WorldWideScience

Sample records for acute sensorineural hearing

  1. Labyrinthine window rupture as a cause of acute sensorineural hearing loss

    OpenAIRE

    Nagai, Tomoyuki; Nagai, Midori

    2011-01-01

    Labyrinthine window rupture (LWR) is one cause of acute sensorineural hearing loss and need for early exploration is clear for good improved hearing. Acute sensorineural hearing loss of 60?dB or more treated from May 2006 to May 2010 were retrospectively analyzed. There were 21 ears of severe deafness, 18 ears of profound deafness, and 10 ears of total deafness. All patients were examined with temporal bone CT. Space-occupying lesions around the labyrinthine windows were suggestive images of ...

  2. Acute profound sensorineural hearing loss as the initial manifestation of acute leukemia--report of a case.

    Science.gov (United States)

    Harada, T; Namiki, S; Kawabata, I

    2000-10-01

    This paper reports an unusual case in which acute lymphocytic leukemia presented acute profound sensorineural hearing loss as the initial manifestation of the disease. The patient is a 55-year-old woman who complained of left hearing loss of sudden onset. Pure tone audiometry revealed profound sensorineural hearing loss of the left ear at mid and low frequencies. The patient was tentatively diagnosed as idiopathic sudden deafness and admitted for the treatment, but her laboratory data indicated that she was at an advanced stage of leukemia. The patient's hearing loss did not improve subjectively until she deceased 1 year after the admission. The mechanism producing acute hearing loss in leukemic patients is reviewed and discussed, and the importance of differentiating possible underlying diseases before we diagnose idiopathic sudden deafness is stressed.

  3. 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.

  4. [Acute low tone sensorineural hearing loss caused by inner ear barotrauma].

    Science.gov (United States)

    Abe, T; Sasamori, S; Oikawa, T; Aigami, T; Endo, Y; Matsuki, C

    1989-09-01

    Three cases of inner ear barotrauma with subjective symptoms and hearing impairment which were similar to the low tone sudden deafness were reported. Case 1 was a 34-year-old man who developed a hearing loss in the next morning of taking an airplane and recovered four days after. Case 2 was a 42-year-old man who developed a hearing loss 2 days after flying in an airplane and hearing loss have recurred 4 times in his right ear for 3 months. Eight months after recovery of previous recurrent attack, a hearing loss occurred in his left ear without flying and recurred twice for 3 weeks. Case 3 was a 25-year-old woman who developed a hearing loss in the right ear after 24 meter depth scuba diving and recurred 4 times for 40 days. Those three patients complained of no vertigo at any attacks and were treated conservatively. From previous reports and the onset and the course of hearing disturbance, acute low tone sensorineural hearing loss in case 1 and case 2 was thought to be caused by circulatory disturbance of the inner ear and in case 3 thought to be caused by inner ear window rupture. But, endolymphatic hydrops was also needed to be take into account in those three cases as a common possible cause. Inner ear barotrauma and so called labyrinthine window rupture were considered to be one of the diseases needed to differentiate from low tone sudden deafness without reference to mono-attack type or recurrent type.

  5. Pediatric Sudden Sensorineural Hearing Loss.

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  7. Sensorineural hearing loss in hemorrhagic dengue?

    Science.gov (United States)

    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.

  8. Sensorineural hearing loss in Lassa fever: two case reports

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    Okokhere Peter O

    2009-01-01

    Full Text Available Abstract Introduction Lassa fever is an acute arena viral haemorrhagic fever with varied neurological sequelae. Sensorineural hearing loss is one of the rare complications which occur usually during the convalescent stage of the infection. Case presentation The cases of two female patients aged 19 and 43 years old, respectively, with clinical features suggestive of Lassa fever and confirmed by immunoserological/Lassa-virus-specific reverse transcriptase polymerase chain reaction are presented. Both patients developed severe sensorineural hearing loss at acute phases of the infections. Conclusion Sensorineural hearing loss from Lassa fever infections can occur in both acute and convalescent stages and is probably induced by an immune response.

  9. 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.

  10. Methadone Induced Sensorineural Hearing Loss

    Directory of Open Access Journals (Sweden)

    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.

  11. Hearing Disorders and Sensorineural Aging

    Directory of Open Access Journals (Sweden)

    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.

  12. 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.

  13. Sudden sensorineural hearing loss following intramuscular administration of penicillin.

    Science.gov (United States)

    Escada, Pedro Alberto; Capucho, Clara; Madeira da Silva, José Francisco

    2004-02-01

    We report a case of sudden hearing loss in a patient with acute exudative tonsillitis, occurring 15 minutes after the intramuscular administration of penicillin. Audiological evaluation documented a profound sensorineural hearing loss of the cochlear type. The mechanism of the hearing loss was probably an immediate hypersensitivity (type I) allergic drug reaction. Penicillin is used frequently for the treatment of several infections. Allergic reactions to penicillin are well known and include urticaria, maculopapular exanthems, angio-oedema, bronchospasm and anaphylaxis, but sudden hearing loss has never been recorded.

  14. 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)

  15. Isolated Contralateral Sudden Sensorineural Hearing Loss: An Unusual Manifestation of Pontine Infarct

    OpenAIRE

    Eluvathingal Muttikkal, Thomas Jose; Kesser, Bradley W.; Mukherjee, Sugoto

    2014-01-01

    Unilateral, acute onset sensorineural hearing loss (“sudden sensorineural hearing loss” [SSNHL]) as an isolated event without other associated neurological deficits usually results from a lesion of the cochlea. Lesions in the ascending central auditory pathways cranial to the cochlear nucleus seldom result in unilateral hearing loss due to decussation of the central auditory pathways at multiple levels. We describe a patient with a tiny acute infarct in the right pons resulting in isolated ac...

  16. 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?

  17. 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...

  18. 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.

  19. 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.

  20. 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.

  1. 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.

  2. An update on drug design strategies to prevent acquired sensorineural hearing loss.

    Science.gov (United States)

    Bodmer, Daniel

    2017-11-01

    Acute sensorineural hearing loss is a dramatic event for the patient. Different pathologies might result in acute sensorineural hearing loss, such as sudden hearing loss, exposure to medications/drugs or loud sound. Current therapeutic approaches include steroids and hyperbaric oxygen in addition to other methods. Research activities of the past have shed light on the molecular mechanisms involved in damage to hair cells, the synapses at the hair cell spiral ganglion junction and the stria vascularis. Molecular events and signaling pathways which underlie damage to these structures have been discovered. Areas covered: This paper summarizes current research efforts involved in investigating the molecular mechanisms involved in acute sensorineural hearing loss. Expert opinion: While progress has been made in unraveling basic mechanisms involved in acute sensorineural hearing loss, it is difficult to translate basic concepts to the clinic. There are often conflicting data in animal and human studies on the effect of a given intervention. There is also a lack of high quality clinical trials (double blind, placebo controlled and high powered). However, this author is confident that research efforts will pay out and that some of these efforts will translate into new therapeutic options for patients with acute hearing loss.

  3. [Bilateral versus unilateral sudden sensorineural hearing loss].

    Science.gov (United States)

    Ni, Maomei; Li, Dehong; Peng, Weihui; Peng, Yikun; Ren, Juanjuan

    2010-01-01

    To analyze the clinical characteristics and treatment effect between bilateral (bi-) and unilateral (uni-) sudden sensorineural hearing loss (SSNHL). Four hundred and eighty cases of SSNHL were retrospective study, which were divided into two groups of bi-SSNHL (n = 40) and uni-SSNHL (n = 440). Clinical characteristics and treatment effects were compared of the two groups. The incidence rate of bi-SSNHL was 8.3 percent and uni-SSNHL was 91.7 percent of all patients with SSNHL. Bi-SSNHL occurs more commonly in patients of old age, diabetes mellitus, and lipid panes abnormalities compared to uni-SSNHL. Twenty-eight ears in the bi-SSNHL group showed hearing recovery (35%), compared with 56.4 percent of patients with uni-SSNHL. Bi-SSNHL and uni-SSNHL may have a completely different clinical characteristics and treatment effect, that implies a different pathophysiology and prognosis. Recognition their different clinical characteristics and treatment effect between bilateral and unilateral SSNHL can help in counseling and managing the patients and correctly evaluate the prognosis.

  4. 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

  5. Sensorineural hearing loss in patients with Kawasaki disease.

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    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.

  6. Sudden bilateral sensorineural hearing loss associated with urticarial vasculitis.

    Science.gov (United States)

    Hall, A C; Leong, A C; Jiang, D; Fitzgerald-O'Connor, A

    2013-07-01

    Bilateral sensorineural hearing loss associated with recurrent urticarial skin lesions may be signs of underlying Muckle-Wells syndrome. Previous reports have described the hearing loss to be progressive in nature. To our knowledge, this paper presents the first published case of sudden onset, bilateral sensorineural hearing loss associated with urticarial vasculitis due to underlying Muckle-Wells syndrome. The patient underwent a cochlear implantation with a modest outcome. Cochlear implantation may help to rehabilitate sudden hearing loss associated with this condition, but early diagnosis may allow treatment with interleukin-1β inhibitors such as anakinra.

  7. [Analysis of sensorineural hearing loss in chronic suppurative otitis media].

    Science.gov (United States)

    Feng, Hongyun; Chen, Ying

    2004-10-01

    To explore the association between chronic suppurative otitis media and sensorineural hearing loss. The files of 147 patients with unilateral chronic suppurative media were reviewed in a retrospective study. Differences between diseased and control ear bone conduction thresholds were analysed by the paired student's t-test over the four frequencies (0.5 kHz, 1.0 kHz, 2.0 kHz, 4.0 kHz). The effect of the presence of cholesteatoma and ossicular erosion on sensorineural hearing loss over the speech frequencies (0.5 kHz, 1.0 kHz, 2.0 kHz) and 4.0 kHz were analysed by one-way ANOVA. Linear regression models were used to clarify the relationships between sensorineural hearing loss and chronic suppurative otitis media. The differences in bone conduction threshold between diseased and control ear were statistically significant. Bone conduction threshold shift at speech frequency was associated with the ages. The presence of cholesteatoma and ossicular erosion was not associated with a significantly increased risk of sensorineural hearing loss. Chronic suppurative otitis media can result in sensorineural hearing loss. The higher frequencies are easier affected than the lower frequencies.

  8. 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.

  9. Sudden sensorineural hearing loss in human monocytic ehrlichiosis.

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    Bhalla, Vidur; Rodgers, Brian; Lin, James

    2017-08-01

    The hallmarks of symptomatic human monocytic ehrlichiosis (HME) include fever, headache, myalgia, nausea, malaise, transaminitis, and blood cell abnormalities. Previous case reports have described isolated cranial nerve palsies in infected patients but not hearing loss. We describe the onset of sudden sensorineural hearing loss in 2 patients with HME-a 31-year-old woman and an 82-year-old woman. The older patient experienced objective and subjective improvement in her hearing after treatment with an antibiotic and steroid taper; the younger patient was lost to audiologic follow-up. Additionally, we discuss the possible mechanisms of the hearing loss in these patients.

  10. 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.

  11. 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.

  12. 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...

  13. 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.

  14. Sensorineural hearing loss in insulin-dependent diabetic patients

    Directory of Open Access Journals (Sweden)

    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.

  15. [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.

  16. Sensori-neural hearing loss following radiotherapy to the nasopharynx

    Energy Technology Data Exchange (ETDEWEB)

    Moretti, J.A.

    1976-04-01

    A retrospective study was done to ascertain the risks of cochlear damage from radiotherapy of the nasopharynx. Audiometric evaluation, pre- and post-radiotherapy, revealed that 7 out of 13 patients had sustained sensori-neural deafness. Contrary to what is generally believed of the resistance of the cochlea to radiotherapeutic damage, eventual loss of hearing can occasionally be expected in patients undergoing radiation therapy for head and neck tumors.

  17. Animal Model of Sensorineural Hearing Loss Associated with Lassa Virus Infection.

    Science.gov (United States)

    Yun, Nadezhda E; Ronca, Shannon; Tamura, Atsushi; Koma, Takaaki; Seregin, Alexey V; Dineley, Kelly T; Miller, Milagros; Cook, Rebecca; Shimizu, Naoki; Walker, Aida G; Smith, Jeanon N; Fair, Joseph N; Wauquier, Nadia; Bockarie, Bayon; Khan, Sheik Humarr; Makishima, Tomoko; Paessler, Slobodan

    2015-12-30

    Approximately one-third of Lassa virus (LASV)-infected patients develop sensorineural hearing loss (SNHL) in the late stages of acute disease or in early convalescence. With 500,000 annual cases of Lassa fever (LF), LASV is a major cause of hearing loss in regions of West Africa where LF is endemic. To date, no animal models exist that depict the human pathology of LF with associated hearing loss. Here, we aimed to develop an animal model to study LASV-induced hearing loss using human isolates from a 2012 Sierra Leone outbreak. We have recently established a murine model for LF that closely mimics many features of human disease. In this model, LASV isolated from a lethal human case was highly virulent, while the virus isolated from a nonlethal case elicited mostly mild disease with moderate mortality. More importantly, both viruses were able to induce SNHL in surviving animals. However, utilization of the nonlethal, human LASV isolate allowed us to consistently produce large numbers of survivors with hearing loss. Surviving mice developed permanent hearing loss associated with mild damage to the cochlear hair cells and, strikingly, significant degeneration of the spiral ganglion cells of the auditory nerve. Therefore, the pathological changes in the inner ear of the mice with SNHL supported the phenotypic loss of hearing and provided further insights into the mechanistic cause of LF-associated hearing loss. Sensorineural hearing loss is a major complication for LF survivors. The development of a small-animal model of LASV infection that replicates hearing loss and the clinical and pathological features of LF will significantly increase knowledge of pathogenesis and vaccine studies. In addition, such a model will permit detailed characterization of the hearing loss mechanism and allow for the development of appropriate diagnostic approaches and medical care for LF patients with hearing impairment. Copyright © 2016, American Society for Microbiology. All Rights

  18. Postural control assessment in students with normal hearing and sensorineural hearing loss.

    Science.gov (United States)

    Melo, Renato de Souza; Lemos, Andrea; Macky, Carla Fabiana da Silva Toscano; Raposo, Maria Cristina Falcão; Ferraz, Karla Mônica

    2015-01-01

    Children with sensorineural hearing loss can present with instabilities in postural control, possibly as a consequence of hypoactivity of their vestibular system due to internal ear injury. To assess postural control stability in students with normal hearing (i.e., listeners) and with sensorineural hearing loss, and to compare data between groups, considering gender and age. This cross-sectional study evaluated the postural control of 96 students, 48 listeners and 48 with sensorineural hearing loss, aged between 7 and 18 years, of both genders, through the Balance Error Scoring Systems scale. This tool assesses postural control in two sensory conditions: stable surface and unstable surface. For statistical data analysis between groups, the Wilcoxon test for paired samples was used. Students with hearing loss showed more instability in postural control than those with normal hearing, with significant differences between groups (stable surface, unstable surface) (p<0.001). Students with sensorineural hearing loss showed greater instability in the postural control compared to normal hearing students of the same gender and age. Copyright © 2014 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  19. 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

  20. Resolution of sudden sensorineural hearing loss following a roller coaster ride.

    Science.gov (United States)

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

    2011-07-01

    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 prior to the episode). Following resolution of the patient's symptoms during a roller coaster ride, pure-tone audiometry showed normal hearing thresholds in both ears. Sudden sensorineural hearing loss is a symptom of cochlear injury and the mechanism of the patient's symptoms was attributed to a patent cochlear aqueduct.

  1. Sensorineural hearing loss following irradiation to the malignant tumor of the head and neck

    Energy Technology Data Exchange (ETDEWEB)

    Murakami, Masafumi; Kobari, Hitomi; Kanno, Hidetaka; Aikawa, Tohru; Anzai, Tomohiro; Okamura, Hiro-oki; Ohtani, Iwao; Hoshino, Toshiaki

    1989-03-01

    We observed sensorineural hearing loss following X-ray irradiation to the malignant tumor of head and neck. There were 24 patients whose auditory organs lied within the irradiation field. Ten of these patients were affected by sensorineural hearing loss. Hearing loss occurred at a high frequency in elderly patients, epipharynx tumor and high dose of irradiation. Many cases revealed high tone hearing loss. Most cases showed about a 20/similar to/30 dB hearing loss, so their impediment seemed not severe in daily life. In some of these cases, we could have temporal bone findings, but there were no particular findings relevant to sensorineural hearing loss. (author).

  2. Static and dynamic balance of children and adolescents with sensorineural hearing loss

    OpenAIRE

    Melo, Renato de Souza; Marinho, Sônia Elvira dos Santos; Freire, Maryelly Evelly Araújo; Souza, Robson Arruda; Damasceno, Hélio Anderson Melo; Raposo, Maria Cristina Falcão

    2017-01-01

    ABSTRACT Objective To assess the static and dynamic balance performance of students with normal hearing and with sensorineural hearing loss. Methods A cross-sectional study assessing 96 students, 48 with normal hearing and 48 with sensorineural hearing loss of both sexes, aged 7 and 18 years. To evaluate static balance, Romberg, Romberg-Barré and Fournier tests were used; and for the dynamic balance, we applied the Unterberger test. Results Hearing loss students showed more changes in s...

  3. Pseudoexfoliation Syndrome and Sensorineural Hearing Loss

    Directory of Open Access Journals (Sweden)

    Ramin Zojaji

    2011-03-01

    Full Text Available Introduction: Pseudoexfoliation syndrome (PXS occurs due to the deposition of extracellular fibrillar materials on the anterior chamber of the eye. This syndrome has been considered to be part of a systemic disease with the potential involvement of the inner ear called sensoroneural hearing loss (SNHL.  In this study, we aimed on evaluating SNHL within PXS patients in Iran to compare them with other international reports. Materials and Methods: In total, 33 patients with PXS and 33 age and sex matched controls were enrolled prospectively in a case-control study. Both groups underwent complete ophthalmologic and otorhinolaryngologic examinations and pure tone audiometry (PTA testing. Six frequencies (0.25, 0.5, 1, 2, 3, 4 and 6 KHz were evaluated for PTA in the same ethnic group in order to select the case and control individuals. Data were analyzed using t-test and chi-square test. Results: Forty-nine out of 66 ears (75.2% in the PXS group and 27 ears (40.9% in the control group had SNHL (P

  4. 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

  5. Hyperbaric oxygen for idiopathic sudden sensorineural hearing loss and tinnitus.

    Science.gov (United States)

    Bennett, Michael H; Kertesz, Tom; Perleth, Matthias; Yeung, Philip; Lehm, Jan P

    2012-10-17

    This is an update of a Cochrane Review first published in The Cochrane Library in Issue 1, 2005 and previously updated in 2007 and 2009.Idiopathic sudden sensorineural hearing loss (ISSHL) is common and has a significant effect on quality of life. Hyperbaric oxygen therapy (HBOT) may improve oxygen supply to the inner ear and result in an improvement in hearing. To assess the benefits and harms of HBOT for treating ISSHL and/or tinnitus. We searched the Cochrane Ear, Nose and Throat Disorders Group Trials Register; the Cochrane Central Register of Controlled Trials (CENTRAL); PubMed; EMBASE; Database of Randomised Trials in Hyperbaric Medicine (DORCTHIM); CINAHL; Web of Science; BIOSIS Previews; Cambridge Scientific Abstracts; ICTRP and additional sources for published and unpublished trials. The date of the most recent search was 2 May 2012, following previous searches in 2009, 2007 and 2004. Randomised studies comparing the effect on ISSHL and tinnitus of HBOT and alternative therapies. Three authors evaluated the quality of trials using the 'Risk of bias' tool and extracted data from the included trials. Seven trials contributed to this review (392 participants). The studies were small and of generally poor quality. Pooled data from two trials did not show any significant improvement in the chance of a 50% increase in hearing threshold on pure-tone average with HBOT (risk ratio (RR) with HBOT 1.53, 95% confidence interval (CI) 0.85 to 2.78, P = 0.16), but did show a significantly increased chance of a 25% increase in pure-tone average (RR 1.39, 95% CI 1.05 to 1.84, P = 0.02). There was a 22% greater chance of improvement with HBOT, and the number needed to treat (NNT) to achieve one extra good outcome was 5 (95% CI 3 to 20). There was also an absolute improvement in average pure-tone audiometric threshold following HBOT (mean difference (MD) 15.6 dB greater with HBOT, 95% CI 1.5 to 29.8, P = 0.03). The significance of any improvement in tinnitus could not be

  6. Modified intratympanic treatment for idiopathic sudden sensorineural hearing loss.

    Science.gov (United States)

    Kara, Emrah; Cetik, Fikret; Tarkan, Ozgür; Sürmelioğlu, Ozgür

    2010-05-01

    Steroids are the only proven drugs in the treatment for idiopathic sudden sensorineural hearing loss. In the recent studies, it has been suggested that, steroids delivered through the intratympanic route obtained higher perilymph levels, resulting in better hearing outcomes. The purpose of this study is to compare the hearing outcomes of the two routes of steroid treatment: intratympanic route and systemic route. In this prospective study, 60 consecutive patients with idiopathic sensorineural hearing loss treated between January 2005 and September 2008 were enrolled: 29 were in the intratympanic steroid group (ITSG) and 31 were in the systemic steroid group (SSG). In the ITSG, 5 intratympanic injections of dexamethasone were performed with the dose of 4 mg/ml, consecutively. Oral methylprednisolone was given at the dose of 1 mg/kg, tapered every 2 days and stopped at 10 days, in the SSG. The pure tone averages (PTA), speech discrimination scores (SDS) and the percentage of the patients who made an improvement more than 10 dB were analyzed on the tenth day and 2 months after the treatment statistically. The improvement in PTA on tenth day and second month after treatment was 31.38 and 37.55 dB, in the ITSG and 19.35 and 20.68 dB in the SSG, respectively. The improvement in SDS in the same time period was 35.24 and 37.52% in the ITSG and 20.13 and 19.61% in the SSG, respectively. Also, 25 of the 29 patients (86.2%) in the ITSG and 16 of the 31 patients (51.6%) in the SSG made an improvement more than 10 dB on PTA in the second month control. Intratympanic steroids gave better hearing results than systemic steroids with no systemic side effects. Studies with more sample sizes will identify the best steroid for injection, application time, frequency and dose.

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

    DEFF Research Database (Denmark)

    Jepsen, Morten Løve; Dau, Torsten

    2011-01-01

    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 discrim...

  8. Regenerative efficacy of mesenchymal stromal cells from human placenta in sensorineural hearing loss.

    Science.gov (United States)

    Kil, Kicheol; Choi, Mi Young; Kong, Ji Sun; Kim, Woo Jin; Park, Kyoung Ho

    2016-12-01

    Hearing loss is a common chronic disorder characterized by decline of auditory function. The global population have suffered from deafness and the transplantation of stem cells is regarded as a therapeutic strategy for this disease. We collected placenta from a total of 13 samples of full term pregnant women and isolated MSCs derived from human placenta and transplanted MSCs on deaf animal model. The normal group and the sensorineural hearing loss (SNHL) group and the experimental (transplanted MSCs) group were compared and estimated hearing level using auditory brainstem response (ABR) recordings and the otoacoustic emission (OAE) test. ABR threshold value and DPOAE level showed that MSCs transplantation groups was improved than the SNHL group. And the number of spiral ganglion neurons were increased in all turn of the cochlea. And there was no evidence of acute immunological rejection and inflammation response was not observed. This study is to evaluate regenerative efficacy of hearing loss by transplanting mesenchymal stromal cells (MSCs) derived from human placenta (amnion and chorion) in deaf animal model. We identified that MSCs transplantation restored auditory impairment and promoted cell regeneration. We hope to overcome sensorineural hearing loss by transplanting stem cells such as mesenchymal stromal cells (MSCs) from easily accessible adult stem cell source in placenta. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  9. Endothelial dysfunction in idiopathic sudden sensorineural hearing loss: a review

    Directory of Open Access Journals (Sweden)

    Nicola Quaranta

    2016-07-01

    Full Text Available 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.

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

    Science.gov (United States)

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

    2016-04-20

    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.

  11. 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.

  12. 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.

  13. [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.

  14. 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.

  15. Mobile Hearing Testing Applications and the Diagnosis of Sudden Sensorineural Hearing Loss: A Cautionary Tale.

    Science.gov (United States)

    Sethi, Rosh K V; Ghanad, Iman; Kanumuri, Vivek; Herrmann, Barbara; Kozin, Elliott D; Remenschneider, Aaron K

    2018-01-01

    Mobile hearing applications (apps) are available for hearing testing, personal sound amplification, as well as hearing aid modulation. Hearing testing apps are gaining popularity, especially in resource-limited settings. The reliability of mobile hearing testing apps, however, is not well characterized. A case study of a single patient with a complaint of sudden hearing loss presenting to a tertiary-care hospital. Comparison of a mobile hearing testing app results with standard audiogram. A commercially available mobile hearing testing app was used after hours to determine if a patient's hearing complaints were consistent with sudden sensorineural hearing loss. The hearing app produced a rudimentary audiogram that was consistent with unilateral SSNHL. Given contraindications to oral treatment, preparations for possible intratympanic dexamethasone after a full audiometric evaluation were completed. Confirmatory audiogram the following day demonstrated normal hearing without evidence of hearing loss. Steroid treatment was aborted and appropriate counseling provided. While mobile hearing testing apps offer improved access to hearing screening in resource-limited settings, caution must be exercised when interpreting data and making clinical decisions based upon results. The role of professional audiologists remains critical. Further testing and validation of specific apps is required.

  16. 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

  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. 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.

  19. 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.

  20. Endothelial progenitor cells in sudden sensorineural hearing loss.

    Science.gov (United States)

    Quaranta, Nicola; Ramunni, Alfonso; De Luca, Concetta; Brescia, Paola; Dambra, Porzia; De Tullio, Giacomina; Vacca, Angelo; Quaranta, Antonio

    2011-04-01

    Endothelial progenitor cells (EPCs) are a unique subtype of circulating cells with properties similar to those of embryonal angioblasts. They have the potential to proliferate and to differentiate into mature endothelial cells. EPCs are reduced in patients with vascular risk factors due to a decreased mobilization, an increased consumption at the site of damage or a reduced half-life. The results of this study confirm the existence of an endothelial dysfunction in patients with sudden sensorineural hearing loss (SSHL) and support the vascular involvement in the pathogenesis of the disease. The aim of this study was to evaluate the concentration of EPCs in patients affected by SSHL. Twenty-one patients affected by SSHL were evaluated. The number of EPCs was analyzed by flow cytometry analysis of peripheral blood CD34+KDR+CD133+ cells. Circulating levels of EPCs were significantly lower in SSHL patients compared with controls. In particular, CD34+KDR+ cells and CD34+CD133+KDR+ cells were significantly reduced (p < 0.05).

  1. Audiovisual Asynchrony Detection and Speech Intelligibility in Noise With Moderate to Severe Sensorineural Hearing Impairment

    NARCIS (Netherlands)

    Baskent, Deniz; Bazo, Danny

    2011-01-01

    Objective: The objective of this study is to explore the sensitivity to intermodal asynchrony in audiovisual speech with moderate to severe sensorineural hearing loss. Based on previous studies, two opposing expectations were an increase in sensitivity, as hearing-impaired listeners heavily rely on

  2. 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 loss; LC-MS: liquid chromatography-mass spectrometry; MS: mass spectrometry; autoAb: autoantibody; 1-DE: one-dimensional electrophoresis. Copyright © 2017 Elsevier Inc. All rights reserved.

  3. 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…

  4. 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

  5. Correlation between auditory brainstem response and hearing prognosis in idiopathic sudden sensorineural hearing loss patients.

    Science.gov (United States)

    Lin, Hung-Che; Chou, Yu-Ching; Wang, Chih-Hung; Hung, Li-Wen; Shih, Cheng-Ping; Kang, Bor-Hwang; Yeh, Wen-Ying; Chen, Hsin-Chien

    2017-12-01

    To investigate the latency and amplitude of auditory brainstem response (ABR) and hearing prognosis in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). Patients with ISSNHL were classified into four different recovery groups. All patients' clinical and demographic features were analyzed. Two-channel ABRs were collected in response to click stimuli at 90dB nHL. ABR amplitudes for wave I and ABR latency for waves I, III, and V were analyzed. One hundred and two patients (54 men and 48 women) were included in the study. Hearing recovery was observed in 72 cases (70.6%). Waves I, III, and V latencies were significantly prolonged in the affected ears compared with the unaffected ears. A smaller wave I amplitude was found in the affected ear compared with the unaffected ear in the three recovery groups. There was a significant association between wave I latency and hearing outcome (p=0.009) with a prolonged trend from complete to slight hearing recovery group. There was a significant correlation between wave I latency and hearing outcome in patients with ISSNHL. The finding may provide diagnostic information and serve as a potential prognostic indicator in patients with ISSNHL. Copyright © 2017 Elsevier B.V. All rights reserved.

  6. 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.

  7. Sudden sensorineural hearing loss: our experience in diagnosis, treatment, and outcome.

    Science.gov (United States)

    Cadoni, Gabriella; Agostino, Stefania; Scipione, Simona; Ippolito, Silvia; Caselli, Andrea; Marchese, Raffaella; Paludetti, Gaetano

    2005-12-01

    To describe our experience concerning sudden sensorineural hearing loss (SSNHL) in a large single-institution series of SSNHL patients and to discuss the results. This was a retrospective study, and the charts of 156 consecutive inpatients (65 males, mean age 44 years, range 10-74 years; 91 females, mean age 46 years, range 15-75 years) with the diagnosis of SSNHL from 1987 to 2000 were reviewed. One hundred forty-three of 156 patients received multidrug therapy (plasma expanders, antiaggregants, steroids), whereas only 13 SSNHL patients received hyperbaric oxygen therapy. Old age, vascular and metabolic risk factors, and cigarette smoking do not a have a high prevalence in the SSNHL population. An etiologic factor was detected in 23 of 156 (15%) cases (16 cases of acute infection, 4 cases of neurovascular conflicts, 2 cases of cerebellar angiomas, 1 case of cochleovestibular schwannoma). The outcome was not related to the laterality, age, or hearing loss type. On the contrary, a statistically significant association between poor recovery and male sex, both tinnitus and vertigo, and the initial severity of the hearing loss was observed. Mostly, SSNHL results in idiopathic disease. At present, diagnostic and therapeutic efforts appear to be inadequate to improve the prognosis of SSNHL. Further studies are needed to obtain better knowledge about the etiopathogenesis of SSNHL so that new therapeutic strategies can be considered in the treatment of this challenging ear disease.

  8. Hyperventilation-induced nystagmus in vestibular schwannoma and unilateral sensorineural hearing loss.

    Science.gov (United States)

    Mandalà, Marco; Giannuzzi, Annalisa; Astore, Serena; Trabalzini, Franco; Nuti, Daniele

    2013-07-01

    We evaluated the incidence and characteristics of hyperventilation-induced nystagmus (HVN) in 49 patients with gadolinium-enhanced magnetic resonance imaging evidence of vestibular schwannoma and 53 patients with idiopathic unilateral sensorineural hearing loss and normal radiological findings. The sensitivity and specificity of the hyperventilation test were compared with other audio-vestibular diagnostic tests (bedside examination of eye movements, caloric test, auditory brainstem responses) in the two groups of patients. The hyperventilation test scored the highest diagnostic efficiency (sensitivity 65.3 %; specificity 98.1 %) of the four tests in the differential diagnosis of vestibular schwannoma and idiopathic unilateral sensorineural hearing loss. Small tumors with a normal caloric response or caloric paresis were associated with ipsilateral HVN and larger tumors and severe caloric deficits with contralateral HVN. These results confirm that the hyperventilation test is a useful diagnostic test for predicting vestibular schwannoma in patients with unilateral sensorineural hearing loss.

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

    Energy Technology Data Exchange (ETDEWEB)

    Lemmerling, M.; De Praeter, G.; Mollet, P.; Mortele, K.; Kunnen, M. [Univ. Hospital, Gent (Belgium). Dept. of Radiol.; Dhooge, I. [Department of Otorhinolaryngology, University Hospital Gent, Gent (Belgium); Mastenbroek, G. [Department of Neurology, Streekziekenhuis De Honte, Terneuzen (Netherlands)

    1998-05-01

    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.) With 5 figs., 10 refs.

  10. Necessity of admission to improve the hearing-recovery rate in idiopathic sudden sensorineural hearing loss.

    Science.gov (United States)

    Kim, Min Jae; Heo, Kyung Wook; Jeon, Pil Hyun; Lee, Jae Jun; Lee, Jun Ho

    2017-11-13

    The purpose of this study was to determine the prognostic potential of admission of a patient for idiopathic sudden sensorineural hearing loss (ISSNHL). Retrospective clinical study. We conducted a retrospective study of 301 patients with ISSNHL. The patients were divided into a study (57 patients at out-patients department (OPD) based treatment, using systemic steroids and intra-tympanic (IT) steroid injection) and a control groups (301 patients at admission, using systemic steroids and IT steroid injection). We compared the success rate (15 dB cut off), Siegel's criteria and hearing gain decibels between two groups. The overall recovery rate of the OPD based treatment group (29.8%) was poor than that of the admission group (52.9%) (p = .002). The odd ratio of OPD based treatment factor was 2.035 when the OPD based treatment and poor prognostic factors were analyzed at logistic regression test (Duration of delayed treatment, 1.073; average hearing loss level, 0.972) (p = .041). Admission must be strongly recommended when the patients with ISSNHL asked about the necessity of admission. We hypothesized that resting, getting out of their social stress, and relief of anxiety might be helpful to their hearing recoveries.

  11. 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…

  12. Neurodevelopmental Disorders in Children with Severe to Profound Sensorineural Hearing Loss: A Clinical Study

    Science.gov (United States)

    Chilosi, Anna M.; Comparini, Alessandro; Scusa, Maria F.; Berrettini, Stefano; Forli, Francesca; Battini, Roberta; Cipriani, Paola; Cioni, Giovanni

    2010-01-01

    Aim: The effects of sensorineural hearing loss (SNHL) are often complicated by additional disabilities, but the epidemiology of associated disorders is not clearly defined. The aim of this study was to evaluate the frequency and type of additional neurodevelopmental disabilities in a sample of children with SNHL and to investigate the relation…

  13. 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:

  14. 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.

  15. Diminished temporal coding with sensorineural hearing loss emerges in background noise.

    Science.gov (United States)

    Henry, Kenneth S; Heinz, Michael G

    2012-10-01

    Behavioral studies in humans suggest that sensorineural hearing loss (SNHL) decreases sensitivity to the temporal structure of sound, but neurophysiological studies in mammals provide little evidence for diminished temporal coding. We found that SNHL in chinchillas degraded peripheral temporal coding in background noise substantially more than in quiet. These results resolve discrepancies between previous studies and help to explain why perceptual difficulties in hearing-impaired listeners often emerge in noisy situations.

  16. 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.

  17. 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.

  18. 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.

  19. Cochlear microphonics in sensorineural hearing loss: lesson from newborn hearing screening.

    Science.gov (United States)

    Ahmmed, Ansar; Brockbank, Christopher; Adshead, June

    2008-08-01

    The diagnostic dilemma surrounding the presence of cochlear microphonics (CM) coupled with significantly elevated auditory brainstem response (ABR) thresholds in babies failing the newborn hearing screening is highlighted. A case report is presented where initial electo-diagnostic assessment could not help in differentiating between Auditory Neuropathy/Auditory Dys-synchrony (AN/AD) and sensorineural hearing loss (SNHL). In line with the protocol and guidelines provided by the national Newborn Hearing Screening Programme in the UK (NHSP) AN/AD was suspected in a baby due to the presence of CM at 85 dBnHL along with click evoked ABR thresholds of 95 dBnHL in one ear and 100 dBnHL in the other ear. Significantly elevated thresholds for 0.5 and 1kHz tone pip ABR fulfilled the audiological diagnostic criteria for AN/AD. However, the possibility of a SNHL could not be ruled out as the 85 dBnHL stimuli presented through inserts for the CM would have been significantly enhanced in the ear canals of the young baby to exceed the threshold level of the ABR that was carried out using headphones. SNHL was eventually diagnosed through clinical and family history, physical examination and imaging that showed enlarged vestibular aqueducts. Presence of CM in the presence of very high click ABR thresholds only suggests a pattern of test results and in such cases measuring thresholds for 0.5 and 1 kHz tone pip ABR may not be adequate to differentiate between SNHL and other conditions associated with AN/AD. There is a need for reviewing the existing AN/AD protocol from NHSP in the UK and new research to establish parameters for CM to assist in the differential diagnosis. A holistic audiological and medical approach is essential to manage babies who fail the newborn hearing screening.

  20. Acute auditory agnosia as the presenting hearing disorder in MELAS.

    Science.gov (United States)

    Miceli, Gabriele; Conti, Guido; Cianfoni, Alessandro; Di Giacopo, Raffaella; Zampetti, Patrizia; Servidei, Serenella

    2008-12-01

    MELAS is commonly associated with peripheral hearing loss. Auditory agnosia is a rare cortical auditory impairment, usually due to bilateral temporal damage. We document, for the first time, auditory agnosia as the presenting hearing disorder in MELAS. A young woman with MELAS (A3243G mtDNA mutation) suffered from acute cortical hearing damage following a single stroke-like episode, in the absence of previous hearing deficits. Audiometric testing showed marked central hearing impairment and very mild sensorineural hearing loss. MRI documented bilateral, acute lesions to superior temporal regions. Neuropsychological tests demonstrated auditory agnosia without aphasia. Our data and a review of published reports show that cortical auditory disorders are relatively frequent in MELAS, probably due to the strikingly high incidence of bilateral and symmetric damage following stroke-like episodes. Acute auditory agnosia can be the presenting hearing deficit in MELAS and, conversely, MELAS should be suspected in young adults with sudden hearing loss.

  1. Static and dynamic balance of children and adolescents with sensorineural hearing loss.

    Science.gov (United States)

    Melo, Renato de Souza; Marinho, Sônia Elvira Dos Santos; Freire, Maryelly Evelly Araújo; Souza, Robson Arruda; Damasceno, Hélio Anderson Melo; Raposo, Maria Cristina Falcão

    2017-01-01

    To assess the static and dynamic balance performance of students with normal hearing and with sensorineural hearing loss. A cross-sectional study assessing 96 students, 48 with normal hearing and 48 with sensorineural hearing loss of both sexes, aged 7 and 18 years. To evaluate static balance, Romberg, Romberg-Barré and Fournier tests were used; and for the dynamic balance, we applied the Unterberger test. Hearing loss students showed more changes in static and dynamic balance as compared to normal hearing, in all tests used (pRomberg, Romberg-Barré, Fournier and Unterberger test p values were, respectively, p=0.004, pRomberg, Romberg-Barré and Fournier tests were, respectively, p=0.007, pRomberg, Romberg-Barré, Fournier and Unterberger tests were p=0.002, pRomberg-Barré, Fournier and Unterberger tests were, respectively, p=0.037, p<0.001 and p=0.037. Hearing-loss students showed more changes in static and dynamic balance comparing to normal hearing of same sex and age groups.

  2. 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

  3. Noonan Syndrome: An Underestimated Cause of Severe to Profound Sensorineural Hearing Impairment. Which Clues to Suspect the Diagnosis?

    Science.gov (United States)

    Ziegler, Alban; Loundon, Natalie; Jonard, Laurence; Cavé, Hélène; Baujat, Geneviève; Gherbi, Souad; Couloigner, Vincent; Marlin, Sandrine

    2017-09-01

    To highlight Noonan syndrome as a clinically recognizable cause of severe to profound sensorineural hearing impairment. New clinical cases and review. Patients evaluated for etiological diagnosis by a medical geneticist in a reference center for hearing impairment. Five patients presenting with confirmed Noonan syndrome and profound sensorineural hearing impairment. Diagnostic and review of the literature. Five patients presented with profound sensorineural hearing impairment and molecularly confirmed Noonan syndrome. Sensorineural hearing impairment has been progressive for three patients. Cardiac echography identified pulmonary stenosis in two patients and was normal for the three other patients. Short stature was found in two patients. Mild intellectual disability was found in one patient. Inconspicuous clinical features as facial dysmorphism, cryptorchidism, or easy bruising were of peculiar interest to reach the diagnosis of Noonan syndrome. Profound sensorineural hearing impairment can be the main feature of Noonan syndrome. Associated features are highly variable; thus, detailed medical history and careful physical examination are mandatory to consider the diagnosis in case of a sensorineural hearing impairment.

  4. Mutations in OTOGL, encoding the inner ear protein otogelin-like, cause moderate sensorineural hearing loss.

    Science.gov (United States)

    Yariz, Kemal O; Duman, Duygu; Zazo Seco, Celia; Dallman, Julia; Huang, Mingqian; Peters, Theo A; Sirmaci, Asli; Lu, Na; Schraders, Margit; Skromne, Isaac; Oostrik, Jaap; Diaz-Horta, Oscar; Young, Juan I; Tokgoz-Yilmaz, Suna; Konukseven, Ozlem; Shahin, Hashem; Hetterschijt, Lisette; Kanaan, Moien; Oonk, Anne M M; Edwards, Yvonne J K; Li, Huawei; Atalay, Semra; Blanton, Susan; Desmidt, Alexandra A; Liu, Xue-Zhong; Pennings, Ronald J E; Lu, Zhongmin; Chen, Zheng-Yi; Kremer, Hannie; Tekin, Mustafa

    2012-11-02

    Hereditary hearing loss is characterized by a high degree of genetic heterogeneity. Here we present OTOGL mutations, a homozygous one base pair deletion (c.1430 delT) causing a frameshift (p.Val477Glufs(∗)25) in a large consanguineous family and two compound heterozygous mutations, c.547C>T (p.Arg183(∗)) and c.5238+5G>A, in a nonconsanguineous family with moderate nonsyndromic sensorineural hearing loss. OTOGL maps to the DFNB84 locus at 12q21.31 and encodes otogelin-like, which has structural similarities to the epithelial-secreted mucin protein family. We demonstrate that Otogl is expressed in the inner ear of vertebrates with a transcription level that is high in embryonic, lower in neonatal, and much lower in adult stages. Otogelin-like is localized to the acellular membranes of the cochlea and the vestibular system and to a variety of inner ear cells located underneath these membranes. Knocking down of otogl with morpholinos in zebrafish leads to sensorineural hearing loss and anatomical changes in the inner ear, supporting that otogelin-like is essential for normal inner ear function. We propose that OTOGL mutations affect the production and/or function of acellular structures of the inner ear, which ultimately leads to sensorineural hearing loss. Copyright © 2012 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

  5. 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.

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

    Science.gov (United States)

    Lalwani, A. K.; Brister, J. R.; Fex, J.; Grundfast, K. M.; Pikus, A. T.; Ploplis, B.; San Agustin, T.; Skarka, H.; Wilcox, E. R.

    1994-01-01

    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' to exon 50 of the dystrophin gene) and at DXS1068 (5' to the brain promoter of the dystrophin gene) were observed. No recombination events were noted with the following markers within the DMD locus: 5'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. PMID:7942846

  7. Diffusion Tensor Imaging of Central Auditory Pathways in Patients with Sensorineural Hearing Loss: A Systematic Review.

    Science.gov (United States)

    Tarabichi, Osama; Kozin, Elliott D; Kanumuri, Vivek V; Barber, Samuel; Ghosh, Satra; Sitek, Kevin R; Reinshagen, Katherine; Herrmann, Barbara; Remenschneider, Aaron K; Lee, Daniel J

    2017-11-01

    Objective The radiologic evaluation of patients with hearing loss includes computed tomography and magnetic resonance imaging (MRI) to highlight temporal bone and cochlear nerve anatomy. The central auditory pathways are often not studied for routine clinical evaluation. Diffusion tensor imaging (DTI) is an emerging MRI-based modality that can reveal microstructural changes in white matter. In this systematic review, we summarize the value of DTI in the detection of structural changes of the central auditory pathways in patients with sensorineural hearing loss. Data Sources PubMed, Embase, and Cochrane. Review Methods We used the Preferred Reporting Items for Systematic Reviews and Meta-Analysis statement checklist for study design. All studies that included at least 1 sensorineural hearing loss patient with DTI outcome data were included. Results After inclusion and exclusion criteria were met, 20 articles were analyzed. Patients with bilateral hearing loss comprised 60.8% of all subjects. Patients with unilateral or progressive hearing loss and tinnitus made up the remaining studies. The auditory cortex and inferior colliculus (IC) were the most commonly studied regions using DTI, and most cases were found to have changes in diffusion metrics, such as fractional anisotropy, compared to normal hearing controls. Detectable changes in other auditory regions were reported, but there was a higher degree of variability. Conclusion White matter changes based on DTI metrics can be seen in patients with sensorineural hearing loss, but studies are few in number with modest sample sizes. Further standardization of DTI using a prospective study design with larger sample sizes is needed.

  8. Sensorineural hearing loss due to Toxoplasma gondii in children: a case-control study.

    Science.gov (United States)

    Noorbakhsh, S; Memari, F; Farhadi, M; Tabatabaei, A

    2008-06-01

    Sensorineural hearing loss (SNHL) can follow congenital toxoplasmosis. Treatment in the first year of life is associated with diminished occurrence of this sequel. In various parts of Iran, the prevalence of antibodies to Toxoplasma gondii ranges from 24% to 57.7%. We evaluate the possible role of Toxoplasma gondii infection on the occurrence of SNHL in children. This case-control study was performed in a tertiary care center in Tehran between 2002 and 2003. This study was carried out based on diagnostic parameters of the American Academy of Otolaryngology criteria for SNHL and a healthy control group. We compared the specific Toxoplasma gondii antibodies (IgM & IgG) measured by ELISA in 95 blood samples of infants with SNHL and 63 healthy matched infants. Acute (IgM) and previous (IgG) immunity to Toxoplasma gondii was found in 12 and 21.2% of SNHL children, respectively. Most cases with previous infections (IgG positive) were children aged less than 1 year old (i.e. maternal immunity), but acute infection (IgM positive) was higher in 3-5 year old age group. Acute infection (IgM) was significantly more frequent in the SNHL group, and previous immunity was higher in the controls (CI 95%, P-value = 0.01; 0.01). With respect to seropositive children, as we were unable to differentiate congenital from acquired cases, we recommend prevention of congenital toxoplasmosis by treatment of Toxoplasma infection in pregnant women and treatment of acquired Toxoplasma gondii infection after birth to minimise the risk of SNHL in children.

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

    OpenAIRE

    Halliday, L. F.; Tuomainen, O.; Rosen, S.

    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 measures of phonological processing, receptive and expressive vocabulary and grammar, word and nonword reading, and parental report of communica...

  10. Saccular dysfunction in children with sensorineural hearing loss and auditory neuropathy/auditory dys-synchrony.

    Science.gov (United States)

    Emami, Seyede Faranak; Farahani, Farhad

    2015-01-01

    There is a correlation between the AN/AD disorder and the saccular dysfunction in children with severe sensorineural hearing loss, which means that saccular dysfunction can be a concomitant sign of AN/AD. In conclusion, the term of audio-vestibular dys-synchrony (AVS) is a more suitable description for this condition. Patients with auditory neuropathy/auditory dys-synchrony (AN/AD) characteristically demonstrate poor neural responses from the vestibulocochlear nerve and brainstem while displaying evidence of intact outer hair cells function. Therefore, the objective of this study is studying of the relationship of the saccular dysfunction with AN/AD disorder in children with sensorineural hearing loss. In this cross-sectional study, 100 children with bilateral severe-to-profound sensorineural hearing losses underwent audiologic tests and cervical vestibular-evoked myogenic potentials (cVEMPs) at the Audiology Department of Hamadan University of Medical Sciences (Hamadan, Iran). Eleven children with bilateral severe sensorineural hearing loss were given to unilateral AN/AD disorder (11 ears), and two children (4 ears) had bilateral AN/AD (total = 13 children). The ears with AN/AD took the form of unrepeatable or absent waves of ABR and presence of OAEs. The statistical analysis of an independent t-test between AN/AD ears as compared to non-AN/AD ears of these 13 children showed that the mean latencies of p13 and the mean latencies of n23 and the mean peak-to-peak amplitude had significant differences.

  11. Unusual magnetic resonance findings in two children with sudden sensorineural hearing loss

    OpenAIRE

    Gasparetto,Emerson L.; Arnolfo de Carvalho Neto; Danny Warszawiak; Isac Bruck; Sérgio Antoniuk; Lúcia H.C. dos Santos

    2005-01-01

    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 inne...

  12. 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.

  13. 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], phearing loss caused by exposure to high sound pressure levels. There was no improvement in the thresholds with vitamin E supplementation. Copyright © 2017 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  14. A Narrow Internal Auditory Canal with Duplication in a Patient with Congenital Sensorineural Hearing Loss

    Energy Technology Data Exchange (ETDEWEB)

    Baik, Hye Won; Yu, Hye On; Kim, Kyung Soo; Kim, Gi Hyeon [Chung-Ang University Hospital, College of Medicine, Seoul (Korea, Republic of)

    2008-07-15

    A narrow internal auditory canal (IAC) with duplication is a rare anomaly of the temporal bone. It is associated with congenital sensorineural hearing loss. Aplasia or hypoplasia of the vestibulocochlear nerve may cause the hearing loss. We present an unusual case of an isolated narrow IAC with duplication that was detected by a CT scan. In this case, the IAC was divided by a bony septum into an empty stenotic inferoposterior portion and a large anterosuperior portion containing the facial nerve that was clearly delineated on MRI.

