hypoacusia. All the laboratory investigation was negative, and brain stem auditory evoked potentials showed deep bilateral deafness in both cases. MRI studies revealed normal inner ears and multifocal white matter areas of slight low signal on T1-weighted images and high signal on FLAIR images. The follow-up MRI studies and neurological examinations did not demonstrate alterations in the previous findings. CONCLUSION: Pediatric patients with SSHL may present cerebral white matter signal abnormalities at the MRI as the only finding. Further studies with larger casuistics need to be conducted to elucidate these findings.OBJETIVO: Descrever os achados de ressonância magnética (RM em dois pacientes pediátricos com perda auditiva neuro-sensorial súbita (PANS. RELATO DOS CASOS: Dois pacientes masculinos (dois anos e três meses e um ano e quarto meses de idade apresentaram-se com surdez súbita. Os exames físico e neurológico foram normais, à exceção da hipoacusia. Toda a investigação laboratorial foi normal, e os potenciais auditivos evocados do tronco cerebral demonstraram surdez profunda bilateral. Os exames de RM evidenciaram estruturas normais dos ouvidos internos e áreas multifocais de discreto hipossinal em T1 e hipersinal em FLAIR na substância branca dos hemisférios cerebrais. As RMs e os exames neurológicos de controle não demonstraram alterações nos achados prévios. CONCLUSÃO: Pacientes pediátricos com PANS podem apresentar à RM alterações de sinal na substância branca dos hemisférios cerebrais como único achado. Estudos futuros com casuísticas maiores deverão ser conduzidos para a elucidação destes

  11. Bilateral sudden sensorineural deafness with vertigo as the sole presenting symptoms of diabetes mellitus — a case report

    OpenAIRE

    Misra, Vilas; Agarwal, C. G.; Bhatia, Naresh; Shukla, G. K.

    2010-01-01

    This Paper reports a late uncontrolled diabetic presenting to an otolaryngologist with sudden severe sensorineural hearing loss of immediate origin with vertigo and tinnitus as the symptoms. Appropriate investigative and treatment measure resulted in deterioration of hearing in the right ear and mild improvement of hearing in the left ear, with no recovery of imbalance.

  12. Bilateral sudden sensorineural deafness with vertigo as the sole presenting symptoms of diabetes mellitus - a case report.

    Science.gov (United States)

    Misra, Vilas; Agarwal, C G; Bhatia, Naresh; Shukla, G K

    2010-06-01

    This Paper reports a late uncontrolled diabetic presenting to an otolaryngologist with sudden severe sensorineural hearing loss of immediate origin with vertigo and tinnitus as the symptoms. Appropriate investigative and treatment measure resulted in deterioration of hearing in the right ear and mild improvement of hearing in the left ear, with no recovery of imbalance.

  13. Hearing status in patients with rheumatoid arthritis.

    Science.gov (United States)

    Ahmadzadeh, A; Daraei, M; Jalessi, M; Peyvandi, A A; Amini, E; Ranjbar, L A; Daneshi, A

    2017-10-01

    Rheumatoid arthritis is thought to induce conductive hearing loss and/or sensorineural hearing loss. This study evaluated the function of the middle ear and cochlea, and the related factors. Pure tone audiometry, speech reception thresholds, speech discrimination scores, tympanometry, acoustic reflexes, and distortion product otoacoustic emissions were assessed in rheumatoid arthritis patients and healthy volunteers. Pure tone audiometry results revealed a higher bone conduction threshold in the rheumatoid arthritis group, but there was no significant difference when evaluated according to the sensorineural hearing loss definition. Distortion product otoacoustic emissions related prevalence of conductive or mixed hearing loss, tympanometry values, acoustic reflexes, and speech discrimination scores were not significantly different between the two groups. Sensorineural hearing loss was significantly more prevalent in patients who used azathioprine, cyclosporine and etanercept. Higher bone conduction thresholds in some frequencies were detected in rheumatoid arthritis patients that were not clinically significant. Sensorineural hearing loss is significantly more prevalent in refractory rheumatoid arthritis patients.

  14. Prevalence of occupational noise induced hearing loss amongst traffic police personnel

    OpenAIRE

    Singh, V K; Mehta, A K

    1999-01-01

    Traffic branch personnel of Pune traffic police were screened for presence of noise induced hearing loss. A very significant number (81.2%) showed sensorineural hearing loss. The various factors responsible for noise induced hearing loss are discussed.

  15. [Metastasis to the temporal bone may cause acute peripheral vestibular syndrome and impaired hearing].

    Science.gov (United States)

    Grubbe Gregersen, Kristine; Hansen, Søren

    2013-05-27

    Metastasis to the petrous apex of the temporal bone may cause acute peripheral vestibular syndrome and impaired hearing or be asymptomatic. Contrast computed tomography should be performed to exclude pathology in the temporal bone in patients with vestibulocochlear deficit, a history of cancer and no findings on cerebral magnetic resonance imaging. We describe a case of a 61-year-old man with metastatic prostatic carcinoma to the temporal bone.

  16. An Evaluation of the BKB-SIN, HINT, QuickSIN, and WIN Materials on Listeners with Normal Hearing and Listeners with Hearing Loss

    Science.gov (United States)

    Wilson, Richard H.; McArdle, Rachel A.; Smith, Sherri L.

    2007-01-01

    Purpose: The purpose of this study was to examine in listeners with normal hearing and listeners with sensorineural hearing loss the within- and between-group differences obtained with 4 commonly available speech-in-noise protocols. Method: Recognition performances by 24 listeners with normal hearing and 72 listeners with sensorineural hearing…

  17. Association between ambient noise exposure, hearing acuity, and risk of acute occupational injury.

    Science.gov (United States)

    Cantley, Linda F; Galusha, Deron; Cullen, Mark R; Dixon-Ernst, Christine; Rabinowitz, Peter M; Neitzel, Richard L

    2015-01-01

    This study aimed to examine the associations between acute workplace injury risk, ambient noise exposure, and hearing acuity, adjusting for reported hearing protection use. In a cohort of 9220 aluminum manufacturing workers studied over six years (33 300 person-years, 13 323 person-jobs), multivariate mixed effects models were used to estimate relative risk (RR) of all injuries as well as serious injuries by noise exposure category and hearing threshold level (HTL) adjusting for recognized and potential confounders. Compared to noise exposure was associated with elevated risk in a monotonic and statistically significant exposure-response pattern for all injuries and serious injuries with higher risk estimates observed for serious injuries [82-84.99 dBA: RR 1.26, 95% confidence interval (95% CI) 0.96-1.64; 85-87.99 dBA: RR 1.39, 95% CI 1.05-1.85; ≥88 dBA: RR 2.29, 95% CI 1.52-3.47]. Hearing loss was associated with increased risk for all injuries, but was not a significant predictor of risk for the subset of more serious injuries. Compared to those without hearing loss, workers with HTL ≥25 dB had 21% increased all injury risk (RR 1.21, 95% CI 1.09-1.33) while those with HTL 10-24.99 dB had 6% increased risk (RR 1.06, 95% CI 1.00-1.13). Reported hearing protection type did not predict injury risk. Noise exposure levels as low as 85 dBA may increase workplace injury risk. HTL was associated with increased risk for all, but not the subset of serious, injuries. Additional study is needed both to confirm the observed associations and explore causal pathways.

  18. Motivation to Address Self-Reported Hearing Problems in Adults with Normal Hearing Thresholds

    Science.gov (United States)

    Alicea, Carly C. M.; Doherty, Karen A.

    2017-01-01

    Purpose: The purpose of this study was to compare the motivation to change in relation to hearing problems in adults with normal hearing thresholds but who report hearing problems and that of adults with a mild-to-moderate sensorineural hearing loss. Factors related to their motivation were also assessed. Method: The motivation to change in…

  19. Timbre perception and object separation with normal and impaired hearing

    OpenAIRE

    Emiroglu, Suzan Selma

    2007-01-01

    Timbre is a combination of all auditory object attributes other than pitch, loudness and duration. A timbre distortion caused by a sensorineural hearing loss not only affects music perception, but may also influence object recognition in general. In order to quantify differences in object segregation and timbre discrimination between normal-hearing and hearing-impaired listeners with a sensorineural hearing loss, a new method for studying timbre perception was developed, which uses cross-fade...

  20. The Use of Drugs to Reduce Hearing Loss Following Acute Acoustic Trauma

    Science.gov (United States)

    2013-10-15

    L - carnitine (ALCAR) and (5) Src-PTK inhibitor, KX1-004 in reducing hearing and sensory cell loss as the result of an acute acoustic trauma that...trauma: (1) L -N-acetylcysteine ( L -NAC); (2) D-Methionine (D-MET); (3) Ebselen SPI-1005; (4) Acetyl- L - carnitine (ALCAR) and (5) Src-PTK inhibitor, KX1...rescue mode with L -N-acetylcysteine, D- Methionine, Ebselen SPI-1005, Acetyl- L - carnitine or Src-PTK inhibitor, KX1-004 following an acoustic trauma

  1. Genes and Syndromic Hearing Loss.

    Science.gov (United States)

    Keats, Bronya J. B.

    2002-01-01

    This article provides a description of the human genome and patterns of inheritance and discusses genes that are associated with some of the syndromes for which hearing loss is a common finding, including: Waardenburg, Stickler, Jervell and Lange-Neilsen, Usher, Alport, mitochondrial encephalomyopathy, and sensorineural hearing loss. (Contains…

  2. Hearing impairment in genotyped Wolfram syndrome patients.

    NARCIS (Netherlands)

    Plantinga, R.F.; Pennings, R.J.E.; Huygen, P.L.M.; Bruno, R.; Eller, P.; Barrett, T.G.; Vialettes, B.; Paquis-Fluklinger, V.; Lombardo, F.; Cremers, C.W.R.J.

    2008-01-01

    OBJECTIVES: Wolfram syndrome is a progressive neurodegenerative syndrome characterized by the features "DIDMOAD" (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness). We sought to study the audiometric data of genotyped Wolfram syndrome patients with sensorineural hearing impairment.

