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Sample records for acute sensorineural hearing

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

    Directory of Open Access Journals (Sweden)

    Kim Dong-Min

    2009-10-01

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

  2. Congenital sensorineural hearing loss

    International Nuclear Information System (INIS)

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

    1984-01-01

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

  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 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.

  5. [Acute sensorineural hearing loss at the Otorhinolaryngology Department of the General Hospital in Subotica 1991-1996].

    Science.gov (United States)

    Rakić, N

    1999-01-01

    Sudden sensorineural hearing loss is an acute hearing loss of a perceptive type, of unknown etiology, in most cases one-sided, or, a every sudden sensorineural deafness that occurs in the period of three days or less. Modern scientific literature points to three possible etiological factors: viral cochleitis, vascular lesion of the labyrinth and unrecognized rupture of the membranous labyrinth. The therapy for this disease is extremely various. There are dozens of reported different (or similar) healing protocols, including vasodilators, Hydroxyethyl starch, low molecular dextran, diuretics, defibrinogenation, fibrmolytic therapy, steroids, prostacyclin, oxygen therapy, hyperbaric oxygen therapy, vitamins, etc. The aim of the study was to recapitulate in brief modern views of etiology and therapy of the sudden sensorineural hearing loss, to analyze cases of this illness within the period of 1991-1996 in our Otorhinolaryngology Department and to compare these data with the same or similar data of other authors. In this study, medical records of patients admitted in the period of 1991-1996 in the Otorhinolaryngology Department of the Health Center in Subotica with a diagnosis of sudden sensorineural hearing loss were used. There were 53 patients with this diagnosis. All patients were treated in the hospital with infusions of rheoactive drugs. During the diagnostic procedure, all patients underwent a complete clinical otorhinolaryngologic examination, detailed anamnesis was taken, acoustic impedance tests (tympanometry) and tonal liminal audiometry were performed. Routine blood test was made (including complete blood picture, sedimentation rate, blood sugar) and a complete urine examination. Sometimes we demanded x-ray of the temporal bones (Schnller and Stenwers). A short increment sensitivity index (SISI) test and Carhart test were also made, if necessary. Function of the vestibular system was tested in cases when the vestibular symptoms were more strongly expressed

  6. 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.

  7. Sensorineural hearing loss and prematurity

    OpenAIRE

    Marlow, E.; Hunt, L.; Marlow, N.

    2000-01-01

    OBJECTIVE—To elucidate clinical antecedents of sensorineural hearing loss (SNHL) in very preterm infants.
DESIGN—Case-control study.
SUBJECTS—Fifteen children < 33 weeks' gestation with significant SNHL born between 1 January 1990 and 31 December 1994, detected within 9 months of birth, and 30 matched control children.
METHODOLOGY—Perinatal variables in the two groups were compared using non-parametric tests and conditional logistic regression (EGRET).
RESULTS—Median birth ...

  8. Sensorineural Hearing Loss following Carbon Monoxide Poisoning

    Directory of Open Access Journals (Sweden)

    Joseph P. Pillion

    2012-01-01

    Full Text Available A case study is presented of a 17-year-old male who sustained an anoxic brain injury and sensorineural hearing loss secondary to carbon monoxide poisoning. Audiological data is presented showing a slightly asymmetrical hearing loss of sensorineural origin and mild-to-severe degree for both ears. Word recognition performance was fair to poor bilaterally for speech presented at normal conversational levels in quiet. Management considerations of the hearing loss are discussed.

  9. [Sensorineural hearing loss due to neonatal hyperbilirubinemia].

    Science.gov (United States)

    Clarós, P; Turcanu, D; Caballero, M; Costa, C; Clavería, M A; Clarós, A; Clarós, A

    2003-01-01

    In this article, the sensorineural hearing loss is presented as a possible sequelae of neonatal hyperbilirubinemia. In our program of early hipoacusia detection, 241 babies were examined from January 1996 until November 1999; 7 cases had a history of hyperbilirubinemia in the neonatal period and 2 of them were diagnosed of sensorineural hearing loss. We discuss how the bilirubin or any other associated factor might have been the cause and this could explain the selective affectation of some children.

  10. Sensorineural hearing loss after magnetic resonance imaging

    DEFF Research Database (Denmark)

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

    2013-01-01

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

  11. 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.

  12. Sudden Sensorineural Hearing Loss; Prognostic Factors

    OpenAIRE

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

    2015-01-01

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

  13. Gd enhanced MRI in sensorineural hearing loss

    International Nuclear Information System (INIS)

    Takenaka, Mika; Tono, Tetsuya; Toyama, Katsuhiro; Kano, Kiyo; Morimitsu, Tamotsu

    1996-01-01

    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)

  14. Sensorineural Hearing Loss after Magnetic Resonance Imaging

    Directory of Open Access Journals (Sweden)

    Abolfazl Mollasadeghi

    2013-01-01

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

  15. 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?

  16. Sensorineural hearing loss after magnetic resonance imaging

    DEFF Research Database (Denmark)

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

    2013-01-01

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

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

    OpenAIRE

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

    2014-01-01

    Sudden sensorineural hearing loss is characterized by acute, idiopathic hearing deterioration. We report here the development and evaluation of “constraint-induced sound therapy”, which is based on a well-established neuro-rehabilitation approach, and which is characterized by the plugging of the intact ear (“constraint”) and the simultaneous, extensive stimulation of the affected ear with music. The sudden sensorineural hearing loss patients who received the constraint-induced sound therapy ...

  18. Mobile phone induced sensorineural hearing loss

    International Nuclear Information System (INIS)

    Al-Dousary, Surayie H.

    2007-01-01

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

  19. Sudden Sensorineural Hearing Loss; Prognostic Factors

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    Arjun Dass

    2015-09-01

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

  20. 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.

  1. Relation between Glaucoma and Sensorineural Hearing Loss

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

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

    2018-02-01

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

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

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    Umberto Volta

    2009-01-01

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

  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. P300 in individuals with sensorineural hearing loss

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    Ana Cláudia Mirandola Barbosa Reis

    2015-04-01

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

  6. P300 in individuals with sensorineural hearing loss.

    Science.gov (United States)

    Reis, Ana Cláudia Mirandola Barbosa; Frizzo, Ana Claudia Figueiredo; Isaac, Myriam de Lima; Garcia, Cristiane Fregonesi Dutra; Funayama, Carolina Araújo Rodrigues; Iório, Maria Cecília Martinelli

    2015-01-01

    Behavioral and electrophysiological auditory evaluations contribute to the understanding of the auditory system and of the process of intervention. To study P300 in subjects with severe or profound sensorineural hearing loss. This was a descriptive cross-sectional prospective study. It included 29 individuals of both genders with severe or profound sensorineural hearing loss without other type of disorders, aged 11 to 42 years; all were assessed by behavioral audiological evaluation and auditory evoked potentials. A recording of the P3 wave was obtained in 17 individuals, with a mean latency of 326.97ms and mean amplitude of 3.76V. There were significant differences in latency in relation to age and in amplitude according to degree of hearing loss. There was a statistically significant association of the P300 results with the degrees of hearing loss (p=0.04), with the predominant auditory communication channels (p<0.0001), and with time of hearing loss. P300 can be recorded in individuals with severe and profound congenital sensorineural hearing loss; it may contribute to the understanding of cortical development and is a good predictor of the early intervention outcome. Copyright © 2014 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

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

    Science.gov (United States)

    Chroni, M; Prappa, E; Kokkevi, I

    2018-04-01

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

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

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    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.

  9. 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.

  10. [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.

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

    Science.gov (United States)

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

    2012-01-01

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

  12. Gipc3 mutations associated with audiogenic seizures and sensorineural hearing loss in mouse and human

    NARCIS (Netherlands)

    Charizopoulou, N.; Lelli, A.; Schraders, M.; Ray, K.; Hildebrand, M.S.; Ramesh, A.; Srisailapathy, C.R.; Oostrik, J.; Admiraal, R.J.C.; Neely, H.R.; Latoche, J.R.; Smith, R.J.; Northup, J.K.; Kremer, J.M.J.; Holt, J.R.; Noben-Trauth, K.

    2011-01-01

    Sensorineural hearing loss affects the quality of life and communication of millions of people, but the underlying molecular mechanisms remain elusive. Here, we identify mutations in Gipc3 underlying progressive sensorineural hearing loss (age-related hearing loss 5, ahl5) and audiogenic seizures

  13. 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

  14. Sensorineural Hearing Loss in Pseudoexfoliation Syndrome

    Directory of Open Access Journals (Sweden)

    Shahin Yazdani

    2008-12-01

    Full Text Available

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

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

    International Nuclear Information System (INIS)

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

    1989-01-01

    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∼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)

  16. 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 static ...

  17. 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...

  18. 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

  19. Speech recognition in individuals with sensorineural hearing loss.

    Science.gov (United States)

    de Andrade, Adriana Neves; Iorio, Maria Cecilia Martinelli; Gil, Daniela

    2016-01-01

    Hearing loss can negatively influence the communication performance of individuals, who should be evaluated with suitable material and in situations of listening close to those found in everyday life. To analyze and compare the performance of patients with mild-to-moderate sensorineural hearing loss in speech recognition tests carried out in silence and with noise, according to the variables ear (right and left) and type of stimulus presentation. The study included 19 right-handed individuals with mild-to-moderate symmetrical bilateral sensorineural hearing loss, submitted to the speech recognition test with words in different modalities and speech test with white noise and pictures. There was no significant difference between right and left ears in any of the tests. The mean number of correct responses in the speech recognition test with pictures, live voice, and recorded monosyllables was 97.1%, 85.9%, and 76.1%, respectively, whereas after the introduction of noise, the performance decreased to 72.6% accuracy. The best performances in the Speech Recognition Percentage Index were obtained using monosyllabic stimuli, represented by pictures presented in silence, with no significant differences between the right and left ears. After the introduction of competitive noise, there was a decrease in individuals' performance. Copyright © 2015 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  20. Speech recognition in individuals with sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Adriana Neves de Andrade

    Full Text Available ABSTRACT INTRODUCTION: Hearing loss can negatively influence the communication performance of individuals, who should be evaluated with suitable material and in situations of listening close to those found in everyday life. OBJECTIVE: To analyze and compare the performance of patients with mild-to-moderate sensorineural hearing loss in speech recognition tests carried out in silence and with noise, according to the variables ear (right and left and type of stimulus presentation. METHODS: The study included 19 right-handed individuals with mild-to-moderate symmetrical bilateral sensorineural hearing loss, submitted to the speech recognition test with words in different modalities and speech test with white noise and pictures. RESULTS: There was no significant difference between right and left ears in any of the tests. The mean number of correct responses in the speech recognition test with pictures, live voice, and recorded monosyllables was 97.1%, 85.9%, and 76.1%, respectively, whereas after the introduction of noise, the performance decreased to 72.6% accuracy. CONCLUSIONS: The best performances in the Speech Recognition Percentage Index were obtained using monosyllabic stimuli, represented by pictures presented in silence, with no significant differences between the right and left ears. After the introduction of competitive noise, there was a decrease in individuals' performance.

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

    Science.gov (United States)

    Melo, Renato de Souza

    2017-09-01

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

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

    Science.gov (United States)

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

    2009-08-01

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

  3. Improvements in Speech Understanding With Wireless Binaural Broadband Digital Hearing Instruments in Adults With Sensorineural Hearing Loss

    OpenAIRE

    Kreisman, Brian M.; Mazevski, Annette G.; Schum, Donald J.; Sockalingam, Ravichandran

    2010-01-01

    This investigation examined whether speech intelligibility in noise can be improved using a new, binaural broadband hearing instrument system. Participants were 36 adults with symmetrical, sensorineural hearing loss (18 experienced hearing instrument users and 18 without prior experience). Participants were fit binaurally in a planned comparison, randomized crossover design study with binaural broadband hearing instruments and advanced digital hearing instruments. Following an adjustment peri...

  4. 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) (ppostural 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.

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

    Science.gov (United States)

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

    2016-04-20

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

  6. 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.

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

    Directory of Open Access Journals (Sweden)

    Yujuan Zhou

    2016-06-01

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

  8. Asymmetric hearing loss in a random population of patients with mild to moderate sensorineural hearing loss.

    Science.gov (United States)

    Segal, Nili; Shkolnik, Mark; Kochba, Anat; Segal, Avichai; Kraus, Mordechai

    2007-01-01

    We evaluated the correlation of asymmetric hearing loss, in a random population of patients with mild to moderate sensorineural hearing loss, to several clinical factors such as age, sex, handedness, and noise exposure. We randomly selected, from 8 hearing institutes in Israel, 429 patients with sensorineural hearing loss of at least 30 dB at one frequency and a speech reception threshold not exceeding 30 dB. Patients with middle ear disease or retrocochlear disorders were excluded. The results of audiometric examinations were compared binaurally and in relation to the selected factors. The left ear's hearing threshold level was significantly higher than that of the right ear at all frequencies except 1.0 kHz (p < .05). One hundred fifty patients (35%) had asymmetric hearing loss (more than 10 dB difference between ears). In most of the patients (85%) the binaural difference in hearing threshold level, at any frequency, was less than 20 dB. Age, handedness, and sex were not found to be correlated to asymmetric hearing loss. Noise exposure was found to be correlated to asymmetric hearing loss.

  9. Sensorineural hearing loss and celiac disease: A coincidental finding

    Science.gov (United States)

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

    2009-01-01

    BACKGROUND Celiac disease (CD) can be associated with a variety of extraintestinal manifestations, including neurological diseases. A new neurological correlation has been found between CD and sensorineural hearing loss (SNHL). OBJECTIVE To verify the association between SNHL and CD, and to establish whether the neurological hearing impairment in CD is related to nonorgan-specific and antineuronal antibodies, as well as the presence of autoimmune disorders. METHODS A sample of 59 consecutive biopsy- and serologically proven CD patients were studied. Among CD patients, 11 were newly diagnosed and 48 were on a gluten-free diet. Hearing function was assessed by audiometric analysis in all CD patients as well as in 59 age- and sex-matched controls. Patients were tested for a panel of immune markers including nonorgan-specific autoantibodies and antineuronal antibodies. RESULTS SNHL was detected in five CD patients (8.5%) and in two controls (3.4%). In one patient, the SNHL was bilateral, whereas the remaining four had a monolateral impairment. The prevalence of SNHL was not significantly different between CD patients and controls. At least one of the antibodies tested for was positive in two of the five CD patients with SNHL and in 12 of the 54 CD patients without SNHL. Antineuronal antibodies to central nervous system antigens were consistently negative in the five CD patients with SNHL. Only one of the five CD patients with SNHL had Hashimoto thyroiditis. CONCLUSIONS SNHL and CD occur coincidentally. Hearing function should be assessed only in CD patients with clinical signs of hearing deficiency. PMID:19668795

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

    Science.gov (United States)

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

    2016-03-01

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

  11. 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.

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

    International Nuclear Information System (INIS)

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

    2011-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Célia Nogueira

    2011-01-01

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

  14. Gradual Recovery from Bilateral Severe Sensorineural Hearing Loss post Motor Vehicle Accident.

    Science.gov (United States)

    Yaroko, A A; Shahrjerdi, B; M D, Md Khairi

    2013-04-01

    Sensorineural hearing loss following trauma is a common finding in daily clinical practice and usually associated with a poor prognosis. Our case illustrates a patient who was involved in motor vehicle accident sustaining bilateral severe to profound sensorineural hearing loss but subsequently recovered fully after two years. Unless there is clear trauma to the cochlea or auditory nerve, a substantial duration of follow up is needed in the treatment of such cases.

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

    Science.gov (United States)

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

    2000-09-01

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

  16. 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.

  17. Gentamicin Exposure and Sensorineural Hearing Loss in Preterm Infants.

    Directory of Open Access Journals (Sweden)

    Aline Fuchs

    Full Text Available To evaluate the impact of gentamicin exposure on sensorineural hearing loss (SNHL in very low birth weight (VLBW infants.Exposure to gentamicin was determined in infants born between 1993 and 2010 at a gestational age < 32 weeks and/or with a birthweight < 1500 g, who presented with SNHL during the first 5 years of life. For each case, we selected two controls matched for gender, gestational age, birthweight, and year of birth.We identified 25 infants affected by SNHL, leading to an incidence of SNHL of 1.58% in our population of VLBW infants. The proportion of infants treated with gentamicin was 76% in the study group and 70% in controls (p = 0.78. The total cumulated dose of gentamicin administered did not differ between the study group (median 10.2 mg/kg, Q1-Q3 1.6-13.2 and the control group (median 7.9 mg/kg, Q1-Q3 0-12.8, p = 0.47. The median duration of gentamicin treatment was 3 days both in the study group and the control group (p = 0.58. Maximum predicted trough serum levels of gentamicin, cumulative area under the curve and gentamicin clearance were not different between cases and controls.The impact of gentamicin on SNHL can be minimized with treatments of short duration, monitoring of blood levels and dose adjustment.

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

    Science.gov (United States)

    de Souza Melo, Renato

    2017-02-01

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

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

    International Nuclear Information System (INIS)

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

    1998-01-01

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

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

    Science.gov (United States)

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

    2015-07-01

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

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

    Science.gov (United States)

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

    2009-02-01

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

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

    Science.gov (United States)

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

    2017-07-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

    Yamamoto, M [Kobe Univ. (Japan). School of Medicine

    1979-08-01

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

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

    International Nuclear Information System (INIS)

    Yamamoto, Matsunori

    1979-01-01

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

  5. 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…

  6. 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…

  7. 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.

  8. Improvements in speech understanding with wireless binaural broadband digital hearing instruments in adults with sensorineural hearing loss.

    Science.gov (United States)

    Kreisman, Brian M; Mazevski, Annette G; Schum, Donald J; Sockalingam, Ravichandran

    2010-03-01

    This investigation examined whether speech intelligibility in noise can be improved using a new, binaural broadband hearing instrument system. Participants were 36 adults with symmetrical, sensorineural hearing loss (18 experienced hearing instrument users and 18 without prior experience). Participants were fit binaurally in a planned comparison, randomized crossover design study with binaural broadband hearing instruments and advanced digital hearing instruments. Following an adjustment period with each device, participants underwent two speech-in-noise tests: the QuickSIN and the Hearing in Noise Test (HINT). Results suggested significantly better performance on the QuickSIN and the HINT measures with the binaural broadband hearing instruments, when compared with the advanced digital hearing instruments and unaided, across and within all noise conditions.

  9. 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.

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

    Science.gov (United States)

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

    2010-01-01

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

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

    Directory of Open Access Journals (Sweden)

    2009-02-01

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

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

    Science.gov (United States)

    Krishnamurti, Sridhar

    2009-03-01

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

  13. 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.

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

    Science.gov (United States)

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

    2017-10-01

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

  15. The current status of audiologic rehabilitation for profound unilateral sensorineural hearing loss.

    Science.gov (United States)

    Bishop, Charles E; Eby, Thomas L

    2010-03-01

    Audiologic rehabilitation of individuals with profound unilateral sensorineural hearing loss (USNHL) has traditionally been limited to the use of air-conduction contralateral routing of sound (CROS) hearing aids. Treatment for these individuals has expanded with new applications of the bone-anchored hearing aid (BAHA), transcranial hearing aid (t-CROS), and the cochlear implant. In this article, the authors review the literature that addresses these various treatment options. Contemporary review Historical information is available that describes the limited efficacy of air-conduction CROS hearing aids in lifting hearing handicap associated with USNHL. Current investigations on providing cross hearing are generally focused on use of the BAHA. Little is known at present whether new developments in hearing aid technology can improve on conventional air-conduction CROS or t-CROS approaches. Interestingly, the cochlear implant seems to be a viable option for individuals with USNHL and tinnitus who also have intact auditory nerve pathways. There is indication in the literature that BAHA provides greater relief of hearing handicap associated with USNHL than CROS hearing aids; however, both have been found to provide limited patient satisfaction and seemingly fall short of restoring true sound localization. Adequate trials have not been performed comparing BAHA with the best CROS hearing aid technology. Transcranial hearing aids and cochlear implants are experimental methods to treat USNHL and hold promise, although there remains a lack of studies available to fully support this.

  16. 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.

  17. 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.

  18. 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

    2018-03-01

    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.

  19. Sudden sensorineural hearing loss: subclinical viral and toxoplasmosis infections as aetiology and how they alter the clinical course.

    Science.gov (United States)

    Kikidis, Dimitrios; Nikolopoulos, Thomas P; Kampessis, Georgios; Stamatiou, Georgios; Chrysovergis, Aristeidis

    2011-01-01

    To explore in a prospective study the evidence of certain viral and toxoplasmosis infections in sudden sensorineural hearing loss (SSHL). 84 consecutive patients with SSHL meeting certain criteria. All patients were assessed for specific IgM antibodies against cytomegalovirus, herpes simplex virus, toxoplasma and Epstein-Barr virus. All were treated with intravenous steroids and assigned to two groups: 76 IgM negative (NV group) and 8 IgM positive (no history of acute infection - V group). The mean hearing level at presentation was 86.5 dB HL (median, 100) in the V group and 60.7 dB HL (median, 61) in the NV group. The difference was statistically significant (p = 0.003). The mean hearing level following treatment was 81.8 dB HL (median, 88) in the V group and 48.7 dB HL (median, 39) in the NV group. The difference was statistically significant (p = 0.004). There was a considerable improvement in hearing after treatment only in the NV group (p toxoplasmoses are involved, the hearing is much worse in comparison to patients with no such indication of infection. An alteration in treatment dosage or method of steroid administration may be needed in such cases. Copyright © 2011 S. Karger AG, Basel.

  20. 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.

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

    DEFF Research Database (Denmark)

    Jepsen, Morten Løve; Dau, Torsten

    2011-01-01

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

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

    International Nuclear Information System (INIS)

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

    1991-01-01

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

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

    Science.gov (United States)

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

    2006-04-01

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

  4. 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

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

    Science.gov (United States)

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

    2016-12-01

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

  6. Congenital inner ear malformations without sensorineural hearing loss.

    Science.gov (United States)

    Yukawa, Kumiko; Horiguchi, Satoshi; Suzuki, Mamoru

    2008-03-01

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

  7. Evaluation of very low birth weight (≤ 1,500 g) as a risk indicator for sensorineural hearing loss.

    Science.gov (United States)

    Borkoski-Barreiro, Silvia A; Falcón-González, Juan C; Limiñana-Cañal, José M; Ramos-Macías, Angel

    2013-01-01

    Hearing plays an essential role in the acquisition, development and maintenance of the properties of the speech and language. Birth weight is an indicator of biological maturation of the newborn. Premature newborns with very low birth weight (VLBW<1,500 g) constitute a group with the highest risk of sensorineural hearing loss. Our objective was to ascertain the degree of hearing loss, sensorineural hearing loss and presence of the association to other risk factors for hearing loss in VLBW infants included in the Universal Hearing Loss Screening Programme at the University Mother-Child Hospital of Gran Canaria (Spain) in the 2007-2010 period. This was a retrospective study of 364 infants with VLBW, measured by transient evoked otoacoustic emissions and auditory brainstem response. There were 112 newborn (30.8%) referred for auditory brainstem response. A diagnosis of hearing loss was given to 22 newborns (2.2%), 14 had conductive hearing loss and 8, sensorineural hearing loss (SNHL), of which 2 had bilateral profound hearing loss. The VLBW newborn presented the association to another risk factor in more than a quarter of the sample studied. All those diagnosed with SNHL were premature. The percentage of VLBW newborns diagnosed with hearing loss is higher than expected in the general population. All those diagnosed with SNHL were premature and presented one or 2 hearing risk factors associated with VLBW. Copyright © 2013 Elsevier España, S.L. All rights reserved.

  8. 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.

  9. 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.

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

    NARCIS (Netherlands)

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

    2001-01-01

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

  11. 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.

  12. 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...

  13. Effects of sensorineural hearing loss on visually guided attention in a multitalker environment.

    Science.gov (United States)

    Best, Virginia; Marrone, Nicole; Mason, Christine R; Kidd, Gerald; Shinn-Cunningham, Barbara G

    2009-03-01

    This study asked whether or not listeners with sensorineural hearing loss have an impaired ability to use top-down attention to enhance speech intelligibility in the presence of interfering talkers. Listeners were presented with a target string of spoken digits embedded in a mixture of five spatially separated speech streams. The benefit of providing simple visual cues indicating when and/or where the target would occur was measured in listeners with hearing loss, listeners with normal hearing, and a control group of listeners with normal hearing who were tested at a lower target-to-masker ratio to equate their baseline (no cue) performance with the hearing-loss group. All groups received robust benefits from the visual cues. The magnitude of the spatial-cue benefit, however, was significantly smaller in listeners with hearing loss. Results suggest that reduced utility of selective attention for resolving competition between simultaneous sounds contributes to the communication difficulties experienced by listeners with hearing loss in everyday listening situations.

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

    Science.gov (United States)

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

    2018-01-01

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

  15. 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.

  16. 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.

  17. 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.

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

    OpenAIRE

    Hosseinabadi, Reza; Jafarzadeh, Sadegh

    2014-01-01

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

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

    Science.gov (United States)

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

    2015-09-01

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

  20. 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.

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

    Science.gov (United States)

    Liang, H; Zhong, S X

    2016-09-07

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

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

    International Nuclear Information System (INIS)

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

    2005-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Mehrdad Rogha

    2017-09-01

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

  4. A comparison of the effects of filtering and sensorineural hearing loss on patients of consonant confusions.

    Science.gov (United States)

    Wang, M D; Reed, C M; Bilger, R C

    1978-03-01

    It has been found that listeners with sensorineural hearing loss who show similar patterns of consonant confusions also tend to have similar audiometric profiles. The present study determined whether normal listeners, presented with filtered speech, would produce consonant confusions similar to those previously reported for the hearing-impaired listener. Consonant confusion matrices were obtained from eight normal-hearing subjects for four sets of CV and VC nonsense syllables presented under six high-pass and six-low pass filtering conditions. Patterns of consonant confusion for each condition were described using phonological features in sequential information analysis. Severe low-pass filtering produced consonant confusions comparable to those of listeners with high-frequency hearing loss. Severe high-pass filtering gave a result comparable to that of patients with flat or rising audiograms. And, mild filtering resulted in confusion patterns comparable to those of listeners with essentially normal hearing. An explanation in terms of the spectrum, the level of speech, and the configuration of this individual listener's audiogram is given.

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

    Science.gov (United States)

    Dedhia, Kavita; Chi, David H

    2016-09-01

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

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

    Science.gov (United States)

    Park, Jungjun; Lombardino, Linda J.

    2012-01-01

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

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

    Science.gov (United States)

    Hosseinabadi, Reza; Jafarzadeh, Sadegh

    2015-01-01

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

  8. Children with minimal sensorineural hearing loss: prevalence, educational performance, and functional status.

    Science.gov (United States)

    Bess, F H; Dodd-Murphy, J; Parker, R A

    1998-10-01

    This study was designed to determine the prevalence of minimal sensorineural hearing loss (MSHL) in school-age children and to assess the relationship of MSHL to educational performance and functional status. To determine prevalence, a single-staged sampling frame of all schools in the district was created for 3rd, 6th, and 9th grades. Schools were selected with probability proportional to size in each grade group. The final study sample was 1218 children. To assess the association of MSHL with educational performance, children identified with MSHL were assigned as cases into a subsequent case-control study. Scores of the Comprehensive Test of Basic Skills (4th Edition) (CTBS/4) then were compared between children with MSHL and children with normal hearing. School teachers completed the Screening Instrument for Targeting Education Risk (SIFTER) and the Revised Behavior Problem Checklist for a subsample of children with MSHL and their normally hearing counterparts. Finally, data on grade retention for a sample of children with MSHL were obtained from school records and compared with school district norm data. To assess the relationship between MSHL and functional status, test scores of all children with MSHL and all children with normal hearing in grades 6 and 9 were compared on the COOP Adolescent Chart Method (COOP), a screening tool for functional status. MSHL was exhibited by 5.4% of the study sample. The prevalence of all types of hearing impairment was 11.3%. Third grade children with MSHL exhibited significantly lower scores than normally hearing controls on a series of subtests of the CTBS/4; however, no differences were noted at the 6th and 9th grade levels. The SIFTER results revealed that children with MSHL scored poorer on the communication subtest than normal-hearing controls. Thirty-seven percent of the children with MSHL failed at least one grade. Finally, children with MSHL exhibited significantly greater dysfunction than children with normal hearing

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

    Science.gov (United States)

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

    1997-10-01

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

  10. 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.

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

    Science.gov (United States)

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

    2014-06-01

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

  12. Clinical analysis of post-irradiation sensorineural hearing loss in patients suffering from nasopharyngeal carcinoma

    International Nuclear Information System (INIS)

    Lu Xueguan; Liu Zhiyong; Zhang Liyuan; Tian Ye

    2005-01-01

    Objective: To investigate the incidence of post-irradiation sensorineural hearing loss (SNHL) in patients suffering from nasopharyngeal carcinoma and to evaluate its potentially contributing factors. Methods: Pure tonetest and impedance audiography were carried out in patients suffering from nasopharyngeal carcinoma with a post-irradiation follow-up time over 1 year. Additionally, the test results were combined with clinical data and analyzed retrospectively. Results: The follow-up time of all patients ranged from 12 to 94 months (median 53 months). The incidences of SNHL at low and high frequencies were 8% and 42% respectively. Univariate analysis showed that patient's age and follow-up time affected the incidence of SNHL at high frequencies (t=2.051, P=0.0269; t=2.978, P=0.0011), but sex, preirradiation subjective hearing loss, irradiation dose and chemotherapy including cisplatin had no significance. Multivariate analysis by Binary Logistic Regression revealed that the risk of SNHL was correlated with patient's age and follow-up time (P=0.02; P=0.009). Conclusion: Post-irradiation SNHL at high frequencies in patients suffering from nasopharyngeal carcinoma is more common than that at low frequencies. The independent prognostic factors for development of SNHL at high frequencies are patient's age and follow-up time. But the role of preirradiation hearing level ,irradiation dose and chemotherapy including cisplatin are not conclusive and further research is needed. (authors)

  13. 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 < 0.05). Mean hearing gains, pure tone averages, speech reception thresholds and subjective recovery rates were significantly better in ozone-treated patients compared with placebo-treated patients (p < 0.05). Ozone therapy is a significant modality for treatment of sudden sensorineural hearing loss; no complications were observed.

  14. Cytokine gene polymorphism associations with congenital cytomegalovirus infection and sensorineural hearing loss.

    Science.gov (United States)

    Kasztelewicz, B; Czech-Kowalska, J; Lipka, B; Milewska-Bobula, B; Borszewska-Kornacka, M K; Romańska, J; Dzierżanowska-Fangrat, K

    2017-10-01

    Cytomegalovirus (CMV) is the most common viral agent of congenital infections and a leading nongenetic cause of sensorineural hearing loss (SNHL). The host immunologic factors that render a developing foetus prone to intrauterine CMV infection and development of hearing loss are unknown. The aim of this study was to assess the potential associations between the polymorphisms within cytokine and cytokine receptors genes, and the risk of congenital CMV infection, and the hearing outcome. A panel of 11 candidate single nucleotide polymorphisms (SNPs): TNF rs1799964, TNF rs1800629, TNFRSF1A rs4149570, IL1B rs16944, IL1B rs1143634, IL10 rs1800896, IL10RA rs4252279, IL12B rs3212227, CCL2 rs1024611, CCL2 rs13900, CCR5 rs333 was genotyped in 470 infants (72 with confirmed intrauterine CMV infection and 398 uninfected controls), and related to congenital CMV infection, and the outcome. In multivariate analysis, the IL1B rs16944 TT and TNF rs1799964 TC genotypes were significantly associated with intrauterine CMV infection (aOR = 2.32; 95% CI, 1.11-4.89; p = 0.032, and aOR = 2.17, 95% CI, 1.25-3.77; p = 0.007, respectively). Twenty-two out of 72 congenitally infected newborns had confirmed SNHL. Carriers of CT or TT genotype of CCL2 rs13900 had increased risk of hearing loss at birth and at 6 months of age (aOR = 3.59; p = 0.028 and aOR = 4.10; p = 0.039, respectively). This is the first study to report an association between SNPs in IL1B, TNF, and CCL2, and susceptibility to congenital CMV infection (IL1B and TNF) and SNHL (CCL2).

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

    Science.gov (United States)

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

    2017-08-01

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

  16. 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

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

    Czech Academy of Sciences Publication Activity Database

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

    2016-01-01

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

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

    Science.gov (United States)

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

    2015-05-01

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

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

    Science.gov (United States)

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

    2016-08-01

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

  20. Safety and otoprotection of metformin in radiation-induced sensorineural hearing loss in the guinea pig.

    Science.gov (United States)

    Mujica-Mota, Mario A; Salehi, Pezhman; Devic, Slobodan; Daniel, Sam J

    2014-05-01

    There is currently no treatment available to prevent radiation-induced sensorineural hearing loss. Metformin has antineoplastic effects and is able to regulate the mitochondrial production of reactive oxygen species after cellular stress, which is one of the mechanisms involved in apoptosis after radiation damage. The objective of this study was to determine the safety and radioprotective properties of metformin against radiation-induced cochlear damage both in vitro and in vivo. In vitro and prospective animal study. Animal Care Facilities of the Montreal Children's Hospital Research Institute. Cultured auditory hair cells (HEI-OC1) were exposed to different concentrations of metformin to determine its safety. Cells were incubated with different metformin concentrations and subjected to radiation. Cell viability after experiments was determined with the 3-(4,5-dimethylthiazol-2-yl)-5-(3-carboxymethoxyphenyl)-2-(4-sulfophenyl)-2H-tetrazolium (MTS) assay. Sixteen guinea pigs were divided in 2 groups: drinking tap water (n = 8) and drinking water containing metformin (n = 8). The animals were unilaterally irradiated for 20 days (total dose 70 Gy), and the ears were divided in 4 groups: control (n = 8), irradiated (n = 8), metformin (n = 8), and experimental (n = 8). Auditory brainstem responses were assessed before and 1, 6, and 16 weeks after completion of radiotherapy. Metformin was not cytotoxic or radioprotective in cultured auditory hair cells. Experimental ears had less hearing loss than radiated ones; however, differences were not statistically significant (P > .05). Metformin is not ototoxic or radioprotective in vitro or in vivo. Ears solely subjected to metformin had better hearing thresholds than the rest of the groups.

  1. Etiology and prevalence rate of bilateral sensorineural hearing impairment in children born in Kobe city over a 10 year period

    International Nuclear Information System (INIS)

    Yoshioka, Mieko; Naito, Yashushi

    2008-01-01

    The study was carried out on children born over a 10 year period from 1997 to 2006 in Kobe city and referred to our center for specialist audiological assessment. A total of 107 cases had a bilateral sensorineural hearing impairment averaging 25 dB or over in the better hearing ear during the study period. To ascertain causes of sensorineural hearing impairment, full medical histories were obtained with detailed family history relevant to hearing impairment and perinatal course for adverse etiological factors. The children were investigated for possible congenital infection and chromosomal anomalies. Children with positive family history of deafness in parents or siblings constituted 11.2% of cases (genetic group). Other etiological groups showed the following distribution: syndromal group 5.6%; inner ear anomalies 5.6%; perinatal group 13.1%; congenital infection 11.2%; chromosomal anomalies 16.8%; multiple congenital anomalies 5.6%; causes unknown 30.9%. The high incidence of causes unknown indicates that steps should be taken to yield a diagnosis. The total number of children born in Kobe city was 117,896 during the period from 1997 to 2005, which gave a prevalence rate of hearing impairment of 0.87/1,000 births. Newborn hearing screening identified many children earlier and also provide the opportunity to finetune the evaluation. (author)

  2. 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....

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

    Science.gov (United States)

    Huo, Liang; Wang, Hua

    2012-12-01

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

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

    Science.gov (United States)

    Manning, Candice; Mermagen, Timothy; Scharine, Angelique

    2017-06-01

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

  5. Sudden Sensorineural Hearing Loss and Polymorphisms in Iron Homeostasis Genes: New Insights from a Case-Control Study

    Directory of Open Access Journals (Sweden)

    Alessandro Castiglione

    2015-01-01

    Full Text Available Background. Even if various pathophysiological events have been proposed as explanations, the putative cause of sudden hearing loss remains unclear. Objectives. To investigate and to reveal associations (if any between the main iron-related gene variants and idiopathic sudden sensorineural hearing loss. Study Design. Case-control study. Materials and Methods. A total of 200 sudden sensorineural hearing loss patients (median age 63.65 years; range 10–92 were compared with 400 healthy control subjects. The following genetic variants were investigated: the polymorphism c.−8CG in the promoter of the ferroportin gene (FPN1; SLC40A1, the two isoforms C1 and C2 (p.P570S of the transferrin protein (TF, the amino acidic substitutions p.H63D and p.C282Y in the hereditary hemochromatosis protein (HFE, and the polymorphism c.–582AG in the promoter of the HEPC gene, which encodes the protein hepcidin (HAMP. Results. The homozygous genotype c.−8GG of the SLC40A1 gene revealed an OR for ISSNHL risk of 4.27 (CI 95%, 2.65–6.89; P=0.001, being overrepresented among cases. Conclusions. Our study indicates that the homozygous genotype FPN1 −8GG was significantly associated with increased risk of developing sudden hearing loss. These findings suggest new research should be conducted in the field of iron homeostasis in the inner ear.

  6. Application of massively parallel sequencing to genetic diagnosis in multiplex families with idiopathic sensorineural hearing impairment.

    Directory of Open Access Journals (Sweden)

    Chen-Chi Wu

    Full Text Available Despite the clinical utility of genetic diagnosis to address idiopathic sensorineural hearing impairment (SNHI, the current strategy for screening mutations via Sanger sequencing suffers from the limitation that only a limited number of DNA fragments associated with common deafness mutations can be genotyped. Consequently, a definitive genetic diagnosis cannot be achieved in many families with discernible family history. To investigate the diagnostic utility of massively parallel sequencing (MPS, we applied the MPS technique to 12 multiplex families with idiopathic SNHI in which common deafness mutations had previously been ruled out. NimbleGen sequence capture array was designed to target all protein coding sequences (CDSs and 100 bp of the flanking sequence of 80 common deafness genes. We performed MPS on the Illumina HiSeq2000, and applied BWA, SAMtools, Picard, GATK, Variant Tools, ANNOVAR, and IGV for bioinformatics analyses. Initial data filtering with allele frequencies (0.95 prioritized 5 indels (insertions/deletions and 36 missense variants in the 12 multiplex families. After further validation by Sanger sequencing, segregation pattern, and evolutionary conservation of amino acid residues, we identified 4 variants in 4 different genes, which might lead to SNHI in 4 families compatible with autosomal dominant inheritance. These included GJB2 p.R75Q, MYO7A p.T381M, KCNQ4 p.S680F, and MYH9 p.E1256K. Among them, KCNQ4 p.S680F and MYH9 p.E1256K were novel. In conclusion, MPS allows genetic diagnosis in multiplex families with idiopathic SNHI by detecting mutations in relatively uncommon deafness genes.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

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

    2018-04-01

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

  9. 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.

  10. Simultaneous versus Sequential Intratympanic Steroid Treatment for Severe-to-Profound Sudden Sensorineural Hearing Loss.

    Science.gov (United States)

    Yoo, Myung Hoon; Lim, Won Sub; Park, Joo Hyun; Kwon, Joong Keun; Lee, Tae-Hoon; An, Yong-Hwi; Kim, Young-Jin; Kim, Jong Yang; Lim, Hyun Woo; Park, Hong Ju

    2016-01-01

    Severe-to-profound sudden sensorineural hearing loss (SSNHL) has a poor prognosis. We aimed to compare the efficacy of simultaneous and sequential oral and intratympanic steroids for this condition. Fifty patients with severe-to-profound SSNHL (>70 dB HL) were included from 7 centers. The simultaneous group (27 patients) received oral and intratympanic steroid injections for 2 weeks. The sequential group (23 patients) was treated with oral steroids for 2 weeks and intratympanic steroids for the subsequent 2 weeks. Pure-tone averages (PTA) and word discrimination scores (WDS) were compared before treatment and 2 weeks and 1 and 2 months after treatment. Treatment outcomes according to the modified American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) criteria were also analyzed. The improvement in PTA and WDS at the 2-week follow-up was 23 ± 21 dB HL and 20 ± 39% in the simultaneous group and 31 ± 29 dB HL and 37 ± 42% in the sequential group; this was not statistically significant. Complete or partial recovery at the 2-week follow-up was observed in 26% of the simultaneous group and 30% of the sequential group; this was also not significant. The improvement in PTA and WDS at the 2-month follow-up was 40 ± 20 dB HL and 37 ± 35% in the simultaneous group and 41 ± 25 dB HL and 48 ± 41% in the sequential group; this was not statistically significant. Complete or partial recovery at the 2-month follow-up was observed in 33% of the simultaneous group and 35% of the sequential group; this was also not significant. Seven patients in the sequential group did not need intratympanic steroid injections for sufficient improvement after oral steroids alone. Simultaneous oral/intratympanic steroid treatment yielded a recovery similar to that produced by sequential treatment. Because the addition of intratympanic steroids can be decided upon based on the improvement after an oral steroid, the sequential regimen can be recommended to avoid unnecessary

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

    Science.gov (United States)

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

    2018-05-01

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

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

    Science.gov (United States)

    Abdala, Carolina; Fitzgerald, Tracy S.