  15. Imported Case of Lassa Fever in Sweden With Encephalopathy and Sensorineural Hearing Deficit.

    Science.gov (United States)

    Grahn, Anna; Bråve, Andreas; Lagging, Martin; Dotevall, Leif; Ekqvist, David; Hammarström, Helena; Karlberg, Helen; Lagerqvist, Nina; Sansone, Martina; Tegnell, Anders; Ulleryd, Peter; Studahl, Marie

    2016-10-01

    We describe an imported case of Lassa fever with both encephalopathy and bilateral sensorineural hearing deficit. Absence of fever during hospitalization, initially nonspecific symptoms, and onset of hearing deficit in a late stage of disease probably contributed to delayed diagnosis (14 days after admittance to hospital). The pathogenesis of neurological manifestations of Lassa fever is poorly understood and no specific treatment was given. A total of 118 personnel had close contact with the patient, but no secondary cases occurred. This case highlights the importance of considering Lassa fever as a differential diagnosis in patients with recent travel to endemic areas.

  16. 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,

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

    Energy Technology Data Exchange (ETDEWEB)

    Grau, C.; Moller, K.; Overgaard, M.; Overgaard, J.; Elbrond, O. (University of Aarhus (Denmark))

    1991-08-01

    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.

  18. [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

  19. MR imaging of 495 consecutive cases with sensorineural hearing loss

    Energy Technology Data Exchange (ETDEWEB)

    Wu, W. [Univ. Hospital, Linkoeping (Sweden). Dept. of Diagnostic Radiology]|[China-Japan Friendship Hospital, Beijing (China); Thuomas, K.AA. [Univ. Hospital, Linkoeping (Sweden). Dept. of Diagnostic Radiology

    1995-11-01

    495 consecutive patients with SNHL and 120 age-matched healthy controls were examined. Spin-echo (SE) and fast spin-echo (FSE) images were used with 1.5 T equipment. An intracranial abnormality was found in 211 (42.6%) of the patients with 95 (19.2%) along the acoustic pathway. Eleven of the 95 patients had sensory hearing loss while 84 had neural hearing loss with the retrocochlear auditory pathway affected by lesions. In 62 of the 84 patients, the internal acoustic canal and cerebellopontine angle were involved. Twenty-two patients had intra-axial lesions. The controls had no pathologic changes along the auditory pathway. (orig./UG).

  20. 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

  1. Long-term Incidence and Degree of Sensorineural Hearing Loss in Otosclerosis.

    Science.gov (United States)

    Ishai, Reuven; Halpin, Christopher F; Shin, Jennifer J; McKenna, Michael J; Quesnel, Alicia M

    2016-12-01

    1) To evaluate the long-term incidence and degree of the sensorineural component of hearing loss (SNHL) in patients with otosclerosis after accounting for expected age-related hearing loss. 2) To identify variables that might predict development of sensorineural hearing loss due to otosclerosis. Retrospective audiometric database and chart review. Tertiary referral center. Consecutive patients with otosclerosis observed between 1994 and 2004, with ≥10 years follow-up, excluding patients with postoperative hearing loss or surgery before the initial audiogram. Bone conduction (BC) thresholds at 0.5, 1, 2, and 4 kHz and Word Recognition. BC threshold change (BCTC) over ≥10 years minus estimated age-related threshold change (ARTC) specific to age and sex for each patient (based on ISO 7029 reference population). Three-hundred fifty-seven ears (290 patients) met study criteria, including 217 ears that had undergone stapedectomy during the study period. Mean follow-up was 14.0 years. The average BCTC after subtracting estimated ARTC was 4.6, 2.6, 3, and 2.7 dB for 0.5, 1, 2, and 4 kHz frequencies, respectively. However, 34% of ears (122 ears) had clinically significant progression of SNHL during the study period (>10 dB BCTC beyond expected ARTC at ≥2 frequencies). Multivariate analysis demonstrated that the probability of developing clinically significant SNHL was higher for women (odds ratio 1.86, p = 0.018) and lower for operated patients (odds ratio 0.46, p = 0.002). The average long-term sensorineural hearing loss due to otosclerosis was statistically significantly more than for age alone at each frequency, but these average values (from 2.6 to 4.6 dB for tested frequencies) were clinically insignificant. Approximately one-third of patients with otosclerosis demonstrated a clinically significant progression of the sensorineural component of hearing loss, with the average BCTC above expected age-related changes ranging from 10.2 to 14.6 dB for

  2. 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.

  3. 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.

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

    Directory of Open Access Journals (Sweden)

    Sheila Uliel

    1980-08-01

    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.

  6. 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.

  7. 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

  8. Sensorineural hearing loss in children: the association with Apgar score. A registry-based study of 392,371 children in Norway.

    Science.gov (United States)

    Kvestad, Ellen; Lie, Kari Kveim; Eskild, Anne; Engdahl, Bo

    2014-11-01

    The causes of congenital permanent hearing loss in children are insufficiently understood. We studied the association of Apgar score 5 min after birth with sensorineural hearing loss diagnosed before the age of 5 years. We performed an epidemiological cohort study with data obtained by linkage between The Medical Birth Registry of Norway and the Norwegian County Registry of Children with Hearing Loss. Cases were 327 children born in Norway during the period 1978-1998 with sensorineural hearing loss. Controls were all children in Norway without sensorineural hearing loss born in the same counties and during the same period as the cases (n=392,044). The associations of Apgar score 5 min after birth with sensorineural hearing loss were estimated as odds ratios (OR) with 95% confidence intervals (CI) by applying logistic regression analyses. Among children with sensorineural hearing loss 0.9% (3/327) had Apgar scoreApgar scoreApgar score 10, after adjustment for birthweight and concurrent birth defects. Most children with sensorineural hearing loss (90%) had Apgar score>8 five minutes after birth. Low Apgar score was associated with childhood sensorineural hearing loss. However, most children with sensorineural hearing loss, had Apgar score>8. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  9. 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...

  10. 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...... estimated in the same listeners. The estimated reduction of cochlear compression was significantly correlated with the increase in the F0DL ratio, while no correlation was found with filter bandwidth. The effects of degraded frequency selectivity and loss of compression were considered in a simplified...

  11. Sensorineural hearing loss in patients with chronic progressive external ophthalmoplegia or Kearns-Sayre syndrome.

    Science.gov (United States)

    Kornblum, C; Broicher, R; Walther, E; Herberhold, S; Klockgether, T; Herberhold, C; Schröder, R

    2005-09-01

    In the present study we assessed the prevalence and nature of hearing loss in patients with chronic progressive external ophthalmoplegia (CPEO) or Kearns-Sayre syndrome (KSS) due to single large-scale mitochondrial DNA (mtDNA) deletion or mtDNA tRNA (Leu (UUR)) A3243G point mutation (A3243G PM). 14 patients with mtDNA deletion and three patients with A3243G PM underwent audiological evaluation comprising pure-tone and speech audiometry as well as transient evoked otoacoustic emissions (OAE). Audiological evaluation revealed hearing impairment in 10/17 patients. Hearing loss was mild to moderate predominantly affecting high frequencies in five patients with subjective hearing problems (three patients with mtDNA deletions, two patients with A3243G PM). Subclinical hearing deficits restricted to high frequencies were seen in further five asymptomatic patients (four patients with mtDNA deletions, one patients with A3243G PM). Audiological findings suggested a cochlear origin of hearing loss in all subjects. Our results demonstrate that CPEO or KSS patients due to mtDNA deletion or A3243G PM are at high risk of developing sensorineural hearing deficits.

  12. 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.

  13. 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.

  14. 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.

  15. 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.

  16. Childhood sensorineural hearing loss: effects of combined exposure with aging or noise exposure later in life.

    Science.gov (United States)

    Aarhus, Lisa; Tambs, Kristian; Nafstad, Per; Bjørgan, Eskil; Engdahl, Bo

    2016-05-01

    The aim of the study was to examine childhood high-frequency sensorineural hearing loss (HF-SNHL) and the effects of combined exposure with aging or noise exposure on HF hearing thresholds in adulthood. Population-based cohort study of 30,003 adults (mean age 40 years) underwent an audiometry and completed a hearing questionnaire. At age 7-13 years, the same people had participated in a longitudinal school hearing investigation, in which 283 participants were diagnosed with HF-SNHL [PTA 3-8 kHz ≥ 25 dB HL (mean 45 dB HL), worse hearing ear], and 29,720 participants had normal hearing thresholds. The effect of childhood HF-SNHL on adult hearing threshold was significantly moderated by age. Age stratified analyses showed that the difference in HF hearing thresholds between adults with and without childhood HF-SNHL was 33 dB (95 % CI 31-34) in young adults (n = 173, aged 20-39 years) and 37 dB (95 % CI 34-39) in middle-aged adults (n = 110, aged 40-56 years). The combined exposure of childhood HF-SNHL and noise exposure showed a simple additive effect. It appears to be a super-additive effect of childhood-onset HF-SNHL and aging on adult hearing thresholds. An explanation might be that already damaged hair cells are more susceptible to age-related degeneration. To exclude possible birth cohort effects, the finding should be confirmed by a study with several audiometries in adulthood.

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

    Directory of Open Access Journals (Sweden)

    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.

  18. Variability in voice fundamental frequency of sustained vowels in speakers with sensorineural hearing loss.

    Science.gov (United States)

    Lee, Guo-She

    2012-01-01

    In a previous study, the low-frequency modulation extent (LFP) of the vocal fundamental frequency (F(0)) showed a significant increase in the presence of binaural noise masking for the healthy individuals. This study was to investigate the F(0) of subjects with sensorineural hearing loss (SNHL) using sustained phonations to explore the changes of F(0) modulations in SNHL. Twenty-three SNHL subjects and 14 age-matched subjects without hearing loss were enrolled in the study. Sustained vocalizations of vowel /a/ for more than 5 seconds were digitally recorded. The F(0) contour of each phonation was acquired using digital signal processing. The modulation extent at different frequencies was obtained using Fourier transformation of F(0) contour. The LFP of F(0) (Voice Foundation. Published by Mosby, Inc. All rights reserved.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  1. 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.

  2. Frequency discrimination and literacy skills in children with mild to moderate sensorineural hearing loss.

    Science.gov (United States)

    Halliday, L F; Bishop, D V M

    2005-10-01

    It has been suggested that specific reading disability (SRD) may be attributable to an impaired ability to perceive spectral differences between sounds that leads to a deficit in frequency discrimination and subsequent problems with language and literacy. The objective of the present study was three-fold. We aimed to (a) determine whether children with mild to moderate sensorineural hearing loss were impaired in their ability to discriminate frequency, (b) assess the extent to which any such deficits may be due to an inability to use information derived from phase locking, and (c) examine whether frequency discrimination abilities were predictive of measures of word and nonword reading and nonword repetition. Difference limens for frequency (DLFs) were obtained for 22 children with mild to moderate hearing loss (SNH group) and 22 age-matched controls (CA group) at central frequencies of 1 kHz, where phase-locking information is available, and 6 kHz, where it is not. A battery of standardized tests of language and literacy was also administered. The SNH group exhibited significantly elevated DLFs at both 1 and 6 kHz relative to controls, despite considerable variability of thresholds in both groups. Although no group differences were found for receptive and expressive vocabulary, receptive grammar, and nonword reading, the SNH group performed worse than controls on word reading and nonword repetition, even though word reading scores were age-appropriate. Frequency discrimination abilities were associated with reading and nonword repetition across groups, but these correlations largely disappeared when the two groups were analyzed separately. Together, these results provide evidence for a dissociation between impaired frequency discrimination and relatively "spared" language and literacy in children with mild to moderate sensorineural hearing loss. These results cast doubt on the assertion that a deficit in frequency discrimination necessarily leads to marked

  3. 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 HzCF, 800 HzCF, 1600 HzCF, 3200 HzCF, or 6400 HzCF, 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 HzCF to 1600 HzCF, due to the severe or profound hearing loss in higher frequency ranges. Using a stringent statistical threshold (p < 0.05; FWE) significant differences between NH and SNHL were only revealed for mid and high-frequency sounds. At a more lenient statistical threshold (p < 0.001, FDRc), however, the size of activation induced by 400 HzCF in PAC was found statistically larger in patients with a prelingual, as compared to a postlingual onset of hearing loss. In addition, this low-frequency range was more extensively represented in the 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

  4. [From gene to disease; DFNA8/12, an autosomal dominant inherited bowl-shaped sensorineural hearing impairment

    NARCIS (Netherlands)

    Cremers, C.W.R.J.; Plantinga, R.F.; Kremer, H.

    2007-01-01

    An autosomal dominant inherited disorder known as DFNA8/12 causes mild-to-moderate/severe mid-frequency or mild-to-severe progressive high-frequency sensorineural hearing impairment. The causative gene, TECTA, encodes alpha-tectorin, the most important non-collagenous component of the tectorial

  5. SENSORINEURAL HEARING PROCESSES AND DISORDERS, HENRY FORD HOSPTIAL INTERNATIONAL SYMPOSIUM (DETROIT, MICHIGAN, MARCH 25-27, 1965).

    Science.gov (United States)

    GRAHMA, A. BRUCE, ED.

    IN ORDER TO PROVIDE BOTH CLINICIAN AND INVESTIGATOR WITH A BETTER UNDERSTANDING OF THE PROBLEMS, CAUSES, IDENTIFICATION, AND HANDLING OF SENSORINEURAL HEARING LOSSES, THE HENRY FORD HOSPITAL INVITED INVESTIGATORS IN PSYCHOACOUSTICS, OTIOLOGY, OTONEUROLOGY, PHSIOLOGY, AND AUDIOLOGY TO SHARE THEIR RESEARCH AND CLINICAL FINDINGS. THE RESULTING 40…

  6. Sudden sensorineural hearing loss and vertigo associated with arterial occlusive disease: three case reports and literature review

    OpenAIRE

    Castro Junior, Ney Penteado de; Almeida, Clemente Isnard Ribeiro de; Campos, Carlos Alberto Herrerias de

    2007-01-01

    Sudden sensorineural hearing loss and vertigo (SSNHLV) has multifactorial causes, of which viral, autoimmune and vascular insufficiency are the most common. The therapeutic management for SSNHLV includes antiviral drugs, corticosteroids, vasodilators, normovolemic hemodilution therapy and hyperbaric oxygen therapy. Vertebrobasilar occlusive disease and carotid occlusive disease are seldom related to SSNHLV. Discussions concerning SSNHLV caused by occlusive vascular disease are important and n...

  7. 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

  8. Sensorineural hearing loss amplifies neural coding of envelope information in the central auditory system of chinchillas.

    Science.gov (United States)

    Zhong, Ziwei; Henry, Kenneth S; Heinz, Michael G

    2014-03-01

    People with sensorineural hearing loss often have substantial difficulty understanding speech under challenging listening conditions. Behavioral studies suggest that reduced sensitivity to the temporal structure of sound may be responsible, but underlying neurophysiological pathologies are incompletely understood. Here, we investigate the effects of noise-induced hearing loss on coding of envelope (ENV) structure in the central auditory system of anesthetized chinchillas. ENV coding was evaluated noninvasively using auditory evoked potentials recorded from the scalp surface in response to sinusoidally amplitude modulated tones with carrier frequencies of 1, 2, 4, and 8 kHz and a modulation frequency of 140 Hz. Stimuli were presented in quiet and in three levels of white background noise. The latency of scalp-recorded ENV responses was consistent with generation in the auditory midbrain. Hearing loss amplified neural coding of ENV at carrier frequencies of 2 kHz and above. This result may reflect enhanced ENV coding from the periphery and/or an increase in the gain of central auditory neurons. In contrast to expectations, hearing loss was not associated with a stronger adverse effect of increasing masker intensity on ENV coding. The exaggerated neural representation of ENV information shown here at the level of the auditory midbrain helps to explain previous findings of enhanced sensitivity to amplitude modulation in people with hearing loss under some conditions. Furthermore, amplified ENV coding may potentially contribute to speech perception problems in people with cochlear hearing loss by acting as a distraction from more salient acoustic cues, particularly in fluctuating backgrounds. Copyright © 2013 Elsevier B.V. All rights reserved.

  9. Non-flat audiograms in sensorineural hearing loss and speech perception

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    Kelly Cristina Lira de Andrade

    2013-06-01

    Full Text Available OBJECTIVE: The audibility thresholds for the sound frequency of 137 upward- and downward-sloping audiograms showing sensorineural hearing loss were selected and analyzed in conjunction with speech recognition thresholds obtained from individuals seen at a public otolaryngology clinic to determine which frequencies in slope audiograms best represent speech recognition thresholds. METHOD: The linear regression model and mean square error were used to determine the associations between the threshold values. RESULT: The mean square error identified larger errors when using thresholds of 500, 1000, and 2000 Hz than when using audibility thresholds of 500, 1000, 2000, and 4000 Hz. The linear regression model showed a higher correlation (91% between the audiogram thresholds for frequencies of 500, 1000, 2000, and 4000 Hz than for the frequencies of 500, 1000, and 2000 Hz (88%. CONCLUSION: Frequencies of 500, 1000, 2000, and 4000 Hz were the most significant in predicting the speech recognition threshold.

  10. Changes of rhythm of vocal fundamental frequency in sensorineural hearing loss and in Parkinson's disease.

    Science.gov (United States)

    Lee, Guo-She; Lin, Sheng-Huang

    2009-12-31

    The neurological control of speech is a complex process that involves phonation organs, respiratory and auditory systems. In the instance of a steady-as-possible sustained phonation, the vocal fundamental frequency (F0) is rhythmic and oscillating in varied degree. The present study examines the changes in the rhythm of F0 in subjects with sensorineural hearing loss (SNHL) as well as in subjects with Parkinson's disease (PD) after being orally administered with dopamine. The sustained steady vocalizations of vowel [a:] from 19 subjects with SNHL and from 13 subjects with normal hearing were collected and statistically compared. In addition, the phonations of 14 subjects with PD before and after oral medication with oral dopamine were collected and statistically compared. The F0 of a phonation was retrieved by digital signal processing of voice signals, and were then analyzed using Fourier transformation to acquire the amplitude of oscillation at different frequency components. Our study showed that subjects with SNHL had significantly larger fluctuation in the low frequency (< 3 Hz) than the subjects with normal hearing. In addition, dopamine medication significantly reduced the fluctuation in the mid-frequency (3-8 Hz) in subjects with PD. Our study indicates that power spectral analysis of F0 may potentially be very useful in the evaluation or detection of SNHL and PD. The rhythms of F0 are produced from neurological controls of phonation and may be used to access clinical diseases by a sustained phonation.

  11. 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.

  12. The Role of High Dose Intratympanic Dexamethasone as Salvage Therapy for Idiopathic Sudden Sensorineural Hearing Loss.

    Science.gov (United States)

    Kordiš, Špela; Battelino, Saba

    2017-11-21

    The aim of this study was to assess the efficacy of a single high dose intratympanic (IT) dexamethasone (DEX) as salvage therapy for idiopathic sudden sensorineural hearing loss (ISSNHL) after unsuccessful treatment with oral corticosteroid (CS). This was a prospective open-label study of 59 patients treated with IT DEX after systemic CS therapy has failed. All patients received high dose (24 mg/mL) IT DEX in a single injection through myringotomy. Of the 59 patients, 40.7% showed improvement in their mean pure tone average (PTA) with IT DEX (p=0.005). The difference in the mean PTA after oral CS treatment only from baseline was not statistically significant (p=0.074). The time from onset of hearing loss to the start of therapy was significantly associated with the outcome (p=0.03). We determined that high dose IT DEX as salvage therapy was beneficial when the primary treatment with oral CS had failed. An early start of the treatment significantly influenced the improvement of hearing.

  13. Risk of sudden sensorineural hearing loss in patients with end-stage renal disease undergoing dialysis.

    Science.gov (United States)

    Wang, I-Kuan; Wang, Ching-Yuan; Muo, Chih-Hsin; Yen, Tzung-Hai; Sung, Fung-Chang

    2017-05-01

    The study evaluated the risk of sudden sensorineural hearing loss (SSHR) in patients with end-stage renal disease (ESRD) undergoing dialysis METHODS: We identified 18 905 patients newly diagnosed with ESRD, comprising 6382 patients on peritoneal dialysis (PD) and 12 523 patients on hemodialysis (HD) using Taiwan's National Health Insurance claims data from 2000 to 2010. A total of 76 520 controls without kidney disease were randomly selected with frequency matched with the ESRD group by age and sex. We further established additional cohorts with 6375 patients on PD and 6375 patients on HD matched by their propensity scores. Cox proportional hazards models were used to examine the effect of ESRD and dialysis modality on the risk of sudden sensorineural hearing loss (SSHL) by the end of 2011. The incidences for SSHR were 0.49, 2.37, 2.95, and 2.12 per 1000 person-years in the control, ESRD (PD and HD), PD, and HD cohorts, respectively. The adjusted HRs of SSHR was 2.71, 3.60, and 2.33 for ESRD, PD, and HD groups (95% CI = 2.17-3.38, 2.71-4.78 and 1.81-2.99, respectively) compared with the control cohort. Patients on PD exhibited 1.53-fold (95% CI = 1.14-2.04) and 1.64-fold (95% CI = 1.19-2.27) higher SSHL risk than their age- and sex-matched and propensity score-matched patients on HD, respectively. Patients with ESRD exhibit a higher risk of SSHL than people without kidney disease. The SSHL risk is greater for patients on PD than on HD. © 2016 Asian Pacific Society of Nephrology.

  14. Effects of sensorineural hearing loss on temporal coding of narrowband and broadband signals in the auditory periphery.

    Science.gov (United States)

    Henry, Kenneth S; Heinz, Michael G

    2013-09-01

    People with sensorineural hearing loss have substantial difficulty understanding speech under degraded listening conditions. Behavioral studies suggest that this difficulty may be caused by changes in auditory processing of the rapidly-varying temporal fine structure (TFS) of acoustic signals. In this paper, we review the presently known effects of sensorineural hearing loss on processing of TFS and slower envelope modulations in the peripheral auditory system of mammals. Cochlear damage has relatively subtle effects on phase locking by auditory-nerve fibers to the temporal structure of narrowband signals under quiet conditions. In background noise, however, sensorineural loss does substantially reduce phase locking to the TFS of pure-tone stimuli. For auditory processing of broadband stimuli, sensorineural hearing loss has been shown to severely alter the neural representation of temporal information along the tonotopic axis of the cochlea. Notably, auditory-nerve fibers innervating the high-frequency part of the cochlea grow increasingly responsive to low-frequency TFS information and less responsive to temporal information near their characteristic frequency (CF). Cochlear damage also increases the correlation of the response to TFS across fibers of varying CF, decreases the traveling-wave delay between TFS responses of fibers with different CFs, and can increase the range of temporal modulation frequencies encoded in the periphery for broadband sounds. Weaker neural coding of temporal structure in background noise and degraded coding of broadband signals along the tonotopic axis of the cochlea are expected to contribute considerably to speech perception problems in people with sensorineural hearing loss. This article is part of a Special Issue entitled "Annual Reviews 2013". Copyright © 2013 Elsevier B.V. All rights reserved.

  15. 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

  16. High Total Cholesterol in Peripheral Blood Correlates with Poorer Hearing Recovery in Idiopathic Sudden Sensorineural Hearing Loss.

    Science.gov (United States)

    Quaranta, Nicola; Squeo, Valentina; Sangineto, Moris; Graziano, Giusi; Sabbà, Carlo

    2015-01-01

    Idiopathic sudden sensorineural hearing loss (ISSHL) is a common otologic emergency whose cause is still unclear. The importance of blood lipids in the pathogenesis of ISSHL is widely reported in literature. In fact elevated levels of low density lipoprotein cholesterol (LDL), total cholesterol (TC) and apolipoprotein B (Apo-B) have been proposed as risk factors for this pathology. No correlation has been described between serum lipid parameters and the prognosis of ISSHL. Aim of the present study was to identify prognostic factors associated with hearing recovery in a group of patients affected by ISSHL. Ninety-four patients with the diagnosis of ISSHL hospitalized between March 2013 and October 2014 were included in this study. Patients' blood sampling and hearing assessments were carried out. Patients were divided into two groups as "recovered" and "unrecovered", according to their response to the treatment. We found a statistically significant higher level of total cholesterol in the unrecovered group compared to the recovered one (p = 0.03). None of the other routine laboratory parameters have shown a statistically significant difference between the patients successfully treated and patients with poor outcomes. Total cholesterol concentrations may be a prognostic factor for recovery in ISSHL and should be assessed together with routine tests in patients with this condition. The other routine laboratory parameters seem to have no effect on the development and prognosis of this pathology.

  17. Relationship between CT findings and sensorineural hearing loss in chronic otitis media.

    Science.gov (United States)

    Yoshida, Haruo; Miyamoto, Ikue; Takahashi, Haruo

    2014-06-01

    To investigate the relationships between the temporal bone CT findings and sensorineural hearing loss in ears with non-cholesteatomatous chronic otitis media (COM). Preoperative bone conduction (BC) hearing thresholds of 266 patients (304 ears) with COM were compared with those of 342 normal individuals (440 ears) by audiometry. The incidence of abnormal BC threshold at lower frequencies (250-1000 Hz) and at higher frequencies (2000-4000 Hz) were examined and the differences between control and COM groups were compared by using χ2 test. In the COM group, the cross-sectional area of the mastoid air cells based on the axial CT image (n=255) were correlated with the results of BC threshold. The percentage in the COM group exceeds 15% in their 50s at lower frequencies while in their 40s at higher frequencies. The BC thresholds were significantly better in the group with normal mastoid area than in those with smaller mastoid area at each decadal age group. The BC impairment from COM becomes worse as the course of the disease progresses and deteriorated from 40s rapidly, especially at higher frequencies and in the group with smaller mastoid area. These results recommend that early treatment, including surgery, should be considered as early as possible before BC impairment occurs, especially for COM cases with smaller mastoid area, which may indicate the existence of more severe otitis media since earlier childhood. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

  18. Novel approaches to treating sensorineural hearing loss. Auditory genetics and necessary factors for stem cell transplant.

    Science.gov (United States)

    Vlastarakos, Petros V; Nikolopoulos, Thomas P; Tavoulari, Evangelia; Kiprouli, Catherine; Ferekidis, Eleftherios

    2008-08-01

    Sensorineural hearing loss is a chronic disease, with a serious impact on human communication and quality of life. Exposure to various factors can lead to irreversible hearing impairment, as the auditory epithelium in humans comprises terminally differentiated cells. By contrast, the inner ear of lower vertebrates and invertebrates shows regenerative capacity. Efforts to regenerate the damaged human inner ear may involve renewed cell proliferation, or transplanting cells that can differentiate into sensory cells. Literature review. Animal studies, in vitro studies, retrospective-cohort studies, community-based case-controls, clinical guidelines, and review articles. Embryonic stem cells, inner ear stem cells, and stem cells from other tissues (i.e., neural tissue, hematopoietic system) may be candidates for restoring the auditory epithelium. Transcriptional regulation of p27kip1 is the primary determinant of terminal mitosis and the final number of postmitotic progenitors of hair and supporting cells. Basic helix-loop-helix transcription factor Math1 was found to be necessary and sufficient for the production of auditory hair cells. Notch signaling seems to play a major role in the regulation of Math1, through lateral inhibition. Brn3c, Gfi1, and Barhl1 are also specific transcription factors that have been implicated in hair cell maintenance and consequent survival. Evidence concerning development, maintenance, and regeneration of hair cells is still at an embryonic stage. Combined data, as attempted in the present study, will lead to a more successful management of deafness.

  19. Recurrence of idiopathic sudden sensorineural hearing loss: a retrospective cohort study.

    Science.gov (United States)

    Wu, Che-Ming; Lee, Kuan-Ji; Chang, Shih-Lun; Weng, Shih-Feng; Lin, Yung-Song

    2014-12-01

    To explore the recurrence rate of idiopathic sudden sensorineural hearing loss (ISSNHL). Using the Taiwan Longitudinal Health Insurance Database, we compared in a retrospective cohort study 45,715 patients with ISSNHL between January 2001 and December 2006 with 45,715 age-, gender-, and comorbidity-matched controls without ISSNHL. We followed each patient from 180 days after the initial diagnosis until the end of 2009 and evaluated the incidence of ISSNHL for 3 years minimum. The cumulative incidence of ISSNHL recurrence was 4.99%. Cox proportional hazard regressions showed that the 50- to 64-year-old age group had a higher adjusted hazard ratio (1.59; 95% confidence interval: 1.40-1.81) than did the 0- to 34-year-old age group. Different comorbidities did not significantly affect the incidence of ISSNHL relapse. The risk of an ISSNHL relapse in patients with a history of ISSNHL was higher than was the risk of a first occurrence of ISSNHL in the Controls. We suggest that physicians counsel patients with a history of ISSNHL to seek medical attention if they have hearing impairments because they may also have a high risk of an ISSNHL relapse. 2B.

  20. Comprehensive Genetic Analysis of Japanese Autosomal Dominant Sensorineural Hearing Loss Patients.

    Directory of Open Access Journals (Sweden)

    Yoh-Ichiro Iwasa

    Full Text Available In general, autosomal dominant inherited hearing loss does not have a founder mutation, with the causative mutation different in each family. For this reason, there has been a strong need for efficient diagnosis methods for autosomal dominant sensorineural hearing loss (ADSNHL patients. This study sought to verify the effectiveness of our analysis algorithm for the screening of ADSNHL patients as well as the usefulness of the massively parallel DNA sequencing (MPS.Seventy-five Japanese ADSNHL patients from 53 ENT departments nationwide participated in this study. We conducted genetic analysis of 75 ADSNHL patients using the Invader assay, TaqMan genotyping assay and MPS-based genetic screening.A total of 46 (61.3% ADSNHL patients were found to have at least one candidate gene variant.We were able to achieve a high mutation detection rate through the combination of the Invader assay, TaqMan genotyping assay and MPS. MPS could be used to successfully identify mutations in rare deafness genes.

  1. 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.

  2. 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

  3. Temporal modulation transfer functions measured from auditory-nerve responses following sensorineural hearing loss.

    Science.gov (United States)

    Kale, Sushrut; Heinz, Michael G

    2012-04-01

    The ability of auditory-nerve (AN) fibers to encode modulation frequencies, as characterized by temporal modulation transfer functions (TMTFs), generally shows a low-pass shape with a cut-off frequency that increases with fiber characteristic frequency (CF). Because AN-fiber bandwidth increases with CF, this result has been interpreted to suggest that peripheral filtering has a significant effect on limiting the encoding of higher modulation frequencies. Sensorineural hearing loss (SNHL), which is typically associated with broadened tuning, is thus predicted to increase the range of modulation frequencies encoded; however, perceptual studies have generally not supported this prediction. The present study sought to determine whether the range of modulation frequencies encoded by AN fibers is affected by SNHL, and whether the effects of SNHL on envelope coding are similar at all modulation frequencies within the TMTF passband. Modulation response gain for sinusoidally amplitude modulated (SAM) tones was measured as a function of modulation frequency, with the carrier frequency placed at fiber CF. TMTFs were compared between normal-hearing chinchillas and chinchillas with a noise-induced hearing loss for which AN fibers had significantly broadened tuning. Synchrony and phase responses for individual SAM tone components were quantified to explore a variety of factors that can influence modulation coding. Modulation gain was found to be higher than normal in noise-exposed fibers across the entire range of modulation frequencies encoded by AN fibers. The range of modulation frequencies encoded by noise-exposed AN fibers was not affected by SNHL, as quantified by TMTF 3- and 10-dB cut-off frequencies. These results suggest that physiological factors other than peripheral filtering may have a significant role in determining the range of modulation frequencies encoded in AN fibers. Furthermore, these neural data may help to explain the lack of a consistent association

  4. Temporal Modulation Transfer Functions Measured From Auditory-Nerve Responses Following Sensorineural Hearing Loss

    Science.gov (United States)

    Kale, Sushrut; Heinz, Michael G.

    2012-01-01

    The ability of auditory-nerve (AN) fibers to encode modulation frequencies, as characterized by temporal modulation transfer functions (TMTFs), generally shows a low-pass shape with a cut-off frequency that increases with fiber characteristic frequency (CF). Because AN-fiber bandwidth increases with CF, this result has been interpreted to suggest that peripheral filtering has a significant effect on limiting the encoding of higher modulation frequencies. Sensorineural hearing loss (SNHL), which is typically associated with broadened tuning, is thus predicted to increase the range of modulation frequencies encoded; however, perceptual studies have generally not supported this prediction. The present study sought to determine whether the range of modulation frequencies encoded by AN fibers is affected by SNHL, and whether the effects of SNHL on envelope coding are similar at all modulation frequencies within the TMTF passband. Modulation response gain for sinusoidally amplitude modulated (SAM) tones was measured as a function of modulation frequency, with the carrier frequency placed at fiber CF. TMTFs were compared between normal-hearing chinchillas and chinchillas with a noise-induced hearing loss for which AN fibers had significantly broadened tuning. Synchrony and phase responses for individual SAM-tone components were quantified to explore a variety of factors that can influence modulation coding. Modulation gain was found to be higher than normal in noise-exposed fibers across the entire range of modulation frequencies encoded by AN fibers. The range of modulation frequencies encoded by noise-exposed AN fibers was not affected by SNHL, as quantified by TMTF 3- and 10-dB cut-off frequencies. These results suggest that physiological factors other than peripheral filtering may have a significant role in determining the range of modulation frequencies encoded in AN fibers. Furthermore, these neural data may help to explain the lack of a consistent association

  5. Speech recognition performance of patients with sensorineural hearing loss under unaided and aided conditions using linear and compression hearing AIDS.

    Science.gov (United States)

    Shanks, Janet E; Wilson, Richard H; Larson, Vern; Williams, David

    2002-08-01

    This study compared speech recognition performance on the Northwestern University Auditory Test No. 6 (NU-6) and the Connected Speech Test (CST) for three hearing aid circuits (peak clipping [PC], compression limiting [CL], and wide dynamic range compression [WDRC]) in adults with symmetrical sensorineural hearing loss. The study also questioned whether or not hearing aid benefit for the three circuits was dependent upon the speech level and the signal-to-babble ratio (S/B) and upon the degree and slope of hearing loss. Unaided speech recognition performance for NU-6 and CST materials presented from a loudspeaker at 0 degrees was measured during Visit 1, and both unaided and aided performance was measured at 3-mo intervals during Visits 2 to 4. The NU-6 was presented in quiet at a conversational speech level of 62 dB SPL. The CST was presented in 10 listening conditions-three S/B (-3, 0, and 3 dB) at each of three speech levels (soft speech at 52 dB SPL, conversational speech at 62 dB SPL, and loud speech at 74 dB SPL) and in quiet at 74 dB SPL. Uncorrelated multi-talker babble was presented from two loudspeakers at 45 degrees on each side of the main speaker. Hearing aid benefit was examined for 360 subjects divided into four groups of hearing loss, pure tone average 10 dB/octave and hearing loss >40 dB HL for the two slope categories. Hearing aid benefit (aided minus unaided performance) measured on the NU-6 in quiet exceeded 31 rau for all three circuits. Although small statistical advantages were found for the WDRC, the differences were approximately 2% and are not considered clinically relevant. Unaided CST performance showed a complex relationship between presentation level and signal-to-babble ratio that was further confounded by the degree of hearing loss. For the two mild hearing loss groups and for each of the three nominal signal-to-babble ratios, CST performance decreased by 20 rau for the -3 dB S/B to 6 rau for the 3 dB S/B as speech level increased

  6. 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....

  7. 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,

  8. PSIP1/LEDGF: a new gene likely involved in sensorineural progressive hearing loss

    Science.gov (United States)

    Girotto, Giorgia; Scheffer, Déborah I.; Morgan, Anna; Vozzi, Diego; Rubinato, Elisa; Di Stazio, Mariateresa; Muzzi, Enrico; Pensiero, Stefano; Giersch, Anne B.; Corey, David P.; Gasparini, Paolo

    2015-01-01

    Hereditary Hearing Loss (HHL) is an extremely heterogeneous disorder. Approximately 30 out of 80 known HHL genes are associated with autosomal dominant forms. Here, we identified PSIP1/LEDGF (isoform p75) as a novel strong candidate gene involved in dominant HHL. Using exome sequencing we found a frameshift deletion (c.1554_1555del leading to p.E518Dfs*2) in an Italian pedigree affected by sensorineural mild-to-moderate HHL but also showing a variable eye phenotype (i.e. uveitis, optic neuropathy). This deletion led to a premature stop codon (p.T519X) with truncation of the last 12 amino acids. PSIP1 was recently described as a transcriptional co-activator regulated by miR-135b in vestibular hair cells of the mouse inner ear as well as a possible protector against photoreceptor degeneration. Here, we demonstrate that it is ubiquitously expressed in the mouse inner ear. The PSIP1 mutation is associated with a peculiar audiometric slope toward the high frequencies. These findings indicate that PSIP1 likely plays an important role in HHL. PMID:26689366

  9. Autocorrelation factors and intelligibility of Japanese monosyllables in individuals with sensorineural hearing loss.

    Science.gov (United States)

    Shimokura, Ryota; Akasaka, Sakie; Nishimura, Tadashi; Hosoi, Hiroshi; Matsui, Toshie

    2017-02-01

    Some Japanese monosyllables contain consonants that are not easily discernible for individuals with sensorineural hearing loss. However, the acoustic features that make these monosyllables difficult to discern have not been clearly identified. Here, this study used the autocorrelation function (ACF), which can capture temporal features of signals, to clarify the factors influencing speech intelligibility. For each monosyllable, five factors extracted from the ACF [Φ(0): total energy; τ1 and ϕ1: delay time and amplitude of the maximum peak; τe: effective duration; Wϕ(0): spectral centroid], voice onset time, speech intelligibility index, and loudness level were compared with the percentage of correctly perceived articulations (144 ears) obtained by 50 Japanese vowel and consonant-vowel monosyllables produced by one female speaker. Results showed that median effective duration [(τe)med] was strongly correlated with the percentage of correctly perceived articulations of the consonants (r = 0.87, p < 0.01). (τe)med values were computed by running ACFs with the time lag at which the magnitude of the logarithmic-ACF envelope had decayed to -10 dB. Effective duration is a measure of temporal pattern persistence, i.e., the duration over which the waveform maintains a stable pattern. The authors postulate that low recognition ability is related to degraded perception of temporal fluctuation patterns.

  10. Visualization of inner ear dysplasias in patients with sensorineural hearing loss.

    Science.gov (United States)

    Klingebiel, R; Bockmühl, U; Werbs, M; Freigang, B; Vorwerk, W; Thieme, N; Lehmann, R

    2001-11-01

    We evaluated a data acquisition and post-processing protocol for inner ear (IE) assessment by MR imaging in patients, suffering from various labyrinth malformations. MR IE studies of 158 consecutive patients (316 IEs) suffering from sensorineural hearing loss without evidence of an acoustic neurinoma were reviewed for pathologies of the IE and internal acoustic meatus. High-resolution MR data of all abnormal IE studies (n=45) were post-processed to previously standardized 3D volume rendered (VR) reconstructions. In 9 patients (5.7%) the following IE dysplasias were detected: malformation of the cochlea (6 IEs), vestibulum (4 IEs), semicircular canals (12 IEs) and vestibular aqueduct/endolymphatic sac (10 IEs). One patient showed evidence of an aplasia of the vestibulocochlear nerve. In 4 patients multiple IE dysplasias were encountered. Comprehensive 3D visualization of all labyrinthine dysplasias was achieved by the use of two VR reconstructions. The overall time for bilateral IE assessment amounted to 30-35 min. The imaging protocol allows for rapid and comprehensive visualization of various IE dysplasias, based on a limited number of VR reconstructions.

  11. Two new cases with microdeletion of 17q23.2 suggest presence of a candidate gene for sensorineural hearing loss within this region

    DEFF Research Database (Denmark)

    Schönewolf-Greulich, Bitten; Ronan, Anne; Ravn, Kristine

    2011-01-01

    . In this report, we describe two new 17q23.2 deletion patients with mild intellectual disability and sensorineural hearing loss. They both had submicroscopic deletions smaller than the common deleted region for the 8 previously described 17q23.2 microdeletion cases. TBX4 was previously suggested...... as the responsible gene for the heart or limb defects observed in 17q23.2 deletion patients, but the present cases do not have these features despite deletion of this gene. The finding of sensorineural hearing loss in 5 of the 10 cases, including the present cases, with a microdeletion at17q23.2, strongly suggests...... the presence of a candidate gene for hearing loss within this region. We screened 41 patients with profound sensorineural hearing loss for mutations of TBX2 and detected no mutations....

  12. A comparison of conventional and in-situ audiometry on participants with varying levels of sensorineural hearing loss.

    Science.gov (United States)

    Kiessling, Jürgen; Leifholz, Melanie; Unkel, Steffen; Pons-Kühnemann, Jörn; Jespersen, Charlotte Thunberg; Pedersen, Jenny Nesgaard

    2015-01-01

    In-situ audiometry is a hearing aid feature that enables the measurement of hearing threshold levels through the hearing instrument using the built-in sound generator and the hearing aid receiver. This feature can be used in hearing aid fittings instead of conventional pure-tone audiometry (PTA), particularly in places where no standard audiometric equipment is available. Differences between conventional and in-situ thresholds are described and discussed for some particular hearing aids. No previous investigation has measured and compared these differences for a number of current hearing aid models by various manufacturers across a wide range of hearing losses. The purpose of this study was to perform a model-based comparison of conventionally and in-situ measured hearing thresholds. Data were collected for a range of hearing aid devices to study and generalize the effects that may occur under clinical conditions. Research design was an experimental and regression study. A total of 30 adults with sensorineural hearing loss served as test persons. They were assigned to three subgroups of 10 subjects with mild (M), moderate to severe (MS), and severe (S) sensorineural hearing loss. All 30 test persons underwent both conventional PTA and in-situ audiometry with four hearing aid models by various manufacturers. The differences between conventionally and in-situ measured hearing threshold levels were calculated and evaluated by an exploratory data analysis followed by a sophisticated statistical modeling process. At 500 and 1500 Hz, almost all threshold differences (conventional PTA minus in-situ data) were negative, i.e., in the low to mid frequencies, hearing loss was overestimated by most devices relative to PTA. At 4000 Hz, the majority of differences (7 of 12) were positive, i.e., in the frequency range above 1500 Hz, hearing loss was frequently underestimated. As hearing loss increased (M→MS→S), the effect of the underestimation decreased. At 500 and 1500 Hz

  13. 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.

  14. 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.

  15. The importance of an extended preoperative trial of BAHA in unilateral sensorineural hearing loss: a prospective cohort study.

    Science.gov (United States)

    Pennings, R J E; Gulliver, M; Morris, D P

    2011-10-01

    The influence of an extended preoperative trial of bone-anchored hearing aid (BAHA) and headband on the decision to proceed with surgery in cases of unilateral sensorineural hearing loss is studied. In this prospective study, the headband was introduced after assessment confirmed suitable candidacy. Those who had an initially favourable response to the headband trial were encouraged to complete a 2-week home trial of device and headband. Patients were asked to pay special attention to situations where they perceived high demands on their hearing, e.g. at the dinner table, in the car, walking outside etc. Counselling regarding the BAHA placement, the need for daily care of the implant site and the financial implications of acquiring the device was also given. Tertiary university hospital. Patients with unilateral sensorineural hearing loss, fulfilling criteria for BAHA surgery, with favourable response to initial 'office trial' of headband. After 2-week home trial, BAHA and headband were returned with a statement of the patient's intentions. Sixteen of 50 (32%) patients who expressed definite interest in BAHA surgery after a positive introduction to the headband in the office did not wish to proceed to surgery having completed a more thorough 2-week trial of device at home. Most patients cited a failure of the device to meet their hearing requirements. Subsequently, we found further attrition with nine more patients (18%) deciding at a still later date, not to proceed with surgery. Ultimately, just 25 of 50 (50%) of our initially enthusiastic group chose not to proceed to surgery. In the group who did proceed, there were no non-users. We propose the importance of an extended preoperative trial where BAHA is considered in the rehabilitation of unilateral sensorineural hearing loss. The trial may foster more realistic expectations of device performance in this challenging group. A 'cooling-off' period before surgery also appears to lead to further attrition with the

  16. Plasticity in the developing auditory cortex: evidence from children with sensorineural hearing loss and auditory neuropathy spectrum disorder.

    Science.gov (United States)

    Cardon, Garrett; Campbell, Julia; Sharma, Anu

    2012-06-01

    The developing auditory cortex is highly plastic. As such, the cortex is both primed to mature normally and at risk for reorganizing abnormally, depending upon numerous factors that determine central maturation. From a clinical perspective, at least two major components of development can be manipulated: (1) input to the cortex and (2) the timing of cortical input. Children with sensorineural hearing loss (SNHL) and auditory neuropathy spectrum disorder (ANSD) have provided a model of early deprivation of sensory input to the cortex and demonstrated the resulting plasticity and development that can occur upon introduction of stimulation. In this article, we review several fundamental principles of cortical development and plasticity and discuss the clinical applications in children with SNHL and ANSD who receive intervention with hearing aids and/or cochlear implants. American Academy of Audiology.

  17. 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

    Directory of Open Access Journals (Sweden)

    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.

  18. 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.

  19. Longitudinal benefit from and satisfaction with the Baha system for patients with acquired unilateral sensorineural hearing loss.

    Science.gov (United States)

    Newman, Craig W; Sandridge, Sharon A; Wodzisz, Lisa M

    2008-12-01

    To analyze the short-, medium-, and long-term benefits from and satisfaction with the Baha for patients with profound unilateral sensorineural hearing loss (SNHL). Prospective clinical study. Head and Neck Institute at the Cleveland Clinic. Eight patients with acquired profound unilateral SNHL. Patients underwent unaided baseline testing and aided Baha testing using laboratory and self-report measures at 6 postfitting intervals. Laboratory measures included the revised Speech Perception in Noise test, Hearing in Noise Test, and localization testing. Disease-specific self-report measures included the Abbreviated Profile of Hearing Aid Benefit, Hearing Handicap Inventory for Adults, and Single-Sided Deafness Questionnaire. The Medical Outcomes Study SF-36 Health Survey was used to assess health-related quality of life. Based on 95% confidence intervals for unaided testing, significant improvements on aided revised Speech Perception in Noise, Abbreviated Profile of Hearing Aid Benefit, and Hearing Handicap Inventory for Adults scores were sustained during the 18-month test interval. Variable performance was observed for the Hearing in Noise Test over time and patients. No acclimatization effects were shown for localization testing or the generic health-related quality-of-life measure. Patients reported satisfaction in a variety of situations as quantified by the Single-Sided Deafness Questionnaire in the long-term. The Baha is effective in reducing psychosocial consequences of unilateral profound SNHL for the long-term. Improvement in speech understanding occurred when the primary signal was spatially separated from background noise. Localization performance did not improve with Baha use. Overall, patients were satisfied with their Baha and would still elect to have this procedure if given a second chance.

  20. 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).

  1. Progressive late-onset sensorineural hearing loss and vestibular impairment with vertigo (DFNA9/COCH): longitudinal analyses in a belgian family.

    NARCIS (Netherlands)

    Lemaire, F.X.; Feenstra, L.; Huygen, P.L.M.; Fransen, E.; Devriendt, K.; Camp, G. van; Vantrappen, G.; Cremers, C.W.R.J.; Wackym, P.A.; Koss, J.C.

    2003-01-01

    OBJECTIVE: To evaluate audiometric and vestibular signs and symptoms in a new DFNA9 family. SETTING: Tertiary referral centers. METHODS: A multigeneration Belgian family with late-onset progressive sensorineural hearing loss and concomitant ves-tibular impairment with an autosomal dominant pattern

  2. Incomplete endochondral ossification of the otic capsule, a variation in children: evaluation of its prevalence and extent in children with and without sensorineural hearing loss.

    Science.gov (United States)

    Sanverdi, S E; Ozgen, B; Dolgun, A; Sarac, S

    2015-01-01

    Endochondral ossification of the otic capsule is a process that continues postnatally; hence, incomplete endochondral ossification is seen as pericochlear hypoattenuation on temporal bone CT scans of children. We determined the prevalence and extent of this entity in a large series and assessed its relation to age and underlying sensorineural hearing loss. Initially, temporal bone CTs of 40 children with sensorineural hearing loss were retrospectively assessed and compared with those of a control group scanned for non-sensorineural hearing loss reasons to assess any difference in the prevalence or extent of incomplete endochondral ossification. Then the CT scans of 510 children (age range, 17 days to 17 years) were retrospectively reviewed, and any observed endochondral ossification areas were classified as mild, moderate, or extensive, according to their extent. Neither the presence nor degree of incomplete endochondral ossification had any significant correlation with the presence of sensorineural hearing loss (P = .08 and P = .1, respectively). Incomplete endochondral ossification was more frequently seen (62% of cases) than complete ossification. There was no statistically significant correlation between incomplete endochondral ossification and sex (P = .8), but an inverse correlation was found between the presence of incomplete endochondral ossification and increasing age (P ossification was the most frequent involvement pattern (44.4%). The pericochlear hypoattenuation in the otic capsule representing incomplete endochondral ossification is a normal finding in children and can be seen as a marked curvilinear hypoattenuation at younger ages in the absence of any clinical disorder. © 2015 by American Journal of Neuroradiology.