  3. Perda auditiva neurossensorial súbita idiopática: evolução na presença de hipertensão arterial sistêmica, diabetes melito e dislipidemias Idiopathic sudden sensorineural hearing loss: evolution in the presence of hypertension, diabetes mellitus and dyslipidemias

    Directory of Open Access Journals (Sweden)

    Jayson Nagaoka

    2010-06-01

    Full Text Available Estudo retrospectivo com o objetivo de avaliar a interferência das doenças associadas na evolução e prognóstico da perda auditiva neurossensorial súbita idiopática. MATERIAL E MÉTODO: Estudo caso controle. Trinta e cinco pacientes com perda auditiva neurossensorial súbita idiopática divididos em dois grupos, um com doenças associadas (hipertensão arterial sistêmica, diabetes melito e dislipidemias e outro sem doenças associadas. Avaliação das variáveis: idade, sexo, doença associada, presença de zumbido, tontura e plenitude aural, presença de microangiopatia cerebral na ressonância magnética, alteração no fundo de olho, tempo de início de tratamento, taxa de melhora auditiva, evolução do índice do reconhecimento da fala. Dados avaliados estatisticamente. RESULTADOS: O grupo com doença associada, quando comparado ao grupo sem doença associada, apresenta idade mais elevada, maior número de pacientes com microangiopatia cerebral na ressonância magnética e recuperação mais lenta do índice de reconhecimento da fala. CONCLUSÃO: Perda auditiva neurossensorial súbita idiopática na presença da hipertensão arterial sistêmica, diabetes melito e dislipidemias, em indivíduos mais velhos, está associada a uma maior prevalência de achados de microangiopatias cerebrais nos exames de ressonância magnética, e à recuperação auditiva mais lenta na melhora do índice de reconhecimento da fala.Retrospective study aiming at evaluating the interference of associate diseases in the evolution and prognosis of idiopathic sudden sensorineural hearing loss. MATERIALS AND METHODS: Case-Control Study. Thirty-five patients with idiopathic sudden sensorineural hearing loss were divided in two groups, one of them with associate diseases (hypertension, diabetes mellitus and dyslipidemias, and another one without co-occurrence of such diseases. The groups were evaluated regarding: age, gender, associate diseases, presence of

  4. Myringoplasty - effects on hearing and contributing factors | Black ...

    African Journals Online (AJOL)

    Indications for myringoplasty are chronic otorrhoea and hearing impairment. Some authors have described poor postoperative hearing results and sensorineural hearing loss associated with the surgery, and question the indication for myringoplasty on an ear with normal hearing « 25 dB). This study of 211 Consecutive ...

  5. Noise Induced Hearing Loss among Khartoum International Airport ...

    African Journals Online (AJOL)

    Background: Noise-induced hearing loss [NIHL] was incriminated to be the second most common sensorineural hearing loss, after age-related hearing loss. Objective: This study aimed to investigate noise induced hearing loss among employees of Khartoum international air port. Material and Methods: The study ...

  6. Current amplification models of sensorineurall and conductive hearing loss

    OpenAIRE

    Ostojić, Sanja; Mikić, Branka; Mirić, Danica

    2012-01-01

    The main function of a hearing aid is to improve auditory and language abilities of hearing impaired users. The amplification model has to be adapted according to age, degree and type of hearing loss. The goal of this paper is to analyze the current amplification models of sensorineural and conductive hearing loss which can provide a high quality of speech perception and sounds at any degree of hearing loss. The BAHA is a surgically implantable system for treatment of conductive hearing loss ...

  7. Effects of cranial radiation on hearing in children with acute lymphocytic leukemia

    International Nuclear Information System (INIS)

    Thibadoux, G.M.; Pereira, W.V.; Hodges, J.M.; Aur, R.J.

    1980-01-01

    The hearing sensitivity of 61 children with acute lymphocytic leukemia who were admitted to our Total Therapy IX study between December 1975 and July 1977 was studied. Their treatment included combined chemotherapy, 2400 rads of cranial radiation, and intrathecal methotrexate. Subjects initially received an otologic examination and middle ear function testing. Audiometric testing was not done until ears were free of outer or middle ear pathology. If the child had no outer or middle ear disease, audiometric thresholds were obtained for the test frequencies: 500, 1000, 2000, 4000, 6000, and 8000 Hz. Pure-tone thresholds were obtained before irradiation (61 patients) and at 6, 12, and 36 months thereafter (49, 46, and 22 patients, respectively). The median age of time of baseline testing was 10 years, 2 months. A paired sample test based on group data was used to test whether there were any significant changes from the threshold values at 6, 12, and 36 months after irradiation. Thresholds were not significantly affected for any test frequency at any test time. Assessments of individual audiograms indicated that none of the children had any significant reductions in hearing levels at the end of the third year after cranial irradiation

  8. Mechanisms of Sensorineural Cell Damage, Death and Survival in the Cochlea

    Directory of Open Access Journals (Sweden)

    Allen Frederic Ryan

    2015-04-01

    Full Text Available The majority of acquired hearing loss, including presbycusis, is caused by irreversible damage to the sensorineural tissues of the cochlea. This article reviews the intracellular mechanisms that contribute to sensorineural damage in the cochlea, as well as the survival signaling pathways that can provide endogenous protection and tissue rescue. These data have primarily been generated in hearing loss not directly related to age. However, there is evidence that similar mechanisms operate in presbycusis. Moreover, accumulation of damage from other causes can contribute to age-related hearing loss. Potential therapeutic interventions to balance opposing but interconnected cell damage and survival pathways, such as antioxidants, anti-apoptotics, and pro-inflammatory cytokine inhibitors, are also discussed.

  9. Niikawa-Kuroki (Kabuki) syndrome with congenital sensorineural deafness: evidence for a wide spectrum of inner ear abnormalities.

    Science.gov (United States)

    Tekin, Mustafa; Fitoz, Suat; Arici, Serap; Cetinkaya, Ergun; Incesulu, Armagan

    2006-05-01

    Hearing loss, mainly due to recurrent otitis media, has been reported in approximately 40% of individuals with Niikawa-Kuroki (Kabuki) syndrome (NKS). Sensorineural hearing loss leading to congenital or prelingual deafness has been described rarely. We have identified two unrelated individuals with Niikawa-Kuroki syndrome among 535 probands who have severe to profound sensorineural deafness. Bilateral absence of the cochlea with dilated dysplastic vestibule and unilateral enlarged vestibule were demonstrated in these two individuals. In conclusion, Niikawa-Kuroki syndrome should be kept in mind when evaluating an individual with congenital deafness and a wide spectrum of inner ear abnormalities occurs in this syndrome.

  10. Is Hearing Impairment Associated with Rheumatoid Arthritis?

    DEFF Research Database (Denmark)

    Emamifar, Amir; Bjoerndal, Kristine; Jensen Hansen, Inger Marie

    2016-01-01

    and ComDisDome to cover all relative reports. The following keywords were used: hearing loss, hearing difficulties, hearing disorders, hearing impairment, sensorineural hearing loss, conductive hearing loss, mixed hearing loss, autoimmune hearing loss, drug ototoxicity, drug-induced hearing loss, hearing......BACKGROUND: Rheumatoid arthritis (RA) is a systemic, inflammatory disease that affects 1% of the population. The auditory system may be involved during the course of disease; however the association of RA and hearing impairment has not been clearly defined. OBJECTIVE: The objective of this review...... is to evaluate published clinical reports related to hearing impairment in patients with RA. Furthermore, we discuss possible pathologies and associated factors as well as new treatment modalities. METHOD: A thorough literature search was performed using available databases including Pubmed, Embase, Cochrane...

  11. Aspects of Hearing Aid Fitting Procedures

    NARCIS (Netherlands)

    R.M. Metselaar (Mick)

    2010-01-01

    textabstractSensorineural hearing loss is a common and chronic disorder that affects almost ten percent of the world population. In the Netherlands, it is also the major disorder in the working population [NCvB, 2008]. Hearing loss leads to restriction in the interaction with others and withdrawal

  12. Sensorineural hearing loss in high school teenagers in Mexico City and its relationship with recreational noise Las alteraciones auditivas de escolares adolescentes en la Ciudad de México pueden estar relacionadas al ruido recreativo

    Directory of Open Access Journals (Sweden)

    María del Consuelo Martínez-Wbaldo

    2009-12-01

    Full Text Available The aim of this study was to measure the frequency of hearing loss in a sample of typical public high-school students exposed to recreational noise, and determine associated risk factors. The sample was made up of 214 teenagers from a high-school in Mexico City; subjects were selected randomly per strata. We applied a questionnaire to identify risk factors for hearing loss and performed a battery of audiologic tests consisting of otoscopy, tympanometry, and pure-tone audiometry. The mean age of the sample was 16 ± 1.07 years; 73% were male and 27%, female. Hearing loss was found in 21% of students. The main hearing loss-related risk factor was exposure to recreational noise: frequent attendance at discotheques and pop-music concerts; use of personal stereos; and noise exposure in school workshops. The high frequency of hearing loss in high school students from one Mexico City school (nearly one fifth of the sample was found to be related to noise exposure mainly during recreational activities.El objetivo de este estudio fue medir la frecuencia de alteraciones auditivas en una muestra de escolares de educación media superior expuestos a ruido recreativo y determinar algunos factores de riesgo asociados. La muestra estuvo constituida por 214 adolescentes de una escuela de la Ciudad de México. Los sujetos fueron seleccionados al azar por medio de una computadora. Se aplicaron cuestionarios con el objeto de identificar los factores de riesgo para alteraciones auditivas y se les practicaron diversos estudios audiológicos consistentes en: otoscopía, timpanometría y una audiometría a tonos puros. La edad media de la muestra fue de 16 ± 1,07 años, 73% fueron del sexo masculino y 27% femenino. Se encontraron alteraciones auditivas en 21% de los estudiantes. Los principales factores de riesgo asociados a alteraciones auditivas fueron: exposición al ruido recreativo al asistir a discotecas, conciertos de música popular, el uso de equipos de

  13. A personal approach to hearing conservation: the key to effective ...

    African Journals Online (AJOL)

    The English term “boilermaker's deafness” was often used during the 1700 and 1800s. It referred to high-frequency sensori-neural hearing loss found in workers exposed to high levels of noise intensity. Second-level noise control relates to hearing conservation programmes (HCPs) and hearing protection (as well as ...

  14. Identifying Hearing Loss By Means Of Iridology | Stearn | African ...

    African Journals Online (AJOL)

    Isolated reports of hearing loss presenting as markings on the iris exist, but to date the effectiveness of iridology to identify hearing loss has not been investigated. This study therefore aimed to determine the efficacy of iridological analysis in the identification of moderate to profound sensorineural hearing loss in adolescents ...