    2003-08-01

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

  13. Total serum bilirubin levels and sensorineural hearing loss in the US adolescents: NHANES 2007-2010.

    Science.gov (United States)

    Zhou, Guoli; Fu, Wenjiang

    2018-02-01

    We aimed to investigate whether current levels of total serum bilirubin are associated with different subtypes of sensorineural hearing loss (SNHL) in adolescents. A set of cross-sectional data from the National Health and Nutrition Examination Survey (NHANES) (2007-2010) was used. A subset of 1404 adolescents was sampled for measurements of total serum bilirubin, tympanometry, and average pure tone threshold at low-frequencies (LPTA: 500, 1000, 2000 Hz) or high-frequencies (HPTA: 3000, 4000, 6000, and 8000 Hz). SNHL was defined as the hearing loss that had type A tympanograms with a peak admittance of 0.3 ml or greater. Associations between serum bilirubin (square-root transformed) and different subtypes of SNHL were evaluated using binary or multinomial logistic regression models with 4-year sampling weights. The bootstrap method was used for estimation of variance and 10-fold cross-validation for assessment of overfitting issue. Total serum bilirubin levels were found to be associated with any high-frequency (HPTA>15 dB in at least one ear, adjusted odds-ratio (OR a )(bootstrap 95% confidence interval) = 3.29(1.31-8.19), p = 0.011), but not with any low-frequency (LPTA>15 dB in at least one ear), SNHL in the US adolescents. Furthermore, high-frequency SNHL with HPTA>15 dB in both ears (bilateral) or HPTA≥25 dB in at least one ear, compared to that with HPTA>15 dB in one ear only (unilateral) or HPTA = 15-25 dB in at least one ear, had a stronger association with total serum bilirubin levels (OR a  = 5.37(1.27-22.65), p = 0.022 for bilateral; OR a  = 2.64(0.84-8.25), p = 0.094 for unilateral; OR a  = 5.00(0.95-26.58), p = 0.058 for HPTA≥25 dB in at least one ear; as well as OR a  = 3.06(1.15-8.25), p = 0.025 for HPTA = 15-25 dB in at least one ear). No severe overfitting problems were found. Our findings suggest that current levels of total serum bilirubin may be informative in predicting and/or targeting high-frequency SNHL

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

    Directory of Open Access Journals (Sweden)

    Jennifer L. Hatton

    2012-01-01

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

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

    Science.gov (United States)

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

    2012-01-01

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

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

    Science.gov (United States)

    Tharpe, Anne Marie; Gustafson, Samantha

    2015-12-01

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

  17. Epidemiology of prelingual sensorineural hearing impairment at a children’s center in Bogotá, Colombia between 1997 and 2008

    OpenAIRE

    Claudia Talero-Gutiérrez; Liliana Romero; Irma Carvajalino; Milciades Ibáñez

    2011-01-01

    Introduction: Hearing loss is a frequent problem in childhood with an incidence of about one case per 1000 births. Control of deafness should be aimed at prevention and early diagnosis in efforts to provide appropriate treatment and stimulate adequate communication in children affected. The objective of this study was to determine the prevalence of different etiologies among deaf children with a diagnosis of prelingual sensorineural hearing loss referred to the Fundación CINDA in Bogotá, Colo...

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

    Directory of Open Access Journals (Sweden)

    Teppei Noda

    2018-02-01

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

  19. Idiopathic sudden sensorineural hearing loss and ménière syndrome: The role of cerebral venous drainage.

    Science.gov (United States)

    Ciccone, M M; Scicchitano, P; Gesualdo, M; Cortese, F; Zito, A; Manca, F; Boninfante, B; Recchia, P; Leogrande, D; Viola, D; Damiani, M; Gambacorta, V; Piccolo, A; De Ceglie, V; Quaranta, N

    2018-02-01

    To evaluate the influence of cerebral venous drainage on the pathogenesis of idiopathic sudden sensorineural hearing loss (ISSHL) and Ménière syndrome (MD). Observational, prospective, cohort study. ENT and Cardiology Departments (University of Bari, Policlinico Hospital, Bari, Italy). We enrolled 59 consecutive patients (32 males, mean age 53.05 + 15.37 years): 40 ISSHL and 19 MD. All patients underwent physical examination, biochemical evaluation (glycemic and lipid profile, viral serology, C reactive protein, etc), audiometric (tonal, vocal, vestibular evoked myogenic potentials and auditory brainstem response test) and impedentiometric examination. The pure tone average (PTA) was calculated for the following frequencies: 250, 500, 1000, 2000, 3000, 4000, 8000. An echo-color Doppler evaluation of the venous cerebral veins, internal jugular (IJV) and vertebral veins (VV) at supine and 90° position was performed. No morphological alterations were found both in patients and controls. There were no signs of stenosis, blocked flow, membranes, etc. We found lower minimum, mean and maximum velocities in distal IJVs (P = .019; P = .013; P = .022; respectively) and left VVs (P = .027; P = .008; P = .001; respectively) in supine (0°) position in both MD and ISSHL patients as compared to controls. The same was for orthostatic position (90°). We found negative correlations between the velocities in extracranial veins and PTA values: therefore, the worst the audiometric performance of the subjects, the lower the velocities in the venous cerebral drainage. Idiopathic sudden sensorineural hearing loss and Ménière syndrome patients showed altered venous flow in IJVs and VVs as compared to controls, independently from posture. This different behavior of venous tone control can influence the ear performance and may have a role in the pathogenesis of both diseases. © 2017 John Wiley & Sons Ltd.

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

    Directory of Open Access Journals (Sweden)

    Aghazadeh-Attari J

    2017-11-01

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

  1. Diabetes mellitus and sensorineural hearing loss: is there an association? Baseline of the Brazilian Longitudinal Study of Adult Health (ELSA-Brasil).

    Science.gov (United States)

    Samelli, Alessandra G; Santos, Itamar S; Moreira, Renata R; Rabelo, Camila M; Rolim, Laurie P; Bensenõr, Isabela J; Lotufo, Paulo A

    2017-01-01

    Although several studies have investigated the effects of diabetes on hearing loss, the relationship between these two conditions remains unclear. Some studies have suggested that diabetes may cause sensorineural hearing loss, whereas others have failed to find an association. The biggest challenge in investigating the association between diabetes and hearing loss is the presence of confounding variables and the complexity of the auditory system. Our study investigated the association between diabetes and sensorineural hearing loss. We evaluated the influence of time from diabetes diagnosis on this association after controlling for age, gender, and hypertension diagnosis and excluding those subjects with exposure to noise. This cross-sectional study evaluated 901 adult and elderly Brazilian Longitudinal Study of Adult Health (ELSA-Brasil) participants from São Paulo, Brazil who underwent audiometry testing as part of ELSA-Brasil's baseline assessment. Hearing thresholds and speech test results were significantly worse in the group with diabetes than in the group without diabetes. However, no significant differences were found between participants with and without diabetes after adjusting for age, gender, and the presence of hypertension. Hearing thresholds were not affected by occupational noise exposure in the groups with and without diabetes. In addition, no association between the duration of diabetes and hearing thresholds was observed after adjusting for age, gender, and hypertension. We found no association between the duration of diabetes and worse hearing thresholds after models were adjusted for age, gender, and the presence of hypertension.

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

    Directory of Open Access Journals (Sweden)

    Shuang Xia

    2017-01-01

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

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

    Directory of Open Access Journals (Sweden)

    I Jen Chang

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

  4. Novel compound heterozygous mutations in MYO7A gene associated with autosomal recessive sensorineural hearing loss in a Chinese family.

    Science.gov (United States)

    Ma, Yalin; Xiao, Yun; Zhang, Fengguo; Han, Yuechen; Li, Jianfeng; Xu, Lei; Bai, Xiaohui; Wang, Haibo

    2016-04-01

    Mutations in MYO7A gene have been reported to be associated with Usher Syndrome type 1B (USH1B) and nonsyndromic hearing loss (DFNB2, DFNA11). Most mutations in MYO7A gene caused USH1B, whereas only a few reported mutations led to DFNB2 and DFNA11. The current study was designed to investigate the mutations among a Chinese family with autosomal recessive hearing loss. In this study, we present the clinical, genetic and molecular characteristics of a Chinese family. Targeted capture of 127 known deafness genes and next-generation sequencing were employed to study the genetic causes of two siblings in the Chinese family. Sanger sequencing was employed to examine those variant mutations in the members of this family and other ethnicity-matched controls. We identified the novel compound heterozygous mutant alleles of MYO7A gene: a novel missense mutation c.3671C>A (p.A1224D) and a reported insert mutation c.390_391insC (p.P131PfsX9). Variants were further confirmed by Sanger sequencing. These two compound heterozygous variants were co-segregated with autosomal recessive hearing loss phenotype. The gene mutation analysis and protein sequence alignment further supported that the novel compound heterozygous mutations were pathogenic. The novel compound heterozygous mutations (c.3671C>A and c.390_391insC) in MYO7A gene identified in this study were responsible for the autosomal recessive sensorineural hearing loss of this Chinese family. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  5. Relative Contributions of Radiation and Cisplatin-Based Chemotherapy to Sensorineural Hearing Loss in Head-and-Neck Cancer Patients

    International Nuclear Information System (INIS)

    Hitchcock, Ying J.; Tward, Jonathan D.; Szabo, Aniko; Bentz, Brandon G.; Shrieve, Dennis C.

    2009-01-01

    Purpose: To investigate the risk of sensorineural hearing loss (SNHL) in patients with head-and-neck cancer and treated with radiation therapy (RT) or concomitant cisplatin-based chemoradiation, the relationship among SNHL and radiation dose to the cochlea, the use of two common cisplatin dose regimens. Methods and Materials: A total of 62 head-and-neck cancer patients treated with curative intent were included in this prospective study. Of the patients, 21 received RT alone, 27 received 40 mg/m 2 weekly cisplatin, 13 received 100 mg/m 2 every 3 weeks during RT, and 1 received RT with weekly epidermal growth factor receptor inhibitor antibody. The effect of chemotherapy and RT dose on hearing was determined using a model that accounted for the age and variability between each ear for each patient. Results: We constructed a model to predict dose-dependent hearing loss for RT or cisplatin-based chemotherapy either alone or in combination. For patients only receiving RT, no significant hearing loss was found at doses to the cochlea of less than 40 Gy. Patients receiving 100 mg/m 2 or 40 mg/m 2 of cisplatin chemotherapy had an estimated +21.5 dB and +9.5 dB hearing loss at 8,000 Hz with low radiation doses (10 Gy), which rose to +38.4 dB and +18.9 dB for high radiation doses (40 Gy). Conclusions: Use of RT alone with doses of less than 40 Gy did not result in clinically significant hearing loss. High-frequency SNHL was profoundly damaged in patients who received concomitant cisplatin when doses of 100 mg/m 2 were used. The threshold cochlear dose for hearing loss with cisplatin-based chemotherapy and RT was predicted to be 10 Gy. The inner ear radiation dose constraints and cisplatin dose intensity should be considered in the treatment of advanced head-and-neck cancer

  6. 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.

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

    Directory of Open Access Journals (Sweden)

    Zhengyue Li

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

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

    Science.gov (United States)

    Li, Youjin; Yang, Jun; Li, Yun

    2011-01-01

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

  9. 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.

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

    Science.gov (United States)

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

    2017-06-01

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

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

    Science.gov (United States)

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

    2017-05-01

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

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

    International Nuclear Information System (INIS)

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

    2010-01-01

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

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

    Science.gov (United States)

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

    2016-09-07

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

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

    Science.gov (United States)

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

    2017-06-01

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

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

    DEFF Research Database (Denmark)

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

    2010-01-01

    Clinical and audiological examination was done in 2 Belgian families with autosomal dominant sensorineural hearing loss (SNHL) linked to DFNA22. Nineteen subjects in family 1 had mild to moderate SNHL starting in the third decade. The hearing loss was characterized by a flat audiogram affecting all......Hz. For all hitherto known DFNA22 families the audiological and clinical characteristics were correlated with the molecular data. This study describes the phenotype of 2 Belgian families with SNHL linked to DFNA22, both with a pathogenic change in the deafness gene MYO6. The phenotypes of all hitherto...

  16. Surdez súbita idiopática: aspectos etiológicos e fisiopatogênicos Idiopathic sudden sensorineural hearing loss: etiopathogenic aspects

    Directory of Open Access Journals (Sweden)

    Paulo Roberto Lazarini

    2006-08-01

    Full Text Available Vários fatores têm sido postulados como causa da surdez súbita idiopática. Por meio de uma revisão bibliográfica foi feita uma análise crítica quanto aos aspectos etiológicos e fisiopatogênicos desta manifestação clínica. Estudos atuais sugerem que os distúrbios vasculares, a ruptura de membranas da orelha interna e as doenças auto-imunes são possíveis causas, mas a afecção viral tem recebido maior atenção nos últimos anos, embora ainda pouco se conheça sobre os mecanismos da surdez súbita idiopática. Os vírus podem causar a perda súbita da audição na infecção aguda, mas a forma latente, com uma possível reativação viral, também tem sido considerada no mecanismo de agressão à cóclea. Apesar de uma alteração da viscosidade sangüínea poder explicar a perda auditiva, estudos experimentais e clínicos não mostram sinais de ossificação e de fibrose na cóclea ou de ruptura de membranas do labirinto. Estes fatos contrapõem, respectivamente, a teoria vascular e a da fístula labiríntica. A eventual presença de anticorpos contra a orelha interna sugere que a surdez súbita idiopática possa ser de natureza auto-imune, fato este também não confirmado pela falta de relação entre os aspectos clínicos e morfológicos da doença auto-imune e da perda auditiva. A surdez súbita idiopática é, ainda, um tema controverso e obscuro em diversos aspectos.Several factors have been postulated to elicit the etiology of idiopathic sudden sensorineural hearing loss. Through a bibliographic review, we made a critical analysis of the different etiopathogenic aspects of its clinical manifestation. The most recent studies concerning the possible causes of sudden hearing loss suggest vascular disorders, rupture of the inner ear membrane and autoimmune diseases; however, viral infections have received a great deal of attention in recent years. Little is known about the mechanism of sudden hearing loss. Viruses can cause

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

    International Nuclear Information System (INIS)

    Kono, Tatsuo

    2008-01-01

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

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

    Science.gov (United States)

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

    2014-04-01

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

  19. Establishment of a model of cochlear lesions in rats to study potential gene therapy for sensorineural hearing loss.

    Science.gov (United States)

    Wang, Ye; Qiao, Li; Chen, Yang; Wen, Liting; Yue, Bo; Qiu, Jianhua; Wu, Shengxi

    2015-12-01

    Sensorineural hearing loss seriously influences a patient's daily life, and no effective treatments exist to date. Gene therapy is a potential treatment for regenerating hair cells to restore hearing. In this study, we established a cochlear lesions model to study hair cell regeneration by co-administration of kanamycin and furosemide. After the injections, we assessed the survival of outer hair cells (OHC), inner hair cells (IHC), supporting cells (SC), spiral ganglion neurons (SGN) and peripheral axons. Moreover, we used two viral vectors to detect the transgene distribution. Our results showed at 12h post-treatment, numerous OHC were missing in the basal turn. At 24h post-treatment, all OHCs in basal half of the cochlea were lost, and by 48h, OHC loss had spread to the apical coil. Four days after the injections, all OHCs were absent. At 1mo post-treatment, the organ of Corti had collapsed. In contrast, most of the SC remained 4d after the injections. The loss of SGN and peripheral axons was consistent with this time course post-treatment. The results of transgene distribution suggested the correlative gene can be transferred into the organ of Corti using adenoviruses (AdV) vectors and lentiviruses (LV) vectors in our cochlear lesion model. We assessed the details of HC death at more time point and chosen the time point for gene transfer in this model. We conclude that this cochlear lesion model would be suitable for the study of hair cell regeneration. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

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

    DEFF Research Database (Denmark)

    Bianchi, Federica; Fereczkowski, Michal; Zaar, Johannes

    2016-01-01

    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......-discrimination performance in listeners with SNHL. Pitch-discrimination thresholds were obtained for 14 normal-hearing (NH) and 10 hearing-impaired (HI) listeners for sine-phase (SP) and random-phase (RP) complex tones. When all harmonics were unresolved, the HI listeners performed, on average, worse than NH listeners...... in the RP condition but similarly to NH listeners in the SP condition. The increase in pitch-discrimination performance for the SP relative to the RP condition (F0DL ratio) was significantly larger in the HI as compared with the NH listeners. Cochlear compression and auditory-filter bandwidths were...

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

    International Nuclear Information System (INIS)

    Chetcuti, Karen; Kumbla, Surekha

    2016-01-01

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

  2. 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.)

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

    NARCIS (Netherlands)

    Baskent, Deniz

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

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

    Directory of Open Access Journals (Sweden)

    M. Amiridavan

    2006-05-01

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

  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. A case-control analysis on the association between erectile dysfunction and sudden sensorineural hearing loss in Taiwan.

    Science.gov (United States)

    Keller, Joseph J; Chen, Yi-Kuang; Lin, Herng-Ching

    2012-05-01

    Although the cause of sudden sensorineural hearing loss (SSNHL) is yet to be elucidated, many theories have been proposed regarding potentially contributory etiologies. One increasingly well-supported theory purports an underlying vascular pathomechanism. If this is the case, SSNHL may also associate with conditions comorbid with vascular diseases, such as erectile dysfunction (ED). However, no studies to date have investigated the association between ED and SSNHL. This study set out to estimate a putative association between ED and having been previously diagnosed with SSNHL using a population-based dataset with a case-control design. This study used administrative claim data from the Taiwan National Health Insurance program. We identified 4,504 patients with ED as the study group and randomly selected 22,520 patients as the comparison group. Conditional logistic regression was used to examine the association between ED and having previously received a diagnosis of SSNHL. The prevalence and risk of SSNHL between cases and controls were calculated. Of the sampled patients, 41 (0.15%) had been diagnosed with SSNHL before the index date; 22 (0.49% of the cases) were from the study group and 19 (0.08% of controls) were from the control group. Conditional logistic regression analysis revealed that after adjusting for the patient's monthly income, geographic location, hypertension, diabetes, hyperlipidemia, coronary heart disease, obesity, and alcohol abuse/alcohol dependence syndrome status, patients with ED were more likely than controls to have been diagnosed with SSNHL before the index date (odds ratio = 6.06, 95% confidence interval = 3.25-11.29). There was an association between ED and prior SSNHL. The results of this study add to the evidence supporting an underlying vascular pathomechanism regarding the development of SSNHL and highlight a need for clinicians dealing with SSNHL patients to be alert to the development of ED. © 2012 International Society for

  7. Sensorineural hearing loss in patients treated for nasopharyngeal carcinoma: a prospective study of the effect of radiation and cisplatin treatment

    International Nuclear Information System (INIS)

    Kwong, Dora L.W.; Wei, William I.; Sham, Jonathan S. T.; Ho, W.K.; Yuen, P.W.; Chua, Daniel T.T.; Au, Dennis K.K.; Wu, P.M.; Choy, Damon T.K.

    1996-01-01

    Purpose: The pattern of sensorineural hearing loss (SNHL) after primary treatment for nasopharyngeal carcinoma (NPC) was studied, and the effect of cisplatin, radiotherapy dose, and fractionation were evaluated. Methods and Materials: One hundred thirty-two patients, 227 ears, and 1100 audiogram reports were analyzed. Methods and Materials: One hundred thirty-two patients, 227 ears, and 1100 audiogram reports were analyzed. Radiotherapy dose ranged from 59.5 to 76.5 Gy. Fifty-two patients received preirradiation cisplatin, total dose 100-185 mg/m 2 . Serial postirradiation bone conduction thresholds at 0.5 kHz, 1 kHz, 2 kHz, and 4 kHz were compared with pretreatment thresholds at respective frequencies. Increase of at least 15 dB was considered as significant and was further grouped as transient or persistent SNHL. Univariate and multivariate analyses were performed to identify predicting factors for persistent SNHL. Results: At median follow-up of 30 months, 24.2% of ears developed persistent SNHL. High frequency was more affected than low frequencies, 22 vs. 5.3%. Males were more affected than females, 29.4 vs. 15.5%, p = 0.0132. Incidence of persistent SNHL increased with age, with 0, 17.2, and 37.4% of patients aged under 30, between 30-50 and over 50 affected, respectively, p = 0.0001. High incidence was found in patient with postirradiation serous otitis media (SOM), 46.9%. Chemotherapy with cisplatin and radiation dose or fractionation had no significant effect. Multivariate analysis confirmed age, sex, and postirradiation SOM as significant prognostic factors for persistent SNHL. Conclusions: Transient and persistent SNHL occurred after radiotherapy, more commonly affecting high frequency. A low dose of preirradiation cisplatin did not increase the risk. A dose fractionation effect of radiotherapy was not confirmed in this study

  8. CEP250 mutations associated with mild cone-rod dystrophy and sensorineural hearing loss in a Japanese family.

    Science.gov (United States)

    Kubota, Daiki; Gocho, Kiyoko; Kikuchi, Sachiko; Akeo, Keiichiro; Miura, Masahiro; Yamaki, Kunihiko; Takahashi, Hiroshi; Kameya, Shuhei

    2018-05-02

    CEP250 encodes the C-Nap1 protein which belongs to the CEP family of proteins. C-Nap1 has been reported to be expressed in the photoreceptor cilia and is known to interact with other ciliary proteins. Mutations of CEP250 cause atypical Usher syndrome which is characterized by early-onset sensorineural hearing loss (SNHL) and a relatively mild retinitis pigmentosa. This study tested the hypothesis that the mild cone-rod dystrophy (CRD) and SNHL in a non-consanguineous Japanese family was caused by CEP250 mutations. Detailed ophthalmic and auditory examinations were performed on the proband and her family members. Whole exome sequencing (WES) was used on the DNA obtained from the proband. Electrophysiological analysis revealed a mild CRD in two family members. Adaptive optics (AO) imaging showed reduced cone density around the fovea. Auditory examinations showed a slight SNHL in both patients. WES of the proband identified compound heterozygous variants c.361C>T, p.R121*, and c.562C>T, p.R188* in CEP250. The variants were found to co-segregate with the disease in five members of the family. The variants of CEP250 are both null variants and according to American College of Medical Genetics and Genomics (ACMG) standards and guideline, these variants are classified into the very strong category (PVS1). The criteria for both alleles will be pathogenic. Our data indicate that mutations of CEP250 can cause mild CRD and SNHL in Japanese patients. Because the ophthalmological phenotypes were very mild, high-resolution retinal imaging analysis, such as AO, will be helpful in diagnosing CEP250-associated disease.

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

    Directory of Open Access Journals (Sweden)

    Cristina Caroça

    2017-02-01

    Full Text Available Abstract Background Rubella infection can affect several organs and cause birth defects that are responsible for congenital rubella syndrome (CRS. Congenital hearing loss is the most common symptom of this syndrome, occurring in approximately 60% of CRS cases. Worldwide, over 100 000 babies are born with CRS every year. There is no specific treatment for rubella, but the disease is preventable by vaccination. Since 1969, the rubella vaccine has been implemented in many countries, but in Africa, only a few countries routinely immunize against rubella. The aim of this study was to estimate the rate of infection from the wild-type rubella virus in São Tomé and Príncipe by determining rubella seroprevalence with a DBS method. The goal of this study was to reinforce the need for implementation of the rubella vaccine in this country. As secondary objectives, the validation of a DBS method was first attempted and an association between seroprevalence and hearing loss was assessed. Methods We collected samples from individuals observed during humanitarian missions in São Tomé and Príncipe. All individuals underwent an audiometric evaluation, and a drop of blood was collected for the dried blood spot (DBS. We define two groups: the case group (individuals with unilateral or bilateral hearing loss (HL and the control group (individuals with two normal ears. Patients were excluded if they suffered from conductive HL, if they showed evidence of possible causes of HL, if they had developmental delay or if they refused to participate in the study. Results Among the 315 subjects, we found 64.1% individuals with IgG for the rubella virus, 32.1% without immunity for the rubella virus and 3.8% who were borderline. In the control group, 62.6% were positive for the rubella IgG, whereas in the case group, 72% were positive. Analyzing both groups, with ages ranging from 2 to 14 years of age and from 15 to 35 years of age, we found a seroprevalence of 50.3% to

  10. 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

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

    Directory of Open Access Journals (Sweden)

    Xin-chang Zhang

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

  12. 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...

  13. 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.

  14. [Connexin gene 26 (GJB2) mutations in patients with hereditary non-syndromic sensorineural loss of hearing in the Republic of Sakha (Yakutia)].

    Science.gov (United States)

    Barashkov, N A; Dzhemileva, L U; Fedorova, S A; Maksimova, N R; Khusnutdinova, E K

    2008-01-01

    The aim of the study was to elucidate the causes of hereditary non-syndromic loss of hearing, a frequent monogene pathology in the Republic of Sakha (Yakutia). A search for mutations in the coding sequence of the connexin 26 gene gap-junction B2 (GJB2) was undertaken in 79 members of 65 unrelated families with the diagnosis of grade III-IV non-syndromic bilateral sensorineural loss of hearing. Five recessive mutations (35delG, V371, 312-326del14, 333-334delAA, R127H) and three polymorphic variants (V271, M34T, E114G) were identified in Yakut patients. Mutations 35delG (41.7%), 312-326dell4 (4.2%), and 333-334delAA (4.2%) were found in Caucasian patients (Russians, Ukrainians, Inguish). Yakuts were carriers of mutations 35delG (2.1%), V371 (2.1%), R127H (1.0%) and sequence variants V271 (6.3%), M34T (1.0%), E114G (1.0%). GJB2 mutations were identified in 50.1% of the Caucasian patients and in 7.2% of the Yakut patients. The low frequency of GJB2 mutations in Yakuts with non-syndromic sensorineural loss of hearing testifies to the presence of mutations of other genes controlling sound perception in this population.

  15. 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

  16. Hearing Loss due to Carbon Monoxide Poisoning

    DEFF Research Database (Denmark)

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

    2013-01-01

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

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

    Science.gov (United States)

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

    2017-08-01

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

  18. Pendred syndrome (goitre and sensorineural hearing loss) maps to chromosome 7 in the region containing the nonsyndromic deafness gene DFNB4.

    Science.gov (United States)

    Coyle, B; Coffey, R; Armour, J A; Gausden, E; Hochberg, Z; Grossman, A; Britton, K; Pembrey, M; Reardon, W; Trembath, R

    1996-04-01

    Inherited causes account for about 50% of individuals presenting with childhood (prelingual) hearing loss, of which 70% are due to mutation in numerous single genes which impair auditory function alone (non-syndromic). The remainder are associated with other developmental anomalies termed syndromic deafness. Genes responsible for syndromic forms of hearing loss include the COL4A5 gene in Alport syndrome and the PAX3 and MITF genes in Waardenburg syndrome. Pendred syndrome is an autosomal recessive disorder associated with developmental abnormalities of the cochlea, sensorineural hearing loss and diffuse thyroid enlargement (goitre). Pendred syndrome is the most common syndromal form of deafness, yet the primary defect remains unknown. We have established a panel of 12 families with two or more affected individuals and used them to search for the location of the Pendred gene by linkage analysis. We excluded localization to four previously mapped nonsyndromic deafness loci but obtained conclusive evidence for linkage of the Pendred syndrome gene to microsatellite markers on chromosome 7q31 (D7S495 Zmax 7.32, Qmax = 0). This region contains a gene, DFNBL, for autosomal recessive non-syndromic sensorineural hearing loss. Multipoint analysis indicates that DFNB4 and Pendred syndrome co-localize to the same 5.5 centiMorgan (cM) interval flanked by D7S501 and D7S523. These data raise the possibility that Pendred syndrome is either allelic with DFNB4 or may represent an inherited contiguous gene disorder, not clinically manifest in the heterozygote.

  19. Genetics Home Reference: sensorineural deafness and male infertility

    Science.gov (United States)

    ... deafness and male infertility Sensorineural deafness and male infertility Printable PDF Open All Close All Enable Javascript ... expand/collapse boxes. Description Sensorineural deafness and male infertility is a condition characterized by hearing loss and ...

  20. [Analysis of mitochondrial 12S rRNA and tRNA(Ser(UCN)) genes in patients with nonsyndromic sensorineural hearing loss from various regions of Russia].

    Science.gov (United States)

    Dzhemileva, L U; Posukh, O L; Tazetdinov, A M; Barashkov, N A; Zhuravskiĭ, S G; Ponidelko, S N; Markova, T G; Tadinova, V N; Fedorova, S A; Maksimova, N R; Khusnutdinova, E K

    2009-07-01

    Mitochondrial DNA (mtDNA) mutations play an important role in etiology of hereditary hearing loss. In various regions of the world, patients suffer from nonsyndromic sensorineural hearing loss initiated by aminoglycoside antibiotics. Mutations that had been shown as pathogenetically important for hearing function disturbance were identified in mitochondrial 12S rRNA and tRNA(Ser(UCN)) genes while pathogenic role of several DNA sequences requires additional studies. This work presents the results of studying the spectrum of mutations and polymorphic variations in mtDNA genes 12S rRNA and tRNA(Ser(UGN)) in 410 patients with nonsyndromal sensoneural hearing impairment/loss from the Volga Ural region, St Petersburg, Yakutia, and Altai and in 520 individuals with normal hearing, which represent several ethnic groups (Russians, Tatars, Bashkirs, Yakuts, Altaians) residing in the Russian Federation. Pathogenetically significant mutation A1555G (12S rRNA) was found in two families (from Yakutia and St Peresburg) with hearing loss, probably caused by treatment with aminoglucosides, and in the population sample of Yakuts with a frequency of 0.83%. Further research is needed to confirm the role in hearing impairment of mutations 961insC, 961insC(n), 961delTinsC(n), T961G, T1095C (12S rRNA) and G7444A, A7445C (tRNA(Ser(UGN revealed in the patients. In addition, in the patients and the population groups, polymorphic mt DNA variants were detected, which are characteristic also of other Eurasian populations both in spectrum and frequency.

  1. Screening of Long Q-T Syndrome in Patients with Congenital Sensorineural Hearing Loss (Jervell and Lange Neilesen Syndrome: Prevention of Fatal Events

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    Farid Matin

    2001-01-01

    Full Text Available Objective:The idiopathic long Q-T syndrome is an infrequently occurring disorder in which affected individuals have an unusual electrocardiographic repolarization abnormality presenting as syncope or loss of consciousness related to ventricular tachycardia or fibrillation. Congenital long Q-T prolongation can be associated with congenital deafness in an autosomal recessive manner (Jervell and Lange-Nielsen syndrome. The purpose of this stuff was to screen this electrocardiographic abnormality in deaf-mute school children in our population, which has not been yet performed. Materials & Methods:  Of 1190 patients with hearing loss, 779 had congenital sensorineural deafness (CSD, aged 13±3.8 years (4-24, 63% female and 37% male. The family history of deafness was as follows: Cardiac axis deviation was found in 56 (7% patients. Electrical conduction abnormalities were found in 12 (15% patients, Wolff-Parkinson-White syndrome, sinus bradycardia, and sinus arrhythmia were found in 2 (0.25%, 4 (0.5%, and 3 (0.38% patients, respectively. The Q-T interval, and Q-Tc duration were 312.6±28.9 ms (200-500 ms, median 320 ms, and 383.6±29.3 ms (232-527 ms, median 413ms, respectively. Long Q-T syndrome was found in 4 (0.5% patients (3F and 1M. Results: Two of these 4 patients had total deafness and 2 had profound hearing loss. None of the patients with mild deafness had Q-T prolongation. Only one of these patients was symptomatic, and had been treated as a case of epilepsy for several years. Conclusion: This data supports the presence of long Q-T syndrome in patients with sensorineural hearing loss in our population, so routine electrocardiographic screening of anyone with congenital deafness is warranted to prevent subsequent associated cardiac arrhythmias and sudden cardiac death.

  2. [Analysis the relationship between SLC26A4 mutation and current diagnosis of inner ear malformation in children with sensorineural hearing loss].

    Science.gov (United States)

    Sun, Baochun; Zhou, Chengyong; Dai, Zhiyao

    2014-11-01

    Explore the relationship between the pathogenic mutations of SLC26A4 gene and inner ear malformation, and analyze the feasibility of genetic testing to help current diagnosis in part of children with sensorineural hearing loss. 2094 cases of children were detected by SLC26A4 with the method of DNA sequence. CT phenotypes of those children were classified according to the method proposed by Sennaroglu. We analyzed the relationship between the pathogenic mutations of gene and the CT phenotypes. (1) 685 cases of inner ear malformations were found in 2094 cases of children with sensorineural hearing loss by CT examination (371 cases of cochlea malformation were consisted of the follow types of malformation. Michel deformity was 6 cases, cochlea aplasia was 8 cases, common cavity deformity was 12 cases, incomplete partition type I was 27 cases, cochlea hypoplasia was 30 cases and Mondini malformation was 288 cases); Vestibular aqueduct was 265 cases; Vestibular/semicircular canal/internal auditory canal were 49 cases, normal was 1409 cases. (2) The DNA sequence results revealed that 465 cases carried pathogenic mutations (Bi-allelic mutations) of SLC26A4 gene, among which 135 cases were homozygous, 330 cases were compound heterozygous. (3) Pathogenic mutations of SLC26A4 gene detected 100% (465/465) in the group related to vestibular aqueduct malformation. The results suggest that pathogenic mutation of SLC26A4 gene is closely related to the CT phenotype of vestibular aqueduct malformation. Detecting of pathogenic mutations for hearing loss is binging the possibility to identify children with inner malformations at an early stage. As a consequence, it will improve the current diagnosis and therapeutical option.

  3. 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

    Directory of Open Access Journals (Sweden)

    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. 

  4. [Aberrant topological properties of whole-brain functional network in chronic right-sided sensorineural hearing loss: a resting-state functional MRI study].

    Science.gov (United States)

    Zhang, Lingling; Liu, Bin; Xu, Yangwen; Yang, Ming; Feng, Yuan; Huang, Yaqing; Huan, Zhichun; Hou, Zhaorui

    2015-02-03

    To investigate the topological properties of the functional brain network in unilateral sensorineural hearing loss patients. In this study, we acquired resting-state BOLD- fMRI data from 19 right-sided SNHL patients and 31 healthy controls with normal hearing and constructed their whole brain functional networks. Two-sample two-tailed t-tests were performed to investigate group differences in topological parameters between the USNHL patients and the controls. Partial correlation analysis was conducted to determine the relationships between the network metrics and USNHL-related variables. Both USNHL patients and controls exhibited small-word architecture in their brain functional networks within the range 0. 1 - 0. 2 of sparsity. Compared to the controls, USNHL patients showed significant increase in characteristic path length and normalized characteristic path length, but significant decrease in global efficiency. Clustering coefficient, local efficiency and normalized clustering coefficient demonstrated no significant difference. Furthermore, USNHL patients exhibited no significant association between the altered network metrics and the duration of USNHL or the severity of hearing loss. Our results indicated the altered topological properties of whole brain functional networks in USNHL patients, which may help us to understand pathophysiologic mechanism of USNHL patients.

  5. Detection of Perinatal Cytomegalovirus Infection and Sensorineural Hearing Loss in Belgian Infants by Measurement of Automated Auditory Brainstem Response▿

    OpenAIRE

    Verbeeck, Jannick; Van Kerschaver, Erwin; Wollants, Elke; Beuselinck, Kurt; Stappaerts, Luc; Van Ranst, Marc

    2008-01-01

    Since auditory disability causes serious problems in the development of speech and in the total development of a child, it is crucial to diagnose possible hearing impairment as soon as possible after birth. This study evaluates the neonatal hearing screening program in Flanders, Belgium. The auditory ability of 118,438 babies was tested using the automated auditory brainstem response. We selected 194 babies with indicative hearing impairment and 332 matched controls to investigate the associa...

  6. Inter-trial coherence as a marker of cortical phase synchrony in children with sensorineural hearing loss and auditory neuropathy spectrum disorder fitted with hearing aids and cochlear implants

    Science.gov (United States)

    Nash-Kille, Amy; Sharma, Anu

    2014-01-01

    Objective Although brainstem dys-synchrony is a hallmark of children with auditory neuropathy spectrum disorder (ANSD), little is known about how the lack of neural synchrony manifests at more central levels. We used time-frequency single-trial EEG analyses (i.e., inter-trial coherence; ITC), to examine cortical phase synchrony in children with normal hearing (NH), sensorineural hearing loss (SNHL) and ANSD. Methods Single trial time-frequency analyses were performed on cortical auditory evoked responses from 41 NH children, 91 children with ANSD and 50 children with SNHL. The latter two groups included children who received intervention via hearing aids and cochlear implants. ITC measures were compared between groups as a function of hearing loss, intervention type, and cortical maturational status. Results In children with SNHL, ITC decreased as severity of hearing loss increased. Children with ANSD revealed lower levels of ITC relative to children with NH or SNHL, regardless of intervention. Children with ANSD who received cochlear implants showed significant improvements in ITC with increasing experience with their implants. Conclusions Cortical phase coherence is significantly reduced as a result of both severe-to-profound SNHL and ANSD. Significance ITC provides a window into the brain oscillations underlying the averaged cortical auditory evoked response. Our results provide a first description of deficits in cortical phase synchrony in children with SNHL and ANSD. PMID:24360131

  7. Biochemical alteration in children with idiopathic nephrotic syndrome associated with an increased risk of sensorineural hearing loss; additional insights in cochlear renal relationship.

    Science.gov (United States)

    El Mashad, Ghada Mohamed; Abo El Fotoh, Wafaa Moustafa M; Zein El Abedein, Ahmed Mahmoud; Abd El Sadek, Fatma Abd El Raoof

    2017-06-01

    Children with Idiopathic Nephrotic Syndrome (INS) are at risk of hearing loss due to the adverse impact of medications and related immunological and genetic factors on both cochlea and kidney. So this work was planned to evaluate hearing status in children with INS and to clarify the possible associated risk factors by interpreting the clinical and laboratory profiles of those children. Ninety children with INS aged 5-14 years [30 patients with steroid-sensitive nephrotic syndrome (SSNS), 30 patients with steroid dependent/frequently relapsing nephrotic syndrome (SDNS/FRNS), and 30 patients with steroid-resistant nephrotic syndrome (SRNS)], and 90 age and sex matched normal controls were enrolled into this study. Laboratory measurements of serum calcium, creatinine, cholesterol, blood urea and other relevant investigations were done. Pure tone audiometry was done with the sensory-neural hearing loss (SNHL) diagnosed when the level bone conduction was >20 dB and the difference in air to the bone gap was children with INS had SNHL, mostly of mild degree HL and primarily occurred at the lower frequencies. A highly significant statistical difference between controls and various types of nephrotic syndrome regarding pure tone audiometry measurements at frequencies 250, 500, 1000 Hz, whereas insignificant difference interpreting pure tone audiometry measurements in 2000, 4000 and 8000 Hz. Children with different phenotypes of nephrotic syndrome are at risk of sensorineural hearing impairment. The hazards associated with this impairment were higher blood pressure, hypercholesterolemia, hypoalbuminemia, and hypocalcemia. Copyright © 2017 Elsevier B.V. All rights reserved.

  8. Systematic review to evaluate the safety, efficacy and economical outcomes of the Vibrant Soundbridge for the treatment of sensorineural hearing loss.

    Science.gov (United States)

    Bruchhage, Karl-Ludwig; Leichtle, Anke; Schönweiler, Rainer; Todt, Ingo; Baumgartner, Wolf-Dieter; Frenzel, Henning; Wollenberg, Barbara

    2017-04-01

    Introduced in the late 90s, the active middle ear implant Vibrant Soundbridge (VSB) is nowadays used for hearing rehabilitation in patients with mild to severe sensorineural hearing loss (SNHL) unable to tolerate conventional hearing aids. In experienced hands, the surgical implantation is fast done, safe and highly standardized. Here, we present a systematic review, after more than 15 years of application, to determine the efficacy/effectiveness and cost-effectiveness, as well as patient satisfaction with the VSB active middle ear implant in the treatment of mild to severe SNHL. A systematic search of electronic databases, investigating the safety and effectiveness of the VSB in SNHL plus medical condition resulted in a total of 1640 papers. After removing duplicates, unrelated articles, screening against inclusion criteria and after in-depth screening, the number decreased to 37 articles. 13 articles were further excluded due to insufficient outcome data. 24 studies remained to be systematically reviewed. Data was searched on safety, efficacy and economical outcomes with the VSB. Safety-oriented outcomes included complication/adverse event rates, damage to the middle/inner ear, revision surgery/explant rate/device failure and mortality. Efficacy outcomes were divided into audiological outcomes, including hearing thresholds, functional gain, speech perception in quiet and noise, speech recognition thresholds, real ear insertion gain and subjective outcomes determined by questionnaires and patient-oriented scales. Data related to quality of life (QALY, ICER) were considered under economical outcomes. The VSB turns out to be a highly reliable and a safe device which significantly improves perception of speech in noisy situations with a high sound quality. In addition, the subjective benefit of the VSB was found to be mostly significant in all studies. Finally, implantation with the VSB proved to be a cost-effective and justified health care intervention.

  9. Tinnitus-related dissociation between cortical and subcortical neural activity in humans with mild to moderate sensorineural hearing loss

    NARCIS (Netherlands)

    Boyen, Kris; de Kleine, Emile; van Dijk, Pim; Langers, Dave R. M.