  3. Resolution of giant basilar artery aneurysm compression and reversal of sensorineural hearing loss with use of a flow diverter: case report.

    Science.gov (United States)

    Mohammad, Laila M; Coon, Alexander L; Carlson, Andrew P

    2017-07-01

    The authors present an unusual case of a 15-year-old boy in whom sensorineural hearing loss and disequilibrium developed in the setting of a giant basilar artery aneurysm. This patient was treated with a flow-diverting stent and had complete resolution of his clinical symptoms including hearing loss. This case demonstrates the efficacy of flow diversion in select pediatric patients with posterior circulation aneurysms. The features that are thought to result in successful treatment are discussed.

  4. 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 a...... reported DFNA22 families with mutations in the MYO6 gene have been studied and compared. It seems that genetic defects that spare the motor domain of the myosin VI protein have a milder phenotype....

  5. A new autosomal-dominant locus (DFNA12) is responsible for a nonsyndromic, midfrequency, prelingual and nonprogressive sensorineural hearing loss.

    Science.gov (United States)

    Govaerts, P J; De Ceulaer, G; Daemers, K; Verhoeven, K; Van Camp, G; Schatteman, I; Verstreken, M; Willems, P J; Somers, T; Offeciers, F E

    1998-11-01

    This study aimed to report on the audiologic findings of a nonsyndromic autosomal-dominant hearing loss of which the gene (DFNA 12) recently was found to map to chromosome 11q22-24. The study also aimed to propose and evaluate an algorithm based on the audiometric findings to discriminate between affected and unaffected family members before genetic linkage analysis. The study design was a retrospective analysis of the audiometric data of genetically affected and unaffected patients. The study was conducted at a tertiary referral center. A total of 17 genetically affected and 54 unaffected family members were studied. Pure-tone audiometry with air and bone conduction and construction and evaluation of an algorithm were performed. The type and degree of hearing loss as compared to age and gender-dependent values according to the International Organization for Standardization 7029 standard were measured. For this comparison, the variable "hearing standard deviations" (HSD) is introduced and is defined as the number of standard deviations that a hearing threshold is lying above the age and gender-related median at the given frequency. A description of the algorithm and an evaluation in terms of alpha- and beta-error also were measured. The hearing loss is nonsyndromic, sensorineural, moderate-to-moderately severe (pure-tone average, 51 dB at age 18 years), with an early onset (probably prelingual) and no progression. It affects all frequencies but mainly the midfrequencies (500, 1,000, and 2,000 Hz). The algorithm consists of an analysis of variance to determine the frequency that is most sensitive for the genetic trait under study and on the ranking of the family members according to their hearing loss (HSD) at this frequency. Individual persons are labeled as "affected" or "unaffected" according to this ranking.

  6. COXPD9 an Evolving Multisystem Disease; Congenital Lactic Acidosis, Sensorineural Hearing Loss, Hypertrophic Cardiomyopathy, Cirrhosis and Interstitial Nephritis.

    Science.gov (United States)

    Bursle, C; Narendra, A; Chuk, R; Cardinal, J; Justo, R; Lewis, B; Coman, D

    2017-01-01

    We present the second report of combined oxidative phosphorylation deficiency-9. The infant presented in the neonatal period with poor feeding, lactic acidosis and sensorineural hearing loss. He subsequently developed a lethal hypertrophic cardiomyopathy during infancy. Cirrhosis and interstitial nephritis were identified at autopsy. Exome sequencing has detected compound heterozygous mutations in the MRPL3 gene which encodes a large mitochondrial ribosome subunit protein. We identified a known heterozygous variant NM_007208 c.950>G (Pro317Arg) in the MRPL3 gene and a novel heterozygous mutation NM_007208 c.49delC p.(Arg17Aspfs*57). Mutations in MRPL3 have previously been shown to alter ribosome assembly and cause abnormal function of multiple respiratory chain complexes. Our case adds to the evolving knowledge of disorders of mitochondrial translation.

  7. ABR thresholds to tonebursts gated with Blackman and linear windows in adults with high-frequency sensorineural hearing loss.

    Science.gov (United States)

    Purdy, Suzanne C; Abbas, Paul J

    2002-08-01

    The goal of this study was to determine whether tonebursts gated on and off using a nonlinear, exact-Blackman-gating function would be a more frequency-specific stimulus for auditory brain stem response audiometry than the more traditional 2-1-2 cycle linearly gated toneburst. Toneburst ABRs were recorded in 10 adults with normal hearing and in 18 adults with sloping high-frequency sensorineural hearing loss. It was hypothesized that any advantage of the Blackman stimuli for frequency-specific threshold assessment should be evident in hearing-impaired subjects with hearing loss confined to the 2000 to 4000 Hz frequency region since spectral splatter in the toneburst stimuli could lead to an underestimation of hearing loss based on the ABR thresholds. ABR stimuli consisted of 2000- and 4000-Hz 2-1-2 (rise-plateau-fall) cycle linearly gated tonebursts and 1-0-1 msec exact-Blackman-gated tonebursts. An additional 0.5-0-0.5 msec 4000-Hz Blackman-gated toneburst was used to investigate whether the difference in rise/fall characteristics of the linearly and Blackman-gated tonebursts could account for any differences in ABR results at 4000 Hz. The ABR toneburst stimuli were calibrated behaviorally in 15 adults with normal hearing. In the normal-hearing listeners toneburst-ABR thresholds generally exceeded behavioral thresholds by 10 to 13 dB for all stimuli. Correlations of 0.85 to 0.96 were obtained between 2000 and 4000 Hz toneburst ABR thresholds and pure-tone audiometric thresholds in the hearing-impaired listeners. Results were similar for Blackman- and linearly gated stimuli. There were no clear differences between Blackman- and linearly gated tonebursts in terms of how well ABR thresholds predicted pure-tone thresholds at 2000 and 4000 Hz. In general audiometric thresholds were predicted with good accuracy (+/-15 dB) by the toneburst ABR thresholds. The 4000-Hz audiometric threshold was underestimated in one subject with a very steeply sloping hearing loss by both

  8. Speech recognition ability of children with unilateral sensorineural hearing loss as a function of amplification, speech stimuli and listening condition.

    Science.gov (United States)

    Kenworthy, O T; Klee, T; Tharpe, A M

    1990-08-01

    The purpose of this investigation was to examine three types of audiological recommendations [unaided, CROS (contralateral routing of signals) and personal FM system] and their impact upon speech recognition ability of children with unilateral sensorineural hearing loss. Each of these recommendations was tested under three listening conditions encountered in a classroom [monaural direct (MD), monaural indirect (MI), midline signal/omnidirectional noise (MS/ON)] with two types of speech materials (Nonsense Syllable Test and an American English adaptation on the Bamford-Kowal-Bench Sentence Lists). These experimental conditions were simulated in a classroom, recorded on audiotape, and played back to subjects under headphones to control such factors as signal-to-noise ratio, earmold fit, and head shadow effects. Six school-age children with unilateral hearing losses between 56 and greater than 120 dB HL (PTA) were evaluated using a repeated measures design. The children experienced the most listening difficulty in the MI condition when they were unaided. The CROS aid improved speech recognition in this condition but degraded speech recognition in the MD condition. The FM system was the only audiological recommendation to produce uniformly high speech recognition scores across all listening conditions with both types of speech materials. Implications for the audiological management of unilaterally hearing-impaired children in the classroom are discussed.

  9. The effect of side of implantation on unilateral cochlear implant performance in patients with prelingual and postlingual sensorineural hearing loss: A systematic review.

    Science.gov (United States)

    Kraaijenga, V J C; Derksen, T C; Stegeman, I; Smit, A L

    2017-09-25

    Cerebral lateralisation of language processing leads to a right ear advantage in normal hearing subjects. The aim of this study was to present a systematic overview of the effect of implantation side on postoperative cochlear implant performance in patients with symmetrical severe to profound sensorineural hearing loss. PubMed, Embase and The Cochrane Library databases. Databases were searched from database inception up to 9 January 2017 for cochlear implant and side and all synonyms. Title, abstract and full-text of retrieved articles were screened for eligibility. Then, directness of evidence and risk of bias were assessed. For the included articles, study characteristics and outcome data (hearing and language development) were extracted. 2541 unique articles were screened, of which twenty were eligible for critical appraisal. No randomised controlled trials were identified. Twelve studies with a high directness of evidence remained for data extraction. Four of six studies including children with pre-lingual sensorineural hearing loss and four of seven studies investigating adults with postlingual sensorineural hearing loss found a right ear advantage in at least one outcome measurement related to cochlear implant performance. The available evidence on the effect of side of implantation is of low quality, as study populations and outcome measures are heterogeneous. The majority of studies reveals evidence for a right ear advantage in prelingually deafened children as well as postlingually deafened adults. In view of the present evidence and as no left ear advantage was identified, we cautiously advise implanting the cochlear implant in the right ear when other prognostic factors do not favour the left ear and sensorineural hearing loss is symmetrical. © 2017 The Authors. Clinical Otolaryngology Published by John Wiley & Sons Ltd.

  10. Impact of Nonaspirin Nonsteroidal Anti-inflammatory Agents and Acetaminophen on Sensorineural Hearing Loss: A Systematic Review

    Science.gov (United States)

    Kyle, Meghann E.; Wang, James C.; Shin, Jennifer J.

    2015-01-01

    Objective To perform a systematic review evaluating the association between sensorineural hearing loss and (1) nonsteroidal anti-inflammatory drugs (NSAIDs) as a class, (2) NSAIDs available over the counter, (3) NSAIDs in short intravenous courses, (4) prescription NSAIDs utilized by patients without systemic inflammatory conditions, (5) prescription NSAIDs in patients with arthritides, and (6) acetaminophen with and without concomitant narcotic usage. Data Sources Computerized searches of PubMed, EMBASE, and the Cochrane Library were updated through May 2014, along with manual searches and inquiries to topic experts. Review Methods The systematic review was performed according to an a priori protocol. Data extraction was performed by 2 independent investigators, and it focused on relevant audiologic measurements, methodological elements related to risk of bias, and potential confounders. Results The 23 criterion-meeting studies included a total of 92,532 participants, with mixed results. Sulindac was the only specific agent to have been studied with formal audiometry in a randomized double-blind placebo-controlled trial in which hearing was the reported primary outcome: Although an effect was seen in the unadjusted analysis (pure tone threshold > 15 dB, 9.3% vs 2.9%; relative risk [RR], 3.2; confidence interval [CI], 1.09-9.55; P = .02), the effect dissipated in the adjusted analysis (P = .09). There was a significant effect on self-reported hearing loss from NSAIDs as a class (RR, 1.21; CI, 1.11-1.33), ibuprofen (RR, 1.13; CI, 1.06-1.19), and acetaminophen (RR, 1.21; CI, 1.11-1.33), but no formal audiometric data confirm or refute this suggested effect. Audiometry has demonstrated profound loss in some instances of acetaminophen-narcotic combination ingestions. Conclusions Data are varied regarding the impact of NSAIDs and acetaminophen on population hearing health. PMID:25560405

  11. 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.

  12. Sensorineural hearing loss and language development following neonatal extracorporeal membrane oxygenation

    NARCIS (Netherlands)

    D. van den Hondel (Desiree); M.J. Madderom (Marlous); A. Goedegebure (Andre); S.J. Gischler (Saskia); P. Mazer (Petra); D. Tibboel (Dick); H. IJsselstijn (Hanneke)

    2012-01-01

    textabstractOBJECTIVE: To determine the prevalence of hearing loss in school-age children who have undergone neonatal extracorporeal membrane oxygenation (ECMO) treatment and to identify any effects of hearing loss on speech- and language development. DESIGN: Prospective longitudinal follow-up

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

    Science.gov (United States)

    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.

  14. Changes of the directional brain networks related with brain plasticity in patients with long-term unilateral sensorineural hearing loss.

    Science.gov (United States)

    Zhang, G-Y; Yang, M; Liu, B; Huang, Z-C; Li, J; Chen, J-Y; Chen, H; Zhang, P-P; Liu, L-J; Wang, J; Teng, G-J

    2016-01-28

    Previous studies often report that early auditory deprivation or congenital deafness contributes to cross-modal reorganization in the auditory-deprived cortex, and this cross-modal reorganization limits clinical benefit from cochlear prosthetics. However, there are inconsistencies among study results on cortical reorganization in those subjects with long-term unilateral sensorineural hearing loss (USNHL). It is also unclear whether there exists a similar cross-modal plasticity of the auditory cortex for acquired monaural deafness and early or congenital deafness. To address this issue, we constructed the directional brain functional networks based on entropy connectivity of resting-state functional MRI and researched changes of the networks. Thirty-four long-term USNHL individuals and seventeen normally hearing individuals participated in the test, and all USNHL patients had acquired deafness. We found that certain brain regions of the sensorimotor and visual networks presented enhanced synchronous output entropy connectivity with the left primary auditory cortex in the left long-term USNHL individuals as compared with normally hearing individuals. Especially, the left USNHL showed more significant changes of entropy connectivity than the right USNHL. No significant plastic changes were observed in the right USNHL. Our results indicate that the left primary auditory cortex (non-auditory-deprived cortex) in patients with left USNHL has been reorganized by visual and sensorimotor modalities through cross-modal plasticity. Furthermore, the cross-modal reorganization also alters the directional brain functional networks. The auditory deprivation from the left or right side generates different influences on the human brain. Copyright © 2015 IBRO. Published by Elsevier Ltd. All rights reserved.

  15. Progressive sensorineural hearing impairment in maternally inherited diabetes mellitus and deafness (MIDD).

    NARCIS (Netherlands)

    Hendrickx, J.J.; Mudde, A.H.; Hart, L.M. 't; Huygen, P.L.M.; Cremers, C.W.R.J.

    2006-01-01

    OBJECTIVE: To study the progression of hearing impairment (HI) and audiological features in patients with the mitochondrial A to G mutation in the tRNA(LEU(RUU)) gene at position 3,243 associated with maternally inherited diabetes and deafness. DESIGN: Retrospective phenotype genotype family study.

  16. Transplantation of mouse-induced pluripotent stem cells into the cochlea for the treatment of sensorineural hearing loss.

    Science.gov (United States)

    Chen, Jing; Guan, Lina; Zhu, Hengtao; Xiong, Shan; Zeng, Liang; Jiang, Hongqun

    2017-11-01

    Mouse-induced pluripotent stem cells (iPSCs) could differentiate into hair cell-like cells and spiral ganglion-like cells after transplantation into mouse cochleae, but it cannot improve the auditory brain response (ABR) thresholds in short term. To evaluate the potential of iPSCs for use as a source of transplants for the treatment of sensorineural hearing loss (SNHL). Establishing SNHL mice model, then injecting the iPSCs or equal volume DMEM basic medium into the cochleae, respectively. Immunofluorescence staining and reverse transcription-polymerase chain reaction (RT-PCR) were used to assess the survival, migration, differentiation of the transplanted iPSCs in cochleae and then recorded the ABR threshold in different time. Hematoxylin-eosin (HE) staining was used to observe the teratoma formation. Four weeks after transplantation, CM-Di1-labeled iPSCs could be found in the modiolus and Rosenthal's canal (RC), and some of them could expressed auditory hair cell markers or spiral ganglion neuron makers in group A, but not found in group B and C. As to the ABR threshold, no significance differences were found between pre- with postoperative in group A or B. In our study, no teratoma was observed in the cochleae.

  17. Pregnancy is not a risk factor for idiopathic sudden sensorineural hearing loss: A nationwide population-based study.

    Science.gov (United States)

    Yen, Ting-Ting; Lin, Ching-Heng; Shiao, Jiun-Yih; Liang, Kai-Li

    2016-01-01

    Sudden sensorineural hearing loss (SSNHL) in pregnancy is rare. It usually occurs in the third trimester. SSNHL in pregnancy does not increase risks during delivery or subsequent stroke. This study aimed to investigate the incidence and to determine the factors associated with SSNHL in pregnancy. Data were retrieved from Taiwan's National Health Insurance Database (NHIRD), covering the years 2000-2009. Patients admitted for SSNHL during pregnancy were enrolled. An age-matched controlled cohort was randomly selected from pregnant women without SSNHL in the NHIRD. The clinical characteristics of both cohorts were collected for further analyses. Thirty-three patients with SSNHL in pregnancy were enrolled. The estimated incidence of SSNHL in pregnancy in Taiwan was 2.71 per 100,000 pregnancies. The incidence of SSNHL in pregnancy was lower than that of the general female population. The incidence of SSNHL in the third trimester was higher compared to the other two. The incidence of SSNHL occurring in the 30-39 years old age group was higher than other groups. Women with better socioeconomic status had a higher incidence of SSNHL. There were no identified systemic diseases before SSNHL. Two patients had pre-eclampsia and one patient had premature delivery. Nevertheless, SSNHL in pregnancy did not increase the risk for stroke.

  18. [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

  19. Dilated cardiomyopathy and sensorineural hearing loss: a heritable syndrome that maps to 6q23-24.

    Science.gov (United States)

    Schönberger, J; Levy, H; Grünig, E; Sangwatanaroj, S; Fatkin, D; MacRae, C; Stäcker, H; Halpin, C; Eavey, R; Philbin, E F; Katus, H; Seidman, J G; Seidman, C E

    2000-04-18

    Dilated cardiomyopathy (DCM) and sensorineural hearing loss (SNHL) are prevalent disorders that occur alone or as components of complex multisystem syndromes. Multiple genetic loci have been identified that, when mutated, cause DCM or SNHL. However, the isolated coinheritance of these phenotypes has not been previously recognized. Clinical evaluations of 2 kindreds demonstrated autosomal-dominant transmission and age-related penetrance of both SNHL and DCM in the absence of other disorders. Moderate-to-severe hearing loss was evident by late adolescence, whereas ventricular dysfunction produced progressive congestive heart failure after the fourth decade. DNA samples from the larger kindred (29 individuals) were used to perform a genome-wide linkage study. Polymorphic loci on chromosome 6q23 to 24 were coinherited with the disease (maximum logarithm of odds score, 4.88 at locus D6S2411). The disease locus must lie within a 2.8 cM interval between loci D6S975 and D6S292, a location that overlaps an SNHL disease locus (DFNA10). However, DFNA10 does not cause cardiomyopathy. The epicardin gene, which encodes a transcription factor expressed in the myocardium and cochlea, was assessed as a candidate gene by nucleotide sequence analysis; no mutations were identified. A syndrome of juvenile-onset SNHL and adult-onset DCM is caused by a mutation at 6q23 to 24 (locus designated CMD1J). Recognition of this cardioauditory disorder allows for the identification of young adults at risk for serious heart disease, thereby enabling early intervention. Definition of the molecular cause of this syndrome may provide new information about important cell physiology common to both the ear and heart.

  20. 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.)

  1. Genome wide analysis in a family with sensorineural hearing loss, autism and mental retardation.

    Science.gov (United States)

    Mosrati, Mohamed Ali; Schrauwen, Isabelle; Kamoun, Hassen; Charfeddine, Ilhem; Fransen, Erik; Ghorbel, Abdelmonem; Van Camp, Guy; Masmoudi, Saber

    2012-12-01

    Hearing loss is a common congenital anomaly with an incidence of 1 in 1000 live births. It has been described together with several other clinical features as fortuitous association or commune genetic syndrome. In this study, we investigated a consanguineous Tunisian family with moderate to profound congenital hearing loss, mental retardation and autistic behaviors. We performed a genome wide microarray analysis study using approximately 300,000 SNPs in a common set of 7 invidious of this family. We identified regions of suggestive linkage with hearing loss on chromosomes 6p12 and 7q34. In addition, we identified a deletion on chromosome 8p in the two autistic individuals. This report presents an illustration of how consanguinity could increase familial clustering of multiple hereditary diseases within the same family. The application of next generation sequencing for this family seems to be a good strategy for further analysis leading to the identification of candidate genes. Copyright © 2012 Elsevier B.V. All rights reserved.

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

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    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

  3. Deep band modulated phrase perception in quiet and noise in individuals with auditory neuropathy spectrum disorder and sensorineural hearing loss

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    Hemanth Narayan Shetty

    2017-01-01

    Full Text Available Context: Deep band modulation (DBM improves speech perception in individuals with learning disability and older adults, who had temporal impairment in them. However, it is unclear on perception of DBM phrases at quiet and noise conditions in individuals with auditory neuropathy spectrum disorder (ANSD and sensorineural hearing loss (SNHL, as these individuals suffer from temporal impairment. Aim: The aim is to study the effect of DBM and noise on phrase perception in individuals with normal hearing, SNHL, and ANSD. Settings and Design: A factorial design was used to study deep-band-modulated phrase perception in quiet and at noise. Materials and Methods: Twenty participants in each group (normal, SNHL, and ANSD were included to assess phrase perception on four lists of each unprocessed (UP and DBM phrases at different signal-to-noise ratios (SNRs (−1, −3, and −5 dB SNR, which were presented at most comfortable level. In addition, a temporal processing was determined by gap detection threshold test. Statistical Analysis: A mixed analysis of variance was used to investigate main and interaction effects of conditions, noise, and groups. Further, a Pearson product moment correlation was used to document relationship between phrase perception and temporal processing among study participants in each experimental condition. Results: In each group, a significant improvement was observed in DBM phrase perception over UP phrase recognition in quiet and noise conditions. Although a significant improvement was observed, the benefit of recognition from DBM over UP is negligible at −5 dB SNR in both SNHL and ANSD groups. In addition, as expected, a significant improvement in phrase perception in each condition was found in normal hearing than SNHL followed by ANSD. Further, in both atypical groups, a strong negative correlation was found between phrase perception and gap detection threshold in each of the experimental condition. Conclusion: This

  4. Effects of sensorineural hearing loss on temporal coding of harmonic and inharmonic tone complexes in the auditory nerve.

    Science.gov (United States)

    Kale, Sushrut; Micheyl, Christophe; Heinz, Michael G

    2013-01-01

    Listeners with sensorineural hearing loss (SNHL) often show poorer thresholds for fundamental-frequency (F0) discrimination and poorer discrimination between harmonic and frequency-shifted (inharmonic) complex tones, than normal-hearing (NH) listeners-especially when these tones contain resolved or partially resolved components. It has been suggested that these perceptual deficits reflect reduced access to temporal-fine-structure (TFS) information and could be due to degraded phase locking in the auditory nerve (AN) with SNHL. In the present study, TFS and temporal-envelope (ENV) cues in single AN-fiber responses to band-pass-filtered harmonic and inharmonic complex tones were -measured in chinchillas with either normal-hearing or noise-induced SNHL. The stimuli were comparable to those used in recent psychophysical studies of F0 and harmonic/inharmonic discrimination. As in those studies, the rank of the center component was manipulated to produce -different resolvability conditions, different phase relationships (cosine and random phase) were tested, and background noise was present. Neural TFS and ENV cues were quantified using cross-correlation coefficients computed using shuffled cross correlograms between neural responses to REF (harmonic) and TEST (F0- or frequency-shifted) stimuli. In animals with SNHL, AN-fiber tuning curves showed elevated thresholds, broadened tuning, best-frequency shifts, and downward shifts in the dominant TFS response component; however, no significant degradation in the ability of AN fibers to encode TFS or ENV cues was found. Consistent with optimal-observer analyses, the results indicate that TFS and ENV cues depended only on the relevant frequency shift in Hz and thus were not degraded because phase locking remained intact. These results suggest that perceptual "TFS-processing" deficits do not simply reflect degraded phase locking at the level of the AN. To the extent that performance in F0- and harmonic/inharmonic discrimination

  5. Hearing loss

    Science.gov (United States)

    Decreased hearing; Deafness; Loss of hearing; Conductive hearing loss; Sensorineural hearing loss; Presbycusis ... Symptoms of hearing loss may include: Certain sounds seeming too loud Difficulty following conversations when two or more people are talking ...

  6. 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.

  7. Benign paroxysmal positional vertigo accompanied by sudden sensorineural hearing loss: a comparative study with idiopathic benign paroxysmal positional vertigo.

    Science.gov (United States)

    Kim, Min-Beom; Ban, Jae Ho

    2012-12-01

    To investigate the clinical characteristics of benign paroxysmal positional vertigo (BPPV) associated with idiopathic sudden sensorineural hearing loss (ISSHL) and to compare them with the characteristics of idiopathic BPPV (i-BPPV). Retrospective case series. We retrospectively analyzed 519 patients with ISSHL and 597 patients with i-BPPV. The ISSHL patients with recent vertigo history before or after admission were tested with video-nystagmography that included the caloric test. BPPV with same-side ISSHL was identified and categorized as secondary BPPV (s-BPPV) using the roll or Dix-Hallpike test. All members of the s-BPPV and i-BPPV groups underwent a daily canalith repositioning procedure (CRP) during the admission periods. We investigated the clinical characteristics, including the number of CRPs performed to achieve successful reposition, canal involvement type, and effect of canal paresis and made comparisons between the s-BPPV and i-BPPV groups. Of the 519 ISSHL patients, 63 (12.1%) were identified as having s-BPPV. Multicanal involvement was more frequent in s-BPPV than i-BPPV patients (P CRPs needed to achieve successful reposition was 4.28 in s-BPPV and 1.34 in i-BPPV (P CRPs required for s-BPPV (P CRPs were required for successful repositioning in patients with s-BPPV than in patients with i-BPPV. Also, the presence of canal paresis in s-BPPV was associated with a greater number of required CRPs. Copyright © 2012 The American Laryngological, Rhinological, and Otological Society, Inc.

  8. 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

  9. Rubella in Sub-Saharan Africa and sensorineural hearing loss: a case control study

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    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

  10. 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 a...

  11. Prevalence of epileptiform discharges in children with sensori-neural hearing loss and behavioral problems compared to their normal hearing peers.

    Science.gov (United States)

    Amirsalari, Susan; Radfar, Shokoufeh; Ajallouyean, Mohammad; Saburi, Amin; Yousefi, Jaleh; Noohi, Sima; Tavallaie, Seyed Abbas; Hassanalifard, Mahdieh; Ghazavi, Yasaman

    2014-01-01

    Overactivity and behavioral problems are common problems in children with prelingually profound sensorineural hearing loss (SNHL). Data on epileptiform electroencephalography (EEG) discharges in deaf children with psychological disorders are so limited. The primary focus of this study was to determine the prevalence of epileptiform discharges (EDs) in children with SNHL and overactivity or behavioral problems. A total of 262 patients with prelingually profound SNHL who were referred to our cochlear implantation center between 2008 and 2010 were enrolled in this study. Children with SNHL who had diagnosis of overactivity and/or behavioral problems by a pediatric psychiatrist, underwent electroencephalography (EEG). EEG analysis was carried out by a board-certified pediatric neurologist. The control group consisted of 45 cases with overactivity or behavioral problems and normal hearing. One hundred thirty-eight children with mean age of 3.5±1.23 year were enrolled in the case group, of whom 88 cases (63.7%) were boy. The control group consisted of 45 cases with mean age of 3.2±1.53 years, of whom 30 (66.6%) cases were male. EDs were detected in 28 (20.02%) children of the case group (with SNHL) in comparison with 4 (8.88%) in the control group (without SNHL), which was statistically significantly different. In this study, we obtained higher frequency of EDs in deaf children with overactivity and/or behavioral problem compared to the children without SNHL. Further studies are required to evaluate the possible association of SNHL with EDs in overactive children.

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

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    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

  13. 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

  14. Predicted effects of sensorineural hearing loss on across-fiber envelope coding in the auditory nerve.

    Science.gov (United States)

    Swaminathan, Jayaganesh; Heinz, Michael G

    2011-06-01

    Cross-channel envelope correlations are hypothesized to influence speech intelligibility, particularly in adverse conditions. Acoustic analyses suggest speech envelope correlations differ for syllabic and phonemic ranges of modulation frequency. The influence of cochlear filtering was examined here by predicting cross-channel envelope correlations in different speech modulation ranges for normal and impaired auditory-nerve (AN) responses. Neural cross-correlation coefficients quantified across-fiber envelope coding in syllabic (0-5 Hz), phonemic (5-64 Hz), and periodicity (64-300 Hz) modulation ranges. Spike trains were generated from a physiologically based AN model. Correlations were also computed using the model with selective hair-cell damage. Neural predictions revealed that envelope cross-correlation decreased with increased characteristic-frequency separation for all modulation ranges (with greater syllabic-envelope correlation than phonemic or periodicity). Syllabic envelope was highly correlated across many spectral channels, whereas phonemic and periodicity envelopes were correlated mainly between adjacent channels. Outer-hair-cell impairment increased the degree of cross-channel correlation for phonemic and periodicity ranges for speech in quiet and in noise, thereby reducing the number of independent neural information channels for envelope coding. In contrast, outer-hair-cell impairment was predicted to decrease cross-channel correlation for syllabic envelopes in noise, which may partially account for the reduced ability of hearing-impaired listeners to segregate speech in complex backgrounds. © 2011 Acoustical Society of America

  15. Predicted effects of sensorineural hearing loss on across-fiber envelope coding in the auditory nervea

    Science.gov (United States)

    Swaminathan, Jayaganesh; Heinz, Michael G.

    2011-01-01

    Cross-channel envelope correlations are hypothesized to influence speech intelligibility, particularly in adverse conditions. Acoustic analyses suggest speech envelope correlations differ for syllabic and phonemic ranges of modulation frequency. The influence of cochlear filtering was examined here by predicting cross-channel envelope correlations in different speech modulation ranges for normal and impaired auditory-nerve (AN) responses. Neural cross-correlation coefficients quantified across-fiber envelope coding in syllabic (0–5 Hz), phonemic (5–64 Hz), and periodicity (64–300 Hz) modulation ranges. Spike trains were generated from a physiologically based AN model. Correlations were also computed using the model with selective hair-cell damage. Neural predictions revealed that envelope cross-correlation decreased with increased characteristic-frequency separation for all modulation ranges (with greater syllabic-envelope correlation than phonemic or periodicity). Syllabic envelope was highly correlated across many spectral channels, whereas phonemic and periodicity envelopes were correlated mainly between adjacent channels. Outer-hair-cell impairment increased the degree of cross-channel correlation for phonemic and periodicity ranges for speech in quiet and in noise, thereby reducing the number of independent neural information channels for envelope coding. In contrast, outer-hair-cell impairment was predicted to decrease cross-channel correlation for syllabic envelopes in noise, which may partially account for the reduced ability of hearing-impaired listeners to segregate speech in complex backgrounds. PMID:21682421

  16. Genetic frequencies related to severe or profound sensorineural hearing loss in Inner Mongolia Autonomous Region

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    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.

  17. Neuroradiological imaging in patients with sensorineural hearing loss prior to cochlear implantation; Neuroradiologische Diagnostik bei Patienten mit sensorineuralem Hoerverlust vor Cochlea-Implantation

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    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.)

  18. Perda auditiva sensorioneural em pacientes com acromegalia em tratamento Sensorineural hearing loss in acromegalic patients under treatment

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    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

  19. Long-Term Progression of Sensorineural Hearing Loss and Tinnitus after Combined Intensity-Modulated Radiation Therapy and Cisplatin-Based Chemotherapy for Nasopharyngeal Carcinoma: A Case Report

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    Ming S. Lee

    2017-08-01

    Full Text Available Sensorineural hearing loss (SNHL is a common adverse effect for nasopharyngeal carcinoma (NPC patients treated with chemoradiotherapy. We report a case of 12-year follow-up from a patient with stage IIB NPC, treated in 2004 with intensity-modulated radiotherapy and cisplatin-based chemotherapy. Pure-tone audiograms were conducted before treatment and at two other points in the 12-year period after treatment. Analysis of the patient’s audiograms reveals that the development of high-frequency SNHL started after treatment and reached a plateau accompanied by tinnitus approximately 32 months after treatment conclusion. After the plateau, high-frequency SNHL continued to develop slowly in the next 10 years, possibly a long-term effect from radiation-induced microvascular change of the hearing apparatus. The continuous high-frequency hearing decline is associated with increased tinnitus pitch in the patient. With experience learned from this case, we recommend hearing tests at regular intervals for at least 3–5 years for NPC patients treated with chemoradiotherapy. Patients need to be educated about tinnitus and counseling can be offered when they begin to feel inconvenienced by tinnitus. These patients also need to be advised against exposure to noise that can aggravate the already compromised hearing apparatus, leading to further hearing loss and worsening tinnitus. Limiting the peak dose and total cumulative dose of cisplatin should be considered based on the patients’ risk factors to achieve a balance between treatment efficacy and long-term adverse effects.

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

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    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 hearing loss with the purpose of studying the cognitive load during

  1. Phonology matters: a comprehensive investigation of reading and spelling skills of school-age children with mild to moderate sensorineural hearing loss.

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    Park, Jungjun; Lombardino, Linda J; Ritter, Michaela

    2013-01-01

    The investigators measured 7 literacy skills in a group of 21 school-age children with mild to moderate sensorineural hearing loss (MSNH group), and compared the scores to those of 2 age-matched groups: children with dyslexia (DYS group) and, as a control, typically developing hearing children (CA group). The MSNH group performed consistently below the CA group but better than the DYS group, an indication that differences in the groups' phonological processing profiles might be an important discriminating feature. Interestingly, the MSNH group showed a selective impairment in word reading accuracy only, whereas their reading rate was relatively unaffected. Children with MSNH who show weak phonological awareness skills seem to compensate by relying on orthographic recognition associated with rapid naming ability. To determine which children with MSNH are at high risk for depressed reading achievement, testing across a widerange of literacy skills should be considered.

  2. Previously Unreported Biallelic Mutation in DNAJC19: Are Sensorineural Hearing Loss and Basal Ganglia Lesions Additional Features of Dilated Cardiomyopathy and Ataxia (DCMA) Syndrome?

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    Ucar, Sema Kalkan; Mayr, Johannes A; Feichtinger, René G; Canda, Ebru; Çoker, Mahmut; Wortmann, Saskia B

    2017-01-01

    Dilated cardiomyopathy (DCM), non-progressive cerebellar ataxia (A), testicular dysgenesis, growth failure, and 3-methylglutaconic aciduria are the hallmarks of DNAJC19 defect (or DCMA syndrome) due to biallelic mutations in DNAJC19. To date DCMA syndrome has been reported in 19 patients from Canada and in two Finnish siblings. The underlying pathomechanism is unknown; however, DNAJC19 is presumed to be involved in mitochondrial membrane related processes (e.g., protein import and cardiolipin remodeling). Here, we report an additional patient with progressive cerebellar atrophy and white matter changes. A Turkish boy presented at age 2 months with dilated cardiomyopathy (initially worsening then stabilizing in the second year of life), growth failure, bilateral cryptorchidism, and facial dysmorphism. Mental and motor developmental were, respectively, moderately and severely delayed. Profound intentional tremor and dyskinesia, spasticity (particularly at the lower extremities), and dystonia were observed. Sensorineural hearing loss was also diagnosed. MRI showed bilateral basal ganglia signal alterations. Plasma lactate levels were increased, as was urinary excretion of 3-methylglutaconic acid. He deceased aged 3 years. Sanger Sequencing of DNAJC19 confirmed the clinical diagnosis of DNAJC19 defect by revealing the previously unreported homozygous stop mutation c.63delC (p.Tyr21*). Investigation of enzymes of mitochondrial energy metabolism revealed decreased activity of cytochrome c oxidase in muscle tissue. Sensorineural hearing loss and bilateral basal ganglia lesions are common symptoms of mitochondrial disorders. This is the first report of an association with DNAJC19 defect.

  3. Simultaneous Labyrinthectomy and Cochlear Implantation for Patients with Single-Sided Ménière’s Disease and Profound Sensorineural Hearing Loss

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    G. Doobe

    2015-01-01

    Full Text Available Objective. To investigate the treatment outcome of a simultaneous labyrinthectomy and cochlear implantation in patients with single-sided Ménière’s disease and profound sensorineural hearing loss. Study Design. Prospective study. Method. Five patients with single-sided Ménière’s disease with active vertigo and functional deafness were included. In all cases, simultaneous cochlear implantation combined with labyrinthectomy surgery was performed. The outcome has been evaluated by the Dizziness Handicap Inventory (DHI and speech recognition. Results. The combined labyrinthectomy and cochlear implantation led in all patients to a highly significant reduction of dizziness up to a restitutio ad integrum. After activation of the cochlear implant and rehabilitation, a mean monosyllabic speech understanding of 69% at 65 dB was observed. Conclusion. For patients with single-sided Ménière’s disease and profound sensorineural hearing loss the simultaneous labyrinthectomy and cochlear implantation are efficient method for the treatment of vertigo as well as the rehabilitation of the auditory system.

  4. Clinical Application of the P1 Cortical Auditory Evoked Potential Biomarker in Children with Sensorineural Hearing Loss and Auditory Neuropathy Spectrum Disorder.

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    Campbell, Julia Dee; Cardon, Garrett; Sharma, Anu

    2011-05-01

    The P1 component of the cortical auditory evoked potential (CAEP) shows clearly documented age-related decreases in latency and changes in morphology in normal hearing children, providing a biomarker for development of the auditory cortical pathways in humans. In hearing-impaired children, auditory deprivation may affect the normal age-related changes in central auditory maturation. Appropriate early intervention with amplification and/or electrical stimulation can provide the necessary stimulation needed to drive progress in central auditory maturation and auditory skill development, however objective measures are needed to evaluate the effectiveness of these treatments in infants and young children. We describe three pediatric cases, where we explored the clinical utility of the P1 as an objective biomarker of auditory cortical development after early intervention. We assessed development of P1 CAEP latency and morphology in two children with sensorineural hearing loss (SNHL) who received intervention with hearing aids (case 1) and cochlear implants (case 2) and a child with Auditory Neuropathy Spectrum Disorder (ANSD) (case 3). Overall, we find that the P1 CAEP serves as useful tool for assessing the effectiveness of early intervention treatment and clinical management of pediatric hearing- impaired patients.

  5. 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.

  6. Neutrophil-to-Lymphocyte Ratio and Platelet-to-Lymphocyte Ratio as Novel Markers for Diagnosis of Sudden Sensorineural Hearing Loss: A Systematic Review and Meta-analysis

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    Mohammad Amin Bahrami

    2016-04-01

    Full Text Available Background: Sudden sensorineural hearing loss (SSNHL is a common otologic disease characterized by a loss of hearing greater than 30 dB in three consecutive frequen­cies which occurs in less than 72 hours. Objective: To investigate the role of neutrophil-to-lymphocyte ratio (NLR and platelet-to-lymphocyte ratio (PLR on sudden sensorineural hearing loss (SSNHL. Search strategy: A PubMed, Science Direct, Scopus, OVID, EMBASE and Google Scholar search (date last searched April 2016 without any time, language and location restriction was done. Inclusion criteria: All case-control studies, which have been investigated the relationship of NLR and PLR with the occurrence of SSNHL were included in our meta-analysis. Results: A total of 5 case-control studies were included in the study. All 5 studies have been reported NLR of patients and control groups (611 patients and 804 controls. Our analysis showed that the mean NLR of patients is 1.12 (0.82-1.43 unit higher than that of controls with 95% CI which is statistically significant. Also, 3 studies have been reported PLR of patients and control groups (512 patients and 705 controls. Our analysis showed that the mean PLR of patients is 0.57 (0.08-1.05 unit higher than that of controls with 95% CI which is statistically significant too. Conclusions: This meta-analysis confirmed the relationship of NLR and PLR with SSNHL. Therefore, these parameters can be considered as new markers in diagnosis of SSNHL. 

  7. Lyme disease: sudden hearing loss as the sole presentation.

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    Espiney Amaro, C; Montalvão, P; Huins, C; Saraiva, J

    2015-02-01

    Lyme disease is an uncommon tick-borne multisystemic infection caused by Borrelia burgdorferi. The most common clinical manifestation is erythema migrans. In this report, a very unusual presentation of this condition is described, in which sudden onset sensorineural hearing loss was the sole presenting symptom. Case report and review of English-language literature. A patient presented with sensorineural hearing loss, with no other symptoms or signs. Acute Lyme infection was detected by laboratory tests. Magnetic resonance imaging showed signs of labyrinthitis of the same inner ear. After hyperbaric oxygen and systemic antibiotic treatment, the patient showed total hearing recovery, and magnetic resonance imaging showed complete resolution of the labyrinthitis. To our knowledge, this is the first reported case of Lyme disease presenting only with sensorineural hearing loss. Borreliosis should be considered as an aetiological factor in sensorineural hearing loss. Adequate treatment may provide total recovery and prevent more severe forms of Lyme disease.

  8. 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

  9. 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.

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    Nash-Kille, Amy; Sharma, Anu

    2014-07-01

    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. 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. 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. Cortical phase coherence is significantly reduced as a result of both severe-to-profound SNHL and ANSD. 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. Copyright © 2013 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

  10. Valproate-induced reversible sensorineural hearing loss: a case report with serial audiometry and pharmacokinetic modelling during a valproate rechallenge.

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    Yeap, Li-Ling; Lim, Kheng-Seang; Lo, Yoke-Lin; Bakar, Mohd Zukiflee Abu; Tan, Chong-Tin

    2014-09-01

    Hearing loss has been reported with valproic acid (VPA) use. However, this is the first case of VPA-induced hearing loss that was tested and confirmed with a VPA rechallenge, supported by serial audiometry and pharmacokinetic modelling. A 39-year-old truck driver with temporal lobe epilepsy was treated with VPA at 400 mg, twice daily, and developed hearing loss after each dose, but recovered within three hours. Hearing loss fully resolved after VPA discontinuation. Audiometry performed five hours after VPA rechallenge showed significant improvement in hearing thresholds. Pharmacokinetic modelling during the VPA rechallenge showed that hearing loss occurred at a level below the therapeutic range. Brainstem auditory evoked potential at three months after VPA discontinuation showed bilateral conduction defect between the cochlear and superior olivary nucleus, supporting a pre-existing auditory deficit. VPA may cause temporary hearing threshold shift. Pre-existing auditory defect may be a risk factor for VPA-induced hearing loss. Caution should be taken while prescribing VPA to patients with pre-existing auditory deficit.

  11. [Analysis of the hereditary etiology of 336 patients with non-syndromic sensorineural hearing loss from Ningxia Hui Autonomous Region of China].

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    Wang, Yan-li; Zhu, Yi-ming; Liu, Xiao-wen; Xu, Bai-cheng; Guo, Yu-fen; Wang, Qiu-ju

    2012-09-01

    To investigate the molecular genetic causes and their characteristics of deafness in Ningxia province, we established screening of three common hereditary deafness genes in 336 deaf and hard-of-hearing patients in this district. Peripheral blood samples were obtained from a total of 336 patients with non-syndromic sensorineural hearing loss in parts of special education schools in Ningxia province to extract genomic DNA. The mitochondrial DNA 12S rRNA m.1555A > G mutation was screened by PCR Alw26I digestion and sequence analysis PCR and direct sequencing were used to analyze the coding region of GJB2 and exons 8 and 19 of SLC26A4. Statistical analysis was performed by using SPSS 11.0 software. Frequencies of different GJB2 or SLC26A4 mutations were compared between Han and Hui people. Among these 336 patients, seven cases (2.08%, 7/336) were found to carry mtDNA 12S rRNA m.1555A > G homozygous mutation, 45 cases (13.39%) were caused by GJB2 mutations and 28 cases (8.33%) had two mutated alleles (homozygote and compound heterozygote) of SLC26A4. In detail, 16.67% (56/336) patients carried GJB2 mutations including 11 single mutant carriers. The allele frequency of c.235delC and c.299_300delAT were 9.52% (64/672) and 2.68% (18/672), respectively, making up 81.19% (82/101) of all pathogenic mutated alleles for GJB2. The single mutant allele carriers of SLC26A4 is 32, and two types (c.919-2A > G and c.2168A > G) accounted for 95.29% (24/27) mutations, totally. We also found that statistically significant differences in c.919-2A > G and c.2168A > G frequencies between Han and Hui people (c.919-2A > G, χ(2) = 8.229, P = 0.004; c.2168A > G, χ(2) = 5.277, P = 0.022). However, there was no statistically significant difference in GJB2 mutation between Han and Hui people. GJB2 mutation was a primary cause for non-syndromic sensorineural hearing loss in Ningxia province, and c.235delC was the most common mutant forms of GJB2. c.919-2A > G and c.2168A > G were common mutant

  12. 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.

  13. 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.

  14. Very good performance with bimodal stimulation in a like-hybrid modality in a patient with profound bilateral sensorineural hearing loss with low-frequencies preservation.

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    Laria, Carla; Auletta, Gennaro; Riccardi, Pasquale; Papa, Carmine; Malesci, Rita; Franzé, Annamaria; Marciano, Elio

    2014-01-01

    In this work we describe the experimental protocol set up to obtain very good results in speech performance and in time course, with a subject presenting profound bilateral sensorineural hearing loss with low-frequencies preservation. We used a bimodal stimulation with a like-hybrid modality. Auditory functions have been analyzed by standard tonal and speech audiometry tests, and verbal perception test. The fitting protocol permitted the subject to reach a perception at 65 dB of 100% in a very short time. The subject showed a sufficient recovery of the language spectral information and a good integration of verbal information with high consonantal recognition is present. This case report shows the importance to realize a correct cochlear implant fitting and that, in the case of bimodal stimulation, it is very important to obtain the mutual adjustment of the two hearing aids. Moreover, this study enhances the importance of realizing a preservative surgery to make the most of cochlear implants capacity. © 2013.

  15. Sensorineural deafness

    Science.gov (United States)

    ... your hearing. The following may be helpful: Hearing aids Telephone amplifiers and other assistive devices Sign language (for those with severe hearing loss) Speech reading (such as lip reading and using visual cues to aid communication) A cochlear implant may be recommended for certain ...

  16. 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.

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    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

  17. 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

  18. 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

    2015-05-01

    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 significantly

  19. CAOS-Episodic Cerebellar Ataxia, Areflexia, Optic Atrophy, and Sensorineural Hearing Loss: A Third Allelic Disorder of the ATP1A3 Gene.

    Science.gov (United States)

    Heimer, Gali; Sadaka, Yair; Israelian, Lori; Feiglin, Ariel; Ruggieri, Alessandra; Marshall, Christian R; Scherer, Stephen W; Ganelin-Cohen, Esther; Marek-Yagel, Dina; Tzadok, Michal; Nissenkorn, Andreea; Anikster, Yair; Minassian, Berge A; Zeev, Bruria Ben

    2015-11-01

    We describe the molecular basis of a distinctive syndrome characterized by infantile stress-induced episodic weakness, ataxia, and sensorineural hearing loss, with permanent areflexia and optic nerve pallor. Whole exome sequencing identified a deleterious heterozygous c.2452 G>A, p.(E818K) variant in the ATP1A3 gene and structural analysis predicted its protein-destabilizing effect. This variant has not been reported in context with rapid-onset dystonia parkinsonism and alternating hemiplegia of childhood, the 2 main diseases associated with ATP1A3. The clinical presentation in the family described here differs categorically from these diseases in age of onset, clinical course, cerebellar over extrapyramidal movement disorder predominance, and peripheral nervous system involvement. While this paper was in review, a highly resembling phenotype was reported in additional patients carrying the same c.2452 G>A variant. Our findings substantiate this variant as the cause of a unique inherited autosomal dominant neurologic syndrome that constitutes a third allelic disease of the ATP1A3 gene. © The Author(s) 2015.

  20. Visualization of inner ear dysplasias in patients with sensorineural hearing loss. High-resolution MR imaging and volume-rendered reconstructions

    Energy Technology Data Exchange (ETDEWEB)

    Klingebiel, R.; Bockmuehl, U. [Charite CM, Humboldt Univ., Berlin (Germany). Dept. of Radiology; Werbs, M. [Charite CM, Humboldt Univ., Berlin (Germany). ENT Dept.; Freigang, B. [O. von Guericke Univ., Magdeburg (Germany). ENT Dept.; Vorwerk, W. [St. Salvator Krankenhaus, Halberstadt (Germany). ENT Dept.; Thieme, N.; Lehmann, R. [Charite CM, Humboldt Univ., Berlin (Germany). Dept. of Radiology

    2001-11-01

    Purpose: We evaluated a data acquisition and post-processing protocol for inner ear (IE) assessment by MR imaging in patients, suffering from various labyrinth malformations. Material and Methods: MR IE studies of 158 consecutive patients (316 IEs) suffering from sensorineural hearing loss without evidence of an acoustic neurinoma were reviewed for pathologies of the IE and internal acoustic meatus. High-resolution MR data of all abnormal IE studies (n=45) were post-processed to previously standardized 3D volume rendered (VR) reconstructions. Results: In 9 patients (5.7%) the following IE dysplasias were detected: malformation of the cochlea (6 IEs), vestibulum (4 IEs), semicircular canals (12 IEs) and vestibular aqueduct/endolymphatic sac (10 IEs). One patient showed evidence of an aplasia of the vestibulocochlear nerve. In 4 patients multiple IE dysplasias were encountered. Comprehensive 3D visualization of all labyrinthine dysplasias was achieved by the use of two VR reconstructions. The overall time for bilateral IE assessment amounted to 30-35 min. Conclusion: The imaging protocol allows for rapid and comprehensive visualization of various IE dysplasias, based on a limited number of VR reconstructions.