  15. Classification of Hearing Loss Disorders Using Teoae-Based Descriptors

    Science.gov (United States)

    Hatzopoulos, Stavros Dimitris

    Transiently Evoked Otoacoustic Emissions (TEOAE) are signals produced by the cochlea upon stimulation by an acoustic click. Within the context of this dissertation, it was hypothesized that the relationship between the TEOAEs and the functional status of the OHCs provided an opportunity for designing a TEOAE-based clinical procedure that could be used to assess cochlear function. To understand the nature of the TEOAE signals in the time and the frequency domain several different analyses were performed. Using normative Input-Output (IO) curves, short-time FFT analyses and cochlear computer simulations, it was found that for optimization of the hearing loss classification it is necessary to use a complete 20 ms TEOAE segment. It was also determined that various 2-D filtering methods (median and averaging filtering masks, LP-FFT) used to enhance of the TEOAE S/N offered minimal improvement (less than 6 dB per stimulus level). Higher S/N improvements resulted in TEOAE sequences that were over-smoothed. The final classification algorithm was based on a statistical analysis of raw FFT data and when applied to a sample set of clinically obtained TEOAE recordings (from 56 normal and 66 hearing-loss subjects) correctly identified 94.3% of the normal and 90% of the hearing loss subjects, at the 80 dB SPL stimulus level. To enhance the discrimination between the conductive and the sensorineural populations, data from the 68 dB SPL stimulus level were used, which yielded a normal classification of 90.2%, a hearing loss classification of 87.5% and a conductive-sensorineural classification of 87%. Among the hearing-loss populations the best discrimination was obtained in the group of otosclerosis and the worst in the group of acute acoustic trauma.

  16. Assessment of hearing aid algorithms using a master hearing aid: the influence of hearing aid experience on the relationship between speech recognition and cognitive capacity.

    Science.gov (United States)

    Rählmann, Sebastian; Meis, Markus; Schulte, Michael; Kießling, Jürgen; Walger, Martin; Meister, Hartmut

    2017-04-27

    Model-based hearing aid development considers the assessment of speech recognition using a master hearing aid (MHA). It is known that aided speech recognition in noise is related to cognitive factors such as working memory capacity (WMC). This relationship might be mediated by hearing aid experience (HAE). The aim of this study was to examine the relationship of WMC and speech recognition with a MHA for listeners with different HAE. Using the MHA, unaided and aided 80% speech recognition thresholds in noise were determined. Individual WMC capacity was assed using the Verbal Learning and Memory Test (VLMT) and the Reading Span Test (RST). Forty-nine hearing aid users with mild to moderate sensorineural hearing loss divided into three groups differing in HAE. Whereas unaided speech recognition did not show a significant relationship with WMC, a significant correlation could be observed between WMC and aided speech recognition. However, this only applied to listeners with HAE of up to approximately three years, and a consistent weakening of the correlation could be observed with more experience. Speech recognition scores obtained in acute experiments with an MHA are less influenced by individual cognitive capacity when experienced HA users are taken into account.

  17. Educating Children Who Are Deaf or Hard of Hearing: Overview. ERIC Digest #E549.

    Science.gov (United States)

    Easterbrooks, Susan

    This digest discusses hearing loss in children. Topics covered include: (1) the causes of hearing loss and the three major types of hearing losses, conductive loss, sensorineural loss, and central auditory processing disorder; (2) the federal definitions of "deaf" (hearing loss which adversely affects educational performance and which is…

  18. Genetic Counseling for Hearing Loss.

    Science.gov (United States)

    Arnos, Kathleen S.

    1997-01-01

    This article addresses epidemiologic and demographic characteristics of hereditary hearing loss and genetic evaluation and counseling. It discusses who should have genetic counseling, the genetic counseling process, and effects of genetics technology on the genetic counseling process. A case study of a 2-year-old with severe sensorineural hearing…

  19. Occupational hearing loss in farmers.

    OpenAIRE

    Plakke, B L; Dare, E

    1992-01-01

    Studies have shown that there is a great deal of high-frequency sensorineural hearing loss among farmers. The studies have failed, however, to differentiate farmers who have occupational noise exposure only from other potential hearing loss etiologies. This study, through extensive case history information, has isolated a farm noise-exposure group and matched its members by age with persons with no significant noise exposure. Results indicate that farmers exposed only to noise from farming ha...

  20. Auditory hair cell defects as potential cause for sensorineural deafness in Wolf-Hirschhorn syndrome

    Directory of Open Access Journals (Sweden)

    Mohi Ahmed

    2015-09-01

    Full Text Available WHSC1 is a histone methyltransferase (HMT that catalyses the addition of methyl groups to lysine 36 on histone 3. In humans, WHSC1 haploinsufficiency is associated with all known cases of Wolf-Hirschhorn syndrome (WHS. The cardinal feature of WHS is a craniofacial dysmorphism, which is accompanied by sensorineural hearing loss in 15% of individuals with WHS. Here, we show that WHSC1-deficient mice display craniofacial defects that overlap with WHS, including cochlea anomalies. Although auditory hair cells are specified normally, their stereocilia hair bundles required for sound perception fail to develop the appropriate morphology. Furthermore, the orientation and cellular organisation of cochlear hair cells and their innervation are defective. These findings identify, for the first time, the likely cause of sensorineural hearing loss in individuals with WHS.

  1. Auditory hair cell defects as potential cause for sensorineural deafness in Wolf-Hirschhorn syndrome.

    Science.gov (United States)

    Ahmed, Mohi; Ura, Kiyoe; Streit, Andrea

    2015-09-01

    WHSC1 is a histone methyltransferase (HMT) that catalyses the addition of methyl groups to lysine 36 on histone 3. In humans, WHSC1 haploinsufficiency is associated with all known cases of Wolf-Hirschhorn syndrome (WHS). The cardinal feature of WHS is a craniofacial dysmorphism, which is accompanied by sensorineural hearing loss in 15% of individuals with WHS. Here, we show that WHSC1-deficient mice display craniofacial defects that overlap with WHS, including cochlea anomalies. Although auditory hair cells are specified normally, their stereocilia hair bundles required for sound perception fail to develop the appropriate morphology. Furthermore, the orientation and cellular organisation of cochlear hair cells and their innervation are defective. These findings identify, for the first time, the likely cause of sensorineural hearing loss in individuals with WHS. © 2015. Published by The Company of Biologists Ltd.

  2. Modeling auditory perception of individual hearing-impaired listeners

    DEFF Research Database (Denmark)

    Jepsen, Morten Løve; Dau, Torsten

    showed that, in most cases, the reduced or absent cochlear compression, associated with outer hair-cell loss, quantitatively accounts for broadened auditory filters, while a combination of reduced compression and reduced inner hair-cell function accounts for decreased sensitivity and slower recovery from...... selectivity. Three groups of listeners were considered: (a) normal hearing listeners; (b) listeners with a mild-to-moderate sensorineural hearing loss; and (c) listeners with a severe sensorineural hearing loss. A fixed set of model parameters were derived for each hearing-impaired listener. The simulations...

  3. Prognostic factors in sudden hearing loss

    OpenAIRE

    Enache, R; Sarafoleanu, I

    2008-01-01

    Sudden hearing loss is a sensorineural hypoacusis, unilateral in most of the cases, with an incidence peak in young adult. This article is the result of a four years retrospective study, in which we have tried to see how age, severity of hearing loss, presence or absence of vertigo, the timing of treatment initiation can influence the hearing recovery. 47 patients were included in this inpatient study. They were investigated (clinical, lab and imaging studies) and the treatment protocol inclu...

  4. THIAMINE–RESPONSIVE MEGALOBLASTIC ANEMIA, SENSORINEURAL DEAFNESS AND DIABETES MELLITUS

    Directory of Open Access Journals (Sweden)

    M. Kadivar R. Moradian

    2006-11-01

    Full Text Available Abstract- The syndrome of diabetes mellitus, sensorineural deafness and megaloblastic anemia dose not result from thiamine deficiency. The previous reported patients had no sign of beriberi, had normal nutrition, and had no evidence of malabsorption. The features of this syndrome with apparent inheritance of autosomal recessive trait may define this puzzling syndrome as a true thiamine dependency state. The first Iranian patient was described by Vossough et al. in 1995. We found nine new cases with diagnostic criteria of thiamine responsive megaloblastic anemia during eight years of our study. In two patients, presentation of diabetes and anemia was concomitant. All of them were deaf with sensorineural hearing loss which was detected in infancy up to two years of age. The presence of congenital valvular heart disease was eliminated by normal echocardiography, but cardiomyopathy was discovered in two. Nonspecific amino-aciduria was discovered in three but urinary screening tests for hereditary orotic aciduria were negative. Ox-Phos biochemistry of muscle mitochondria which demonstrates severe defect in complexes I, III, IV in diabetes mellitus associated with deafness, were done but was unremarkable in our patients. Urinary methylmalonic acid and methyl malonyl carnitine by GS/MS and TMS was done in our patients and showed abnormal results in six patients. Thiamine gene, SLC 19A2, was detected in four patients.

  5. Vision and hearing impairments and their associations with falling and loss of instrumental activities in daily living in acute hospitalized older persons in five Nordic hospitals.

    Science.gov (United States)

    Grue, Else Vengnes; Ranhoff, Anette Hylen; Noro, Anja; Finne-Soveri, Harriet; Jensdóttir, Anna Birna; Ljunggren, Gunnar; Bucht, Gösta; Björnson, Leif Jan; Jonsén, Elisabeth; Schroll, Marianne; Jónsson, Palmi V

    2009-12-01

    Many older people believe sensory problems are inevitably, a part of growing old, and avoid assessment and help. Such problems are often also overlooked by health professionals. The aim of this study was to find the prevalence of hearing and vision impairment and their associations with loss of instrumental activities in daily living (IADL) and risk of falling in patients aged 75 years or older, admitted to a medical ward in an acute hospital in each of the five Nordic countries. The Minimum Data Set for Acute Care was used for data collection in 770 patients. Premorbid data, admission data and history of falls over 3 months were obtained on admission by interview and observation. Hearing impairment was present if the patient required a quiet setting to be able to hear normal speech. Vision impairment was defined as unable to read regular print in a newspaper. Bivariate and logistic regression analyses were performed. Forty-eight per cent of the patients had a hearing impairment, 32.3% had vision impairment and 20.1% had both. Hearing impairment was associated with falling but not in the logistic regression model. Hearing and vision impairment were associated with loss of IADL but only combined impairment was independently. Hearing and vision impairments were frequent among older patients in the medical wards. Falling was associated with hearing loss and IADL loss with hearing, vision and combined impairments. Sensory loss was also associated with fear of falling. It is recommended routinely to screen sensory functions in older patients in a medical setting. Intervention studies are needed to determine whether improvements in hearing and vision can prevent falls and further loss of function in this patient population.