    Tinnitus is a phantom sound percept that is strongly associated with peripheral hearing loss. However, only a fraction of hearing-impaired subjects develops tinnitus. This may be based on differences in the function of the brain between those subjects that develop tinnitus and those that do not. In

  10. [Combined treatment supported by piracetam and/or acyclovir in idiopathic sudden sensorineural hearing loss: experience with 81 cases].

    Science.gov (United States)

    Karakurt, Süleyman Emre; Ozkul, Mehmet Doğan; Cukurova, Ibrahim; Demirhan, Erhan; Yiğitbaşi, Orhan Gazi

    2009-01-01

    To investigate the efficiency of piracetam and acyclovir in treating sudden hearing loss. Eightyone patients (44 males, 37 females; mean age 40.4 year; range 18 to 72 years) who had treatment between January 2002 and December 2006 with diagnosis of idiopathic sudden hearing loss were evaluated retrospectively. These patients were divided into four groups according to the treatment they received. The patients who had combined treatment constituted the first group; those who had combined treatment and piracetam the second; those who had combined treatment and acyclovir the third; those who had combined treatment, acyclovir, and piracetam the fourth group. For the four treatment groups, in the pre-and post-treatment (10th day) evaluation of the treatment efficiency made by calculation of the hearing thresholds in 250-8000 Hz frequencies, no significant difference between the groups was determined (p>0.05). No additional benefit was obtained with acyclovir and piracetam in treatment.

  11. Drug-induced Defibrinogenation as New Treatment Approach of Acute Hearing Loss in an Animal Model for Inner Ear Vascular Impairment.

    Science.gov (United States)

    Weiss, Bernhard G; Bertlich, Mattis; Bettag, Stephan A; Desinger, Hendrik; Ihler, Friedrich; Canis, Martin

    2017-06-01

    Disturbance of cochlear microcirculation is considered to be the final common pathway of various inner ear diseases. Hyperfibrinogenemia causing increased plasma viscosity is a known risk factor for sudden sensorineural hearing loss and may lead to a critical reduction of cochlear blood flow. The aim of this study was to evaluate the effect of a substantial reduction of plasma fibrinogen levels by drug-induced defibrinogenation for the treatment of acute hearing loss in vivo. Acute hearing loss was induced by hyperfibrinogenemia (i.v. injection of 330 mg/kg BW fibrinogen), using a guinea pig animal model. Parameters of cochlear microcirculation and hearing thresholds were quantified by intravital microscopy and evoked response audiometry. After obtaining baseline values, the course of hearing loss and disturbances of microcirculation were investigated under influence of intravenous defibrinogenation therapy (ancrod), corticosteroid, or placebo treatment, using 5 animals/group. Acute hyperfibrinogenemia caused hearing loss from 10 ± 7 to 26 ± 10 dB SPL at baseline. Drug-induced reduction of fibrinogen levels showed a significant increase of cochlear microcirculation (1.6-fold) and recovered hearing threshold (11 ± 6 dB SPL). Placebo or corticosteroid treatment had no effect on hearing loss (35 ± 7 dB SPL and 32 ± 18 dB SPL, respectively). Acute hyperfibrinogenemia resulted in hearing loss. Drug-induced reduction of elevated fibrinogen levels caused an increase in cochlear blood flow and a decrease in hearing thresholds. Placebo or corticosteroid treatment had no effect. Reduction of plasma fibrinogen levels could serve as a clinical treatment option for acute hearing loss.

  12. Alteration of frequency range for binaural beats in acute low-tone hearing loss.

    Science.gov (United States)

    Karino, Shotaro; Yamasoba, Tatsuya; Ito, Ken; Kaga, Kimitaka

    2005-01-01

    The effect of acute low-tone sensorineural hearing loss (ALHL) on the interaural frequency difference (IFD) required for perception of binaural beats (BBs) was investigated in 12 patients with unilateral ALHL and 7 patients in whom ALHL had lessened. A continuous pure tone of 30 dB sensation level at 250 Hz was presented to the contralateral, normal-hearing ear. The presence of BBs was determined by a subjective yes-no procedure as the frequency of a loudness-balanced test tone was gradually adjusted around 250 Hz in the affected ear. The frequency range in which no BBs were perceived (FRNB) was significantly wider in the patients with ALHL than in the controls, and FRNBs became narrower in the recovered ALHL group. Specifically, detection of slow BBs with a small IFD was impaired in this limited (10 s) observation period. The significant correlation between the hearing level at 250 Hz and FRNBs suggests that FRNBs represent the degree of cochlear damage caused by ALHL.

  13. A canonical splice site mutation in GIPC3 causes sensorineural hearing loss in a large Pakistani family

    NARCIS (Netherlands)

    Siddiqi, S.; Ismail, M.; Oostrik, J.; Munawar, S.; Mansoor, A.; Kremer, H.; Qamar, R.; Schraders, M.

    2014-01-01

    With homozygosity mapping we have identified two large homozygous regions on chromosome 3q13.11-q13.31 and chromosome 19p13.3-q31.32 in a large Pakistani family suffering from autosomal recessive nonsyndromic hearing impairment (arNSHI). The region on chromosome 19 overlaps with the previously

  14. Is sensorineural hearing loss a possible side effect of nasopharyngeal and parotid irradiation? A systematic review of the literature

    International Nuclear Information System (INIS)

    Raaijmakers, Esther; Engelen, Antoine M.

    2002-01-01

    Background and purpose: Little has been published about permanent hearing loss due to radiotherapy, thus making it a rather unknown phenomenon. Therefore, we performed a study of the literature over the last 20 years. Materials and methods: Sixteen relevant clinical studies were found, reporting mostly on nasopharyngeal or parotid gland treatments. Hearing loss was measured using a pure tone audiogram. Studies were assessed using a simple scoring list. Nine studies were used for further analysis. Data on the pure tone audiogram were pooled. Results: Results showed that, especially in the higher frequencies (≥4 kHz), loss can be measured. When data were pooled, in 42±3% of the patients a hearing loss was found of 10 dB or more at 4 kHz. Averaged over all measured frequencies the effect is less prominent but still statistically significant (18±2%). No significant difference between nasopharyngeal and parotid gland treatment was found (P<0.05). Conclusion: Only a few studies, mostly concerning small patient numbers, have investigated hearing damage due to radiotherapy. So far there has been no consensus on the subject. However, in this systematic review we found a significant effect. Dose to the inner ear therefore deserves more attention, especially in dose escalation studies and inverse planning

  15. Effect of edaravone on acute brainstem-cerebellar infarction with vertigo and sudden hearing loss.

    Science.gov (United States)

    Inoue, Yuta; Yabe, Takao; Okada, Kazunari; Nakamura, Yuka

    2014-06-01

    We report 2 cases with acute brainstem and brainstem-cerebellar infarction showed improvement of their signs and symptoms after administration of edaravone. Case 1, a 74-year-old woman who experienced sudden vertigo, also had dysarthria and left hemiplegia. Magnetic resonance imaging (MRI) showed an abnormal region in the right ventrolateral medulla oblongata. The patient's vertigo and hemiplegia improved completely after treatment. Case 2, a 50-year-old man who experienced sudden vertigo and sensorineural hearing loss (SNHL), developed dysarthria after admission. MRI revealed acute infarction in the right cerebellar hemisphere. Magnetic resonance angiography revealed dissection of the basilar artery and occlusion of the right anterior inferior cerebellar artery. The patient's vertigo and hearing remarkably improved. We have described 2 patients whose early symptoms were vertigo and sudden SNHL, but who were later shown to have ischemic lesions of the central nervous system. Edaravone is neuroprotective drug with free radical-scavenging actions. Free radicals in the ear are responsible for ischemic damage. Edaravone, a free radical scavenger, may be useful in the treatment of vertigo and SNHL. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

  16. 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

  17. Open-Fit Domes and Children with Bilateral High-Frequency Sensorineural Hearing Loss: Benefits and Outcomes.

    Science.gov (United States)

    Johnstone, Patti M; Yeager, Kelly R; Pomeroy, Marnie L; Hawk, Nicole

    2018-04-01

    Open-fit domes (OFDs) coupled with behind-the-ear (BTE) hearing aids were designed for adult listeners with moderate-to-severe bilateral high-frequency hearing loss (BHFL) with little to no concurrent loss in the lower frequencies. Adult research shows that BHFL degrades sound localization accuracy (SLA) and that BTE hearing aids with conventional earmolds (CEs) make matters worse. In contrast, research has shown that OFDs enhance spatial hearing percepts in adults with BHFL. Although the benefits of OFDs have been studied in adults with BHFL, no published studies to date have investigated the use of OFDs in children with the same hearing loss configuration. This study seeks to use SLA measurements to assess efficacy of bilateral OFDs in children with BHFL. To measure SLA in children with BHFL to determine the extent to which hearing loss, age, duration of CE use, and OFDs affect localization accuracy. A within-participant experimental design using repeated measures was used to determine the effect of OFDs on localization accuracy in children with BHFL. A between-participant experimental design was used to compare localization accuracy between children with BHFL and age-matched controls with normal hearing (NH). Eighteen children with BHFL who used CE and 18 age-matched NH controls. Children in both groups were divided into two age groups: older children (10-16 yr) and younger children (6-9 yr). All testing was done in a sound-treated booth with a horizontal array of 15 loudspeakers (radius of 1 m). The stimulus was a spondee word, "baseball": the level averaged 60 dB SPL and randomly roved (±8 dB). Each child was asked to identify the location of a sound source. Localization error was calculated across the loudspeaker array for each listening condition. A significant interaction was found between immediate benefit from OFD and duration of CE usage. Longer CE usage was associated with degraded localization accuracy using OFDs. Regardless of chronological age

  18. Interstitial keratitis and sensorineural hearing loss as a manifestation of rheumatoid arthritis: clinical lessons from a rare complication

    Science.gov (United States)

    Lee, Lennard YW; Akhtar, Mohammed Majid; Kirresh, Othman; Gibson, Terence

    2012-01-01

    Cogan's syndrome or non-syphilitic interstitial keratitis with vestibule-auditory dysfunction is a serious and under-recognised complication of rheumatoid arthritis. It is an autoimmune condition characterised by inflammatory infiltrates on the cornea and extensive vestibulocochlear damage. If left untreated, patients progress to develop profound hearing loss. We present a case that was incorrectly diagnosed and treated as conjunctivitis by several emergency departments prior to being correctly recognised as Cogan's syndrome. PMID:23242087

  19. Interstitial keratitis and sensorineural hearing loss as a manifestation of rheumatoid arthritis: clinical lessons from a rare complication

    OpenAIRE

    Lee, Lennard YW; Akhtar, Mohammed Majid; Kirresh, Othman; Gibson, Terence

    2012-01-01

    Cogan's syndrome or non-syphilitic interstitial keratitis with vestibule-auditory dysfunction is a serious and under-recognised complication of rheumatoid arthritis. It is an autoimmune condition characterised by inflammatory infiltrates on the cornea and extensive vestibulocochlear damage. If left untreated, patients progress to develop profound hearing loss. We present a case that was incorrectly diagnosed and treated as conjunctivitis by several emergency departments prior to being correct...

  20. Facial Nerve Schwannoma Involving Middle Cranial Fossa: When the Unilateral Sensorineural Hearing Loss Guide to the Correct Diagnosis

    OpenAIRE

    De Stefano, Alessandro; Dispenza, Francesco; Kulamarva, Gautham

    2011-01-01

    The Facial Nerve Schwannoma is a rare tumor and it seldom involved the middle cranial fossa. Facial nerve schwannoma has various manifestations, including facial palsy but unfortunately facial nerve is very resistant to compression and often facial nerve paralysis or a facial weakness are not present. We present a case of giant facial nerve schwannoma involved the middle cranial fossa without facial nerve paralysis. In these cases the unilateral hearing loss (if present) guide to a correct di...

  1. Effects of hearing aids in the balance, quality of life and fear to fall in elderly people with sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Lacerda, Clara Fonseca

    2012-01-01

    Full Text Available Introduction: The aging process provokes structural modifications and functional to it greets, compromising the postural control and central processing. Studies have boarded the necessity to identify to the harmful factors of risk to aged the auditory health and security in stricken aged by auditory deficits and with alterations of balance. Objective: To evaluate the effect of auditory prosthesis in the quality of life, the balance and the fear of fall in aged with bilateral auditory loss. Method: Carried through clinical and experimental study with 56 aged ones with sensorineural auditory loss, submitted to the use of auditory prosthesis of individual sonorous amplification (AASI. The aged ones had answered to the questionnaires of quality of life Short Form Health Survey (SF-36, Falls Efficacy International Scale- (FES-I and the test of Berg Balance Scale (BBS. After 4 months, the aged ones that they adapted to the use of the AASI had been reevaluated. Results: It had 50% of adaptation of the aged ones to the AASI. It was observed that the masculine sex had greater difficulty in adapting to the auditory device and that the variable age, degree of loss, presence of humming and vertigo had not intervened with the adaptation to auditory prosthesis. It had improvement of the quality of life in the dominance of the State General Health (EGS and Functional Capacity (CF and of the humming, as well as the increase of the auto-confidence after adaptation of auditory prosthesis. Conclusion: The use of auditory prosthesis provided the improvement of the domains of the quality of life, what it reflected consequently in one better auto-confidence and in the long run in the reduction of the fear of fall in aged with sensorineural auditory loss.

  2. Sudden bilateral sensorineural hearing loss after intravenous cocaine injection: a case report and review of the literature.

    Science.gov (United States)

    Stenner, Markus; Stürmer, Konrad; Beutner, Dirk; Klussmann, Jens Peter

    2009-12-01

    Little is known about the effects of intravenous abuse of cocaine, especially on the inner ear. We report on a 26-year-old man who presented to our outpatient department with a sudden severe hearing loss after intravenous injection of cocaine. The audiogram on admission showed symmetric air conduction levels up to 80 dB at 4 kHz. After treatment with intravenous sodium chloride, prednisolone, and pentoxifylline, the audiogram 2 days later showed a bilateral normacusis. A review of the literature on the topic is given and possible reasons for inner ear damages caused by cocaine are discussed.

  3. 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.

  4. AUDIOME: a tiered exome sequencing-based comprehensive gene panel for the diagnosis of heterogeneous nonsyndromic sensorineural hearing loss.

    Science.gov (United States)

    Guan, Qiaoning; Balciuniene, Jorune; Cao, Kajia; Fan, Zhiqian; Biswas, Sawona; Wilkens, Alisha; Gallo, Daniel J; Bedoukian, Emma; Tarpinian, Jennifer; Jayaraman, Pushkala; Sarmady, Mahdi; Dulik, Matthew; Santani, Avni; Spinner, Nancy; Abou Tayoun, Ahmad N; Krantz, Ian D; Conlin, Laura K; Luo, Minjie

    2018-03-29

    PurposeHereditary hearing loss is highly heterogeneous. To keep up with rapidly emerging disease-causing genes, we developed the AUDIOME test for nonsyndromic hearing loss (NSHL) using an exome sequencing (ES) platform and targeted analysis for the curated genes.MethodsA tiered strategy was implemented for this test. Tier 1 includes combined Sanger and targeted deletion analyses of the two most common NSHL genes and two mitochondrial genes. Nondiagnostic tier 1 cases are subjected to ES and array followed by targeted analysis of the remaining AUDIOME genes.ResultsES resulted in good coverage of the selected genes with 98.24% of targeted bases at >15 ×. A fill-in strategy was developed for the poorly covered regions, which generally fell within GC-rich or highly homologous regions. Prospective testing of 33 patients with NSHL revealed a diagnosis in 11 (33%) and a possible diagnosis in 8 cases (24.2%). Among those, 10 individuals had variants in tier 1 genes. The ES data in the remaining nondiagnostic cases are readily available for further analysis.ConclusionThe tiered and ES-based test provides an efficient and cost-effective diagnostic strategy for NSHL, with the potential to reflex to full exome to identify causal changes outside of the AUDIOME test.Genetics in Medicine advance online publication, 29 March 2018; doi:10.1038/gim.2018.48.

  5. 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

  6. A novel gene for Usher syndrome type 2: mutations in the long isoform of whirlin are associated with retinitis pigmentosa and sensorineural hearing loss.

    Science.gov (United States)

    Ebermann, Inga; Scholl, Hendrik P N; Charbel Issa, Peter; Becirovic, Elvir; Lamprecht, Jürgen; Jurklies, Bernhard; Millán, José M; Aller, Elena; Mitter, Diana; Bolz, Hanno

    2007-04-01

    Usher syndrome is an autosomal recessive condition characterized by sensorineural hearing loss, variable vestibular dysfunction, and visual impairment due to retinitis pigmentosa (RP). The seven proteins that have been identified for Usher syndrome type 1 (USH1) and type 2 (USH2) may interact in a large protein complex. In order to identify novel USH genes, we followed a candidate strategy, assuming that mutations in proteins interacting with this "USH network" may cause Usher syndrome as well. The DFNB31 gene encodes whirlin, a PDZ scaffold protein with expression in both hair cell stereocilia and retinal photoreceptor cells. Whirlin represents an excellent candidate for USH2 because it binds to Usherin (USH2A) and VLGR1b (USH2C). Genotyping of microsatellite markers specific for the DFNB31 gene locus on chromosome 9q32 was performed in a German USH2 family that had been excluded for all known USH loci. Patients showed common haplotypes. Sequence analysis of DFNB31 revealed compound heterozygosity for a nonsense mutation, p.Q103X, in exon 1, and a mutation in the splice donor site of exon 2, c.837+1G>A. DFNB31 mutations appear to be a rare cause of Usher syndrome, since no mutations were identified in an additional 96 USH2 patients. While mutations in the C-terminal half of whirlin have previously been reported in non-syndromic deafness (DFNB31), both alterations identified in our USH2 family affect the long protein isoform. We propose that mutations causing Usher syndrome are probably restricted to exons 1-6 that are specific for the long isoform and probably crucial for retinal function. We describe a novel genetic subtype for Usher syndrome, which we named USH2D and which is caused by mutations in whirlin. Moreover, this is the first case of USH2 that is allelic to non-syndromic deafness.

  7. Cost-effectiveness analysis of a non-contrast screening MRI protocol for vestibular schwannoma in patients with asymmetric sensorineural hearing loss

    International Nuclear Information System (INIS)

    Crowson, Matthew G.; Rocke, Daniel J.; Kaylie, David M.; Hoang, Jenny K.; Weissman, Jane L.

    2017-01-01

    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.)

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

    International Nuclear Information System (INIS)

    Choi, Yoon Jung; Park, Sang Yoo; Kim, Myung Soon; Sung, Ki Jun

    2004-01-01

    The purpose of this study is to evaluate the significance of the hypoplastic canal for the cochlear nerve in patients with sensorineural hearing loss (SNHL) and the relationship between the hypoplastic bony canal and aplasia or hypoplasia of the cochlear nerve. A retrospective review of high resolution temporal CT(HRCT) and MRI findings was conducted. The narrow bony canal of the cochlear nerve and the relative size of the internal auditory canal were correlated with the cochlear nerve deficiency on MRI. The comparative size of the component nerves (facial, cochlear, superior vestibular, inferior vestibular nerve), and the relative size of the internal auditory canal and the bony canal of the cochlear nerve were measured. The clinical history and the results of the clinical examination were reviewed for each patient. High resolution MRI showed aplasia of the common vestibulocochlear nerve in one patient and a deficiency of the cochlear nerve in 9 patients. These abnormalities occurred in association with a prominent narrowing of the canal for the cochlear nerve and a stenosis of the internal auditory canal, which was observed on temporal bone CT in 9 patients with congenital SNHL. Three patients had normal IAC, despite the presence of a hypoplastic cochlear nerve on the side on which they had SNHL. In one patient, the narrowing of the canal for the cochlear nerve and internal auditory canal were not found to be associated with acquired SNHL. The hypoplastic bony canal for the cochlear nerve might be more highly indicative of congenital cochlear nerve deficiency than that of the narrow internal auditory canal, and the position of the crista falciformis should also be carefully

  9. 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.

  10. 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.)

  11. Congenital malformations of the inner ear and the vestibulocochlear nerve in children with sensorineural hearing loss: evaluation with CT and MRI.

    Science.gov (United States)

    Westerhof, J P; Rademaker, J; Weber, B P; Becker, H

    2001-01-01

    The purpose of this work was to study the diagnostic value of CT and MRI in children with sensorineural hearing loss and to analyze anatomic abnormalities of the inner ear and the vestibulocochlear nerve in this patient group. We evaluated 42 inner ears in 21 children with congenital deafness who had congenital inner ear malformations and who were candidates for cochlear implants. All patients were studied with high resolution MR and helical CT examinations. The MR study included a T2-weighted 3D fast SE sequence. We describe and tabulate the anatomic abnormalities. Special attention was given to abnormalities of the vestibulocochlear nerve. The field of view in the plane according to the length axis of the internal auditory canal (IAC) was 4 cm. Additional continuous parasagittal reformations perpendicular to the length axis of the IAC were studied with a field of view of 3 cm. CT and MRI allowed accurate identification of malformations of the inner ear in children with congenital deafness. We identified 99 malformations, with a majority of patients demonstrating multiple abnormalities. Common imaging findings were Mondini abnormality and Mondini variants (12/42) and fusion of the lateral or superior semicircular canal with the vestibule (12/42). MRI demonstrated in 9 of 21 patients a rudimentary or absent vestibulocochlear nerve in the auditory canal. CT and MRI are important modalities to analyze the inner ear in children who are candidates for cochlear implants. MRI with an extremely small field of view should be used to study possible abnormalities of the vestibulocochlear nerves. This may alter clinical care and allow cochlear implant placement in patients whose electrodiagnostic studies suggest that the implant should not be performed. The detailed analysis of abnormalities of the inner ear might establish prognostic factors.

  12. [Brain plastic alterations in subjects with chronic right-sided sensorineural hearing loss: a resting-state MRI study].

    Science.gov (United States)

    Zhang, L L; Gong, J P; Xu, Y W; Liu, B

    2016-06-21

    To investigate the nodal properties and reorganization of whole-brain functional network in subjects with severe right-sided SNHL. From June 2012 to June 2013, a total of 19 patients with severe right-sided SNHL were collected from Zhongda Hospital or the recruitment advertising along with 31 healthy controls.Based on the graph-theoretical analysis, the whole-brain functional networks were constructed using the BOLD-fMRI data of all subjects.Two sample two-tailed t-tests were used to investigate the differences between two groups in nodal metrics, such as node degree, node betweenness, node global efficiency and node local efficiency.All metrics were corrected by multiple comparisons.Partial correlation analysis was used to estimate the relationship between the significant metrics and the duration or severity of hearing loss. The right-sided SNHL showed significantly increased betweenness centrality in left supramarginal gyrus and right fusiform.However, other nodal parameters showed no statistical difference.Besides, patients exhibited no significant association between the altered metrics and clinical variables. Alterations of local topological properties may underlie cerebral cross-modal plastic reorganization in visual or speech-related regions in severe right-sided SNHL patients.

  13. Vestibular Evoked Myogenic Potential Produced by Bone-Conducted Stimuli: A Study on its Basics and Clinical Applications in Patients with Conductive and Sensorineural Hearing Loss and a Group with Vestibular Schawannoma.

    Science.gov (United States)

    Mahdi, Parvane; Amali, Amin; Pourbakht, Akram; Karimi Yazdi, Alireza; Bassam, Ali

    2013-06-01

    Vestibular evoked myogenic potential (VEMP) has recently been broadly studied in vestibular disorders. As it is evoked by loud sound stimulation, even mild conductive hearing loss may affect VEMP results. Bone-conducted (BC) stimulus is an alternative stimulation for evoking this response. This study aims to assess the characteristics of BC-VEMP in different groups of patients. We performed a cross sectional analysis on 20 healthy volunteers with normal pure-tone audiometry as a control group; and on a group of patients consisted of 20 participants with conductive hearing loss, five with bilateral sensorineural hearing loss and four with vestibular schawannoma. AC and BC-VEMP were performed in all participants. In control group the VEMP responses to both kinds of stimuli had an acceptable morphology and consisted of p13 and n23 waves. Latency value of these main components in each type of stimulus was not significantly different (P>0.05). However, the mean amplitude was larger in BC modality than AC stimulation (P=0.025). In the group with conductive hearing loss, the VEMP response was absent in fifteen (46.87%) of the 32 ears using the AC method, whereas all (100%) displayed positive elicitability of VEMP by BC method. Normal VEMP responses in both stimuli were evoked in all patients with sensorineural hearing loss. In patients with unilateral vestibular schwannomas (VS), 2 (50.00%) had neither AC-VEMP nor BC-VEMP. Auditory stimuli delivered by bone conduction can evoke VEMP response. These responses are of vestibular origin and can be used in vestibular evaluation of patients with conductive hearing loss.

  14. 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.

  15. Cogan's syndrome mimicking acute Lyme arthritis.

    Science.gov (United States)

    Schwegmann, J P; Enzenauer, R J

    1995-05-01

    A pediatric case of Cogan's syndrome mimicking acute Lyme arthritis is described. A 12-year-old black boy was admitted to the pediatric service for presumed right knee septic arthritis. Symptoms included acute pain and swelling with decreased range-of-motion. Although the patient's right knee symptoms and positive Lyme serology were consistent with a diagnosis of Lyme arthritis, the presence of sensorineural hearing loss and interstitial keratitis with inflammatory arthritis suggested a diagnosis of Cogan's syndrome. Subsequent Western blot analysis was negative for Borrelia burgdorferi antigens. The patient had dramatic clinical improvement of musculoskeletal and ophthalmologic complaints shortly after receiving high-dose corticosteroids, although residual sensorineural hearing loss persisted.

  16. [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.

  17. Potencial evocado auditivo de tronco encefálico por via óssea em indivíduos com perda auditiva sensorioneural Brainstem auditory evoked potential in subjects with sensorineural hearing losses

    Directory of Open Access Journals (Sweden)

    Luciana Castelo Branco Camurça Fernandes

    2012-01-01

    Full Text Available OBJETIVO: caracterizar os resultados do PEATE por via óssea em indivíduos com perda auditiva sensorioneural leve, comparando esses dados com o grupo controle, formado por indivíduos audiologicamente normais. MÉTODO: a amostra foi constituída por 40 adultos, de ambos os sexos, com faixa etária de 18 a 55 anos, distribuídos em um grupo controle de 30 indivíduos com audição normal e um grupo estudo composto de 10 indivíduos com perda auditiva sensorioneural de grau leve. O PEATE foi realizado com equipamento EP15, da marca Interacoustics. O estímulo utilizado foi o clique com taxa de apresentação de 27,7/s, em um total de 2000 estímulos, com polaridade de rarefação por VA e alternada para VO e filtro passa-banda de 50Hz e 3000Hz. RESULTADOS: em indivíduos com perda sensorioneural de grau leve, não houve diferenças estatisticamente significantes entre o limiar do PEATE por via aérea e óssea, estando esses limiares equivalentes, com GAP aéreo-ósseo menor que 10dB. A latência da onda V no limiar eletrofisiológico e a 50 dBnNA foram menores que as referidas latências observadas em indivíduos com audição normal. CONCLUSÃO: foram encontrados limiares eletrofisiológicos por via óssea equivalente aos limiares obtidos por via aérea, com presença de GAP aéreo-ósseo menor que 10dBnNA. Assim a utilização do PEATE por VO fornece dados para uma caracterização mais detalhada do tipo da perda auditiva.PURPOSE: to characterize the results of ABR via bone in subjects with mild sensorineural hearing loss, comparing these data with the control group made up by subjects with normal hearing. METHOD: the sample consisted of 40 adults of both genders, 18 - 55 year old, divided into a control group of 30 subjects with normal hearing and a study group made up of 10 subjects with mild sensorineural hearing loss. ABR was carried out with Interacoustics brand EP15. The stimulus was the click presentation rate of 27.7 / s, for a total of

  18. [A comparative study on efficacy of glucocorticoids, mineralocorticoids and vasoactive drugs on reversing hearing loss in patients suffering idiopathic sensorineural cochlear hypoacusis. A preliminary clinical trial].

    Science.gov (United States)

    Campos-Bañales, Eugenia María; López-Campos, Daniel; de Serdio-Arias, José Luis; Esteban-Rodriguez, J; García-Sáinz, Mar; Muñoz-Cortés, Álvaro; López-Aguado, Daniel

    2015-01-01

    Sensory neural hearing loss (SNHL) is a disorder characterised by an important deterioration of the auditory function. Re-establishing normal ion homeostasis of the endolymph could be related to hearing recovery and it might be mediated by mineralocorticoids. The main purpose of this preliminary, randomized controlled clinical trial was assessing the recovery of idiopathic sensory neural cochlear hearing loss (SNHL) by comparing the efficacy of 2 types of steroids versus vasodilators. The 3-month intervention involved 70 patients, allocated into 4 different groups: a control with no medication, consisting of 14 patients (8 men and 6 women); a vasodilator group of 21 patients (11 men and 10 women); a glucocorticoid group with 16 patients (10 men and 6 women); and a mineralocorticoid therapy group, consisting of 19 patients (11 men and 8 women). The level of hearing loss and its topography were estimated using Liminal Tone Audiometry (LTA) and Auditory Brainstem Response (ABR). Our research found overall greater efficacy of mineralocorticoids versus glucocorticoids and vasodilators. There was better response in women than in men and it was higher from the left ear, regardless of patient gender. The hearing gain was significantly superior in the mineralocorticoid group, followed by the glucocorticoid group. However, the responses to vasodilators were lesser and of low statistical significance. Copyright © 2014 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

  19. Reconhecimento de fala no nível de máximo conforto em pacientes adultos com perda auditiva neurossensorial Speech recognition in the maximum comfort level in adults with sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Zuleica Costa Zaboni

    2009-01-01

    Full Text Available OBJETIVO: Pesquisar o Índice Percentual de Reconhecimento de Fala (IPRF no nível de máximo de conforto em adultos com perda auditiva neurossensorial de grau leve a moderadamente severo até 60 dB NA. MÉTODOS: Os indivíduos avaliados foram agrupados de acordo com o grau de perda auditiva (Grupos I, II e III. Feito isso, os grupos foram subdivididos (IA, IB, IIA, IIB, IIIA, IIIB conforme explicado a seguir. No subgrupo A de cada um dos grupos, foi determinado o IPRF a 40 dB NS, obtendo-se as respostas iniciando-se pela orelha direita. O paciente foi solicitado a informar quão confortável estava o som neste nível de apresentação dos estímulos, utilizando-se para obter a informação, uma escala com quatro descritores: baixo, confortável, alto e alto demais. Depois disso, foi pesquisado o nível de máximo conforto (NMC e nessa intensidade foi obtido o IPRF. No subgrupo B de cada um dos grupos, foi realizado o mesmo procedimento, porém obtendo-se primeiramente o IPRF no NMC, e a seguir a 40 dB NS. Nesse subgrupo, o teste foi iniciado na orelha esquerda. RESULTADOS: Após a avaliação dos indivíduos dos três grupos foi calculado o nível de audição médio em que os indivíduos referiram maior conforto que variou de 25 a 32,95 dB NS. No NMC de apresentação dos estímulos, houve maior percentagem de acertos de palavras. CONCLUSÃO: A avaliação do IPRF no nível de máximo conforto para indivíduos com perda auditiva neurossensorial de grau leve a moderadamente severo, proporciona melhores resultados de Reconhecimento de Fala.PURPOSE: To obtain the Percentage Index of Speech Recognition (PISR at maximum comfortable level (MCL in adults with mild to moderately severe (up to 60 dB sensorineural hearing loss. METHODS: The subjects evaluated were grouped according to the degree of hearing loss (Groups I, II and III. The groups were further divided into subgroups (IA,IB, IIA, IIB, IIIA, IIIB as it follows. In the subgroup A of each

  20. Clinical and logopaedic results of simultaneous and sequential bilateral implants in children with severe and/or profound bilateral sensorineural hearing loss: A literature review.

    Science.gov (United States)

    López-Torrijo, Manuel; Mengual-Andrés, Santiago; Estellés-Ferrer, Remedios

    2015-06-01

    This article carries out a literature review of the advantages and limitations of the simultaneous bilateral cochlear implantation (SCI) compared to those of the sequential bilateral cochlear implantation (SBCI) and the unilateral cochlear implantation (UCI). The variables analysed in said comparison are: safety and surgical technique, SCI incidence, effectiveness, impact of the inter-implant interval, costs and financing, impact on brain plasticity, impact on speech and language development, main benefits, main disadvantages and concerns, and predictive factors of prognosis. Although the results are not conclusive, all variables analysed seem to point towards observable benefits of SCI in comparison with SBCI or UCI. This tendency should be studied in more depth in multicentre studies with higher methodological rigour, more comprehensive samples and periods and other determining variables (age at the time of implantation, duration and degree of the hearing loss, rehabilitation methodologies used, family involvement, etc.). Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  1. [Intratympanic injection therapy for therapy refractory acute hearing loss: A safe option for secondary treatment].

    Science.gov (United States)

    Mühlmeier, G; Maier, S; Maier, M; Maier, H

    2015-10-01

    High-dose corticosteroids are currently recommended for idiopathic sudden sensorineural hearing loss (ISSNHL) treatment. Intratympanic injections (ITI) are of growing importance, especially in cases of therapy resistance. The selection of patients for this procedure in SSNHL has not been adequately examined so far. A total of 77 patients with ISSNHL after ineffective systemic pretreatment underwent intratympanic administration of dexamethasone and hyaluronic acid. Improvement after treatment was determined by pure tone audiometry for both ears before and of the treated ear after ITI. In this study 34 female and 43 male patients with mean age of 57 years showed a pre-ITI hearing loss of 35 dB in the lower frequencies and 69 dB in the higher frequencies. The mean hearing gain was 10 dB and the response rate was 62%. Absolute hearing gain revealed significant improvements at 500 Hz, 1 kHz and 2 kHz. Under inclusion of contralateral thresholds there were hardly any differences up to 4 kHz. In a detailed analysis of responders moderate improvements could be observed even in higher frequencies. Overall, no relevant adverse events occurred. Treatment of ISSNHL resistant to systemic regimens by ITI of steroids provides an option that offers additional prospects of auditory improvement for affected patients. The presented results indicate that these modalities are also valid for patients with pancochlear ISSNHL.

  2. Вікові особливості механічної памяті у школярів з нейросенсорною приглухуватістю = Age features of mechanical memory in schoolchildren with sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Mykola Klishch

    2016-08-01

    AGE FEATURES OF MECHANICAL MEMORY IN SCHOOLCHILDREN WITH SENSORINEURAL HEARING LOSS I.Horbachevsky Ternopil State Medical University (Ternopil Summary. We studied indices of short-term mechanical memory for figures, numbers, syllables and geometric figures in schoolchildren of different age periods (elementary school-age – 7-10 years, middle school-age – 11-14 years, senior school-age – 15-17 years with sensorineural hearing loss and normal hearing. For this purpose we used package "Memory" of computer diagnostic complex "Effecton Studio". It was found out that with age indices of verbal (figures, numbers, syllables and non-verbal (images memory increase in both study groups, but in the group of schoolchildren with sensorineural hearing loss of all age periods indices of non-verbal memory were significantly lower, and indices of verbal memory – significantly higher than in schoolchildren with normal hearing. It was concluded that children with hearing derivation remember images better than children with normal hearing and have well-developed short-term visual memory for non-verbal stimuli, which can be used in planning of remedial work. Keywords. Sensorineural hearing loss, memory, age features.

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

    African Journals Online (AJOL)

    Again, the occurrence of Sensorineural Hearing Loss was more than other types of hearing loss. Noise, Fever, Presbycusis, Sickness, Meningitis and Meniere\\'s diseases were the major causes of Sensorineural Hearing Loss. Conductive Hearing Loss was attributed in the main to Wax, Foreign Bodies, Otitis Media, and ...

  4. 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.

  5. 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

  6. 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....

  7. Epidemiological and clinical aspects of ear nose and throat sensorineural emergencies in the Yaoundé reference hospital.

    Science.gov (United States)

    Djomou, François; Nkouo, Yves Christian Andjock; Mindja, Eko David; Nchinda, Choffor; Meka, Luc; Mbamyah-Lyonga, Emilia; Ndjolo, Alexis

    2016-01-01

    Sensorineural emergencies (SNE) are rare clinical situations. Few patients consult early explaining subsequent difficulty in having accurate data and management. Three clinical conditions are considered SNE in otolaryngology; they include sudden sensorineural hearing loss (SSHL), Bell's palsy and acute vertigo. There is very little data available on sensorineural emergencies in our setting. The aim of this study was to provide preliminary data on the management of Ear Nose and Throat (ENT) sensorineural emergency cases in Yaoundé Reference Hospital. A descriptive retrospective study was carried out based on data collected over a period of 5 years, January 2010 to July 2014 at the Yaoundé Reference Hospital. Information was obtained from patients' files collected from the archives of the institution. Patients presenting with SSHL, Bell's palsy, acute vertigo who consulted during the study period were included in the study. A total of 22 patients were included in the study out of 6406 patients who consulted at the ENT Unit. The prevalence of SNE in ENT consultations was 0.003, distributed as follows; 13 patients (59.1%) of SNE had Bell's palsy, seven (31.8%) had vestibular neuritis and two (9.1%) had SSHL. The prevalence of SNE was low with idiopathic Bell's palsy being the most frequent. There was a general delay in arrival of patients hence delay in diagnosis. This delay could equally be a factor for treatment failure and poor prognosis. More effort should be made in terms of population sensitization about the necessity of getting early medical attention.

  8. 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

  9. Perda auditiva sensório-neural na otite média crônica supurativa em pacientes com e sem colesteatoma Sensorineural hearing loss in chronic suppurative otitis media with and without cholesteatoma

    Directory of Open Access Journals (Sweden)

    Alexandre Fernandes de Azevedo

    2007-10-01

    Full Text Available Perda auditiva sensório-neural (PASN relacionada a otite média crônica supurativa (OMCS foi estudada para esclarecer a participação do colesteatoma nesse contexto. OBJETIVO: Avaliar ocorrência de PASN na OMCS, correlacionando com colesteatoma, duração da doença e idade. CASUÍSTICA E MÉTODOS: Estudo retrospectivo de 115 pacientes com OMCS com e sem colesteatoma submetidos à cirurgia. Incluíram-se pacientes com doença unilateral, orelha contralateral normal e idade inferior a 60 anos. RESULTADOS: Idade média foi de 26 anos, sendo 58 homens e 57 mulheres. Tempo médio de duração da doença otológica de 12,4 anos. Limiar auditivo médio foi de 40 dB na orelha com OMCS e 22dB na orelha normal (P=0,002. Observou-se colesteatoma em 78 dos 115 casos. Na orelha com OMCS, ocorreram 15 (13% casos de PASN, sendo 7 associadas à colesteatoma e 8 não associadas. Seis casos de PASN foram severa/profunda, correlacionando-se com idade ajustada (P=0,003, ausência de colesteatoma (P=0,01, mas não com duração da doença (P=0,458. CONCLUSÃO: PASN ocorreu em 13% dos pacientes com OMCS, correlacionando-se com o aumento da idade, mas não com a presença de colesteatoma ou com maior duração da doença otológica.Sensorineural hearing loss (SNHL related to chronic suppurative otitis media (CSOM was studied to clarify the involvement of cholesteatomas in this context. AIM: to evaluate SNHL related to CSOM and its association with cholesteatomas, disease duration and patients’ ages. METHODS: Retrospective analysis of 115 patients with CSOM with and without cholesteatoma submitted to surgical treatment. Inclusion criteria were active unilateral disease, normal contralateral ear and age below 60 years. RESULTS: The average age was 26.3 years, 58 males and 57 females. The duration of ear disease was, in average, 12.4 years. The average threshold of hearing was 40 dB in CSOM ear and 22 dB in the normal contralateral ear (P=0.002. CSOM with

  10. Behavioral training promotes multiple adaptive processes following acute hearing loss.

    Science.gov (United States)

    Keating, Peter; Rosenior-Patten, Onayomi; Dahmen, Johannes C; Bell, Olivia; King, Andrew J

    2016-03-23

    The brain possesses a remarkable capacity to compensate for changes in inputs resulting from a range of sensory impairments. Developmental studies of sound localization have shown that adaptation to asymmetric hearing loss can be achieved either by reinterpreting altered spatial cues or by relying more on those cues that remain intact. Adaptation to monaural deprivation in adulthood is also possible, but appears to lack such flexibility. Here we show, however, that appropriate behavioral training enables monaurally-deprived adult humans to exploit both of these adaptive processes. Moreover, cortical recordings in ferrets reared with asymmetric hearing loss suggest that these forms of plasticity have distinct neural substrates. An ability to adapt to asymmetric hearing loss using multiple adaptive processes is therefore shared by different species and may persist throughout the lifespan. This highlights the fundamental flexibility of neural systems, and may also point toward novel therapeutic strategies for treating sensory disorders.

  11. Bilateral sudden sensorineural deafness with vertigo as the sole presenting symptoms of diabetes mellitus — a case report

    OpenAIRE

    Misra, Vilas; Agarwal, C. G.; Bhatia, Naresh; Shukla, G. K.