  1. Changes in tonal audiometry in children with progressive sensorineural hearing loss and history of Neonatal Intensive Care Unit discharge. A 20 year long-term follow-up.

    Science.gov (United States)

    Martínez-Cruz, Carlos F; Poblano, Adrián; García-Alonso Themann, Patricia

    2017-10-01

    Newborns from Neonatal intensive care units (NICU) are at high-risk for sensorineural hearing loss (SNHL) a follow-up is needed for early diagnosis and intervention. Our objective here was to describe the features and changes of SNHL at different periods during a follow-up of almost 20 years. Risk factors for SNHL during development were analyzed. The audiological examination included: Brainstem auditory evoked potentials (BAEP), and Transient evoked otoacoustic emissions (TEOAE). At birth; tonal audiometry (between 125 and 8000 Hz), and tympanometry were performed at 5, 10, 15, and 20 years of age. Sixty-five percent of cases presented bilateral absence of BAEP. At 5 years of age, the most frequent SNHL level was severe (42.5%), followed by moderate (22.5%), and profound level (20%), in all cases, the SNHL was symmetrical with a predominance of lesion for the high frequencies. Exchange transfusion was associated with a higher degree of SNHL (OR = 6.00, CI = 1.11-32.28, p < 0.02). In 55%, SNHL remained stable, but in 40% of the cases it was progressive. At the end of the study six cases with moderate loss progressed to the severe level and seven cases with severe level progressed to profound. Forty percent of infants with SNHL discharged from NICU may present a progression in the hearing loss. Exchange transfusion was associated with a higher degree of SNHL. NICU graduates with SNHL merit a long-term audiological follow-up throughout their lifespan. Copyright © 2017 Elsevier B.V. All rights reserved.

  2. Comparação entre critérios de recuperação auditiva na perda neurossensorial súbita Comparison of hearing recovery criteria in sudden sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    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

  3. 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.

  4. Binaural integration of periodically alternating speech following cochlear implantation in subjects with profound sensorineural unilateral hearing loss.

    Science.gov (United States)

    Wesarg, Thomas; Richter, Nicole; Hessel, Horst; Günther, Stefanie; Arndt, Susan; Aschendorff, Antje; Laszig, Roland; Hassepass, Frederike

    2015-01-01

    In cochlear implant (CI) recipients with unilateral hearing loss (UHL) and normal hearing (NH) in the contralateral ear, the central auditory system receives signals of different auditory modalities, i.e. electrically via the CI ear as well as acoustically via the NH ear. The present study investigates binaural integration of bimodal stimulation in the central auditory system of 10 CI subjects with UHL by applying a modified version of the Rapidly Alternating Speech Perception (RASP) test to characterise speech recognition ability under monotic and dichotic listening arrangements. Subsequently, the results for each monotic and dichotic test condition were compared to quantify the binaural benefit from CI usage. The study results demonstrate significantly improved speech recognition under dichotic compared to monotic listening conditions, providing evidence that there is binaural integration of acoustically and electrically transmitted speech segments in the central nervous system at brainstem and cortical levels. In contrast to more commonly used tests of binaural integration, such as localisation, the RASP test provides the clinical option to investigate binaural integration involving structures at the cortical level. © 2015 S. Karger AG, Basel.

  5. 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.

  6. 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.)

  7. Prevalence of Mutations in Deafness-Causing Genes in Cochlear Implanted Patients with Profound Nonsyndromic Sensorineural Hearing Loss in Shandong Province, China.

    Science.gov (United States)

    Luo, Jianfen; Bai, Xiaohui; Zhang, Fengguo; Xiao, Yun; Gu, Lintao; Han, Yuechen; Fan, Zhaomin; Li, Jianfeng; Xu, Lei; Wang, Haibo

    2017-11-01

    The mutations of GJB2, SLC26A4, and mtDNA12SrRNA are the most common inherited causes of nonsyndromic sensorineural hearing loss (NSHL) in China, yet previous genetic screenings were mainly carried on patients with moderate-to-profound impairment. We aimed to detect the mutation frequencies in NSHL population within a more specified range of severity. Patients with profound NSHL who had undergone cochlear implantation in the Shandong Provincial Hospital (Shandong, China) were recruited. The majority (n  =  472) were between 0.7 and 6 years old, and the remaining (n  =  63) were between 6 and 70 years old. In total, 115 mutation alleles of the three genes were screened with SNP scan assay. Of the patients, 19.44% (104/535) were found to have GJB2 mutations, and the most common allele was c.235delC, followed by c.299_300delAT and c.109G>A. SLC26A4 mutations were detected in 13.46% patients (72/535), and the most common allele was c.919-2A>G (IVS7-2A>G), followed by c.1174A>T and c.2168A>G. Seven patients (1.31%) carried mutations in mtDNA12SrRNA, with the alleles of m.1555A>G and m.1494C>T. We found the allele frequency of c.109G>A (GJB2) was relatively lower in the profound NSHL population in comparison to the moderate-to-profound ones, and the c.1174A>T (SLC26A4) relatively higher. It suggests those mutations may be connected with the degree of deafness, which needs more observations and analyses to support. © 2017 John Wiley & Sons Ltd/University College London.

  8. The significance of a hypoplastic bony canal for the cochlear nerve in patients with sensorineural hearing loss: CT and MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Yoon Jung; Park, Sang Yoo; Kim, Myung Soon; Sung, Ki Jun [College of Medicine, Yonsei Univ., Wonju (Korea, Republic of)

    2004-04-01

    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.

  9. 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

  10. 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.

  11. 38 CFR 17.149 - Sensori-neural aids.

    Science.gov (United States)

    2010-07-01

    ... medical treatment. (c) VA will furnish needed hearing aids to those veterans who have service-connected... 38 Pensions, Bonuses, and Veterans' Relief 1 2010-07-01 2010-07-01 false Sensori-neural aids. 17... Prosthetic, Sensory, and Rehabilitative Aids § 17.149 Sensori-neural aids. (a) Notwithstanding any other...

  12. Prospective mutation screening of three common deafness genes in a large Taiwanese Cohort with idiopathic bilateral sensorineural hearing impairment reveals a difference in the results between families from hospitals and those from rehabilitation facilities.

    Science.gov (United States)

    Wu, Chen-Chi; Chen, Pei-Jer; Chiu, Yu-Hsun; Lu, Ying-Chang; Wu, Ming-Chueh; Hsu, Chuan-Jen

    2008-01-01

    Accurate epidemiological data on common deafness genes are essential to improve the efficiency and to reduce the cost of molecular diagnosis. They may depend on several factors, including a clear delineation of the source of patients being studied. In the present study, we hypothesize that patients with idiopathic sensorineural hearing loss recruited from different sources might reveal discrepancies in the epidemiological results of genetic screening, because patients from different sources might demonstrate distinct clinical or audiologic features and thus result in biased selection of subjects. To elucidate the relative importance of common deafness genes in Taiwanese and to verify our hypothesis, we conducted a prospective project screening mutations in GJB2, SLC26A4 and mitochondrial 12S rRNA gene in a total of 420 Taiwanese families with idiopathic bilateral sensorineural hearing loss, of which 325 families were recruited from hospitals and 95 from hearing rehabilitation facilities. Allele frequencies of common mutations in these three genes and distributions of the corresponding genotypes were then compared between the two groups. The allele frequencies of mutations in SLC26A4, GJB2 and mitochondrial 12S rRNA in the probands of the 420 families were 14.4, 21.7 and 3.8%, respectively. The allele frequency of SLC26A4 mutations in the hospital group was significantly higher than that in the rehabilitation facility group (16.2 vs. 8.4%, chi(2)-test, p < 0.05), whereas no difference in the frequencies of GJB2 mutations and mitochondrial 12S rRNA mutations was found between the two groups. Distributions of probands classified by SLC26A4 genotypes were also different between the two groups (chi(2)-test, p < 0.05). Accordingly, a discrepancy in the genetic screening results might exist between different sources of idiopathic hearing-impaired patients. Further analysis of audiological results and construction of a logistic regression model showed that different

  13. Temporal Response Properties of the Auditory Nerve in Implanted Children with Auditory Neuropathy Spectrum Disorder and Implanted Children with Sensorineural Hearing Loss.

    Science.gov (United States)

    He, Shuman; Abbas, Paul J; Doyle, Danielle V; McFayden, Tyler C; Mulherin, Stephen

    2016-01-01

    This study aimed to (1) characterize temporal response properties of the auditory nerve in implanted children with auditory neuropathy spectrum disorder (ANSD), and (2) compare results recorded in implanted children with ANSD with those measured in implanted children with sensorineural hearing loss (SNHL). Participants included 28 children with ANSD and 29 children with SNHL. All subjects used cochlear nucleus devices in their test ears. Both ears were tested in 6 children with ANSD and 3 children with SNHL. For all other subjects, only one ear was tested. The electrically evoked compound action potential (ECAP) was measured in response to each of the 33 pulses in a pulse train (excluding the second pulse) for one apical, one middle-array, and one basal electrode. The pulse train was presented in a monopolar-coupled stimulation mode at 4 pulse rates: 500, 900, 1800, and 2400 pulses per second. Response metrics included the averaged amplitude, latencies of response components and response width, the alternating depth and the amount of neural adaptation. These dependent variables were quantified based on the last six ECAPs or the six ECAPs occurring within a time window centered around 11 to 12 msec. A generalized linear mixed model was used to compare these dependent variables between the 2 subject groups. The slope of the linear fit of the normalized ECAP amplitudes (re. amplitude of the first ECAP response) over the duration of the pulse train was used to quantify the amount of ECAP increment over time for a subgroup of 9 subjects. Pulse train-evoked ECAPs were measured in all but 8 subjects (5 with ANSD and 3 with SNHL). ECAPs measured in children with ANSD had smaller amplitude, longer averaged P2 latency and greater response width than children with SNHL. However, differences in these two groups were only observed for some electrodes. No differences in averaged N1 latency or in the alternating depth were observed between children with ANSD and children with

  14. [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.

  15. The relationship between vestibular aqueduct diameter and sensorineural hearing loss is linear: a review and meta-analysis of large case series.

    Science.gov (United States)

    Spencer, C R

    2012-11-01

    Inner ear homeostasis is dependent on the vestibular aqueduct and its content, the endolymphatic duct. Narrow and enlarged vestibular aqueducts have both been associated with hearing loss in Ménière's and large vestibular aqueduct syndromes. This review investigated the correlation between vestibular aqueduct diameter and pure tone average, and the effect of measurement site (i.e. the midpoint or the external aperture). A systematic review of the literature and meta-analysis of large case series published on the Allied and Complementary Medicine, British Nursing Index, Cumulative Index to Nursing and Allied Health, Embase, Health Business Elite, Health Management Information Consortium, Medline, PsycInfo and PubMed databases. References and personal books were also scrutinised. A linear relationship between vestibular aqueduct diameter and hearing loss was observed, with a projected increase of 6 dBHL per unit of vestibular aqueduct diameter (95 per cent confidence interval, 2-10; p = 0.003). This relationship was independent of measurement site. This dose-dependent or linear relationship supports the role of flow and/or pressure change as aetiological factors in the pathogenesis of hearing loss, as per Poiseuille's law. This aetiological association is strengthened by the fact that the observed relationship is independent of measurement site.

  16. 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

    2017-11-17

    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 < 0.002). Positive family history of SSNHL seems to be negative prognostic factors in the response to treatment (p < 0.001). The response to treatment was not related to the pattern (p = 0.04) and initial severity of 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.

  17. Sickle Cell Trait, Malaria and Sensorineural Hearing Loss–A Case-Control Study from São Tomé and Príncipe

    OpenAIRE

    Caroça, C; Pereira de Lima, J; Campelo, P; Carolino, E; H. Caria; Paço, J; Nunes-Silva, S.

    2016-01-01

    Background: Hearing loss is a problem with higher incidence in South Asia, Asia Pacific and sub-Saharan Africa. In these countries there is also associated history of anemia and malaria. Objective: This study aims to identify a putative role of Beta globin mutation - sickle cell trait and HL in São Tomé and Príncipe population. Methods: A retrospective case-control study of a convenience sample was collected during Otolaryngologist Humanitarian Missions in São Tomé and Príncipe...

  18. 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.

  19. Validity of hearing impairment calculation methods for prediction of self-reported hearing handicap

    National Research Council Canada - National Science Library

    Andrew B John; Brian M Kreisman; Stephen Pallett

    2012-01-01

    .... The present study evaluated the ability of various arithmetic hearing impairment calculations to predict a self-reported hearing handicap in a sample of presenting with sensorineural hearing loss. 204 adults (127 male, 77 female...

  20. 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 of ...... of febrile neutropenia. Testing for the A1555G mutation is recommended in patients frequently treated with aminoglycosides....

  1. 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

  2. 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.

  3. 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

  4. 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

    2017-11-21

    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.

  5. [Clinical statistics of recurrent acute low-tone senseorineural hearing loss].

    Science.gov (United States)

    Imamura, Shun-ichi; Honda, Hideyuki; Miyata, Masanori; Mizukoshi, Akihito; Masuyama, Keisuke

    2007-07-01

    Recently, acute low-tone senseorineural hearing loss (ALHL) has become common, and its good prognosis is known well. On the other hand, several reports have suggested that ALHL is frequently associated with Meniere's disease. We retrospectively examined the clinical course of 357 cases that were diagnosed and treated as ALHL at our hospitals. Forty-four of these cases that showed high-tone hearing loss in association with age-related changes were classified as atypical cases. The clinical futures of 49 "poor prognosis cases", who experienced recurrent hearing loss and/or profound hearing loss, are reported. Eight of the 49 cases who experienced recurrences had progressive hearing loss upto middle or high tones. Seventeen cases complained of vertiginous sensation, and 8 of these cases experienced recurrent attacks of vertigo and were diagnosed as having Meniere's disease. The former seventeen cases accounted for 34.7% of the "poor prognosis cases", and the latter eight accounted for 16.3% of these cases. Our results suggest that the hearing loss is more frequently associated with Meniere's disease in cases who experience recurrent hearing loss. Thus, cases initially diagnosed as ALHL may include some cases of progressive hearing loss and Meniere's disease. Even in cases in which hearing improvement is obtained, careful clinical observation is necessary, especially in older patients with bilateral affliction and atypical presentation. ALHL has been generally considered to have a good prognosis, however our examination revealed a relatively high frequency of recurrences, progressive hearing loss and complication by vertigo. We recommend, based on this evidence, that careful explanation of this disease is necessary at time of initial informed consent.

  6. [The very severe sensorineural deafness patients caused by rubella virus infection: two cases report].

    Science.gov (United States)

    Ma, Jing; Wan, Lang; Xu, Fen

    2015-09-01

    To explore the audiological features in children who were sever sensorineural hearing loss infected with rubella virus. There were two cases of rubella virus infection in children who were deaf, they conducted the distortion product otoacoustic emission, ABR and auditory steady-state evoked response (ASSR) examination, then analyzed the results comprehensively. Two patients' mothers were prompted to have infected rubella virus during the early three months pregnant period by history and laboratory tests. The two patients were not detected deafness gene mutation. Audiology results implied the two patients were very severe binaural sensorineural deafness, so they were recommended to equipped with hearing aids and cochlear implant surgery. Early pregnancy women infected with rubella virus can cause very severe offspring sensorineural deafness. The crowd whose mother were suspected to infect with rubella virus in early pregnancy, that should be tracked and detected hearing in order to achieve early detection, early intervention and early treatment.

  7. 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 ...

  8. 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.

  9. Hearing in children with fetal alcohol spectrum disorder (FASD).

    Science.gov (United States)

    Cohen-Kerem, Raanan; Bar-Oz, Benjamin; Nulman, Irena; Papaioannou, Vicky A; Koren, Gideon

    2007-01-01

    Alcohol is the most prevalent human teratogen affected by early exposure of the fetus. Although not listed as a major part of the fetal alcohol spectrum disorder (FASD), different texts list hearing loss as a characteristic of the FASD, based on several small studies. To characterize hearing in children with FASD, diagnosed in the Motherisk Program in Toronto. Cross sectional cohort study. Academic referral center. Children 4-16 years of age that met criteria for FASD, with no other known risk factor for sensorineural hearing loss. A consecutive sample of 41 children (13 girls, mean age 8.9 +/-3 years) was collected. Physical examination, audiometry and tympanometry. External and middle ear pathology on physical examination, pure tone average (PTA), speech reception threshold (SRT), discrimination and tympanometry. Results were compared to reference values in the normal population. Hearing loss equal or greater than 16dB hearing-level in each frequency tested was considered to be clinically significant. A total of 5 (11.2%) of children had hearing loss of at least 16dB hearing-level, mostly unilateral. SRT was within the normal range in 40 (98%) of children with FASD and discrimination was normal in all children. None had auricular or external canal dysmorphology. 14.7% of the children had frequent episodes of acute otitis media. Middle ear effusion was detected in 8 ears (9.8%). The prevalence of mild sensorineural hearing loss in children diagnosed with FASD (16dB hearing-level or greater) was not higher than expected in this age group. However, because children with FASD are academically and behaviorally challenged, early detection of hearing loss and early intervention is warranted.

  10. Therapeutic effects of orally administrated antioxidant drugs on acute noise-induced hearing loss.

    Science.gov (United States)

    Choi, C-H; Du, X; Floyd, R A; Kopke, R D

    2014-03-01

    The objective of this study was to investigate the dose-dependent therapeutic effect of the orally administrated antioxidant drugs [4-hydroxy alpha-phenyl-tert-butylnitrone (4-OHPBN) and N-acetyl-L-cysteine (NAC)] on acute noise-induced hearing loss because oral administration is the most commonly used method of drug administration due to its convenience, safety, and economical efficiency. Thirty chinchilla were exposed to a 105 dB octave band noise centered at 4 kHz for 6 h and randomly assigned to a control group (saline only) and three experimental groups [4-OHPBN (10 mg/kg) plus NAC (20 mg/kg), 4-OHPBN (20 mg/kg) plus NAC (50 mg/kg), and 4-OHPBN (50 mg/kg) plus NAC (100 mg/kg)]. The drugs were orally administrated beginning 4 h after noise exposure and then administered twice daily for the next 2 days. Permanent auditory brainstem response threshold shifts, distortion product otoacoustic emission threshold shifts, and the percentage of missing outer hair cell were determined. The oral administration significantly reduced permanent hearing threshold shift, distortion product otoacoustic emission threshold shift, and the percentage of missing outer hair cell in a dose-dependent manner. This result demonstrates that orally administered drugs can treat acute noise-induced hearing loss in a dose-dependent manner. This suggests that oral administration was effective in treating acute noise-induced hearing loss as in intraperitoneal administration.

  11. 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...

  12. 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…

  13. 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

  14. 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.

  15. Motivation to Address Self-Reported Hearing Problems in Adults With Normal Hearing Thresholds.

    Science.gov (United States)

    Alicea, Carly C M; Doherty, Karen A

    2017-12-20

    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. The motivation to change in relation to self-reported hearing problems was measured using the University of Rhode Island Change Assessment (McConnaughy, Prochaska, & Velicer, 1983). The relationship between objective and subjective measures and an adult's motivation was examined. The level of hearing handicap did not differ significantly between adults with normal hearing who reported problems hearing in background noise and adults who had a mild-to-moderate sensorineural hearing loss. Hearing handicap, personal distress, and minimization of hearing loss were factors significantly related to motivation. Age, degree of hearing loss, speech-in-noise scores, working memory, and extended high-frequency average thresholds were not significantly related to their motivation. Adults with normal hearing thresholds but self-reported hearing problems had the same level of hearing handicap and were equally motivated to take action for their hearing problems as age-matched adults with a mild-to-moderate sensorineural hearing loss. Hearing handicap, personal distress, and minimization of hearing loss were most strongly correlated with an individual's motivation to change.

  16. Differences between acoustic trauma and other types of acute noise-induced hearing loss in terms of treatment and hearing prognosis.

    Science.gov (United States)

    Wada, Tetsuro; Sano, Hajime; Nishio, Shin-Ya; Kitoh, Ryosuke; Ikezono, Tetsuo; Iwasaki, Satoshi; Kaga, Kimitaka; Matsubara, Atsushi; Matsunaga, Tatsuo; Murata, Takaaki; Naito, Yasushi; Suzuki, Mikio; Takahashi, Haruo; Tono, Testuya; Yamashita, Hiroshi; Hara, Akira; Usami, Shin-Ichi

    2017-01-01

    To evaluate the differences between acoustic trauma (AT) and other types of acute noise-induced hearing loss (ANIHL), we performed a literature search and case reviews. The literature search based on online databases was completed in September 2016. Articles on ANIHL and steroid treatment for human subjects were reviewed. The source sounds and treatment sequelae of our accumulated cases were also reviewed. Hearing loss caused by gun-shots and explosions was categorized into the AT group, while hearing loss caused by concerts and other noises was categorized into the ANIHL group. Systemic steroid treatment did not appear to be effective, at least in the AT group, based on both the literature and our case reviews. However, effective recovery after treatment including steroids was observed in the ANIHL group. The difference in hearing recovery between the AT and ANIHL groups was statistically significant (p = .030), although differences in age, days from the onset to treatment and pretreatment hearing levels were not significant. Hearing recovery from AT is very poor, whereas, ANIHL is recoverable to some extent. Therefore, it is essential to differentiate between these two groups for accurate prediction of the hearing prognosis and evaluation of treatment effects.

  17. Acute acoustic trauma: how to manage and how to prevent?

    Science.gov (United States)

    Mardassi, Ali; Turki, Senda; Mbarek, Hajer; Hachicha, Amani; Benzarti, Sonia; Abouda, Maher

    2016-11-01

    The consequence of an exposure to intense sounds can be a temporary or permanent hearing loss and even with a rapid therapeutic management, severe sensorineural sequelae may persist. the authors report a retrospective study about 64 patients followed for an acute acoustic trauma during a period of 8 years (2006 to 2013). For all the cases, a clinical examination associated to a pure-tone audiometry was conducted. Hearing levels were measured at the frequencies 500, 1000, 2000 and 3000 Hertz. Auditory evoked potentials were performed in 17 cases. The therapeutic and evolutive data were detailed and discussed. All our patients were male with a mean age of 34 years. The cause of acoustic trauma was a firing of a gun near the ear in 48 cases, an explosion near the ear in 5 cases and a sudden exposure to loud noises near military planes in 11 cases. Clinical complaints were acute hearing loss with tinnitus. Audiometric exams found a sensorineural hearing loss with a hearing level average of 38 decibels (dB) +/- 14 SD. The therapy consisted of systemic cortisteroids associated in all the cases to peripheral vasodilators.  It was given intravenously during 10 days and then orally with vasodilators during one to 3 months. Hyperbaric oxygenotherapy have been administrated for 25 patients. The follow-up consisted of questioning about symptoms, clinical examination and pure-tone audiometry. A good evolution was noted in 52 cases (81%) and the mean of hearing level after therapy was: 24 dB +/- 12 dB. Despite a prolonged therapy with vaso-active drugs, tinnitus persisted in 36 cases. Controlling noise and its harmful effects through technical devices and safety professionals programs are the best way to reduce the frequency and the sensorineural sequelae due to acute acoustic trauma.

  18. [Active electronic hearing implants for middle and inner ear hearing loss--a new era in ear surgery. III: prospects for inner ear hearing loss].

    Science.gov (United States)

    Zenner, H P; Leysieffer, H

    1997-10-01

    The perspectives for active hearing implants lie in the treatment of patients with sensorineural hearing loss (SNHL). The majority of patients with SNHL suffer from a cochlea amplifier (CA) failure which is discernible by a positive recruitment and loss of otoacoustic emissions (OAE). Therefore, the electronic implant is expected to partially replace functions of the CA. Thus, the implant is thought to function as a CAI (cochlea amplifier implant). An approved implant for routine use is not yet available. Clinical studies have thus far only used the high energy consuming (HEC), narrow-band, electromagnetic floating-mass transducer, as well as the Maniglia-HEC implant. The high energie consuming, yet broadband Canadian Fredrickson implant is soon to be used in humans. Of the piezoelectrical implants, a German CAI (Tübingen implant) at present consisting of a piezoelectrical transducer and a microphone has thus far been acutely implanted in first patient. It is a low energy consuming (LEC), broad-band implantable system for patients with sensorineural hearing loss. Routine surgical treatment of patients with sensorineural hearing loss with a CAI will only be achieved if complete implants (with transducer, microphones, batteries, and control unit) are made available. They combine distinct acoustic superiority with invisibility (end of stigmatization), an open ear canal, and hopefully, the end of feedback whistling. Among the implants mentioned, the German CAI is the only LEC implant. Its energy requirements are so low that with today's technologie implantable batteries (e.g., in pacemakers), the additional implantation of an energy carrier seems feasible. Since the implantable microphone is already available in the German system, the only essential part missing for a totally implantable CAI is the implantable control unit.

  19. 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.

  20. 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 ...

  1. 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 ...

  2. Enlarged Vestibular Aqueduct Syndrome: Sudden Hearing Loss in a Child with a Cerebral Shunt.

    Science.gov (United States)

    Wendt, Wendi-Jo; Hashikawa, Andrew

    2017-07-04

    Enlarged vestibular aqueduct syndrome (EVAS) is the most common congenital ear anomaly that causes sensorineural hearing loss in children and may predispose a child to sudden hearing loss from sudden pressure changes or minor head trauma. We report a case of a 4-year-old boy with a history of a ventriculoperitoneal shunt and migraines who presented to the emergency department with parental and child care provider reports of acute hearing loss, without a history of trauma, infection, or hardware malfunction, who was diagnosed with bilateral EVAS. Diagnosis of EVAS occurs with specific temporal bone imaging with either high-resolution, thin-cut computed tomography or magnetic resonance imaging scans. Enlarged vestibular aqueduct syndrome is typically refractory to medical treatment and often results in hearing loss that is too severe to benefit from amplification, requiring cochlear implantation.

  3. 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

  4. Hearing screenings on children under three years at risk of hearing impairment.

    Directory of Open Access Journals (Sweden)

    Marcelino Lizano Rabelo

    2011-09-01

    Full Text Available This article presents the results of newborn hearing screenings on infants under three years at risk of hearing impairment at Paediatric Hospital of Sancti Spíritus¸on from 2008 to 2010. All children at risk of hearing impairment were tested by an auditory brainstem response (ABR for the positive diagnosis of hearing impairment. Results: Over the period, 398 infants were screened, among whom 36 (8,98% were diagnosed as deaf or hard of hearing. twenty seven children (6,8% had a sensorineural hearing impairment, The most important risk factors for sensorineural hearing loss were: severe birth asphyxia; mechanic ventilation, and antibiotics.Conclusion: Our hearing screening on infants at risk allowed 36 children diagnosed as hearing impairment, all of then received early management.

  5. First report of prevalence c.IVS1+1G>A and del (GJB6-13S1854) mutations in Syrian families with non-syndromic sensorineural hearing loss.

    Science.gov (United States)

    Al-Achkar, Walid; Al-Halabi, Bassel; Ali, Bashar; Moassass, Faten

    2017-01-01

    Mutations in GJB2 and GJB6 genes are a frequent cause of congenital non-syndromic hearing loss (NSHL). Mutational screening has usually focused on coding region of GJB2 gene. A few studies have been conducted on the non-coding region and exon 1. c.IVS1+1G>A (a splice site mutation in GJB2 gene have been detected as disruptive mutation. Del (GJB6 D13S1830) is found in many populations, but del (GJB6 D13S1854) is reported from a few restricted countries. This study was carried out to investigate the prevalence of splice site mutation c.IVS1+1G>A and two common deletions in GJB6 gene as the genetic etiology of hearing impairment in 70 Syrian families. The frequency of the c.IVS1+1G>A mutation and two deletions were determined by PCR-RFLP and A multiplex PCR assay. Our results showed a high prevalence of IVS1+1G>A mutation (20%) and del(GJB6-D13S1854) (15.7%) in deaf families. The homozygous genotype (c.IVS1+1G>A/c.IVS1+1G>A) was observed in one family and the compound heterozygous genotypes (c.35delG/c.IVS1+1G>A) and (c.IVS1+1G>A/V153I) were observed in 7 families and one family respectively. Also, the heterozygous state (c.IVS1+1G>A/unknown) was detected in 5 families. The study of del((GJB6-D13S1854) was showed a compound heterozygous genotype del((GJB6-D13S1854)/c.IVS1+1G>A) in the same families (5 families) having heterozygous genotype of c.IVS1+1G>A mutation. Also, del(GJB6-D13S1854) is combined with c.35delG mutation in 2 families and it was observed in the heterozygous state del(GJB6-D13S1854)/unknown) in 4 families. In contrast, the del(GJB6-D13S1830) described in many population was absent in our patients. Our findings indicate to significant contribution of the splice site mutation and del(GJB6-D13S1854) in our deaf families and these mutations were important causes of hearing impairment. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  6. 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.

  7. 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

  8. Is Hearing Impairment Associated with Rheumatoid Arthritis?

    DEFF Research Database (Denmark)

    Emamifar, Amir; Bjoerndal, Kristine; Jensen Hansen, Inger Marie

    2016-01-01

    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...... 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...

  9. 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 ...

  10. Objective Estimation of Frequency-Specific Pure-Tone Hearing Thresholds following Bone-Conduction Hearing Aid Stimulation

    OpenAIRE

    Rahne, Torsten; Ehelebe, Thomas

    2014-01-01

    Patients suffering from conductive or mixed hearing loss may benefit from bone-conduction hearing systems (BAHS). The amount of amplification provided by the hearing system is selected based on the individual’s sensorineural frequency-specific threshold. With patients who are not able to provide thresholds behaviorally, such as young children, objective methods are required to estimate the unaided and aided hearing threshold and thus the success of the hearing system fitting. In a prospective...

  11. Sudden hearing loss in children.

    Science.gov (United States)

    Ječmenica, Jovana; Bajec-Opančina, Aleksandra

    2014-08-01

    Sudden sensorineural hearing loss (SSHL) is defined as a 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 very rare in children. Sudden hearing loss is a symptom that suggests that there is a problem in the inner ear, surrounding structures, or the whole organism. The etiology and development of this disorder are still not fully understood. The literature contains numerous models of the pathogenesis of SSHL, with childhood SSHL having certain peculiarities. In practical terms, the multifactorial nature of SSHL is important in the choice of diagnostic methods and treatment methods. It is important to determine the cause and effect relationship between the underlying disease and hearing loss. © The Author(s) 2014.

  12. The role of alternative GJB2 transcription in screening for neonatal sensorineural deafness in Austria.

    Science.gov (United States)

    Parzefall, Thomas; Lucas, Trevor; Koenighofer, Martin; Ramsebner, Reinhard; Frohne, Alexandra; Czeiger, Shelly; Baumgartner, Wolf-Dieter; Schoefer, Christian; Gstoettner, Wolfgang; Frei, Klemens

    2017-04-01

    Alterations within a novel putative Exon 1a within the gap junction beta 2 (GJB2) gene may play a role in the development of genetic hearing impairment in Austria. Mutations in the GJB2 gene are the most common cause of hereditary sensorineural deafness. Genome-wide screening for alternative transcriptional start sites in the human genome has revealed the presence of an additional GJB2 exon (E1a). This study tested the hypothesis of whether alternative GJB2 transcription involving E1a may play a role in the development of congenital sensorineural deafness in Austria. GJB2 E1a and flanking regions were sequenced in randomized normal hearing control subjects and three different patient groups with non-syndromic hearing impairment (NSHI), and bioinformatic analysis was performed. Statistical analysis of disease association was carried out using the Cochran-Armitage test for trend. A single change 2410 bp proximal to the translational start site (c.-2410T > C, rs7994748, NM_004004.5:c.-23 + 792T > C) was found to be significantly associated with the common c.35delG GJB2 mutation (p = .009). c.35delG in combination with c.-2410CC occurred at a 6.9-fold increased frequency compared to the control group. Additionally, one patient with idiopathic congenital hearing loss was found to be homozygous c.-2410CC.

  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. Imaging of postoperative sensorineural complications of stapes surgery: a pictorial essay.

    Science.gov (United States)

    Ayache, Denis; Lejeune, Delphine; Williams, Marc T

    2007-01-01

    Sensorineural hearing loss and/or vertigo are rare but severe complications of stapes surgery for otosclerosis, ranging from 0.2 to 3%. Management of such complications depends on the underlying cause: intravestibular protrusion of the prosthesis, perilymph fistula, labyrinthitis, and reparative granuloma extending into the vestibule. Surgery is mandatory in cases of intravestibular prosthesis or of persistent perilymph fistula. In cases of suppurative labyrinthitis or reparative granuloma extending into the vestibule, prognosis is usually poor, despite aggressive medical therapy or revision surgery. CT scan or magnetic resonance imaging can frequently help to determine the cause of the inner ear complication of stapedectomy. Demonstrative cases are presented to illustrate the prominent place of imaging in managing sensorineural complications of stapes surgery.

  15. Functionality of hearing aids: state-of-the-art and future model-based solutions.

    Science.gov (United States)

    Kollmeier, Birger; Kiessling, Jürgen

    2016-12-13

    A review about technical and perceptual factors in hearing aid technology, research and development is provided, covering current commercial solutions, underlying models of hearing loss for usage in hearing devices and emerging future technical solutions for hearing aid functionalities. A chain of techniques has provided incremental, but steady increases in user benefit, e.g. in the fields of hearing aid amplification, feedback suppression, dynamic compression, noise reduction and situation adaptation. The models describing the perceptual consequences of sensorineural hearing impairment describe the effects on the acoustical level, the neurosensory level and the cognitive level and provide the framework for compensatory (or even substitutional) functions of hearing aids in terms of the attenuation component, the distortion component and the neural component of the hearing loss. A major factor is the requirement of a strong individualisation of hearing aid solutions calling for an appropriate assessment of the different sensorineural components of a hearing loss, especially with respect to bilateral and binaural hearing aid solutions.

  16. LIPID PROFILE IN SUDDEN SENSORINEURAL HEARING LOSS- A PROSPECTIVE STUDY

    National Research Council Canada - National Science Library

    Anil H. T; S. D. Mahamood Pasha

    2017-01-01

    .... Relevant history was obtained. Examination and investigations were conducted. RESULTS Statistical analysis showed that there was significant difference between the means of lipid profile of the patients and the control group...

  17. 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.

  18. Transient evoked otoacoustic emissions testing for screening of sensorineural deafness in puppies.

    Science.gov (United States)

    McBrearty, A; Penderis, J

    2011-01-01

    Transient evoked otoacoustic emissions (TEOAE) are widely used for human neonatal deafness screening, but have not been reported for clinical use in dogs. To investigate the feasibility of TEOAE testing in conscious puppies and the ability of TEOAE testing to correctly identify deaf and hearing ears, as defined by brainstem auditory evoked response (BAER). Forty puppies from 10 litters. Prospective study on puppies presented for hearing assessment as part of a congenital deafness BAER screening program. Hearing status was determined using BAER. TEOAE testing was performed after the BAER assessment and the results of the TEOAE testing were compared with the hearing status for each ear. Parameters were tested for normality using the D'Agostino Pearson test and comparisons between the deaf and hearing ears were made using Mann-Whitney tests. TEOAE testing was readily performed in puppies presented for congenital deafness screening. Using analysis parameters based on those used in human neonatal hearing screening, TEOAE testing correctly identified all deaf ears, as defined by BAER testing, with a sensitivity of 100% (95% CI: 56-100%) for diagnosing deafness and specificity of 78% (95% CI: 66-87%). TEOAE testing is an effective screening modality for identifying congenital sensorineural deafness in dogs. In light of the simpler and less expensive equipment, TEOAE testing has the potential to improve access to hearing screening and through this reduce the prevalence of congenital deafness in the dog. Copyright © 2011 by the American College of Veterinary Internal Medicine.

  19. 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

  20. The place of hyperbaric oxygen therapy and ozone therapy in sudden hearing loss

    National Research Council Canada - National Science Library

    Ergun Taşdöven, Gülin; Derin, Alper Tunga; Yaprak, Neslihan; Özçağlar, Hasan Ümit

    2016-01-01

    ...: This study assessed the efficacy of hyperbaric oxygen therapy or ozone therapy in the treatment of idiopathic sudden sensorineural hearing loss, when either therapy was included with steroid treatment. Methods...

  1. 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

  2. Characteristics of hearing-impairment among patients in Ghana ...

    African Journals Online (AJOL)

    kemrilib

    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.

  3. Munchausen Syndrome by Proxy: Mother Fabricates Infant's Hearing Impairment.

    Science.gov (United States)

    Kahn, Gerri; Goldman, Ellen

    1991-01-01

    Case study reports a case of Munchausen Syndrome by Proxy, a form of child abuse in which the mother presents a child for treatment for a condition she herself has invented or created. This case study describes the ways in which a mother obtained a diagnosis of sensorineural hearing loss as well as amplification for her normally hearing infant.…

  4. 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.

  5. Subjective hearing impairment after subarachnoid haemorrhage : Prevalence and risk factors

    NARCIS (Netherlands)

    Vos, Erik M.; Greebe, Paut; Visser-Meily, J. M Anne; Rinkel, Gabriel J E; Vergouwen, Mervyn D I

    2017-01-01

    Background Sensorineural hearing impairment is a key symptom in patients with superficial siderosis of the central nervous system, a disease caused by chronic or intermittent haemorrhage into the subarachnoid space. We investigated the prevalence and risk factors of subjective hearing impairment

  6. The Use of Drugs to Reduce Hearing Loss Following Acute Acoustic Trauma

    Science.gov (United States)

    2013-10-15

    Henderson, D., Hamernik, R.P., Dosanjh, D.S. and Mills, J. (eds), (1976). Effects of Noise on Hearing. Raven Press, NY. Hamernik, R.P., Henderson, D. and...Salvi, R. (eds), (1982). New Perspectives on Noise Induced Hearing Loss. Raven Press, NY. Salvi, R.J., Henderson, D., Hamernik, R.P. and Colletti, V...D. (1978). Discharge patterns in the cochlear nucleus of the chinchilla following noise induced asymptotic threshold shift. J. Exp. Brain Res. 32

  7. Spectral Tilt Change in Stop Consonant Perception by Listeners with Hearing Impairment

    Science.gov (United States)

    Alexander, Joshua M.; Kluender, Keith R.

    2009-01-01

    Purpose: To evaluate how perceptual importance of spectral tilt is altered when formant information is degraded by sensorineural hearing loss. Method: Eighteen listeners with mild to moderate hearing impairment (HI listeners) and 20-23 listeners with normal hearing (NH listeners) identified synthesized stimuli that varied in second formant…

  8. 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.

  9. Mutations in TUBB4B Cause a Distinctive Sensorineural Disease.

    Science.gov (United States)

    Luscan, Romain; Mechaussier, Sabrina; Paul, Antoine; Tian, Guoling; Gérard, Xavier; Defoort-Dellhemmes, Sabine; Loundon, Natalie; Audo, Isabelle; Bonnin, Sophie; LeGargasson, Jean-François; Dumont, Julien; Goudin, Nicolas; Garfa-Traoré, Meriem; Bras, Marc; Pouliet, Aurore; Bessières, Bettina; Boddaert, Nathalie; Sahel, José-Alain; Lyonnet, Stanislas; Kaplan, Josseline; Cowan, Nicholas J; Rozet, Jean-Michel; Marlin, Sandrine; Perrault, Isabelle

    2017-12-07

    Leber congenital amaurosis (LCA) is a neurodegenerative disease of photoreceptor cells that causes blindness within the first year of life. It occasionally occurs in syndromic metabolic diseases and plurisystemic ciliopathies. Using exome sequencing in a multiplex family and three simplex case subjects with an atypical association of LCA with early-onset hearing loss, we identified two heterozygous mutations affecting Arg391 in β-tubulin 4B isotype-encoding (TUBB4B). Inspection of the atomic structure of the microtubule (MT) protofilament reveals that the β-tubulin Arg391 residue contributes to a binding pocket that interacts with α-tubulin contained in the longitudinally adjacent αβ-heterodimer, consistent with a role in maintaining MT stability. Functional analysis in cultured cells overexpressing FLAG-tagged wild-type or mutant TUBB4B as well as in primary skin-derived fibroblasts showed that the mutant TUBB4B is able to fold, form αβ-heterodimers, and co-assemble into the endogenous MT lattice. However, the dynamics of growing MTs were consistently altered, showing that the mutations have a significant dampening impact on normal MT growth. Our findings provide a link between sensorineural disease and anomalies in MT behavior and describe a syndromic LCA unrelated to ciliary dysfunction. Copyright © 2017 American Society of Human Genetics. All rights reserved.

  10. [Hearing disorders in 3- to 12-year-olds].

    Science.gov (United States)

    Roman, Stéphane; Elziere, Maya; Nicollas, Richard; Triglia, Jean-Michel

    2011-04-01

    The hearing disorders of the child between 3 and 12 years are dominated by the conductive hearing loss. The questioning, looking for risk factors, as well as the otoscopy are essential to detect and diagnose a hearing disorder. Screening tests help to identify the children with risk of hearing loss. Then, the child is sent for diagnosis towards an ENT specialist or a specialized structure. In spite of the frequency of the conductive hearing loss, in case of the slightest doubt, a sensorineural hearing loss must be always looked for by a puretone audiometry examination via the headphones.

  11. Distortion Product Otoacoustic Emissions in acute acoustic trauma.

    Science.gov (United States)

    Oeken, Jens

    1998-01-01

    Acute acoustic traumas are caused by exposure to extremely high noise levels ranging from milliseconds to several hours' duration. In pure tone audiometry they range from the C5 dip to basomediocochlear sensorineural hearing loss. Their pathogenesis is assumed to consist of micromechanical-traumatic and biochemical-metabolic damage to the outer hair cells. In order to establish the changes to the DPOAE (distortion products of otoacoustic emissions), 17 patients were examined after sustaining acute acoustic trauma. The causes included firework explosions, anti-tank rocket launchers, vehicle tyre bursting, rock concerts, hand-gun shots, sub-machine gun fire, hand grenade explosion, exploding car battery. The pure tone audiogram, tympanogram, tinnitus maskability and DPOAE (both DP-gram and growth rate in various frequencies) were determined in all patients. If the event had occurred some time ago, measurements were taken only once; in acute cases measurements were repeated at different times. In nine patients with persistent hearing impairment, clear DPs were found in the unaffected frequencies but were completely absent in the affected frequency range. Four of these patients were unilaterally and two patients were bilaterally affected; three patients had a different (not noise-induced) hearing loss on the opposite side. In eight patients with regressive hearing loss, DPs were by contrast detectable throughout the entire frequency range, their amplitudes only rising slightly as hearing recovered. Of these eight patients, three were unilaterally and five bilaterally affected. DPOAE seem to indicate the likelihood of recovery of hearing threshold after an acute acoustic trauma. In cases with DPs completely absent in the affected frequency range, the prognosis seems to be much worse than in cases with present DPs in the frequency range of hearing.

  12. Review of environmental factors affecting hearing.

    OpenAIRE

    Mills, J H; Going, J A

    1982-01-01

    The major nongenetic causes of sensorineural hearing loss are exposure to noise, aging, ototoxic drugs, viral and bacterial infections, and interactions between these factors. Regarding exposure to continuous noise, the data base from laboratory and field studies indicates that a risk of hearing loss is present when noise levels exceed 75-80 dBA. As noise level, duration and number of exposures increase so does risk. The data base for other forms of noise (intermittent, impact) is not as esta...

  13. 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

  14. Alström Syndrome with Acute Pancreatitis: A Case Report

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    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.

  15. Syndrome of congenital cataracts, sensorineural deafness, Down syndrome-like facial appearance, short stature, and mental retardation: two additional cases.

    Science.gov (United States)

    Keppler-Noreuil, Kim; Welch, Judy; Baker-Lange, Katherine

    2007-11-01

    An apparently new syndrome of congenital cataracts, sensorineural deafness, Down syndrome-like facial appearance, short stature, and mental retardation was described by Gripp et al. 1996. The authors reported on two unrelated patients with congenital cataracts, sensorineural deafness, distinctive facial appearance, mental retardation, postnatal short stature, and skeletal changes. We report on two additional patients with findings most similar to the reported patients by Gripp et al. 1996, including bilateral congenital cataracts, hearing loss, craniofacial abnormalities, short stature, skeletal abnormalities, and developmental delay. Both of the patients reported herein had chromosome microarray analysis, which showed normal results in Patient 2 but abnormal results in Patient 1 and his mother who both had a chromosome 11q25 subtelomere deletion. Patient 1 and his mother's findings are atypical for the common findings reported in Jacobsen syndrome (11q terminal deletion syndrome), and consistent with the patients reported by Gripp et al. 1996. The etiology for these cases has been unknown. The microarray results on Patient 1 suggest that the other patients with findings of developmental delay, short stature, congenital cataracts, sensorineural hearing loss, and similar craniofacial features may have either a microdeletion of chromosome 11q terminal region or haploinsufficiency of a gene localized to this region. Copyright 2007 Wiley-Liss, Inc.

  16. 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.

  17. Delayed diagnosis of a patient with Usher syndrome 1C in a Louisiana Acadian family highlights the necessity of timely genetic testing for the diagnosis and management of congenital hearing loss.

    Science.gov (United States)

    Umrigar, Ayesha; Musso, Amanda; Mercer, Danielle; Hurley, Annette; Glausier, Cassondra; Bakeer, Mona; Marble, Michael; Hicks, Chindo; Tsien, Fern

    2017-01-01

    Advances in sequencing technologies and increased understanding of the contribution of genetics to congenital sensorineural hearing loss have led to vastly improved outcomes for patients and their families. Next-generation sequencing and diagnostic panels have become increasingly reliable and less expensive for clinical use. Despite these developments, the diagnosis of genetic sensorineural hearing loss still presents challenges for healthcare providers. Inherited sensorineural hearing loss has high levels of genetic heterogeneity and variable expressivity. Additionally, syndromic hearing loss (hearing loss and additional clinical abnormalities) should be distinguished from non-syndromic (hearing loss is the only clinical symptom). Although the diagnosis of genetic sensorineural hearing loss can be challenging, the patient's family history and ethnicity may provide critical information, as certain genetic mutations are more common in specific ethnic populations. The early identification of the cause of deafness can benefit patients and their families by estimating recurrence risks for future family planning and offering the proper interventions to improve their quality of life. Collaboration between pediatricians, audiologists, otolaryngologists, geneticists, and other specialists are essential in the diagnosis and management of patients with hearing disorders. An early diagnosis is vital for proper management and care, as some clinical manifestations of syndromic sensorineural hearing loss are not apparent at birth and have a delayed age of onset. We present a case of Usher syndrome (congenital deafness and childhood-onset blindness) illustrating the challenges encountered in the diagnosis and management of children presenting with congenital genetic sensorineural hearing loss, along with helpful resources for clinicians and families.

  18. 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.

  19. 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....

  20. Audiometry and other hearing tests.

    Science.gov (United States)

    Davies, R A

    2016-01-01

    Hearing tests of the peripheral auditory system are well established and the pure-tone audiogram is generally regarded as the screening test of choice in adults. It allows the distinction to be made between conductive, i.e., outer- and middle-ear, and sensorineural, i.e., cochlear, hearing loss, and also to describe the configuration of the hearing thresholds in terms of severity and the frequency affected. Electrophysiologic testing with auditory potentials, e.g., the auditory brainstem response, can identify sites of lesion in the eighth nerve, brainstem, and more centrally. However, it is only in the last two decades that a battery of central auditory tests has been established that can probe the central pathways in more details, i.e., when the pure-tone audiogram may be normal, and yet the patient still has symptoms of hearing dysfunction. © 2016 Elsevier B.V. All rights reserved.

  1. A controlled comparison of auditory steady-state responses and pure-tone audiometry in patients with hearing loss.

    Science.gov (United States)

    Wadhera, Raman; Hernot, Sharad; Gulati, Sat Paul; Kalra, Vijay

    2017-01-01

    We performed a prospective interventional study to evaluate correlations between hearing thresholds determined by pure-tone audiometry (PTA) and auditory steady-state response (ASSR) testing in two types of patients with hearing loss and a control group of persons with normal hearing. The study was conducted on 240 ears-80 ears with conductive hearing loss, 80 ears with sensorineural hearing loss, and 80 normal-hearing ears. We found that mean threshold differences between PTA results and ASSR testing at different frequencies did not exceed 15 dB in any group. Using Pearson correlation coefficient calculations, we determined that the two responses correlated better in patients with sensorineural hearing loss than in those with conductive hearing loss. We conclude that measuring ASSRs can be an excellent complement to other diagnostic methods in determining hearing thresholds.

  2. Thyroid carcinoma, cataract and hearing loss in a patient after irradiation for facial hemangioma

    Energy Technology Data Exchange (ETDEWEB)

    Talmi, Y.; Kalmanovitch, M.; Zohar, Y.

    1988-01-01

    A rare case of a patient irradiated on the right side of the face for a large hemangioma during early childhood is reported. This patient suffers from sensorineural hearing loss in the right ear and a cataract of the right eye, and she underwent surgery for Hurtle cell carcinoma of the thyroid gland. The literature discussing radiation-induced hearing loss is reviewed.