  6. The Care and Use of a Child's Hearing Aid: A Practical Guide for Parents, Teachers, and Children.

    Science.gov (United States)

    Lewis, Michael A.

    The booklet is intended to provide practical information on hearing aids for hearing impaired children, their families and teachers. Illustrations are provided to describe the hearing process. The nature of hearing loss is considered in terms of types (conductive, sensori-neural, and mixed), causes, degrees, and educational implications. Four…

  7. [FEDERAL CLINICAL RECOMMENDATIONS IN DIAGNOSIS, TREATMENT AND PREVENTION OF HEARING LOSS DUE TO NOISE].

    Science.gov (United States)

    Adeninskaya, E E; Bukhtiarov, I V; Bushmanov, A Iu; Dayhes, N A; Denisov, E I; Izmerov, N F; Mazitova, N N; Pankova, V B; Preobrazhenskaya, E A; Prokopenko, L V; Simonova, N I; Tavartkiladze, G A; Fedina, I N

    2016-01-01

    Noise induced hearing loss is a slowly developing hearing impairment, caused by occupational exposure to excessive noise levels, constitutes a lesion of the auditory analyzer and clinically manifested as chronic bilateral sensorineural hearing loss. Currently, there is not a treatment that provide a cure of sensorineural hearing loss. Regular, individually tailored treatment should be directed to the pathogenic mechanisms and specific clinical symptoms of hearing loss, as well as the prevention of complications. We recommend using non-drug therapies that can improve blood flow in labyrinth, tissue and cellular metabolism.

  8. Reversible hearing loss following cryptococcal meningitis: case study.

    Science.gov (United States)

    Neo, W L; Durisala, N; Ho, E C

    2016-07-01

    Sensorineural hearing loss is a recognised complication of cryptococcal meningitis. The mechanism of hearing loss in patients with cryptococcal meningitis is different from that in bacterial meningitis. An immune-competent man with cryptococcal meningitis presented with sudden onset, bilateral, severe to profound sensorineural hearing loss and vestibular dysfunction. He was initially evaluated for cochlear implantation. However, he had a significant recovery; he no longer required surgery and was able to cope without a hearing aid. Typically, cochlear implantation is performed with some urgency in patients with hearing loss post-bacterial meningitis, because of the risk of labyrinthitis ossificans. However, this process has not been described in patients with cryptococcal meningitis. Furthermore, patients with hearing loss associated with cryptococcal meningitis have shown varying degrees of reversibility. In this case report, hearing loss from cryptococcal meningitis is compared with that from bacterial meningitis, and the need for cochlear implantation in patients with cryptococcal meningitis is discussed.

  9. Dexamethasone loaded nanoparticles exert protective effects against Cisplatin-induced hearing loss by systemic administration.

    Science.gov (United States)

    Sun, Changling; Wang, Xueling; Chen, Dongye; Lin, Xin; Yu, Dehong; Wu, Hao

    2016-04-21

    Ototoxicity is one of the most important adverse effects of cisplatin chemotherapy. As a common treatment of acute sensorineural hearing loss, systemic administration of steroids was demonstrated ineffective against cisplatin-induced hearing loss (CIHL) in published studies. The current study aimed to evaluate the potential protective effect of dexamethasone (DEX) encapsulated in polyethyleneglycol-coated polylactic acid (PEG-PLA) nanoparticles (DEX-NPs) against cisplatin-induced hearing loss following systemic administration. DEX was fabricated into PEG-PLA nanoparticles using emulsion and evaporation technique as previously reported. DEX or DEX-NPs was administered intraperitoneally to guinea pigs 1h before cisplatin administration. Auditory brainstem response (ABR) threshold shifts were measured at four frequencies (4, 8, 16, and 24kHz) 1 day before and three days after cisplatin injection. Cochlear morphology was examined to evaluate inner ear injury induced by cisplatin exposure. A single dose of DEX-NPs 1h before cisplatin treatment resulted in a significant preservation of the functional and structural properties of the cochlea, which was equivalent to the effect of multidose (3 days) DEX injection. In contrast, no significant protective effect was observed by single dose injection of DEX. The results of histological examination of the cochleae were consistent with the functional measurements. In conclusion, a single dose DEX-NPs significantly attenuated cisplatin ototoxicity in guinea pigs after systemic administration at both histological and functional levels indicating the potential therapeutic benefits of these nanoparticles for enhancing the delivery of DEX in acute sensorineural hearing loss. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  10. Characteristics of hearing-impairment among patients in Ghana ...

    African Journals Online (AJOL)

    Noise, Fever, Presbycusis, Sickness, Meningitis and Meniere's diseases were the major causes of Sensorineural Hearing Loss. Conductive Hearing Loss was attributed in the main to Wax, Foreign Bodies, Otitis Media, and Traumas. These findings have important implications on the need of resources for rehabilitation.

  11. 2 SISTERS WITH MENTAL-RETARDATION, CATARACT, ATAXIA, PROGRESSIVE HEARING-LOSS, AND POLYNEUROPATHY

    NARCIS (Netherlands)

    BEGEER, JH; SCHOLTE, FA; VANESSEN, AJ

    1991-01-01

    Two sisters are described with a disorder characterised by mental retardation, congenital cataract, progressive spinocerebellar ataxia, sensorineural deafness, and signs of peripheral neuropathy. Progressive hearing loss, ataxia, and polyneuropathy became evident in the third decade. The

  12. Risk factors for hearing loss in elderly

    Directory of Open Access Journals (Sweden)

    Kelly Vasconcelos Chaves Martins

    2012-06-01

    Full Text Available Objective: To identify risk factors related to sensorineural hearing loss in elderly. Methods: The sample consisted of 60 selected elderly, divided into two groups: the Case Group, composed by 30 individuals, 21 females and 9 males, aged at least 60 years, presenting sensorineural hearing loss, and the Control Group, composed by 30 individuals matched on gender and age, with normal hearing. The patients were submitted to audiological anamnesis and tonal audiometry. The hearing impairment was defined according to average threshold greater than 35dBNA, in the frequencies of 1,000; 2,000 and 4,000 Hz, in the best ear. Results: Statistically significant odds ratios were: a to audiological history: noise exposure and family history of deafness; b to situations involving hearing difficulty: television, church, telephone, silent environment, spatial location of sound, difficulty with voices and noisy environment; c to otologic history: tinnitus, otorrhea and nausea; and d to medical history: visual problems, smoke, alcohol, thyroid problems and kidney disease. Conclusion: The findings of this study highlighted, for sensorineural hearing loss, risk factors related to audiologic, otologic and medical history, and to situations involving hearing difficulty.

  13. MicroRNAs in sensorineural diseases of the ear

    Directory of Open Access Journals (Sweden)

    Kathy eUshakov

    2013-12-01

    Full Text Available Non-coding microRNAs have a fundamental role in gene regulation and expression in almost every multicellular organism. Only discovered in the last decade, microRNAs are already known to play a leading role in many aspects of disease. In the vertebrate inner ear, microRNAs are essential for controlling development and survival of hair cells. Moreover, dysregulation of microRNAs has been implicated in sensorineural hearing impairment, as well as in other ear diseases such as cholesteatomas, vestibular schwannomas and otitis media. Due to the inaccessibility of the ear in humans, animal models have provided the optimal tools to study microRNA expression and function, in particular mice and zebrafish. A major focus of current research has been to discover the targets of the microRNAs expressed in the inner ear, in order to determine the regulatory pathways of the auditory and vestibular systems. The potential for microRNA manipulation in development of therapeutic tools for hearing impairment is as yet unexplored, paving the way for future work in the field.

  14. Screening of Connexin 26 in Nonsyndromic Hearing Loss

    Directory of Open Access Journals (Sweden)

    Moreira, Danielle

    2014-04-01

    Full Text Available Introduction The first locus for nonsyndromic autosomal recessive hearing loss is on chromosome 13q11–22. The 35delG mutation is present in 80% of cases in which GJB2 is involved, which makes the study of this mutation very important. The viability and benefits of screening for mutations in the connexin 26 gene are now beginning to change the diagnostic evaluation and identification of the etiology of hearing loss. Objective To investigate the occurrence of the 35delG mutation in patients with nonsyndromic sensorineural hearing loss and their first degree relatives. Methods This transversal study included 72 patients from the local hospital. The patients were divided into three groups: group A, sensorineural hearing loss (n = 58; group B, first-degree relatives of group A with sensorineural hearing loss (n = 09; and group C, first-degree relatives of patients from group A without hearing loss (n = 05. All patients had audiological evaluation and genetic testing of the 35delG mutation. Results The 35delG mutation was found in four heterozygous mutations (three of them found in the same family. The other heterozygous mutation was found in a female patient with bilateral, moderate, prelingual, sensorineural hearing loss. A single homozygous mutation was found in a male patient, with severe sensorineural hearing loss in his right ear and profound hearing loss in the left ear. Conclusions The 35delG mutation was found in 7% of the cases. The test is easy to perform and inexpensive, but it is necessary to investigate other genes related to hearing loss.

  15. Screening of connexin 26 in nonsyndromic hearing loss.

    Science.gov (United States)

    Moreira, Danielle; Silva, Daniela da; Lopez, Priscila; Mantovani, Jair Cortez

    2015-01-01

    Introduction The first locus for nonsyndromic autosomal recessive hearing loss is on chromosome 13q11-22. The 35delG mutation is present in 80% of cases in which GJB2 is involved, which makes the study of this mutation very important. The viability and benefits of screening for mutations in the connexin 26 gene are now beginning to change the diagnostic evaluation and identification of the etiology of hearing loss. Objective To investigate the occurrence of the 35delG mutation in patients with nonsyndromic sensorineural hearing loss and their first degree relatives. Methods This transversal study included 72 patients from the local hospital. The patients were divided into three groups: group A, sensorineural hearing loss (n = 58); group B, first-degree relatives of group A with sensorineural hearing loss (n = 09); and group C, first-degree relatives of patients from group A without hearing loss (n = 05). All patients had audiological evaluation and genetic testing of the 35delG mutation. Results The 35delG mutation was found in four heterozygous mutations (three of them found in the same family). The other heterozygous mutation was found in a female patient with bilateral, moderate, prelingual, sensorineural hearing loss. A single homozygous mutation was found in a male patient, with severe sensorineural hearing loss in his right ear and profound hearing loss in the left ear. Conclusions The 35delG mutation was found in 7% of the cases. The test is easy to perform and inexpensive, but it is necessary to investigate other genes related to hearing loss.