    2010-01-01

    This Paper reports a late uncontrolled diabetic presenting to an otolaryngologist with sudden severe sensorineural hearing loss of immediate origin with vertigo and tinnitus as the symptoms. Appropriate investigative and treatment measure resulted in deterioration of hearing in the right ear and mild improvement of hearing in the left ear, with no recovery of imbalance.

  12. Bilateral sudden sensorineural deafness with vertigo as the sole presenting symptoms of diabetes mellitus - a case report.

    Science.gov (United States)

    Misra, Vilas; Agarwal, C G; Bhatia, Naresh; Shukla, G K

    2010-06-01

    This Paper reports a late uncontrolled diabetic presenting to an otolaryngologist with sudden severe sensorineural hearing loss of immediate origin with vertigo and tinnitus as the symptoms. Appropriate investigative and treatment measure resulted in deterioration of hearing in the right ear and mild improvement of hearing in the left ear, with no recovery of imbalance.

  13. Long-term hearing outcomes after recurrent acute otitis media during early childhood.

    Science.gov (United States)

    Krakau, Mattias; Dagöö, Britta Rynnel; Hellström, Sten; Granath, Anna

    2017-12-01

    To survey long-term hearing outcomes and middle ear pathology in a 30-year follow-up in individuals with onset of recurrent acute otitis media (rAOM) before three years of age. 28 adults, aged 30.1-31.8 years, who originally - at the age of 12-32 months - participated in a study on rAOM between 1979 and 1983, were re-examined regarding self-reported ear problems, current tympanic membrane changes and audiology. Thirteen subjects had suffered from rAOM during early childhood and 15 subjects served as a control group. Recurrent acute otitis media subjects reported hearing problems comparable to those of the controls. Pure tone audiometry, at 125-8000 Hz, did not differ between groups. The rAOM group had a trend for impaired high-frequency (9000-14,000 Hz) threshold levels (9000-14,000 Hz); implying that their cochlear function seemed to have deteriorated. Adults, who suffered from recurrent acute otitis media as infants, did not show any clinically significant hearing loss for pure tone audiometry when compared to controls, but there was a trend for impaired results regarding extended high frequency audiometry (9-14 kHz). Children suffering from rAOM will be at low risk of developing hearing loss and severe middle ear disease.

  14. 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.

  15. Prevalence of occupational noise induced hearing loss amongst traffic police personnel

    OpenAIRE

    Singh, V K; Mehta, A K

    1999-01-01

    Traffic branch personnel of Pune traffic police were screened for presence of noise induced hearing loss. A very significant number (81.2%) showed sensorineural hearing loss. The various factors responsible for noise induced hearing loss are discussed.

  16. 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…

  17. 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

  18. Hearing impairment in genotyped Wolfram syndrome patients.

    NARCIS (Netherlands)

    Plantinga, R.F.; Pennings, R.J.E.; Huygen, P.L.M.; Bruno, R.; Eller, P.; Barrett, T.G.; Vialettes, B.; Paquis-Fluklinger, V.; Lombardo, F.; Cremers, C.W.R.J.

    2008-01-01

    OBJECTIVES: Wolfram syndrome is a progressive neurodegenerative syndrome characterized by the features "DIDMOAD" (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness). We sought to study the audiometric data of genotyped Wolfram syndrome patients with sensorineural hearing impairment.

  19. Genes and Syndromic Hearing Loss.

    Science.gov (United States)

    Keats, Bronya J. B.

    2002-01-01

    This article provides a description of the human genome and patterns of inheritance and discusses genes that are associated with some of the syndromes for which hearing loss is a common finding, including: Waardenburg, Stickler, Jervell and Lange-Neilsen, Usher, Alport, mitochondrial encephalomyopathy, and sensorineural hearing loss. (Contains…

  20. 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.

  1. 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

  2. Clinical and audiologic characteristics of patients with sensorineural tinnitus and its association with psychological aspects: an analytic retrospective study.

    Science.gov (United States)

    Al-Swiahb, Jamil Nasser; Hwang, Eul Seung; Kong, Ji Sun; Kim, Woo Jin; Yeo, Sang Won; Park, Shi Nae

    2016-12-01

    This study was performed to analyze clinical and audiologic characteristics of sensorineural tinnitus and to investigate the associating factors reflecting psychological aspects of stress and depression of the patients. This is a retrospective analytical study conducted in a tinnitus clinic of a tertiary referral center of a university hospital. The medical records of 216 patients suffering from sensorineural tinnitus were thoroughly evaluated to determine correlations between clinical and audiological characteristics, including age, sex, predisposing or etiologic factors, hearing levels up to extended high frequencies, and tinnitus severity. Psychological aspects of stress and depression were also evaluated and analyzed to seek the associations with tinnitus severity. All data were stored in our database bank and were statistically analyzed. Our study subjects showed a slight male predominance. The highest percentage of tinnitus was found in patients of 60-80 years old. Only 32.5 % of tinnitus patients were subjectively aware of their hearing loss, whereas 73 % of subjects had hearing deficits in some frequencies in their audiogram. Hearing impairments were of the low-frequency sensorineural type in 18.2 % of patients and were limited to the high frequencies in 77.9 % of patients. Tinnitus was unilateral in 51 % of patients and had a tonal nature in 45 % of patients. In total, 45.8 % of patients with high-frequency sensorineural hearing loss had high-pitched tinnitus. There were significant correlations between tinnitus severity, loudness and annoyance. Correlations with THI (Tinnitus Handicap Inventory) and Beck depression index scores were also found. Sensorineural tinnitus was related with hearing loss in some frequencies nevertheless of patients' own awareness of hearing loss. Loudness and annoyance of tinnitus seems to be two important factors reflecting psychological problems of patients' stress and depression.

  3. Current amplification models of sensorineurall and conductive hearing loss

    OpenAIRE

    Ostojić, Sanja; Mikić, Branka; Mirić, Danica

    2012-01-01

    The main function of a hearing aid is to improve auditory and language abilities of hearing impaired users. The amplification model has to be adapted according to age, degree and type of hearing loss. The goal of this paper is to analyze the current amplification models of sensorineural and conductive hearing loss which can provide a high quality of speech perception and sounds at any degree of hearing loss. The BAHA is a surgically implantable system for treatment of conductive hearing loss ...

  4. Effects of cranial radiation on hearing in children with acute lymphocytic leukemia

    International Nuclear Information System (INIS)

    Thibadoux, G.M.; Pereira, W.V.; Hodges, J.M.; Aur, R.J.

    1980-01-01

    The hearing sensitivity of 61 children with acute lymphocytic leukemia who were admitted to our Total Therapy IX study between December 1975 and July 1977 was studied. Their treatment included combined chemotherapy, 2400 rads of cranial radiation, and intrathecal methotrexate. Subjects initially received an otologic examination and middle ear function testing. Audiometric testing was not done until ears were free of outer or middle ear pathology. If the child had no outer or middle ear disease, audiometric thresholds were obtained for the test frequencies: 500, 1000, 2000, 4000, 6000, and 8000 Hz. Pure-tone thresholds were obtained before irradiation (61 patients) and at 6, 12, and 36 months thereafter (49, 46, and 22 patients, respectively). The median age of time of baseline testing was 10 years, 2 months. A paired sample test based on group data was used to test whether there were any significant changes from the threshold values at 6, 12, and 36 months after irradiation. Thresholds were not significantly affected for any test frequency at any test time. Assessments of individual audiograms indicated that none of the children had any significant reductions in hearing levels at the end of the third year after cranial irradiation

  5. Auditory hair cell defects as potential cause for sensorineural deafness in Wolf-Hirschhorn syndrome.

    Science.gov (United States)

    Ahmed, Mohi; Ura, Kiyoe; Streit, Andrea

    2015-09-01

    WHSC1 is a histone methyltransferase (HMT) that catalyses the addition of methyl groups to lysine 36 on histone 3. In humans, WHSC1 haploinsufficiency is associated with all known cases of Wolf-Hirschhorn syndrome (WHS). The cardinal feature of WHS is a craniofacial dysmorphism, which is accompanied by sensorineural hearing loss in 15% of individuals with WHS. Here, we show that WHSC1-deficient mice display craniofacial defects that overlap with WHS, including cochlea anomalies. Although auditory hair cells are specified normally, their stereocilia hair bundles required for sound perception fail to develop the appropriate morphology. Furthermore, the orientation and cellular organisation of cochlear hair cells and their innervation are defective. These findings identify, for the first time, the likely cause of sensorineural hearing loss in individuals with WHS. © 2015. Published by The Company of Biologists Ltd.

  6. Auditory hair cell defects as potential cause for sensorineural deafness in Wolf-Hirschhorn syndrome

    Directory of Open Access Journals (Sweden)

    Mohi Ahmed

    2015-09-01

    Full Text Available WHSC1 is a histone methyltransferase (HMT that catalyses the addition of methyl groups to lysine 36 on histone 3. In humans, WHSC1 haploinsufficiency is associated with all known cases of Wolf-Hirschhorn syndrome (WHS. The cardinal feature of WHS is a craniofacial dysmorphism, which is accompanied by sensorineural hearing loss in 15% of individuals with WHS. Here, we show that WHSC1-deficient mice display craniofacial defects that overlap with WHS, including cochlea anomalies. Although auditory hair cells are specified normally, their stereocilia hair bundles required for sound perception fail to develop the appropriate morphology. Furthermore, the orientation and cellular organisation of cochlear hair cells and their innervation are defective. These findings identify, for the first time, the likely cause of sensorineural hearing loss in individuals with WHS.

  7. [Cochlear implantation in a child with congenital sensorineural deafness due to 35 DELG mutation in GJB2 (connexin 26) gene].

    Science.gov (United States)

    Teriutin, F M; Barashkov, N A; Dzhemileva, L U; Posukh, O L; Fedotova, E E; Gurinova, E E; Fedorova, S A; Tavartkiladze, G A; Khusnutdinova, E K

    2009-01-01

    This paper reports the first case of cochlear implantation performed in this country in a child with congenital non-syndromic sensorineural loss of hearing having hereditary etiology and attributable to autosomal-recessive 35 delG mutation in locus DFNB1 (13q.11-q12) of GJB2 (connexin 26) gene.

  8. Acute necrosis after Gamma Knife surgery in vestibular schwannoma leading to multiple cranial nerve palsies.

    Science.gov (United States)

    Kapitza, Sandra; Pangalu, Athina; Horstmann, Gerhard A; van Eck, Albert T; Regli, Luca; Tarnutzer, Alexander A

    2016-08-01

    We discuss a rare acute complication after Gamma Knife therapy (Elekta AB, Stockholm, Sweden) in a single patient. A 52-year-old woman presented with vertigo, facial weakness and hearing loss emerging 48hours following Gamma Knife radiosurgery for a right-sided vestibular schwannoma. Neurological examination 6days after symptom onset showed right-sided facial palsy, spontaneous left-beating nystagmus and pathologic head-impulse testing to the right. Pure-tone audiogram revealed right-sided sensorineural hearing loss. A diagnosis of acute vestibulocochlear and facial neuropathy was made. Brain MRI demonstrated focal contrast sparing within the schwannoma, likely related to acute radiation necrosis. Acute multiple cranial neuropathies of the cerebellopontine angle after Gamma Knife treatment should raise suspicion of acute tissue damage within the schwannoma and should result in urgent MRI. Treatment with steroids may be considered based on accompanying swelling and edema. Copyright © 2016 Elsevier Ltd. All rights reserved.

  9. 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.

  10. 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.......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...

  11. 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...

  12. Classification of Hearing Loss Disorders Using Teoae-Based Descriptors

    Science.gov (United States)

    Hatzopoulos, Stavros Dimitris

    Transiently Evoked Otoacoustic Emissions (TEOAE) are signals produced by the cochlea upon stimulation by an acoustic click. Within the context of this dissertation, it was hypothesized that the relationship between the TEOAEs and the functional status of the OHCs provided an opportunity for designing a TEOAE-based clinical procedure that could be used to assess cochlear function. To understand the nature of the TEOAE signals in the time and the frequency domain several different analyses were performed. Using normative Input-Output (IO) curves, short-time FFT analyses and cochlear computer simulations, it was found that for optimization of the hearing loss classification it is necessary to use a complete 20 ms TEOAE segment. It was also determined that various 2-D filtering methods (median and averaging filtering masks, LP-FFT) used to enhance of the TEOAE S/N offered minimal improvement (less than 6 dB per stimulus level). Higher S/N improvements resulted in TEOAE sequences that were over-smoothed. The final classification algorithm was based on a statistical analysis of raw FFT data and when applied to a sample set of clinically obtained TEOAE recordings (from 56 normal and 66 hearing-loss subjects) correctly identified 94.3% of the normal and 90% of the hearing loss subjects, at the 80 dB SPL stimulus level. To enhance the discrimination between the conductive and the sensorineural populations, data from the 68 dB SPL stimulus level were used, which yielded a normal classification of 90.2%, a hearing loss classification of 87.5% and a conductive-sensorineural classification of 87%. Among the hearing-loss populations the best discrimination was obtained in the group of otosclerosis and the worst in the group of acute acoustic trauma.

  13. Assessment of hearing aid algorithms using a master hearing aid: the influence of hearing aid experience on the relationship between speech recognition and cognitive capacity.

    Science.gov (United States)

    Rählmann, Sebastian; Meis, Markus; Schulte, Michael; Kießling, Jürgen; Walger, Martin; Meister, Hartmut

    2017-04-27

    Model-based hearing aid development considers the assessment of speech recognition using a master hearing aid (MHA). It is known that aided speech recognition in noise is related to cognitive factors such as working memory capacity (WMC). This relationship might be mediated by hearing aid experience (HAE). The aim of this study was to examine the relationship of WMC and speech recognition with a MHA for listeners with different HAE. Using the MHA, unaided and aided 80% speech recognition thresholds in noise were determined. Individual WMC capacity was assed using the Verbal Learning and Memory Test (VLMT) and the Reading Span Test (RST). Forty-nine hearing aid users with mild to moderate sensorineural hearing loss divided into three groups differing in HAE. Whereas unaided speech recognition did not show a significant relationship with WMC, a significant correlation could be observed between WMC and aided speech recognition. However, this only applied to listeners with HAE of up to approximately three years, and a consistent weakening of the correlation could be observed with more experience. Speech recognition scores obtained in acute experiments with an MHA are less influenced by individual cognitive capacity when experienced HA users are taken into account.

  14. Educating Children Who Are Deaf or Hard of Hearing: Overview. ERIC Digest #E549.

    Science.gov (United States)

    Easterbrooks, Susan

    This digest discusses hearing loss in children. Topics covered include: (1) the causes of hearing loss and the three major types of hearing losses, conductive loss, sensorineural loss, and central auditory processing disorder; (2) the federal definitions of "deaf" (hearing loss which adversely affects educational performance and which is…

  15. External ear anomalies and hearing impairment in Noonan Syndrome.

    Science.gov (United States)

    van Trier, Dorothée C; van Nierop, Josephine; Draaisma, Jos M Th; van der Burgt, Ineke; Kunst, Henricus; Croonen, Ellen A; Admiraal, Ronald J C

    2015-06-01

    This is the first cohort in which hearing impairment and external ear anomalies in Noonan Syndrome are described extensively. Retrospective analysis of the otorhinolaryngological and clinical genetic data from 97 Noonan Syndrome (NS) patients. Forty-four NS patients were seen by an otorhinolaryngologist for the analysis of hearing impairment. In our cohort 80 of the 97 patients were genetically tested. In 71 of these mutations were found: in 48 patients a mutation in PTPN11, in 10 patients in SOS1, in 5 patients in SHOC2, in 5 patients in RAF1, in 1 patient in MAP2K2, in 1 patient in KRAS and in 1 patient in A2ML1. External ear anomalies were reported in 75 NS patients (77%). In 69 patients the ears were low-set, 28 patients had posteriorly rotated ears, 14 patients showed protruding ears and 18 had thickened helices. Hearing impairment was detected in 34 NS patients. Nine patients had sensorineural hearing impairment, two a permanent conductive hearing impairment, two other patients had mixed hearing impairment and 20 patients had conductive hearing impairment in the past, caused by otitis media with effusion. Their temporary conductive hearing impairment resolved between the ages of 2 and 18 years. Sensorineural hearing impairment varied between mild high-frequency hearing impairment and profound (uni- and bilateral) hearing impairment and was progressive in three patients. Four NS patients received cochlear implants for their severe sensorineural hearing impairment. The cohort is small for genotype-phenotype correlations, but sensorineural hearing impairment, especially the bilateral severe hearing impairment, was only seen in patients with a PTPN11 mutation. NS is characterized by dysmorphic external ear anomalies and both sensorineural and conductive hearing impairment. Audiological examinations are recommended in all patients with Noonan Syndrome. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  16. Occupational hearing loss in farmers.

    OpenAIRE

    Plakke, B L; Dare, E

    1992-01-01

    Studies have shown that there is a great deal of high-frequency sensorineural hearing loss among farmers. The studies have failed, however, to differentiate farmers who have occupational noise exposure only from other potential hearing loss etiologies. This study, through extensive case history information, has isolated a farm noise-exposure group and matched its members by age with persons with no significant noise exposure. Results indicate that farmers exposed only to noise from farming ha...

  17. Modeling auditory perception of individual hearing-impaired listeners

    DEFF Research Database (Denmark)

    Jepsen, Morten Løve; Dau, Torsten

    showed that, in most cases, the reduced or absent cochlear compression, associated with outer hair-cell loss, quantitatively accounts for broadened auditory filters, while a combination of reduced compression and reduced inner hair-cell function accounts for decreased sensitivity and slower recovery from...... selectivity. Three groups of listeners were considered: (a) normal hearing listeners; (b) listeners with a mild-to-moderate sensorineural hearing loss; and (c) listeners with a severe sensorineural hearing loss. A fixed set of model parameters were derived for each hearing-impaired listener. The simulations...

  18. Hearing Status in Pediatric Renal Transplant Recipients.

    Science.gov (United States)

    Gulleroglu, Kaan; Baskin, Esra; Aydin, Erdinc; Ozluoglu, Levent; Moray, Gokhan; Haberal, Mehmet

    2015-08-01

    Renal transplant provides a long-term survival. Hearing impairment is a major factor in subjective health status. Status of hearing and the cause of hearing impairment in the pediatric renal transplant group have not been evaluated. Here, we studied to evaluate hearing status in pediatric renal transplant patients and to determine the factors that cause hearing impairment. Twenty-seven pediatric renal transplant recipients were investigated. All patients underwent audiologic assessment by means of pure-tone audiometry. The factors on hearing impairment were performed. Sensorineural hearing impairment was found in 17 patients. There was marked hearing impairment for the higher frequencies between 4000 and 8000 Hz. Sudden hearing loss developed in 2 patients, 1 of them had tinnitus. Decrease of speech understanding was found in 8 patients. The cyclosporine level was significantly high in patients with hearing impairment compared with group without hearing impairment. Cyclosporine levels also were found to be statistically significantly high when compared with the group with decrease of speech understanding and the group without decrease of speech understanding. Similar relations cannot be found between tacrolimus levels and hearing impairment and speech understanding. Sensorineural hearing impairment prevalence was high in pediatric renal transplant recipients when compared with the general population of children. Cyclosporine may be responsible for causing hearing impairment after renal transplant. We suggest that this effect is a dose-dependent toxicity.

  19. 38 CFR 17.149 - Sensori-neural aids.

    Science.gov (United States)

    2010-07-01

    ... 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 provision of this part, VA will furnish needed sensori-neural aids (i.e., eyeglasses, contact lenses...

  20. 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.

  1. Antenatal Bartter's syndrome with sensorineural deafness

    OpenAIRE

    Bhamkar, R. P.; Gajendragadkar, A.

    2009-01-01

    Bartter's syndrome is a group of inherited, salt-losing tubulopathies presenting as metabolic alkalosis with normotensive hyperreninemia and hyperaldosteronism. We report here the first case of a neonate with bilateral, sensorineural deafness, a variant of antenatal Bartter's syndrome from an Indian community.

  2. Hear, Hear!

    Science.gov (United States)

    Rittner-Heir, Robbin

    2000-01-01

    Examines the problem of acoustics in school classrooms; the problems it creates for student learning, particularly for students with hearing problems; and the impediments to achieving acceptable acoustical levels for school classrooms. Acoustic guidelines are explored and some remedies for fixing sound problems are highlighted. (GR)

  3. 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.

  4. The Care and Use of a Child's Hearing Aid: A Practical Guide for Parents, Teachers, and Children.

    Science.gov (United States)

    Lewis, Michael A.

    The booklet is intended to provide practical information on hearing aids for hearing impaired children, their families and teachers. Illustrations are provided to describe the hearing process. The nature of hearing loss is considered in terms of types (conductive, sensori-neural, and mixed), causes, degrees, and educational implications. Four…

  5. [FEDERAL CLINICAL RECOMMENDATIONS IN DIAGNOSIS, TREATMENT AND PREVENTION OF HEARING LOSS DUE TO NOISE].

    Science.gov (United States)

    Adeninskaya, E E; Bukhtiarov, I V; Bushmanov, A Iu; Dayhes, N A; Denisov, E I; Izmerov, N F; Mazitova, N N; Pankova, V B; Preobrazhenskaya, E A; Prokopenko, L V; Simonova, N I; Tavartkiladze, G A; Fedina, I N

    2016-01-01

    Noise induced hearing loss is a slowly developing hearing impairment, caused by occupational exposure to excessive noise levels, constitutes a lesion of the auditory analyzer and clinically manifested as chronic bilateral sensorineural hearing loss. Currently, there is not a treatment that provide a cure of sensorineural hearing loss. Regular, individually tailored treatment should be directed to the pathogenic mechanisms and specific clinical symptoms of hearing loss, as well as the prevention of complications. We recommend using non-drug therapies that can improve blood flow in labyrinth, tissue and cellular metabolism.

  6. Congenital hereditary endothelial dystrophy with progressive sensorineural deafness (Harboyan syndrome

    Directory of Open Access Journals (Sweden)

    Abramowicz Marc

    2008-10-01

    Full Text Available Abstract Harboyan syndrome is a degenerative corneal disorder defined as congenital hereditary endothelial dystrophy (CHED accompanied by progressive, postlingual sensorineural hearing loss. To date, 24 cases from 11 families of various origin (Asian Indian, South American Indian, Sephardi Jewish, Brazilian Portuguese, Dutch, Gypsy, Moroccan, Dominican have been reported. More than 50% of the reported cases have been associated with parental consanguinity. The ocular manifestations in Harboyan syndrome include diffuse bilateral corneal edema occurring with severe corneal clouding, blurred vision, visual loss and nystagmus. They are apparent at birth or within the neonatal period and are indistinguishable from those characteristic of the autosomal recessive CHED (CHED2. Hearing deficit in Harboyan is slowly progressive and typically found in patients 10–25 years old. There are no reported cases with prelinglual deafness, however, a significant hearing loss in children as young as 4 years old has been detected by audiometry, suggesting that hearing may be affected earlier, even at birth. Harboyan syndrome is caused by mutations in the SLC4A11 gene located at the CHED2 locus on chromosome 20p13-p12, indicating that CHED2 and Harboyan syndrome are allelic disorders. A total of 62 different SLC4A11 mutations have been reported in 98 families (92 CHED2 and 6 Harboyan. All reported cases have been consistent with autosomal recessive transmission. Diagnosis is based on clinical criteria, detailed ophthalmological assessment and audiometry. A molecular confirmation of the clinical diagnosis is feasible. A variety of genetic, metabolic, developmental and acquired diseases presenting with clouding of the cornea should be considered in the differential diagnosis (Peters anomaly, sclerocornea, limbal dermoids, congenital glaucoma. Audiometry must be performed to differentiate Harboyan syndrome from CHED2. Autosomal recessive types of CHED (CHED2 and

  7. Mutations in SPATA5 Are Associated with Microcephaly, Intellectual Disability, Seizures, and Hearing Loss

    NARCIS (Netherlands)

    Tanaka, Akemi J.; Cho, Megan T.; Millan, Francisca; Juusola, Jane; Retterer, Kyle; Joshi, Charuta; Niyazov, Dmitriy; Garnica, Adolfo; Gratz, Edward; Deardorff, Matthew; Wilkins, Alisha; Ortiz-Gonzalez, Xilma; Mathews, Katherine; Panzer, Karin; Brilstra, Eva; Van Gassen, Koen L I; Volker-Touw, Catharina M L; van Binsbergen, Ellen; Sobreira, Nara; Hamosh, Ada; McKnight, Dianalee; Monaghan, Kristin G.; Chung, Wendy K.

    2015-01-01

    Using whole-exome sequencing, we have identified in ten families 14 individuals with microcephaly, developmental delay, intellectual disability, hypotonia, spasticity, seizures, sensorineural hearing loss, cortical visual impairment, and rare autosomal-recessive predicted pathogenic variants in

  8. 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.

  9. 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.

  10. Dexamethasone loaded nanoparticles exert protective effects against Cisplatin-induced hearing loss by systemic administration.

    Science.gov (United States)

    Sun, Changling; Wang, Xueling; Chen, Dongye; Lin, Xin; Yu, Dehong; Wu, Hao

    2016-04-21

    Ototoxicity is one of the most important adverse effects of cisplatin chemotherapy. As a common treatment of acute sensorineural hearing loss, systemic administration of steroids was demonstrated ineffective against cisplatin-induced hearing loss (CIHL) in published studies. The current study aimed to evaluate the potential protective effect of dexamethasone (DEX) encapsulated in polyethyleneglycol-coated polylactic acid (PEG-PLA) nanoparticles (DEX-NPs) against cisplatin-induced hearing loss following systemic administration. DEX was fabricated into PEG-PLA nanoparticles using emulsion and evaporation technique as previously reported. DEX or DEX-NPs was administered intraperitoneally to guinea pigs 1h before cisplatin administration. Auditory brainstem response (ABR) threshold shifts were measured at four frequencies (4, 8, 16, and 24kHz) 1 day before and three days after cisplatin injection. Cochlear morphology was examined to evaluate inner ear injury induced by cisplatin exposure. A single dose of DEX-NPs 1h before cisplatin treatment resulted in a significant preservation of the functional and structural properties of the cochlea, which was equivalent to the effect of multidose (3 days) DEX injection. In contrast, no significant protective effect was observed by single dose injection of DEX. The results of histological examination of the cochleae were consistent with the functional measurements. In conclusion, a single dose DEX-NPs significantly attenuated cisplatin ototoxicity in guinea pigs after systemic administration at both histological and functional levels indicating the potential therapeutic benefits of these nanoparticles for enhancing the delivery of DEX in acute sensorineural hearing loss. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  11. 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.

  12. Hearing loss in a glue sniffer.

    Science.gov (United States)

    Williams, D M

    1988-10-01

    A case is presented of a 27-year-old glue sniffing woman with sensorineural hearing loss, optic atrophy and global brain damage. This form of addiction has not received much attention as a cause of otologic catastrophes, and should be borne in mind where similar cases come to the otolaryngologist.

  13. [Presbycusis - Age Related Hearing Loss].

    Science.gov (United States)

    Fischer, N; Weber, B; Riechelmann, H

    2016-07-01

    Presbycusis or age related hearing loss can be defined as a progressive, bilateral and symmetrical sensorineural hearing loss due to age related degeneration of inner ear structures. It can be considered a multifactorial complex disorder with environmental and genetic factors. The molecular, electrophysiological and histological damage at different levels of the inner ear cause a progressive hearing loss, which usually affects the high frequencies of hearing. The resulting poor speech recognition has a negative impact on cognitive, emotional and social function in older adults. Recent investigations revealed an association between hearing impairment and social isolation, anxiety, depression and cognitive decline in elderly. These findings emphasize the importance of diagnosis and treating hearing loss in the elderly population. Hearing aids are the most commonly used devices for treating presbycusis. The technical progress of implantable hearing devices allows an effective hearing rehabilitation even in elderly with severe hearing loss. However, most people with hearing impairments are not treated adequately. © Georg Thieme Verlag KG Stuttgart · New York.

  14. Management of Conductive Hearing Loss in Children.

    Science.gov (United States)

    Dougherty, William; Kesser, Bradley W

    2015-12-01

    Conductive hearing loss (CHL), far more common than sensorineural hearing loss in children, can be acquired or congenital, can range from mild to moderately severe, and can be caused by a simple cerumen impaction, middle ear fluid, or complex middle ear abnormalities with or without the absence of the ear canal (congenital aural atresia). This article presents evidence-based recommendations for the evaluation and management of the child with both acquired and congenital CHL. Copyright © 2015 Elsevier Inc. All rights reserved.

  15. Delayed neuropsychiatric sequelae after acute hydrogen sulfide poisoning: affection of motor function, memory, vision and hearing.

    Science.gov (United States)

    Tvedt, B; Edland, A; Skyberg, K; Forberg, O

    1991-10-01

    A shipyard worker was poisoned by hydrogen sulfide (H2S), and rescued after 15-20 min. He regained consciousness after 2 days. Three days later his condition deteriorated, and he was more or less comatose for a month. When he woke up, he was amnesic, nearly blind, had reduced hearing, and had a moderate spastic tetraparesis and ataxia. Two months after the accident, he had greatly improved. Audiograms showed hearing loss with maximum at 2000 Hz and significantly poorer speech discrimination. EEG showed generalized dysrhythmia. At follow-up 5 years later he had not been able to resume his work, and had slight motor, memory and visual symptoms. CT and MRI showed slight cerebral atrophy. EEG and evoked responses were normal.

  16. Radiation Therapy and Hearing Loss

    International Nuclear Information System (INIS)

    Bhandare, Niranjan; Jackson, Andrew; Eisbruch, Avraham; Pan, Charlie C.; Flickinger, John C.; Antonelli, Patrick; Mendenhall, William M.

    2010-01-01

    A review of literature on the development of sensorineural hearing loss after high-dose radiation therapy for head-and-neck tumors and stereotactic radiosurgery or fractionated stereotactic radiotherapy for the treatment of vestibular schwannoma is presented. Because of the small volume of the cochlea a dose-volume analysis is not feasible. Instead, the current literature on the effect of the mean dose received by the cochlea and other treatment- and patient-related factors on outcome are evaluated. Based on the data, a specific threshold dose to cochlea for sensorineural hearing loss cannot be determined; therefore, dose-prescription limits are suggested. A standard for evaluating radiation therapy-associated ototoxicity as well as a detailed approach for scoring toxicity is presented.

  17. Psycholinguistic abilities in cochlear implant and hearing impaired children

    OpenAIRE

    Hassan, Hatem Ezzeldin; Eldin, Sally Taher Kheir; Al Kasaby, Rasha Mohamed

    2014-01-01

    Background: Many congenitally sensorineural hearing loss (SNHL) children and cochlear implant (CI) recipients develop near-normal language skills. However, there is a wide variation in individual outcomes following cochlear implantation, or using hearing aids. Some CI recipients or Hearing aids users never develop useable speech and oral language skills. The causes of this enormous variation in outcomes are only partly understood at the present time. So, the aim of this study was to assess th...

  18. The Hearing Outcomes of Cochlear Implantation in Waardenburg Syndrome

    OpenAIRE

    Koyama, Hajime; Kashio, Akinori; Sakata, Aki; Tsutsumiuchi, Katsuhiro; Matsumoto, Yu; Karino, Shotaro; Kakigi, Akinobu; Iwasaki, Shinichi; Yamasoba, Tatsuya

    2016-01-01

    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. F...

  19. Masker phase effects in normal-hearing and hearing-impaired listeners: evidence for peripheral compression at low signal frequencies

    DEFF Research Database (Denmark)

    Oxenham, Andrew J.; Dau, Torsten

    2004-01-01

    curvature. Results from 12 listeners with sensorineural hearing loss showed reduced masker phase effects, when compared with data from normal-hearing listeners, at both 250- and 1000-Hz signal frequencies. The effects of hearing impairment on phase-related masking differences were not well simulated...... are affected by a common underlying mechanism, presumably related to cochlear outer hair cell function. The results also suggest that normal peripheral compression remains strong even at 250 Hz....

  20. Sudden bilateral hearing loss after organophosphate inhalation

    Directory of Open Access Journals (Sweden)

    Mehmet Akif Dundar

    2016-12-01

    Full Text Available Sudden bilateral hearing loss are seen rarely and the toxic substance exposure constitutes a small part of etiology. A Fifty-eight-year-old woman admitted to our clinic with sudden bilateral hearing loss shortly after chlorpyrifos-ethyl exposure. Otolaryngologic examination findings were normal. The patient had 40 dB sensorineural hearing loss (SNHL on the right ear and 48 dB SNHL on the left ear. Additional diagnostic tests were normal. The conventional treatment for sudden hearing loss was performed. On the second week following organophosphate (OP exposure the patient's hearing loss almost completely resolved. OP's are heavily used in agriculture and should be taken into consideration as an etiologic factor in sudden hearing loss. Keywords: Organophosphates, Hearing loss, Sudden

  1. Association of folate with hearing is dependent on the 5, 10-methylenetetrahydrofolate reductase 677C-->T mutation

    NARCIS (Netherlands)

    Durga, J.; Anteunis, L.J.C.; Schouten, E.G.; Bots, M.L.; Kok, F.J.; Verhoef, P.

    2006-01-01

    Vascular disease and its risk factors have been associated with the age-related hearing loss. We examined the association of elevated plasma homocysteine and its determinants with hearing levels. Pure-tone air conduction thresholds in 728 individuals with sensorineural hearing loss were not

  2. 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.

  3. 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.

  4. 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....

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

    OpenAIRE

    Emmanuel D Kitcher; Grace Ocansey; Benjamin Abaidoo; Alidu Atule

    2014-01-01

    Noise induced hearing loss (NIHL) is an irreversible sensorineural hearing loss associated with exposure to high levels of excessive noise. Prevention measures are not well established in developing countries. This comparative cross sectional study aims to determine the prevalence of hearing loss in both a group of high risk workers and a control group and to assess their knowledge of the effects of noise on hearing health. A total of 101 market mill workers and 103 controls employed within m...

  6. Conductive hearing loss in four dogs associated with the use of ointment-based otic medications.

    Science.gov (United States)

    Cole, Lynette K; Rajala-Schultz, Päivi J; Lorch, Gwendolen

    2018-04-17

    Hearing loss (HL) is classified as conductive when sound transmission is compromised in the ear canal or middle ear, or sensorineural when there is an abnormality of the receptor cells of the cochlea or auditory pathway. Hearing in dogs is evaluated using the brainstem auditory evoked response (BAER) test. Our objective was to characterize BAER findings pre- and post-ear flushing in four dogs with acute HL following application of an ointment-based otic medication containing betamethasone, clotrimazole and gentamicin in a mineral oil-based system containing a plasticized hydrocarbon gel. Dogs, ranging from 9 to 11 years old, that had been treated with the otic medication for one to three weeks prior to hearing loss and on otoscopic examination had evidence of medication in the horizontal ear canals. Dogs were anaesthetized for an ear flush to remove the medication from the ear canals. Hearing was assessed using BAER testing, measurements were initiated with 116 decibel peak equivalent sound pressure level (dBpeSPL) click. Estimated threshold was defined as the lowest intensity in dB in which wave V was still present. Post-ear flush the estimated threshold improved in both ears of all dogs (mean 22.3 dB; range 13-41 dB), confirming conductive HL due to the otic medication. All owners noted an improvement in their dog's hearing post-ear flush, validating the BAER findings. These results emphasize the importance of an ear flush to remove otic medications in dogs that experience acute HL, to determine if the HL is conductive, and if so, to restore hearing. © 2018 ESVD and ACVD.

  7. State of the art in perceptual design of hearing aids

    Science.gov (United States)

    Edwards, Brent W.; van Tasell, Dianne J.

    2002-05-01

    Hearing aid capabilities have increased dramatically over the past six years, in large part due to the development of small, low-power digital signal processing chips suitable for hearing aid applications. As hearing aid signal processing capabilities increase, there will be new opportunities to apply perceptually based knowledge to technological development. Most hearing loss compensation techniques in today's hearing aids are based on simple estimates of audibility and loudness. As our understanding of the psychoacoustical and physiological characteristics of sensorineural hearing loss improves, the result should be improved design of hearing aids and fitting methods. The state of the art in hearing aids will be reviewed, including form factors, user requirements, and technology that improves speech intelligibility, sound quality, and functionality. General areas of auditory perception that remain unaddressed by current hearing aid technology will be discussed.

  8. Viral Causes of Hearing Loss: A Review for Hearing Health Professionals

    Science.gov (United States)

    Cohen, Brandon E.; Durstenfeld, Anne

    2014-01-01

    A number of viral infections can cause hearing loss. Hearing loss induced by these viruses can be congenital or acquired, unilateral or bilateral. Certain viral infections can directly damage inner ear structures, others can induce inflammatory responses which then cause this damage, and still others can increase susceptibility or bacterial or fungal infection, leading to hearing loss. Typically, virus-induced hearing loss is sensorineural, although conductive and mixed hearing losses can be seen following infection with certain viruses. Occasionally, recovery of hearing after these infections can occur spontaneously. Most importantly, some of these viral infections can be prevented or treated. For many of these viruses, guidelines for their treatment or prevention have recently been revised. In this review, we outline many of the viruses that cause hearing loss, their epidemiology, course, prevention, and treatment. PMID:25080364

  9. Comparing Analog and Digital Hearing Aids in Reducing Hearing Disability

    Directory of Open Access Journals (Sweden)

    Ghassem Mohammad Khani

    2004-06-01

    Full Text Available Objective: Comparing analog and digital hearing aids reducing disability caused by hearing deficiency among moderate to severe sensorineural hearing-impaired persons. Method and Material: This descriptive-analytic study was carried out on two groups of subjects participated in this study in some audiology clinics of hearing aid since May 2002 to October 2003. Twenty subjects wore analog hearing aids and twenty one subjects wore digital hearing aids. In this study , no subject had previous middle ear or psychological problems. APHAB questionnaire was completed before using hearing aid and 2 months after to determine benefit of hearing aid use. Results: Total score mean of APHAB inventory before and after use of analoge hearing aids were 52.215+6.420 and 32.300+3.443 respectively. Also total score mean of APHAB inventory before and after use of digital hearing aids were 54.9252+9.028 and 26.321+10.916 respectively. There was no significant difference between total mean score of APHAB inventory before and after using analog and digital hearing aids (P=0.058.While there was significant difference between total mean score of APHAB questionnaire before and after use of analog hearing aids (P<0.001 and also before and after use of digital hearing aids (P<0.001. Moreover age, gender , litracy level , occupation , degree of hearing loss and manner of hearing aid usage did not have significant effect on APHAB results. Configuration of loss had siginficant effect on aversiveness subscale before and after use of analog hearing aids (P=0.008. Previous experience and duration of hearing aid usage had significant effect on aversiveness subscale before and after use of digital hearing aids (P=0.043 and (P=0.024, respectively , while all of these three items did not have significant effect on total mean score of APHAB inventory and also total mean scores of three subscales of ease of communication , reverberation and background noise. Conclusion: Comparing to

  10. Newborn hearing screening.

    Science.gov (United States)

    Stewart, D L; Pearlman, A

    1994-11-01

    Congenital deafness is a relatively common problem with an incidence of 1/300 to 1/1000. Most states have no mass screening program for hearing loss, but the state of Kentucky compiles a High Risk Registry which is a historical survey of parents relating to risk factors for hearing loss. Unfortunately this survey can miss 50% of those who have a hearing deficit. If not detected prior to discharge, there is often a delay in diagnosis of deafness which prevents early intervention. We report 2 years' experience at Kosair Children's Hospital where 1,987 infants admitted to well baby, intermediate, or intensive care nurseries were screened using the ALGO-1 screener (Natus Medical Inc, Foster City, CA) which is a modified auditory brain stem evoked response (ABR). Our screening of this population led to an 11% incidence of referral for complete audiological evaluation. There were no significant complications. Forty-eight infants were found to have nonspecified, sensorineural, or conductive hearing loss. The positive predictive value of the test was 96%. Therefore, we feel that the use of the modified ABR in the newborn is a timely, cost efficient method of screening for hearing loss and should be used for mass screening of all newborns.

  11. Genetic mutation susceptibility of hearing loss in child with severe neonatal jaundice

    International Nuclear Information System (INIS)

    Zahedi, F.D.; Rahman, R.A.; Abdullah, A.

    2015-01-01

    This case report demonstrates a case of 5-year-old non-syndromic Malay boy who passed the hearing screening test however he was confirmed has bilateral profound sensorineural hearing loss diagnosed at 3 months of age by brain stem evoked response (BSER). He has background history of severe neonatal jaundice and male siblings of hearing impairment. The antenatal and birth history was uneventful apart from maternal hypothyroidism. His other two elder brothers have bilateral sensorineural hearing loss and history of severe neonatal jaundice as well. The ear examinations, computed tomography scan and magnetic resonance imaging revealed normal findings. Right sided cochlear implantation was done at the age of 3 years old and he is still under audiology follow-up. Conclusion: Genetic studies are important to determine the cause of genetic mutation in susceptibility to hearing impairment that run in his family after severe neonatal jaundice. Those baby with risk of developing hearing loss required diagnostic hearing assessment. (author)

  12. Audiometric Characteristics of a Dutch DFNA10 Family With Mid-Frequency Hearing Impairment

    NARCIS (Netherlands)

    Beelen, E. van; Oonk, A.M.M.; Leijendeckers, J.M.; Hoefsloot, E.H.; Pennings, R.J.E.; Feenstra, I.; Dieker, H.J.; Huygen, P.L.M.; Snik, A.F.M.; Kremer, H.; Kunst, H.P.M.