  3. Working Memory, Sleep, and Hearing Problems in Patients with Tinnitus and Hearing Loss Fitted with Hearing Aids.

    Science.gov (United States)

    Zarenoe, Reza; Hällgren, Mathias; Andersson, Gerhard; Ledin, Torbjörn

    2017-02-01

    Tinnitus is a common condition and there is a need to evaluate effects of tinnitus management in relation to moderating factors such as degree of hearing loss. As it is possible that tinnitus influences concentration, and thus is likely to disturb cognitive processing, the role of cognitive functioning also needs to be investigated. To compare a group of patients with sensorineural hearing loss and tinnitus to a control group with only sensorineural hearing loss (and no tinnitus). To investigate working memory, sleep, and hearing problems measured before and after hearing rehabilitation. A prospective study. The sample consisted of 100 patients, 50 with hearing loss and tinnitus, and 50 controls with hearing loss but no tinnitus. All patients were between 40 and 82 yr old and had a pure-tone average (PTA; average of 0.5, 1, 2, and 4 kHz) hearing aids with regard to their working memory capacity, sleep quality, hearing problems, speech recognition, and tinnitus annoyance. Eight patients dropped out of the study. Thus, a total of 92 patients were included for analysis, with 46 in each group. As a consequence of unplanned age and PTA differences between the groups, an age-matched subsample (n = 30 + 30) was selected for further analysis. Tests including the Reading Span, Hearing-in-Noise Test (HINT), Tinnitus Handicap Inventory (THI), Hearing Handicap Inventory for the Elderly (HHIE), and Pittsburgh Sleep Quality Index (PSQI) were administered before and after hearing aid rehabilitation. There were no between-group differences at baseline in the full sample (n = 92), with the exception of the THI (p hearing loss and tinnitus group had significantly higher scores. Pre/post changes were significant for both groups on the Reading Span, and HHIE. However, these improvements were significantly larger for the patients in the hearing loss and tinnitus group on the Reading Span test (p hearing loss also exhibited significantly improved THI scores at follow-up, compared to

  4. 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.

  5. 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.

  6. A role of the microphthalmia-associated transcription factor in congenital sensorineural deafness and eye pigmentation in Dalmatian dogs.

    Science.gov (United States)

    Stritzel, S; Wöhlke, A; Distl, O

    2009-02-01

    Microphthalmia-associated transcription factor (MITF) is involved in white spotting and deafness associated with lack of pigmentation in human and mice. In the present study, we employed MITF-associated markers to evaluate MITF as a candidate for canine congenital sensorineural deafness (CCSD) in Dalmatian dogs. We performed an association study using MITF flanking and intragenic markers for 88 Dalmatian dogs of different hearing and eye pigmentation status. A significant association was identified for MITF-related markers with CCSD and blue iris colour. We conclude that MITF might play a role in CCSD and blue eye colour in Dalmatian dogs.

  7. Long-term follow-up in distal renal tubular acidosis with sensorineural deafness.

    Science.gov (United States)

    Peces, R

    2000-11-01

    A 20-year-old man presented with failure to thrive and bilateral genu valgum. On the basis of growth failure, skeletal deformity, hyperchloremic metabolic acidosis with alkaline urine and hypokalemia, nephrocalcinosis, and hearing loss, a diagnosis of distal renal tubular acidosis (DRTA) with sensorineural deafness was made. The genu valgum was treated by corrective osteotomy. Skeletal deformity was corrected and impaired growth improved after sustained therapy of metabolic acidosis with alkali supplementation. During an 8-year follow-up period the patient's glomerular filtration rate remained stable, the nephrocalcinosis did not progress, and his height increased 10 cm. Although nephrolithiasis led to atrophy of the right kidney, at last follow-up, when the patient was 44 years old, his creatinine clearance was 50 ml/min per 1.73 m2 body surface.

  8. Auditory Memory deficit in Elderly People with Hearing Loss.

    Science.gov (United States)

    Shahidipour, Zahra; Geshani, Ahmad; Jafari, Zahra; Jalaie, Shohreh; Khosravifard, Elham

    2013-06-01

    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. 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. Significant difference was observed in DVMT between elderly people with normal hearing and those with hearing loss (Pauditory verbal memory. This result depicts the importance of auditory intervention to make better communicational skills and therefore auditory memory in this population.

  9. Auditory Memory deficit in Elderly People with Hearing Loss

    Directory of Open Access Journals (Sweden)

    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

  10. 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 ...

  11. 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…

  12. Binaural speech intelligibility in noise for hearing-impaired listeners

    NARCIS (Netherlands)

    Bronkhorst, A.W.; Plomp, R.

    1989-01-01

    The effect of head-induced interaural time delay (ITD) and interaural level differences (ILD) on binaural speech intelligibility in noise was studied for listeners with symmetrical and asymmetrical sensorineural hearing losses. The material, recorded with a KEMAR manikin in an anechoic room,

  13. Speech perception in noise in unilateral hearing loss.

    Science.gov (United States)

    Mondelli, Maria Fernanda Capoani Garcia; Dos Santos, Marina de Marchi; José, Maria Renata

    2016-01-01

    Unilateral hearing loss is characterized by a decrease of hearing in one ear only. In the presence of ambient noise, individuals with unilateral hearing loss are faced with greater difficulties understanding speech than normal listeners. To evaluate the speech perception of individuals with unilateral hearing loss in speech perception with and without competitive noise, before and after the hearing aid fitting process. The study included 30 adults of both genders diagnosed with moderate or severe sensorineural unilateral hearing loss using the Hearing In Noise Test - Hearing In Noise Test-Brazil, in the following scenarios: silence, frontal noise, noise to the right, and noise to the left, before and after the hearing aid fitting process. The study participants had a mean age of 41.9 years and most of them presented right unilateral hearing loss. In all cases evaluated with Hearing In Noise Test, a better performance in speech perception was observed with the use of hearing aids. Using the Hearing In Noise Test-Brazil test evaluation, individuals with unilateral hearing loss demonstrated better performance in speech perception when using hearing aids, both in silence and in situations with a competing noise, with use of hearing aids. Copyright © 2015 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  14. OBJECTIVE HEARING DISORDER DIAGNOSTIC METHODS IN YOUNGER CHILDREN

    Directory of Open Access Journals (Sweden)

    E. E. Savel'eva

    2014-01-01

    Full Text Available Objective: comparative analysis of objective hearing function examination methods in children and identification of the factors affecting examination results. Patients and methods. We studied hearing in 473 children of 3 months – 5 years of age with sensorineural hearing loss and surdity. The control group was comprised of 30 children with normal hearing. Along with the standard clinical examination of ENT-organs, we performed tympanometry and reflexometry, examination of delayed evoked otoacoustic emission and reflection-source frequency otoacoustic emission, registered short-latency auditory evoked potentials and auditory steady state response (ASSR in all children. We also conducted behavioral audiometry in children of 2-3 years of age and play audiometry in older children. Results. Various hearing loss risk factors are revealed in anamneses of most children (77% with sensorineural hearing loss and surdity. The most sensitive (Se = 100% and specific (Sp = 98.3% method of diagnosing hearing level in children is the registration of short-latency brainstem auditory evoked potentials. Conclusions. The most reliable results of hearing thresholds identification are obtained when classic psychoacoustic hearing function examination methods are combined with modern electrophysiological examination method and hearing loss grade verification using surdopedagogic tests.

  15. [neurologic Semiology In A Population Of Hearing Impaired Children].

    OpenAIRE

    V. M. Gonçalves; Piovesana, A M; Moura-Ribeiro, M V

    2015-01-01

    A random sample of 42 sensorineural hearing impaired children (severe and bilateral) was studied, from special classes in Campinas, with chronological ages varying between 4 and 7 years old. The children of this sample were compared with two control groups of 42 children of the same chronological age, from regular classes of private and public schools. All of them were submitted to the traditional neurological examination. Hearing impaired children showed differences as to head circumference ...

  16. Hearing Status of the Blind School Boys

    Directory of Open Access Journals (Sweden)

    Bahram Jalaei

    2003-09-01

    Full Text Available Objective: A survey on hearing state of blind schoolboys in Tehran. Method and Material: This cross-sectional descriptive survey was conducted on 640 ears of 320 blind schoolboys of 5-23 years old as a hearing screening in Tehran blind schools, 2000-01. Results: 20.94% was hearing-impaired of whom 13.75% had bilateral and 7.19% had unilateral hearing loss. 15.16% of students suffered from sensory-neural hearing loss (SNHL, 1.56% from conductive hearing loss (CHL and 0.62% from mixed hearing loss (MHL, that is, SNHL occurred 10 times more than CHL. Hearing loss was often mild and moderate, and audiogram configuration was primarily high tone loss (HTL. On the whole, 8.13% of students needed medical treatment, 15.13% needed audiological rehabilitation and 3.44% of them were in need of both medical and rehabilitation Intervention. General information about hearing problem was very low among students, their parents and teachers, which was 26.87%, 20.9% and 22.39%, respectively. There is significant relation between SNHL and parents' relationship. Conclusion: The prevalence of hearing loss in blinds is higher than in normal-visions. It is often sensorineural. So, it is important to identify these children through hearing screening (Using more high frequencies and to conduct medical as well as rehabilitative intervention.

  17. Myringoplasty - effects on hearing and contributing factors

    African Journals Online (AJOL)

    The most significant factors influencing this are the grade of the surgeon and the size of the perforation. The average pre-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.

  18. Efficacy of 3 commonly used hearing aid circuits: A crossover trial. NIDCD/VA Hearing Aid Clinical Trial Group.

    Science.gov (United States)

    Larson, V D; Williams, D W; Henderson, W G; Luethke, L E; Beck, L B; Noffsinger, D; Wilson, R H; Dobie, R A; Haskell, G B; Bratt, G W; Shanks, J E; Stelmachowicz, P; Studebaker, G A; Boysen, A E; Donahue, A; Canalis, R; Fausti, S A; Rappaport, B Z

    2000-10-11

    Numerous studies have demonstrated that hearing aids provide significant benefit for a wide range of sensorineural hearing loss, but no carefully controlled, multicenter clinical trials comparing hearing aid efficacy have been conducted. To compare the benefits provided to patients with sensorineural hearing loss by 3 commonly used hearing aid circuits. Double-blind, 3-period, 3-treatment crossover trial conducted from May 1996 to February 1998. Eight audiology laboratories at Department of Veterans Affairs medical centers across the United States. A sample of 360 patients with bilateral sensorineural hearing loss (mean age, 67.2 years; 57% male; 78.6% white). Patients were randomly assigned to 1 of 6 sequences of linear peak clipper (PC), compression limiter (CL), and wide dynamic range compressor (WDRC) hearing aid circuits. All patients wore each of the 3 hearing aids, which were installed in identical casements, for 3 months. Results of tests of speech recognition, sound quality, and subjective hearing aid benefit, administered at baseline and after each 3-month intervention with and without a hearing aid. At the end of the experiment, patients ranked the 3 hearing aid circuits. Each circuit markedly improved speech recognition, with greater improvement observed for soft and conversationally loud speech (all 52-dB and 62-dB conditions, Phearing aid circuits more frequently (41.6%) than the WDRC (29.8%) and the PC (28.6%) (P =.001 for CL vs both WDRC and PC). Each circuit provided significant benefit in quiet and noisy listening situations. The CL and WDRC circuits appeared to provide superior benefits compared with the PC, although the differences between them were much less than the differences between the aided vs unaided conditions. JAMA. 2000;284:1806-1813.

  19. [Speech perception with hearing aids in comparison to pure-tone hearing loss].

    Science.gov (United States)

    Hoppe, U; Hast, A; Hocke, T

    2014-06-01

    Speech perception is the most important social task of the auditory system. Consequently, speech audiometry is essential to evaluate hearing aid benefit. The aim of the study was to describe the correlation between pure-tone hearing loss and speech perception. In particular, pure-tone audiogram, speech audiogram, and speech perception with hearing aids were compared. In a retrospective study, 102 hearing aid users with bilateral sensorineural hearing loss were included. Pure-tone loss (PTA) was correlated to monosyllabic perception at 65 dB with hearing aid and with maximum monosyllabic perception with headphones. Speech perception as a function of hearing loss can be represented by a sigmoid function. However, for higher degrees of hearing loss, substantial deviations are observed. Maximum monosyllabic perception with headphones is usually not achieved with hearing aids at standard speech levels of 65 dB. For larger groups, average pure-tone hearing loss and speech perception correlate significantly. However, prognosis for individuals is not possible. In particular for higher degrees of hearing loss substantial deviations could be observed. Speech performance with hearing aids cannot be predicted sufficiently from speech audiograms. Above the age of 80, speech perception is significantly worse.

  20. 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.

  1. Disability certificate for individuals with hearing impairment – Time to rethink

    OpenAIRE

    Hemanth Narayan Shetty; Jijo Pottackal Mathai; Ajith Kumar Uppunda

    2017-01-01

    Background: A retrospective research design was used to investigate the effect of degree of hearing loss on speech identification scores (SISs) and aided improvement in individuals with sensorineural hearing loss (SNHL) and auditory neuropathy spectrum disorder (ANSD). The present criteria (PWD Act, 2001) to issue disability certificate is suitable for individuals with SNHL, as there is a good agreement between their degree of hearing loss and speech understanding. In contrast, individuals wi...

  2. Prevalence of minimal hearing loss in South Korea.

    Science.gov (United States)

    Choi, Ji Eun; Ahn, Jungmin; Park, Hyun Woo; Baek, Sun-Young; Kim, Seonwoo; Moon, Il Joon

    2017-01-01

    This study evaluated the prevalence of minimal hearing loss (MHL) in South Korea based on the 2010 to 2012 Korea National Health and Nutrition Examination Survey. A total of 16,630 representative individuals (older than 12 years) who completed ear examinations and structured questionnaires were analyzed. Only participants who had normal tympanic membranes were included. MHL was categorized into the following three groups: 1) unilateral sensorineural hearing loss (USHL, pure-tone average (PTA) ≥ 15 dB in the affected ear), 2) bilateral sensorineural hearing loss (BSHL, 15 dB ≤ PTA hearing loss (HFSHL, two or more high-frequency thresholds > 25 dB in either ear). To evaluate clinical symptoms, subjective hearing status, tinnitus, and quality of life of each MHL group were compared to those of normal-hearing listeners. The use of hearing aids (HAs) was also investigated in the MHL population. The prevalence of normal hearing and MHL were 58.4% and 37.4%, respectively. In univariate analyses, the prevalence of MHL increased with age. It was significantly increased in males. Regarding clinical symptoms, 13.0% and 92.1% of participants with MHL reported difficulties with hearing and annoying tinnitus, respectively. In multivariate analyses, these proportions were significantly higher in the MHL groups than in normal-hearing listeners. Participants with MHL also showed significantly lower Euro Qol-5D index scores than did normal-hearing listeners. Regarding hearing rehabilitation, among minimally hearing impaired participants with subjective hearing loss, only 0.47% of individuals used HAs. Our results reveal that MHL is common in South Korea. It is associated with significant subjective hearing loss, tinnitus, and poor quality of life. Therefore, clinicians need to pay attention to this special group and provide proper counselling and rehabilitative management.

  3. Prevalence of minimal hearing loss in South Korea

    Science.gov (United States)

    Choi, Ji Eun; Ahn, Jungmin; Park, Hyun Woo; Baek, Sun-Young; Kim, Seonwoo; Moon, Il Joon

    2017-01-01

    This study evaluated the prevalence of minimal hearing loss (MHL) in South Korea based on the 2010 to 2012 Korea National Health and Nutrition Examination Survey. A total of 16,630 representative individuals (older than 12 years) who completed ear examinations and structured questionnaires were analyzed. Only participants who had normal tympanic membranes were included. MHL was categorized into the following three groups: 1) unilateral sensorineural hearing loss (USHL, pure-tone average (PTA) ≥ 15 dB in the affected ear), 2) bilateral sensorineural hearing loss (BSHL, 15 dB ≤ PTA hearing loss (HFSHL, two or more high-frequency thresholds > 25 dB in either ear). To evaluate clinical symptoms, subjective hearing status, tinnitus, and quality of life of each MHL group were compared to those of normal-hearing listeners. The use of hearing aids (HAs) was also investigated in the MHL population. The prevalence of normal hearing and MHL were 58.4% and 37.4%, respectively. In univariate analyses, the prevalence of MHL increased with age. It was significantly increased in males. Regarding clinical symptoms, 13.0% and 92.1% of participants with MHL reported difficulties with hearing and annoying tinnitus, respectively. In multivariate analyses, these proportions were significantly higher in the MHL groups than in normal-hearing listeners. Participants with MHL also showed significantly lower Euro Qol-5D index scores than did normal-hearing listeners. Regarding hearing rehabilitation, among minimally hearing impaired participants with subjective hearing loss, only 0.47% of individuals used HAs. Our results reveal that MHL is common in South Korea. It is associated with significant subjective hearing loss, tinnitus, and poor quality of life. Therefore, clinicians need to pay attention to this special group and provide proper counselling and rehabilitative management. PMID:28196098

  4. [Hearing in old age. Hearing loss is never physiologic].

    Science.gov (United States)

    Leithäuser, D

    1999-06-17

    The common form of hardness of hearing seen in old age is not, in the first instance, due to physiological age-related changes as is, for example, presbyopia, but rather to a complex sensorineural pattern of injury. The underlying causes are not yet fully understood. Typical medical risk factors such as hypertension, diabetes mellitus, etc. apparently play a smaller role than increasing environmental noise and modern life styles. The various forms of presbyacusis and their presentation in the pure tone audiogram are discussed.

  5. 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…

  6. 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.

  7. No Association Between Time of Onset of Hearing Loss (Childhood Versus Adulthood) and Self-Reported Hearing Handicap in Adults

    Science.gov (United States)

    Tambs, Kristian; Engdahl, Bo

    2015-01-01

    Purpose This study examined the association between time of onset of hearing loss (childhood vs. adulthood) and self-reported hearing handicap in adults. Methods 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. Results 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). Conclusion 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. PMID:26649831

  8. 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.

  9. [Basic knowledge on the efficacy of hearing aids depending on the type of hearing impairment for Ear, Nose & Throat specialists].

    Science.gov (United States)

    Steffens, T; Marcrum, S C

    2017-12-13

    For Ear, Nose & Throat specialists, the physiological and psychoacoustical deficits related to hearing impairment and the compensatory capabilities of hearing aids are topics of prime importance. In conductive hearing loss, the foremost deficit is decreased audibility, for which hearing aids can compensate almost entirely through the use of level independent gain. In the instance of sensorineural hearing loss, however, the irreversible loss of outer and inner hair cells causes a distorted sound perception, which is particularly troublesome when trying to understand speech in noisy environments. Unfortunately, this distortion cannot be compensated through the use of hearing aids. Nevertheless, in particular listening environments, its effects can be lessened by reducing background noise levels through the use of directional microphones and, to a lesser extent, digital noise reduction. Noise reduction is in many cases also the main effect to improve speech discrimination in retrocochlear hearing loss.

  10. 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.

  11. 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 hearing loss. The study comprised nine patients (six males, three females) who underwent middle-ear implantation with Carina fully implantable active middle-ear implants to treat bilateral moderate-to-severe mixed hearing loss. The patients initially used conventional hearing aids and subsequently received the Carina implants. The hearing thresholds with implants and hearing aids were compared. There were no significant differences between: the pre-operative and post-operative air and bone conduction thresholds (p > 0.05), the thresholds with hearing aids and Carina implants (p > 0.05), or the pre-operative (mean, 72.8 ± 19 per cent) and post-operative (mean, 69.9 ± 24 per cent) speech discrimination scores (p > 0.05). One of the patients suffered total sensorineural hearing loss three months following implantation despite an initial 38 dB functional gain. All except one patient showed clinical improvements after implantation according to quality of life questionnaire (Glasgow Benefit Inventory) scores. Acceptance of Carina implants is better than with conventional hearing aids in patients with mixed hearing loss, although both yield similar hearing amplification. Cosmetic reasons appear to be critical for patient acceptance.

  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. Double blind comparison of three hearing aid circuits with new hearing aid users.

    Science.gov (United States)

    Hayes, D E; Cormier, K L

    2000-01-01

    This study was a double blind comparison of three types of hearing aid circuits: Class A linear peak clipping, Class D compression limiting and K-Amp wide dynamic range compression. Subjective ratings, speech perception tests, real ear measurements and questionnaire data were obtained from a group of 17 new hearing aid users with mild to moderate sensorineural hearing loss. The results indicate a similar performance for all three circuits. We saw no evidence of performance degradation due to saturation distortion, even in the presence of high levels of speech and noise. Our primary conclusions include recommending K-Amps to new hearing aid users with mild to moderate hearing loss, mostly on the basis of battery life, while cautioning about the use of compression knee-point controls and recognizing that Class A and Class D amplifiers are virtually equivalent in every performance measurement.

  14. Hearing loss in congenital toxoplasmosis detected by newborn screening.

    Science.gov (United States)

    Andrade, Gláucia Manzan Queiroz de; Resende, Luciana Macedo de; Goulart, Eugênio Marcos Andrade; Siqueira, Arminda Lucia; Vitor, Ricardo Wagner de Almeida; Januario, José Nelio

    2008-01-01

    Congenital toxoplasmosis may cause sensorineural deficit in up to 20% of the patients and proper treatment in the first year improves prognosis. In Brazil, this infections impact on hearing impairment is unknown. To evaluate hearing of newborns with congenital toxoplasmosis identified by the newborn screening service. This prospective study analyzed children with congenital toxoplasmosis identified by newborn screening (IgM anti-T.gondii) in Belo Horizonte during 2003/2004. The presence of IgM and/or IgA in the first 6 months or IgG at 12 months-of age in serology was used as case definition. Hearing tests were carried out at the time of diagnosis and 12 months later, including behavioral audiometry, evoked otoacoustic emission and brainstem evoked responses audiometry. Among 30.808 screened children (97% of live births), 20 had congenital toxoplasmosis, 15 (75%) were asymptomatic at birth. Nineteen children were evaluated by hearing tests. Four had sensorineural impairment (21.1%). One child had other risk factors for hearing impairment; the other three had no other risk factors but toxoplasmosis. Two properly children treated still had hearing loss, in disagreement with current literature. Results suggest that congenital toxoplasmosis, common in Brazil, is a risk factor for hearing impairment and its impact on hearing loss deserves further studies.

  15. Hearing loss and cochlear damage in experimental pneumococcal meningitis, with special reference to the role of neutrophil granulytes

    DEFF Research Database (Denmark)

    Brandt, CT; Caye-Thomsen, P; Lund, SP

    2006-01-01

    Hearing loss is a well-known sequelae from meningitis, affecting up to 25% of survivors. However, the principal components of the infectious and inflammatory reaction responsible for the sensorineural hearing loss remain to be identified. The present study aimed to investigate the impact...... of an augmented neutrophil response on the development of hearing loss and cochlear damage in a model of experimental pneumococcal meningitis in rats. Hearing loss and cochlear damage were assessed by distortion product oto-acoustic emissions (DPOAE), auditory brainstem response (ABR) and histopathology in rats...... infection. Pretreatment with G-CSF increased hearing loss 24 h after infection and on day 8 compared to untreated rats (Mann-Whitney, P = 0.012 and P = 0.013 respectively). The increased sensorineural hearing loss at day 8 was associated with significantly decreased spiral ganglion cell counts (P = 0...

  16. HEARING CORRECTION SYSTEM BASED ON MOBILE COMPUTING PLATFORM

    Directory of Open Access Journals (Sweden)

    E. S. Azarov

    2014-01-01

    Full Text Available An implementation of digital hearing aid based on the mobile computing platform iPhone is proposed. The developed signal processing scheme has a low delay, takes into account characteristics of the computing platform and allows to perform the correction of sensorineural hearing loss. The cor-rection is carried out by linear frequency-dependent amplification and wideband dynamic range com-pression of the signal. In order to take into consideration magnitude characteristic of loudspeakers, the audiometry is performed using iPhone directly. The experimental results of with proposed hearing aid are given.

  17. [The age effect in evaluation of hearing aid benefits by speech audiometry].

    Science.gov (United States)

    Müller, A; Hocke, T; Hoppe, U; Mir-Salim, P

    2016-03-01

    Hearing loss is one of the most common disabilities in the elderly. The aim of this study was to investigate the relationship between pure-tone hearing loss and maximum monosyllabic perception and speech perception with hearing aids. The focus of the investigation was elderly patients. In this prospective study, 188 patients with sensorineural hearing loss were included. The pure-tone audiogram (4FPTA), the Freiburg speech intelligibility test with headphones and the word recognition score with hearing aids at 65 dB SPL were measured and evaluated. An increasing age was associated with higher discrepancy between the maximum speech perception and speech understanding with hearing aids. The mean difference between maximum monosyllabic perception and speech perception with hearing aids is about 20% in the elderly population. The intended goal of hearing aid prescription, the match between maximum monosyllabic perception and word recognition score with hearing aids within 5 to 10%, is not achieved in the elderly population.

  18. [The assessment of hearing impairment in patients over 60 years of age using hearing aids].

    Science.gov (United States)

    Skrzypek, Aleksandra; Sekula, Alicja; Deryło, Maria Bratumiła; Kuśmierczyk, Joanna; Talar, Marcin

    2014-01-01

    To assess the hearing impairment in people over 60 years old using hearing aids. This was a single-center study, but it is planned to extend it further to the whole country. The study was focused on patients with hearing aids. During the assessment 57 people were included in the observation in order to control the status of their hearing loss and benefit from traditional hearing aids as well as the possibility to apply the auditory implants in case of a little benefit from hearing aids. The otoscopy and pure tone audiometry were performed as well as the questionnaires on demographic and epidemiological data of patients were collected as well as the quality of their life with hearing aids was subjectively assessed. The results show that 91% of patients have sensorineural hearing loss (SHL), the remaining 9%--severe mixed hearing loss. Severe SHL was found in 22 patients, the moderate hearing loss was observed in 37%, and the profound SHL was the case in 5 patients. Minimal SHL was observed in 7% of patients (n=4). More than 73% of the study subjects were male (n=38). The average age of the patients who completed the survey was 74 years old. Thirty-five patients used their hearing aid over 3 years and less than 70% of them used it every day all day. Hearing aid was not actively used by 10 patients. Over the last year 51.92% of the patients underwent a hearing examination. The bone anchored hearing aid was suggested to 2% of subjects and the cochlear implant was offered to 10 patients. The data analysis shows the need to educate and inform the elderly about alternative methods of hearing loss treatment. Copyright © 2013 Polish Otorhinolaryngology - Head and Neck Surgery Society. Published by Elsevier Urban & Partner Sp. z.o.o. All rights reserved.

  19. The Hearing Outcomes of Cochlear Implantation in Waardenburg Syndrome

    Directory of Open Access Journals (Sweden)

    Hajime Koyama

    2016-01-01

    Full Text Available Objectives. This study aimed to determine the feasibility of cochlear implantation for sensorineural hearing loss in patients with Waardenburg syndrome. Method. A retrospective chart review was performed on patients who underwent cochlear implantation at the University of Tokyo Hospital. Clinical classification, genetic mutation, clinical course, preoperative hearing threshold, high-resolution computed tomography of the temporal bone, and postoperative hearing outcome were assessed. Result. Five children with Waardenburg syndrome underwent cochlear implantation. The average age at implantation was 2 years 11 months (ranging from 1 year 9 months to 6 years 3 months. Four patients had congenital profound hearing loss and one patient had progressive hearing loss. Two patients had an inner ear malformation of cochlear incomplete partition type 2. No surgical complication or difficulty was seen in any patient. All patients showed good hearing outcome postoperatively. Conclusion. Cochlear implantation could be a good treatment option for Waardenburg syndrome.

  20. How Well Can Centenarians Hear?

    Science.gov (United States)

    Mao, Zhongping; Zhao, Lijun; Pu, Lichun; Wang, Mingxiao; Zhang, Qian; He, David Z. Z.

    2013-01-01

    With advancements in modern medicine and significant improvements in life conditions in the past four decades, the elderly population is rapidly expanding. There is a growing number of those aged 100 years and older. While many changes in the human body occur with physiological aging, as many as 35% to 50% of the population aged 65 to 75 years have presbycusis. Presbycusis is a progressive sensorineural hearing loss that occurs as people get older. There are many studies of the prevalence of age-related hearing loss in the United States, Europe, and Asia. However, no audiological assessment of the population aged 100 years and older has been done. Therefore, it is not clear how well centenarians can hear. We measured middle ear impedance, pure-tone behavioral thresholds, and distortion-product otoacoustic emission from 74 centenarians living in the city of Shaoxing, China, to evaluate their middle and inner ear functions. We show that most centenarian listeners had an “As” type tympanogram, suggesting reduced static compliance of the tympanic membrane. Hearing threshold tests using pure-tone audiometry show that all centenarian subjects had varying degrees of hearing loss. More than 90% suffered from moderate to severe (41 to 80 dB) hearing loss below 2,000 Hz, and profound (>81 dB) hearing loss at 4,000 and 8,000 Hz. Otoacoustic emission, which is generated by the active process of cochlear outer hair cells, was undetectable in the majority of listeners. Our study shows the extent and severity of hearing loss in the centenarian population and represents the first audiological assessment of their middle and inner ear functions. PMID:23755251

  1. How well can centenarians hear?

    Directory of Open Access Journals (Sweden)

    Zhongping Mao

    Full Text Available With advancements in modern medicine and significant improvements in life conditions in the past four decades, the elderly population is rapidly expanding. There is a growing number of those aged 100 years and older. While many changes in the human body occur with physiological aging, as many as 35% to 50% of the population aged 65 to 75 years have presbycusis. Presbycusis is a progressive sensorineural hearing loss that occurs as people get older. There are many studies of the prevalence of age-related hearing loss in the United States, Europe, and Asia. However, no audiological assessment of the population aged 100 years and older has been done. Therefore, it is not clear how well centenarians can hear. We measured middle ear impedance, pure-tone behavioral thresholds, and distortion-product otoacoustic emission from 74 centenarians living in the city of Shaoxing, China, to evaluate their middle and inner ear functions. We show that most centenarian listeners had an "As" type tympanogram, suggesting reduced static compliance of the tympanic membrane. Hearing threshold tests using pure-tone audiometry show that all centenarian subjects had varying degrees of hearing loss. More than 90% suffered from moderate to severe (41 to 80 dB hearing loss below 2,000 Hz, and profound (>81 dB hearing loss at 4,000 and 8,000 Hz. Otoacoustic emission, which is generated by the active process of cochlear outer hair cells, was undetectable in the majority of listeners. Our study shows the extent and severity of hearing loss in the centenarian population and represents the first audiological assessment of their middle and inner ear functions.

  2. Use of the ages and stages questionnaire in young children who are deaf/hard of hearing as a screening for additional disabilities.

    Science.gov (United States)

    Wiley, Susan; Meinzen-Derr, Jareen

    2013-05-01

    Children with sensorineural hearing loss have a high rate of additional disabilities beyond those expected by the impact of hearing loss on language and are identified later than children without hearing loss. Identifying these developmental delays and allowing earlier implementation of interventions specific to these concerns would improve care. To determine whether the Ages and Stages Questionnaire (ASQ) can identify unmet needs or unidentified developmental delays among young children with bilateral sensorineural hearing loss. Fifty children with any degree of bilateral sensorineural hearing loss, 6months to 36months of age recruited from a tertiary referral center and regional early intervention programs for children who are deaf/hard of hearing. The ASQ and comprehensive developmental evaluations with the Revised Gesell Developmental Schedules. Sensitivities and specificities of the ASQ were calculated. While 32% of our population had a developmental delay outside the domain of communication, the ASQ had poor sensitivity on the overall score without the communication domain as well as for fine motor, cognitive, personal-social domains. It had good sensitivity for the domains of communication and gross motor skills and good specificity (ranging 83-85%) on specific domains as well as for the overall score (70%). The ASQ does not provide an effective mean for identifying additional developmental concerns in young children with bilateral sensorineural hearing loss. Further studies are needed to determine how to identify additional disabilities in a timely manner among young children with hearing loss. Copyright © 2012 Elsevier Ltd. All rights reserved.

  3. Profound mixed hearing loss: bone-anchored hearing aid system or cochlear implant?

    Science.gov (United States)

    Verhaegen, Veronique J O; Mulder, Jef J S; Mylanus, Emmanuel A M; Cremers, Cor W R J; Snik, Ad F M

    2009-10-01

    We evaluated 5 patients who were changed over from a Baha Cordelle to a cochlear implant (CI). Moreover, the level of phoneme score was determined at which a Baha Cordelle user was better helped with a CI. We offer descriptive case reports and a retrospective evaluation of speech recognition in Baha Cordelle users and CI users. In the CI users with noncompromised cochleas, the 10th percentile of the aided phoneme score in quiet at 65 dB sound pressure level (PS65) was 42%. We consider this PS65 as the cutoff level for switching from a Baha Cordelle to a CI. When patients with mixed hearing loss were using the Baha Cordelle, the PS65 of 42% was obtained at a mean sensorineural hearing loss component of about 70 dB hearing level (HL). This 70-dB HL component was used to consider Baha Cordelle users for cochlear implantation. The results of the 5 patients support these transition criteria. A CI is a valuable option in patients with mixed hearing loss when the sensorineural hearing loss component exceeds 70 dB HL or when the PS65 with a Baha Cordelle is less than about 40%. In such patients, the implantation procedure should be individualized on the basis of the clinical findings in the middle ear and mastoid cavity.

  4. Prevalence of hearing loss in children with 22q11.2 deletion syndrome.

    Science.gov (United States)

    Jiramongkolchai, Pawina; Kumar, Manvinder S; Chinnadurai, Sivakumar; Wootten, Christopher T; Goudy, Steven L

    2016-08-01

    The purpose of this study was to determine the prevalence and characterize the types of hearing loss in pediatric patients with 22q11.2 deletion syndrome (22q11DS). Fifty-eight patients were identified via retrospective chart review performed of patients with 22q11DS between 1996 and 2014. Patient demographics, pertinent family history, associated comorbidities, and degree and type of hearing loss were gathered for each patient. A literature review of the National Library of Medicine's database with a focus on hearing loss and 22q11DS was performed. 22 patients (38%) were found to have hearing impairment: 68% with conductive hearing loss, 14% with sensorineural hearing loss, and 18% with mixed hearing loss. Patients with hearing loss regardless of type had a higher prevalence of developmental delay (55%), cleft palate (23%), articulation disorders (77%), and a greater need for tympanostomy tubes (73%) compared to patients with normal hearing. Temporal bone computed tomography scans of 5 patients revealed a variety of abnormalities in the middle and/or inner ears. Hearing impairment occurs in up to 38% of 22q11DS patients of both conductive and sensorineural types, with the conductive type being the most common. These patients have a greater need for tympanostomy tubes and a higher prevalence of developmental delay and speech articulation disorders. Early hearing screening and treatment is warranted in this population. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  5. Hearing Loss in Children With Craniofacial Microsomia.

    Science.gov (United States)

    Mitchell, Ryan M; Saltzman, Babette S; Norton, Susan J; Harrison, Robert G; Heike, Carrie L; Luquetti, Daniela V; Sie, Kathleen C Y

    2017-11-01

      To evaluate the association between craniofacial phenotype and hearing loss in children with craniofacial microsomia.   Retrospective cohort study.   Tertiary care children's hospital.   Individuals with craniofacial microsomia.   Ear-specific audiograms and standardized phenotypic classification of facial characteristics.   A total of 79 participants were included in the study. The mean age was 9 years (range, 1 to 23 years) and approximately 60% were boys. Facial anomalies were bilateral in 39 participants and unilateral in 40 participants (24 right, 16 left). Microtia (hypoplasia of the ear) was the most common feature (94%), followed by mandibular hypoplasia (76%), soft tissue deficiency (60%), orbital hypoplasia or displacement (53%), and facial nerve palsy (32%). Sixty-five individuals had hearing loss (12 bilateral and 53 unilateral). Hearing loss was conductive in 73% of affected ears, mixed in 10%, sensorineural in 1%, and indeterminate in 16%. Hypoplasia of the ear or mandible was frequently associated with ipsilateral hearing loss, although contralateral hearing loss occurred in 8% of hemifaces.   Hearing loss is strongly associated with malformations of the ipsilateral ear in craniofacial microsomia and is most commonly conductive. Hearing loss can occur contralaterally to the side with malformations in children with apparent hemifacial involvement. Children with craniofacial microsomia should receive early diagnostic hearing assessments.

  6. Experiences of adult patients hearing loss postlingually with Cochlear Implant

    Directory of Open Access Journals (Sweden)

    Teresa María Lizcano Tejado

    2013-09-01

    Full Text Available Hearing loss is a significant public health problem. The incidence is difficult to establish because of the lack of data in people under age three, but is estimated about 1 per thousand for severe and profound hearing loss.A cochlear implant (CI is a device that converts sounds into electrical energy that triggers a sensation of hearing. The IC is indicated in patients with severe bilateral sensorineural hearing loss with null or poor benefit use of hearing aids.The general objective of this project is to understand the experiences of adult patients with severe-profound sensorineural hearing loss with IC postlingually throughout the implementation process.A personal vision of those implemented will allow us to learn how to face the possibility to hear and interact with their environment, applying this information to improve health care provided to them and identifying those areas where such assistance should be improved. Also allow us to compare the initial expectations and have been achieved, creating realistic expectations for future candidates.For its development we have designed a qualitative study, based on the principles and procedures of grounded theory, semistructured interviews, participant observation and discussion groups.The data will be analyzed using the software Nudist ViVo 9.

  7. [Multicenter trial for sudden hearing loss therapy - planning and concept].

    Science.gov (United States)

    Plontke, S K; Girndt, M; Meisner, C; Probst, R; Oerlecke, I; Richter, M; Steighardt, J; Dreier, G; Weber, A; Baumann, I; Plößl, S; Löhler, J; Laszig, R; Werner, J A; Rahne, T

    2016-04-01

    Systemic steroids are widely used worldwide as a standard of care for primary therapy of idiopathic sudden sensorineural hearing loss (ISSHL). The German ISSHL guideline recommends high-dose steroids for primary therapy of ISSHL, without evidence from randomized controlled trials (RCTs). The rationale for the treatment of ISSHL using high dose steroids is only based on retrospective cohort studies.This article describes the planning and initiation of a multicenter, national, randomized, controlled clinical trial entitled Efficacy and safety of high dose glucocorticosteroid treatment for idiopathic sudden sensorineural hearing loss - a three-armed, randomized, triple-blind, multicenter trial (HODOKORT). This clinical trial aims to compare standard dose with two types of high-dose steroids for primary systemic therapy with respect to their efficacy in improving hearing, and thus communication ability, in patients with idiopathic sudden sensorineural hearing loss.This study is funded by the "Clinical Trials with High Patient Relevance" research program in the health research framework of the German Federal Ministry of Education and Research. It is one of two studies by the German Study Center of Clinical Trials of the German Society of Otorhinolaryngology, Head and Neck Surgery (DSZ-HNO). Planning and initiation was done in cooperation with the DSZ-HNO, the Coordination Center of Clinical Trials of the Martin-Luther-University Halle-Wittenberg, and the Study Center of the University Hospital Freiburg.

  8. Visual impairment in severe and profound sensorineural deafness.

    OpenAIRE

    Armitage, I M; Burke, J. P.; Buffin, J T

    1995-01-01

    The frequency of reversible and irreversible visual impairment was determined in children with severe and profound sensorineural deafness, as subnormal vision can adversely affect their educational and social development. Eighty three of 87 such children attending an audiology service were examined to assess the incidence and severity of visual impairment. Each child underwent a detailed ophthalmic assessment. The criteria for visual impairment were visual acuity < 6/9 Snellen or equivalent a...

  9. Epilepsy, ataxia, sensorineural deafness, tubulopathy, and KCNJ10 mutations.

    Science.gov (United States)

    Bockenhauer, Detlef; Feather, Sally; Stanescu, Horia C; Bandulik, Sascha; Zdebik, Anselm A; Reichold, Markus; Tobin, Jonathan; Lieberer, Evelyn; Sterner, Christina; Landoure, Guida; Arora, Ruchi; Sirimanna, Tony; Thompson, Dorothy; Cross, J Helen; van't Hoff, William; Al Masri, Omar; Tullus, Kjell; Yeung, Stella; Anikster, Yair; Klootwijk, Enriko; Hubank, Mike; Dillon, Michael J; Heitzmann, Dirk; Arcos-Burgos, Mauricio; Knepper, Mark A; Dobbie, Angus; Gahl, William A; Warth, Richard; Sheridan, Eamonn; Kleta, Robert

    2009-05-07

    Five children from two consanguineous families presented with epilepsy beginning in infancy and severe ataxia, moderate sensorineural deafness, and a renal salt-losing tubulopathy with normotensive hypokalemic metabolic alkalosis. We investigated the genetic basis of this autosomal recessive disease, which we call the EAST syndrome (the presence of epilepsy, ataxia, sensorineural deafness, and tubulopathy). Whole-genome linkage analysis was performed in the four affected children in one of the families. Newly identified mutations in a potassium-channel gene were evaluated with the use of a heterologous expression system. Protein expression and function were further investigated in genetically modified mice. Linkage analysis identified a single significant locus on chromosome 1q23.2 with a lod score of 4.98. This region contained the KCNJ10 gene, which encodes a potassium channel expressed in the brain, inner ear, and kidney. Sequencing of this candidate gene revealed homozygous missense mutations in affected persons in both families. These mutations, when expressed heterologously in xenopus oocytes, caused significant and specific decreases in potassium currents. Mice with Kcnj10 deletions became dehydrated, with definitive evidence of renal salt wasting. Mutations in KCNJ10 cause a specific disorder, consisting of epilepsy, ataxia, sensorineural deafness, and tubulopathy. Our findings indicate that KCNJ10 plays a major role in renal salt handling and, hence, possibly also in blood-pressure maintenance and its regulation. 2009 Massachusetts Medical Society

  10. Current perception threshold and the HAVS Stockholm sensorineural scale.

    Science.gov (United States)

    House, Ron; Krajnak, Kristine; Manno, Michael; Lander, Lina

    2009-10-01

    It is important to determine which tests of sensorineural dysfunction identify the neurological damage from hand-arm vibration exposure. To examine the association between the hand-arm vibration syndrome (HAVS) Stockholm sensorineural scale stages and tests of peripheral neurological function including measurement of current perception threshold (CPT) and nerve conduction. All the subjects were men who were assessed for HAVS with a medical and occupational history and physical examination to determine the Stockholm stage, CPT testing at frequencies of 5, 250 and 2000 Hz for the median and ulnar nerves and measurement of nerve conduction carried out in a blinded fashion. A total of 155 of the 157 recruited subjects agreed to take part in the study, a 99% participation rate. CPT was statistically significantly increased (P Stockholm sensorineural Stages 1 and > or =2 in comparison to Stage 0 for every frequency and nerve combination. However, CPT could not discriminate well between Stages 1 and > or =2. There was no association between median or ulnar neuropathy measured by nerve conduction and the Stockholm stages. Polychotomous multinomial logistic regression indicated that the CPT measurements at 2000 Hz, corresponding to damage to large myelinated nerve fibres, were most predictive of both Stockholm Stages 1 and > or =2 in comparison to Stage 0. Neuropathy measured by nerve conduction was unrelated to the Stockholm scale stages. CPT was increased above Stage 0 but did not distinguish well between the higher stages of the Stockholm scale.

  11. Contiguous deletion of SLC6A8 and BAP31 in a patient with severe dystonia and sensorineural deafness.

    Science.gov (United States)

    Osaka, Hitoshi; Takagi, Atsushi; Tsuyusaki, Yu; Wada, Takahito; Iai, Mizue; Yamashita, Sumimasa; Shimbo, Hiroko; Saitsu, Hirotomo; Salomons, Gajja S; Jakobs, Cornelis; Aida, Noriko; Toshihiro, Shinka; Kuhara, Tomiko; Matsumoto, Naomichi

    2012-05-01

    We report here a 6-year-old boy exhibiting severe dystonia, profound intellectual and developmental disability with liver disease, and sensorineural deafness. A deficient creatine peak in brain (1)H-MR spectroscopy and high ratio of creatine/creatinine concentration in his urine lead us to suspect a creatine transporter (solute carrier family 6, member 8; SLC6A8) deficiency, which was confirmed by the inability to take up creatine into fibroblasts. We found a large ~19 kb deletion encompassing exons 5-13 of SLC6A8 and exons 5-8 of the B-cell receptor-associated protein (BAP31) gene. This case is the first report in which the SLC6A8 and BAP31 genes are both deleted. The phenotype of BAP31 mutations has been reported only as a part of Xq28 deletion syndrome or contiguous ATP-binding cassette, sub-family D, member 1 (ABCD1)/DXS1375E (BAP31) deletion syndrome [MIM ID #300475], where liver dysfunction and sensorineural deafness have been suggested to be attributed to the loss of function of BAP31. Our case supports the idea that the loss of BAP31 is related to liver dysfunction and hearing loss. Copyright © 2012 Elsevier Inc. All rights reserved.

  12. Assessment of the risk factors for hearing loss in adult Nigerian ...

    African Journals Online (AJOL)

    Background: A reduction in hearing sensitivity is common in adults and was previously considered to be normal as age increases. However, other health variables may play a role in the sensory changes. This prospective, comparative, hospital‑based study assessed the risk factors (RFs) associated with sensorineural ...

  13. Working Memory and Speech Comprehension in Older Adults with Hearing Impairment

    Science.gov (United States)

    Nagaraj, Naveen K.

    2017-01-01

    Purpose: This study examined the relationship between working memory (WM) and speech comprehension in older adults with hearing impairment (HI). It was hypothesized that WM would explain significant variance in speech comprehension measured in multitalker babble (MTB). Method: Twenty-four older (59-73 years) adults with sensorineural HI…

  14. The effect of cochlear implantation on tinnitus in patients with bilateral hearing loss : A systematic review

    NARCIS (Netherlands)

    Ramakers, Geerte G J; van Zon, Alice; Stegeman, Inge; Grolman, Wilko

    2015-01-01

    OBJECTIVES: To present an overview of the effect of cochlear implantation on tinnitus in adults with bilateral sensorineural hearing loss. DATA SOURCES: PubMed, Cochrane Library, CINAHL, and Embase databases were searched for articles from database inception up to January 13, 2015. METHODS: A

  15. Relations between psychophysical data and speech perception for hearing-impaired subjects. II

    NARCIS (Netherlands)

    Dreschler, W. A.; Plomp, R.

    1985-01-01

    Twenty-one sensorineurally hearing-impaired adolescents were studied with an extensive battery of tone-perception, phoneme-perception, and speech-perception tests. Tests on loudness perception, frequency selectivity, and temporal resolution at the test frequencies of 500, 1000, and 2000 Hz were

  16. Genetic and Pharmacological Intervention for Treatment/Prevention of Hearing Loss

    Science.gov (United States)

    Cotanche, Douglas A.

    2008-01-01

    Twenty years ago it was first demonstrated that birds could regenerate their cochlear hair cells following noise damage or aminoglycoside treatment. An understanding of how this structural and functional regeneration occurred might lead to the development of therapies for treatment of sensorineural hearing loss in humans. Recent experiments have…

  17. MYO15A splicing mutations in hearing loss: A review literature and report of a novel mutation.

    Science.gov (United States)

    Motavaf, Mahsa; Soveizi, Mahdieh; Maleki, Majid; Mahdieh, Nejat

    2017-05-01

    Sensorineural hearing loss (SNHL) is the most prevalent genetic sensory defect in humans, affecting about 1 in 1000 newborns around the world. Non-syndromic SNHL accounts for nearly 70% of hereditary hearing loss and 80% of SNHL cases show an autosomal recessive mode of inheritance (ARNSHL). In the present study, we applied targeted-exome sequencing to a family with a single proband affected by congenital sensorineural hearing loss. 127 known genes were sequenced to find the causative mutation. One novel homozygous donor splice site mutation, c.4596 + 1G > A (IVS12 + 1G > A) was found in MYO15A gene. Analysis of this mutation within the family showed that the mutation segregates with hearing loss. New DNA sequencing technologies could lead to identification of the disease causing variants especially in highly heterogeneous disorders such as hearing loss. Copyright © 2017 Elsevier B.V. All rights reserved.

  18. [The problems of hearing impairment in the flying staff of commercial aviation in Russia].

    Science.gov (United States)

    Pankova, V B; Bushmanov, A Iu

    2014-01-01

    The authors discuss the problems pertaining to the growing incidence of hearing impairment in the members of the flying staff employed in commercial aviation of Russia and the main criteria used to elucidate the causes behind occupational diseases of the organs of hearing. Special attention is given to the principal normative documents regulating the methodological basis on which the acoustic factor in the aircraft cockpit is evaluated, peculiarities of occupational sensorineural hearing impairment and the methods for its detection. The main errors in the determination of the relationship between the working conditions and the diseases of the organs of hearing are discussed.