  16. Sudden bilateral hearing loss after organophosphate inhalation

    OpenAIRE

    Dundar, Mehmet Akif; Derin, Serhan; Aricigil, Mitat; Eryilmaz, Mehmet Akif

    2016-01-01

    Sudden bilateral hearing loss are seen rarely and the toxic substance exposure constitutes a small part of etiology. A Fifty-eight-year-old woman admitted to our clinic with sudden bilateral hearing loss shortly after chlorpyrifos-ethyl exposure. Otolaryngologic examination findings were normal. The patient had 40 dB sensorineural hearing loss (SNHL) on the right ear and 48 dB SNHL on the left ear. Additional diagnostic tests were normal. The conventional treatment for sudden hearing loss was...

  17. [Presbycusis - Age Related Hearing Loss].

    Science.gov (United States)

    Fischer, N; Weber, B; Riechelmann, H

    2016-07-01

    Presbycusis or age related hearing loss can be defined as a progressive, bilateral and symmetrical sensorineural hearing loss due to age related degeneration of inner ear structures. It can be considered a multifactorial complex disorder with environmental and genetic factors. The molecular, electrophysiological and histological damage at different levels of the inner ear cause a progressive hearing loss, which usually affects the high frequencies of hearing. The resulting poor speech recognition has a negative impact on cognitive, emotional and social function in older adults. Recent investigations revealed an association between hearing impairment and social isolation, anxiety, depression and cognitive decline in elderly. These findings emphasize the importance of diagnosis and treating hearing loss in the elderly population. Hearing aids are the most commonly used devices for treating presbycusis. The technical progress of implantable hearing devices allows an effective hearing rehabilitation even in elderly with severe hearing loss. However, most people with hearing impairments are not treated adequately. © Georg Thieme Verlag KG Stuttgart · New York.

  18. Myringoplasty - effects on hearing and contributing factors

    African Journals Online (AJOL)

    operative air-bone gap was closed from. 23,7 dB to 13,9 dB with a 4,5% incidence of postoperative sensorineural hearing loss. Postoperatively 77,9% of patients had an air-bone gap of less than 20 dB. We conclude that myringoplasty is a beneficial.

  19. Delayed neuropsychiatric sequelae after acute hydrogen sulfide poisoning: affection of motor function, memory, vision and hearing.

    Science.gov (United States)

    Tvedt, B; Edland, A; Skyberg, K; Forberg, O

    1991-10-01

    A shipyard worker was poisoned by hydrogen sulfide (H2S), and rescued after 15-20 min. He regained consciousness after 2 days. Three days later his condition deteriorated, and he was more or less comatose for a month. When he woke up, he was amnesic, nearly blind, had reduced hearing, and had a moderate spastic tetraparesis and ataxia. Two months after the accident, he had greatly improved. Audiograms showed hearing loss with maximum at 2000 Hz and significantly poorer speech discrimination. EEG showed generalized dysrhythmia. At follow-up 5 years later he had not been able to resume his work, and had slight motor, memory and visual symptoms. CT and MRI showed slight cerebral atrophy. EEG and evoked responses were normal.

  20. Management of Conductive Hearing Loss in Children.

    Science.gov (United States)

    Dougherty, William; Kesser, Bradley W

    2015-12-01

    Conductive hearing loss (CHL), far more common than sensorineural hearing loss in children, can be acquired or congenital, can range from mild to moderately severe, and can be caused by a simple cerumen impaction, middle ear fluid, or complex middle ear abnormalities with or without the absence of the ear canal (congenital aural atresia). This article presents evidence-based recommendations for the evaluation and management of the child with both acquired and congenital CHL. Copyright © 2015 Elsevier Inc. All rights reserved.

  1. The Use of Drugs to Reduce Hearing Loss Following Acute Acoustic Trama

    Science.gov (United States)

    2012-07-01

    Methionine, Acetyl L - Carnitine or the Src protein tyrosine kinase inhibitor, KX1-004 and compared to untreated control subjects. Hearing loss...noise exposure: (1) L -N-acetylcysteine ( L -NAC); (2) D-Methionine (D-MET); (3) Ebselen SPI-1005; (4) Acetyl- L - carnitine (ALCAR) and (5) Src-PTK inhibitor...4) Acetyl- L - carnitine (ALCAR) and (5) Src-PTK inhibitor, KX1-004. Five reference/replication experimental groups and 3 control groups will be run

  2. Association between pseudoexfoliation syndrome and sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Ahmed Mohamed Kamal Elshafei

    2015-01-01

    The prevalence and severity of SNHL increases in cases of PXF compared with age-matched controls. This is not affected by the laterality of ocular involvement, or the presence or absence of cataract or glaucoma. This confirms the systemic nature of the disease and may add to difficulties during ophthalmic surgery when performed under local anesthesia due to difficulty in communication.

  3. Alström Syndrome with Acute Pancreatitis: A Case Report

    Directory of Open Access Journals (Sweden)

    Wen-Chih Wu

    2003-07-01

    Full Text Available We report the case of a 21-year-old female with Alström syndrome who also suffered from acute pancreatitis of obscure manifestation. The patient had underlying cone-rod dystrophy of the retinas, nystagmus, obesity, progressive sensorineural hearing impairment, diabetes mellitus, and hypertriglyceridemia, compatible with the clinical diagnosis of Alström syndrome. Serial examinations showed liver dysfunction and pancreatitis. In treating a patient with poor communication (i.e. cone-rod dystrophy and hearing impairment suffering from acute illness, understanding the underlying disease and the potential for pancreatitis with hypertriglyceridemia is necessary. It is also a challenge to treat a patient with multiple system involvement. In conclusion, Alström syndrome is a disease of systemic multi-organ involvement, and hepatic disease and pancreatitis, possibly due to dyslipidemia, appear to be manifestations of Alström syndrome.

  4. Radiation Therapy and Hearing Loss

    International Nuclear Information System (INIS)

    Bhandare, Niranjan; Jackson, Andrew; Eisbruch, Avraham; Pan, Charlie C.; Flickinger, John C.; Antonelli, Patrick; Mendenhall, William M.

    2010-01-01

    A review of literature on the development of sensorineural hearing loss after high-dose radiation therapy for head-and-neck tumors and stereotactic radiosurgery or fractionated stereotactic radiotherapy for the treatment of vestibular schwannoma is presented. Because of the small volume of the cochlea a dose-volume analysis is not feasible. Instead, the current literature on the effect of the mean dose received by the cochlea and other treatment- and patient-related factors on outcome are evaluated. Based on the data, a specific threshold dose to cochlea for sensorineural hearing loss cannot be determined; therefore, dose-prescription limits are suggested. A standard for evaluating radiation therapy-associated ototoxicity as well as a detailed approach for scoring toxicity is presented.

  5. Masker phase effects in normal-hearing and hearing-impaired listeners: evidence for peripheral compression at low signal frequencies

    DEFF Research Database (Denmark)

    Oxenham, Andrew J.; Dau, Torsten

    2004-01-01

    curvature. Results from 12 listeners with sensorineural hearing loss showed reduced masker phase effects, when compared with data from normal-hearing listeners, at both 250- and 1000-Hz signal frequencies. The effects of hearing impairment on phase-related masking differences were not well simulated...... are affected by a common underlying mechanism, presumably related to cochlear outer hair cell function. The results also suggest that normal peripheral compression remains strong even at 250 Hz....

  6. Congenital hereditary endothelial dystrophy with progressive sensorineural deafness (Harboyan syndrome

    Directory of Open Access Journals (Sweden)

    Abramowicz Marc

    2008-10-01

    Full Text Available Abstract Harboyan syndrome is a degenerative corneal disorder defined as congenital hereditary endothelial dystrophy (CHED accompanied by progressive, postlingual sensorineural hearing loss. To date, 24 cases from 11 families of various origin (Asian Indian, South American Indian, Sephardi Jewish, Brazilian Portuguese, Dutch, Gypsy, Moroccan, Dominican have been reported. More than 50% of the reported cases have been associated with parental consanguinity. The ocular manifestations in Harboyan syndrome include diffuse bilateral corneal edema occurring with severe corneal clouding, blurred vision, visual loss and nystagmus. They are apparent at birth or within the neonatal period and are indistinguishable from those characteristic of the autosomal recessive CHED (CHED2. Hearing deficit in Harboyan is slowly progressive and typically found in patients 10–25 years old. There are no reported cases with prelinglual deafness, however, a significant hearing loss in children as young as 4 years old has been detected by audiometry, suggesting that hearing may be affected earlier, even at birth. Harboyan syndrome is caused by mutations in the SLC4A11 gene located at the CHED2 locus on chromosome 20p13-p12, indicating that CHED2 and Harboyan syndrome are allelic disorders. A total of 62 different SLC4A11 mutations have been reported in 98 families (92 CHED2 and 6 Harboyan. All reported cases have been consistent with autosomal recessive transmission. Diagnosis is based on clinical criteria, detailed ophthalmological assessment and audiometry. A molecular confirmation of the clinical diagnosis is feasible. A variety of genetic, metabolic, developmental and acquired diseases presenting with clouding of the cornea should be considered in the differential diagnosis (Peters anomaly, sclerocornea, limbal dermoids, congenital glaucoma. Audiometry must be performed to differentiate Harboyan syndrome from CHED2. Autosomal recessive types of CHED (CHED2 and

  7. Sudden bilateral hearing loss after organophosphate inhalation

    Directory of Open Access Journals (Sweden)

    Mehmet Akif Dundar

    2016-12-01

    Full Text Available Sudden bilateral hearing loss are seen rarely and the toxic substance exposure constitutes a small part of etiology. A Fifty-eight-year-old woman admitted to our clinic with sudden bilateral hearing loss shortly after chlorpyrifos-ethyl exposure. Otolaryngologic examination findings were normal. The patient had 40 dB sensorineural hearing loss (SNHL on the right ear and 48 dB SNHL on the left ear. Additional diagnostic tests were normal. The conventional treatment for sudden hearing loss was performed. On the second week following organophosphate (OP exposure the patient's hearing loss almost completely resolved. OP's are heavily used in agriculture and should be taken into consideration as an etiologic factor in sudden hearing loss. Keywords: Organophosphates, Hearing loss, Sudden

  8. Effects of noise reduction on speech intelligibility, perceived listening effort, and personal preference in hearing-impaired listeners

    NARCIS (Netherlands)

    Brons, Inge; Houben, Rolph; Dreschler, Wouter A.