    2016-01-01

    OBJECTIVES: Mutations in EYA4 can cause nonsyndromic autosomal dominant sensorineural hearing impairment (DFNA10) or a syndromic variant with hearing impairment and dilated cardiomyopathy. A mutation in EYA4 was found in a Dutch family, causing DFNA10. This study is focused on characterizing the

  13. 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.

  14. 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.

  15. About Hearing

    Science.gov (United States)

    ... Info to Go » Hearing-Related » About Hearing About Hearing Each child who is deaf or hard of ... the ear to the brain. Implications: When the hearing mechanism is not functioning Hearing may be impacted ...

  16. Vowel production of Mandarin-speaking hearing aid users with different types of hearing loss.

    Directory of Open Access Journals (Sweden)

    Yu-Chen Hung

    Full Text Available In contrast with previous research focusing on cochlear implants, this study examined the speech performance of hearing aid users with conductive (n = 11, mixed (n = 10, and sensorineural hearing loss (n = 7 and compared it with the speech of hearing control. Speech intelligibility was evaluated by computing the vowel space area defined by the Mandarin Chinese corner vowels /a, u, i/. The acoustic differences between the vowels were assessed using the Euclidean distance. The results revealed that both the conductive and mixed hearing loss groups exhibited a reduced vowel working space, but no significant difference was found between the sensorineural hearing loss and normal hearing groups. An analysis using the Euclidean distance further showed that the compression of vowel space area in conductive hearing loss can be attributed to the substantial lowering of the second formant of /i/. The differences in vowel production between groups are discussed in terms of the occlusion effect and the signal transmission media of various hearing devices.

  17. 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

  18. 75 FR 30693 - Revised Medical Criteria for Evaluating Hearing Loss

    Science.gov (United States)

    2010-06-02

    ... pathways from the inner ear to the brain. Persons with the degree of sensorineural hearing loss required in... commenter recommended that we raise the average air conduction threshold in proposed listing 102.10A to... with an IQ of 35-55; the WHO's is similar, with an IQ of 35-49. In either case, the mental retardation...

  19. 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…

  20. Congenital hearing loss. Is CT enough? | Agha | Alexandria Journal ...

    African Journals Online (AJOL)

    Patients and methods: This is a prospective study including 60 patients, 24 males and 36 females aged from 1 to 7 years, who were presented by unilateral or bilateral congenital conductive (CHL) or/and sensorineural hearing loss (SNHL). All patients were evaluated by HRCT scan with post-processing multiplanar ...

  1. Benefit of Analog, Programmable and Digital Hearing Aids

    Directory of Open Access Journals (Sweden)

    Jamileh Fatahi

    2006-12-01

    Full Text Available Background and Aims: As the hearing aid technology progressively promotes toward replacing analog hearing aids with digital and programmable ones, comparison of the patient satisfaction of those kinds of hearing aids by means of a valuable tool seems so necessary. So, the aim of this study was to compare self-reported benefit of analog, digitally controlled programmable and digital hearing aids for reducing disability caused by hearing impairment in mild to severe sensorineural hearing impaired persons. Materials and Methods: This cross-sectional study was performed on 90 persons with mild to severe sensorineural hearing loss dividing into three groups: 43 subjects were fitted with digital, 15 with programmable, 32 with analog hearing aids. After pure tone audiometry, Abbreviated profile of hearing aid benefit (APHAB was completed before and one month after using hearing aids to determine the benefit of them. Results: Global APHAB mean scores for digital, programmable and analog hearing aids were 49.05, 33.19 and 39.53, respectively. Ease of Communication subscale mean scores were 53.46 for digitals, 37.66 for programmables and 39.09 for analogs. Background noise subscale mean scores for digital programmable and analog hearing aids were 46.36, 25.53 and 35.31, respectively. Global and also both subscale mean scores showed significant difference between digital hearing aids and programmable and analog ones. There was no significant difference between reverberation subscale mean scores of three groups. Conclusion: It seems digital hearing aids may be more beneficial to reduce disability caused by hearing loss than analog and programmable hearing aids are.

  2. Imaging of post-traumatic hearing loss.

    Science.gov (United States)

    Mazón, M; Pont, E; Albertz, N; Carreres-Polo, J; Más-Estellés, F

    Hearing loss is the most frequent complication of temporal bone trauma. The role of the radiologist is of great importance; the adequacy and selection of the imaging technique, as well as its correct interpretation, are crucial to establish the diagnosis, prognosis and enable the selection of appropriate treatment. With the aim of systematizing the most relevant concepts in the evaluation of image studies in this scenario, this review will be outlined according to the hearing loss type. The potential lesions of its components will be assessed; In each case the most appropriate imaging technique will be suggested and the findings will be described and depicted. In postraumatic hearing loss, computed tomography is the initial technique of choice and will allow the detection of alterations that cause conductive hearing loss; magnetic resonance imaging will be useful in the evaluation of sensorineural hearing loss. Copyright © 2017 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.

  3. 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.

  4. Hypoparathyroidism, sensorineural deafness, and renal dysgenesis syndrome with a mutation

    Directory of Open Access Journals (Sweden)

    Yong Suk Shim

    2015-03-01

    Full Text Available Hypoparathyroidism, sensorineural deafness, and renal dysgenesis syndrome is an autosomal dominant disease caused by mutations in the GATA3 gene on chromosome 10p15. We identified a patient diagnosed with hypoparathyroidism who also had a family history of hypoparathyroidism and sensorineural deafness, present in the father. The patient was subsequently diagnosed and found to be a heterozygote for an insertion mutation c.255_256ins4 (GTGC in exon 2 of GATA3. His father was also confirmed to have the same mutation in GATA3.

  5. Addition of host genetic variants in a prediction rule for post meningitis hearing loss in childhood: a model updating study

    NARCIS (Netherlands)

    Sanders, Marieke S.; de Jonge, Rogier C. J.; Terwee, Caroline B.; Heymans, Martijn W.; Koomen, Irene; Ouburg, Sander; Spanjaard, Lodewijk; Morré, Servaas A.; van Furth, A. Marceline

    2013-01-01

    Sensorineural hearing loss is the most common sequela in survivors of bacterial meningitis (BM). In the past we developed a validated prediction model to identify children at risk for post-meningitis hearing loss. It is known that host genetic variations, besides clinical factors, contribute to

  6. 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…

  7. Identifying hearing loss by means of iridology.

    Science.gov (United States)

    Stearn, Natalie; Swanepoel, De Wet

    2006-11-13

    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. A controlled trial was conducted with an iridologist, blind to the actual hearing status of participants, analyzing the irises of participants with and without hearing loss. Fifty hearing impaired and fifty normal hearing subjects, between the ages of 15 and 19 years, controlled for gender, participated in the study. An experienced iridologist analyzed the randomised set of participants' irises. A 70% correct identification of hearing status was obtained by iridological analyses with a false negative rate of 41% compared to a 19% false positive rate. The respective sensitivity and specificity rates therefore came to 59% and 81%. Iridological analysis of hearing status indicated a statistically significant relationship to actual hearing status (P iridology were not comparable to those of traditional audiological screening procedures.

  8. ANALYTICAL STUDY OF SENSORY NEURAL HEARING LOSS IN CSOM WITH AND WITHOUT CHOLESTEATOMA

    Directory of Open Access Journals (Sweden)

    Kabikanta Samantaray

    2016-07-01

    Full Text Available BACKGROUND Chronic suppurative otitis media still remains a major cause of conductive hearing loss in our country. But, a few patients also display an added sensorineural hearing loss. MATERIALS & METHODS Hundred patients with CSOM undergoing surgery at our department were included in the study. The affected ears formed the ‘CSOM Group’ and the normal ears formed the ‘Control group’. Detailed otological history, clinical, surgical and audiometric findings were recorded and analysed. RESULTS It was inferred that CSOM associated with sensorineural hearing loss was found in small number of patients only. No correlation was established between duration of discharge and sensorineural loss. CONCLUSION Though greater SN loss was seen in patients of CSOM with cholesteatoma it was not statistically significant. Whether an early surgery in CSOM can prevent SN loss or not needs further studies.

  9. Acute cortical deafness in a child with MELAS syndrome.

    Science.gov (United States)

    Pittet, Marie P; Idan, Roni B; Kern, Ilse; Guinand, Nils; Van, Hélène Cao; Toso, Seema; Fluss, Joël

    2016-05-01

    Auditory impairment in mitochondrial disorders are usually due to peripheral sensorineural dysfunction. Central deafness is only rarely reported. We report here an 11-year-old boy with MELAS syndrome who presented with subacute deafness after waking up from sleep. Peripheral hearing loss was rapidly excluded. A brain MRI documented bilateral stroke-like lesions predominantly affecting the superior temporal lobe, including the primary auditory cortex, confirming the central nature of deafness. Slow recovery was observed in the following weeks. This case serves to illustrate the numerous challenges caused by MELAS and the unusual occurrence of acute cortical deafness, that to our knowledge has not be described so far in a child in this setting.

  10. Hearing loss in Usher syndrome type II is nonprogressive.

    Science.gov (United States)

    Reisser, Christoph F V; Kimberling, William J; Otterstedde, Christian R

    2002-12-01

    Usher syndrome is an autosomal recessive disorder characterized by sensorineural hearing loss and progressive visual loss secondary to retinitis pigmentosa. In the literature, a possible progression of the moderate to severe hearing loss in Usher syndrome type II (Usher II) is controversial. We studied the development of the hearing loss of 125 patients with a clinical diagnosis of Usher syndrome type II intraindividually and interindividually by repeatedly performing complete audiological and neuro-otologic examinations. Our data show a very characteristic slope of the hearing curve in all Usher II patients and no clinically relevant progression of the hearing loss over up to 17 years. The subjective impression of a deterioration of the communicative abilities of Usher II patients must therefore be attributed to the progressive visual loss. The patients should be reassured that changes in their hearing abilities are unlikely and should be provided with optimally fitted modern hearing aids.

  11. Vibrant Soundbridge rehabilitation of conductive and mixed hearing loss.

    Science.gov (United States)

    Lüers, Jan-Christoffer; Hüttenbrink, Karl-Bernd

    2014-12-01

    The Vibrant Soundbridge is the world's most often implanted active middle ear implant or hearing aid. During the last few years, the device indications have expanded from sensorineural hearing loss to conductive and mixed hearing loss. Titanium couplers have led to improved contact of the floating mass transducer with the middle ear structures. The resulting hearing gain is satisfying for most patients, but so far, there is no clear audiologic advantage over conventional hearing aids. Currently, the indications are mainly related to intolerance of conventional hearing aids (eg, chronic otitis externa), severe mixed hearing loss with a destructed middle ear and certain medical diagnosis (eg, congenital atresia). Copyright © 2014 Elsevier Inc. All rights reserved.

  12. [The specific microbiological and clinical features of acute otitis media].

    Science.gov (United States)

    Gurov, A V; Levina, Yu V; Guseva, A L; Elchueva, Z G; Efimova, S P; Gordienko, M V

    The objective of the present study was to elucidate the specific features of the clinical course of acute otitis media as well as the peculiarities of the vestibular function and the microbial paysage associated with this pathological condition under the present-day conditions. The study included 135 patients presenting with acute otitis media (AOM) at different stages of the disease. The discharge obtained from the tympanic cavity of all the patients was examined with the use of polymerase chain reaction in real time, audiological and vestibulogical methods. The distinctive features of acute otitis medium associated with Streptococcus pneumoniae infection were found to be the intense pain syndrome with the symptoms of intoxication, well apparent inflammatory changes in the tympanic membrane as revealed by otoscopy, the increased frequency of sensorineural impairment of hearing, and the characteristic type B tympanometric curve. Typical of AOM associated with Haemophilus influenza infection are the mild pain syndrome, weak changes in the tympanic membrane as revealed by otoscopy, conductive hearing loss, and the type C tympanometric curve.

  13. 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.

  14. 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.

  15. Prevalence, heritability and genetic correlations of congenital sensorineural deafness and pigmentation phenotypes in the Border Collie.

    Science.gov (United States)

    De Risio, Luisa; Lewis, Tom; Freeman, Julia; de Stefani, Alberta; Matiasek, Lara; Blott, Sarah

    2011-06-01

    The objectives of this study were to estimate prevalence, heritability and genetic correlations of congenital sensorineural deafness (CSD) and pigmentation phenotypes in the Border Collie. Entire litters of Border Collies that presented to the Animal Health Trust (1994-2008) for assessment of hearing status by brain stem auditory evoked response (BAER) at 4-10 weeks of age were included. Heritability and genetic correlations were estimated using residual maximum likelihood (REML). Of 4143 puppies that met the inclusion criteria, 97.6% had normal hearing status, 2.0% were unilaterally deaf and 0.4% were bilaterally deaf. Heritability of deafness as a trichotomous trait (normal/unilaterally deaf/bilaterally deaf) was estimated at 0.42 using multivariate analysis. Genetic correlations of deafness with iris colour and merle coat colour were 0.58 and 0.26, respectively. These results indicate that there is a significant genetic effect on CSD in Border Collies and that some of the genes determining deafness also influence pigmentation phenotypes. Copyright © 2010 Elsevier Ltd. All rights reserved.

  16. Neurological features of epilepsy, ataxia, sensorineural deafness, tubulopathy syndrome.

    OpenAIRE

    Cross, J. H.; Arora, R.; Heckemann, R. A.; Gunny, R.; Chong, K.; Carr, L.; Baldeweg, T.; Differ, A. M.; Lench, N.; Varadkar, S.; Sirimanna, T.; Wassmer, E.; Hulton, S. A.; Ognjanovic, M.; Ramesh, V.

    2013-01-01

    Recently, we reported a previously unrecognized symptom constellation comprising epilepsy, ataxia, sensorineural deafness, and tubulopathy (EAST syndrome) associated with recessive mutations in the KCNJ10 gene. Here, we provide a detailed characterization of the clinical features of the syndrome to aid patient management with respect to diagnosis, prognostic counselling, and identification of best treatment modalities.

  17. Hearing impairment in genotyped Wolfram syndrome patients.

    Science.gov (United States)

    Plantinga, Rutger F; Pennings, Ronald J E; Huygen, Patrick L M; Bruno, Rocco; Eller, Philipp; Barrett, Timothy G; Vialettes, Bernard; Paquis-Fluklinger, Veronique; Lombardo, Fortunato; Cremers, Cor W R J

    2008-07-01

    Wolfram syndrome is a progressive neurodegenerative syndrome characterized by the features "DIDMOAD" (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness). We sought to study the audiometric data of genotyped Wolfram syndrome patients with sensorineural hearing impairment. Pure tone threshold data of 23 Wolfram syndrome patients were used for cross-sectional analysis in subgroups (age less than 16 years or between 19 and 25 years, gender, and origin). All subgroups, with 1 exception, showed a fairly similar type of hearing impairment with, on average, thresholds of about 25 dB (range, 0 to 65 dB) at 0.25 to 1 kHz, gently sloping downward to about 60 dB (range, 25 to 95 dB) at 8 kHz. The subgroup of Dutch women, which was excluded from the calculations of the average hearing thresholds, showed a higher degree of hearing impairment. Only the latter subgroup showed progression; however, contrary to the previous longitudinal analysis, progression was not significant in the present cross-sectional analysis, presumably because of the high degree of cross-subject variability. This unique collection of audiometric data from genotyped Wolfram syndrome patients shows no substantial progression in sensorineural hearing impairment with advancing age, no relation to the types of WFS1 mutations identified, and, with exclusion of the subgroup of Dutch female patients, no significant sex-related differences.

  18. Development of conductive hearing loss due to posterior semicircular canal dehiscence.

    Science.gov (United States)

    Kubota, Marie; Kubo, Kazuhiko; Yasui, Tetsuro; Matsumoto, Nozomu; Komune, Shizuo

    2015-06-01

    We herein report a case of posterior semicircular canal dehiscence (SCD) syndrome who had been audiologically followed up for eight years. The patient originally had sensorineural hearing loss. The audiogram had gradually transformed to pure conductive hearing loss. The posterior SCD was identified in CT scan. The reported case showed the possibility to distinguish the mechanism at play underlying the typical conductive hearing loss in SCD patients by tracing the transition of the hearing loss pattern. This information is of much help to predict the hearing outcomes if surgical intervention were chosen for the treatment. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  19. The Hearing Outcomes of Cochlear Implantation in Waardenburg Syndrome.

    Science.gov (United States)

    Koyama, Hajime; Kashio, Akinori; Sakata, Aki; Tsutsumiuchi, Katsuhiro; Matsumoto, Yu; Karino, Shotaro; Kakigi, Akinobu; Iwasaki, Shinichi; Yamasoba, Tatsuya

    2016-01-01

    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. 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.

  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. 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

  3. Hearing loss among patients with Turner's syndrome: literature review

    Directory of Open Access Journals (Sweden)

    Cresio Alves

    2014-06-01

    Full Text Available INTRODUCTION: Turner's syndrome (TS is caused by a partial or total deletion of an X chromosome, occurring in 1:2,000 to 1:5,000 live born females. Hearing loss is one of its major clinical manifestations. However, there are few studies investigating this problem. OBJECTIVES: To review the current knowledge regarding the epidemiology, etiology, clinical manifestations and diagnosis of hearing impairment in patients with TS. METHODS: A bibliographic search was performed in the Medline and Lilacs databanks (1980-2012 to identify the main papers associating Turner's syndrome, hearing impairment and its clinical outcomes. CONCLUSIONS: Recurrent otitis media, dysfunction of the Eustachian tube, conductive hearing loss during infancy and sensorineural hearing loss in adolescence are the audiologic disorders more common in ST. The karyotype appears to be important in the hearing loss, with studies demonstrating an increased prevalence in patients with monosomy 45,X or isochromosome 46,i(Xq. Morphologic studies of the cochlea are necessary to help out in the clarifying the etiology of the sensorineural hearing loss.

  4. 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.

  5. Hearing devices for children with unilateral hearing loss: Patient- and parent-reported perspectives.

    Science.gov (United States)

    Purcell, Patricia L; Jones-Goodrich, Rose; Wisneski, Meghan; Edwards, Todd C; Sie, Kathleen C Y

    2016-11-01

    Management of children with unilateral hearing loss is not standardized. The primary goal of this study was to elicit patient- and parent-reported perspectives regarding usage of hearing devices in pediatric UHL and to suggest a basic algorithmic approach to management. Our tertiary care center recruited families of youth ages 5-19 years with unilateral hearing loss from January 2014 through October 2015. Parents of all youths completed a 36-item survey, and some youth ages 11-19 years participated in hour-long interviews. We assessed patterns of hearing device usage among participants, and performed qualitative data analysis to understand factors considered by youths when deciding whether or not to use a hearing device. Survey information was collected for 50 patients. Distribution of hearing loss severity in affected ear was mild 14%, moderate 26%, severe 22%, and profound 38%. The majority of children had sensorineural hearing loss (57%), followed by mixed (32%), and then conductive (11%). 34 children (68%) had tried a hearing device; 20 continued to use the device. Retention rates were similar among children with different degrees of hearing loss: mild 66%, moderate 50%, severe 60%, profound 64%. Sixteen children tried a wireless contralateral routing of signal (CROS) device, and 15 tried a behind-the-ear (BTE) hearing aid. Retention rates for CROS and BTE devices were 69% and 47%, respectively. The most common reason for cessation of use was discomfort, followed by lack of benefit. A majority of children with unilateral hearing loss who tried a hearing device continued to use it, and retention rates were similar across all degrees of hearing loss. These findings suggest that personal hearing devices should be included in management protocols. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  6. Formal auditory training in adult hearing aid users

    Directory of Open Access Journals (Sweden)

    Daniela Gil

    2010-01-01

    Full Text Available INTRODUCTION: Individuals with sensorineural hearing loss are often able to regain some lost auditory function with the help of hearing aids. However, hearing aids are not able to overcome auditory distortions such as impaired frequency resolution and speech understanding in noisy environments. The coexistence of peripheral hearing loss and a central auditory deficit may contribute to patient dissatisfaction with amplification, even when audiological tests indicate nearly normal hearing thresholds. OBJECTIVE: This study was designed to validate the effects of a formal auditory training program in adult hearing aid users with mild to moderate sensorineural hearing loss. METHODS: Fourteen bilateral hearing aid users were divided into two groups: seven who received auditory training and seven who did not. The training program was designed to improve auditory closure, figure-to-ground for verbal and nonverbal sounds and temporal processing (frequency and duration of sounds. Pre- and post-training evaluations included measuring electrophysiological and behavioral auditory processing and administration of the Abbreviated Profile of Hearing Aid Benefit (APHAB self-report scale. RESULTS: The post-training evaluation of the experimental group demonstrated a statistically significant reduction in P3 latency, improved performance in some of the behavioral auditory processing tests and higher hearing aid benefit in noisy situations (p-value < 0,05. No changes were noted for the control group (p-value <0,05. CONCLUSION: The results demonstrated that auditory training in adult hearing aid users can lead to a reduction in P3 latency, improvements in sound localization, memory for nonverbal sounds in sequence, auditory closure, figure-to-ground for verbal sounds and greater benefits in reverberant and noisy environments.

  7. Congenital stapes malformation: Rare conductive hearing loss in a patient with Waardenburg syndrome.

    Science.gov (United States)

    Melzer, Jonathan M; Eliason, Michael; Conley, George S

    2016-04-01

    Waardenburg syndrome is a known autosomal dominant cause of congenital hearing loss. It is characterized by a distinctive phenotypic appearance and often involves sensorineural hearing loss. Temporal bone abnormalities and inner ear dysmorphisms have been described in association with the disease. However, middle ear abnormalities as causes of conductive hearing loss are not typically seen in Waardenburg syndrome. We discuss a case of an 8-year-old female who meets diagnostic criteria for Waardenburg syndrome type 3 and who presented with a bilateral conductive hearing loss associated with congenital stapes fixation. We discuss management strategy in this previously unreported phenotype. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

  8. 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

  9. Masking and Partial Masking in Listeners with a High-Frequency Hearing Loss

    NARCIS (Netherlands)

    Smits, J.T.S.; Duifhuis, H.

    1982-01-01

    3 listeners with sensorineural hearing loss ranging from moderate to moderate-severe starting at frequencies higher than 1 kHz participated in two masking experiments and a partial masking experiment. In the first masking experiment, fM = 1 kHz and LM = 50 dB SPL, higher than normal masked

  10. 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

  11. Hypokalemic salt-losing tubulopathy with chronic renal failure and sensorineural deafness.

    Science.gov (United States)

    Jeck, N; Reinalter, S C; Henne, T; Marg, W; Mallmann, R; Pasel, K; Vollmer, M; Klaus, G; Leonhardt, A; Seyberth, H W; Konrad, M

    2001-07-01

    To characterize a rare inherited hypokalemic salt-losing tubulopathy with linkage to chromosome 1p31. We conducted a retrospective analysis of the clinical data for 7 patients in whom cosegregation of the disease with chromosome 1p31 had been demonstrated. In addition, in 1 kindred, prenatal diagnosis in the second child was established, allowing a prospective clinical evaluation. Clinical presentation of the patients was homogeneous and included premature birth attributable to polyhydramnios, severe renal salt loss, normotensive hyperreninemia, hypokalemic alkalosis, and excessive hyperprostaglandin E-uria, which suggested the diagnosis of hyperprostaglandin E syndrome/antenatal Bartter syndrome. However, the response to indomethacin was only poor, accounting for a more severe variant of the disease. The patients invariably developed chronic renal failure. The majority had extreme growth retardation, and motor development was markedly delayed. In addition, all patients turned out to be deaf. The hypokalemic salt-losing tubulopathy with chronic renal failure and sensorineural deafness represents not only genetically but also clinically a disease entity distinct from hyperprostaglandin E syndrome/antenatal Bartter syndrome. A pleiotropic effect of a single gene defect is most likely causative for syndromic hearing loss.

  12. 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.

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

    Science.gov (United States)

    Kitcher, Emmanuel D; Ocansey, Grace; Abaidoo, Benjamin; Atule, Alidu

    2014-01-01

    Noise induced hearing loss (NIHL) is an irreversible sensorineural hearing loss associated with exposure to high levels of excessive noise. Prevention measures are not well established in developing countries. This comparative cross sectional study aims to determine the prevalence of hearing loss in both a group of high risk workers and a control group and to assess their knowledge of the effects of noise on hearing health. A total of 101 market mill workers and 103 controls employed within 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 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.

  14. 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.

  15. Familial X/Y Translocation Encompassing ARSE in Two Moroccan Siblings with Sensorineural Deafness.

    Science.gov (United States)

    Amasdl, Saadia; Smaili, Wiam; Natiq, Abdelhafid; Hassani, Amale; Sbiti, Aziza; Agadr, Aomar; Sanlaville, Damien; Sefiani, Abdelaziz

    2017-01-01

    Unbalanced translocations involving X and Y chromosomes are rare and associated with a contiguous gene syndrome. The clinical phenotype is heterogeneous including mainly short stature, chondrodysplasia punctata, ichthyosis, hypogonadism, and intellectual disability. Here, we report 2 brothers with peculiar gestalt, short stature, and hearing loss, who harbor an X/Y translocation. Physical examination, brainstem acoustic potential evaluation, bone age, hormonal assessment, and X-ray investigations were performed. Because of their dysmorphic features, karyotyping, FISH, and aCGH were carried out. The probands had short stature, hypertelorism, midface hypoplasia, sensorineural hearing loss, normal intelligence as well as slight radial and ulnar bowing with brachytelephalangy. R-banding identified a derivative X chromosome with an abnormally expanded short arm. The mother was detected as a carrier of the same aberrant X chromosome. aCGH disclosed a 3.1-Mb distal deletion of chromosome region Xp22.33pter. This interval encompasses several genes, especially the short stature homeobox (SHOX) and arylsulfatase (ARSE) genes. The final karyotype of the probands was: 46,Y,der(X),t(X;Y)(p22;q12).ish der(X)(DXYS129-,DXYS153-)mat.arr[hg19] Xp22.33(61091_2689408)×1mat,Xp22.33(2701273_3258404)×0mat,Yq11.222q12 (21412851_59310245)×2. Herein, we describe a Moroccan family with a maternally inherited X/Y translocation and discuss the genotype-phenotype correlations according to the deleted genes. © 2017 S. Karger AG, Basel.

  16. A case report of hearing loss post use of hydroxychloroquine in a HIV-infected patient

    OpenAIRE

    Khalili, Hossein; Dastan, Farzaneh; Dehghan Manshadi, Seyed Ali

    2014-01-01

    Objective A case with reversible symmetrical sensorineural hearing loss following hydroxychloroquine therapy is described. Case summary A 57-year-old, human immunodeficiency virus (HIV) positive man was referred to the HIV clinic of Imam Khomeini Hospital, Tehran with chief complaint of bilateral slowly progressive hearing loss starting from two months ago. The man had history of rheumatoid arthritis diagnosed from 3 months ago and was administered hydroxychloroquine 200 mg and prednisolone 5...

  17. Maternal and Placental Factors Associated with Congenital Hearing Loss in Very Preterm Neonates

    OpenAIRE

    Shin Hye Kim; Byung Yoon Choi; Jaehong Park; Eun Young Jung; Soo-Hyun Cho; Kyo Hoon Park

    2017-01-01

    Sensorineural hearing loss (SNHL) is a multifactorial disease that more frequently affects preterm newborns. Although a number of maternal conditions have been reported to be associated with preterm birth, little information is available concerning maternal risk factors for the development of SNHL. We aimed to identify maternal and placental risk factors associated with a “refer” result on the newborn hearing screening (NHS) test and subsequently confirmed SNHL in very preterm neonates. Me...

  18. Hearing assessment in pre-school children with speech delay.

    Science.gov (United States)

    Psillas, George; Psifidis, Anestis; Antoniadou-Hitoglou, Magda; Kouloulas, Athanasios

    2006-09-01

    The purpose of this study was to detect any underlying hearing loss among the healthy pre-school children with speech delay. 76 children, aged from 1 to 5 years, underwent a thorough audiological examination consisting of tympanometry, free field testing, otoacoustic emission recordings and auditory brainstem responses (ABRs). If hearing was normal, then they were evaluated by a child neurologist-psychiatrist. According to our findings, the children were classified into 3 groups; those with normal hearing levels (group I, 52 children, 68.4%), sensorineural hearing loss (group II, 22 children, 28.9%) and conductive hearing loss (group III, 2 children, 2.6%). In group I, speech delay was attributed to pervasive developmental disorder (PDD), which represents high-functioning autistic children (37 cases). Other causes were specific language impairment (SLI)-expressive (3 cases), bilingualism (2 cases), and unknown etiology (10 cases). More than half (59%) of the children diagnosed with PDD evidenced significant language impairment limited to more than two words. Children with SLI-expressive and bilingualism used a maximum of two words. In group II, 13 children suffered from profound hearing loss in both ears, 3 from severe, 3 had profound hearing loss in one ear and severe in the other, 2 from moderate, and 1 had moderate in one ear and severe in the other. No child had mild sensorineural hearing loss. The children with profound hearing loss in at least one ear had total language impairment using no word at all (10 cases), or a maximum of two words (6 cases). When hearing loss was moderate to severe, then the speech vocabulary was confined to several words (more than two words-6 cases). Only two children suffering from conductive hearing loss both presented with complete lack of speech. A great number of healthy pre-school children with speech delay were found to have normal hearing. In this case, the otolaryngologist should be aware of the possible underlying clinical

  19. Effects of musical training and hearing loss on pitch discrimination

    DEFF Research Database (Denmark)

    Santurette, Sébastien; Bianchi, Federica; Dau, Torsten

    2018-01-01

    content of the sound and whether the harmonics are resolved by the auditory frequency analysis operated by cochlear processing. F0DLs are also heavily influenced by the amount of musical training received by the listener and by the spectrotemporal auditory processing deficits that often accompany...... sensorineural hearing loss. This paper reviews the latest evidence for how musical training and hearing loss affect pitch discrimination performance, based on behavioral F0DL experiments with complex tones containing either resolved or unresolved harmonics, carried out in listeners with different degrees...... of hearing loss and musicianship. A better understanding of the interaction between these two factors is crucial to determine whether auditory training based on musical tasks or targeted towards specific auditory cues may be useful to hearing-impaired patients undergoing hearing rehabilitation....

  20. Hearing loss in children with primary ciliary dyskinesia.

    Science.gov (United States)

    Kreicher, Kathryn L; Schopper, Heather K; Naik, Akash N; Hatch, Jonathan L; Meyer, Ted A

    2018-01-01

    To evaluate the type and severity of hearing impairment in pediatric patients with primary ciliary dyskinesia (PCD) and relate these measures to patient demographics, treatment options, and other otologic factors. A retrospective analysis of children with a diagnosis of PCD, Kartagener's syndrome, or situs inversus in the AudGen Database was conducted. Audiograms were analyzed for type of hearing loss (HL), severity, laterality, and progression. Medical charts were reviewed to identify factors that influence severity and progression of hearing loss. 56 patients met inclusion criteria and 42 patients had HL. 66.6% had bilateral and 33.3% had unilateral loss (70 total ears with HL). Conductive hearing loss (CHL) was the most common type of HL, though 30% of children had some sensorineural component to their hearing loss. 92.9% of children with HL received at least one diagnosis of otitis media, but HL did not improve in the majority (77.8%) of ears in our study regardless of ear tube placement. Slight to mild CHL and all types of otitis media are prevalent among patients with PCD, and some of these children have sensorineural hearing loss (SNHL). All patients diagnosed with situs inversus at birth should be evaluated by an otolaryngologist. Copyright © 2017 Elsevier B.V. All rights reserved.

  1. Evaluation of Extended-Wear Hearing Technology for Children with Hearing Loss.

    Science.gov (United States)

    Wolfe, Jace; Schafer, Erin; Martella, Natalie; Morais, Mila; Mann, Misty

    2015-01-01

    Research shows that many older children and teenagers who have mild to moderately severe sensorineural hearing loss do not use their hearing instruments during all waking hours. A variety of reasons may contribute toward this problem, including concerns about cosmetics associated with hearing aid use and the inconvenience of daily maintenance associated with hearing instruments. Extended-wear hearing instruments are inserted into the wearer's ear canal by an audiologist and are essentially invisible to outside observers. The goal of this study was to evaluate the potential benefits and limitations associated with use of extended-wear hearing instruments in a group of children with hearing loss. A two-way repeated measures design was used to examine performance differences obtained with the participants' daily-wear hearing instruments versus that obtained with extended-wear hearing instruments. Sixteen children, ages 10-17 yr old, with sensorineural hearing loss ranging from mild to moderately severe. Probe microphone measures were completed to evaluate the aided output of device. Behavioral test measures included word recognition in quiet, sentence recognition in noise, aided warble-tone thresholds, and psychophysical loudness scaling. Questionnaires were also administered to evaluate subjective performance with each hearing technology. Data logging suggested that many participants were not using their daily-wear hearing instruments during all waking hours (mean use was less than 6 h/day). Real ear probe microphone measurements indicated that a closer fit to the Desired Sensation Level Version 5 prescriptive targets was achieved with the children's daily-wear instruments when compared to the extended-wear instruments. There was no statistically significant difference in monosyllabic word recognition at 50 or 60 dBA obtained with the two hearing technologies. Sentence recognition in noise obtained with use of the extended-wear devices was, however, significantly

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

    Directory of Open Access Journals (Sweden)

    Lieber Po-Hung Li

    Full Text Available 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

  3. Hearing Loss and Kidney Dysfunction: Finding a Unifying Diagnosis

    Directory of Open Access Journals (Sweden)

    Praveena Iruku

    2013-01-01

    Full Text Available Microscopic polyangiitis (MPA is a systemic vasculitis that affects small caliber vessels, with renal and lung compromise. Diagnosis can be challenging; timely diagnosis and treatment are important to prevent devastating complication, particularly renal failure. We present a case of a patient with microscopic polyangiitis presented with renal and pulmonary involvements with concomitant sensorineural hearing loss. We provide diagnostic, therapeutic, and prognostic keys to microscopic polyangiitis.

  4. 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.

  5. Localization of A Novel Autosomal Recessive Non-Syndromic Hearing Impairment Locus (DFNB38) to 6q26–q27 in a Consanguineous Kindred from Pakistan

    OpenAIRE

    Ansar, Muhammad; Ramzan, Mohammad; Pham, Thanh L.; Yan, Kai; Jamal, Syed Muhammad; Haque, Sayedul; Ahmad, Wasim; Leal, Suzanne M.

    2003-01-01

    For autosomal recessive nonsyndromic hearing impairment over 30 loci have been mapped and 19 genes have been identified. DFNB38, a novel locus for autosomal recessive nonsyndromic hearing impairment, was localized in a consanguineous Pakistani kindred to 6q26–q27. The affected family members present with profound prelingual sensorineural hearing impairment and use sign language for communications. Linkage was established to microsatellite markers located on chromosome 6q26–q27 (Multipoint lod...

  6. 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.

  7. Hearing thresholds in adult Nigerians with diabetes mellitus: a case–control study

    Directory of Open Access Journals (Sweden)

    Nwosu JN

    2017-05-01

    Full Text Available Jones Ndubuisi Nwosu, Ethel Nkechi Chime Department of Otorhinolaryngology, College of Medicine, University of Nigeria, Enugu Campus, Enugu, Nigeria Objectives: To determine the prevalence, types and severity of hearing loss and associated factors in a hospital population of adult Nigerians with diabetes mellitus.Subjects and methods: This study was a prospective hospital-based study conducted at the Otorhinolaryngology and Diabetic Clinics of the University of Nigeria Teaching Hospital (UNTH Ituku-Ozalla, Enugu, for a period of 12 months. Consecutively presenting eligible adult diabetics and their age- and sex-matched healthy controls were recruited. Each case and control participant had clinical and otologic examination, followed by pure tone audiometry. Data were analyzed using descriptive and comparative statistics.Results: There were 224 patients and 192 control participants. The patients comprised 112 males and 112 females (sex ratio=1:1, whose mean age was 47.6 years (range: 26–80 years. The prevalence of hearing loss was 46.9%. This comprised 43.8% sensorineural and 3.1% conductive hearing losses. The distribution of hearing loss by severity was mild 25.0%, moderate 15.6% and severe 6.3%. The controls comprised 96 males and 96 females whose mean age was 44.6 years (range: 25–79 years. The prevalence of hearing loss was significantly higher overall and by type (sensorineural hearing loss, conductive hearing loss in cases compared with controls.Conclusion: The prevalence of hearing loss among diabetic adults at UNTH, Enugu, is comparatively high. Hearing loss is predominantly sensorineural and often mild to moderate in severity. Routine audiometric evaluation of all adult diabetics at UNTH is recommended. Keywords: adults, diabetes mellitus, hearing threshold

  8. 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

  9. 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

  10. [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.

  11. Hearing Aids

    Science.gov (United States)

    ... primarily useful in improving the hearing and speech comprehension of people who have hearing loss that results ... and you can change the program for different listening environments—from a small, quiet room to a ...

  12. Predicting hearing thresholds and occupational hearing loss with multiple-frequency auditory steady-state responses.

    Science.gov (United States)

    Hsu, Ruey-Fen; Ho, Chi-Kung; Lu, Sheng-Nan; Chen, Shun-Sheng

    2010-10-01

    An objective investigation is needed to verify the existence and severity of hearing impairments resulting from work-related, noise-induced hearing loss in arbitration of medicolegal aspects. We investigated the accuracy of multiple-frequency auditory steady-state responses (Mf-ASSRs) between subjects with sensorineural hearing loss (SNHL) with and without occupational noise exposure. Cross-sectional study. Tertiary referral medical centre. Pure-tone audiometry and Mf-ASSRs were recorded in 88 subjects (34 patients had occupational noise-induced hearing loss [NIHL], 36 patients had SNHL without noise exposure, and 18 volunteers were normal controls). Inter- and intragroup comparisons were made. A predicting equation was derived using multiple linear regression analysis. ASSRs and pure-tone thresholds (PTTs) showed a strong correlation for all subjects (r = .77 ≈ .94). The relationship is demonstrated by the equationThe differences between the ASSR and PTT were significantly higher for the NIHL group than for the subjects with non-noise-induced SNHL (p tool for objectively evaluating hearing thresholds. Predictive value may be lower in subjects with occupational hearing loss. Regardless of carrier frequencies, the severity of hearing loss affects the steady-state response. Moreover, the ASSR may assist in detecting noise-induced injury of the auditory pathway. A multiple linear regression equation to accurately predict thresholds was shown that takes into consideration all effect factors.

  13. Hear, hear, what, what

    Energy Technology Data Exchange (ETDEWEB)

    1985-02-01

    Noise is now the most serious health hazard in industry. People risk suffering severe damage to their hearing and health generally through exposure to noise levels which exceed the risk limit of 95-90 dB(A). Stress related problems are often directly the result of exposure to noise. Furthermore, noise leads to reduced productivity, increased absenteeism and compensation claims. In a series of eight reports, Bilson technologists detail the specific noise problems commonly encountered in each of the major areas of industry. These range from the concrete and construction industry through sawmilling, wood working, mining and mechanical engineering, to textile and food manufacture. This report discusses the noise in mining, considering drills, load haul dump machines, surface mines, earth moving equipment, treatment plants, frequency spectra of noise, and finally, choice of hearing protection.

  14. Newborn Hearing Screening and Early Diagnostic in the NICU

    Directory of Open Access Journals (Sweden)

    Maria Francisca Colella-Santos

    2014-01-01

    Full Text Available The aim was to describe the outcome of neonatal hearing screening (NHS and audiological diagnosis in neonates in the NICU. The sample was divided into Group I: neonates who underwent NHS in one step and Group II: neonates who underwent a test and retest NHS. NHS procedure was automated auditory brainstem response. NHS was performed in 82.1% of surviving neonates. For GI, referral rate was 18.6% and false-positive was 62.2% (normal hearing in the diagnostic stage. In GII, with retest, referral rate dropped to 4.1% and false-positive to 12.5%. Sensorineural hearing loss was found in 13.2% of infants and conductive in 26.4% of cases. There was one case of auditory neuropathy spectrum (1.9%. Dropout rate in whole process was 21.7% for GI and 24.03% for GII. We concluded that it was not possible to perform universal NHS in the studied sample or, in many cases, to apply it within the first month of life. Retest reduced failure and false-positive rate and did not increase evasion, indicating that it is a recommendable step in NHS programs in the NICU. The incidence of hearing loss was 2.9%, considering sensorineural hearing loss (0.91%, conductive (1.83% and auditory neuropathy spectrum (0.19%.

  15. 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.

  16. 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 ...

  17. 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.