  19. Is cognitive function in adults with hearing impairment improved by the use of hearing AIDS?

    Science.gov (United States)

    Choi, A Young; Shim, Hyun Joon; Lee, Sung Hee; Yoon, Sang Won; Joo, Eun-Jeong

    2011-06-01

    In the present study, we investigated whether speech-related cognitive function and speech recognition ability under background noise in adults with hearing impairment are improved with the use of hearing aids. Participants were recruited from the ENT Department of Eulji Hospital from September 2008 to July 2009. The study group comprised 18 participants (mean age, 69.5±8.3 years) with sensorineural hearing loss who were fitted with hearing aids, and the control group comprised 11 participants of equivalent age (mean age, 63.1±11.8 years) who were not fitted with hearing aids. All participants were assessed using the computerized Korean visual verbal learning test (VVLT) and words-in-noise (WIN) test prior to fitting of hearing aids for the study group and initially for the control group. Both groups were reassessed in both tests after 6 months. For each group, differences in the results between the two assessments were compared using the Friedman test. There was no difference in mean age between the study group and control group. In the study group, total VVLT score (reflecting short-term memory) was significantly improved from before hearing aid use to 6 months after hearing aid use (Plearning ability) was also significantly improved from before hearing aid use to 6 months after hearing aid use (P0.05). The speech-related cognitive function of individuals with hearing impairment improved after using hearing aids. This finding indicates that hearing aids may induce acclimatization of the central auditory system.

  20. Congenital sensorineural deafness in dalmatian dogs associated with quantitative trait loci.

    Science.gov (United States)

    Kluth, Susanne; Distl, Ottmar

    2013-01-01

    A genome-wide association study (GWAS) was performed for 235 Dalmatian dogs using the canine Illumina high density bead chip to identify quantitative trait loci (QTL) associated with canine congenital sensorineural deafness (CCSD). Data analysis was performed for all Dalmatian dogs and in addition, separately for brown-eyed and blue-eyed dogs because of the significant influence of eye colour on CCSD in Dalmatian dogs. Mixed linear model analysis (MLM) revealed seven QTL with experiment-wide significant associations (-log10P>5.0) for CCSD in all Dalmatian dogs. Six QTL with experiment-wide significant associations for CCSD were found in brown-eyed Dalmatian dogs and in blue-eyed Dalmatian dogs, four experiment-wide significant QTL were detected. The experiment-wide CCSD-associated SNPs explained 82% of the phenotypic variance of CCSD. Five CCSD-loci on dog chromosomes (CFA) 6, 14, 27, 29 and 31 were in close vicinity of genes shown as causative for hearing loss in human and/or mouse.

  1. Congenital sensorineural deafness in dalmatian dogs associated with quantitative trait loci.

    Directory of Open Access Journals (Sweden)

    Susanne Kluth

    Full Text Available A genome-wide association study (GWAS was performed for 235 Dalmatian dogs using the canine Illumina high density bead chip to identify quantitative trait loci (QTL associated with canine congenital sensorineural deafness (CCSD. Data analysis was performed for all Dalmatian dogs and in addition, separately for brown-eyed and blue-eyed dogs because of the significant influence of eye colour on CCSD in Dalmatian dogs. Mixed linear model analysis (MLM revealed seven QTL with experiment-wide significant associations (-log10P>5.0 for CCSD in all Dalmatian dogs. Six QTL with experiment-wide significant associations for CCSD were found in brown-eyed Dalmatian dogs and in blue-eyed Dalmatian dogs, four experiment-wide significant QTL were detected. The experiment-wide CCSD-associated SNPs explained 82% of the phenotypic variance of CCSD. Five CCSD-loci on dog chromosomes (CFA 6, 14, 27, 29 and 31 were in close vicinity of genes shown as causative for hearing loss in human and/or mouse.

  2. Occupational hearing loss of market mill workers in the city of Accra, Ghana

    Directory of Open Access Journals (Sweden)

    Emmanuel D Kitcher

    2014-01-01

    Full Text Available 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 markets in the city of Accra, Ghana, were evaluated using a structured questionnaire and pure tone audiometry. The questionnaire assessed factors including self-reported hearing loss, tinnitus, knowledge on the effects of noise on hearing health and the use of hearing protective devices. Pure tone audiometric testing was conducted for both mill workers and controls. Noise levels at the work premises of the mill workers and controls were measured. Symptoms of hearing loss were reported by 24 (23.76% and 8 (7.7% mill workers and controls respectively. Fifty-five (54.5% and fifty-four (52.37% mill workers and controls exhibited knowledge of the effects of noise on hearing health. Five (5.0% mill workers used hearing protective devices. There was significant sensorineural hearing loss and the presence of a 4 kHz audiometric notch among mill workers when compared with controls for the mean thresholds of 2 kHz, 3 kHz and 4 kHz (P = 0. 001. The prevalence of hearing loss in the better hearing ears of the mill workers and controls was 24.8% and 4.8% respectively (P < 0.5. The prevalence of hearing loss, which may be characteristic of NIHL in the better hearing ears of the mill workers and controls was 24.8% and 4.8% respectively. The majority of mill workers did not use hearing protection.

  3. [Phenotypic trends and breeding values for canine congenital sensorineural deafness in Dalmatian dogs].

    Science.gov (United States)

    Blum, Meike; Distl, Ottmar

    2014-01-01

    In the present study, breeding values for canine congenital sensorineural deafness, the presence of blue eyes and patches have been predicted using multivariate animal models to test the reliability of the breeding values for planned matings. The dataset consisted of 6669 German Dalmatian dogs born between 1988 and 2009. Data were provided by the Dalmatian kennel clubs which are members of the German Association for Dog Breeding and Husbandry (VDH). The hearing status for all dogs was evaluated using brainstem auditory evoked potentials. The reliability using the prediction error variance of breeding values and the realized reliability of the prediction of the phenotype of future progeny born in each one year between 2006 and 2009 were used as parameters to evaluate the goodness of prediction through breeding values. All animals from the previous birth years were used for prediction of the breeding values of the progeny in each of the up-coming birth years. The breeding values based on pedigree records achieved an average reliability of 0.19 for the future 1951 progeny. The predictive accuracy (R2) for the hearing status of single future progeny was at 1.3%. Combining breeding values for littermates increased the predictive accuracy to 3.5%. Corresponding values for maternal and paternal half-sib groups were at 3.2 and 7.3%. The use of breeding values for planned matings increases the phenotypic selection response over mass selection. The breeding values of sires may be used for planned matings because reliabilities and predictive accuracies for future paternal progeny groups were highest.

  4. Pengaruh Hemodialisis Terhadap Kejadian Kurang Pendengaran Sensorineural Pada Penderita Gagal Ginjal Kronik

    Directory of Open Access Journals (Sweden)

    Loriana Ulfa

    2016-06-01

    Full Text Available Sensorineural hearing loss (SNHL can occur in patients with chronic renal failure (CRF who receive hemodialysis (HD. SNHL is caused by hemodialysis occured in any frequency. The factors affect the disease include, among others, age, hypertension, and diabetes mellitus (DM. The effect of hemodialysis onSNHL occurrence in patients with CRF was analyzed in this study. It was a cohort study on 52 patients with CRF who met the inclusion criteria. The sample consists of two groups, 26 patients with CRF who received hemodialysis and 26 patients with CRF who did not received hemodialysis. Timpanoaudiometry was performed on HD group (patient with HD before the first HD and after the third HD. Timpanoaudiometry was performed on HD group (patient without HD at the same time with the first group. The data on age, hypertension, and DM were obtained from medical records. The results were analyzed with Chi-Square test. It was found that 30.7% SNHL patients were in the HD group consisting of 26.9% mild degree patients and 3.8% moderate degree patients . Mean of hearing threshold decrease was 8.13 ± 5.30. There was no patient with SNHL in the non-HD group. Analysis with Chi-Square test showed that hemodialysis affects SNHL occurrence (p=0.004. Age (p=0.084, hypertension (p=0.215, and DM (p=0.683 do not affect SNHL occurrence. It is concluded that hemodialysis affects SNHL occurrence whileage, hypertension, and DM do not. [MKB. 2016;48(2:98–104

  5. Cancer treatment in determination of hearing loss.

    Science.gov (United States)

    Oliveira, Priscila Feliciano de; Oliveira, Camila Silva; Andrade, Joice Santos; Santos, Tamara Figueiredo do Carmo; Oliveira-Barreto, Aline Cabral de

    2016-01-01

    Chemotherapy and radiotherapy in oncology have repercussions in hearing health, and can damage structures of the inner ear. These repercussions usually, result in a bilateral and irreversible hearing loss. To identify sensorineural hearing loss cases with complaints of tinnitus and difficulty in speech understanding and investigate their relationship with the types of chemotherapy and radiotherapy the patients received. Cross-sectional, clinical, observational, analytical, historical cohort study of 58 subjects treated in a public hospital in the state of Sergipe, diagnosed with neoplasia. The subjects were submitted to anamnesis, conventional pure tone audiometry, and speech recognition threshold. Of the 116 ears, 25.9% presented sensorioneural hearing loss characterized by changes in high frequencies. There was a positive correlation between hearing loss and the association of chemotherapy and radiotherapy (p=0.035; R=0.196). The auditory complaint analysis shows that most of the subjects had tinnitus and speech understanding difficulty, even with a normal auditory threshold. Cancer treatment causes hearing loss, associated with the administration of chemotherapy and radiotherapy. Cyclophosphamide increased the risk of causing hearing loss. Complaints of tinnitus and speech understanding difficulty were observed. Copyright © 2015 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  6. Inflectional morphology in German hearing-impaired children.

    Science.gov (United States)

    Penke, Martina; Wimmer, Eva; Hennies, Johannes; Hess, Markus; Rothweiler, Monika

    2016-01-01

    Despite modern hearing aids, children with hearing impairment often have only restricted access to spoken language input during the 'critical' years for language acquisition. Specifically, a sensorineural hearing impairment affects the perception of voiceless coronal consonants which realize verbal affixes in German. The aim of this study is to explore if German hearing-impaired children have problems in producing and/or acquiring inflectional suffixes expressed by such phonemes. The findings of two experiments (an elicitation task and a picture-naming task) conducted with a group of hearing-impaired monolingual German children (age 3-4 years) demonstrate that difficulties in perceiving specific phonemes relate to the avoidance of these same sounds in speech production independent of the grammatical function these phonemes have.

  7. Hearing Screening

    Science.gov (United States)

    Johnson-Curiskis, Nanette

    2012-01-01

    Hearing levels are threatened by modern life--headsets for music, rock concerts, traffic noises, etc. It is crucial we know our hearing levels so that we can draw attention to potential problems. This exercise requires that students receive a hearing screening for their benefit as well as for making the connection of hearing to listening.

  8. About Hearing

    Science.gov (United States)

    ... with unilateral hearing should be monitored closely for linguistic, educational, or social gaps. For more information about ... see: Info to Go/Hearing Aids For more information about cochlear implants, see: Info ... resources about hearing, see: Hearing and Amplification (My Baby's ...

  9. Neuromagnetic index of hemispheric asymmetry prognosticating the outcome of sudden hearing loss.

    Science.gov (United States)

    Li, Lieber Po-Hung; Shiao, An-Suey; Chen, Kuang-Chao; Lee, Po-Lei; Niddam, David M; Chang, Shyue-Yih; Hsieh, Jen-Chuen

    2012-01-01

    The longitudinal relationship between central plastic changes and clinical presentations of peripheral hearing impairment remains unknown. Previously, we reported a unique plastic pattern of "healthy-side dominance" in acute unilateral idiopathic sudden sensorineural hearing loss (ISSNHL). This study aimed to explore whether such hemispheric asymmetry bears any prognostic relevance to ISSNHL along the disease course. Using magnetoencephalography (MEG), inter-hemispheric differences in peak dipole amplitude and latency of N100m to monaural tones were evaluated in 21 controls and 21 ISSNHL patients at two stages: initial and fixed stage (1 month later). Dynamics/Prognostication of hemispheric asymmetry were assessed by the interplay between hearing level/hearing gain and ipsilateral/contralateral ratio (I/C) of N100m latency and amplitude. Healthy-side dominance of N100m amplitude was observed in ISSNHL initially. The pattern changed with disease process. There is a strong correlation between the hearing level at the fixed stage and initial I/C(amplitude) on affected-ear stimulation in ISSNHL. The optimal cut-off value with the best prognostication effect for the hearing improvement at the fixed stage was an initial I/C(latency) on affected-ear stimulation of 1.34 (between subgroups of complete and partial recovery) and an initial I/C(latency) on healthy-ear stimulation of 0.76 (between subgroups of partial and no recovery), respectively. This study suggested that a dynamic process of central auditory plasticity can be induced by peripheral lesions. The hemispheric asymmetry at the initial stage bears an excellent prognostic potential for the treatment outcomes and hearing level at the fixed stage in ISSNHL. Our study demonstrated that such brain signature of central auditory plasticity in terms of both N100m latency and amplitude at defined time can serve as a prognostication predictor for ISSNHL. Further studies are needed to explore the long-term temporal scenario

  10. The effect of symmetrical and asymmetrical hearing impairment on music quality perception.

    Science.gov (United States)

    Cai, Yuexin; Zhao, Fei; Chen, Yuebo; Liang, Maojin; Chen, Ling; Yang, Haidi; Xiong, Hao; Zhang, Xueyuan; Zheng, Yiqing

    2016-09-01

    The purpose of this study was to investigate the effect of symmetrical, asymmetrical and unilateral hearing impairment on music quality perception. Six validated music pieces in the categories of classical music, folk music and pop music were used to assess music quality in terms of its 'pleasantness', 'naturalness', 'fullness', 'roughness' and 'sharpness'. 58 participants with sensorineural hearing loss [20 with unilateral hearing loss (UHL), 20 with bilateral symmetrical hearing loss (BSHL) and 18 with bilateral asymmetrical hearing loss (BAHL)] and 29 normal hearing (NH) subjects participated in the present study. Hearing impaired (HI) participants had greater difficulty in overall music quality perception than NH participants. Participants with BSHL rated music pleasantness and naturalness to be higher than participants with BAHL. Moreover, the hearing thresholds of the better ears from BSHL and BAHL participants as well as the hearing thresholds of the worse ears from BSHL participants were negatively correlated to the pleasantness and naturalness perception. HI participants rated the familiar music pieces higher than unfamiliar music pieces in the three music categories. Music quality perception in participants with hearing impairment appeared to be affected by symmetry of hearing loss, degree of hearing loss and music familiarity when they were assessed using the music quality rating test (MQRT). This indicates that binaural symmetrical hearing is important to achieve a high level of music quality perception in HI listeners. This emphasizes the importance of provision of bilateral hearing assistive devices for people with asymmetrical hearing impairment.

  11. [Influence of hearing aids on monosyllabic test score and subjective everyday hearing].

    Science.gov (United States)

    Thümmler, R; Liebscher, T; Hoppe, U

    2016-08-01

    Pure tone and speech audiometry are essential methods for examining the indication for hearing aids, as well as for hearing aid evaluation. Additionally, the subjective benefit of hearing aids has to be evaluated with appropriate questionnaires. The aim of the present study was to investigate the correlation between speech audiometry data and the results of a simple and user-friendly questionnaire, as well as to provide normative data for subjective benefit. Data from 136 hearing aid users with bilateral sensorineural hearing loss were analyzed retrospectively. Pure tone thresholds and Freiburg monosyllabic speech perception in the binaural situation were measured at 65 dB in quiet and in noise (signal-to-noise ratio, SNR = +5 dB), with and without hearing aids. Additionally, subjective hearing in everyday life was recorded using the 12-item Oldenburg Inventory. Improvement of speech perception with hearing aids for the Freiburg monosyllabic test in quiet was 32.0 percentage points on average; in noise, there was an average improvement of 16.4 percentage points. There was a strong correlation between the results of pure tone and speech audiometry. With hearing aids, patients scored their everyday hearing using the Oldenburg Inventory on average 1.4 scale points better than without hearing aids. Results of the Oldenburg Inventory correlate with both pure tone and speech audiometry. Hearing aid evaluation should include both speech audiometry and systematic measurement of the subjective benefit using a suitable questionnaire. In combination, the Freiburg monosyllabic test and the Oldenburg Inventory allow for quick and comprehensive evaluation.

  12. Management of sudden hearing loss with hyperbaric oxygen therapy.

    Science.gov (United States)

    Gaitanou, K; Fildissis, G; Vavasis, P; Kalentzos, V; Baltopoulos, G

    2014-01-01

    Idiopathic sudden sensorineural hearing loss (ISSHL) is defined as the sensorineural hearing loss of a minimum of 30 dB in at least three frequencies for three days or more. This study aims to evaluate the clinical efficacy of hyperbaric oxygen therapy (HBO2) in the management of idiopathic sudden sensorineural hearing loss. Patients with ISSHL received treatment including oral corticosteroids and HBO2. HBO2 protocol included five phases of five sessions each. ISSHL was assessed by measuring the tonal audiogram before and after each phase. Tinnitus was assessed using a questionnaire and a visual analogue scale at the beginning and the end of the study. Secondary assessment points included changes in the intensity and the improvement of tinnitus. 56 patients were included in the study. All patients completed Phases I and II of HBO2, 43 completed Phase III, 13 completed Phase IV, and six completed all five phases. Overall, a significant improvement was noted between the initial and final audiogram after HBO2 (p problems were also significantly reduced (p < 0.001). This study affirms previous findings that the use of HBO2 and vasodilators are efficacious in the treatment of ISSHL. Our findings also suggest benefit for the treatment of tinnitus.

  13. Hearing Loss in Patients with Nasopharyngeal Carcinoma after Chemotherapy and Radiation

    Directory of Open Access Journals (Sweden)

    Ling-Feng Wang

    2003-04-01

    Full Text Available In light of the possible adverse effects of radiation on hearing, we conducted a study to evaluate the long-term sensorineural hearing status following radiotherapy (RT in patients suffering from nasopharyngeal carcinoma. Audiologic examinations were performed at regular intervals before and after RT. We also analyzed the effects of age, chemotherapy, pre-RT hearing status, and post-RT otitis media with effusion (OME on post-RT hearing change. A total of 150 patients (261 ears were enrolled in this study and followed up for a mean of 43.8 months. After RT, 8.9-28.8% of ears had at least a 10 dB loss in bone conduction threshold at speech frequency, which was defined as an average of hearing threshold at 0.5 kHz, 1 kHz, and 2 kHz, while the percentage was 18-34.2% at 4 kHz. Patient age was related to these changes at speech frequency, and the presence of post-RT OME was related to significant loss at both speech frequency and 4 kHz. Pre-RT hearing status and chemotherapy did not influence hearing change. To sum up, sensorineural hearing loss began as early as after completion of RT. Early changes may be transient, but the effect of radiation on hearing tended to be chronic and progressive.

  14. Long-term hearing preservation after microsurgical excision of vestibular schwannoma.

    Science.gov (United States)

    Woodson, Erika Ann; Dempewolf, Ryan Douglas; Gubbels, Samuel Paul; Porter, Aaron Thomas; Oleson, Jacob Jay; Hansen, Marlan Rex; Gantz, Bruce Jay

    2010-09-01

    To examine long-term hearing outcomes after microsurgical excision of vestibular schwannoma (VS). Retrospective case review. Tertiary referral center. Forty-nine subjects at a single institution who had undergone microsurgical excision of a VS via middle cranial fossa (MCF) approach between 1994 and 2007 with immediate postoperative (PO) hearing preservation and for whom long-term audiograms were available. Diagnostic. Word Recognition Score (WRS) is defined by speech discrimination scores (SDS) greater than 70% (grade I), 50% to 70% (grade II), less than 50% (grade III), and 0% (grade IV). For subjects with more than 2 years of follow-up, WRS I hearing was present PO in 42 of 49 patients and was preserved at the latest follow-up in 38 (90%) of 42 patients. No subjects fell beyond WRS II. WRS I hearing was maintained in 23 (88%) of 26 patients with more than 5 years of follow-up. Postoperative WRS I to II hearing was maintained in 28 (96%) of 29 patients with more than 5 years of follow-up. The patient who lost significant hearing in the ear operated on had sensorineural hearing loss that paralleled deterioration in her ear that was not operated on. Most subjects maintain their initial PO SDS after microsurgical VS removal, and therefore, the initial PO WRS is predictive of long-term hearing. Postsurgical changes do not alter the natural rate or pattern of progressive bilateral sensorineural hearing loss in individual subjects.

  15. Clinical results of hearing aid with noise-level-controlled selective amplification.

    Science.gov (United States)

    Ono, H; Kanzaki, J; Mizoi, K

    1983-01-01

    We have developed a hearing aid which reduces the low-frequency energy of the input sound according to the input noise level and the spectrum of the environmental noise. The results of speech discrimination tests using speech in noise in 70 hearing-impaired subjects showed improvement in word discrimination scores in noise of over 15% for 50 of 53 subjects with sensorineural hearing loss. This group of hearing-impaired patients was subdivided into categories according to (a) audiogram type [flat, sloping (i.e. gradual high-frequency loss) and abruptly falling (steep high-frequency slope)]; (b) degree of hearing loss (less than or more than 50 dB average loss) and (c) etiology (familial deafness, presbyacusis). Results on 17 subjects with conductive or mixed hearing impairments showed no improvement, but expressed a preference for this system. The subject reported that the sound was more natural and quiet compared with the conventional AGC hearing aids.

  16. Hearing loss in Pompe disease revisited: results from a study of 24 children.

    Science.gov (United States)

    van Capelle, Carine I; Goedegebure, Andre; Homans, Nienke C; Hoeve, Hans L J; Reuser, Arnold J; van der Ploeg, Ans T

    2010-10-01

    Little information is available regarding the auditory function in Pompe patients. Hearing loss has been reported in classic infantile patients, but it is still unknown whether central nervous system involvement interferes with auditory function and whether enzyme replacement therapy can improve hearing. Auditory function has not been studied in children with milder forms of the disease. We analyzed repetitive auditory brainstem response measurements and pure tone audiometry in 24 children with Pompe disease. Only 1 of 13 patients with milder phenotypes showed recurrent conductive hearing loss, while 10 out of 11 classic infantile patients had sensorineural hearing defects. These patients also had a high prevalence of conductive hearing loss. Five patients showed evidence of mild retrocochlear pathology, suggestive of glycogen accumulation in the central nervous system. Hearing loss persisted during therapy in all patients. The results emphasize the need for careful monitoring of auditory function in classic infantile Pompe patients, and for early implementation of hearing aids to protect speech and language development.

  17. Extended high frequency audiometry can diagnose sub-clinic involvement in a seemingly normal hearing systemic lupus erythematosus population.

    Science.gov (United States)

    Lasso de la Vega, Mar; Villarreal, Ithzel María; López Moya, Julio; García-Berrocal, José Ramón

    2017-02-01

    Sensorineural hearing loss must be considered within the clinical picture of systemic lupus erythematosus. The results confirm the usefulness of extended high-frequency audiometry in the audiologic testing of these patients, enabling the possibility of modifying or applying a preventive treatment for a possible hearing loss. Hearing involvement is usually under-diagnosed with routine auditory examination. This study proposes the use of extended high-frequency audiometry to achieve a correct detection of a possible asymptomatic hypoacusis in early stages of the disease. The aim of this study is to analyze the hearing levels in extended high-frequencies in these patients and to correlate the hearing loss with the severity of the disease and the immunological parameters. A descriptive cross-sectional study was performed. Fifty-five patients with systemic lupus erythematosus were included in the study. The control group consisted of 71 patients paired by age and sex with the study population. Both a pure tone audiometry and an extended high-frequency audiometry (8-18 KHz) were performed. In total, 70% were diagnosed with sensorineural hearing loss with extended high-frequency audiometry, overcoming the results obtained with pure tone audiometry (30.9%). Statistically significant correlations were found within the patients regarding sensorineural hearing loss related with age, disease activity and cryoglobulinemia.

  18. [Neurologic semiology in a population of hearing impaired children].

    Science.gov (United States)

    Gonçalves, V M; Piovesana, A M; de Moura-Ribeiro, M V

    1993-09-01

    A random sample of 42 sensorineural hearing impaired children (severe and bilateral) was studied, from special classes in Campinas, with chronological ages varying between 4 and 7 years old. The children of this sample were compared with two control groups of 42 children of the same chronological age, from regular classes of private and public schools. All of them were submitted to the traditional neurological examination. Hearing impaired children showed differences as to head circumference and muscle tonus. In the other examined items we found motor hyperactivity, cerebellar and ocular syndromes although there were no significant differences between the groups.

  19. Development and analysis of a low-cost screening tool to identify and classify hearing loss in children: a proposal for developing countries.

    Science.gov (United States)

    Samelli, Alessandra Giannella; Rabelo, Camila Maia; Vespasiano, Ana Paula Chaparin

    2011-01-01

    A lack of attention has been given to hearing health in primary care in developing countries. A strategy involving low-cost screening tools may fill the current gap in hearing health care provided to children. Therefore, it is necessary to establish and adopt lower-cost procedures that are accessible to underserved areas that lack other physical or human resources that would enable the identification of groups at risk for hearing loss. The aim of this study was to develop and analyze the efficacy of a low-cost screening tool to identify and classify hearing loss in children. A total of 214 2-to-10 year-old children participated in this study. The study was conducted by providing a questionnaire to the parents and comparing the answers with the results of a complete audiological assessment. Receiver operating characteristic (ROC) curves were constructed, and discriminant analysis techniques were used to classify each child based on the total score. We found conductive hearing loss in 39.3% of children, sensorineural hearing loss in 7.4% and normal hearing in 53.3%. The discriminant analysis technique provided the following classification rule for the total score on the questionnaire: 0 to 4 points - normal hearing; 5 to 7 points - conductive hearing loss; over 7 points - sensorineural hearing loss. Our results suggest that the questionnaire could be used as a screening tool to classify children with normal hearing or hearing loss and according to the type of hearing loss based on the total questionnaire score.

  20. [Hearing screening at nursery schools: results of an evaluation study].

    Science.gov (United States)

    Weichbold, Viktor; Rohrer, Monika; Winkler, Cornelia; Welzl-Müller, Kunigunde

    2004-07-31

    This study aimed to evaluate the hearing screening of pre-school children at nursery schools in Tyrol, Austria. 47 nursery schools with a total of 2199 enrolled children participated in the study. At the screening, the children were presented a series of tones at frequencies 0.5 kHz (25dB), 1 kHz, 2 kHz, 3 kHz, and 4 kHz (20 dB each) from portable audiometers. The tones were presented over headphones for each ear separately and at irregular intervals. Failure to respond to any of the frequencies was considered failure of the screening. Parents were then advised in written form to have the child examined by an ENT-specialist. 1832 individuals were screened (coverage: 83% of nursery school children; corresponding to at least 63% of all Tyrolean children aged 3 to 5 years). Of these, 390 failed the test (referral rate: 21% of all screened). Examination through an ENT-specialist occurred with 217 children, and this confirmed the positive test in 139 children (hit rate: 64%). In most cases, a temporary conductive hearing loss due to external or middle ear problems (glue ear, tube dysfunction, cerumen, otitis media) was diagnosed. A sensorineural hearing loss was found in 4 children (in 3 of them bilateral). The need for therapy was recognized in 81 children (4% of all screened). Pre-school hearing screening identifies children with ear and hearing problems that need therapeutical intervention. Although the hearing problems are mostly of a temporary nature, some may require monitoring over some period. Also some children with permanent sensorineural hearing loss may be detected through this measure. Hearing screening is an efficient means of assessing ear and hearing problems in pre-school children. However, the follow-up rate needs to be improved for optimizing the efficacy.

  1. Hearing Loss in HIV-Infected Children in Lilongwe, Malawi

    Science.gov (United States)

    Hrapcak, Susan; Kuper, Hannah; Bartlett, Peter; Devendra, Akash; Makawa, Atupele; Kim, Maria; Kazembe, Peter; Ahmed, Saeed

    2016-01-01

    Introduction With improved access to antiretroviral therapy (ART), HIV infection is becoming a chronic illness. Preliminary data suggest that HIV-infected children have a higher risk of disabilities, including hearing impairment, although data are sparse. This study aimed to estimate the prevalence and types of hearing loss in HIV-infected children in Lilongwe, Malawi. Methods This was a cross-sectional survey of 380 HIV-infected children aged 4–14 years attending ART clinic in Lilongwe between December 2013-March 2014. Data was collected through pediatric quality of life and sociodemographic questionnaires, electronic medical record review, and detailed audiologic testing. Hearing loss was defined as >20 decibels hearing level (dBHL) in either ear. Predictors of hearing loss were explored by regression analysis generating age- and sex-adjusted odds ratios. Children with significant hearing loss were fitted with hearing aids. Results Of 380 patients, 24% had hearing loss: 82% conductive, 14% sensorineural, and 4% mixed. Twenty-one patients (23% of those with hearing loss) were referred for hearing aid fitting. There was a higher prevalence of hearing loss in children with history of frequent ear infections (OR 7.4, 4.2–13.0) and ear drainage (OR 6.4, 3.6–11.6). Hearing loss was linked to history of WHO Stage 3 (OR 2.4, 1.2–4.5) or Stage 4 (OR 6.4, 2.7–15.2) and history of malnutrition (OR 2.1, 1.3–3.5), but not to duration of ART or CD4. Only 40% of caregivers accurately perceived their child’s hearing loss. Children with hearing impairment were less likely to attend school and had poorer emotional (p = 0.02) and school functioning (p = 0.04). Conclusions There is an urgent need for improved screening tools, identification and treatment of hearing problems in HIV-infected children, as hearing loss was common in this group and affected school functioning and quality of life. Clear strategies were identified for prevention and treatment, since most

  2. Hearing Loss in HIV-Infected Children in Lilongwe, Malawi.

    Directory of Open Access Journals (Sweden)

    Susan Hrapcak

    Full Text Available With improved access to antiretroviral therapy (ART, HIV infection is becoming a chronic illness. Preliminary data suggest that HIV-infected children have a higher risk of disabilities, including hearing impairment, although data are sparse. This study aimed to estimate the prevalence and types of hearing loss in HIV-infected children in Lilongwe, Malawi.This was a cross-sectional survey of 380 HIV-infected children aged 4-14 years attending ART clinic in Lilongwe between December 2013-March 2014. Data was collected through pediatric quality of life and sociodemographic questionnaires, electronic medical record review, and detailed audiologic testing. Hearing loss was defined as >20 decibels hearing level (dBHL in either ear. Predictors of hearing loss were explored by regression analysis generating age- and sex-adjusted odds ratios. Children with significant hearing loss were fitted with hearing aids.Of 380 patients, 24% had hearing loss: 82% conductive, 14% sensorineural, and 4% mixed. Twenty-one patients (23% of those with hearing loss were referred for hearing aid fitting. There was a higher prevalence of hearing loss in children with history of frequent ear infections (OR 7.4, 4.2-13.0 and ear drainage (OR 6.4, 3.6-11.6. Hearing loss was linked to history of WHO Stage 3 (OR 2.4, 1.2-4.5 or Stage 4 (OR 6.4, 2.7-15.2 and history of malnutrition (OR 2.1, 1.3-3.5, but not to duration of ART or CD4. Only 40% of caregivers accurately perceived their child's hearing loss. Children with hearing impairment were less likely to attend school and had poorer emotional (p = 0.02 and school functioning (p = 0.04.There is an urgent need for improved screening tools, identification and treatment of hearing problems in HIV-infected children, as hearing loss was common in this group and affected school functioning and quality of life. Clear strategies were identified for prevention and treatment, since most hearing loss was conductive in nature, likely due to

  3. Hearing Loss in HIV-Infected Children in Lilongwe, Malawi.

    Science.gov (United States)

    Hrapcak, Susan; Kuper, Hannah; Bartlett, Peter; Devendra, Akash; Makawa, Atupele; Kim, Maria; Kazembe, Peter; Ahmed, Saeed

    2016-01-01

    With improved access to antiretroviral therapy (ART), HIV infection is becoming a chronic illness. Preliminary data suggest that HIV-infected children have a higher risk of disabilities, including hearing impairment, although data are sparse. This study aimed to estimate the prevalence and types of hearing loss in HIV-infected children in Lilongwe, Malawi. This was a cross-sectional survey of 380 HIV-infected children aged 4-14 years attending ART clinic in Lilongwe between December 2013-March 2014. Data was collected through pediatric quality of life and sociodemographic questionnaires, electronic medical record review, and detailed audiologic testing. Hearing loss was defined as >20 decibels hearing level (dBHL) in either ear. Predictors of hearing loss were explored by regression analysis generating age- and sex-adjusted odds ratios. Children with significant hearing loss were fitted with hearing aids. Of 380 patients, 24% had hearing loss: 82% conductive, 14% sensorineural, and 4% mixed. Twenty-one patients (23% of those with hearing loss) were referred for hearing aid fitting. There was a higher prevalence of hearing loss in children with history of frequent ear infections (OR 7.4, 4.2-13.0) and ear drainage (OR 6.4, 3.6-11.6). Hearing loss was linked to history of WHO Stage 3 (OR 2.4, 1.2-4.5) or Stage 4 (OR 6.4, 2.7-15.2) and history of malnutrition (OR 2.1, 1.3-3.5), but not to duration of ART or CD4. Only 40% of caregivers accurately perceived their child's hearing loss. Children with hearing impairment were less likely to attend school and had poorer emotional (p = 0.02) and school functioning (p = 0.04). There is an urgent need for improved screening tools, identification and treatment of hearing problems in HIV-infected children, as hearing loss was common in this group and affected school functioning and quality of life. Clear strategies were identified for prevention and treatment, since most hearing loss was conductive in nature, likely due to

  4. Cortical Electrophysiological Markers of Language Abilities in Children with Hearing Aids: A Pilot Study

    Directory of Open Access Journals (Sweden)

    David Bakhos

    2014-01-01

    Full Text Available Objective. To investigate cortical auditory evoked potentials (CAEPs in pediatric hearing aid (HA users, with and without language impairment. Design. CAEPs were measured in 11 pediatric HA users (age: 8–12 years with moderate bilateral sensorineural hearing loss (HL; participants were classified according to language ability. CAEPs were also measured for a control group of 11 age-matched, normal-hearing (NH children. Results. HL children without language impairment exhibited normal CAEPs. HL children with language impairment exhibited atypical temporal CAEPs, characterized by the absence of N1c; frontocentral responses displayed normal age-related patterns. Conclusion. Results suggest that abnormal temporal brain function may underlie language impairment in pediatric HA users with moderate sensorineural HL.

  5. The Role of Insulin-Like Growth Factor 1 in the Progression of Age-Related Hearing Loss.

    Science.gov (United States)

    Rodríguez-de la Rosa, Lourdes; Lassaletta, Luis; Calvino, Miryam; Murillo-Cuesta, Silvia; Varela-Nieto, Isabel

    2017-01-01

    Aging is associated with impairment of sensorial functions and with the onset of neurodegenerative diseases. As pari passu circulating insulin-like growth factor 1 (IGF-1) bioavailability progressively decreases, we see a direct correlation with sensory impairment and cognitive performance in older humans. Age-related sensory loss is typically caused by the irreversible death of highly differentiated neurons and sensory receptor cells. Among sensory deficits, age-related hearing loss (ARHL), also named presbycusis, affects one third of the population over 65 years of age and is a major factor in the progression of cognitive problems in the elderly. The genetic and molecular bases of ARHL are largely unknown and only a few genes related to susceptibility to oxidative stress, excitotoxicity, and cell death have been identified. IGF-1 is known to be a neuroprotective agent that maintains cellular metabolism, activates growth, proliferation and differentiation, and limits cell death. Inborn IGF-1 deficiency leads to profound sensorineural hearing loss both in humans and mice. IGF-1 haploinsufficiency has also been shown to correlate with ARHL. There is not much information available on the effect of IGF-1 deficiency on other human sensory systems, but experimental models show a long-term impact on the retina. A secondary action of IGF-1 is the control of oxidative stress and inflammation, thus helping to resolve damage situations, acute or made chronic by aging. Here we will review the primary actions of IGF-1 in the auditory system and the underlying molecular mechanisms.

  6. The Role of Insulin-Like Growth Factor 1 in the Progression of Age-Related Hearing Loss

    Directory of Open Access Journals (Sweden)

    Lourdes Rodríguez-de la Rosa

    2017-12-01

    Full Text Available Aging is associated with impairment of sensorial functions and with the onset of neurodegenerative diseases. As pari passu circulating insulin-like growth factor 1 (IGF-1 bioavailability progressively decreases, we see a direct correlation with sensory impairment and cognitive performance in older humans. Age-related sensory loss is typically caused by the irreversible death of highly differentiated neurons and sensory receptor cells. Among sensory deficits, age-related hearing loss (ARHL, also named presbycusis, affects one third of the population over 65 years of age and is a major factor in the progression of cognitive problems in the elderly. The genetic and molecular bases of ARHL are largely unknown and only a few genes related to susceptibility to oxidative stress, excitotoxicity, and cell death have been identified. IGF-1 is known to be a neuroprotective agent that maintains cellular metabolism, activates growth, proliferation and differentiation, and limits cell death. Inborn IGF-1 deficiency leads to profound sensorineural hearing loss both in humans and mice. IGF-1 haploinsufficiency has also been shown to correlate with ARHL. There is not much information available on the effect of IGF-1 deficiency on other human sensory systems, but experimental models show a long-term impact on the retina. A secondary action of IGF-1 is the control of oxidative stress and inflammation, thus helping to resolve damage situations, acute or made chronic by aging. Here we will review the primary actions of IGF-1 in the auditory system and the underlying molecular mechanisms.

  7. The effect of simulated unilateral hearing loss on horizontal sound localization accuracy and recognition of speech in spatially separate competing speech.

    Science.gov (United States)

    Asp, Filip; Jakobsson, Anne-Marie; Berninger, Erik

    2018-01-01

    Unilateral hearing loss (UHL) occurs in 25% of cases of congenital sensorineural hearing loss. Due to the unilaterally reduced audibility associated with UHL, everyday demanding listening situations may be disrupted despite normal hearing in one ear. The aim of this study was to quantify acute changes in recognition of speech in spatially separate competing speech and sound localization accuracy, and relate those changes to two levels of temporary induced UHL (UHL30 and UHL43; suffixes denote the average hearing threshold across 0.5, 1, 2, and 4 kHz) for 8 normal-hearing adults. A within-subject repeated-measures design was used (normal binaural conditions, UHL30 and UHL43). The main outcome measures were the threshold for 40% correct speech recognition and the overall variance in sound localization accuracy quantified by an Error Index (0 = perfect performance, 1.0 = random performance). Distinct and statistically significant deterioration in speech recognition (2.0 dB increase in threshold, p  0.05), while sound localization was additionally impaired (Error Index increase of 0.33, p < 0.01) with an associated large increase in individual variability. Qualitative analyses on a subject-by-subject basis showed that high-frequency audibility was important for speech recognition, while low-frequency audibility was important for horizontal sound localization accuracy. While the data might not be entirely applicable to individuals with long-standing UHL, the results suggest a need for intervention for mild-to-moderate UHL. Copyright © 2017 The Authors. Published by Elsevier B.V. All rights reserved.

  8. Etiology of hearing loss in children.

    Directory of Open Access Journals (Sweden)

    José Ignacio BENITO-OREJAS

    2017-06-01

    Full Text Available Introduction and objective: The neonatal hearing loss is one of the most common disabilities, with lifelong implications for the child and his family. The implementation of the universal newborn hearing screening and the development in molecular medicine, genetic and integrative neuroscience has perfected the early diagnosis of the hearing loss children and consequently its intervention. With this work, we want to clarify the audiological aspects and causes of the permanent hearing loss diagnosed during the past 20 years. Method: We reviewed retrospectively the records of the children diagnosed with less than 3 years of age of permanent hearing loss, during the period 1994-2015, in a tertiary center. Evaluate the time of home, laterality, type and degree of hearing loss. Depending on the background, genetic testing and other complementary explorations, we present the results of our diagnostic study. Results: In the study-population (n = 183, 71% of the permanent hearing loss > 30 dB HL was diagnosed at birth (congenital. Its main features are the bilaterality (81%, the predominance sensorineural (85% and the grade profound (42% or moderate (30%, more prevalent in the unilateral forms. About the etiologic diagnosis, a 47% of the cases are of origin genetic (29% of which are syndromic, a 25% of cause environmental and a 28% unknown. Discussion: Our results are consistent for the generally accepted distribution of causes, but there are discrepancies in the literature. Despite the different tests used, we had to infer the etiology in 62% of children with hearing loss, finally unknown by 28%. Conclusions: We consider fundamental the monitoring for a consensus standardized etiological protocol that orient in the diagnostic process of hearing loss in children.

  9. [From gene to disease; genetic causes of hearing loss and visual impairment sometimes accompanied by vestibular problems (Usher syndrome)

    NARCIS (Netherlands)

    Pennings, R.J.E.; Kremer, J.M.J.; Deutman, A.F.; Kimberling, W.J.; Cremers, C.W.R.J.

    2002-01-01

    Usher syndrome is an autosomal recessively inherited disease, characterised by sensorineural hearing loss, tapetoretinal degeneration and in some cases vestibular problems. Based on the clinical heterogeneity, the disease can be classified into three clinical types (I, II and III), which have their

  10. Hearing Problems

    Science.gov (United States)

    ... Read MoreDepression in Children and TeensRead MoreBMI Calculator Hearing ProblemsLoss in the ability to hear or discriminate ... This flow chart will help direct you if hearing loss is a problem for you or a ...

  11. Congenital Sensorineural Deafness in Australian Stumpy-Tail Cattle Dogs Is an Autosomal Recessive Trait That Maps to CFA10

    Science.gov (United States)

    Sommerlad, Susan; McRae, Allan F.; McDonald, Brenda; Johnstone, Isobel; Cuttell, Leigh; Seddon, Jennifer M.; O'Leary, Caroline A.

    2010-01-01

    Background Congenital sensorineural deafness is an inherited condition found in many dog breeds, including Australian Stumpy-tail Cattle Dogs (ASCD). This deafness is evident in young pups and may affect one ear (unilateral) or both ears (bilateral). The genetic locus/loci involved is unknown for all dog breeds. The aims of this study were to determine incidence, inheritance mechanism, and possible association of congenital sensorineural deafness with coat colour in ASCD and to identify the genetic locus underpinning this disease. Methodology/Principal Findings A total of 315 ASCD were tested for sensorineural deafness using the brain stem auditory evoked response (BAER) test. Disease penetrance was estimated directly, using the ratio of unilaterally to bilaterally deaf dogs, and segregation analysis was performed using Mendel. A complete genome screen was undertaken using 325 microsatellites spread throughout the genome, on a pedigree of 50 BAER tested ASCD in which deafness was segregating. Fifty-six dogs (17.8%) were deaf, with 17 bilaterally and 39 unilaterally deaf. Unilaterally deaf dogs showed no significant left/right bias (p = 0.19) and no significant difference was observed in frequencies between the sexes (p = 0.18). Penetrance of deafness was estimated as 0.72. Testing the association of red/blue coat colour and deafness without accounting for pedigree structure showed that red dogs were 1.8 times more likely to be deaf (p = 0.045). The within family association between red/blue coat colour and deafness was strongly significant (p = 0.00036), with red coat colour segregating more frequently with deafness (COR = 0.48). The relationship between deafness and coat speckling approached significance (p = 0.07), with the lack of statistical significance possibly due to only four families co-segregating for both deafness and speckling. The deafness phenotype was mapped to CFA10 (maximum linkage peak on CFA10 −log10 p-value = 3

  12. Congenital sensorineural deafness in Australian stumpy-tail cattle dogs is an autosomal recessive trait that maps to CFA10.

    Directory of Open Access Journals (Sweden)

    Susan Sommerlad

    Full Text Available BACKGROUND: Congenital sensorineural deafness is an inherited condition found in many dog breeds, including Australian Stumpy-tail Cattle Dogs (ASCD. This deafness is evident in young pups and may affect one ear (unilateral or both ears (bilateral. The genetic locus/loci involved is unknown for all dog breeds. The aims of this study were to determine incidence, inheritance mechanism, and possible association of congenital sensorineural deafness with coat colour in ASCD and to identify the genetic locus underpinning this disease. METHODOLOGY/PRINCIPAL FINDINGS: A total of 315 ASCD were tested for sensorineural deafness using the brain stem auditory evoked response (BAER test. Disease penetrance was estimated directly, using the ratio of unilaterally to bilaterally deaf dogs, and segregation analysis was performed using Mendel. A complete genome screen was undertaken using 325 microsatellites spread throughout the genome, on a pedigree of 50 BAER tested ASCD in which deafness was segregating. Fifty-six dogs (17.8% were deaf, with 17 bilaterally and 39 unilaterally deaf. Unilaterally deaf dogs showed no significant left/right bias (p = 0.19 and no significant difference was observed in frequencies between the sexes (p = 0.18. Penetrance of deafness was estimated as 0.72. Testing the association of red/blue coat colour and deafness without accounting for pedigree structure showed that red dogs were 1.8 times more likely to be deaf (p = 0.045. The within family association between red/blue coat colour and deafness was strongly significant (p = 0.00036, with red coat colour segregating more frequently with deafness (COR = 0.48. The relationship between deafness and coat speckling approached significance (p = 0.07, with the lack of statistical significance possibly due to only four families co-segregating for both deafness and speckling. The deafness phenotype was mapped to CFA10 (maximum linkage peak on CFA10 -log10 p

  13. Congenital sensorineural deafness in Australian stumpy-tail cattle dogs is an autosomal recessive trait that maps to CFA10.

    Science.gov (United States)

    Sommerlad, Susan; McRae, Allan F; McDonald, Brenda; Johnstone, Isobel; Cuttell, Leigh; Seddon, Jennifer M; O'Leary, Caroline A

    2010-10-12

    Congenital sensorineural deafness is an inherited condition found in many dog breeds, including Australian Stumpy-tail Cattle Dogs (ASCD). This deafness is evident in young pups and may affect one ear (unilateral) or both ears (bilateral). The genetic locus/loci involved is unknown for all dog breeds. The aims of this study were to determine incidence, inheritance mechanism, and possible association of congenital sensorineural deafness with coat colour in ASCD and to identify the genetic locus underpinning this disease. A total of 315 ASCD were tested for sensorineural deafness using the brain stem auditory evoked response (BAER) test. Disease penetrance was estimated directly, using the ratio of unilaterally to bilaterally deaf dogs, and segregation analysis was performed using Mendel. A complete genome screen was undertaken using 325 microsatellites spread throughout the genome, on a pedigree of 50 BAER tested ASCD in which deafness was segregating. Fifty-six dogs (17.8%) were deaf, with 17 bilaterally and 39 unilaterally deaf. Unilaterally deaf dogs showed no significant left/right bias (p = 0.19) and no significant difference was observed in frequencies between the sexes (p = 0.18). Penetrance of deafness was estimated as 0.72. Testing the association of red/blue coat colour and deafness without accounting for pedigree structure showed that red dogs were 1.8 times more likely to be deaf (p = 0.045). The within family association between red/blue coat colour and deafness was strongly significant (p = 0.00036), with red coat colour segregating more frequently with deafness (COR = 0.48). The relationship between deafness and coat speckling approached significance (p = 0.07), with the lack of statistical significance possibly due to only four families co-segregating for both deafness and speckling. The deafness phenotype was mapped to CFA10 (maximum linkage peak on CFA10 -log10 p-value = 3.64), as was both coat colour and speckling. Fine

  14. The effect of hearing aids and frequency modulation technology on results from the communication profile for the hearing impaired.

    Science.gov (United States)

    Lewis, M Samantha; Valente, Michael; Horn, Jane Enrietto; Crandell, Carl

    2005-04-01

    Hearing impairment has been associated with decline in psychosocial function. Previous investigations have reported that the utilization of hearing aids can ameliorate these reductions in psychosocial function. To date, few investigations have examined the effects of frequency modulation technology on hearing handicap, adjustment to hearing loss, and communicative strategies. The purpose of this investigation was to examine these effects and to compare them to the benefits obtained when using hearing aids alone. Subjects ranged in age from 34 to 81 years and had mean pure-tone thresholds consistent with a bilateral moderate to severe sloping sensorineural hearing loss. All subjects wore hearing aids only and hearing aids plus FM system in a randomized fashion. The Communication Profile for the Hearing Impaired (CPHI) was administered prior to fitting the study devices and once a month for three months in each of the two conditions. A statistically significant difference between device conditions was obtained for the Importance of Communication in Work Situations subscale. Additionally, statistically significant differences over time were noted in several CPHI subscales. Despite statistical significance, none of these results were clinically significant. The implications of these results will be discussed.

  15. Evaluation of Speech Intelligibility and Sound Localization Abilities with Hearing Aids Using Binaural Wireless Technology.

    Science.gov (United States)

    Ibrahim, Iman; Parsa, Vijay; Macpherson, Ewan; Cheesman, Margaret

    2013-01-02

    Wireless synchronization of the digital signal processing (DSP) features between two hearing aids in a bilateral hearing aid fitting is a fairly new technology. This technology is expected to preserve the differences in time and intensity between the two ears by co-ordinating the bilateral DSP features such as multichannel compression, noise reduction, and adaptive directionality. The purpose of this study was to evaluate the benefits of wireless communication as implemented in two commercially available hearing aids. More specifically, this study measured speech intelligibility and sound localization abilities of normal hearing and hearing impaired listeners using bilateral hearing aids with wireless synchronization of multichannel Wide Dynamic Range Compression (WDRC). Twenty subjects participated; 8 had normal hearing and 12 had bilaterally symmetrical sensorineural hearing loss. Each individual completed the Hearing in Noise Test (HINT) and a sound localization test with two types of stimuli. No specific benefit from wireless WDRC synchronization was observed for the HINT; however, hearing impaired listeners had better localization with the wireless synchronization. Binaural wireless technology in hearing aids may improve localization abilities although the possible effect appears to be small at the initial fitting. With adaptation, the hearing aids with synchronized signal processing may lead to an improvement in localization and speech intelligibility. Further research is required to demonstrate the effect of adaptation to the hearing aids with synchronized signal processing on different aspects of auditory performance.