    2014-01-01

    This study evaluates the perceptual effects of single-microphone noise reduction in hearing aids. Twenty subjects with moderate sensorineural hearing loss listened to speech in babble noise processed via noise reduction from three different linearly fitted hearing aids. Subjects performed (a)

  9. Analysis of caloric test responses in sudden hearing loss.

    Science.gov (United States)

    Shih, Cheng-Ping; Chou, Yu-Ching; Chen, Hsin-Chien; Lee, Jih-Chin; Chu, Yueng-Hsiang; Wang, Chih-Hung

    2017-02-01

    Sudden sensorineural hearing loss is characterized by a rapid-onset hearing loss that develops within 3 days. Vertigo may also be present. We conducted a retrospective study to investigate whether the severity of a loss of caloric function is associated with the initial hearing loss and with hearing recovery. Our study population was made up of 135 patients-67 men and 68 women, aged 25 to 71 years (mean: 50.9)-with sudden sensorineural hearing loss who had undergone bithermal caloric testing. We compared various patient factors according to patients' hearing level and their response to caloric testing. We also analyzed the canal paresis (CP) value in patients with an abnormal caloric response according to three factors: disease severity, vertigo, and hearing recovery, and we evaluated the correlation between the loss of caloric function and hearing outcomes. We found that an abnormal caloric response was significantly associated with a profound hearing loss at presentation, the presence of vertigo, and poor hearing recovery. Among patients with an abnormal caloric response, the CP value was significantly correlated with hearing recovery (r = 0.503, p = 0.001). Poor hearing recovery was seen in 80% of patients with a CP value of ≥40% but in only 25% of patients with a value of hearing recovery was worse in the patients with a CP value of ≥40% (p = 0.002). We conclude that a CP value of ≥40% is a significant prognostic factor for an unfavorable treatment outcome.

  10. Speech-evoked auditory brainstem responses in children with hearing loss.

    Science.gov (United States)

    Koravand, Amineh; Al Osman, Rida; Rivest, Véronique; Poulin, Catherine

    2017-08-01

    The main objective of the present study was to investigate subcortical auditory processing in children with sensorineural hearing loss. Auditory Brainstem Responses (ABRs) were recorded using click and speech/da/stimuli. Twenty-five children, aged 6-14 years old, participated in the study: 13 with normal hearing acuity and 12 with sensorineural hearing loss. No significant differences were observed for the click-evoked ABRs between normal hearing and hearing-impaired groups. For the speech-evoked ABRs, no significant differences were found for the latencies of the following responses between the two groups: onset (V and A), transition (C), one of the steady-state wave (F), and offset (O). However, the latency of the steady-state waves (D and E) was significantly longer for the hearing-impaired compared to the normal hearing group. Furthermore, the amplitude of the offset wave O and of the envelope frequency response (EFR) of the speech-evoked ABRs was significantly larger for the hearing-impaired compared to the normal hearing group. Results obtained from the speech-evoked ABRs suggest that children with a mild to moderately-severe sensorineural hearing loss have a specific pattern of subcortical auditory processing. Our results show differences for the speech-evoked ABRs in normal hearing children compared to hearing-impaired children. These results add to the body of the literature on how children with hearing loss process speech at the brainstem level. Copyright © 2017 Elsevier B.V. All rights reserved.

  11. Conductive hearing loss in four dogs associated with the use of ointment-based otic medications.

    Science.gov (United States)

    Cole, Lynette K; Rajala-Schultz, Päivi J; Lorch, Gwendolen

    2018-04-17

    Hearing loss (HL) is classified as conductive when sound transmission is compromised in the ear canal or middle ear, or sensorineural when there is an abnormality of the receptor cells of the cochlea or auditory pathway. Hearing in dogs is evaluated using the brainstem auditory evoked response (BAER) test. Our objective was to characterize BAER findings pre- and post-ear flushing in four dogs with acute HL following application of an ointment-based otic medication containing betamethasone, clotrimazole and gentamicin in a mineral oil-based system containing a plasticized hydrocarbon gel. Dogs, ranging from 9 to 11 years old, that had been treated with the otic medication for one to three weeks prior to hearing loss and on otoscopic examination had evidence of medication in the horizontal ear canals. Dogs were anaesthetized for an ear flush to remove the medication from the ear canals. Hearing was assessed using BAER testing, measurements were initiated with 116 decibel peak equivalent sound pressure level (dBpeSPL) click. Estimated threshold was defined as the lowest intensity in dB in which wave V was still present. Post-ear flush the estimated threshold improved in both ears of all dogs (mean 22.3 dB; range 13-41 dB), confirming conductive HL due to the otic medication. All owners noted an improvement in their dog's hearing post-ear flush, validating the BAER findings. These results emphasize the importance of an ear flush to remove otic medications in dogs that experience acute HL, to determine if the HL is conductive, and if so, to restore hearing. © 2018 ESVD and ACVD.

  12. Systemic steroid reduces long-term hearing loss in experimental pneumococcal meningitis

    DEFF Research Database (Denmark)

    Worsøe, Lise Lotte; Brandt, C.T.; Lund, S.P.

    2010-01-01

    Sensorineural hearing loss is a common complication of pneumococcal meningitis. Treatment with corticosteroids reduces inflammatory response and may thereby reduce hearing loss. However, both experimental studies and clinical trials investigating the effect of corticosteroids on hearing loss have...... generated conflicting results. The objective of the present study was to determine whether systemic steroid treatment had an effect on hearing loss and cochlear damage in a rat model of pneumococcal meningitis....

  13. Occupational hearing loss of market mill workers in the city of Accra, Ghana

    OpenAIRE

    Emmanuel D Kitcher; Grace Ocansey; Benjamin Abaidoo; Alidu Atule

    2014-01-01

    Noise induced hearing loss (NIHL) is an irreversible sensorineural hearing loss associated with exposure to high levels of excessive noise. Prevention measures are not well established in developing countries. This comparative cross sectional study aims to determine the prevalence of hearing loss in both a group of high risk workers and a control group and to assess their knowledge of the effects of noise on hearing health. A total of 101 market mill workers and 103 controls employed within m...

  14. UNILATERAL SENSORINEURAL DEAFNESS IN SCHOOL CHILDREN; A CLINICAL AND AUDIOLOGICAL EVALUATION AT A TERTIARY HOSPITAL OF TELANGANA

    Directory of Open Access Journals (Sweden)

    Harish Chandra Reddy

    2016-01-01

    Full Text Available INTRODUCTION Interpersonal communication, day to day activities, learning language and acquiring grades in the school are important parts in the life of school going children. Unilateral or bilateral Loss of hearing affects education and the child feels handicapped socially, emotionally, and scholastically. Such children lose grades and retained in the same class affecting them emotionally. The present study was conducted in order to find out the various causes of unilateral Sensorineural Hearing Loss (USNHL in children and to study role of various audiological findings in diagnosing the cause of impaired hearing in children. AIMS AND OBJECTIVES To find out the possible etiological causes and study the role of various audiological tests in finding the causes of unilateral impaired hearing in children. MATERIALS AND METHODS A prospective study was conducted in the Department of ENT of a tertiary Hospital attached to a Medical College in Warangal, Telangana. A total of 346 children aged between 4 and 15 years attended presenting with complaints of impaired hearing. Among them 73 children with unilateral sensorineural deafness were included in the study. After a thorough ENT examination subjective tests like pure tone audiometry and behavioral observation audiometry and objective tests like impedance audiometry, Oto-acoustic emissions (OAE, and brainstem evoked response audiometry (BERA were performed. RESULTS While identifying the possible etiological cause of USNHL, OAE revealed 56.16% REFER and 43.83% PASS results. Impedance audiometric results showed 71.22% as Type A Tympanogram. Pure tone audiometric results were mostly showed severe and profound hearing losses together accounting to 87.66%. BERA findings were mostly on severe and profound hearing loss (90.40%. No children with syndromic or cranio-facial abnormalities were found. CONCLUSIONS A prospective Hospital based study of school children with USNHL showed acquired nature of the

  15. Viral Causes of Hearing Loss: A Review for Hearing Health Professionals

    Science.gov (United States)

    Cohen, Brandon E.; Durstenfeld, Anne

    2014-01-01

    A number of viral infections can cause hearing loss. Hearing loss induced by these viruses can be congenital or acquired, unilateral or bilateral. Certain viral infections can directly damage inner ear structures, others can induce inflammatory responses which then cause this damage, and still others can increase susceptibility or bacterial or fungal infection, leading to hearing loss. Typically, virus-induced hearing loss is sensorineural, although conductive and mixed hearing losses can be seen following infection with certain viruses. Occasionally, recovery of hearing after these infections can occur spontaneously. Most importantly, some of these viral infections can be prevented or treated. For many of these viruses, guidelines for their treatment or prevention have recently been revised. In this review, we outline many of the viruses that cause hearing loss, their epidemiology, course, prevention, and treatment. PMID:25080364

  16. Sudden bilateral hearing loss after organophosphate inhalation.

    Science.gov (United States)

    Dundar, Mehmet Akif; Derin, Serhan; Aricigil, Mitat; Eryilmaz, Mehmet Akif

    2016-12-01

    Sudden bilateral hearing loss are seen rarely and the toxic substance exposure constitutes a small part of etiology. A Fifty-eight-year-old woman admitted to our clinic with sudden bilateral hearing loss shortly after chlorpyrifos-ethyl exposure. Otolaryngologic examination findings were normal. The patient had 40 dB sensorineural hearing loss (SNHL) on the right ear and 48 dB SNHL on the left ear. Additional diagnostic tests were normal. The conventional treatment for sudden hearing loss was performed. On the second week following organophosphate (OP) exposure the patient's hearing loss almost completely resolved. OP's are heavily used in agriculture and should be taken into consideration as an etiologic factor in sudden hearing loss.

  17. Newborn hearing screening.

    Science.gov (United States)

    Stewart, D L; Pearlman, A

    1994-11-01

    Congenital deafness is a relatively common problem with an incidence of 1/300 to 1/1000. Most states have no mass screening program for hearing loss, but the state of Kentucky compiles a High Risk Registry which is a historical survey of parents relating to risk factors for hearing loss. Unfortunately this survey can miss 50% of those who have a hearing deficit. If not detected prior to discharge, there is often a delay in diagnosis of deafness which prevents early intervention. We report 2 years' experience at Kosair Children's Hospital where 1,987 infants admitted to well baby, intermediate, or intensive care nurseries were screened using the ALGO-1 screener (Natus Medical Inc, Foster City, CA) which is a modified auditory brain stem evoked response (ABR). Our screening of this population led to an 11% incidence of referral for complete audiological evaluation. There were no significant complications. Forty-eight infants were found to have nonspecified, sensorineural, or conductive hearing loss. The positive predictive value of the test was 96%. Therefore, we feel that the use of the modified ABR in the newborn is a timely, cost efficient method of screening for hearing loss and should be used for mass screening of all newborns.