  18. Sound localization in noise in hearing-impaired listeners.

    Science.gov (United States)

    Lorenzi, C; Gatehouse, S; Lever, C

    1999-06-01

    The present study assesses the ability of four listeners with high-frequency, bilateral symmetrical sensorineural hearing loss to localize and detect a broadband click train in the frontal-horizontal plane, in quiet and in the presence of a white noise. The speaker array and stimuli are identical to those described by Lorenzi et al. (in press). The results show that: (1) localization performance is only slightly poorer in hearing-impaired listeners than in normal-hearing listeners when noise is at 0 deg azimuth, (2) localization performance begins to decrease at higher signal-to-noise ratios for hearing-impaired listeners than for normal-hearing listeners when noise is at +/- 90 deg azimuth, and (3) the performance of hearing-impaired listeners is less consistent when noise is at +/- 90 deg azimuth than at 0 deg azimuth. The effects of a high-frequency hearing loss were also studied by measuring the ability of normal-hearing listeners to localize the low-pass filtered version of the clicks. The data reproduce the effects of noise on three out of the four hearing-impaired listeners when noise is at 0 deg azimuth. They reproduce the effects of noise on only two out of the four hearing-impaired listeners when noise is at +/- 90 deg azimuth. The additional effects of a low-frequency hearing loss were investigated by attenuating the low-pass filtered clicks and the noise by 20 dB. The results show that attenuation does not strongly affect localization accuracy for normal-hearing listeners. Measurements of the clicks' detectability indicate that the hearing-impaired listeners who show the poorest localization accuracy also show the poorest ability to detect the clicks. The inaudibility of high frequencies, "distortions," and reduced detectability of the signal are assumed to have caused the poorer-than-normal localization accuracy for hearing-impaired listeners.

  19. Investigating the Impact of Hearing Aid Use and Auditory Training on Cognition, Depressive Symptoms, and Social Interaction in Adults With Hearing Loss: Protocol for a Crossover Trial.

    Science.gov (United States)

    Nkyekyer, Joanna; Meyer, Denny; Blamey, Peter J; Pipingas, Andrew; Bhar, Sunil

    2018-03-23

    Sensorineural hearing loss is the most common sensory deficit among older adults. Some of the psychosocial consequences of this condition include difficulty in understanding speech, depression, and social isolation. Studies have shown that older adults with hearing loss show some age-related cognitive decline. Hearing aids have been proven as successful interventions to alleviate sensorineural hearing loss. In addition to hearing aid use, the positive effects of auditory training-formal listening activities designed to optimize speech perception-are now being documented among adults with hearing loss who use hearing aids, especially new hearing aid users. Auditory training has also been shown to produce prolonged cognitive performance improvements. However, there is still little evidence to support the benefits of simultaneous hearing aid use and individualized face-to-face auditory training on cognitive performance in adults with hearing loss. This study will investigate whether using hearing aids for the first time will improve the impact of individualized face-to-face auditory training on cognition, depression, and social interaction for adults with sensorineural hearing loss. The rationale for this study is based on the hypothesis that, in adults with sensorineural hearing loss, using hearing aids for the first time in combination with individualized face-to-face auditory training will be more effective for improving cognition, depressive symptoms, and social interaction rather than auditory training on its own. This is a crossover trial targeting 40 men and women between 50 and 90 years of age with either mild or moderate symmetric sensorineural hearing loss. Consented, willing participants will be recruited from either an independent living accommodation or via a community database to undergo a 6-month intensive face-to-face auditory training program (active control). Participants will be assigned in random order to receive hearing aid (intervention) for

  20. Investigating the Impact of Hearing Aid Use and Auditory Training on Cognition, Depressive Symptoms, and Social Interaction in Adults With Hearing Loss: Protocol for a Crossover Trial

    Science.gov (United States)

    Meyer, Denny; Blamey, Peter J; Pipingas, Andrew; Bhar, Sunil

    2018-01-01

    Background Sensorineural hearing loss is the most common sensory deficit among older adults. Some of the psychosocial consequences of this condition include difficulty in understanding speech, depression, and social isolation. Studies have shown that older adults with hearing loss show some age-related cognitive decline. Hearing aids have been proven as successful interventions to alleviate sensorineural hearing loss. In addition to hearing aid use, the positive effects of auditory training—formal listening activities designed to optimize speech perception—are now being documented among adults with hearing loss who use hearing aids, especially new hearing aid users. Auditory training has also been shown to produce prolonged cognitive performance improvements. However, there is still little evidence to support the benefits of simultaneous hearing aid use and individualized face-to-face auditory training on cognitive performance in adults with hearing loss. Objective This study will investigate whether using hearing aids for the first time will improve the impact of individualized face-to-face auditory training on cognition, depression, and social interaction for adults with sensorineural hearing loss. The rationale for this study is based on the hypothesis that, in adults with sensorineural hearing loss, using hearing aids for the first time in combination with individualized face-to-face auditory training will be more effective for improving cognition, depressive symptoms, and social interaction rather than auditory training on its own. Methods This is a crossover trial targeting 40 men and women between 50 and 90 years of age with either mild or moderate symmetric sensorineural hearing loss. Consented, willing participants will be recruited from either an independent living accommodation or via a community database to undergo a 6-month intensive face-to-face auditory training program (active control). Participants will be assigned in random order to receive

  1. 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...

  2. Mutation analysis of SLC26A4 for Pendred syndrome and nonsyndromic hearing loss by high-resolution melting

    DEFF Research Database (Denmark)

    Chen, Neng; Tranebjærg, Lisbeth; Rendtorff, Nanna Dahl

    2011-01-01

    Pendred syndrome and DFNB4 (autosomal recessive nonsyndromic congenital deafness, locus 4) are associated with autosomal recessive congenital sensorineural hearing loss and mutations in the SLC26A4 gene. Extensive allelic heterogeneity, however, necessitates analysis of all exons and splice sites...

  3. A common SLC26A4-linked haplotype underlying non-syndromic hearing loss with enlargement of the vestibular aqueduct

    DEFF Research Database (Denmark)

    Chattaraj, Parna; Munjal, Tina; Honda, Keiji

    2017-01-01

    BACKGROUND: Enlargement of the vestibular aqueduct (EVA) is the most common radiological abnormality in children with sensorineural hearing loss. Mutations in coding regions and splice sites of the SLC26A4 gene are often detected in Caucasians with EVA. Approximately one-fourth of patients with E...

  4. [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

  5. Mutational spectrum of the WFS1 gene in Wolfram syndrome, nonsyndromic hearing impairment, diabetes mellitus, and psychiatric disease

    NARCIS (Netherlands)

    Cryns, K; Sivakumaran, TA; Van den Ouweland, JMW; Pennings, RJE; Cremers, CWRJ; Flothmann, K; Young, TL; Smith, RJH; Lesperance, MM; Van Camp, G

    2003-01-01

    WFS1 is a novel gene and encodes an 890 amino-acid glycoprotein (wolframin), predominantly localized in the endoplasmic reticulum. Mutations in WFS1 underlie autosomal recessive Wolfram syndrome and autosomal dominant low frequency sensorineural hearing impairment (LFSNHI) DFNA6/14. In addition,

  6. Mutational spectrum of the WFS1 gene in Wolfram syndrome, nonsyndromic hearing impairment, diabetes mellitus, and psychiatric disease.

    NARCIS (Netherlands)

    Cryns, K.; Sivakumaran, T.A.; Ouweland, J.M.W. van den; Pennings, R.J.E.; Cremers, C.W.R.J.; Flothmann, K.; Young, T.L.; Smith, R.J.H.; Lesperance, M.M.; Camp, G. van

    2003-01-01

    WFS1 is a novel gene and encodes an 890 amino-acid glycoprotein (wolframin), predominantly localized in the endoplasmic reticulum. Mutations in WFS1 underlie autosomal recessive Wolfram syndrome and autosomal dominant low frequency sensorineural hearing impairment (LFSNHI) DFNA6/14. In addition,

  7. Delayed diagnosis of childhood deafness: the value of false negatives in the Programme for Early Detection of Neonatal Hearing Loss.

    Science.gov (United States)

    Martínez-Pacheco, María C; Ferrán de la Cierva, Luis; García-Purriños, Francisco J

    Despite its importance, the existence of false negatives (patients who are told they hear well, but they have some degree of hipacusia) is rarely evaluated in programs for early detection of hearing loss. The aim of this study is to determine the variables that can lead to a delayed diagnosis, especially the existence of false negatives and the lack of registration of risk factors. A retrospective study of prevalence has been carried out, in which the medical records of children diagnosed with sensorineural hearing loss born within 2005 and 2012 in the health centers of study have been analyzed. Of the 32 children with sensorineural hearing loss, 16 passed the OAE, 12 did not passed the OAE, and in four they were not carried out. Of the children who passed the OAE, 57% have severe hearing loss. 66% of children with hearing loss presented a risk factor for hearing loss at birth, being the most frecuent family history of hearing loss, but only 7% of those with family history of hearing loss were included in the risk group. The results of the study indicate that the late diagnosis of hearing loss is related to the presence of false negatives to the OAE and the non-registration of risk factors. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

  8. A case of antenatal Bartter syndrome with sensorineural deafness.

    Science.gov (United States)

    Lee, Hyun Seung; Cheong, Hae Il; Ki, Chang-Seok

    2010-10-01

    Bartter syndrome type IV, also known as Bartter syndrome with sensorineural deafness (BSND), is caused by loss-of-function mutations in the BSND gene, which encodes barttin, an accessory subunit of chloride channels located in the kidney and inner ear. Patients with BS IV have a highly variable clinical phenotype. This report concerns a Korean male patient with antenatal Bartter syndrome due to a homozygous BSND p.G47R mutation, who presented with severe perinatal symptoms followed by a relatively benign course with preserved renal function after early infancy. In addition, the clinical features and the laboratory data of the patient were compared with those of previously reported patients with the same mutation.

  9. Inflammatory and immune responses in the cochlea: potential therapeutic targets for sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Masato eFujioka

    2014-12-01

    Full Text Available The inner ear was previously assumed to be an immune-privileged organ due to the existence of its tight junction-based blood-labyrinth barrier. However, studies performed during the past decade revealed that the mesenchymal region of the cochlea, including its lateral wall, is a common site of inflammation. Neutrophils do not enter this region, which is consistent with the old dogma; however, bone marrow-derived resident macrophages are always present in the spiral ligament of the lateral wall and are activated in response to various types of insults, including noise exposure, ischemia, mitochondrial damage and surgical stress. Recent studies have also revealed another type of immune cell, called perivascular melanocyte-like macrophages (PVM/Ms, in the stria vascularis. These dedicated antigen-presenting cells also control vascular contraction and permeability. This review discusses a series of reports regarding inflammatory/immune cells in the cochlear lateral wall, the pathways involved in cochlear damage and their potential as therapeutic targets.

  10. Characteristics of hearing loss in HDR (hypoparathyroidism, sensorineural deafness, renal dysplasia) syndrome

    NARCIS (Netherlands)

    van Looij, Marjolein A. J.; Meijers-Heijboer, Hanne; Beetz, Rolf; Thakker, Rajesh V.; Christie, Paul T.; Feenstra, Lou W.; van Zanten, Bert G. A.

    2006-01-01

    Haploinsufficiency of the zinc finger transcription factor GATA3 causes the triad of hypoparathyroidism, deafness and renal dysplasia, known by its acronym HDR syndrome. The purpose of the current study was to describe in detail the auditory phenotype in human HDR patients and compare these to

  11. 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…

  12. Hearing Impairment Among Children Referred to a Public Audiology Clinic in Gaborone, Botswana.

    Science.gov (United States)

    Banda, Francis M; Powis, Kathleen M; Mokoka, Agnes B; Mmapetla, Moalosi; Westmoreland, Katherine D; David, Thuso; Steenhoff, Andrew P

    2018-01-01

    Objective . To describe and quantify hearing impairment among children referred to the audiology clinic in Princess Marina Hospital, a public referral hospital in Botswana. Methods . In a retrospective case series, we reviewed medical records of children aged 10 years and younger whose hearing was assessed between January 2006 and December 2015 at the audiology clinic of Princess Marina Hospital in Gaborone, Botswana. Results . Of 622 children, 50% were male, and median age was 6.7 years (interquartile range = 5.0-8.3). Hearing impairment was diagnosed in 32% of clinic attendees, comprising sensorineural (23%), conductive (25%), and mixed (11%) hearing loss, while 41% of children with diagnosed hearing impairment did not have a classification type. Hearing impairment was mild in 22.9%, moderate in 22.4%, severe in 19.4%, profound in 16.9%, and of undocumented severity in 18.4%. Children younger than 5 years were 2.7 times (95% confidence interval = 1.29-5.49; P = .008) more likely to be diagnosed with sensorineural hearing impairment compared with those older than 5 years. By contrast, children older than 5 years were 9.6 times (95% confidence interval = 2.22-41.0; P = .002) more likely to be diagnosed with conductive hearing loss compared with those under 5 years. Conclusion . Hearing impairment was common among children referred to this audiology clinic in Botswana. Of those with hearing impairment, more than a third had moderate or severe deficits, suggesting that referrals for hearing assessments are not occurring early enough. Hearing awareness programs individually tailored to parents, educators, and health care workers are needed. Neonatal and school hearing screening programs would also be beneficial.

  13. 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

    2010-10-01

    Full Text Available 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

  14. Consumer Preferences for Hearing Aid Attributes

    Science.gov (United States)

    Lataille, Angela T.; Buttorff, Christine; White, Sharon; Niparko, John K.

    2012-01-01

    Low utilization of hearing aids has drawn increased attention to the study of consumer preferences using both simple ratings (e.g., Likert scale) and conjoint analyses, but these two approaches often produce inconsistent results. The study aims to directly compare Likert scales and conjoint analysis in identifying important attributes associated with hearing aids among those with hearing loss. Seven attributes of hearing aids were identified through qualitative research: performance in quiet settings, comfort, feedback, frequency of battery replacement, purchase price, water and sweat resistance, and performance in noisy settings. The preferences of 75 outpatients with hearing loss were measured with both a 5-point Likert scale and with 8 paired-comparison conjoint tasks (the latter being analyzed using OLS [ordinary least squares] and logistic regression). Results were compared by examining implied willingness-to-pay and Pearson’s Rho. A total of 56 respondents (75%) provided complete responses. Two thirds of respondents were male, most had sensorineural hearing loss, and most were older than 50; 44% of respondents had never used a hearing aid. Both methods identified improved performance in noisy settings as the most valued attribute. Respondents were twice as likely to buy a hearing aid with better functionality in noisy environments (p < .001), and willingness to pay for this attribute ranged from US$2674 on the Likert to US$9000 in the conjoint analysis. The authors find a high level of concordance between the methods—a result that is in stark contrast with previous research. The authors conclude that their result stems from constraining the levels on the Likert scale. PMID:22514094

  15. 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. PMID:25999874

  16. Advances in the Understanding of the Genetic Causes of Hearing Loss in Children Inform a Rational Approach to Evaluation.

    Science.gov (United States)

    Carey, John C; Palumbos, Janice C

    2016-10-01

    Hearing loss represents the most common sensory disability of children. Remarkable advances in the identification of genes underlying nonsyndromic and syndromic hearing loss in just the last 2 decades have led to the ability to determine the specific genetic cause of hearing loss in many children. Surprisingly one gene, GJB2, encoding the protein connexin-26, accounts for about 20 % of sensorineural hearing loss (including in India) and is considered the first tier test in evaluating an infant with unexplained congenital hearing loss. Using the knowledge of the etiology of hearing loss, the authors propose a diagnostic reasoning process for the assessment of a child in the pediatric setting. Second tier testing consists of the multiple gene panels using whole exome sequencing strategies, and is becoming available in some regions of the world including the US. Referral to medical genetics is always a consideration in a child with no explanation for the hearing loss and in families with questions about recurrence risk.

  17. 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.

  18. Do Hearing Protectors Protect Hearing?

    Science.gov (United States)

    Groenewold, Matthew R.; Masterson, Elizabeth A.; Themann, Christa L.; Davis, Rickie R.

    2015-01-01

    Background We examined the association between self-reported hearing protection use at work and incidence of hearing shifts over a 5-year period. Methods Audiometric data from 19,911 workers were analyzed. Two hearing shift measures—OSHA standard threshold shift (OSTS) and high-frequency threshold shift (HFTS)—were used to identify incident shifts in hearing between workers’ 2005 and 2009 audiograms. Adjusted odds ratios were generated using multivariable logistic regression with multi-level modeling. Results The odds ratio for hearing shift for workers who reported never versus always wearing hearing protection was nonsignificant for OSTS (OR 1.23, 95% CI 0.92–1.64) and marginally significant for HFTS (OR 1.26, 95% CI 1.00–1.59). A significant linear trend towards increased risk of HFTS with decreased use of hearing protection was observed (P = 0.02). Conclusion The study raises concern about the effectiveness of hearing protection as a substitute for noise control to prevent noise-induced hearing loss in the workplace. Am. J. Ind. Med. 57:1001–1010, 2014. Published 2014. This article is a U.S. Government work and is in the public domain in the USA. PMID:24700499

  19. Vertigo with sudden hearing loss: audio-vestibular characteristics.

    Science.gov (United States)

    Pogson, Jacob M; Taylor, Rachael L; Young, Allison S; McGarvie, Leigh A; Flanagan, Sean; Halmagyi, G Michael; Welgampola, Miriam S

    2016-10-01

    Acute vertigo with sudden sensorineural hearing loss (SSNHL) is a rare clinical emergency. Here, we report the audio-vestibular test profiles of 27 subjects who presented with these symptoms. The vestibular test battery consisted of a three-dimensional video head impulse test (vHIT) of semicircular canal function and recording ocular and cervical vestibular-evoked myogenic potentials (oVEMP, cVEMP) to test otolith dysfunction. Unlike vestibular neuritis, where the horizontal and anterior canals with utricular function are more frequently impaired, 74 % of subjects with vertigo and SSNHL demonstrated impairment of the posterior canal gain (0.45 ± 0.20). Only 41 % showed impairment of the horizontal canal gains (0.78 ± 0.27) and 30 % of the anterior canal gains (0.79 ± 0.26), while 38 % of oVEMPs [asymmetry ratio (AR) = 41.0 ± 41.3 %] and 33 % of cVEMPs (AR = 47.3 ± 41.2 %) were significantly asymmetrical. Twenty-three subjects were diagnosed with labyrinthitis/labyrinthine infarction in the absence of evidence for an underlying pathology. Four subjects had a definitive diagnosis [Ramsay Hunt Syndrome, vestibular schwannoma, anterior inferior cerebellar artery (AICA) infarction, and traction injury]. Ischemia involving the common-cochlear or vestibulo-cochlear branches of the labyrinthine artery could be the simplest explanation for vertigo with SSNHL. Audio-vestibular tests did not provide easy separation between ischaemic and non-ischaemic causes of vertigo with SSNHL.

  20. Hearing Impairment

    Science.gov (United States)

    ... Ear Hears Think about how you can feel speakers vibrate on your sound system or feel your throat vibrate when you speak. Sound, which is made up of invisible waves of energy, causes these vibrations. Hearing begins when sound waves that travel through ...

  1. Sensorineural Deafness, Distinctive Facial Features and Abnormal Cranial Bones

    Science.gov (United States)

    Gad, Alona; Laurino, Mercy; Maravilla, Kenneth R.; Matsushita, Mark; Raskind, Wendy H.

    2008-01-01

    The Waardenburg syndromes (WS) account for approximately 2% of congenital sensorineural deafness. This heterogeneous group of diseases currently can be categorized into four major subtypes (WS types 1-4) on the basis of characteristic clinical features. Multiple genes have been implicated in WS, and mutations in some genes can cause more than one WS subtype. In addition to eye, hair and skin pigmentary abnormalities, dystopia canthorum and broad nasal bridge are seen in WS type 1. Mutations in the PAX3 gene are responsible for the condition in the majority of these patients. In addition, mutations in PAX3 have been found in WS type 3 that is distinguished by musculoskeletal abnormalities, and in a family with a rare subtype of WS, craniofacial-deafness-hand syndrome (CDHS), characterized by dysmorphic facial features, hand abnormalities, and absent or hypoplastic nasal and wrist bones. Here we describe a woman who shares some, but not all features of WS type 3 and CDHS, and who also has abnormal cranial bones. All sinuses were hypoplastic, and the cochlea were small. No sequence alteration in PAX3 was found. These observations broaden the clinical range of WS and suggest there may be genetic heterogeneity even within the CDHS subtype. PMID:18553554

  2. 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...

  3. The development and standardization of Self-assessment for Hearing Screening of the Elderly.

    Science.gov (United States)

    Kim, Gibbeum; Na, Wondo; Kim, Gungu; Han, Woojae; Kim, Jinsook

    2016-01-01

    The present study aimed to develop and standardize a screening tool for elderly people who wish to check for themselves their level of hearing loss. The Self-assessment for Hearing Screening of the Elderly (SHSE) consisted of 20 questions based on the characteristics of presbycusis using a five-point scale: seven questions covered general issues related to sensorineural hearing loss, seven covered hearing difficulty under distracting listening conditions, two covered hearing difficulty with fast-rated speech, and four covered the working memory function during communication. To standardize SHSE, 83 elderly participants took part in the study: 25 with normal hearing, and 22, 23, and 13 with mild, moderate, and moderate-to-severe sensorineural hearing loss, respectively, according to their hearing sensitivity. All were retested 3 weeks later using the same questionnaire to confirm its reliability. In addition, validity was assessed using various hearing tests such as a sentence test with background noise, a time-compressed speech test, and a digit span test. SHSE and its subcategories showed good internal consistency. SHSE and its subcategories demonstrated high test-retest reliability. A high correlation was observed between the total scores and pure-tone thresholds, which indicated gradually increased SHSE scores of 42.24%, 55.27%, 66.61%, and 78.15% for normal hearing, mild, moderate, and moderate-to-severe groups, respectively. With regard to construct validity, SHSE showed a high negative correlation with speech perception scores in noise and a moderate negative correlation with scores of time-compressed speech perception. However, there was no statistical correlation between digit span results and either the SHSE total or its subcategories. A confirmatory factor analysis supported three factors in SHSE. We found that the developed SHSE had valuable internal consistency, test-retest reliability, and convergent and construct validity. These results suggest that

  4. 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…

  5. Clinical study on unilateral hearing loss in children

    International Nuclear Information System (INIS)

    Masuda, Sawako; Usui, Satoko

    2007-01-01

    A series of 60 children was studied (aged 0 to 10 years, 32 boys, 28 girls) with severe unilateral sensorineural hearing loss of unknown etiology. There were two peaks, at 0 and 6 years. In 19 children, hearing loss was identified during a conservative general health checkup for school or preschool children. In 21 children aged 0 years, 16 were suspected of hearing loss by newborn hearing screening. Temporal bone computed tomography scans were examined in 51 patients. Sixteen ears (31.4%) with hearing loss had inner ear and/or internal auditory canal abnormalities. In one patient, the anomaly was the presence of a bony wall dividing the internal auditory canal into two separate compartments associated with severe inner ear hypoplasia. Two patients had a common cavity. In one of these patients, the anomaly was revealed because of severe bacterial meningitis, and another was detected by newborn hearing screening. Six patients had a narrow internal auditory canal, 4 had a narrow internal auditory canal and hypoplastic cochlea, and 1 had a narrow internal auditory canal and cystic vestibule, and lateral semicircular canal dysplasia. Two patients had a cystic vestibule and lateral semicircular canal dysplasia. One case showed fluctuation of the hearing level in the contralateral ear with normal hearing during the observation period at an average of 20 months. The number of children whose unilateral hearing loss is detected early by newborn hearing screening has enormously increased. Strategies for follow-up, early intervention, and support for families are necessary for young children with unilateral hearing impairment. (author)

  6. Noise-Induced Hearing Loss (NIHL: literature review with a focus on occupational medicine

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    Metidieri, Mirella Melo

    2013-04-01

    Full Text Available According to the Ministry of Health (2006, Noise-Induced Hearing Loss (NIHL is hearing loss caused by prolonged exposure to noise. It is characterized as sensorineural hearing loss and is usually bilateral, irreversible, and progressive while the exposure to noise continues. A NIHL is a predictable and preventable disease with an epidemiologically relevant prevalence in urban communities. The hearing loss begins and predominates in the frequencies of 3, 4, and 6 kHz and eventually progresses to 8, 2, 1, 0.5, and 0.25 kHz. In Brazil, regulatory standard 15 limits the exposure to continuous noise to no more than 4 hours' exposure to 90 dBA and a maximum level of 85 dB for a full 8-hour working period. As NIHL is a preventable and predictable disease, preventive action by professionals may be able to change the prevalence of hearing loss in noisy environments.

  7. Noise-Induced Hearing Loss (NIHL): literature review with a focus on occupational medicine.

    Science.gov (United States)

    Metidieri, Mirella Melo; Rodrigues, Hugo Fernandes Santos; Filho, Francisco José Motta Barros de Oliveira; Ferraz, Daniela Pereira; Neto, Antonio Fausto de Almeida; Torres, Sandro

    2013-04-01

    According to the Ministry of Health (2006), Noise-Induced Hearing Loss (NIHL) is hearing loss caused by prolonged exposure to noise. It is characterized as sensorineural hearing loss and is usually bilateral, irreversible, and progressive while the exposure to noise continues. A NIHL is a predictable and preventable disease with an epidemiologically relevant prevalence in urban communities. The hearing loss begins and predominates in the frequencies of 3, 4, and 6 kHz and eventually progresses to 8, 2, 1, 0.5, and 0.25 kHz. In Brazil, regulatory standard 15 limits the exposure to continuous noise to no more than 4 hours' exposure to 90 dBA and a maximum level of 85 dB for a full 8-hour working period. As NIHL is a preventable and predictable disease, preventive action by professionals may be able to change the prevalence of hearing loss in noisy environments.

  8. Inner ear malformations in patients with sensorineural heating loss: detection with gradient-echo (3DFT-CISS) MRI

    Energy Technology Data Exchange (ETDEWEB)

    Casselman, J.W. [Dept. of Radiology, A.Z. St.-Jan Brugge, Bruges (Belgium); Kuhweide, R. [Dept. of Otorhinolaryngology, A.Z. St.-Jan Brugge, Bruges (Belgium); Ampe, W. [Dept. of Otorhinolaryngology, A.Z. St.-Jan Brugge, Bruges (Belgium); D`Hont, G.D. [Dept. of Otorhinolaryngology, A.Z. St.-Jan Brugge, Bruges (Belgium); Offeciers, E.F. [ENT Dept., Sint-Augustinus Medical Inst., Univ. of Antwerp (Belgium); Faes, W.K. [Dept. of Radiology, A.Z. St.-Jan Brugge, Bruges (Belgium); Pattyn, G. [Dept. of Radiology, A.Z. St.-Jan Brugge, Bruges (Belgium)

    1996-04-01

    The sensitivity of different MRI sequences in the detection of inner ear malformations in patients presenting with sensorineural hearing loss (SNHL) and/or vertigo was evaluated. We studied 650 patients presenting with SNHL and/or vertigo, clinically not suspected of having inner ear malformations. The sensitivity of T1-weigted, Gd-enhanced T1-weighted and (when available) T2-weighted spin-echo images, and three-dimensional Fourier transformation-constructive interference in steady state (3DFT-CISS) gradient-echo images, to unexpected malformations was assessed. Inner ear malformations were found in 15 (2.3%) of these patients. Enlargement of the endolymphatic duct and sac was the most frequent malformation, found in 11 patients. The 3DFT-CISS images showed all lesions; the other sequences were less sensitive and the pathology was missed, partially or only retrospectively seen in 11 of the 15 patients. Therefore, in addition to the routine unenhanced and Gd-enhanced T1-weighted and T2-weighted images, thin gradient-echo (3DFT-CISS) images are necessary to detect all clinically unexpected inner ear malformations in patients presenting with vertigo and/or SNHL. (orig.)

  9. Inner ear malformations in patients with sensorineural heating loss: detection with gradient-echo (3DFT-CISS) MRI

    International Nuclear Information System (INIS)

    Casselman, J.W.; Kuhweide, R.; Ampe, W.; D'Hont, G.D.; Offeciers, E.F.; Faes, W.K.; Pattyn, G.

    1996-01-01

    The sensitivity of different MRI sequences in the detection of inner ear malformations in patients presenting with sensorineural hearing loss (SNHL) and/or vertigo was evaluated. We studied 650 patients presenting with SNHL and/or vertigo, clinically not suspected of having inner ear malformations. The sensitivity of T1-weigted, Gd-enhanced T1-weighted and (when available) T2-weighted spin-echo images, and three-dimensional Fourier transformation-constructive interference in steady state (3DFT-CISS) gradient-echo images, to unexpected malformations was assessed. Inner ear malformations were found in 15 (2.3%) of these patients. Enlargement of the endolymphatic duct and sac was the most frequent malformation, found in 11 patients. The 3DFT-CISS images showed all lesions; the other sequences were less sensitive and the pathology was missed, partially or only retrospectively seen in 11 of the 15 patients. Therefore, in addition to the routine unenhanced and Gd-enhanced T1-weighted and T2-weighted images, thin gradient-echo (3DFT-CISS) images are necessary to detect all clinically unexpected inner ear malformations in patients presenting with vertigo and/or SNHL. (orig.)

  10. Hearing Aids

    Science.gov (United States)

    ... listen to TV or your music player, play videogames, or use your phone. Talk to your audiologist ... your audiologist several times, but it's worth the benefit of being able to hear your friends and ...

  11. 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.

  12. Comparisons of hearing threshold changes in male workers with unilateral conductive hearing loss exposed to workplace noise: a retrospective cohort study for 8 years.

    Science.gov (United States)

    Park, Sang Jin; Sung, Joo Hyun; Sim, Chang Sun; Yun, Seok Hyeon; Yeom, Jeong Han; Kwon, Joong-Keun; Lee, Jiho

    2016-01-01

    The purpose of this study was to investigate hearing threshold changes of workers with unilateral conductive hearing loss who were exposed to workplace noise for 8-years. Among 1819 workers at a shipyard in Ulsan, 78 subjects with an air-bone gap ≥10 dBHL in unilateral ears were selected. Factors that could affect hearing were acquired from questionnaires, physical examinations, and biochemistry examinations. Paired t-test was conducted to compare the hearing threshold changes over time between conductive hearing loss (CHL) ear and sensorineural hearing loss (SNHL) ear. The study included male subjects aged 48.7 ± 2.9, having worked for 29.8 ± 2.7 years. Hearing thresholds increased significantly in CHL ears and SNHL ears at all frequencies (0.5-6 kHz) during follow-up period (p hearing threshold changes was lower in ears with conductive hearing loss than in contralateral ears. This is suggested as a protective effect against noise exposure.

  13. Hearing Loss in Infants with Microcephaly and Evidence of Congenital Zika Virus Infection - Brazil, November 2015-May 2016.

    Science.gov (United States)

    Leal, Mariana C; Muniz, Lilian F; Ferreira, Tamires S A; Santos, Cristiane M; Almeida, Luciana C; Van Der Linden, Vanessa; Ramos, Regina C F; Rodrigues, Laura C; Neto, Silvio S Caldas

    2016-09-02

    Congenital infection with Zika virus causes microcephaly and other brain abnormalities (1). Hearing loss associated with other congenital viral infections is well described; however, little is known about hearing loss in infants with congenital Zika virus infection. A retrospective assessment of a series of 70 infants aged 0-10 months with microcephaly and laboratory evidence of Zika virus infection was conducted by the Hospital Agamenon Magalhães in Brazil and partners. The infants were enrolled during November 2015-May 2016 and had screening and diagnostic hearing tests. Five (7%) infants had sensorineural hearing loss, all of whom had severe microcephaly; however, one child was tested after receiving treatment with an ototoxic antibiotic. If this child is excluded, the prevalence of sensorineural hearing loss was 5.8% (four of 69), which is similar to that seen in association with other congenital viral infections. Additional information is needed to understand the prevalence and spectrum of hearing loss in children with congenital Zika virus infection; all infants born to women with evidence of Zika virus infection during pregnancy should have their hearing tested, including infants who appear normal at birth.

  14. 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.

  15. A novel mutation in the WFS1 gene identified in a Taiwanese family with low-frequency hearing impairment

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    Chung Shing-Fang

    2007-05-01

    Full Text Available Abstract Background Wolfram syndrome gene 1 (WFS1 accounts for most of the familial nonsyndromic low-frequency sensorineural hearing loss (LFSNHL which is characterized by sensorineural hearing losses equal to and below 2000 Hz. The current study aimed to contribute to our understanding of the molecular basis of LFSNHL in an affected Taiwanese family. Methods The Taiwanese family with LFSNHL was phenotypically characterized using audiologic examination and pedigree analysis. Genetic characterization was performed by direct sequencing of WFS1 and mutation analysis. Results Pure tone audiometry confirmed that the family members affected with LFSNHL had a bilateral sensorineural hearing loss equal to or below 2000 Hz. The hearing loss threshold of the affected members showed no progression, a characteristic that was consistent with a mutation in the WFS1 gene located in the DFNA6/14/38 locus. Pedigree analysis showed a hereditarily autosomal dominant pattern characterized by a full penetrance. Among several polymorphisms, a missense mutation Y669H (2005T>C in exon 8 of WFS1 was identified in members of a Taiwanese family diagnosed with LFSNHL but not in any of the control subjects. Conclusion We discovered a novel heterozygous missense mutation in exon 8 of WFS1 (i.e., Y669H which is likely responsible for the LFSNHL phenotype in this particular Taiwanese family.

  16. Efficient estimates of cochlear hearing loss parameters in individual listeners

    DEFF Research Database (Denmark)

    Fereczkowski, Michal; Jepsen, Morten Løve; Dau, Torsten

    2013-01-01

    It has been suggested that the level corresponding to the knee-point of the basilar membrane (BM) input/output (I/O) function can be used to estimate the amount of inner- and outer hair-cell loss (IHL, OHL) in listeners with a moderate cochlear hearing impairment Plack et al. (2004). According...... to Jepsen and Dau (2011) IHL + OHL = HLT [dB], where HLT stands for total hearing loss. Hence having estimates of the total hearing loss and OHC loss, one can estimate the IHL. In the present study, results from forward masking experiments based on temporal masking curves (TMC; Nelson et al., 2001...... estimates of the knee-point level. Further, it is explored whether it is possible to estimate the compression ratio using only on-frequency TMCs. 10 normal-hearing and 10 hearing-impaired listeners (with mild-to-moderate sensorineural hearing loss) were tested at 1, 2 and 4 kHz. The results showed...

  17. Hearing Loss After Radiotherapy for Pediatric Brain Tumors: Effect of Cochlear Dose

    International Nuclear Information System (INIS)

    Hua, Chiaho; Bass, Johnnie K.; Khan, Raja; Kun, Larry E.; Merchant, Thomas E.

    2008-01-01

    Purpose: To determine the effect of cochlear dose on sensorineural hearing loss in pediatric patients with brain tumor treated by using conformal radiation therapy (CRT). Patients and Methods: We studied 78 pediatric patients (155 ears) with localized brain tumors treated in 1997-2001 who had not received platinum-based chemotherapy and were followed up for at least 48 months. They were evaluated prospectively by means of serial pure-tone audiograms (250 Hz-8 kHz) and/or auditory brainstem response before and every 6 months after CRT. Results: Hearing loss occurred in 14% (11 of 78) of patients and 11% (17 of 155) of cochleae, with onset most often at 3-5 years after CRT. The incidence of hearing loss was low for a cochlear mean dose of 30 Gy or less and increased at greater than 40-45 Gy. Risk was greater at high frequencies (6-8 kHz). In children who tested abnormal for hearing, average hearing thresholds increased from a less than 25 decibel (dB) hearing level (HL) at baseline to a mean of 46 ± 13 (SD) dB HL for high frequencies, 41 ± 7 dB HL for low frequencies, and 38 ± 6 dB HL for intermediate frequencies. Conclusions: Sensorineural hearing loss is a late effect of CRT. In the absence of other factors, including ototoxic chemotherapy, increase in cochlear dose correlates positively with hearing loss in pediatric patients with brain tumor. To minimize the risk of hearing loss for children treated with radiation therapy, a cumulative cochlear dose less than 35 Gy is recommended for patients planned to receive 54-59.4 Gy in 30-33 treatment fractions

  18. A case of improved hearing with cochlear implantation in Gaucher disease type 1.

    Science.gov (United States)

    Endo, Shiori; Mizuta, Kunihiro; Yamatodani, Takashi; Nakanishi, Hiroshi; Hosokawa, Kumiko; Misawa, Kiyoshi; Hosokawa, Seiji; Mineta, Hiroyuki

    2018-06-01

    Gaucher disease is a lysosomal storage disorder that is caused by congenital defective function of the enzyme glucocerebrosidase. Glucocerebroside that is not hydrolyzed by glucocerebrosidase mainly accumulates in the reticular tissue. We describe a Japanese boy with Gaucher disease type 1 who developed bilateral profound sensorineural hearing loss within approximately 4years. We performed cochlear implantation initially on his right ear and again on his left ear 5 months later. The cochlear implants were successfully utilized with a speech discrimination score of 95% on a Japanese sentence recognition test. There are many reports of central hearing loss in Gaucher disease type 2 or 3. However, to the best of our knowledge, this is the first report of profound inner ear hearing loss with Gaucher disease. It also appears to be the first record of cochlear implantation for Gaucher disease. Cochlear implants may be useful for sensorineural hearing loss in patients with Gaucher disease without neurological symptoms other than hearing loss. Copyright © 2017 Elsevier B.V. All rights reserved.

  19. 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.

  20. Hearing, speech, language, and vestibular disorders in the fetal alcohol syndrome: a literature review.

    Science.gov (United States)

    Church, M W; Kaltenbach, J A

    1997-05-01

    Fetal alcohol syndrome (FAS) is characterized in part by mental impairment, as well as craniofacial and ocular anomalies. These conditions are traditionally associated with childhood hearing disorders, because they all have a common embryonic origin in malformations of the first and second branchial arches, and have similar critical periods of vulnerability to toxic insult. A review of human and animal research indicates that there are four types of hearing disorders associated with FAS. These are: (1) a developmental delay in auditory maturation, (2) sensorineural hearing loss, (3) intermittent conductive hearing loss due to recurrent serous otitis media, and (4) central hearing loss. The auditory and vestibular systems share the same peripheral apparatuses (the inner ear and eighth cranial nerve) and are embryologically and structurally similar. Consequently, vestibular disorders in FAS children might be expected. The evidence for vestibular dysfunction in FAS is ambiguous, however. Like other syndromes associated with craniofacial anomalies, hearing disorders, and mental impairment, FAS is also characterized by a high prevalence of speech and language pathology. Hearing disorders are a form of sensory deprivation. If present during early childhood, they can result in permanent hearing, language, and mental impairment. Early identification and intervention to treat hearing, language, and speech disorders could therefore result in improved outcome for the FAS child. Specific recommendations are made for intervention and future research.

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

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    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.

  2. Polish universal neonatal hearing screening program-4-year experience (2003-2006).

    Science.gov (United States)

    Szyfter, Witold; Wróbel, Maciej; Radziszewska-Konopka, Marzanna; Szyfter-Harris, Joanna; Karlik, Michał

    2008-12-01

    The aim of this paper is to share our experience and observations in running the Universal Neonatal Hearing Screening Program on a national level, present results and indicate some problems that have arisen during these 4 years. Polish Universal Neonatal Hearing Screening Program started back in 2002 in all neonatal units in Poland. Implemented testing methods consisted of test of transient evoked otoacoustic emission (TEOAE) performed in all new born children in their first 2-3 days of life and auditory brainstem response testing (ABR) conducted on children, who did not meet the TEOAE pass criteria. Additional questionnaire registered information on ototoxic drugs and family history of hearing impairment in every newborn. Diagnosed children were further referred for treatment and rehabilitation. After 4 years of running the program (between 2003 and 2006) a total number of 1,392,427 children were screened for hearing impairment, what stands for 96.3% of all delivered babies, registered in Poland. The screening program enabled to identify and refer for further treatment 2485 children with various types of hearing loss, 312 with profound (0.02% of population) and 145 with severe sensorineural hearing loss (0.11% of population). Our results indicate the accuracy of newborn hearing screening which remain an issue. Although improvement is needed in both intervention systems and diagnostic follow-up of hospitals, the Polish Universal Neonatal Hearing Program fully has achieved the main goal, the identification and treatment of hearing impaired children.

  3. Revalence of Hearing Loss and the Related Factors in BuAli Hospital

    Directory of Open Access Journals (Sweden)

    Shohreh Jalaei

    2004-06-01

    Full Text Available Objective:Determining the prevalence of hearing loss and correlated factors in clients referring to audiology clinic of Buali hospital. Method and Material: This cross-sectional analytic-descriptive survery was carried out on six thousand and twenty ears of 3010 clients (1651 ,a;e amd 1359 female in Audiology clinic of Buali hospital , during Sept 2000 to Sept 2001. Results: Hearing loss is the most common reason for referring the clients (20.5%. 1319 ears (21.9% Showed sensorineural hearing impairment and conductive and mixed hearing loss are observed in 1059 (17.6% and 234 (3.9% ears, respectedly. Hearing loss degree most cases is mild (14% in both ears. There is no significant difference between male and female hearing threshold means (p>0.05 ‘ but a significant difference between hearing thresholds is observed in terms of age (p<0.05. Audiogram configuration in most cases is flat (55.1%. Otoscopic examination reveals abnormal condition in 2333 ears (38.8% . 37.2% of the studied cases have abnormal tympanogram mostly type B (15.9%. 2.2% of the clients wear hearing aid that mostly have B.T.E ones (1.5%. 6.2% of the clients , depends on their hearing impairment type and degree need rehabilitation services. Conclusion: The results are Valid only in the context of this study and it’s generaliztion needs further researches.