  16. Evaluation of speech intelligibility and sound localization abilities with hearing aids using binaural wireless technology

    Directory of Open Access Journals (Sweden)

    Iman Ibrahim

    2012-12-01

    Full Text Available Wireless synchronization of the digital signal processing (DSP features between two hearing aids in a bilateral hearing aid fitting is a fairly new technology. This technology is expected to preserve the differences in time and intensity between the two ears by co-ordinating the bilateral DSP features such as multichannel compression, noise reduction, and adaptive directionality. The purpose of this study was to evaluate the benefits of wireless communication as implemented in two commercially available hearing aids. More specifically, this study measured speech intelligibility and sound localization abilities of normal hearing and hearing impaired listeners using bilateral hearing aids with wireless synchronization of multichannel Wide Dynamic Range Compression (WDRC. Twenty subjects participated; 8 had normal hearing and 12 had bilaterally symmetrical sensorineural hearing loss. Each individual completed the Hearing in Noise Test (HINT and a sound localization test with two types of stimuli. No specific benefit from wireless WDRC synchronization was observed for the HINT; however, hearing impaired listeners had better localization with the wireless synchronization. Binaural wireless technology in hearing aids may improve localization abilities although the possible effect appears to be small at the initial fitting. With adaptation, the hearing aids with synchronized signal processing may lead to an improvement in localization and speech intelligibility. Further research is required to demonstrate the effect of adaptation to the hearing aids with synchronized signal processing on different aspects of auditory performance.

  17. Evaluation of speech intelligibility and sound localization abilities with hearing aids using binaural wireless technology

    Directory of Open Access Journals (Sweden)

    Iman Ibrahim

    2013-01-01

    Full Text Available Wireless synchronization of the digital signal processing (DSP features between two hearing aids in a bilateral hearing aid fitting is a fairly new technology. This technology is expected to preserve the differences in time and intensity between the two ears by co-ordinating the bilateral DSP features such as multichannel compression, noise reduction, and adaptive directionality. The purpose of this study was to evaluate the benefits of wireless communication as implemented in two commercially available hearing aids. More specifically, this study measured speech intelligibility and sound localization abilities of normal hearing and hearing impaired listeners using bilateral hearing aids with wireless synchronization of multichannel Wide Dynamic Range Compression (WDRC. Twenty subjects participated; 8 had normal hearing and 12 had bilaterally symmetrical sensorineural hearing loss. Each individual completed the Hearing in Noise Test (HINT and a sound localization test with two types of stimuli. No specific benefit from wireless WDRC synchronization was observed for the HINT; however, hearing impaired listeners had better localization with the wireless synchronization. Binaural wireless technology in hearing aids may improve localization abilities although the possible effect appears to be small at the initial fitting. With adaptation, the hearing aids with synchronized signal processing may lead to an improvement in localization and speech intelligibility. Further research is required to demonstrate the effect of adaptation to the hearing aids with synchronized signal processing on different aspects of auditory performance.

  18. Biotechnology in the Treatment of Sensorineural Hearing Loss: Foundations and Future of Hair Cell Regeneration

    Science.gov (United States)

    Parker, Mark A.

    2011-01-01

    Purpose: To provide an overview of the methodologies involved in the field of hair cell regeneration. First, the author provides a tutorial on the biotechnological foundations of this field to assist the reader in the comprehension and interpretation of the research involved in hair cell regeneration. Next, the author presents a review of stem…

  19. Autozygosity mapping of autosomal recessive non-syndromic sensorineural hearing loss (ARNSSNHL)

    Energy Technology Data Exchange (ETDEWEB)

    Brown, K.A.; Nobel, A.; Markham, A.F. [Univ. of Leeds (United Kingdom)] [and others

    1994-09-01

    Congenital deafness affects about 1 in 2000 persons and is of genetic origin in approximately half these cases. The majority of congenital deafness is non-syndromic and over 75% of cases are compatible with autosomal recessive inheritance. Mapping of the loci responsible for ARNSSNHL will be complicated by genetic heterogeneity. Our approach to isolating genes involved in ARNSSNHL is by autozygosity mapping which involves the genetic analysis of children resulting from consanguineous marriages with the aim of identifying regions of homozygosity unique to the genomes of affected individuals which have been inherited from a common ancestor. The population employed in this study is the Pakistani community of Leeds, Bradford and Manchester in the UK which originated from the Mirpur region of Pakistan. Microsatellite analysis of the genome with markers spaced, on average, 10 cM apart is in progress and the investigation of 15 consanguineous families has identified one family which shows linkage to human chromosome 13q. This family appears to be linked to the same autosomal recessive deafness locus as two Tunisian families recently described and confirms that this chromosome 13q locus is also responsible, although as a minor contributor, to the deafness observed in the Pakistani population.

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

    NARCIS (Netherlands)

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

    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,

  1. NLRP3 mutation and cochlear autoinflammation cause syndromic and nonsyndromic hearing loss DFNA34 responsive to anakinra therapy

    Science.gov (United States)

    Nakanishi, Hiroshi; Kawashima, Yoshiyuki; Kurima, Kiyoto; Chae, Jae Jin; Ross, Astin M.; Pinto-Patarroyo, Gineth; Patel, Seema K.; Muskett, Julie A.; Ratay, Jessica S.; Chattaraj, Parna; Park, Yong Hwan; Grevich, Sriharsha; Brewer, Carmen C.; Hoa, Michael; Kim, H. Jeffrey; Broderick, Lori; Hoffman, Hal M.; Aksentijevich, Ivona; Kastner, Daniel L.; Goldbach-Mansky, Raphaela; Griffith, Andrew J.

    2017-01-01

    The NLRP3 inflammasome is an intracellular innate immune sensor that is expressed in immune cells, including monocytes and macrophages. Activation of the NLRP3 inflammasome leads to IL-1β secretion. Gain-of-function mutations of NLRP3 result in abnormal activation of the NLRP3 inflammasome, and cause the autosomal dominant systemic autoinflammatory disease spectrum, termed cryopyrin-associated periodic syndromes (CAPS). Here, we show that a missense mutation, p.Arg918Gln (c.2753G > A), of NLRP3 causes autosomal-dominant sensorineural hearing loss in two unrelated families. In family LMG446, hearing loss is accompanied by autoinflammatory signs and symptoms without serologic evidence of inflammation as part of an atypical CAPS phenotype and was reversed or improved by IL-1β blockade therapy. In family LMG113, hearing loss segregates without any other target-organ manifestations of CAPS. This observation led us to explore the possibility that resident macrophage/monocyte-like cells in the cochlea can mediate local autoinflammation via activation of the NLRP3 inflammasome. The NLRP3 inflammasome can indeed be activated in resident macrophage/monocyte-like cells in the mouse cochlea, resulting in secretion of IL-1β. This pathway could underlie treatable sensorineural hearing loss in DFNA34, CAPS, and possibly in a wide variety of hearing-loss disorders, such as sudden sensorineural hearing loss and Meniere’s disease that are elicited by pathogens and processes that stimulate innate immune responses within the cochlea. PMID:28847925

  2. Psychometric evaluation of a Swedish version of the communication strategies scale of the communication profile for the hearing impaired.

    Science.gov (United States)

    Hallberg, L R; Eriksson-Mangold, M; Carlsson, S G

    1992-06-01

    The Communication Strategies scale of the Communication Profile for the Hearing Impaired (CPHI) was translated into Swedish and used in several studies of people with hearing impairment (Hallberg & Carlsson, in press; Hallberg, Erlandsson, & Carlsson, 1991). In this study the scale was evaluated in terms of descriptive statistics, corrected item-total correlations, principal component analysis, and internal consistency reliability. Agreement with results from American studies is surprisingly good. Normative data based on three samples are presented: a general Swedish hearing-impaired sample with predominantly sensorineural hearing loss (N = 199), a subgroup of 105 younger subjects with noise-induced hearing loss, and a subgroup of 39 older subjects with sensorineural hearing loss due to heredity and/or old age. A significantly more frequent use of maladaptive behaviors (p less than .001) and verbal communication strategies (p less than .01) was reported by older subjects with age-related and/or hereditary hearing loss than by younger subjects with noise-induced hearing loss. The Communication Strategies scale seems to be an adequately reliable and clinically useful instrument for assessing adaptive and maladaptive strategies in hearing-impaired subjects.

  3. Working memory and intelligibility of hearing-aid processed speech.

    Science.gov (United States)

    Souza, Pamela E; Arehart, Kathryn H; Shen, Jing; Anderson, Melinda; Kates, James M

    2015-01-01

    Previous work suggested that individuals with low working memory capacity may be at a disadvantage in adverse listening environments, including situations with background noise or substantial modification of the acoustic signal. This study explored the relationship between patient factors (including working memory capacity) and intelligibility and quality of modified speech for older individuals with sensorineural hearing loss. The modification was created using a combination of hearing aid processing [wide-dynamic range compression (WDRC) and frequency compression (FC)] applied to sentences in multitalker babble. The extent of signal modification was quantified via an envelope fidelity index. We also explored the contribution of components of working memory by including measures of processing speed and executive function. We hypothesized that listeners with low working memory capacity would perform more poorly than those with high working memory capacity across all situations, and would also be differentially affected by high amounts of signal modification. Results showed a significant effect of working memory capacity for speech intelligibility, and an interaction between working memory, amount of hearing loss and signal modification. Signal modification was the major predictor of quality ratings. These data add to the literature on hearing-aid processing and working memory by suggesting that the working memory-intelligibility effects may be related to aggregate signal fidelity, rather than to the specific signal manipulation. They also suggest that for individuals with low working memory capacity, sensorineural loss may be most appropriately addressed with WDRC and/or FC parameters that maintain the fidelity of the signal envelope.

  4. Bone-Anchored Hearing Aid: patient satisfaction with the Cordelle device.

    Science.gov (United States)

    Ho, Eu Chin; Monksfield, Peter; Egan, Elizabeth; Reid, Andrew; Proops, David

    2009-09-01

    To assess patient satisfaction with the Bone-Anchored Hearing Aid (BAHA) Cordelle device and to identify any user- and device-related issues. Retrospective, anonymized, cross-sectional postal survey using the Entific Medical Systems questionnaire. Tertiary referral center. Fifty adult patients who had been using the BAHA Cordelle for more than 6 months. Hearing rehabilitation using BAHA Cordelle. Patients' satisfaction with the BAHA Cordelle in a variety of real-life hearing scenarios, their actual use of the device, and other issues with the device. Response rate was 66%. Responders were happy with their Cordelle in quiet situations and 1-to-1 conversations but less so in noisy or in group situations.However, approximately 80% of Cordelle users will use their device everyday for more than 8 h/d, and the overall satisfaction level is high. Compared with ear-level BAHA patients, Cordelle users are older and have mixed hearing loss with poorer sensorineural reserve, making hearing rehabilitation more challenging. The findings of this study support the use of the BAHA Cordelle as a viable, safe, low-morbidity hearing rehabilitation option in patients with mixed hearing loss with poor sensorineural reserve. Patients should be counseled appropriately to help manage their expectations.

  5. Prevalence of Internal Auditory Canal Diverticulum and Its Association with Hearing Loss and Otosclerosis.

    Science.gov (United States)

    Pippin, K J; Muelleman, T J; Hill, J; Leever, J; Staecker, H; Ledbetter, L N

    2017-11-01

    Focal low-attenuation outpouching or diverticulum at the anterolateral internal auditory canal is an uncommon finding on CT of the temporal bone. This finding has been described as cavitary otosclerosis in small case reports and histology series. The purpose of this study was to establish the prevalence of internal auditory canal diverticulum and its association with classic imaging findings of otosclerosis and/or hearing loss. Temporal bone CT scans of 807 patients, obtained between January 2013 and January 2016, were retrospectively reviewed to identify internal auditory canal diverticula and/or classic imaging findings of otosclerosis. Clinical evaluations for hearing loss were reviewed for patients with internal auditory canal diverticula and/or otosclerosis. Internal auditory canal diverticula were found in 43 patients (5%); classic otosclerosis, in 39 patients (5%); and both findings, in 7 patients (1%). Most temporal bones with only findings of internal auditory canal diverticula (91%) demonstrated hearing loss, with 63% of this group demonstrating sensorineural hearing loss. The hearing loss classification distribution was significantly different (P < .01) from that in the classic otosclerosis group and in the group with both diverticula and otosclerosis. Internal auditory canal diverticula are not uncommon on CT examinations of the temporal bone and most commonly occur without classic imaging findings of otosclerosis. These lesions are associated with sensorineural hearing loss, and referral for hearing evaluation may be appropriate when present. © 2017 by American Journal of Neuroradiology.

  6. [Hearing loss in attended patients in otorhinolaryngology service at IMSS in Guadalajara].

    Science.gov (United States)

    Chávez-Delgado, María Estela; Alvarez-Raygoza, Yolanda; Celis de la-Rosa, Alfredo; Virgen-Enciso, Maricela; Castro-Castañeda, Sergio

    2008-01-01

    to know the etiology, topography, degree of hearing loss and coexisting morbidity in outpatient otorhinolaryngology service at Instituto Mexicano del Seguro Social, Guadalajara, Jalisco. descriptive and transversal study of 393 patients older than 12 years, were studied during 12 months. They were sent to the otorhinolaryngology service from five hospitals in Guadalajara. Three population groups were made (adolescents, adults and elderly); medical history, otoscopy and conventional tone audiometry was carried out. the average age of patients was 51.2 (SD 4.2 years), 57.8 % were women and 58.5 % adults. Of the 786 examined ears, 91.9 % (95% CI = 89.8-93.7) had hearing loss; chronic suppurative otitis media with or without sensorineural factor, was the most common of hearing loss, affecting 25.4 % (95% CI = 22.3-28.7) of the 723 hearing loss ears; after mixed cochleopathies (21.8 %, 95% CI = 18.8-25). Sensorineural hearing loss occurred in 85.6 % (95% CI = 82.8-88) and 31 % (95% CI= 27.4-34.3) had moderate hearing loss. The most common coexisting morbidity were high blood pressure and diabetes mellitus. our data suggest that hearing loss in population study is a treatable pathology, this information will aid in the promotion of preventive measures for opportune detection of otitis and comorbidity and to implement detection campaigns in population of risk, schools and labor centers.

  7. Hearing handicap in adults with unilateral deafness and bilateral hearing loss.

    Science.gov (United States)

    Iwasaki, Satoshi; Sano, Hajime; Nishio, Shinya; Takumi, Yutaka; Okamoto, Makito; Usami, Shin-ichi; Ogawa, Kaoru

    2013-06-01

    To assess the perception of hearing handicap in adult patients with unilateral sudden sensorineural hearing loss (SNHL) compared with those with bilateral SNHL or unilateral congenital SNHL. Retrospective chart review. Multicenter department of otolaryngology referrals. Seventy-one subjects in the unilateral severe-profound (>70 dB) sudden SNHL group (Group 1), 17 subjects in the unilateral prelingual or congenital SNHL group (Group 2), and 121 subjects in the bilateral SNHL group (Group 3). Questionnaire. Hearing Handicap Inventory for Adults (HHIA) and visual analogue scale (VAS) measurements of hearing handicap. Average levels of hearing loss were 92 dB in Group 1, 109 dB in Group 2, and 67 dB in Group 3. The relative percentage scores of HHIA and VAS compared with Group 3 were 72.6% and 81.0% in Group 1 and 25.4% and 50.3% in Group 2, respectively. A mild correlation between the HHIA subscale or VAS scores and degree of hearing loss could be found in Group 3. No significant correlation was found between the HHIA subscale or VAS scores and duration of hearing loss in Group 1 or Group 3. Higher scores were obtained in male subjects than in female subjects. Patients in Group 1 who were troubled by tinnitus scored significantly higher in the HHIA. In multiple logistic regression analysis, presence of tinnitus, older age, higher average hearing loss level, and group (bilateral SNHL>unilateral sudden SNHL>unilateral precongenital SNHL) revealed a significant positive association with high score (>42) of HHIA (odds ratio, 3.171, 1.021, 1.031, and 6.690, respectively). The results of HHIA and VAS suggest that not only patients with bilateral SNHL but also those with unilateral sudden SNHL, particularly those who have tinnitus, experience a hearing handicap.

  8. Effect of hearing aid release time and presentation level on speech perception in noise in elderly individuals with hearing loss.

    Science.gov (United States)

    Pottackal Mathai, Jijo; Mohammed, Hasheem

    2017-02-01

    To investigate the effect of compression time settings and presentation levels on speech perception in noise for elderly individuals with hearing loss. To compare aided speech perception performance in these individuals with age-matched normal hearing subjects. Twenty (normal hearing) participants within the age range of 60-68 years and 20 (mild-to-moderate sensorineural hearing loss) in the age range of 60-70 years were randomly recruited for the study. In the former group, SNR-50 was determined using phonetically balanced sentences that were mixed with speech-shaped noise presented at the most comfortable level. In the SNHL group, aided SNR-50 was determined at three different presentation levels (40, 60, and 80 dB HL) after fitting binaural hearing aids that had different compression time settings (fast and slow). In the SNHL group, slow compression time settings showed significantly better SNR-50 compared to fast release time. In addition, the mean of SNR-50 in the SNHL group was comparable to normal hearing participants while using a slow release time. A hearing aid with slow compression time settings led to significantly better speech perception in noise, compared to that of a hearing aid that had fast compression time settings.

  9. Hearing loss in children with growth hormone deficiency.

    Science.gov (United States)

    Muus, John S; Weir, Forest W; Kreicher, Kathryn L; Bowlby, Deborah A; Discolo, Christopher M; Meyer, Ted A

    2017-09-01

    Although insulin-like growth factor 1 (IGF-1) has been shown to be important for inner-ear development in animal models, little is known about the otologic and audiologic findings of children with growth hormone deficiency (GHD). The goal of this study is to evaluate the prevalence, type, and severity of hearing impairment in children with GHD. Audiologic, otologic, and demographic data were recorded for children with a diagnosis of GHD in the AudGen database. Data for each patient were selected based on the first encounter with available complete audiometric data or the first encounter with a type of hearing loss documented. The patients were then stratified by type and severity of hearing loss, and otologic issues were documented. A separate cohort comprised of children with GHD without hearing loss was compared as a control. 209 children with GHD met inclusion criteria. 173 (83%) of these patients had hearing loss. 79% of losses were bilateral and 21% were unilateral (309 total ears with hearing loss). 293 of the 309 ears with hearing loss had audiograms with ear-specific thresholds; 47 had conductive, 24 had sensorineural, 65 had mixed and 157 had undefined hearing loss with incomplete audiograms. Pure-tone averages (PTA) were higher among patients with mixed hearing loss compared to patients with all other loss types. Hearing loss is prevalent in children with GHD with a predisposition to be bilateral. These findings suggest the need for increased awareness and routine hearing screening for patients with GHD. Further studies may elucidate the etiology of the hearing impairment in children with GHD to better aid pediatricians, endocrinologists, otolaryngologists and audiologists when assessing and managing these children. Copyright © 2017 Elsevier B.V. All rights reserved.

  10. Types of Hearing Aids

    Science.gov (United States)

    ... Consumer Devices Consumer Products Hearing Aids Types of Hearing Aids Share Tweet Linkedin Pin it More sharing ... are some features for hearing aids? What are hearing aids? Hearing aids are sound-amplifying devices designed ...

  11. Refinement of the locus for non-syndromic sensorineural deafness ...

    Indian Academy of Sciences (India)

    Unknown

    2004-04-05

    Bergstrom et al. 1971). Several X-linked forms of isolated bilateral hearing impairment have been reported and their classification is based on the mode of onset and audiogram data (Gorlin 1995). Genetically, the locus DFN1 had been ...

  12. Refinement of the locus for non-syndromic sensorineural deafness ...

    Indian Academy of Sciences (India)

    Non-syndromic X-linked deafness is a rare form of genetic deafness in humans accounting for a small proportion of all hereditary hearing loss. Different clinical forms of non-syndromic X-linked deafness have been described, and most of these have been mapped. Here, we report a Chinese family affected by a congenital ...

  13. Working Memory and Speech Recognition in Noise under Ecologically Relevant Listening Conditions: Effects of Visual Cues and Noise Type among Adults with Hearing Loss

    Science.gov (United States)

    Miller, Christi W.; Stewart, Erin K.; Wu, Yu-Hsiang; Bishop, Christopher; Bentler, Ruth A.; Tremblay, Kelly

    2017-01-01

    Purpose: This study evaluated the relationship between working memory (WM) and speech recognition in noise with different noise types as well as in the presence of visual cues. Method: Seventy-six adults with bilateral, mild to moderately severe sensorineural hearing loss (mean age: 69 years) participated. Using a cross-sectional design, 2…

  14. Hearing Aids

    Science.gov (United States)

    ... of hearing loss. Hearing loss can have a negative effect on communication, relationships, school/work performance, and emotional ... the President's Council of Advisors on Science and Technology (PCAST), and the ... in Effect Guidance Document: Conditions for Sale for Air-Conduction ...

  15. Lungfish Hearing

    DEFF Research Database (Denmark)

    Christensen, Christian Bech; Madsen, Peter Teglberg; Christensen-Dalsgaard, Jakob

    Recent research has shown that tympanic middle ears evolved independently in the major vertebrate groups and represent independent experiments in terrestrial hearing. Furthermore, the tympanic ear emerged quite late – ap - proximately 120 mya after the origin of the tetrapods and approximately 70...... my after the first truly terrestrial tetrapods emerged. One of the major challenges is to understand the transitional stages from tetrapod ancestors to the tympanic tetrapod ear, for example how a non-tympanic ear functions in terrestrial hearing. Lungfish are the closest living relatives...... and urodeles. Based on ABR and vibration measurements also on amphib - ians, lizards, snakes and alligators we can outline scenarios for the initial adaptations of the middle ear to non-tympanic hearing and assess the selection pressures later adapting the middle ear for tympanic hearing. Hearing by bone...

  16. A study on hearing threshold profile in traffic police personnel

    Directory of Open Access Journals (Sweden)

    Shelke BN, Aundhkar VG, Adgaonkar BD, Somwanshi SD, Gavkare AM, Ghuge SH

    2013-10-01

    Full Text Available Introduction: Noise is one of the causes of preventable sensori-neural loss. The traffic police personnel (TPP busy in controlling traffic at heavy traffic junctions suffer from the ill effects of noise and air pollution. Aim and objectives: The objective of this study was to assess the hearing threshold at various frequencies of the traffic police persons exposed to the vehicular noise and comparison with controls not exposed to noise. Material and methods: Thirty TPP and thirty controls were evaluated by clinical methods and subjected to the Pure Tone Audiometry (PTA in ENT department. Audiogram recorded by using conventional techniques in both ears. RESULTS: There was a significant difference in the hearing thresholds at frequency 2000 Hz, 4000 Hz and 8000 Hz of right and left ear between the two groups. Conclusion: This study concludes an increased risk of noise induced hearing loss (NIHL for the environmental noise exposed subjects.

  17. Neurologic Semiology In A Population Of Hearing Impaired Children [semiologia Neurológica Numa População De Crianças Deficientes Auditivas.

    OpenAIRE

    Goncalves V.M.; Piovesana A.M.; De Moura-Ribeiro M.V.

    1993-01-01

    A random sample of 42 sensorineural hearing impaired children (severe and bilateral) was studied, from special classes in Campinas, with chronological ages varying between 4 and 7 years old. The children of this sample were compared with two control groups of 42 children of the same chronological age, from regular classes of private and public schools. All of them were submitted to the traditional neurological examination. Hearing impaired children showed differences as to head circumference ...

  18. Benefits of active middle ear implants over hearing aids in patients with sloping high tone hearing loss: comparison with hearing aids.

    Science.gov (United States)

    Lee, J M; Jeon, J H; Moon, I S; Choi, J Y

    2017-06-01

    In this retrospective chart review we compared the subjective and objective benefits of active middle ear implants (AMEIs) with conventional hearing aids (HAs) in patients with sloping high tone hearing loss. Thirty-four patients with sensorineural hearing loss were treated with AMEIs. Of these, six had sloping high tone hearing loss and had worn an HA for more than 6 months. Objective assessments, a pure-tone audiogram, as well as a word recognition test, and the Korean version of the Hearing in Noise Test (K-HINT), and a subjective assessment, the Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire, were performed. Tests were conducted under three circumstances: 1) the unaided state before surgery; 2) the HA-aided state before surgery; and 3) the AMEI-aided state 3 months after surgery. The average high-frequency hearing gain (≥ 2 kHz) was significantly better with AMEIs than with HAs. Although the result had no statistical significance, AMEIs showed a superior word recognition score (WRS) compared to HAs. However, the most comfortable hearing level at which the WRS was tested was significantly decreased with an AMEI compared to an HA. In the K-HINT, patients with an AMEI showed greater recognition than those fitted with an HA under both quiet and noisy conditions. The APAHB scores revealed that patients were more satisfied with an AMEI rather than an HA on all subscales. The use of vibroplasty in patients with sloping high tone loss resulted in positive hearing outcomes when compared to conventional HAs. Based on the data from this study, AMEIs provided better objective and subjective results and could, therefore, be a better alternative for the treatment of sloping hearing loss. © Copyright by Società Italiana di Otorinolaringologia e Chirurgia Cervico-Facciale, Rome, Italy.

  19. Clinical need for a Baha trial in patients with single-sided sensorineural deafness. Analysis of a Baha database of 196 patients.

    Science.gov (United States)

    Desmet, Jolien; Bouzegta, Rajae; Hofkens, Anouk; De Backer, Annemarie; Lambrechts, Peggy; Wouters, Kristien; Claes, Jozef; De Bodt, Marc; Van de Heyning, Paul

    2012-03-01

    A bone-anchored hearing aid (Baha) is used in patients with single-sided sensorineural deafness (SSD) to overcome the head shadow effect. Of all the patients with SSD, treated at our hospital, 196 patients used a Baha on trial between November 2001 and April 2010. The objective of this study is to evaluate what factors determine the decision of a SSD patient whether or not to opt for a Baha device following a Baha trial period. 196 patients with SSD were enrolled for a trial period of 2 weeks at the Antwerp University Hospital, a tertiary referral centre. 93% of these patients suffered from an acquired hearing loss. 44% of all the patients (87/196) chose to wear a Baha device after the trail period, either on an abutment or on a headband. The collected data were analysed to determine correlations between the decision of a patient following a Baha trial period on the one hand, and Fletcher Index ipsi- and contralaterally, bone conduction hearing thresholds at the better hearing ear, aetiology, age at the start of the trial period, duration of hearing loss at the start of the trial period and the type of device used during the trial period, on the other hand. Although 66% of all the patients (109/196) declined the Baha after a trial, reasons not to choose a Baha were diverse and no crucial factors could be found that determine the success of a Baha trial period. Lack of improvement concerning speech understanding in noise was the most important reason mentioned by patients who declined the Baha. The authors advocate that all patients, suffering from SSD, should be offered the opportunity to try a Baha device as no factors could be found that determine the decision of a patient following the trial period.

  20. Auditory function and hearing loss in children and adults with Williams syndrome: cochlear impairment in individuals with otherwise normal hearing.

    Science.gov (United States)

    Marler, Jeffrey A; Sitcovsky, Jessica L; Mervis, Carolyn B; Kistler, Doris J; Wightman, Frederic L

    2010-05-15

    Hearing loss is common in school-age individuals with Williams syndrome (WS) and extensive in adults. Prior studies with relatively small sample sizes suggest that hearing loss in WS has an early onset and may be progressive, yet the auditory phenotype and the scope of the hearing loss have not been adequately characterized. We used standard audiometric tools: Otoscopy, tympanometry, air-conduction (bone conduction when available) behavioral testing, and distortion product otoacoustic emissions (DPOAEs) to measure hearing sensitivity and outer hair cell function. We tested 81 individuals with WS aged 5.33-59.50 years. Sixty-three percent of the school-age and 92% of the adult participants had mild to moderately-severe hearing loss. The hearing loss in at least 50% was sensorineural. DPOAE testing corroborated behavioral results. Strikingly, 12 of 14 participants with hearing within normal limits bilaterally had 4,000-Hz DPOAE input/output (DPOAE IO) functions indicative of outer hair cell damage and impaired cochlear compression. Our results indicate that hearing loss is very common in WS. Furthermore, individuals with WS who have "normal" hearing as defined by behavioral thresholds may actually have sub-clinical impairments or undetected cochlear pathology. Our findings suggest outer hair cell dysfunction in otherwise normal hearing individuals. The DPOAE IO in this same group revealed growth functions typically seen in groups with noise-induced damage. Given this pattern of findings, individuals with WS may be at increased risk of noise-induced hearing loss. Recommendations regarding audiological testing for individuals with WS and accommodations for these individuals in both academic and nonacademic settings are provided.

  1. PREVALENCE OF NOISE-INDUCED HEARING LOSS POLICE PERSONNEL COMING FOR HEALTH CHECKUP AT GOVERNMENT THENI MEDICAL COLLEGE AND HOSPITALS

    OpenAIRE

    Indra Thirugnanam; Arul Sundaresh Kumar; Padmanabam; Valarmathi; Ramesh

    2017-01-01

    BACKGROUND Occupational settings and transport is the prominent sources of noise that affect health. Noise-Induced Hearing Loss (NIHL) is sensory neural hearing loss due to exposure to intense impulse or continuous sound. Exposure to noise can be occupational or non-occupational. The audiologic profile of NIHL is the presence of sensorineural hearing loss that is most pronounced in the high-frequency region between 3,000 Hz and 6,000 Hz of the audiogram and the greatest amount of ...

  2. Objective estimation of frequency-specific pure-tone hearing thresholds following bone-conduction hearing aid stimulation.

    Science.gov (United States)

    Rahne, Torsten; Ehelebe, Thomas

    2014-01-01

    Patients suffering from conductive or mixed hearing loss may benefit from bone-conduction hearing systems (BAHS). The amount of amplification provided by the hearing system is selected based on the individual's sensorineural frequency-specific threshold. With patients who are not able to provide thresholds behaviorally, such as young children, objective methods are required to estimate the unaided and aided hearing threshold and thus the success of the hearing system fitting. In a prospective study with ten adult Baha softband users, aided and unaided frequency-specific thresholds were estimated. Aided thresholds to tone bursts via Baha stimulation were obtained behaviorally and electrophysiologically using cortical auditory evoked potentials (CAEPs) and were compared to pure-tone thresholds using routine clinical audiometry. For all stimulation frequencies, the frequency-specific electrophysiological and behavioral hearing thresholds measured with Baha stimulation were highly correlated and not different. Increased thresholds were observed only with the 0.5 kHz Baha stimulation as compared to the pure-tone audiogram. Objective measurement of frequency-specific hearing thresholds with CAEPs is applicable to BAHS users.

  3. Objective Estimation of Frequency-Specific Pure-Tone Hearing Thresholds following Bone-Conduction Hearing Aid Stimulation

    Directory of Open Access Journals (Sweden)

    Torsten Rahne

    2014-01-01

    Full Text Available Patients suffering from conductive or mixed hearing loss may benefit from bone-conduction hearing systems (BAHS. The amount of amplification provided by the hearing system is selected based on the individual’s sensorineural frequency-specific threshold. With patients who are not able to provide thresholds behaviorally, such as young children, objective methods are required to estimate the unaided and aided hearing threshold and thus the success of the hearing system fitting. In a prospective study with ten adult Baha softband users, aided and unaided frequency-specific thresholds were estimated. Aided thresholds to tone bursts via Baha stimulation were obtained behaviorally and electrophysiologically using cortical auditory evoked potentials (CAEPs and were compared to pure-tone thresholds using routine clinical audiometry. For all stimulation frequencies, the frequency-specific electrophysiological and behavioral hearing thresholds measured with Baha stimulation were highly correlated and not different. Increased thresholds were observed only with the 0.5 kHz Baha stimulation as compared to the pure-tone audiogram. Objective measurement of frequency-specific hearing thresholds with CAEPs is applicable to BAHS users.

  4. Examination of Hearing in a Rheumatoid Arthritis Population: Role of Extended-High-Frequency Audiometry in the Diagnosis of Subclinical Involvement

    Directory of Open Access Journals (Sweden)

    Mar Lasso de la Vega

    2016-01-01

    Full Text Available Objective. The aim of this study is to analyze the high-frequency hearing levels in patients with rheumatoid arthritis and to determine the relationship between hearing loss, disease duration, and immunological parameters. Materials and Methods. A descriptive cross-sectional study including fifty-three patients with rheumatoid arthritis was performed. The control group consisted of 71 age- and sex-matched patients from the study population (consecutively recruited in Madrid “Area 9,” from January 2010 to February 2011. Both a pure tone audiometry and an extended-high-frequency audiometry were performed. Results. Extended-high-frequency audiometry diagnosed sensorineural hearing loss in 69.8% of the patients which exceeded the results obtained with pure tone audiometry (43% of the patients. This study found significant correlations in patients with sensorineural hearing loss related to age, sex, and serum anti-cardiolipin (aCL antibody levels. Conclusion. Sensorineural hearing loss must be considered within the clinical context of rheumatoid arthritis. Our results demonstrated that an extended-high-frequency audiometry is a useful audiological test that must be performed within the diagnostic and follow-up testing of patients with rheumatoid arthritis, providing further insight into a disease-modifying treatment or a hearing loss preventive treatment.

  5. Examination of Hearing in a Rheumatoid Arthritis Population: Role of Extended-High-Frequency Audiometry in the Diagnosis of Subclinical Involvement.

    Science.gov (United States)

    Lasso de la Vega, Mar; Villarreal, Ithzel Maria; Lopez-Moya, Julio; Garcia-Berrocal, Jose Ramon

    2016-01-01

    Objective. The aim of this study is to analyze the high-frequency hearing levels in patients with rheumatoid arthritis and to determine the relationship between hearing loss, disease duration, and immunological parameters. Materials and Methods. A descriptive cross-sectional study including fifty-three patients with rheumatoid arthritis was performed. The control group consisted of 71 age- and sex-matched patients from the study population (consecutively recruited in Madrid "Area 9," from January 2010 to February 2011). Both a pure tone audiometry and an extended-high-frequency audiometry were performed. Results. Extended-high-frequency audiometry diagnosed sensorineural hearing loss in 69.8% of the patients which exceeded the results obtained with pure tone audiometry (43% of the patients). This study found significant correlations in patients with sensorineural hearing loss related to age, sex, and serum anti-cardiolipin (aCL) antibody levels. Conclusion. Sensorineural hearing loss must be considered within the clinical context of rheumatoid arthritis. Our results demonstrated that an extended-high-frequency audiometry is a useful audiological test that must be performed within the diagnostic and follow-up testing of patients with rheumatoid arthritis, providing further insight into a disease-modifying treatment or a hearing loss preventive treatment.

  6. Examination of Hearing in a Rheumatoid Arthritis Population: Role of Extended-High-Frequency Audiometry in the Diagnosis of Subclinical Involvement

    Science.gov (United States)

    Lasso de la Vega, Mar; Villarreal, Ithzel Maria; Lopez-Moya, Julio; Garcia-Berrocal, Jose Ramon

    2016-01-01

    Objective. The aim of this study is to analyze the high-frequency hearing levels in patients with rheumatoid arthritis and to determine the relationship between hearing loss, disease duration, and immunological parameters. Materials and Methods. A descriptive cross-sectional study including fifty-three patients with rheumatoid arthritis was performed. The control group consisted of 71 age- and sex-matched patients from the study population (consecutively recruited in Madrid “Area 9,” from January 2010 to February 2011). Both a pure tone audiometry and an extended-high-frequency audiometry were performed. Results. Extended-high-frequency audiometry diagnosed sensorineural hearing loss in 69.8% of the patients which exceeded the results obtained with pure tone audiometry (43% of the patients). This study found significant correlations in patients with sensorineural hearing loss related to age, sex, and serum anti-cardiolipin (aCL) antibody levels. Conclusion. Sensorineural hearing loss must be considered within the clinical context of rheumatoid arthritis. Our results demonstrated that an extended-high-frequency audiometry is a useful audiological test that must be performed within the diagnostic and follow-up testing of patients with rheumatoid arthritis, providing further insight into a disease-modifying treatment or a hearing loss preventive treatment. PMID:27239375

  7. Consequences of a screening programme on the prevalence of congenital hereditary sensorineural deafness in the Australian Cattle Dog.

    Science.gov (United States)

    Sommerlad, S F; Morton, J M; Johnstone, I; O'Leary, C A; Seddon, J M

    2014-12-01

    Genetic disease testing programmes are used in domestic animal breeds to guide selective breeding with the aim of reducing disease prevalence. We assessed the change in the prevalence of canine congenital hereditary sensorineural deafness (CHSD) in litters of Australian Cattle Dogs following the introduction of a brainstem auditory evoked response (BAER) testing programme. We studied 608 pups from 122 litters from 10 breeding kennels. Despite 10 years of testing (1998-2008), no substantial reduction in prevalence of CHSD was evident in these 10 breeding kennels. Even for the subset of litters in which both parents were BAER tested as normal hearing (305 pups from 58 litters), there was no evidence of substantial reduction in prevalence. Odds ratios for CHSD in pups for each extra year since testing in the kennel commenced were 1.01 (95% CI, 0.88-1.17) and 1.03 (95% CI, 0.82-1.30) respectively for these populations. Amongst 284 dogs from 54 litters with extended pedigrees and both parents BAER-tested normal hearing, observed prevalences of CHSD were highest in pups with no BAER-tested normal grandparents (17% or 5/29) and lowest in pups with all four grandparents tested normal (0% or 0/9). In pups for which one, two and three grandparents tested negative, prevalences of CHSD were 12% (9/74), 9% (9/101) and 8% (6/71) respectively. Hence, testing programmes based on phenotypic screening may not lead to a substantial reduction in recessive genetic disease prevalence over the medium term, even when only tested normal parents are used. Exclusive breeding of litters in which both parents and all four grandparents are BAER-tested normal is expected to reduce CHSD prevalence in pups to the greatest extent over the long term. © 2014 Stichting International Foundation for Animal Genetics.

  8. Cochlear Implantation in Adults With Asymmetric Hearing Loss: Benefits of Bimodal Stimulation.

    Science.gov (United States)

    van Loon, Maarten Caspar; Smits, Cas; Smit, Conrad F; Hensen, Erik F; Merkus, Paul

    2017-07-01

    This study addresses the outcome of cochlear implantation in addition to hearing aid use in patients with asymmetric sensorineural hearing loss. Prospective longitudinal study. Tertiary referral center. Seven adults with asymmetric sensorineural hearing loss, i.e., less than 30% aided speech recognition in their worst hearing ear and 60 to 85% speech recognition in their best hearing ear. All patients had a postlingual onset of their hearing loss and less than 20 years of auditory deprivation of their worst hearing ear. Cochlear implantation in the functionally deaf ear. Speech recognition in quiet, speech recognition in noise, spatial speech recognition, localization abilities, music appreciation, and quality of life. Measurements were performed before cochlear implantation and 3, 6, and 12 months after cochlear implantation. Before cochlear implantation, the average speech recognition of the ear fitted with a hearing aid was 74%. Cochlear implantation eventually resulted in an average speech recognition of 75%. Bimodal stimulation yielded speech recognition scores of 82, 86, and 88% after 3, 6, and 12 months, respectively. At all time intervals, bimodal stimulation resulted in a significantly better speech recognition as compared with stimulation with only hearing aid or only cochlear implant (CI). Speech recognition in noise and spatial speech recognition significantly improved as well as the ability to localize sounds and the quality of life. This study demonstrated that patients are able to successfully integrate electrical stimulation with contralateral acoustic amplification and benefit from bimodal stimulation. Therefore, we think that cochlear implantation should be considered in this particular group of patients, even in the presence of substantial residual hearing on the contralateral side.

  9. Hearing Impairments

    Science.gov (United States)

    Cavender, Anna; Ladner, Richard E.

    For many people with hearing impairments, the degree of hearing loss is only a small aspect of their disability and does not necessarily determine the types of accessibility solutions or accommodations that may be required. For some people, the ability to adjust the audio volume may be sufficient. For others, translation to a signed language may be more appropriate. For still others, access to text alternatives may be the best solution. Because of these differences, it is important for researchers in Web accessibility to understand that people with hearing impairments may have very different cultural-linguistic traditions and personal backgrounds.

  10. Efficacy of hearing-aid based telephone strategies for listeners with moderate-to-severe hearing loss.

    Science.gov (United States)

    Picou, Erin M; Ricketts, Todd A

    2013-01-01

    Understanding speech over the telephone when listening in noisy environments may present a significant challenge for listeners with moderate-to-severe hearing loss. The purpose of this study was to compare speech recognition and subjective ratings across several hearing aid-based telephone listening strategies for individuals with moderate-to-severe sensorineural hearing loss. Speech recognition and subjective ratings were evaluated for a simulated telephone signal. The strategies evaluated included acoustic telephone, unilateral telecoil, unilateral wireless streaming, and bilateral wireless streaming. Participants were seated in a noisy room for all evaluations. Eighteen adults, aged 49-88 yr, with moderate-to-severe sensorineural hearing loss participated. Speech recognition scores on the Connected Speech Test were converted to rationalized arcsine units and analyzed using analysis of variance testing and Tukey post hoc analyses. Subjective ratings of ease and comfort were also analyzed in this manner. Speech recognition performance was poorest with acoustic coupling to the telephone and best with bilateral wireless routing. Telecoil coupling resulted in better speech recognition performance than acoustic coupling, but was significantly poorer than bilateral wireless routing. Furthermore, unilateral wireless routing and telecoil coupling generally led to similar speech recognition performance, except in lower-level background noise conditions, for which unilateral routing resulted in better performance than the telecoil. For people with moderate-to-severe sensorineural hearing loss, acoustic telephone listening with a hearing aid may not lead to acceptable performance in noise. Although unilateral routing options (telecoil and wireless streaming) improved performance, speech recognition performance and subjective ratings of ease and comfort were best when bilateral wireless routing was used. These results suggest that wireless routing is a potentially beneficial

  11. Hearing Impairment

    Science.gov (United States)

    ... Fitness Diseases & Conditions Infections Drugs & Alcohol School & Jobs Sports Expert Answers (Q&A) Staying Safe Videos for ... known as noise-induced hearing loss (NIHL) . Personal music players are among the chief culprits of NIHL ...

  12. Hearing Conservation

    Science.gov (United States)

    1995-01-01

    the federal standard. Footnote** See Title 29 Code of Federal Regulations (CFR) 1910.95 "Occupational Noise Exposure." (Back to text) | USDOL | CONTACT INFORMATION | DISCLAIMER | 15 of 15 OSHA 3074 - Hearing Conservation

  13. Effects of sound amplification in self-perception of tinnitus and hearing loss in the elderly

    Directory of Open Access Journals (Sweden)

    Tiago de Melo Araujo

    Full Text Available ABSTRACT OBJECTIVE: To determine the effect of the use of hearing aids in self-perception of tinnitus and hearing loss in the elderly. METHODS: A total of 24 elderly patients between 60 and 70 years of age with moderate-grade sensorineural hearing loss were evaluated and divided into two groups according to the presence or absence of tinnitus. All volunteers were fitted with binaural micro-channel hearing aids of the same brand and model and submitted to tests, scales, and questionnaires relevant to this topic. The evaluations were performed before and after one and three months of effective use of these hearing aids. RESULTS: Acoustic stimulation through the effective use of hearing aids caused a reduction in the perception of tinnitus sound intensity (especially in evaluations with the prosthesis on and in nuisance associated with this symptom and with hearing loss. In addition, all participants were satisfied with the use of hearing aids. CONCLUSION: The continuous use of hearing aids is beneficial for the treatment of tinnitus and hearing loss, bringing satisfaction to users.

  14. Middle ear and hearing disorders of schoolchildren aged 7-10 years in South Sinai, Egypt.

    Science.gov (United States)

    Yamamah, G; Mabrouk, A; Ghorab, E; Ahmady, M; Abdulsalam, H

    2012-03-01

    Hearing loss among schoolchildren in developing countries is reported to be a significant health problem. Data on child hearing loss in South Sinai, a remote governorate of Egypt, are lacking. Middle ear diseases and hearing impairment were assessed among 453 primary-school children aged 7-10 years in South Sinai (906 ears). Otoscopic examination, tympanometry and pure tone audiometry (PTA) were done. Ear disease was found in 27.5% of the ears examined. The commonest cause was secretory otitis media (10.8%), followed by occluded earwax (9.5%). Mild and moderate hearing loss affected 8.5% of the sample, while sensorineural hearing loss affected 2.4%; only 0.4% had moderate and severe hearing loss. Hearing impairment affects 19.3% of this age group in South Sinai. None of the children with hearing impairment had been previously diagnosed or was receiving treatment and support. Hearing and middle ear screening at schools is recommended for early detection and management of middle ear and hearing problems.

  15. Bone-anchored hearing aid (BAHA: indications, functional results, and comparison with reconstructive surgery of the ear

    Directory of Open Access Journals (Sweden)

    Bento, Ricardo Ferreira

    2012-01-01

    Full Text Available Introduction: The bone-anchored hearing aid (BAHA is a bone conduction hearing device that transmits sound directly into the inner ear. It is mainly used in patients with conductive hearing loss associated with aural atresia, but it is also used in those with mixed and sensorineural hearing loss. Goals: To review the main indications for BAHA, to analyze the audiometric results and its benefits for patients and compare them with other treatment modalities, and to compare the literature data with our sample of 13 patients. Method: The research was performed using a database covering works in English, Spanish, and Portuguese, with no limitations in the years when the procedures were performed. We compared the literature data with our results for the 13 patients who underwent BAHA implantation between the years 2000 and 2009. Results: Most of the studies showed that BAHA has great advantages over reconstructive surgery in terms of hearing results, complications, and disease recurrence. The postoperative results for our 13 patients were satisfactory and comparable with the results from the literature, with closure of the air-bone gap in 7 patients and achieving an air-bone gap of 10 dB in 6 patients. No postoperative complications were observed. Conclusion: BAHA is a better treatment option than reconstructive surgery for patients with bilateral deafness. It is a relatively simple surgical procedure with few complications and good hearing results. Recent studies have examined its use in conductive and unilateral sensorineural hearing loss.

  16. Bone-anchored hearing aid (BAHA): indications, functional results, and comparison with reconstructive surgery of the ear.

    Science.gov (United States)

    Bento, Ricardo Ferreira; Kiesewetter, Alessandra; Ikari, Liliane Satomi; Brito, Rubens

    2012-07-01

     The bone-anchored hearing aid (BAHA) is a bone conduction hearing device that transmits sound directly into the inner ear. It is mainly used in patients with conductive hearing loss associated with aural atresia, but it is also used in those with mixed and sensorineural hearing loss.  To review the main indications for BAHA, to analyze the audiometric results and its benefits for patients and compare them with other treatment modalities, and to compare the literature data with our sample of 13 patients.  The research was performed using a database covering works in English, Spanish, and Portuguese, with no limitations in the years when the procedures were performed. We compared the literature data with our results for the 13 patients who underwent BAHA implantation between the years 2000 and 2009.  Most of the studies showed that BAHA has great advantages over reconstructive surgery in terms of hearing results, complications, and disease recurrence. The postoperative results for our 13 patients were satisfactory and comparable with the results from the literature, with closure of the air-bone gap in 7 patients and achieving an air-bone gap of 10 dB in 6 patients. No postoperative complications were observed.  BAHA is a better treatment option than reconstructive surgery for patients with bilateral deafness. It is a relatively simple surgical procedure with few complications and good hearing results. Recent studies have examined its use in conductive and unilateral sensorineural hearing loss.

  17. Pattern of Noise Induced Hearing Loss and its Relation with Duration of Exposure in Traffic Police Personnel

    Directory of Open Access Journals (Sweden)

    Mrityunjay Gupta

    2015-06-01

    Full Text Available Background: Occupational noise exposure is a major and preventable cause of hearing loss. Traffic policemen are constantly exposed to noise pollution. Noise induced hearing loss (NIHL denotes a selective 4000 Hz hearing loss (unilateral or bilateral. Objective: To assess the prevalence pattern of NIHL and its relation with duration of exposure to noise in traffic policemen. Material and methods: Otoscopy, tuning fork tests and Pure Tone Audiometry (PTA were performed in 150 apparently healthy police personnel. Results: Rinne’s and Weber’s test were normal in all the subjects. Schwabach’s test showed normal bone conduction in normal subjects as well as those with NIHL. PTA revealed that 33 (22% of sample subjects were suffering from NIHL; 8 (5.3% had sensorineural hearing loss whereas 109 (72.7% performed normally in the test. None of the subjects with sensorineural hearing loss showed the characteristic notch at 4 kHz. Majority of the subjects (69.7% with NIHL had mild to moderate grades of hearing loss in both ears. No subject had profound hearing loss. Significant association was observed between NIHL and duration of exposure. Steps need to be taken at the level of employers as well as individuals to counter occupational noise threat.