  18. Inner ear and facial nerve complications of acute otitis media with focus on bacteriology and virology.

    Science.gov (United States)

    Hydén, Dag; Akerlind, Britt; Peebo, Markus

    2006-05-01

    sensorineural hearing loss and vertigo, three had vertigo as an isolated symptom and one, with bilateral AOM, had bilateral sensorineural hearing loss. Seven patients had a combination of facial palsy and inner ear symptoms (unilateral sensorineural hearing loss in three, unilateral sensorineural hearing loss and vertigo in two, bilateral sensorineural hearing loss and vertigo in one, with bilateral AOM, and vertigo alone in one). One patient had an isolated facial palsy. Healing was complete in 11 of the 20 patients. In seven patients a minor defect remained at follow-up (a sensorineural hearing loss at higher frequencies in all). Only two patients had obvious defects (a pronounced hearing loss in combination with a moderate to severe facial palsy (House-Brackman grade 4) in one, distinct vestibular symptoms and a total caloric loss in combination with a high-frequency loss in the other. Eight patients had positive bacteriological cultures from middle ear contents: Streptococcus pneumoniae in two, beta-hemolytic Streptococcus group A in two, beta-hemolytic Streptococcus group A together with Staphylococcus aureus in one, Staph. aureus alone in one and coagulase-negative staphylococci (interpreted as pathogens) in two. In the 12 patients with negative cultures, there was a probable bacteriological cause due to the outcome in SR/CRP and leukocyte count in five. In four patients serological testing showed a concomitant viral infection that was interpreted to be the cause (varicella zoster virus in two, herpes simplex virus in one and adenovirus in one.) In three there was a probable viral cause despite negative viral antibody test due to normal outcome in SR/CRP, normal leukocyte count, serous fluid at myringotomy and a relatively short pre-complication antibiotic treatment period.

  19. Genetic mutation susceptibility of hearing loss in child with severe neonatal jaundice

    International Nuclear Information System (INIS)

    Zahedi, F.D.; Rahman, R.A.; Abdullah, A.

    2015-01-01

    This case report demonstrates a case of 5-year-old non-syndromic Malay boy who passed the hearing screening test however he was confirmed has bilateral profound sensorineural hearing loss diagnosed at 3 months of age by brain stem evoked response (BSER). He has background history of severe neonatal jaundice and male siblings of hearing impairment. The antenatal and birth history was uneventful apart from maternal hypothyroidism. His other two elder brothers have bilateral sensorineural hearing loss and history of severe neonatal jaundice as well. The ear examinations, computed tomography scan and magnetic resonance imaging revealed normal findings. Right sided cochlear implantation was done at the age of 3 years old and he is still under audiology follow-up. Conclusion: Genetic studies are important to determine the cause of genetic mutation in susceptibility to hearing impairment that run in his family after severe neonatal jaundice. Those baby with risk of developing hearing loss required diagnostic hearing assessment. (author)

  20. Current amplification models of sensorineurall and conductive hearing loss

    Directory of Open Access Journals (Sweden)

    Ostojić Sanja

    2012-01-01

    Full Text Available The main function of a hearing aid is to improve auditory and language abilities of hearing impaired users. The amplification model has to be adapted according to age, degree and type of hearing loss. The goal of this paper is to analyze the current amplification models of sensorineural and conductive hearing loss which can provide a high quality of speech perception and sounds at any degree of hearing loss. The BAHA is a surgically implantable system for treatment of conductive hearing loss that works through direct bone conduction. BAHA is used to help people with chronic ear infections, congenital external auditory canal atresia and single sided deafness who cannot benefit from conventional hearing aids. The last generation of hearing aid for sensorineural hearing loss is cochlear implant. Bimodal amplification improves binaural hearing. Hearing aids alone do not make listening easier in all situations. The things that can interfere with listening are background noises, distance from a sound and reverberation or echo. The device used most often today is the Frequency Modulated (FM system.

  1. Does the use of recombinant TSH in preparation for I-131 scintigraphy scan affect hearing function?

    Directory of Open Access Journals (Sweden)

    Mehtap Doğan

    2018-03-01

    Conclusion: Sensorineural hearing loss was detected, especially at low frequencies, in patients with DTC after surgical treatment whose hormone replacement therapy was withdrawn but not in those receiving rhTSH. It is therefore preferred to use rhTSH when preparing for I-131 scintigraphy scan in patients at risk for hearing loss.

  2. Audiometric Characteristics of a Dutch DFNA10 Family With Mid-Frequency Hearing Impairment

    NARCIS (Netherlands)

    Beelen, E. van; Oonk, A.M.M.; Leijendeckers, J.M.; Hoefsloot, E.H.; Pennings, R.J.E.; Feenstra, I.; Dieker, H.J.; Huygen, P.L.M.; Snik, A.F.M.; Kremer, H.; Kunst, H.P.M.

    2016-01-01

    OBJECTIVES: Mutations in EYA4 can cause nonsyndromic autosomal dominant sensorineural hearing impairment (DFNA10) or a syndromic variant with hearing impairment and dilated cardiomyopathy. A mutation in EYA4 was found in a Dutch family, causing DFNA10. This study is focused on characterizing the

  3. Hearing impairment in children with congenital cytomegalovirus (CMV) infection based on distortion product otoacoustic emissions (DPOAE) and brain evoked response audiometry stimulus click (BERA Click) examinations

    Science.gov (United States)

    Airlangga, T. J.; Mangunatmadja, I.; Prihartono, J.; Zizlavsky, S.

    2017-08-01

    Congenital cytomegalovirus (congenital CMV) infection is a leading factor of nongenetic sensorineural hearing loss in children. Hearing loss caused by CMV infection does not have a pathognomonic configuration hence further research is needed. The development of knowledge on hearing loss caused by congenital CMV infection is progressing in many countries. Due to a lack of research in the context of Indonesia, this study assesses hearing impairment in children with congenital CMV infection in Indonesia, more specifically in the Cipto Mangunkusumo Hospital. Our objective was to profile hearing impairment in children 0-5 years of age with congenital CMV infection using Distortion Product Otoacoustic Emissions (DPOAE) and Brain Evoked Response Audiometry Stimulus Click (BERA Click) examinations. This cross-sectional study was conducted in the Cipto Mangunkusum Hospital from November, 2015 to May 2016 with 27 children 0-5 years of age with congenital CMV infection. Of individual ears studied, 58.0% exhibited sensorineural hearing loss. There was a significant relationship between developmental delay and incidence of sensorineural hearing loss. Subjects with a developmental delay were 6.57 times more likely (CI 95%; 1.88-22.87) to experience sensorineural hearing loss. Congenital CMV infection has an important role in causing sensorineural hearing loss in children.

  4. Vowel production of Mandarin-speaking hearing aid users with different types of hearing loss.

    Directory of Open Access Journals (Sweden)

    Yu-Chen Hung

    Full Text Available In contrast with previous research focusing on cochlear implants, this study examined the speech performance of hearing aid users with conductive (n = 11, mixed (n = 10, and sensorineural hearing loss (n = 7 and compared it with the speech of hearing control. Speech intelligibility was evaluated by computing the vowel space area defined by the Mandarin Chinese corner vowels /a, u, i/. The acoustic differences between the vowels were assessed using the Euclidean distance. The results revealed that both the conductive and mixed hearing loss groups exhibited a reduced vowel working space, but no significant difference was found between the sensorineural hearing loss and normal hearing groups. An analysis using the Euclidean distance further showed that the compression of vowel space area in conductive hearing loss can be attributed to the substantial lowering of the second formant of /i/. The differences in vowel production between groups are discussed in terms of the occlusion effect and the signal transmission media of various hearing devices.

  5. Auditory Memory deficit in Elderly People with Hearing Loss

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    Zahra Shahidipour

    2013-06-01

    Full Text Available Introduction: Hearing loss is one of the most common problems in elderly people. Functional side effects of hearing loss are various. Due to the fact that hearing loss is the common impairment in elderly people; the importance of its possible effects on auditory memory is undeniable. This study aims to focus on the hearing loss effects on auditory memory.   Materials and Methods: Dichotic Auditory Memory Test (DVMT was performed on 47 elderly people, aged 60 to 80; that were divided in two groups, the first group consisted of elderly people with hearing range of 24 normal and the second one consisted of 23 elderly people with bilateral symmetrical ranged from mild to moderate Sensorineural hearing loss in the high frequency due to aging in both genders.   Results: Significant difference was observed in DVMT between elderly people with normal hearing and those with hearing loss (P

  6. The benefit of amplification on auditory working memory function in middle-aged and young-older hearing impaired adults

    OpenAIRE

    Doherty, Karen A.; Desjardins, Jamie L.

    2015-01-01

    Untreated hearing loss can interfere with an individual’s cognitive abilities and intellectual function. Specifically, hearing loss has been shown to negatively impact working memory function, which is important for speech understanding, especially in difficult or noisy listening conditions. The purpose of the present study was to assess the effect of hearing aid use on auditory working memory function in middle-aged and young-older adults with mild to moderate sensorineural hearing loss. P...

  7. Vocabulary and Working Memory in Children Fit with Hearing Aids

    Science.gov (United States)

    Stiles, Derek J.; McGregor, Karla K.; Bentler, Ruth A.

    2012-01-01

    Purpose: To determine whether children with mild-to-moderately severe sensorineural hearing loss (CHL) present with disturbances in working memory and whether these disturbances relate to the size of their receptive vocabularies. Method: Children 6 to 9 years of age participated. Aspects of working memory were tapped by articulation rate, forward…

  8. Effect of hemodialysis on the hearing function of patients with ...

    African Journals Online (AJOL)

    Sensorineural hearing impairment (SHI) has been reported in chronic renal failure (CRF) patients with a prevalence of 20-40%. The aetiopathogenetic mechanisms reported included osmotic alteration resulting in loss of hair cells and in some, complications of haemodialysis have been hypothesized. We have in the past ...

  9. Congenital hearing loss. Is CT enough? | Agha | Alexandria Journal ...

    African Journals Online (AJOL)

    Patients and methods: This is a prospective study including 60 patients, 24 males and 36 females aged from 1 to 7 years, who were presented by unilateral or bilateral congenital conductive (CHL) or/and sensorineural hearing loss (SNHL). All patients were evaluated by HRCT scan with post-processing multiplanar ...