  4. Effects of Hearing Loss and Fast-Acting Compression on Amplitude Modulation Perception and Speech Intelligibility

    DEFF Research Database (Denmark)

    Wiinberg, Alan; Jepsen, Morten Løve; Epp, Bastian

    2018-01-01

    Objective: The purpose was to investigate the effects of hearing-loss and fast-acting compression on speech intelligibility and two measures of temporal modulation sensitivity. Design: Twelve adults with normal hearing (NH) and 16 adults with mild to moderately severe sensorineural hearing loss......, the MDD thresholds were higher for the group with hearing loss than for the group with NH. Fast-acting compression increased the modulation detection thresholds, while no effect of compression on the MDD thresholds was observed. The speech reception thresholds obtained in stationary noise were slightly...... of the modulation detection thresholds, compression does not seem to provide a benefit for speech intelligibility. Furthermore, fast-acting compression may not be able to restore MDD thresholds to the values observed for listeners with NH, suggesting that the two measures of amplitude modulation sensitivity...

  5. 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.

  6. Peripheral hearing loss reduces the ability of children to direct selective attention during multi-talker listening.

    Science.gov (United States)

    Holmes, Emma; Kitterick, Padraig T; Summerfield, A Quentin

    2017-07-01

    Restoring normal hearing requires knowledge of how peripheral and central auditory processes are affected by hearing loss. Previous research has focussed primarily on peripheral changes following sensorineural hearing loss, whereas consequences for central auditory processing have received less attention. We examined the ability of hearing-impaired children to direct auditory attention to a voice of interest (based on the talker's spatial location or gender) in the presence of a common form of background noise: the voices of competing talkers (i.e. during multi-talker, or "Cocktail Party" listening). We measured brain activity using electro-encephalography (EEG) when children prepared to direct attention to the spatial location or gender of an upcoming target talker who spoke in a mixture of three talkers. Compared to normally-hearing children, hearing-impaired children showed significantly less evidence of preparatory brain activity when required to direct spatial attention. This finding is consistent with the idea that hearing-impaired children have a reduced ability to prepare spatial attention for an upcoming talker. Moreover, preparatory brain activity was not restored when hearing-impaired children listened with their acoustic hearing aids. An implication of these findings is that steps to improve auditory attention alongside acoustic hearing aids may be required to improve the ability of hearing-impaired children to understand speech in the presence of competing talkers. Copyright © 2017 Elsevier B.V. All rights reserved.

  7. Stapedectomy in tympanosclerosis, hearing results

    International Nuclear Information System (INIS)

    Al-Qahtani, M.; Hagr, A.

    2008-01-01

    The aim of present study was to evaluate the results of using stapedectomy to treat tympanosclerosis fixation of footplate. It is a retrospective review of tympanosclerosis of the oval window with stapes fixation, after undergoing surgery. Eight patients underwent stapedectomy for tympanosclerotic stapes. Postoperative post-op air-bone gap ABG closure to within 10 dB was achieved in 25% of the cases. An ABG of less than 20 db was obtained in 87.5% of the cases. The mean preoperative pre-op ABGs were 31.09 dB. Significant post-op improvement of air conduction thresholds more than, or equal to 20dB was found in 62.5% of cases. No significant sensorineural hearing loss was seen in this series with a mean pre-op bone-conduction threshold BC of 23.43 dB and a mean post-op BC threshold of 16.25 dB. The mean follow-up period was 6.5 months. (author)

  8. 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

  9. Influence of directionality and maximal power output on speech understanding with bone anchored hearing implants in single sided deafness

    OpenAIRE

    Krempaska, Silvia; Koval, Juraj; Schmid, Christoph; Pfiffner, Flurin; Kurz, Anja; Kompis, Martin

    2014-01-01

    Bone-anchored hearing implants (BAHI) are routinely used to alleviate the effects of the acoustic head shadow in single-sided sensorineural deafness (SSD). In this study, the influence of the directional microphone setting and the maximum power output of the BAHI sound processor on speech understanding in noise in a laboratory setting were investigated. Eight adult BAHI users with SSD participated in this pilot study. Speech understanding in noise was measured using a new Slovak speech-in-noi...

  10. Enhancing Auditory Selective Attention Using a Visually Guided Hearing Aid

    Science.gov (United States)

    2017-01-01

    Purpose Listeners with hearing loss, as well as many listeners with clinically normal hearing, often experience great difficulty segregating talkers in a multiple-talker sound field and selectively attending to the desired “target” talker while ignoring the speech from unwanted “masker” talkers and other sources of sound. This listening situation forms the classic “cocktail party problem” described by Cherry (1953) that has received a great deal of study over the past few decades. In this article, a new approach to improving sound source segregation and enhancing auditory selective attention is described. The conceptual design, current implementation, and results obtained to date are reviewed and discussed in this article. Method This approach, embodied in a prototype “visually guided hearing aid” (VGHA) currently used for research, employs acoustic beamforming steered by eye gaze as a means for improving the ability of listeners to segregate and attend to one sound source in the presence of competing sound sources. Results The results from several studies demonstrate that listeners with normal hearing are able to use an attention-based “spatial filter” operating primarily on binaural cues to selectively attend to one source among competing spatially distributed sources. Furthermore, listeners with sensorineural hearing loss generally are less able to use this spatial filter as effectively as are listeners with normal hearing especially in conditions high in “informational masking.” The VGHA enhances auditory spatial attention for speech-on-speech masking and improves signal-to-noise ratio for conditions high in “energetic masking.” Visual steering of the beamformer supports the coordinated actions of vision and audition in selective attention and facilitates following sound source transitions in complex listening situations. Conclusions Both listeners with normal hearing and with sensorineural hearing loss may benefit from the acoustic

  11. 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.

  12. 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

  13. 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.

  14. Conductive hearing loss and middle ear pathology in young infants referred through a newborn universal hearing screening program in Australia.

    Science.gov (United States)

    Aithal, Sreedevi; Aithal, Venkatesh; Kei, Joseph; Driscoll, Carlie

    2012-10-01

    over time with 66.67% of ATSI infants reviewed showing persistent conductive hearing loss compared to 17.86% of non-ATSI infants. Medical management of 17 infants with persistent conductive hearing loss included monitoring, antibiotic treatment, examination under anesthesia, and grommet insertion. Conductive hearing loss was found to be a common diagnosis among infants referred through screening. ATSI infants had significantly higher rates of middle ear pathology and conductive hearing loss at birth and showed poor resolution of middle ear pathology over time compared to non-ATSI infants. Future research using a direct measure of middle ear function as an adjunct to the automated auditory brainstem response screening tool to distinguish conductive from sensorineural hearing loss may facilitate prioritization of infants for assessment, thus reducing parental anxiety and streamlining the management strategies for the respective types of hearing loss. American Academy of Audiology.

  15. Correlative mRNA and protein expression of middle and inner ear inflammatory cytokines during mouse acute otitis media.

    Science.gov (United States)

    Trune, Dennis R; Kempton, Beth; Hausman, Frances A; Larrain, Barbara E; MacArthur, Carol J

    2015-08-01

    Although the inner ear has long been reported to be susceptible to middle ear disease, little is known of the inflammatory mechanisms that might cause permanent sensorineural hearing loss. Recent studies have shown inner ear tissues are capable of expressing inflammatory cytokines during otitis media. However, little quantitative information is available concerning cytokine gene expression in the inner ear and the protein products that result. Therefore, this study was conducted of mouse middle and inner ear during acute otitis media to measure the relationship between inflammatory cytokine genes and their protein products with quantitative RT-PCR and ELISA, respectively. Balb/c mice were inoculated transtympanically with heat-killed Haemophilus influenzae and middle and inner ear tissues collected for either quantitative RT-PCR microarrays or ELISA multiplex arrays. mRNA for several cytokine genes was significantly increased in both the middle and inner ear at 6 h. In the inner ear, these included MIP-2 (448 fold), IL-6 (126 fold), IL-1β (7.8 fold), IL-10 (10.7 fold), TNFα (1.8 fold), and IL-1α (1.5 fold). The 24 h samples showed a similar pattern of gene expression, although generally at lower levels. In parallel, the ELISA showed the related cytokines were present in the inner ear at concentrations higher by 2-122 fold higher at 18 h, declining slightly from there at 24 h. Immunohistochemistry with antibodies to a number of these cytokines demonstrated they occurred in greater amounts in the inner ear tissues. These findings demonstrate considerable inflammatory gene expression and gene products in the inner ear following acute otitis media. These higher cytokine levels suggest one potential mechanism for the permanent hearing loss seen in some cases of acute and chronic otitis media. Copyright © 2015 Elsevier B.V. All rights reserved.

  16. Simulating auditory and visual sensorineural prostheses: a comparative review

    Science.gov (United States)

    Hallum, L. E.; Dagnelie, G.; Suaning, G. J.; Lovell, N. H.

    2007-03-01

    Microelectronic vision prosthesis proposes to render luminous spots (so-called phosphenes) in the visual field of the otherwise blind subject by way of an implanted array of stimulating electrodes, and in doing so restore some spatial vision. There are now many research teams worldwide working towards a therapeutic device, analogous to the cochlear implant, for the profoundly blind. Despite the similarities between the cochlear implant and vision prostheses, there are few instances in the literature where the two approaches are compared and contrasted with a mind to informing the science and engineering of the latter. This is the focus of the present review; specifically, our interest is psychophysics and signal processing. Firstly, we examine the cochlear implant, and review a handful of psychophysical work: the acoustic simulation of cochlear implants and the method used. We focus on the use of normally hearing subjects (played coloured noise bands or sine waves) as a means of investigating cochlear-implant efficacy and speech processing algorithms. These results provide guidance to vision researchers, for they address the interpretation of simulation data, and flag key areas, such as 'artificial' perception in the presence of noise, that require experimental work in coming years. Secondly, we provide an up-to-date review of the body of analogous psychophysical work: the visual simulation, involving normal observers, of microelectronic vision prosthesis. These simulations allow predictions as to the likely clinical efficacy of the prosthesis; indeed, results to date suggest that a number on the order of 100 implanted electrodes will afford subjects mobility and recognition of faces (and other complex stimuli), while even fewer electrodes facilitate reading printed text and very simple visuomanual tasks. Further, the simulations allow investigations of image and signal processing strategies, plus they provide researchers in the field, and other interested persons

  17. Development of a digital hearing aid to meet the Brazilian Government's Ordinance 587 (APAC).

    Science.gov (United States)

    Penteado, Silvio Pires; Bento, Ricardo Ferreira

    2010-01-01

    The treatment of sensorineural hearing loss is based on hearing aids, also known as individual sound amplification devices. The hearing aids purchased by the Brazilian Government, aiming at fulfilling public policies, are based on dedicated components, which bring about benefits, but also render them expensive and may impair repair services after manufacture's warranty expires. to design digital behind-the-ear hearing aids built from standardized components coming from the very supply chain of these manufacturers. experimental. to identify the supply chain of these manufacturers, request samples and set up hearing aids in the laboratory. The developed hearing aids did not show lesser electroacoustic characteristics when compared to those acquired by the Government, also being tested by the same reference international technical standard. It is possible to develop digital behind-the-ear hearing aids based on off-the-shelf components from hearing aid manufacturers' supply chain. Their advantages include low operational costs - for acquisition (with clear advantages for the Government) and service (advantage for the patient).

  18. 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.

  19. 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.

  20. 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.

  1. Measurements on Hearing

    DEFF Research Database (Denmark)

    Poulsen, Torben

    1996-01-01

    Background material for measurements of hearing for grammar school pupils. The note gives the necessary background for the exercise 'Measurement on Hearing'. The topics comprise sound and decibel, the ear, basic psychoacoustics, hearing threshold, audiometric measurement methods, speech and speech...

  2. Hearing Disorders and Deafness

    Science.gov (United States)

    ... enough to enjoy talking with friends or family. Hearing disorders make it hard, but not impossible, to ... often be helped. Deafness can keep you from hearing sound at all. What causes hearing loss? Some ...

  3. Prospective study of the effects of ventilation tubes on hearing after radiotherapy for carcinoma of nasopharynx

    Energy Technology Data Exchange (ETDEWEB)

    Chowdhury, C.R.; Ho, J.H.; Wright, A.; Tsao, S.Y.; Au, G.K.; Tung, Y.

    1988-03-01

    In patients with nasopharyngeal carcinoma, deafness sometimes occurs following radiotherapy. It is usually conductive, but may be sensorineural. Tinnitus is present frequently and usually is distressing. The role of ventilation tubes (grommets) in relieving these problems was assessed in a prospective randomized controlled trial of 115 patients. In the group with grommets, there was an improvement in hearing, with a reduction of the averaged air-bone gap (p less than .01). This was not found in the control group without ventilation tubes, who developed a larger conductive loss (p less than .01) and, in addition, a slight deterioration of the averaged bone conduction threshold (p less than .01). Surprisingly, the group with grommets did not develop this sensorineural loss (p less than .01). In addition, tinnitus was improved significantly by the insertion of ventilation tubes (p less than .01). Neither necrosis nor stenosis of the external auditory meatus was found in either group.

  4. Prospective study of the effects of ventilation tubes on hearing after radiotherapy for carcinoma of nasopharynx

    International Nuclear Information System (INIS)

    Chowdhury, C.R.; Ho, J.H.; Wright, A.; Tsao, S.Y.; Au, G.K.; Tung, Y.

    1988-01-01

    In patients with nasopharyngeal carcinoma, deafness sometimes occurs following radiotherapy. It is usually conductive, but may be sensorineural. Tinnitus is present frequently and usually is distressing. The role of ventilation tubes (grommets) in relieving these problems was assessed in a prospective randomized controlled trial of 115 patients. In the group with grommets, there was an improvement in hearing, with a reduction of the averaged air-bone gap (p less than .01). This was not found in the control group without ventilation tubes, who developed a larger conductive loss (p less than .01) and, in addition, a slight deterioration of the averaged bone conduction threshold (p less than .01). Surprisingly, the group with grommets did not develop this sensorineural loss (p less than .01). In addition, tinnitus was improved significantly by the insertion of ventilation tubes (p less than .01). Neither necrosis nor stenosis of the external auditory meatus was found in either group

  5. Novel association of achalasia with hereditary sensory and motor neuropathy with sensorineural deafness.

    Science.gov (United States)

    Asthana, A K; Lubel, J S; Kohn, G P

    2016-08-01

    Achalasia is a primary esophageal motility disorder. Unlike diffuse esophageal spasm, it has not previously been described in association with hereditary sensory and motor neuropathy (HSMN). An 18-year-old-male with HSMN with sensorineural deafness presented with a 2-day history of dysphagia to solids and liquids. Achalasia was diagnosed after extensive investigations, and his symptoms resolved with endoscopic and definitive surgical management. His monozygotic twin brother had also been diagnosed with HSMN and suffered from chronic dysphagia, which was also subsequently diagnosed with achalasia. This is the first case to illustrate an association between HSMN with sensorineural deafness and achalasia. © 2013 Wiley Periodicals, Inc. and the International Society for Diseases of the Esophagus.

  6. Hearing: Noise-Induced Hearing Loss

    Science.gov (United States)

    ... reduce your exposure to noise by choosing quiet leisure activities rather than noisy ones. Develop the habit ... the degree of your hearing loss, you may benefit from using a hearing aid (a device you ...

  7. Hearing Loss in otitis media with effusion- Types and management- A study of hundred cases

    Directory of Open Access Journals (Sweden)

    Sriram Nathan

    2012-01-01

    Full Text Available Introduction- Serous Ottis media or otitis media with effusion is a common cause of hearing loss of acute onset. The common presentation will be a block or reduced hearing possible after a travel or flight. Most of the cases present with conductive hearing loss while some may present with mixed or even pure sensory hearing loss. Background- we study hundred patients diagnosed with serous otitis media for the type of hearing loss and characterize the management strategy. Conclusion- Serous otitis media is a common cause of hearing loss which is mostly conductive and is amenable to treatment while some rare cases which may persist may require assistive hearing with amplification. Although sensorineral hearing loss is rare in otitis media, it is seen in practice along with mixed hearing loss. The pathophysiology of the neural affection of hearing loss remains a mystery although many theories exist.

  8. Determination of Hearing Loss Prevalence in Preschool Children of Ahwaz

    Directory of Open Access Journals (Sweden)

    Mozafar Sarafraz

    2011-03-01

    Full Text Available Introduction: Children learn to communicate by hearing sounds. If there is hearing loss, the cognitive and speaking abilities and language learning will deteriorate. Early detection and intervention are important factors in the successful treatment of hearing loss in children. Hearing loss (HL is divided into two main groups: conductive hearing loss (CHL and sensorineural hearing loss (SNHL, the prevalence of the former being higher in children, many whose causes are easy to detect and treat. Material and Methods: In this descriptive, cross-sectional study, 785 children, aged 6-7 years, entering elementary school Grade 1 in the school year 2010/2011, were randomly selected from 10% of Ahwaz Hearing Loss Screening Centers, and their audiograms were studied. The collected data were analyzed using SPSS and descriptive statistics. Results: Out of the 785 preschool children examined in this study, 77 children (9.8% suffered from HL (42.9% female and 57.1% male, 59.7% from CHL, and 40.3% from SNHL. Twenty-six percent suffered from bilateral HL and 74% from unilateral HL. Thirty-eight point ninety-six percent had abnormal tympanometry, 61% of whom were Type B. Most of the children (53% had mild HL. Thirty-one point two percent of parents were aware of their children's HL. Conclusion: Considering the high prevalence of HL, especially SNHL, in this study, which is usually permanent but detectable at the neonatal ages, raising public awareness and early screening of ear diseases, which can lead to the detection and treatment in most cases, seem to be vital.

  9. Hearing loss and enlarged internal auditory canal in children.

    Science.gov (United States)

    Santos, Saturnino; Domínguez, M Jesús; Cervera, Javier; Suárez, Alicia; Bueno, Antonio; Bartolomé, Margarita; López, Rafael

    2014-01-01

    Among the temporal bone abnormalities that can be found in the etiological study of paediatric sensorineural hearing loss (SNHL) by imaging techniques, those related to the internal auditory canal (IAC) are the least frequent. The most prevalent of these abnormalities that is associated with SNHL is stenotic IAC due to its association with cochlear nerve deficiencies. Less frequent and less concomitant with SNHL is the finding of an enlarged IAC (>8mm). Retrospective and descriptive review of clinical associations, imaging, audiological patterns and treatment of 9 children with hearing loss and enlarged IAC in the period 1999 to 2012. Two groups of patients are described. The first, without association with vestibulocochlear dysplasias, consisted of: 2 patients with SNHL without other temporal bone or systemic abnormalities, one with bilateral mixed HL from chromosome 18q deletion, one with a genetic X-linked DFN3 hearing loss, one with unilateral hearing loss in neurofibromatosis type 2 with bilateral acoustic neuroma, and one with unilateral hearing loss with cochlear nerve deficiency. The second group, with association with vestibulocochlear dysplasias, was comprised of: one patient with moderate bilateral mixed hearing loss in branchio-oto-renal syndrome, one with profound unilateral SNHL with recurrent meningitis, and another with profound bilateral SNHL with congenital hypothyroidism. The presence of an enlarged IAC in children can be found in different clinical and audiological settings with relevancies that can range from life-threatening situations, such as recurrent meningitis, to isolated hearing loss with no other associations. Copyright © 2013 Elsevier España, S.L. All rights reserved.

  10. The development and standardization of Self-assessment for Hearing Screening of the Elderly

    Directory of Open Access Journals (Sweden)

    Kim G

    2016-06-01

    Full Text Available Gibbeum Kim,1 Wondo Na,1 Gungu Kim,1 Woojae Han,2 Jinsook Kim2 1Department of Speech Pathology and Audiology, Hallym University Graduate School, Chuncheon, Republic of Korea; 2Division of Speech Pathology and Audiology, Research Institute of Audiology and Speech Pathology, College of Natural Sciences, Hallym Universtiy, Chuncheon, Republic of Korea Purpose: The present study aimed to develop and standardize a screening tool for elderly people who wish to check for themselves their level of hearing loss. Methods: The Self-assessment for Hearing Screening of the Elderly (SHSE consisted of 20 questions based on the characteristics of presbycusis using a five-point scale: seven questions covered general issues related to sensorineural hearing loss, seven covered hearing difficulty under distracting listening conditions, two covered hearing difficulty with fast-rated speech, and four covered the working memory function during communication. To standardize SHSE, 83 elderly participants took part in the study: 25 with normal hearing, and 22, 23, and 13 with mild, moderate, and moderate-to-severe sensorineural hearing loss, respectively, according to their hearing sensitivity. All were retested 3 weeks later using the same questionnaire to confirm its reliability. In addition, validity was assessed using various hearing tests such as a sentence test with background noise, a time-compressed speech test, and a digit span test. Results: SHSE and its subcategories showed good internal consistency. SHSE and its subcategories demonstrated high test–retest reliability. A high correlation was observed between the total scores and pure-tone thresholds, which indicated gradually increased SHSE scores of 42.24%, 55.27%, 66.61%, and 78.15% for normal hearing, mild, moderate, and moderate-to-severe groups, respectively. With regard to construct validity, SHSE showed a high negative correlation with speech perception scores in noise and a moderate negative

  11. The relationship of speech intelligibility with hearing sensitivity, cognition, and perceived hearing difficulties varies for different speech perception tests

    Science.gov (United States)

    Heinrich, Antje; Henshaw, Helen; Ferguson, Melanie A.

    2015-01-01

    Listeners vary in their ability to understand speech in noisy environments. Hearing sensitivity, as measured by pure-tone audiometry, can only partly explain these results, and cognition has emerged as another key concept. Although cognition relates to speech perception, the exact nature of the relationship remains to be fully understood. This study investigates how different aspects of cognition, particularly working memory and attention, relate to speech intelligibility for various tests. Perceptual accuracy of speech perception represents just one aspect of functioning in a listening environment. Activity and participation limits imposed by hearing loss, in addition to the demands of a listening environment, are also important and may be better captured by self-report questionnaires. Understanding how speech perception relates to self-reported aspects of listening forms the second focus of the study. Forty-four listeners aged between 50 and 74 years with mild sensorineural hearing loss were tested on speech perception tests differing in complexity from low (phoneme discrimination in quiet), to medium (digit triplet perception in speech-shaped noise) to high (sentence perception in modulated noise); cognitive tests of attention, memory, and non-verbal intelligence quotient; and self-report questionnaires of general health-related and hearing-specific quality of life. Hearing sensitivity and cognition related to intelligibility differently depending on the speech test: neither was important for phoneme discrimination, hearing sensitivity alone was important for digit triplet perception, and hearing and cognition together played a role in sentence perception. Self-reported aspects of auditory functioning were correlated with speech intelligibility to different degrees, with digit triplets in noise showing the richest pattern. The results suggest that intelligibility tests can vary in their auditory and cognitive demands and their sensitivity to the challenges that

  12. Hearing outcomes of the active bone conduction system Bonebridge® in conductive or mixed hearing loss.

    Science.gov (United States)

    Carnevale, Claudio; Til-Pérez, Guillermo; Arancibia-Tagle, Diego J; Tomás-Barberán, Manuel D; Sarría-Echegaray, Pedro L

    2018-05-18

    The active transcutaneous bone conduction implant Bonebridge ® , is indicated for patients affected by bilateral conductive/mixed hearing loss or unilateral sensorineural hearing loss, showing hearing outcomes similar to other percutaneous bone conduction implants, but with a lower rate of complications. The aim of this study was to analyze the hearing outcomes in a series of 26 patients affected by conductive or mixed hearing loss and treated with Bonebridge ® . 26 of 30 patients implanted with Bonebridge ® between October 2012 and May 2017, were included in the study. We compared the air conduction thresholds at the frequencies 500, 1000, 2000, 3000, 4000Hz, the SRT50% and the percentage of correct answers at an intensity of 50dB with and without the implant. "Pure tone average" with the implant was 34.91dB showing an average gain of 33.46dB. Average SRT 50% with the implant was 34.33dB, whereas before the surgery no patient achieved 50% of correct answers at a sound intensity of 50dB. The percentage of correct answers at 50dB changed from 11% without the implant to 85% with it. We only observed one complication consisting of an extrusion of the implant in a patient with a history of 2 previous rhytidectomies. The hearing outcomes obtained in our study are similar to those published in the literature. Bonebridge ® represents an excellent alternative in the treatment of conductive or mixed hearing loss, and with a lower rate of complications. Copyright © 2018 Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. Publicado por Elsevier España, S.L.U. All rights reserved.

  13. Dynamic relation between working memory capacity and speech recognition in noise during the first 6 months of hearing aid use.

    Science.gov (United States)

    Ng, Elaine H N; Classon, Elisabet; Larsby, Birgitta; Arlinger, Stig; Lunner, Thomas; Rudner, Mary; Rönnberg, Jerker

    2014-11-23

    The present study aimed to investigate the changing relationship between aided speech recognition and cognitive function during the first 6 months of hearing aid use. Twenty-seven first-time hearing aid users with symmetrical mild to moderate sensorineural hearing loss were recruited. Aided speech recognition thresholds in noise were obtained in the hearing aid fitting session as well as at 3 and 6 months postfitting. Cognitive abilities were assessed using a reading span test, which is a measure of working memory capacity, and a cognitive test battery. Results showed a significant correlation between reading span and speech reception threshold during the hearing aid fitting session. This relation was significantly weakened over the first 6 months of hearing aid use. Multiple regression analysis showed that reading span was the main predictor of speech recognition thresholds in noise when hearing aids were first fitted, but that the pure-tone average hearing threshold was the main predictor 6 months later. One way of explaining the results is that working memory capacity plays a more important role in speech recognition in noise initially rather than after 6 months of use. We propose that new hearing aid users engage working memory capacity to recognize unfamiliar processed speech signals because the phonological form of these signals cannot be automatically matched to phonological representations in long-term memory. As familiarization proceeds, the mismatch effect is alleviated, and the engagement of working memory capacity is reduced. © The Author(s) 2014.

  14. [Natural history of occupational hearing loss induced by noise].

    Science.gov (United States)

    de Almeida, S I; Albernaz, P L; Zaia, P A; Xavier, O G; Karazawa, E H

    2000-01-01

    To evaluate the clinical and audiometric characteristics of occupational hearing loss induced by noise, according to age and time of exposition in years. 222 patients with occupational sensorineural hearing loss induced by noise were studied retrospectively, correlating the auditive clinical claims, alterations of audiometric thresholds at frequencies of 250 Hz to 8000 Hz, speech discrimination indicator with age and time of exposure. As a control group were used the audiometric threshold of a population of same medium age, without morbid antecedents of hearing illness, as preconized by ISO 1999 (1990). The group were divided into subgroups and three decades of exposure were analyzed. It was verified that the clinical claims of hipoacusia increases according to the age and time of exposure. The frequency of tinnitus is constant. The audiometric thresholds in the second decade of exposure present variations that depend on the age. The several audiometric curves are parallel, but they are not horizontal. The worst thresholds were found in the high frequencies from 3000 Hz to 8000 Hz, as a clinical and physiopathological consequences of the commitment of basal areas of cochlea. The speech discrimination showed to be worst according to the increase of age and time of exposure. Patients with hearing loss disacusia induced by occupational noise present characteristic audiometric thresholds that vary according to age and time of exposure to noise. These characteristics defined and resumed in audiometric curves can constitute a standard of comparison, evaluation and control for exposed populations.

  15. Can Baby Hear?

    Science.gov (United States)

    ... born in the United States are deaf or hard-of-hearing. Research shows that early intervention with hearing devices and educational services can help children with hearing loss to develop language skills at the same rate as their hearing peers. ...

  16. Hard of Hearing?

    DEFF Research Database (Denmark)

    T Christensen, Vibeke

    This summary presents the results of a study of the impact of reduced hearing in relation to labour-market attachment and working life. Reduced hearing contributes to early retirement. Many people with impaired hearing are not aware of the impact of their hearing problems on their working life an...

  17. Targeted surveillance for postnatal hearing loss: a program evaluation.

    Science.gov (United States)

    Beswick, Rachael; Driscoll, Carlie; Kei, Joseph; Glennon, Shirley

    2012-07-01

    The importance of monitoring hearing throughout early childhood cannot be understated. However, there is a lack of evidence available regarding the most effective method of monitoring hearing following the newborn screen. The goal of this study was to describe a targeted surveillance program using a risk factor registry to identify children with a postnatal hearing loss. All children who were born in Queensland, Australia between September 2004 and December 2009, received a bilateral 'pass' on newborn hearing screening, and had at least one risk factor, were referred for targeted surveillance and were included in this study. The cohort was assessed throughout early childhood in accordance with Queensland's diagnostic assessment protocols. During the study period, 7320 (2.8% of 261,328) children were referred for targeted surveillance, of which 56 were identified with a postnatal hearing loss (0.77%). Of these, half (50.0%) were identified with a mild hearing loss, and 64.3% were identified with a sensorineural hearing loss. In regards to risk factors, syndrome, craniofacial anomalies, and severe asphyxia had the highest yield of positive cases of postnatal hearing loss for children referred for targeted surveillance, whereas, low birth weight, bacterial meningitis, and professional concern had a particularly low yield. Limitations of the targeted surveillance program were noted and include: (1) a lost contact rate of 32.4%; (2) delays in first surveillance assessment; (3) a large number of children who required on-going monitoring; and (4) extensive diagnostic assessments were completed on children with normal hearing. Examination of the lost contact rate revealed indigenous children were more likely to be documented as lost contact. In addition, children with one risk factor only were significantly more likely to not attend a surveillance appointment. Positive cases of postnatal hearing loss were detected through the targeted surveillance program. However, the

  18. CERN hearing day

    CERN Multimedia

    2005-01-01

    1 in 10 people suffer from hearing loss - do you? The Medical Service invites everyone working on the CERN site to participate in the NATIONAL HEARING DAY on: Thursday 10th March 2005 From 9am to 4pm The Infirmary, Blg. 57, Ground Floor We will be offering hearing tests (audiograms), as well as information and advice on hearing loss, tinnitus, etc. Deafness does not just affect the elderly: in Europe, 50% of the hearing-impaired are under the age of 55. Exposure to excessive noise is one of the main reasons for hearing problems but PREVENTION IS POSSIBLE. For example, hearing protection devices can prevent 80% of tinnitus cases.

  19. CERN hearing day

    CERN Multimedia

    2005-01-01

    1 in 10 people suffer from hearing loss - do you? The Medical Service invites everyone working on CERN premises to participate in the National Hearing Day on: Thursday 10th March From 9am to 4pm The Infirmary, Blg. 57, Gr.Fl. We will be offering hearing tests (audiogram); information, advice on hearing loss, tinnitus and more. Deafness does not just affect the elderly: in Europe, 50% the hearing-impaired are under the age of 55. Exposure to excessive noise is one of the main reasons for hearing loss. But PREVENTION IS POSSIBLE AND EFFECTIVE: for example, Hearing protection devices could reduce tinnitus cases by 80%.

  20. CERN hearing day

    CERN Document Server

    2005-01-01

    1 in 10 people suffer from hearing loss - do you? The Medical Service invites everyone working on CERN premises to participate in the National Hearing Day on: Thursday 10th March From 9am to 4pm The Infirmary, Blg. 57, Gr.Fl. We will be offering hearing tests (audiogram); information, advice on hearing loss, tinnitus and more. Deafness does not just affect the elderly: in Europe, 50% the hearing-impaired are under the age of 55. Exposure to excessive noise is one of the main reasons for hearing loss. But prevention is possible and effective: for example, Hearing protection devices could reduce tinnitus cases by 80%.

  1. CERN hearing day

    CERN Multimedia

    2005-01-01

    1 in 10 people suffer from hearing loss ? do you? The Medical Service invites everyone working on the CERN site to participate in the NATIONAL HEARING DAY on: Thursday 10th March 2005 From 9am to 4pm The Infirmary, Blg. 57, Ground Floor We will be offering hearing tests (audiograms), as well as information and advice on hearing loss, tinnitus, etc. Deafness does not just affect the elderly: in Europe, 50% of the hearing-impaired are under the age of 55. Exposure to excessive noise is one of the main reasons for hearing problems but prevention is possible. For example, hearing protection devices can prevent 80% of tinnitus cases.

  2. The Master Hearing Aid

    Science.gov (United States)

    Curran, James R.

    2013-01-01

    As early as the 1930s the term Master Hearing Aid (MHA) described a device used in the fitting of hearing aids. In their original form, the MHA was a desktop system that allowed for simulated or actual adjustment of hearing aid components that resulted in a changed hearing aid response. Over the years the MHA saw many embodiments and contributed to a number of rationales for the fitting of hearing aids. During these same years, the MHA was viewed by many as an inappropriate means of demonstrating hearing aids; the audio quality of the desktop systems was often superior to the hearing aids themselves. These opinions and the evolution of the MHA have molded the modern perception of hearing aids and the techniques used in the fitting of hearing aids. This article reports on a history of the MHA and its influence on the fitting of hearing aids. PMID:23686682

  3. Does tinnitus, hearing asymmetry, or hearing loss predispose to occupational injury risk?

    Science.gov (United States)

    Cantley, Linda F; Galusha, Deron; Cullen, Mark R; Dixon-Ernst, Christine; Tessier-Sherman, Baylah; Slade, Martin D; Rabinowitz, Peter M; Neitzel, Richard L

    2015-02-01

    To determine the relative contributions of tinnitus, asymmetrical hearing loss, low frequency hearing loss (pure tone average of 0.5, 1, 2, 3 kHz; PTA.5123), or high frequency hearing loss (pure tone average of 4, 6 kHz; PTA46), to acute injury risk among a cohort of production and maintenance workers at six aluminum manufacturing plants, adjusting for ambient noise exposure and other recognized predictors of injury risk. Retrospective analysis. The study considered 9920 workers employed during 2003 to 2008. The cohort consisted of 8818 workers (89%) whose complete records were available. Adjusting for noise exposure and other recognized injury predictors, a 25% increased acute injury risk was observed among workers with a history of tinnitus in conjunction with high-frequency hearing loss (PTA46). Low frequency hearing loss may be associated with minor, yet less serious, injury risk. We did not find evidence that asymmetry contributes to injury risk. These results provide evidence that tinnitus, combined with high-frequency hearing loss, may pose an important safety threat to workers, especially those who work in high-noise exposed environments. These at risk workers may require careful examination of their communication and hearing protection needs.

  4. Detection threshold for sound distortion resulting from noise reduction in normal-hearing and hearing-impaired listeners.

    Science.gov (United States)

    Brons, Inge; Dreschler, Wouter A; Houben, Rolph

    2014-09-01

    Hearing-aid noise reduction should reduce background noise, but not disturb the target speech. This objective is difficult because noise reduction suffers from a trade-off between the amount of noise removed and signal distortion. It is unknown if this important trade-off differs between normal-hearing (NH) and hearing-impaired (HI) listeners. This study separated the negative effect of noise reduction (distortion) from the positive effect (reduction of noise) to allow the measurement of the detection threshold for noise-reduction (NR) distortion. Twelve NH subjects and 12 subjects with mild to moderate sensorineural hearing loss participated in this study. The detection thresholds for distortion were determined using an adaptive procedure with a three-interval, two-alternative forced-choice paradigm. Different levels of distortion were obtained by changing the maximum amount of noise reduction. Participants were also asked to indicate their preferred NR strength. The detection threshold for overall distortion was higher for HI subjects than for NH subjects, suggesting that stronger noise reduction can be applied for HI listeners without affecting the perceived sound quality. However, the preferred NR strength of HI listeners was closer to their individual detection threshold for distortion than in NH listeners. This implies that HI listeners tolerate fewer audible distortions than NH listeners.

  5. The Effects of Hearing Aid Directional Microphone and Noise Reduction Processing on Listening Effort in Older Adults with Hearing Loss.

    Science.gov (United States)

    Desjardins, Jamie L

    2016-01-01

    Older listeners with hearing loss may exert more cognitive resources to maintain a level of listening performance similar to that of younger listeners with normal hearing. Unfortunately, this increase in cognitive load, which is often conceptualized as increased listening effort, may come at the cost of cognitive processing resources that might otherwise be available for other tasks. The purpose of this study was to evaluate the independent and combined effects of a hearing aid directional microphone and a noise reduction (NR) algorithm on reducing the listening effort older listeners with hearing loss expend on a speech-in-noise task. Participants were fitted with study worn commercially available behind-the-ear hearing aids. Listening effort on a sentence recognition in noise task was measured using an objective auditory-visual dual-task paradigm. The primary task required participants to repeat sentences presented in quiet and in a four-talker babble. The secondary task was a digital visual pursuit rotor-tracking test, for which participants were instructed to use a computer mouse to track a moving target around an ellipse that was displayed on a computer screen. Each of the two tasks was presented separately and concurrently at a fixed overall speech recognition performance level of 50% correct with and without the directional microphone and/or the NR algorithm activated in the hearing aids. In addition, participants reported how effortful it was to listen to the sentences in quiet and in background noise in the different hearing aid listening conditions. Fifteen older listeners with mild sloping to severe sensorineural hearing loss participated in this study. Listening effort in background noise was significantly reduced with the directional microphones activated in the hearing aids. However, there was no significant change in listening effort with the hearing aid NR algorithm compared to no noise processing. Correlation analysis between objective and self

  6. Noise-induced and age-related hearing loss:  new perspectives and potential therapies [version 1; referees: 4 approved

    Directory of Open Access Journals (Sweden)

    M Charles Liberman

    2017-06-01

    Full Text Available The classic view of sensorineural hearing loss has been that the primary damage targets are hair cells and that auditory nerve loss is typically secondary to hair cell degeneration. Recent work has challenged that view. In noise-induced hearing loss, exposures causing only reversible threshold shifts (and no hair cell loss nevertheless cause permanent loss of >50% of the synaptic connections between hair cells and the auditory nerve. Similarly, in age-related hearing loss, degeneration of cochlear synapses precedes both hair cell loss and threshold elevation. This primary neural degeneration has remained a “hidden hearing loss” for two reasons: 1 the neuronal cell bodies survive for years despite loss of synaptic connection with hair cells, and 2 the degeneration is selective for auditory nerve fibers with high thresholds. Although not required for threshold detection when quiet, these high-threshold fibers are critical for hearing in noisy environments. Research suggests that primary neural degeneration is an important contributor to the perceptual handicap in sensorineural hearing loss, and it may be key to the generation of tinnitus and other associated perceptual anomalies. In cases where the hair cells survive, neurotrophin therapies can elicit neurite outgrowth from surviving auditory neurons and re-establishment of their peripheral synapses; thus, treatments may be on the horizon.

  7. Systemic Polyarteritis Nodosa as the Cause of Sudden Onset Bilateral Sensorineural Hearing Loss Following Lassa Virus Infection

    Science.gov (United States)

    2016-07-05

    weakness; 5) mono- or polyneuropathy; 6) hypertension ; 7) elevated BUN or creatinine; 8) antibodies to hepatitis B surface antigen in serum; 9...day 6, with those of the two survivors peaking 14 days after infection followed by virus clearance by day 28 (Fig. 1C). Blood chemistry and...Surg 109, 559 (1993); published online EpubSep ( 14. J. Churg, A. Churg, Idiopathic and secondary vasculitis: a review. Mod Pathol 2, 144-160 (1989

  8. Comparative assessment of amphibious hearing in pinnipeds.

    Science.gov (United States)

    Reichmuth, Colleen; Holt, Marla M; Mulsow, Jason; Sills, Jillian M; Southall, Brandon L

    2013-06-01

    Auditory sensitivity in pinnipeds is influenced by the need to balance efficient sound detection in two vastly different physical environments. Previous comparisons between aerial and underwater hearing capabilities have considered media-dependent differences relative to auditory anatomy, acoustic communication, ecology, and amphibious life history. New data for several species, including recently published audiograms and previously unreported measurements obtained in quiet conditions, necessitate a re-evaluation of amphibious hearing in pinnipeds. Several findings related to underwater hearing are consistent with earlier assessments, including an expanded frequency range of best hearing in true seals that spans at least six octaves. The most notable new results indicate markedly better aerial sensitivity in two seals (Phoca vitulina and Mirounga angustirostris) and one sea lion (Zalophus californianus), likely attributable to improved ambient noise control in test enclosures. An updated comparative analysis alters conventional views and demonstrates that these amphibious pinnipeds have not necessarily sacrificed aerial hearing capabilities in favor of enhanced underwater sound reception. Despite possessing underwater hearing that is nearly as sensitive as fully aquatic cetaceans and sirenians, many seals and sea lions have retained acute aerial hearing capabilities rivaling those of terrestrial carnivores.

  9. Could Neonatal Hypernatremia Dehydration Influence Hearing Status?

    Directory of Open Access Journals (Sweden)

    Hassan Boskabadi

    2014-01-01

    Full Text Available Introduction: Neonatal hypernatremia dehydration (NHD is a dangerous condition in neonates, which is accompanied by acute complications (renal failure, cerebral edema, and cerebral hemorrhage and chronic complications (developmental delay. Children begin learning language from birth, and hearing impairment interferes with this process. We assessed the hearing status of infants with hypernatremia dehydration.   Materials and Methods: In a case-control study in 110 infants presenting at the Ghaem Hospital (Mashhad, Iran between 2007 and 2011, we examined the incidence of hearing impairment in infants suffering from hypernatremia dehydration (serum sodium >150 mEq/L in comparison with infants with normal sodium level (serum sodium ≤150 mEq/L.   Results: Three of 110 cases examined in the study group showed a transient hearing impairment. A mean serum sodium level of 173mg/dl was reported among hearing-impaired infants.   Conclusion:  Transient hearing impairment was higher in infants with hypernatremia; although this difference was not significant (P>0.05. Hearing impairment was observed in cases of severe hypernatremia.  