  18. Hearing Loss in Children

    Science.gov (United States)

    ... Hearing Loss Homepage Facts Noise-Induced Hearing Loss Genetics of Hearing Loss Screening & Diagnosis Types of Hearing Loss About Sound Treatment & Intervention Services Learning Language Bacterial Meningitis Studies Data & Statistics EHDI Annual Data 2015 ...

  19. Genetics of Hearing Loss

    Science.gov (United States)

    ... Hearing Loss Homepage Facts Noise-Induced Hearing Loss Genetics of Hearing Loss Screening & Diagnosis Types of Hearing Loss About Sound Treatment & Intervention Services Learning Language Bacterial Meningitis Studies Data & Statistics EHDI Annual Data 2015 ...

  20. Managing Hearing Loss

    Science.gov (United States)

    ... page please turn JavaScript on. Feature: Hearing Loss Managing Hearing Loss Past Issues / Spring 2015 Table of ... not a cure. Read More "Hearing Loss" Articles Managing Hearing Loss / Symptoms, Devices, Prevention & Research / Screening Newborns / ...

  1. What's Hearing Loss?

    Science.gov (United States)

    ... Weight for Me? Your Teeth Heart Murmurs What's Hearing Loss? KidsHealth > For Kids > What's Hearing Loss? Print ... problem can also develop later in life. How Hearing Works To understand how and why hearing loss ...

  2. Processing the telephone speech signal for the hearing impaired.

    Science.gov (United States)

    Terry, M; Bright, K; Durian, M; Kepler, L; Sweetman, R; Grim, M

    1992-04-01

    Speech intelligibility scores from 16 subjects with sensorineural hearing loss were evaluated using a digitized version of the California Consonant Test that was presented via headphones through a 300 to 3000 Hz bandpass filter to simulate the telephone band. Each subject was tested with an unprocessed signal that was frequency-equalized to compensate for the individual's hearing loss, and a signal that was equalized and compressed by the use of a compressor compression technique. Subjects were tested at three sound pressure levels above a pure-tone average threshold for frequencies 1 and 2 kHz. Two digital signal processing techniques designed to compensate for high-frequency hearing loss were examined: frequency domain processing and time domain processing. Frequency domain involved modification of the short-term spectrum obtained through a fast Fourier transform, whereas time domain processing involved passing the signal through a bank of finite impulse response filters. Both techniques showed significant intelligibility improvements (15-30%). In a second experiment, 16 additional subjects with high-frequency hearing loss compared an amplified telephone signal to three processed signals: (1) 6 dB per octave emphasis; (2) a signal frequency equalized for their hearing loss; and (3) a signal that was equalized for their hearing loss and was compressed according to their uncomfortable loudness levels. Most subjects preferred the signal with the 6 dB per octave emphasis.

  3. Long-term versus short-term hearing aid benefit.

    Science.gov (United States)

    Surr, R K; Cord, M T; Walden, B E

    1998-06-01

    This study compared hearing aid benefit obtained 6 weeks and a minimum of 1 year after fitting to determine if changes occurred over time. Fifteen individuals with mild-to-moderate sensorineural hearing losses, who were successful users of linear amplification, were fitted binaurally with the Resound BT2 Personal Hearing System. These hearing aids are programmable in two frequency bands that provide wide dynamic range compression (WDRC) amplification. The manufacturer's recommended loudness growth in octave bands (LGOB) and audiogram programming algorithm and fitting procedures were used. Following an initial 6-week period and again following a minimum of 1 year of use, the Profile of Hearing Aid Benefit (PHAB) was administered. Similarly, speech recognition performance was tested using the Connected Speech Test (CST) in a six-talker speech babble at 50 dBA, +10 signal-to-noise (S/N); 60 dBA, +5 SNR; and 70 dBA, +2 SNR; and in quiet with a reverberation time of 0.78 seconds. Significant aided benefit was shown. These short-term benefit scores for the PHAB and CST were compared with those obtained after 1 year of full-time use. Results revealed no significant change in hearing aid benefit with long-term use, suggesting that a 6-week acclimatization period is sufficiently long for clinical trials of this type of WDRC amplification.

  4. [Hearing disorders in the children presenting with various chronic diseases].

    Science.gov (United States)

    Barilyak, V V; Tsygankova, E R; Meleshina, N A; Tishkova, I G; Barilyak, R Yu

    2015-01-01

    The objective of the present study was to improve the effectiveness of early diagnostics and prophylaxis of hearing disturbances in the children presenting with various chronic diseases. As is known, there are several dozens of neurologic disorders associated with hearing disorders. By way of example, there is a group of nephropathologies responsible for the loss of hearing in the children. Specifically, hearing impairment can be one of the symptoms of diabetes mellitus. The patients presenting with mucopolysaccharidosis make up a group at risk of hearing impairment. Sensorineural loss of hearing is widespread among the children presenting with coeliac disease. The protocols for the treatment of certain pathologies envisage the application of certain medications possessed of the cytotoxic activity, such as preparations for chemo- and radiotherapy or cytostatic agents that suppress cell proliferation when applied for the management of some autoimmune diseases. It is concluded that cooperation between health providers representing different medical disciplines may be instrumental in the organization of efficacious screening for the detection of children with severe chronic pathology.

  5. Incidence and relative risk of hearing disorders in professional musicians

    Science.gov (United States)

    Schink, Tania; Kreutz, Gunter; Busch, Veronika; Pigeot, Iris; Ahrens, Wolfgang

    2014-01-01

    Background Hearing disorders have been associated with occupational exposure to music. Musicians may benefit from non-amplified and low-intensity music, but may also have high risks of music-induced hearing loss. Aims To compare the incidence of hearing loss (HL) and its subentities in professional musicians with that in the general population. Methods We performed a historical cohort study among insurants between 19 and 66 years who were employed subject to social insurance contributions. The study was conducted with data from three German statutory health insurance providers covering the years 2004–2008 with about 7 million insurants. Incidence rates with 95% CIs of HL and the subentities noise-induced hearing loss (NIHL), conductive HL, sensorineural HL, conductive and sensorineural HL, as well as tinnitus were estimated stratified by age, sex and federal state. A Cox regression analysis was conducted to estimate adjusted HRs and two-sided 95% CIs for HL and its subentities. Results More than 3 million insurants were eligible, of whom 2227 were identified as professional musicians (0.07%). During the 4-year observation period, 283 697cases of HL were seen, 238 of them among professional musicians (0.08%), leading to an unadjusted incidence rate ratio of 1.27. The adjusted hazard ratio of musicians was 1.45 (95% CI 1.28 to 1.65) for HL and 3.61 (95% CI 1.81 to 7.20) for NIHL. Conclusions Professional musicians have a high risk of contracting hearing disorders. Use of already available prevention measures should reduce the incidence of HL in professional musicians. PMID:24790053

  6. [Hearing loss and airway problems in children with mucopolysaccharidoses].

    Science.gov (United States)

    Santos, Saturnino; López, Laura; González, Luis; Domínguez, M Jesús

    2011-01-01

    Mucopolysaccharidoses (MPS) are a group of systemic diseases characterised by a genetic deficiency of lysosomal enzymes that causes the accumulation of glycosaminoglycans in different tissues. The onset of symptoms usually occurs in early childhood, causing problems of otitis media, hearing loss and airway obstruction in the ENT area. Describing the audiological findings and airway pathology found in 9 children diagnosed as having MPS. A retrospective review was performed of the clinical and audiological findings, exploratory results and therapeutic ENT procedures for 9 children diagnosed with MPS in an ENT service at a tertiary paediatric public centre in the period 2007-2010. Subtypes found were 4 MPS type I, 2 moderate MPS type II, 1 severe MPS type II, 1 MPS type IV and 1 MPS type VI. All patients presented chronic middle ear effusions. A child developed mild bilateral sensorineural hearing loss; another case was diagnosed as mixed hearing loss. The remaining auditory pattern was moderate bilateral conductive hearing loss. Four patients showed secondary obstructive sleep apnoea/hypopnoea syndrome (OSAHS) due to Waldeyer ring hyperplasia; surgery could not be performed on one of them because of cervical spinal cord compression from mucopolysaccharide deposits. In 2 cases, there was OSAHS relapse. Children with MPS are at increased risk for developing sensorineural hearing loss. The OSAHS syndrome appears in greater proportion than in the general child population, and recurrences may occur more frequently after surgery. Such children can also be risk patients in airway management. Copyright © 2011 Elsevier España, S.L. All rights reserved.

  7. Acquisition of who-question comprehension in German children with hearing loss.

    Science.gov (United States)

    Wimmer, Eva; Rothweiler, Monika; Penke, Martina

    2017-05-01

    For children with sensorineural hearing loss the ability to understand wh-questions might be particularly challenging because they often have only restricted access to spoken language input during optimal periods of language acquisition. In previous research it has been suggested that this restricted input during critical stages in language acquisition might lead to syntactic deficits that persist into adolescence. In this study we want to pursue this issue by investigating the comprehension of wh-questions in German children with bilateral sensorineural hearing loss. We report results of a who-question comprehension task in a group of 21 3- to 4-year-old German hard-of-hearing children compared to a group of age-matched children with normal hearing. The group data and individual performance patterns suggest that the syntactic comprehension difficulties observed in some, but not all, of the children with hearing loss reflect a delay in the acquisition of who-question comprehension rather than a persistent syntactic deficit. Follow-up data elicited from a subgroup of children confirm this supposition. Copyright © 2017 Elsevier Inc. All rights reserved.

  8. Concept and Evaluation of a New Piezoelectric Transducer for an Implantable Middle Ear Hearing Device.

    Science.gov (United States)

    Liu, Houguang; Cheng, Jinlei; Yang, Jianhua; Rao, Zhushi; Cheng, Gang; Yang, Shanguo; Huang, Xinsheng; Wang, Mengli

    2017-11-02

    Implantable middle ear hearing devices (IMEHDs) have been developed as a new technology to overcome the limitations of conventional hearing aids. The piezoelectric cantilever transducers currently used in the IMEHDs have the advantages of low power consumption and ease of fabrication, but generate less high-frequency output. To address this problem, we proposed and designed a new piezoelectric transducer based on a piezoelectric stack for the IMEHD. This new transducer, attached to the incus body with a coupling rod, stimulates the ossicular chain in response to the expansion-and-contraction of its piezoelectric stack. To test its feasibility for hearing loss compensation, a bench testing of the transducer prototype and a temporal bone experiment were conducted, respectively. Bench testing results showed that the new transducer did have a broad frequency bandwidth. Besides, the transducer was found to have a low total harmonic distortion (<0.75%) in all frequencies, and small release time (1 ms). The temporal bone experiment further proved that the transducer has the capability to produce sufficient vibrations to compensate for severe sensorineural hearing loss, especially at high frequencies. This property benefits the treatment of the most common sloping high-frequency sensorineural hearing loss. To produce a 100 dB SPL equivalent sound pressure at 1 kHz, its power consumption is 0.49 mW, which is low enough for the transducer to be utilized in the IMEHD.

  9. Concept and Evaluation of a New Piezoelectric Transducer for an Implantable Middle Ear Hearing Device

    Directory of Open Access Journals (Sweden)

    Houguang Liu

    2017-11-01

    Full Text Available Implantable middle ear hearing devices (IMEHDs have been developed as a new technology to overcome the limitations of conventional hearing aids. The piezoelectric cantilever transducers currently used in the IMEHDs have the advantages of low power consumption and ease of fabrication, but generate less high-frequency output. To address this problem, we proposed and designed a new piezoelectric transducer based on a piezoelectric stack for the IMEHD. This new transducer, attached to the incus body with a coupling rod, stimulates the ossicular chain in response to the expansion-and-contraction of its piezoelectric stack. To test its feasibility for hearing loss compensation, a bench testing of the transducer prototype and a temporal bone experiment were conducted, respectively. Bench testing results showed that the new transducer did have a broad frequency bandwidth. Besides, the transducer was found to have a low total harmonic distortion (<0.75% in all frequencies, and small release time (1 ms. The temporal bone experiment further proved that the transducer has the capability to produce sufficient vibrations to compensate for severe sensorineural hearing loss, especially at high frequencies. This property benefits the treatment of the most common sloping high-frequency sensorineural hearing loss. To produce a 100 dB SPL equivalent sound pressure at 1 kHz, its power consumption is 0.49 mW, which is low enough for the transducer to be utilized in the IMEHD.

  10. Perioperative Hearing Evaluations for Patients Undergoing Tympanostomy Tube Placement.

    Science.gov (United States)

    Whittemore, Kenneth R; Dornan, Briana K; Dargie, Jenna M; Zhou, Guangwei

    2016-11-01

    Obtaining hearing thresholds is an important step in the evaluation of a child with otitis media because decreased hearing in the presence of a chronic middle ear effusion factors into the decision to place tympanostomy tubes (TTs). To provide evidence regarding appropriate use of perioperative hearing evaluations in conjunction with TTs. Case series with medical record review of all patients aged 0 to 24 years who received TTs at a tertiary pediatric care facility from June 1, 2010, through June 1, 2011. Medical records were abstracted by 1 researcher for surgical, audiometric, tympanometric, clinical, and patient demographic data. The data analysis was performed between December 1, 2014, and June 1, 2015. Audiometric data were examined to determine the number of patients with hearing loss preoperatively and postoperatively, with the intention to describe the population with no prior hypothesis regarding results. Of 2274 patients identified, 910 (40.0%) were female. Median (interquartile range) age at TT placement was 2.62 (1.48-4.94) years. A total of 1757 (77.3%) underwent audiometric evaluation preoperatively, 1742 (76.6%) postoperatively, and 1395 (61.3%) both preoperatively and postoperatively; 170 (7.5%) had no audiometric testing. Within 1 year after surgery, 271 (11.9%) of patients had evidence of nonfunctional tubes. Postoperatively, 19.9% (347 of the 1742 patients who received a postoperative evaluation) had hearing loss. In all, 89 (3.9%) patients had a permanent sensorineural hearing loss, and 15 (0.66%) had a persistent conductive hearing loss. A postoperative audiometric examination should be performed in children who have hearing loss when evaluated before TT placement to determine whether resolution of the hearing loss was obtained.

  11. HEARING LOSS IN DECOMPRESSION

    Science.gov (United States)

    hearing loss is often reported. Most of this can readily be attributed to the residual effects of repeated aerotitis media, and is not sudden. A review of the topic of sudden deafness reveals that every large hospital where such records have been published sees every month about 1-2 patients whose sudden deafness is not easily explained. Possible causes have been suggested: acute neuritis of the VIIIth nerve, virus infection, vascular accident, vasomotor neurosis, acoustic trauma at levels of noise not usually noxious, collagen disease, transient ischemia from violent

  12. Effect of day-case unilateral cochlear implantation in adults on general and disease-specific quality of life, postoperative complications and hearing results, tinnitus, vertigo and cost-effectiveness : Protocol for a randomised controlled trial

    NARCIS (Netherlands)

    Derks, Laura S M; Wegner, Inge; Smit, Adriana L.; Thomeer, Hans G X M; Topsakal, Vedat; Grolman, Wilko

    2016-01-01

    INTRODUCTION: Cochlear implantation is an increasingly common procedure in the treatment of severe to profound sensorineural hearing loss (SNHL) in children and adults. It is often performed as a day-case procedure. The major drive towards day-case surgery has been from a logistical, economical and

  13. [Hearing loss due to chemotherapy and radiation therapy in young children].

    Science.gov (United States)

    Gruss, Irit; Handzel, Ophir; Ingber, Sara; Beiser, Michael

    2012-01-01

    Modern treatment of pediatric cancer patients has improved survival and life expectancy. However, treatment is associated with significant side-effects, including hearing loss. Hearing impairment has an important impact on language, communication and social skills, as well as on academic performance in school. 1. Characterize hearing loss caused by treatment of pediatric cancer. 2. Define the impact of hearing loss on language development. 3. Describe techniques for intervention. A retrospective review of ten children averaging five years of age who were referred to MICHA, a center specializing in treating hearing impaired children. For each child, at least four hearing tests were obtained, various language acquisition tests administered and methods of interventions recorded. All the subjects had high-tone symmetric sensorineural hearing loss; at 4000 Hz it reached 80 dB HL. Low frequency tones were better preserved. Pure-tone thresholds were worse than speech reception thresholds. Hearing aids improved hearing thresholds. Nine out of the ten children experienced a delay in language acquisition. The treatment plan included: hearing aids, hearing training, intervention in language and communication skills, emotional support and preparation for elementary school. Hearing loss amongst pediatric cancer patients as a consequence of treatment is extremely common. Delays in acquisition of language skills are seen in the majority of these children. Pediatric cancer patients should be referred to a proper diagnostic and intervention program. They are likely to benefit from monitoring the hearing before, during and after treatment, and from interventions to improve their hearing, language and communication skills. In addition, audiometry and otoacoustic emissions should be used to identify early damage to the inner ear. Noise and further exposure to ototoxic medications should be avoided.

  14. Temporal integration of loudness in listeners with hearing losses of primarily cochlear origin

    DEFF Research Database (Denmark)

    Buus, Søren; Florentine, Mary; Poulsen, Torben

    1999-01-01

    To investigate how hearing loss of primarily cochlear origin affects the loudness of brief tones, loudness matches between 5- and 200-ms tones were obtained as a function of level for 15 listeners with cochlear impairments and for seven age-matched controls. Three frequencies, usually 0.5, 1, and 4......-frequency hearing losses (slopes >50 dB/octave) showed larger-than-normal maximal amounts of temporal integration (40 to 50 dB). This finding is consistent with the shallow loudness functions predicted by our excitation-pattern model for impaired listeners [, in Modeling Sensorineural Hearing Loss, edited by W....... Jesteadt (Erlbaum, Mahwah, NJ, 1997), pp. 187–198]. Loudness functions derived from impaired listeners' temporal-integration functions indicate that restoration of loudness in listeners with cochlear hearing loss usually will require the same gain whether the sound is short or long. ©1999 Acoustical...

  15. Telephone speech enhancement for hearing-impaired listeners using multi-channel compression

    Science.gov (United States)

    Natarajan, Harikrishna P.; Krishnamurthy, Ashok K.; Feth, Lawrence L.

    2002-05-01

    Elderly listeners with sensorineural hearing impairment usually have difficulty with telephone communication if they do not use a hearing aid or telephone amplifier. In many cases, these devices may not be readily available or maybe uncomfortable to use. We describe here an alternative strategy to enhance the speech by pre-processing the signal before sending it over the telephone line. The pre-processing is based on a multi-channel compression algorithm that modifies the critical band spectrum of the speech signal to bring it within the dynamic hearing range of the listener, while also compensating for the effects of the telephone line. We describe the algorithm and the results of a simulation study in which the Articulation Index (AI) is used to measure the intelligibility enhancement, and the Glasberg, Moore and Stone (1999) model is used to simulate the effects of the hearing loss.

  16. Improvements in speech perception with an experimental nonlinear frequency compression hearing device.

    Science.gov (United States)

    Simpson, Andrea; Hersbach, Adam A; McDermott, Hugh J

    2005-05-01

    The performance of an experimental frequency compression hearing device was evaluated using tests of speech understanding in quiet. The device compressed frequencies above a programmable cut-off, resulting in those parts of the input signal being shifted to lower frequencies. Below the cut-off, signals were amplified without frequency shifting. Subjects were experienced hearing aid users with moderate-to-severe sensorineural hearing loss and sloping audiograms. Their recognition of monosyllabic words was tested using the experimental device in comparison with conventional hearing aids. Of the 17 subjects, eight showed a significant score improvement (p frequencies by the experimental device in comparison with the conventional aids. However, a subsequent study found that increasing the high-frequency gain in the conventional aids did not produce equivalent perceptual benefits.

  17. Successful bone-anchored hearing aid implantation in a patient with osteogenesis imperfecta.

    Science.gov (United States)

    Coutinho, M B; Marques, C; Mendes, G J; Gonçalves, C

    2015-11-01

    To report a case of successful bone-anchored hearing aid implantation in an adult patient with type III osteogenesis imperfecta, which is commonly regarded as a contraindication to this procedure. A 45-year-old man with type III osteogenesis imperfecta presented with mixed hearing loss. There was a mild sensorineural component in both ears, with an air-bone gap between 45 and 50 dB HL. He was implanted with a bone-anchored hearing aid. The audiological outcome was good, with no complications and good implant stability (as measured by resonance frequency analysis). To our knowledge, this is the first recorded case of bone-anchored hearing aid implantation in a patient with osteogenesis imperfecta.

  18. Relationships Among Factors Relevant to Abdominal Fat and Age-Related Hearing Loss.

    Science.gov (United States)

    Lee, Young; Park, Mina

    2017-12-01

    Metabolic syndrome is related with abdominal fat and with age-related hearing loss (ARHL). In this study, we evaluated the association between a variety of factors relevant to abdominal fat (FRAs) and hearing thresholds. We reviewed retrospectively the medical records of 2,602 subjects aged over 40 years with symmetrical sensorineural hearing loss who underwent abdominal fat computed tomography (CT) scans. Univariate and multivariate linear regression analyses were used to demonstrate the association between each FRA and hearing thresholds at low and high frequencies. Four of 5 FRAs were associated with hearing thresholds at high frequencies in males. All FRAs examined showed a relationship with hearing thresholds at low frequencies in females. Diabetes mellitus (DM) among clinical factors and visceral adipose tissue (VAT) among the 5 FRAs were the most reflective of hearing thresholds in both males and females. We found that FRAs were associated with hearing loss with frequency specific characteristics according to sex and reinforced that DM and VAT is particularly an important role for hearing.

  19. Universal newborn hearing screening: preliminary experience at the University Hospital of Cagliari

    Directory of Open Access Journals (Sweden)

    Giulia Pinna

    2012-10-01

    Full Text Available Bilateral congenital or acquired sensorineural hearing loss is a pathological condition affecting 1-2 children per 1,000 live births; it represents a major issue in public health because its late identification can negatively affect speech and language development. The aim of hearing screening is to obtain diagnosis and management of hearing loss as soon as possible; in fact early diagnosis and treatment allow children with congenital hearing impairment to acquire adequate linguistic competence. The present study reports our preliminary experience in newborn hearing screening at Neonatology services of University of Cagliari (Italy. During the first semester of surveillance, between January 2012 and June 2012, hearing screening was performed on a total of 901 babies using two different methods, TEOAEs in healthy neonates and automated ABR in high-risk babies. All infants were screened prior to hospital discharge; in some cases, especially for preterm infants of Neonatal Intensive Care Unit and Puericulture Institute, the screening was performed after discharge, to achieve a possible better global and acoustic maturation; 5 cases of hearing impairment were found. In the present study the Authors confirmed that it is possible to start a universal hearing screening in a relatively short time reaching the percentages suggested by Joint Committee on Infant Hearing.

  20. Audiological evaluation and self-assessed hearing problems in subjects with single-sided congenital external ear malformations and associated conductive hearing loss.

    Science.gov (United States)

    Priwin, Claudia; Jönsson, Radi; Magnusson, Lennart; Hultcrantz, Malou; Granström, Gösta

    2007-04-01

    Previously, unilateral hearing impairment (UHI) has been considered of little consequence. However, a recent meta-analysis of children with UHI displayed educational and behavioural problems and possible delays of speech and language development. Further, patients with UHI consequently report hearing difficulties. Our study investigated hearing function, possible inner ear protection, and self-assessed hearing problems in 57 subjects aged between 3-80 years with single-sided congenital ear malformations and conductive UHI. Pure-tone thresholds and speech recognition (quiet, noise) were measured, and all patients completed a self-assessment questionnaire. Pure-tone thresholds corresponding to sensorineural function did not significantly differ between the normal (air conduction) and affected ear (bone conduction). However, speech recognition in both quiet and in noise was normal on the non-affected side but significantly worse on the malformed side. A moderate to high degree of self-assessed hearing problems were reported. In conclusion, hearing function in the affected ear was found to be subnormal in terms of supra threshold signal processing. Furthermore, a high degree of hearing difficulty was reported. Therefore, active treatment, surgery, or hearing amplification, might be considered.

  1. Sensorineural deafness, abnormal genitalia, synostosis of metacarpals and metatarsals 4 and 5, and mental retardation

    DEFF Research Database (Denmark)

    Mendioroz, Jacobo; Fernández-Toral, Joaquín; Suárez, Etelvina

    2005-01-01

    In 1988 Pfeiffer and Kapferer reported on a patient with sensorineural deafness, psychomotor delay, hypospadias, cerebral manifestations, and bilateral synostosis of the 4th and 5th metacarpals and metatarsals. Synostosis of the 4th and 5th metacarpals and metatarsals is a very rare defect that has...

  2. Development and analysis of a low-cost screening tool to identify and classify hearing loss in children: a proposal for developing countries

    Directory of Open Access Journals (Sweden)

    Alessandra Giannella Samelli

    2011-01-01

    Full Text Available OBJECTIVE: A lack of attention has been given to hearing health in primary care in developing countries. A strategy involving low-cost screening tools may fill the current gap in hearing health care provided to children. Therefore, it is necessary to establish and adopt lower-cost procedures that are accessible to underserved areas that lack other physical or human resources that would enable the identification of groups at risk for hearing loss. The aim of this study was to develop and analyze the efficacy of a low-cost screening tool to identify and classify hearing loss in children. METHODS: A total of 214 2-to-10 year-old children participated in this study. The study was conducted by providing a questionnaire to the parents and comparing the answers with the results of a complete audiological assessment. Receiver operating characteristic (ROC curves were constructed, and discriminant analysis techniques were used to classify each child based on the total score. RESULTS: We found conductive hearing loss in 39.3% of children, sensorineural hearing loss in 7.4% and normal hearing in 53.3%. The discriminant analysis technique provided the following classification rule for the total score on the questionnaire: 0 to 4 points - normal hearing; 5 to 7 points - conductive hearing loss; over 7 points - sensorineural hearing loss. CONCLUSION: Our results suggest that the questionnaire could be used as a screening tool to classify children with normal hearing or hearing loss and according to the type of hearing loss based on the total questionnaire score

  3. Hearing loss and cochlear damage in experimental pneumococcal meningitis, with special reference to the role of neutrophil granulytes

    DEFF Research Database (Denmark)

    Brandt, CT; Caye-Thomsen, P; Lund, SP

    2006-01-01

    of an augmented neutrophil response on the development of hearing loss and cochlear damage in a model of experimental pneumococcal meningitis in rats. Hearing loss and cochlear damage were assessed by distortion product oto-acoustic emissions (DPOAE), auditory brainstem response (ABR) and histopathology in rats...... infection. Pretreatment with G-CSF increased hearing loss 24 h after infection and on day 8 compared to untreated rats (Mann-Whitney, P = 0.012 and P = 0.013 respectively). The increased sensorineural hearing loss at day 8 was associated with significantly decreased spiral ganglion cell counts (P = 0...... pathology compared to controls. In conclusion, the inflammatory host reaction contributes significantly to the development of hearing loss in experimental meningitis....

  4. Performance of hearing skills in children with auditory neuropathy spectrum disorder using cochlear implant: a systematic review,

    Directory of Open Access Journals (Sweden)

    Nayara Freitas Fernandes

    2015-02-01

    Full Text Available Introduction: Currently, there are no doubts about the benefits of cochlear implants for the development of children with severe or profound hearing loss. However, there is still no consensus among researchers and professionals regarding the benefits for the improvement of hearing skills in children with auditory neuropathy spectrum disorder using cochlear implants. Objective: Review the available evidence in the literature to answer the following: "What is the performance of hearing skills in children with auditory neuropathy spectrum disorder using cochlear implants?" Methods: Systematic review of the literature through electronic database consultation, considering publications in the period 2002-2013. Results: Twenty-two studies met the criteria and were included in the systematic review. Conclusion: The analyzed studies demonstrated that after cochlear implant surgery, individuals with auditory neuropathy spectrum disorder improved their performance of hearing skills and had similar performance to that of children with sensorineural hearing loss using cochlear implant.

  5. Preferred Compression Threshold and Release Time in Quiet and Noisy Conditions for Elderly Korean Hearing Aid Users.

    Science.gov (United States)

    Han, Woojae; Lee, Junghak; Kim, Jinsook; Lee, Kyeongwon; Kim, Dongwook

    2017-09-01

    Listeners with sensorineural hearing loss want to compensate for their narrow dynamic range from appropriate compression functions of hearing aids. The present study aimed to determine which compression threshold and release time of nonlinear hearing aids might affect speech recognition and speech quality judgments. Ten elderly listeners with symmetrical moderate-to-severe sensorineural hearing loss participated in the study. To compare two compression threshold (31 dB SPL vs. 61 dB SPL) and two release time (50 ms vs. 500 ms) conditions, subjects' speech recognition was measured using the Korean version of the Hearing in Noise Test in both quiet and noisy situations. All subjects were also asked to rate the degree of speech quality in terms of loudness, sharpness, clarity, and background noise immediately after completing the speech recognition test. Although no significant difference emerged in speech recognition thresholds between the two compression thresholds in the quiet situation, the compression threshold of 31 dB SPL showed a lower (or better) signal-to-noise ratio of speech recognition compared to that of 61 dB SPL. The release times of 50 and 500 ms did not statistically differ in both quiet and noisy situations. Subjective categories were found to be louder and clearer in the 50 ms release time for quiet and noisy, respectively. We expect that these patterns of current results will apply for better-fitting protocol of elderly Korean hearing aid users.

  6. [Children's hearing behavior observations and high risk individual genetic screening for late-onset hearing loss early detection and intervention exploring a basic-level hospitals model].

    Science.gov (United States)

    Guo, Yilian; Zeng, Xiangli; Liu, Ting; Zou, Yudi; Ye, Yanchou

    2015-09-01

    To explore the methods to detect and intervene children's late-onset hearing loss early which are suitable for basic-level hospitals. Udiology and imaging diagnosis had been given to the children who passed the newborn hearing screening but showed auditory behavior disorders in the growth process, and individualized interventions were given according to the results of diagnosis. Seven children with high risk for hereditary deafness were sent to superior hospital and had molecular screening of common mutations of inherited deafness carried out, then corresponding prevention guidance and intervention were given to them. Fifty-two cases with late-onset hearing loss or verbal disorders were detected by auditory behavior observations,including 4 cases of auditory neuropathy, 4 cases of unilateral sensorineural deafness, 27 cases of secretory otitis media. 13 cases of bilateral sensorineural deafness and 4 cases of autism. Seven newborns with high risk of hereditary deafness were sent to the Third Affiliated Hospital of Sun Yat-Sen University and received molecular screening of common mutations of inherited deafness. One case with GJB2 compound heterozygous mutations was detected and followed up to 4 years old, he was found bilateral moderate hearing loss and accepted the hearing aids at 2 years old. Mitochondrial DNA 1555 a > G heterogeneity mutation in 2 cases and GJB2 235 delC single heterozygous mutations in 3 cases, no mutation in 1 case, all these 6 cases have been followed-up until now, their hearing are normal. Children's auditory behavior observations and the superior hospitals referral performing high risk individual screening for newborns with high risk for hereditary deafness can detect children's late-onset hearing loss in time, this model is suitable for basic-level hospitals.

  7. awuah 2 .pub

    African Journals Online (AJOL)

    User

    Keywords: Acute otitis media, hearing loss, tympanometry, Pure-tone Audiometry. INTRODUCTION. Middle ear infection is eminently amenable to treatment, and early effective management will prevent progression to Chronic Suppurative. Otitis Media and subsequent Cholesteatoma and Sensorineural hearing loss.

  8. Hearing Loss in Osteogenesis Imperfecta: Characteristics and Treatment Considerations

    Science.gov (United States)

    Pillion, Joseph P.; Vernick, David; Shapiro, Jay

    2011-01-01

    Osteogenesis imperfecta (OI) is the most common heritable disorder of connective tissue. It is associated with fractures following relatively minor injury, blue sclerae, dentinogenesis imperfecta, increased joint mobility, short stature, and hearing loss. Structures in the otic capsule and inner ear share in the histologic features common to other skeletal tissues. OI is due to mutations involving several genes, the most commonly involved are the COL1A1 or COL1A2 genes which are responsible for the synthesis of the proalpha-1 and proalpha-2 polypeptide chains that form the type I collagen triple helix. A genotype/phenotype relationship to hearing loss has not been established in OI. Hearing loss is commonly found in OI with prevalence rates ranging from 50 to 92% in some studies. Hearing loss in OI may be conductive, mixed, or sensorineural and is more common by the second or third decade. Treatment options such as hearing aids, stapes surgery, and cochlear implants are discussed. PMID:22567374

  9. The Sound of Silence: Mouse Models for Hearing Loss

    Directory of Open Access Journals (Sweden)

    Sumantra Chatterjee

    2011-01-01

    Full Text Available Sensorineural hearing loss is one of the most common disabilities in humans. It is estimated that about 278 million people worldwide have slight to extreme hearing loss in both ears, which results in an economic loss for the country and personal loss for the individual. It is thus critical to have a deeper understanding of the causes for hearing loss to better manage and treat the affected individuals. The mouse serves as an excellent model to study and recapitulate some of these phenotypes, identify new genes which cause deafness, and to study their roles in vivo and in detail. Mutant mice have been instrumental in elucidating the function and mechanisms of the inner ear. The development and morphogenesis of the inner ear from an ectodermal layer into distinct auditory and vestibular components depends on well-coordinated gene expression and well-orchestrated signaling cascades within the otic vesicle and interactions with surrounding layers of tissues. Any disruption in these pathways can lead to hearing impairment. This review takes a look at some of the genes and their corresponding mice mutants that have shed light on the mechanism governing hearing impairment (HI in humans.

  10. Working memory and intelligibility of hearing-aid processed speech

    Directory of Open Access Journals (Sweden)

    Pamela eSouza

    2015-05-01

    Full Text Available Previous work suggested that individuals with low working memory capacity may be at a disadvantage in adverse listening environments, including situations with background noise or substantial modification of the acoustic signal. This study explored the relationship between patient factors (including working memory capacity and intelligibility and quality of modified speech for older individuals with sensorineural hearing loss. The modification was created using a combination of hearing aid processing (wide-dynamic range compression and frequency compression applied to sentences in multitalker babble. The extent of signal modification was quantified via an envelope fidelity index. We also explored the contribution of components of working memory by including measures of processing speed and executive function. We hypothesized that listeners with low working memory capacity would perform more poorly than those with high working memory capacity across all situations, and would also be differentially affected by high amounts of signal modification. Results showed a significant effect of working memory capacity for speech intelligibility, and an interaction between working memory, amount of hearing loss and signal modification. Signal modification was the major predictor of quality ratings. These data add to the literature on hearing-aid processing and working memory by suggesting that the working memory-intelligibility effects may be related to aggregate signal fidelity, rather than on the specific signal manipulation. They also suggest that for individuals with low working memory capacity, sensorineural loss may be most appropriately addressed with wide-dynamic range compression and/or frequency compression parameters that maintain the fidelity of the signal envelope.

  11. Role of p19ink4d in the pathogenesis of hearing loss.

    Science.gov (United States)

    Lai, Ruosha; Li, Jingkun; Hu, Peng; Wen, Jie; Jie, Qing; Dong, Yunpeng; Peng, Tao; Liu, Xuezhong; Xie, Dinghua

    2015-01-01

    This study aimed to investigate the p19 expression in cisplatin-treated rats and the role of p19 in the degeneration of inner ear cells. It also searched for p19 gene alterations in patients with profound sensorineural deafness. P19ink4d is essential for the postmitotic maintenance of hair cells. It is presumed that a mutation in the functional homolog of p19 or a disturbance in its regulated expression can be the underlying cause of hearing loss. Experiments were conducted on male and female Sprague-Dawley rats (aged 6-7 weeks, 280-320 g) with thresholds of auditory brainstem responses hearing loss (SNHL) were recruited at the second Xiangya Hospital of Central South University between 2005 and 2013, and genomic DNA for deafness gene analysis was obtained from peripheral blood samples of the patients and their lineal relatives. It was found that the p19 expression increased over time in the inner ear cells after cisplatin administration, but the p19 mRNA and protein levels significantly decreased in rats with manifested hearing loss induced by cisplatin. However, no mutation existed within the coding exons of p19 in the patients with profound sensorineural deafness. To conclude, the results support the concept that p19 may play an important role in the ototoxic effects of cisplatin and is probably involved in the pathogenesis of hearing loss.

  12. AAO: Autoimmune and Autoinflammatory (Disease) in Otology: What is New in Immune-Mediated Hearing Loss.

    Science.gov (United States)

    Vambutas, Andrea; Pathak, Shresh

    2016-10-01

    Autoinflammatory diseases are a family of immune-mediated, rare diseases, some of which, exhibit sensorineural hearing loss (SNHL), suggesting potentially similar mechanisms of molecular pathogenesis between autoinflammatory-mediated hearing loss and autoimmune inner ear disease (AIED) may exist. The purpose of this review is to compare the clinical features of autoimmune and autoinflammatory diseases that affect hearing, discuss the limitations of our knowledge, and highlight potential new disease mechanisms and therapeutics. Pubmed Literature Review; Google Scholar Literature review. A focused comparison of AIED with a number of autoinflammatory diseases that manifest with sensorineural hearing loss was performed. The pathogenesis of these diseases is reviewed in the context of the innate and adaptive immune system, cytokine expression and genetic polymorphisms. AIED, since first described by Cogan and Lehnhardt and first clinically characterized by McCabe, has remained an enigmatic disease, with limited advances in both new diagnostics and new therapeutics. Since the discovery of autoinflammatory diseases, a number of systemic autoimmune diseases have either been re-classed as autoinflammatory diseases or identified to have features of autoinflammatory disease. AIED has clinical features of both autoimmune and autoinflammatory disease. It is critical that autoinflammatory diseases be correctly identified, as failure to do so may result in systemic amyloidosis and kidney damage.

  13. Comparison of linear gain and wide dynamic range compression hearing aid circuits II: aided loudness measures.

    Science.gov (United States)

    Jenstad, L M; Pumford, J; Seewald, R C; Cornelisse, L E

    2000-02-01

    The goal of this study was to test the theoretical advantages of a single-channel wide dynamic range compression (WDRC) circuit fitted using the DSL method for increased dynamic range and normalized loudness growth. Ten adolescents and young adults with moderate to severe sensorineural hearing loss were fitted monaurally with the Siemens Viva 2 Pro behind-the-ear instrument set to DSL 4.0 targets for both linear gain and WDRC processing. Threshold, upper limit of comfort and loudness growth were measured in the unaided, linear gain and WDRC conditions for warble tones, environmental sounds and speech. Twelve adult listeners with normal hearing also were tested monaurally in the unaided condition to provide normative data for comparison purposes. The WDRC hearing aid provided a greater input dynamic range than the linear circuit for all stimuli. The dynamic range was normalized for more subjects with the WDRC than the linear hearing aid. In addition, exponential loudness growth functions fitted to the loudness growth data showed that, on average, loudness growth was more normalized with the WDRC hearing aid fitted to DSL[i/o] targets than the linear hearing aid fitted to DSL[i/o] targets. WDRC processing, fitted using the DSL[i/o] method, has potential applications in hearing aid fittings for listeners with moderate to severe hearing loss because it provides an audible, comfortable and tolerable amplified signal across a wider range of inputs than linear gain processing, without the need for volume control adjustments.

  14. Hearing loss and speech perception in noise difficulties in Fanconi anemia.

    Science.gov (United States)

    Verheij, Emmy; Oomen, Karin P Q; Smetsers, Stephanie E; van Zanten, Gijsbert A; Speleman, Lucienne

    2017-10-01

    Fanconi anemia is a hereditary chromosomal instability disorder. Hearing loss and ear abnormalities are among the many manifestations reported in this disorder. In addition, Fanconi anemia patients often complain about hearing difficulties in situations with background noise (speech perception in noise difficulties). Our study aimed to describe the prevalence of hearing loss and speech perception in noise difficulties in Dutch Fanconi anemia patients. Retrospective chart review. A retrospective chart review was conducted at a Dutch tertiary care center. All patients with Fanconi anemia at clinical follow-up in our hospital were included. Medical files were reviewed to collect data on hearing loss and speech perception in noise difficulties. In total, 49 Fanconi anemia patients were included. Audiograms were available in 29 patients and showed hearing loss in 16 patients (55%). Conductive hearing loss was present in 24.1%, sensorineural in 20.7%, and mixed in 10.3%. A speech in noise test was performed in 17 patients; speech perception in noise was subnormal in nine patients (52.9%) and abnormal in two patients (11.7%). Hearing loss and speech perception in noise abnormalities are common in Fanconi anemia. Therefore, pure tone audiograms and speech in noise tests should be performed, preferably already at a young age, because hearing aids or assistive listening devices could be very valuable in developing language and communication skills. 4. Laryngoscope, 127:2358-2361, 2017. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

  15. Hearing rehabilitation with single-stage bilateral vibroplasty in a child with Franceschetti syndrome.

    Science.gov (United States)

    Sargsyan, Sona; Rahne, Torsten; Kösling, Sabrina; Eichler, Gerburg; Plontke, Stefan K

    2014-05-01

    Hearing is of utmost importance for normal speech and social development. Even children who have mild or unilateral permanent hearing loss may experience difficulties with understanding speech, as well as problems with educational and psycho-social development. The increasing advantages of middle-ear implant technologies are opening new perspectives for restoring hearing. Active middle-ear implants can be used in children and adolescents with hearing loss. In addition to the well-documented results for improving speech intelligibility and quality of hearing in sensorineural hearing loss active middle-ear implants are now successfully used in patients with conductive and mixed hearing loss. In this article we present a case of successful, single-stage vibroplasty, on the right side with the fixation of the FMT on the stapes and PORP CLiP vibroplasty on the left side in a 6-year-old girl with bilateral mixed hearing loss and multiple dyslalia associated with Franceschetti syndrome (mandibulofacial dysostosis). CT revealed bilateral middle-ear malformations as well as an atretic right and stenotic left external auditory canal. Due to craniofacial dysmorphia airway and (post)operative, management is significantly more difficult in patients with a Franceschetti syndrome which in this case favoured a single-stage bilateral procedure. No intra- or postoperative surgical complications were reported. The middle-ear implants were activated 4 weeks after surgery. In the audiological examination 6 months after surgery, the child showed 100% speech intelligibility with activated implants on each side.

  16. Effect of a Bluetooth-implemented hearing aid on speech recognition performance: subjective and objective measurement.

    Science.gov (United States)

    Kim, Min-Beom; Chung, Won-Ho; Choi, Jeesun; Hong, Sung Hwa; Cho, Yang-Sun; Park, Gyuseok; Lee, Sangmin

    2014-06-01

    The object was to evaluate speech perception improvement through Bluetooth-implemented hearing aids in hearing-impaired adults. Thirty subjects with bilateral symmetric moderate sensorineural hearing loss participated in this study. A Bluetooth-implemented hearing aid was fitted unilaterally in all study subjects. Objective speech recognition score and subjective satisfaction were measured with a Bluetooth-implemented hearing aid to replace the acoustic connection from either a cellular phone or a loudspeaker system. In each system, participants were assigned to 4 conditions: wireless speech signal transmission into hearing aid (wireless mode) in quiet or noisy environment and conventional speech signal transmission using external microphone of hearing aid (conventional mode) in quiet or noisy environment. Also, participants completed questionnaires to investigate subjective satisfaction. Both cellular phone and loudspeaker system situation, participants showed improvements in sentence and word recognition scores with wireless mode compared to conventional mode in both quiet and noise conditions (P Bluetooth-implemented hearing aids helped to improve subjective and objective speech recognition performances in quiet and noisy environments during the use of electronic audio devices.

  17. Is BPPV a Prognostic Factor in Idiopathic Sudden Sensory Hearing Loss?

    Science.gov (United States)

    Lee, No Hee; Ban, Jae Ho

    2010-12-01

    The prognostic significance of vertigo in patients with idiopathic sudden sensorineural hearing loss (SSNHL) remains a matter of debate because vertigo is associated with many different vestibular disorders. The purpose of this study is to determine the role of benign paroxysmal positional vertigo (BPPV) as a prognostic factor in patients with SSNHL. We conducted a retrospective study of 298 patients with SSNHL. Hearing outcomes were evaluated by assessments of pre-treatment hearing and hearing gain. Comparative multivariate analyses between prognostic factors and hearing outcome were conducted. Thirty-eight (12.7%) SSNHL patients were found to also have BPPV. BPPV showed significant negative prognostic factors in hearing outcome on multivariate analysis (odds ratio, 0.15). In comparison to average pure tone audiometry (PTA), patients diagnosed with SSNHL with BPPV exhibited poorer hearing in pre- and post-treatment PTA compared to SSNHL without BPPV. Old age (>60 years), pre-treatment hearing, and canal paresis were significant outcome predictors. BPPV in SSNHL patients, representing definitive vestibular damage, was closely related to poor prognosis.

  18. Development of a Shortened Version of the Spatial Hearing Questionnaire (SHQ-S) for Screening Spatial-Hearing Ability.

    Science.gov (United States)

    Ou, Hua; Perreau, Ann; Tyler, Richard S

    2017-09-18

    The Spatial Hearing Questionnaire (SHQ) was developed to address how to measure spatial-hearing ability in complex listening situations (Tyler, Perreau, & Ji, 2009). It has been translated and validated into various languages, including Chinese, Dutch, French, and Persian. Although the SHQ contains only 24 items, it could be time-consuming in a busy clinic to administer. The purposes of this study were to develop and validate a shortened version of the SHQ (SHQ-S) and to compare self-perceived spatial-hearing ability across adults with normal hearing (NH), hearing loss (HL), and cochlear implants (CIs). This was a retrospective study. The full version of the SHQ was administered to measure self-perceived spatial-hearing ability for 51 adults with NH at Augustana College, 47 adults with essentially mild to moderately severe sensorineural HL at Illinois State University, and 72 adult CI users at the University of Iowa. Exploratory factor analysis was performed for the full version for the data collected from adults with NH and HL. Appropriate items were chosen to develop the SHQ-S from the results of the exploratory factor analysis. Confirmatory factor analysis was then applied to test the factor structure of the SHQ-S for all participants. One-way analysis of variance was used to compare the self-perceived spatial-hearing performance scores between the 3 groups. The exploratory factor analysis revealed scores loaded on 2 factors. Six items from the full version were chosen accordingly. The results of the confirmatory factor analysis indicated that that a shortened version of 6 items is sufficient to measure spatial-hearing ability. The internal consistency reliability of the SHQ-S was high. The main effect of the one-way analysis of variance was significant for the groups, F(2, 167) = 36.0, p < .0001. The comparisons with the Tukey adjustment indicated that the NH group reported significantly better spatial-hearing ability than either the HL or the CI group (both

  19. Permanent Childhood Hearing Impairment: Aetiological Evaluation of Infants identified through the Irish Newborn Hearing Screening Programme

    LENUS (Irish Health Repository)

    Smith, A

    2017-11-01

    The Newborn Hearing Screening Programme (NHSP) was established in Cork University Maternity Hospital (CUMH) in April 2011. Between April 2011 and July 2014, 42 infants were identified with a Permanent Childhood Hearing Impairment (PCHI). Following this diagnosis, infants underwent a paediatric assessment according to recognised guidelines with the intention of identifying the underlying aetiology of the PCHI. The aim of this study was to assess the findings of this aetiological workup via retrospective chart review. PCHI data was obtained from the eSP database. This is a web based information system (eSP) used to track each baby through the screening and referral process A retrospective chart review of these patients was performed. Sixteen (38%) infants were diagnosed with a bilateral sensorineural hearing loss. Two infants had congenital CMV infection. A Connexin 26 gene mutation was detected in one infant. Two infants were diagnosed with Waardenburg syndrome, One with Pendred syndrome and one with Pfeiffer syndrome. Five babies underwent cochlear implantation. Through adherence to the recommended protocol a possible cause of PCHI may be determined. This study has identified areas of future improvement for this service in Ireland.

  20. Hearing rehabilitation in cerebral palsy: development of language and hearing after cochlear implantation

    Directory of Open Access Journals (Sweden)

    Anacléia Melo da Silva Hilgenberg

    2015-06-01

    Full Text Available INTRODUCTION: Auditory rehabilitation in children with bilateral severe-to-profound sensorineural hearing loss with cochlear implant has been developed in recent decades; however, the rehabilitation of children with cerebral palsy still remains a challenge to otolaryngology and speech therapy professionals. OBJECTIVE: To verify the effectiveness of cochlear implants in the development of auditory and language skills in children with cerebral palsy. METHODS: A prospective analytical study. The evaluation of auditory responses to speech test was applied to the children in this study at regular intervals following implantation. Standardized tests that assess and quantify the development of auditory and language skills were administered and speech therapy video records and speech therapy files were analyzed. All children went through individually tailored intensive audiological rehabilitation programs following cochlear implantation. RESULTS: Two participants had gradual auditory and language development when compared to other participants who reached advanced levels in hearing and oral language classifications. CONCLUSION: The use of the Cochlear implant enabled participants to reach advanced stages of hearing and language skills in three of the five participants with cerebral palsy in this study. This electronic device is a viable therapeutic option for children with cerebral palsy to help them achieve complex levels of auditory and language skills.