  10. Prognostic factors in sudden hearing loss

    Science.gov (United States)

    Enache, R

    2008-01-01

    Sudden hearing loss is a sensorineural hypoacusis, unilateral in most of the cases, with an incidence peak in young adult. This article is the result of a four years retrospective study, in which we have tried to see how age, severity of hearing loss, presence or absence of vertigo, the timing of treatment initiation can influence the hearing recovery. 47 patients were included in this inpatient study. They were investigated (clinical, lab and imaging studies) and the treatment protocol included vasodilators, anti–inflammatory agents, vitamins, plasma expander. In conclusion, a good prognosis in the hearing recovery was associated with absence of vertigo, early treatment (the first 7 days), and hearing loss less than 50dB. Age had no influence upon the recovery process. PMID:20108511

  11. Elderly With Different Types of Hearing Loss and Comorbidities: Satisfaction With Hearing Aids

    Directory of Open Access Journals (Sweden)

    Dashti

    2015-07-01

    Full Text Available Background Hearing loss is developing when age is rising. Initiation and progression rates of hearing loss vary among different individuals and groups. Objectives The current study aimed to determine satisfaction of the elderly with their hearing aids in different types of hearing loss and comorbidities. Patients and Methods The study was conducted on 40 elderly subjects suffering from hearing loss and using hearing aids. The data collection method included assessment of hearing loss in addition to using a questionnaire to estimate respondents' satisfaction with their hearing aids in daily life. The Persian version of the Satisfaction with Amplification in Daily Life (SADL questionnaire was administered. The data were analyzed using descriptive and inferential statistics by SPSS software version19. Results The mean satisfaction scores of the elderly were 4.83 ± 0.51 and 5.36 ± 0.30 in the sensorineural loss groups. There was no significant difference between different comorbidities. There was a significant difference between satisfaction level of cost and services subscales in the symmetrical styles of hearing loss (P value = 0.04. Conclusions The findings of the study indicated a high satisfaction of the elderly with their hearing aids, considering the type of hearing loss. Despite all the efforts to improve the audiologic services during verification process, the elderly should be consulted specifically in order to fit their hearing aid as well as their expectations from aid.

  12. Acute Hearing Loss Caused by Decreasing Anterior Inferior Cerebellar Arterial Perfusion in a Patient with Vertebral Artery Stenosis.

    Science.gov (United States)

    Fukuda, Rintaro; Miyamoto, Nobukazu; Hayashida, Arisa; Ueno, Yuji; Yamashiro, Kazuo; Tanaka, Ryota; Hattori, Nobutaka

    2017-06-01

    We report a case of bilateral hearing loss caused by decreased vascular flow in the anterior inferior cerebellar artery (AICA) territory. A 74-year-old man who experienced right hearing loss 5 months ago presented with bilateral deafness and right cerebellar ataxia; however, no ischemic lesion was detected in the bilateral AICA area. After stroke treatment, hearing loss was improved. One month later, we obtained blood flow improvement in the left AICA territory on single-photon-emission computed tomography and vertebral artery stenosis on magnetic resonance angiography. Therefore, clinicians should recognize that bilateral hearing loss may be related to stroke in the vertebrobasilar artery area. Copyright © 2017 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  13. No Association Between Time of Onset of Hearing Loss (Childhood Versus Adulthood) and Self-Reported Hearing Handicap in Adults.

    Science.gov (United States)

    Aarhus, Lisa; Tambs, Kristian; Engdahl, Bo

    2015-12-01

    This study examined the association between time of onset of hearing loss (childhood vs. adulthood) and self-reported hearing handicap in adults. This is a population-based cohort study of 2,024 adults (mean = 48 years) with hearing loss (binaural pure-tone average 0.5-4 kHz ≥ 20 dB HL) who completed a hearing handicap questionnaire. In childhood, the same persons (N = 2,024) underwent audiometry in a school investigation (at ages 7, 10, and 13 years), in which 129 were diagnosed with sensorineural hearing loss (binaural pure-tone average 0.5-4 kHz ≥ 20 dB HL), whereas 1,895 had normal hearing thresholds. Hearing handicap was measured in adulthood as the sum-score of various speech perception and social impairment items (15 items). The sum-score increased with adult hearing threshold level (p handicap sum-score between the group with childhood-onset hearing loss (n = 129) and the group with adult-onset hearing loss (n = 1,895; p = .882). Self-reported hearing handicap in adults increased with hearing threshold level. After adjustment for adult hearing threshold level, this cohort study revealed no significant association between time of onset of hearing loss (childhood vs. adulthood) and self-reported hearing handicap.

  14. Electromagnetic semi-implantable hearing device: phase I. Clinical trials.

    Science.gov (United States)

    McGee, T M; Kartush, J M; Heide, J C; Bojrab, D I; Clemis, J D; Kulick, K C

    1991-04-01

    Conventional hearing aids have improved significantly in recent years; however, amplification of sound within the external auditory canal creates a number of intrinsic problems, including acoustic feedback and the need for a tight ear mold to increase usable gain. Nonacoustic alternatives which could obviate these encumbrances have not become practical due to inefficient coupling (piezoelectric techniques) or unfeasible power requirements (electromagnetic techniques). Recent technical advances, however, prompted a major clinical investigation of a new electromagnetic, semi-implantable hearing device. This study presents the details of clinical phase I, in which an electromagnetic driver was coupled with a target magnet temporarily affixed onto the lateral surface of the malleus of six hearing aid users with sensorineural losses. The results indicate that the electromagnetic hearing device provides sufficient gain and output characteristics to benefit individuals with sensorineural hearing loss. Significant improvements compared to conventional hearing aids were noted in pure-tone testing and, to a lesser degree, in speech discrimination. Subjective responses were quite favorable, indicating that the electromagnetic hearing device 1. produces no acoustic feedback; 2. works well in noisy environments; and 3. provides a more quiet, natural sound than patients' conventional hearing aids. These favorable results led to phase II of the project, in which patients with surgically amendable mixed hearing losses were implanted with the target magnet incorporated within a hydroxyapatite ossicular prosthesis. The results of this second-stage investigation were also encouraging and will be reported separately.

  15. A Review of the Literature on Large Vestibular Aqueduct Syndrome for Teachers of the Deaf and Hard of Hearing.

    Science.gov (United States)

    Vause, Kellie; Beattie, R. G.

    1997-01-01

    This review describes the history, clinical features, methods of diagnosis, and treatment of (1) large vestibular aqueduct syndrome (LVAS), a condition involving congenital hearing loss that is purely sensorineural or mixed in nature, and (2) progressive stepwise hearing losses following minor head trauma. Emphasis is on prevention of further…

  16. Impact of Early Intervention on Expressive and Receptive Language Development among Young Children with Permanent Hearing Loss

    Science.gov (United States)

    Meinzen-Derr, Jareen; Wiley, Susan; Choo, Daniel I.

    2011-01-01

    Along with early detection, early intervention (EI) is critical for children identified with hearing loss. Evidence indicates that many children with sensorineural hearing loss experience improved language abilities if EI services were initiated at an "early" age. The present study's objectives were to determine the impact of a state EI program on…

  17. Vibrant Soundbridge rehabilitation of conductive and mixed hearing loss.

    Science.gov (United States)

    Lüers, Jan-Christoffer; Hüttenbrink, Karl-Bernd

    2014-12-01

    The Vibrant Soundbridge is the world's most often implanted active middle ear implant or hearing aid. During the last few years, the device indications have expanded from sensorineural hearing loss to conductive and mixed hearing loss. Titanium couplers have led to improved contact of the floating mass transducer with the middle ear structures. The resulting hearing gain is satisfying for most patients, but so far, there is no clear audiologic advantage over conventional hearing aids. Currently, the indications are mainly related to intolerance of conventional hearing aids (eg, chronic otitis externa), severe mixed hearing loss with a destructed middle ear and certain medical diagnosis (eg, congenital atresia). Copyright © 2014 Elsevier Inc. All rights reserved.

  18. ANALYTICAL STUDY OF SENSORY NEURAL HEARING LOSS IN CSOM WITH AND WITHOUT CHOLESTEATOMA

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    Kabikanta Samantaray

    2016-07-01

    Full Text Available BACKGROUND Chronic suppurative otitis media still remains a major cause of conductive hearing loss in our country. But, a few patients also display an added sensorineural hearing loss. MATERIALS & METHODS Hundred patients with CSOM undergoing surgery at our department were included in the study. The affected ears formed the ‘CSOM Group’ and the normal ears formed the ‘Control group’. Detailed otological history, clinical, surgical and audiometric findings were recorded and analysed. RESULTS It was inferred that CSOM associated with sensorineural hearing loss was found in small number of patients only. No correlation was established between duration of discharge and sensorineural loss. CONCLUSION Though greater SN loss was seen in patients of CSOM with cholesteatoma it was not statistically significant. Whether an early surgery in CSOM can prevent SN loss or not needs further studies.

  19. Hearing aid-related satisfaction based on type and degree of hearing loss in elderly

    Directory of Open Access Journals (Sweden)

    Farzad FarajiKhiavi

    2015-02-01

    Full Text Available Background and Aim: Hearing loss is one of the most prevalent chronic diseases in the elderly; using a hearing aid to alleviate auditory impairment can positively affect their quality of life. This research aimed to determine the level of satisfaction concerning hearing aids in elderly people with hearing impairment based on the type and degree of hearing loss.Methods: An analytic cross-sectional research design was used ; the sample included 40 elderly people who used hearing aids. According to the World Health Organization (WHO age classification, participants were divided into two age groups: 65-74 years (n=20 and 75-90 years (n=20. Satisfaction levels were assessed using a standard satisfaction with amplification in daily life (SADL questionnaire.Results: Satisfaction levels in the 65-74 age group were significantly higher than that in the 75-90 age group (p=0.02. Participants with mixed hearing loss revealed higher satisfaction levels than participants with sensorineural hearing loss (p=0.02. On the negative effects dimension, participants with severe hearing loss exhibited significantly higher satisfaction levels than participants with moderate or moderate to severe hearing loss (p=0.01.Conclusion: Total satisfaction mean scores were relatively high in the elderly participants . Negative features could be reduced via careful consultation regarding the aids’ amplifying capabilities and limitations in groups with moderate or moderate to severe hearing loss.

  20. Comparison of Carina active middle-ear implant with conventional hearing aids for mixed hearing loss.

    Science.gov (United States)

    Savaş, V A; Gündüz, B; Karamert, R; Cevizci, R; Düzlü, M; Tutar, H; Bayazit, Y A

    2016-04-01

    To compare the auditory outcomes of Carina middle-ear implants with those of conventional hearing aids in patients with moderate-to-severe mixed