  10. Measurement of conductive hearing loss in mice.

    Science.gov (United States)

    Qin, Zhaobing; Wood, Melissa; Rosowski, John J

    2010-05-01

    In order to discriminate conductive hearing loss from sensorineural impairment, quantitative measurements were used to evaluate the effect of artificial conductive pathology on distortion-product otoacoustic emissions (DPOAEs), auditory brainstem responses (ABRs) and laser-Doppler vibrometry (LDV) in mice. The conductive manipulations were created by perforating the pars flaccida of the tympanic membrane, filling or partially filling the middle-ear cavity with saline, fixing the ossicular chain, and interrupting the incudo-stapedial joint. In the saline-filled and ossicular-fixation groups, averaged DPOAE thresholds increased relative to the control state by 20-36 and 25-39 dB, respectively with the largest threshold shifts occurring at frequencies less than 20kHz, while averaged ABR thresholds increased 12-19 and 12-25 dB, respectively without the predominant low-frequency effect. Both DPOAE and ABR thresholds were elevated by less than 10 dB in the half-filled saline condition; no significant change was observed after pars flaccida perforation. Conductive pathology generally produced a change in DPOAE threshold in dB that was 1.5-2.5 times larger than the ABR threshold change at frequencies less than 30 kHz; the changes in the two thresholds were nearly equal at the highest frequencies. While mild conductive pathology (ABR threshold shifts of conductive hearing losses (ABR threshold shifts >10 dB) were associated with significant deceases in DPOAE growth rate. Our LDV measurements are consistent with others and suggest that measurements of umbo velocity are not an accurate indicator of conductive hearing loss produced by ossicular lesions in mice. Copyright (c) 2009 Elsevier B.V. All rights reserved.

  11. Sound localization under perturbed binaural hearing.

    NARCIS (Netherlands)

    Wanrooij, M.M. van; Opstal, A.J. van

    2007-01-01

    This paper reports on the acute effects of a monaural plug on directional hearing in the horizontal (azimuth) and vertical (elevation) planes of human listeners. Sound localization behavior was tested with rapid head-orienting responses toward brief high-pass filtered (>3 kHz; HP) and broadband

  12. Quality of life in individuals with tinnitus with and without hearing loss

    Directory of Open Access Journals (Sweden)

    Gregorina Silva Ribeiro Rocha

    Full Text Available ABSTRACT Purpose: to evaluate the perception of the level of quality of life in subjects with tinnitus, with and without hearing loss. Methods: a cross-sectional quantitative study. A total of 189 subjects (mean age 53.06 years divided into four groups. Group 1: subjects with normal auditory thresholds without tinnitus complaint; Group 2: subjects with normal auditory thresholds and tinnitus complaint; Group 3: subjects with sensorineural hearing loss without tinnitus complaint; Group 4: subjects with sensorineural hearing loss and with tinnitus complaint. Levels of quality of life were investigated through the World Health Organization Quality Of Life (WHOQOL website - brief and psycho-emotional and functional aspects of patients with tinnitus through the Tinnitus Handicap Inventory (THI. Statistical analyzes, comparisons among groups and descriptive analysis were performed, considering a significance level of 5%. Results: the overall mean scores of quality of life in group 4 (56.07 were smaller than those of group 1 (64.67 (p<0.05. The subjects with tinnitus complaint presented a moderate level of disturbance of the symptom. Conclusion: tinnitus interferes in the quality of life of individuals who had preserved or altered auditory thresholds. Therefore, means to reduce the discomfort caused by tinnitus symptom should be developed, in order to improve patients' quality of life.

  13. Prevalence of congenital hereditary sensorineural deafness in Australian Cattle Dogs and associations with coat characteristics and sex

    Directory of Open Access Journals (Sweden)

    Sommerlad Susan F

    2012-10-01

    Full Text Available Abstract Background Congenital hereditary sensorineural deafness (CHSD occurs in many dog breeds, including Australian Cattle Dogs. In some breeds, CHSD is associated with a lack of cochlear melanocytes in the stria vascularis, certain coat characteristics, and potentially, abnormalities in neuroepithelial pigment production. This study investigates phenotypic markers for CHSD in 899 Australian Cattle Dogs. Results Auditory function was tested in 899 Australian Cattle Dogs in family groups using brainstem auditory evoked response testing. Coat colour and patterns, facial and body markings, gender and parental hearing status were recorded. Deafness prevalence among all 899 dogs was 10.8% with 7.5% unilaterally deaf, and 3.3% bilaterally deaf, and amongst pups from completely tested litters (n = 696 was 11.1%, with 7.5% unilaterally deaf, and 3.6% bilaterally deaf. Univariable and multivariable analyses revealed a negative association between deafness and bilateral facial masks (odds ratio 0.2; P ≤ 0.001. Using multivariable logistic animal modelling, the risk of deafness was lower in dogs with pigmented body spots (odds ratio 0.4; P = 0.050. No significant associations were found between deafness and coat colour. Within unilaterally deaf dogs with unilateral facial masks, no association was observed between the side of deafness and side of mask. The side of unilateral deafness was not significantly clustered amongst unilaterally deaf dogs from the same litter. Females were at increased risk of deafness (odds ratio from a logistic animal model 1.9; P = 0.034 after adjusting for any confounding by mask type and pigmented body spots. Conclusions Australian Cattle Dogs suffer from CHSD, and this disease is more common in dogs with mask-free faces, and in those without pigmented body patches. In unilaterally deaf dogs with unilateral masks, the lack of observed association between side of deafness and side of mask suggests that if CHSD is due to

  14. The role of bone conduction hearing aids in congenital unilateral hearing loss: A systematic review.

    Science.gov (United States)

    Liu, C Carrie; Livingstone, Devon; Yunker, Warren K

    2017-03-01

    To systematically review the literature on the audiological and/or quality of life benefits of a bone conduction hearing aid (BCHA) in children with congenital unilateral conductive or sensorineural deafness. A systematic search was performed according to the PRISMA guidelines using the PubMed, Medline, and Embase databases. Data were collected on the following outcomes of interest: speech reception threshold, speech discrimination, sound localization, and quality of life measures. Given the heterogeneity of the data for quantitative analysis, the results are qualitatively summarized. Eight studies were included in the review. Four studies examined the audiological outcomes associated with bone conduction hearing aid implantation. There was a consistent gain in speech reception thresholds and speech discrimination, especially in noisy environments. Results pertaining to sound localization was inconsistent. The studies that examined quality of life measures reported a high usage rate of BCHAs among children. Quality of life improvements are reported with suggested benefit in the subdomain of learning. Given the potential benefits of a BCHA, along with the fact that it can be safely trialed using a headband, it is reasonable to trial a BCHA in children with congenital unilateral deafness. Should the trial offer audiological and/or quality of life benefits for the individual child, then BCHA implantation can be considered. Copyright © 2017 Elsevier B.V. All rights reserved.

  15. Negotiating hearing disability and hearing disabled identities

    DEFF Research Database (Denmark)

    Lykke Hindhede, Anette

    2012-01-01

        Using disability theory as a framework and social science theories of identity to strengthen the arguments, this paper explores empirically how working-age adults confront the medical diagnosis of hearing impairment. For most participants hearing impairment threatens the stability of social...... interaction and the construction of hearing disabled identities is seen as shaped in the interaction with the hearing impaired person‟s surroundings. In order to overcome the potential stigmatisation the „passing‟ as normal becomes predominant. For many the diagnosis provokes radical redefinitions of the self....... The discursively produced categorisation and subjectivity of senescence mean that rehabilitation technologies such as hearing aids identify a particular life-style (disabled) which determines their social significance. Thus wearing a hearing aid works against the contemporary attempt to create socially ideal...

  16. Evaluating the feasibility of integrating salivary testing for congenital CMV into the Newborn Hearing Screening Programme in the UK.

    Science.gov (United States)

    Kadambari, Seilesh; Luck, Suzanne; Davis, Adrian; Walter, Simone; Agrup, Charlotte; Atkinson, Claire; Stimson, Laura; Williams, Eleri; Berrington, Janet; Griffiths, Paul; Sharland, Mike

    2015-08-01

    Congenital cytomegalovirus (cCMV) accounts for 20% of all childhood sensorineural hearing loss (SNHL) but is not routinely tested for at birth. Valganciclovir has been shown to prevent hearing deterioration and improve neurocognitive outcomes if started in the first month of life. This study aimed to assess the feasibility of integrating testing for cCMV using salivary swabs into the Newborn Hearing Screening Programme (NHSP). Parents of newborns newborn hearing screen for further audiological testing, were approached by hearing screeners to obtain a saliva sample for CMV DNA polymerase chain reaction (PCR). Eighty percent (203/255) of newborns who were eligible had a saliva swab taken by the hearing screener. Over 99% of results were delivered within the first month of life. Two newborns were identified with cCMV and both seen on day 10 of life by the paediatric specialist. All saliva samples tested delivered a result using real-time PCR. It is feasible for hearing screeners to obtain saliva swabs to test for CMV DNA using real-time PCR in newborns referred after their initial hearing screen. Rapid diagnostic testing for cCMV needs a more detailed clinical and cost-effectiveness analysis.

  17. Hearing Aid and children

    Directory of Open Access Journals (Sweden)

    Jamileh Fatahi

    2002-07-01

    Full Text Available In order to develop oral communication, hearing impaired infants and young children must be able to hear speech comfortably and consistently. To day children with all degrees of hearing loss may be condidates for some kinds of amlification. As children differ from adults, many Factors should be consider in hearing aid selection, evaluation and fitting. For example the child age when he or she is candidate for custom instruments? Do we consider programmable Hearing aid? Are multi memory instruments appropriate for them? What about directional microphones? What style of hearing aid do we select? In this paper such questions are Answered.

  18. Congenital hearing impairment

    Energy Technology Data Exchange (ETDEWEB)

    Robson, Caroline D. [Children' s Hospital and Harvard Medical School, Division of Neuroradiology, Department of Radiology, Boston, MA (United States)

    2006-04-15

    Establishing the etiology of congenital hearing impairment can significantly improve treatment for certain causes of hearing loss and facilitates genetic counseling. High-resolution CT and MRI have contributed to the evaluation and management of hearing impairment. In addition, with the identification of innumerable genetic loci and genetic defects involved in hearing loss, genetic testing has emerged as an invaluable tool in the assessment of hearing impairment. Some of the common forms of congenital hearing loss are reviewed and their imaging features illustrated. (orig.)

  19. Congenital hearing impairment

    International Nuclear Information System (INIS)

    Robson, Caroline D.

    2006-01-01

    Establishing the etiology of congenital hearing impairment can significantly improve treatment for certain causes of hearing loss and facilitates genetic counseling. High-resolution CT and MRI have contributed to the evaluation and management of hearing impairment. In addition, with the identification of innumerable genetic loci and genetic defects involved in hearing loss, genetic testing has emerged as an invaluable tool in the assessment of hearing impairment. Some of the common forms of congenital hearing loss are reviewed and their imaging features illustrated. (orig.)

  20. GJB2 (Connexin-26) mutations are not frequent among hearing impaired patients in East Greenland

    DEFF Research Database (Denmark)

    Homoe, P.; Koch, A.; Rendtorff, Nanna Dahl

    2012-01-01

    Objective: Investigate genetic causes of HI among the Inuit populations in the Arctic with a high prevalence of hearing impairment (HI). Design: A cross-sectional survey with population-based controls. Study sample: Forty-five patients, with sensorineural or mixed HI and an available blood sample...... for GJB2 sequencing from DNA, were selected from 166 East Greenlanders by specialist audiology examination, including pure-tone air and bone conduction audiometry from 125 Hz to 8000 Hz. Controls were 108 East- and 109 West-Greenlanders. Results: Forty-five patients with HI were included, 24 males and 21...

  1. Estrogen and hearing from a clinical point of view; characteristics of auditory function in women with Turner syndrome.

    Science.gov (United States)

    Hederstierna, Christina; Hultcrantz, Malou; Rosenhall, Ulf

    2009-06-01

    Turner syndrome is a chromosomal aberration affecting 1:2000 newborn girls, in which all or part of one X chromosome is absent. This leads to ovarial dysgenesis and little or no endogenous estrogen production. These women have, among many other syndromal features, a high occurrence of ear and hearing problems, and neurocognitive dysfunctions, including reduced visual-spatial abilities; it is assumed that estrogen deficiency is at least partially responsible for these problems. In this, study 30 Turner women aged 40-67, with mild to moderate hearing loss, performed a battery of hearing tests aimed at localizing the lesion causing the sensorineural hearing impairment and assessing central auditory function, primarily sound localization. The results of TEOAE, ABR and speech recognition scores in noise were all indicative of cochlear dysfunction as the cause of the sensorineural impairment. Phase audiometry, a test for sound localization, showed mild disturbances in the Turner women compared to the reference group, suggesting that auditory-spatial dysfunction is another facet of the recognized neurocognitive phenotype in Turner women.

  2. Prescriptive amplification recommendations for hearing losses with a conductive component and their impact on the required maximum power output: an update with accompanying clinical explanation.

    Science.gov (United States)

    Johnson, Earl E

    2013-06-01

    Hearing aid prescriptive recommendations for hearing losses having a conductive component have received less clinical and research interest than for losses of a sensorineural nature; as a result, much variation remains among current prescriptive methods in their recommendations for conductive and mixed hearing losses (Johnson and Dillon, 2011). The primary intent of this brief clinical note is to demonstrate differences between two algebraically equivalent expressions of hearing loss, which have been approaches used historically to generate a prescription for hearing losses with a conductive component. When air and bone conduction thresholds are entered into hearing aid prescriptions designed for nonlinear hearing aids, it was hypothesized that that two expressions would not yield equivalent amounts of prescribed insertion gain and output. These differences are examined for their impact on the maximum power output (MPO) requirements of the hearing aid. Subsequently, the MPO capabilities of two common behind-the-ear (BTE) receiver placement alternatives, receiver-in-aid (RIA) and receiver-in-canal (RIC), are examined. The two expressions of hearing losses examined were the 25% ABG + AC approach and the 75% ABG + BC approach, where ABG refers to air-bone gap, AC refers to air-conduction threshold, and BC refers to bone-conduction threshold. Example hearing loss cases with a conductive component are sampled for calculations. The MPO capabilities of the BTE receiver placements in commercially-available products were obtained from hearing aids on the U.S. federal purchasing contract. Prescribed gain and the required MPO differs markedly between the two approaches. The 75% ABG + BC approach prescribes a compression ratio that is reflective of the amount of sensorineural hearing loss. Not all hearing aids will have the MPO capabilities to support the output requirements for fitting hearing losses with a large conductive component particularly when combined with

  3. JS-X syndrome: A multiple congenital malformation with vocal cord paralysis, ear deformity, hearing loss, shoulder musculature underdevelopment, and X-linked recessive inheritance.

    Science.gov (United States)

    Hoeve, Hans L J; Brooks, Alice S; Smit, Liesbeth S

    2015-07-01

    We report on a family with a not earlier described multiple congenital malformation. Several male family members suffer from laryngeal obstruction caused by bilateral vocal cord paralysis, outer and middle ear deformity with conductive and sensorineural hearing loss, facial dysmorphisms, and underdeveloped shoulder musculature. The affected female members only have middle ear deformity and hearing loss. The pedigree is suggestive of an X-linked recessive inheritance pattern. SNP-array revealed a deletion and duplication on Xq28 in the affected family members. A possible aetiology is a neurocristopathy with most symptoms expressed in structures derived from branchial arches. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  4. Mutations in a Novel Isoform of TRIOBP That Encodes a Filamentous-Actin Binding Protein Are Responsible for DFNB28 Recessive Nonsyndromic Hearing Loss

    OpenAIRE

    Shahin, Hashem; Walsh, Tom; Sobe, Tama; Abu Sa’ed, Judeh; Abu Rayan, Amal; Lynch, Eric D.; Lee, Ming K.; Avraham, Karen B.; King, Mary-Claire; Kanaan, Moein

    2005-01-01

    In a large consanguineous Palestinian kindred, we previously mapped DFNB28—a locus associated with recessively inherited, prelingual, profound sensorineural hearing impairment—to chromosome 22q13.1. We report here that mutations in a novel 218-kDa isoform of TRIOBP (TRIO and filamentous actin [F-actin] binding protein) are associated with DFNB28 hearing loss in a total of nine Palestinian families. Two nonsense mutations (R347X and Q581X) truncate the protein, and a potentially deleterious mi...

  5. Prevalence of hearing impairment in elderly patients referred to the audiology service in Manaus, Amazon

    Directory of Open Access Journals (Sweden)

    Karla Geovanna Moraes Crispim

    2012-12-01

    Full Text Available Objective: To describe the audiological findings (type, audiometric configuration, degree of hearing loss and its association with gender and age in subjects aged over 60 years, treated at outpatient clinic of specialties of Manaus. Methods: A cross-sectional epidemiological descriptive study conducted with the universe of elderly who underwent audiometry from January to December 2010, with a total of 574 subjects. To classify the type, degree and configuration of hearing loss, we used the criteria adopted by Santos & Russo, Davis & Silverman and Silman & Silverman, respectively. Statistical analysis was performed using measures of central tendency, dispersion and frequency distributions. To check for statistically significant differences, we used the chi-square test, with a significance level of 5% (p ≤ 0.05. Results: The prevalence of 94.4% (n = 542 of hearing loss, sensorineural predominantly (85.5%, n = 491 and mild (60%, n = 188 in females and degree moderate or greater in males (50%, n = 130, downward sloping (54.2%, n = 311 in both sexes. The percentage of normality was low, with 261 (3.4% for men and 313 (7.3% for women. Conclusion: The prevalence of hearing loss increased with age, being equal to 100% in individuals 80 years or older, and men have the worst hearing thresholds. There is need for greater awareness about hearing loss in the elderly, needing additional population-based and multicentric studies in order to support public policies.

  6. [Hearing capacity and speech production in 417 children with facial cleft abnormalities].

    Science.gov (United States)

    Schönweiler, R; Schönweiler, B; Schmelzeisen, R

    1994-11-01

    Children with cleft palates often suffer from chronic conductive hearing losses, delayed language acquisition and speech disorders. This study presents results of speech and language outcomes in relation to hearing function and types of palatal malformations found. 417 children with cleft palates were examined during followup evaluations that extended over several years. Disorders were studied as they affected the ears, nose and throat, audiometry and speech and language pathology. Children with isolated cleft lips were excluded. Among the total group, 8% had normal speech and language development while 92% had speech or language disorders. 80% of these latter children had hearing problems that predominantly consisted of fluctuating conductive hearing losses caused by otitis media with effusion. 5% had sensorineural hearing losses. Fifty-eight children (14%) with rhinolalia aperta were not improved by speech therapy and required velopharyngoplasties, using a cranial-based pharyngeal flap. Language skills did not depend on the type of cleft palate presents but on the frequency and amount of hearing loss found. Otomicroscopy and audiometric follow-ups with insertions of ventilation tubes were considered to be most important for language development in those children with repeated middle ear infections. Speech or language therapy was necessary in 49% of the children.

  7. A Prevalence Study of Hearing Loss among Primary School Children in the South East of Iran

    Directory of Open Access Journals (Sweden)

    Aqeel Absalan

    2013-01-01

    Full Text Available Hearing impairment substantially affects child’s ability to normally acquire the spoken language. Such negative effects create problems for the child not only in terms of communication but also in terms of achievement in school as well as social and emotional growth. The aim of this research is to study the prevalence of hearing disorders and its relationship to age and gender among primary school students of Zahedan, Iran. In this cross-sectional and descriptive analytical study, 1500 students from elementary schools were screened for hearing loss. The selection of samples was performed using multistage sampling method. Primary information was obtained through direct observation, otoscopy, and audiometric and tympanometric screenings. Data was obtained and analyzed via ANOVA test. Statistical analysis showed a significant correlation between the age and the prevalence of middle ear abnormal function. Conductive hearing loss in males and females was 8.8% and 7.1%, respectively. In addition, 1% and 0.7% of male and female students, respectively, suffered from sensorineural hearing loss. Results indicated that 20.2% of students of elementary schools in Zahedan needed medical treatment for their problems. Therefore, it is recommended that the hearing screening of school-age children should be included in annual school health programs in this region.

  8. Comparison of muzzle suppression and ear-level hearing protection in firearm use.

    Science.gov (United States)

    Branch, Matthew Parker

    2011-06-01

    To compare noise reduction of commercially available ear-level hearing protection (muffs/inserts) to that of firearm muzzle suppressors. Experimental sound measurements under consistent environmental conditions. None. Muzzle suppressors for 2 pistol and 2 rifle calibers were tested using the Bruel & Kjaer 2209 sound meter and Bruel & Kjaer 4136 microphone calibrated with the Bruel & Kjaer Pistonphone using Military-Standard 1474D placement protocol. Five shots were recorded unsuppressed and 10 shots suppressed under consistent environmental conditions. Sound reduction was then compared with the real-world noise reduction rate of the best available ear-level protectors. All suppressors offered significantly greater noise reduction than ear-level protection, usually greater than 50% better. Noise reduction of all ear-level protectors is unable to reduce the impulse pressure below 140 dB for certain common firearms, an international standard for prevention of sensorineural hearing loss. Modern muzzle-level suppression is vastly superior to ear-level protection and the only available form of suppression capable of making certain sporting arms safe for hearing. The inadequacy of standard hearing protectors with certain common firearms is not recognized by most hearing professionals or their patients and should affect the way hearing professionals counsel patients and the public.

  9. Interactions between amplitude modulation and frequency modulation processing: Effects of age and hearing loss.

    Science.gov (United States)

    Paraouty, Nihaad; Ewert, Stephan D; Wallaert, Nicolas; Lorenzi, Christian

    2016-07-01

    Frequency modulation (FM) and amplitude modulation (AM) detection thresholds were measured for a 500-Hz carrier frequency and a 5-Hz modulation rate. For AM detection, FM at the same rate as the AM was superimposed with varying FM depth. For FM detection, AM at the same rate was superimposed with varying AM depth. The target stimuli always contained both amplitude and frequency modulations, while the standard stimuli only contained the interfering modulation. Young and older normal-hearing listeners, as well as older listeners with mild-to-moderate sensorineural hearing loss were tested. For all groups, AM and FM detection thresholds were degraded in the presence of the interfering modulation. AM detection with and without interfering FM was hardly affected by either age or hearing loss. While aging had an overall detrimental effect on FM detection with and without interfering AM, there was a trend that hearing loss further impaired FM detection in the presence of AM. Several models using optimal combination of temporal-envelope cues at the outputs of off-frequency filters were tested. The interfering effects could only be predicted for hearing-impaired listeners. This indirectly supports the idea that, in addition to envelope cues resulting from FM-to-AM conversion, normal-hearing listeners use temporal fine-structure cues for FM detection.

  10. Radiotherapy-induced hearing loss in patients with laryngeal and hypopharyngeal carcinomas.

    Science.gov (United States)

    Gudelj, Goran; Trotić, Robert; Herceg, Tonko; Parazajder, Domagoj; Vagić, Davor; Geber, Goran

    2014-09-01

    The purpose of this study was to investigate a hypothesized correlation of development of a sensorineural hearing loss and radiotherapy in patients with laryngeal and hypopharyngeal carcinoma. This prospective study included a total of 50 patients, which after strict exclusion critera (audiologic problems before RT primary tumors of the auditory system, spread of the primary tumor to any part of the auditory system) resulted in 23 analyzed patients, ranging between 50 and 76 years of age, with a mean age of 60. Audiometry measuring frequency-specific thresholds was performed in three time points: one month before radiotherapy, one and six months after radiotherapy. A significant statistical difference in hearing tresholds after radiotherapy was found in 6 out of 23 patients. An obvious tendency towards hearing loss without statistical significance at 250 and 4000 Hz was found for a whole tested population (p < or = 0.3 with Bonferroni correction). Observed tendency towards hearing loss after radiotherapy of laryngeal carcinoma was related to side of the tumor and less severe when chemotherapy was not added as adjuvant therapy. These results should help to decrease a rate of hearing loss by careful planing of ear protection, by using observed frequencies as relevant markers of hearing loss and by reconsidering adjuvant chemoterapy during radiotherapy of laryngeal carcinoma.

  11. Auditory brainstem response screening for hearing loss in high risk neonates.

    Science.gov (United States)

    Watson, D R; McClelland, R J; Adams, D A

    1996-07-01

    The present paper reports the findings of a 7 year study evaluating the use of the auditory brainstem response (ABR) as the basis of a hearing screening procedure in a group of newborns at increased risk of hearing impairment. A Special Care Baby Unit (SCBU) population of 417 infants with diverse clinical backgrounds and treatment histories was tested for hearing impairment at birth using ABR audiometry. Some 332 passed the original screen at 30 dBnHL test level in both ears. Of the failure group, 18 did not survive and 32 had some degree of hearing impairment confirmed, nine of which were sensorineural in origin. An increased incidence of persistent middle ear disease was also noted in the failure group. A detailed operational analysis demonstrates that provided appropriate pass/fail criteria are adopted, the ABR technique offers excellent sensitivity and specificity for the detection of significant hearing loss in the test population. Furthermore, the study establishes that implementation of an ABR-based screening programme could reduce the average age at detection of permanent hearing loss by 7 months. A cost assessment shows that the introduction of such a targetted screening procedure could be done at a reasonable outlay.

  12. Three novel GJB2 (connexin 26) variants associated with autosomal dominant syndromic and nonsyndromic hearing loss.

    Science.gov (United States)

    DeMille, Desiree; Carlston, Colleen M; Tam, Oliver H; Palumbos, Janice C; Stalker, Heather J; Mao, Rong; Zori, Roberto T; Viskochil, David H; Park, Albert H; Carey, John C

    2018-04-01

    Connexin 26 (Cx26), encoded by the GJB2 gene, is a key protein involved in the formation of gap junctions in epithelial organs including the inner ear and palmoplantar epidermis. Pathogenic variants in GJB2 are responsible for approximately 50% of inherited sensorineural deafness. The majority of these variants are associated with autosomal recessive inheritance; however, rare reports of dominantly co-segregating variants have been published. Since we began offering GJB2 testing in 2003, only about 2% of detected GJB2 variants from our laboratory have been classified as dominant. Here we report three novel dominant GJB2 variants (p.Thr55Ala, p.Gln57_Pro58delinsHisSer, and p.Trp44Gly); two associated with syndromic sensorineural hearing loss and one with nonsyndromic hearing loss. In the kindred with the p.Thr55Ala variant, the proband and his father present with only leukonychia as a cutaneous finding of their syndromic hearing loss. This phenotype has been previously documented in conjunction with palmoplantar hyperkeratosis, but isolated leukonychia is a novel finding likely associated with the unique threonine to alanine change at codon 55 (other variants at this codon have been reported in cases of nonsyndromic hearing loss). This report contributes to the short list of GJB2 variants associated with autosomal dominant hearing loss, highlights the variability of skin and nail findings associated with such cases, and illustrates the occurrence of both syndromic and nonsyndromic presentations with changes in the same gene. © 2018 Wiley Periodicals, Inc.

  13. Noise and Hearing Protection

    Science.gov (United States)

    ... ENTCareers Marketplace Find an ENT Doctor Near You Noise and Hearing Protection Noise and Hearing Protection Patient ... it is. How can I tell if a noise is dangerous? People differ in their sensitivity to ...

  14. Genes and Hearing Loss

    Science.gov (United States)

    ... ENTCareers Marketplace Find an ENT Doctor Near You Genes and Hearing Loss Genes and Hearing Loss Patient ... mutation may only have dystopia canthorum. How Do Genes Work? Genes are a road map for the ...

  15. Buying a Hearing Aid

    Science.gov (United States)

    ... the aids? Start using your hearing aids in quiet surroundings, gradually building up to noisier environments. Then eventually work up to wearing your hearing aids all waking hours. Keep a diary to help you remember your ...

  16. OI Issues: Hearing Loss

    Science.gov (United States)

    ... signals normally to the brain. In addition, hearing losses are classified according to the degree of severity: • Mild, • Moderate, • Severe, • Profound. Hearing losses are also classified according to the sound frequency ...

  17. Hearing Loss: Screening Newborns

    Science.gov (United States)

    ... of this page please turn JavaScript on. Feature: Hearing Loss Screening Newborns Past Issues / Spring 2015 Table ... deafness, which account for most cases. Screening Newborns' Hearing Now Standard In 1993, children born in the ...

  18. Hearing and the cochlea

    Science.gov (United States)

    ... like structure that contains the receptor organ for hearing. The cochlea contains the spiral organ of Corti, which is the receptor organ for hearing. It consists of tiny hair cells that translate ...

  19. Occupational hearing loss

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/001048.htm Occupational hearing loss To use the sharing features on this page, please enable JavaScript. Occupational hearing loss is damage to the inner ear from noise ...

  20. Vocabulary and working memory in children fit with hearing aids.

    Science.gov (United States)

    Stiles, Derek J; McGregor, Karla K; Bentler, Ruth A

    2012-02-01

    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. Children 6 to 9 years of age participated. Aspects of working memory were tapped by articulation rate, forward and backward digit span in the auditory and visual modalities, Corsi span, parent surveys, and a sequential encoding task. Articulation rate, digit spans, and Corsi spans were also administered in low-level broadband noise. CHL and children with normal hearing (CNH) demonstrated auditory advantage in forward serial recall. CHL demonstrated slower articulation rates than CNH, but similar memory spans. CHL with poor executive function presented with poorer performance on the Corsi span task. The presence of background noise had no effect on performance in either group. CHL presented with significantly smaller receptive vocabularies than their CNH peers. Across groups, receptive vocabulary size was positively correlated with digit span in quiet, Corsi span in noise, and articulation rate. In the presence of mild-to-moderately severe hearing loss, children demonstrated resilient working memory systems. For all children, working memory and vocabulary were related; that is, children with poorer working memory had smaller vocabulary sizes.

  1. International hearing protector standardization

    DEFF Research Database (Denmark)

    Poulsen, Torben

    2002-01-01

    Hearing protectors shall fulfill some minimum requirements to their performance. As hearing protector manufacturers sell the products all over the world, the testing and certification of hearing protectors has become an international issue. The ISO working group WG17 under the headlines Acoustics...

  2. Hearing Aids Communication

    DEFF Research Database (Denmark)

    Globally, hearing loss is the second most frequent disability. About 80% of the persons affected by hearing loss do not use hearing aids. The goal of this edited volume is to present a theoretically founded, interdisciplinary approach geared at understanding and improving social interaction...

  3. Deafness and Hearing Loss.

    Science.gov (United States)

    National Information Center for Children and Youth with Disabilities, Washington, DC.

    This brief overview provides information on the definition, incidence, and characteristics of children with hearing impairments and deafness. The federal definitions of hearing impairment and deafness are provided. The different types of hearing loss are noted, including: (1) conductive (caused by diseases or obstructions in the outer or middle…

  4. Hearing poorly with skill

    DEFF Research Database (Denmark)

    Day, Dennis

    2012-01-01

    This paper offers an account of ongoing research into hearing. I offer a characterization of 'skil- led practitioners' from an Ethnomethodological perspective. The skilled practitioner in question is a generic 'hard of hearing' person. The ambition is that such a characterization, both in its...... making and its final state, may be an intrinsic part of design practices concerning the development of hearing aids....

  5. Deterioration of noise-induced hearing loss among bottling factory workers.

    Science.gov (United States)

    Ologe, F E; Olajide, T G; Nwawolo, C C; Oyejola, B A

    2008-08-01

    Repeated exposure to excessive noise will eventually lead to an irreversible increase in hearing thresholds. In theory, the damage reflects both the intensity of the noise and the duration of exposure. This is not linear with respect to duration of exposure; rather, the worker may experience a disproportionate loss in the early years of exposure. A prospective study surveying workers of the production section (i.e. most noise-exposed area) of a bottling factory was carried out in December 2003 and in December 2005. A self-administered questionnaire was used to extract information about worker's demographic characteristics, drug intake, and medical and occupational history, as well as information on the use of hearing protection devices. Noise mapping of the various departments of the factory was carried out. Otological examination, tympanometry and audiometry were also carried out on selected subjects. Eighty-four workers, 76 (90.5 per cent) men and eight (9.5 per cent) women, were studied. Their mean age was 33.0 +/- 7.6 years in 2003 and 35.0 +/- 7.6 years in 2005. The recorded noise levels in the factory production section ranged between 91.5 and 98.7 dBA. The prevalence of sensorineural hearing loss among workers was noted to be 64.9 and 86.9 per cent for test one (2003) and test two (2005), respectively. The degree of hearing deterioration within the two years of this study was 1.0-3.2 dB for the right ear and 1.6-3.4 dB for the left ear. This deterioration was at discrete frequencies. More than half (53.6 per cent) of the workers did not have a hearing protection device. Of the 46.4 per cent who did, only 38.5 per cent claimed to have used it regularly. These findings showed that there was a high prevalence of mild sensorineural hearing loss and significant hearing deterioration among workers, due to exposure to excessive noise over a two-year period. The study demonstrates the practical importance of serial audiometry for noise-exposed workers as a means of

  6. Correlation between Serum Aldosterone Level and Hearing Condition of Elderly Patients Referred to Otolaryngology Services of Hamadan, Western Iran

    Directory of Open Access Journals (Sweden)

    Dr. Farhad Farahani

    2010-06-01

    Full Text Available Background and Aim: Recently, more attention was paid to the direct protective effect of aldosterone against hearing impairment in elderly patients. The aim of this study was determination of possible correlation between serum aldosterone level and hearing condition of elderly patients that referred to the Otolaryngology services of Hamadan in 2005-2006.Methods: In this case control study 54 (27 males,27 females persons above 60 years old were evaluated. They contained twenty eight cases with normal hearing and 26 cases with presbycusis. Persons with any abnormal biochemical finding or history of conditions that predispose them to the sensorineural hearing loss (SNHL were excluded. In both groups serum level of sodium, potassium and aldosterone were measured and hearing condition evaluated by puretone, speech and immitance audiometry.Results: Statistical relationship between serum aldostrone level and hearing condition, sex, configuration of audiogram and speech discrimination score (SDS were not significant. In addition, no significant relationship between sodium and potassium levels with hearing condition was found (p>0.05.Conclusion: This study could not confirm protective effect of aldostrone against presbycusis. This discrepancy may originate from epidemiologic differences, laboratory errors or small sample size.

  7. Identification of conductive hearing loss using air conduction tests alone: reliability and validity of an automatic test battery.

    Science.gov (United States)

    Convery, Elizabeth; Keidser, Gitte; Seeto, Mark; Freeston, Katrina; Zhou, Dan; Dillon, Harvey

    2014-01-01

    The primary objective of this study was to determine whether a combination of automatically administered pure-tone audiometry and a tone-in-noise detection task, both delivered via an air conduction (AC) pathway, could reliably and validly predict the presence of a conductive component to the hearing loss. The authors hypothesized that performance on the battery of tests would vary according to hearing loss type. A secondary objective was to evaluate the reliability and validity of a novel automatic audiometry algorithm to assess its suitability for inclusion in the test battery. Participants underwent a series of hearing assessments that were conducted in a randomized order: manual pure-tone air conduction audiometry and bone conduction audiometry; automatic pure-tone air conduction audiometry; and an automatic tone-in-noise detection task. The automatic tests were each administered twice. The ability of the automatic test battery to: (a) predict the presence of an air-bone gap (ABG); and (b) accurately measure AC hearing thresholds was assessed against the results of manual audiometry. Test-retest conditions were compared to determine the reliability of each component of the automatic test battery. Data were collected on 120 ears from normal-hearing and conductive, sensorineural, and mixed hearing-loss subgroups. Performance differences between different types of hearing loss were observed. Ears with a conductive component (conductive and mixed ears) tended to have normal signal to noise ratios (SNR) despite impaired thresholds in quiet, while ears without a conductive component (normal and sensorineural ears) demonstrated, on average, an increasing relationship between their thresholds in quiet and their achieved SNR. Using the relationship between these two measures among ears with no conductive component as a benchmark, the likelihood that an ear has a conductive component can be estimated based on the deviation from this benchmark. The sensitivity and

  8. Rapid word-learning in normal-hearing and hearing-impaired children: effects of age, receptive vocabulary, and high-frequency amplification.

    Science.gov (United States)

    Pittman, A L; Lewis, D E; Hoover, B M; Stelmachowicz, P G

    2005-12-01

    This study examined rapid word-learning in 5- to 14-year-old children with normal and impaired hearing. The effects of age and receptive vocabulary were examined as well as those of high-frequency amplification. Novel words were low-pass filtered at 4 kHz (typical of current amplification devices) and at 9 kHz. It was hypothesized that (1) the children with normal hearing would learn more words than the children with hearing loss, (2) word-learning would increase with age and receptive vocabulary for both groups, and (3) both groups would benefit from a broader frequency bandwidth. Sixty children with normal hearing and 37 children with moderate sensorineural hearing losses participated in this study. Each child viewed a 4-minute animated slideshow containing 8 nonsense words created using the 24 English consonant phonemes (3 consonants per word). Each word was repeated 3 times. Half of the 8 words were low-pass filtered at 4 kHz and half were filtered at 9 kHz. After viewing the story twice, each child was asked to identify the words from among pictures in the slide show. Before testing, a measure of current receptive vocabulary was obtained using the Peabody Picture Vocabulary Test (PPVT-III). The PPVT-III scores of the hearing-impaired children were consistently poorer than those of the normal-hearing children across the age range tested. A similar pattern of results was observed for word-learning in that the performance of the hearing-impaired children was significantly poorer than that of the normal-hearing children. Further analysis of the PPVT and word-learning scores suggested that although word-learning was reduced in the hearing-impaired children, their performance was consistent with their receptive vocabularies. Additionally, no correlation was found between overall performance and the age of identification, age of amplification, or years of amplification in the children with hearing loss. Results also revealed a small increase in performance for both

  9. Disrupted bone remodeling leads to cochlear overgrowth and hearing loss in a mouse model of fibrous dysplasia.

    Directory of Open Access Journals (Sweden)

    Omar Akil

    Full Text Available Normal hearing requires exquisite cooperation between bony and sensorineural structures within the cochlea. For example, the inner ear secretes proteins such as osteoprotegrin (OPG that can prevent cochlear bone remodeling. Accordingly, diseases that affect bone regulation can also result in hearing loss. Patients with fibrous dysplasia develop trabecular bone overgrowth resulting in hearing loss if the lesions affect the temporal bones. Unfortunately, the mechanisms responsible for this hearing loss, which could be sensorineural and/or conductive, remain unclear. In this study, we used a unique transgenic mouse model of increased Gs G-protein coupled receptor (GPCR signaling induced by expression of an engineered receptor, Rs1, in osteoblastic cells. These ColI(2.3+/Rs1+ mice showed dramatic bone lesions that histologically and radiologically resembled fibrous dysplasia. We found that ColI(2.3+/Rs1+ mice showed progressive and severe conductive hearing loss. Ossicular chain impingement increased with the size and number of dysplastic lesions. While sensorineural structures were unaffected, ColI(2.3+/Rs1+ cochleae had abnormally high osteoclast activity, together with elevated tartrate resistant acid phosphatase (TRAP activity and receptor activator of nuclear factor kappa-B ligand (Rankl mRNA expression. ColI(2.3+/Rs1+ cochleae also showed decreased expression of Sclerostin (Sost, an antagonist of the Wnt signaling pathway that normally increases bone formation. The osteocyte canalicular networks of ColI(2.3+/Rs1+ cochleae were disrupted and showed abnormal osteocyte morphology. The osteocytes in the ColI(2.3+/Rs1+ cochleae showed increased expression of matrix metalloproteinase 13 (MMP-13 and TRAP, both of which can support osteocyte-mediated peri-lacunar remodeling. Thus, while the ossicular chain impingement is sufficient to account for the progressive hearing loss in fibrous dysplasia, the deregulation of bone remodeling extends to the

  10. Music and hearing aids.

    Science.gov (United States)

    Madsen, Sara M K; Moore, Brian C J

    2014-10-31

    The signal processing and fitting methods used for hearing aids have mainly been designed to optimize the intelligibility of speech. Little attention has been paid to the effectiveness of hearing aids for listening to music. Perhaps as a consequence, many hearing-aid users complain that they are not satisfied with their hearing aids when listening to music. This issue inspired the Internet-based survey presented here. The survey was designed to identify the nature and prevalence of problems associated with listening to live and reproduced music with hearing aids. Responses from 523 hearing-aid users to 21 multiple-choice questions are presented and analyzed, and the relationships between responses to questions regarding music and questions concerned with information about the respondents, their hearing aids, and their hearing loss are described. Large proportions of the respondents reported that they found their hearing aids to be helpful for listening to both live and reproduced music, although less so for the former. The survey also identified problems such as distortion, acoustic feedback, insufficient or excessive gain, unbalanced frequency response, and reduced tone quality. The results indicate that the enjoyment of listening to music with hearing aids could be improved by an increase of the input and output dynamic range, extension of the low-frequency response, and improvement of feedback cancellation and automatic gain control systems. © The Author(s) 2014.

  11. Music and Hearing Aids

    Directory of Open Access Journals (Sweden)

    Sara M. K. Madsen

    2014-10-01

    Full Text Available The signal processing and fitting methods used for hearing aids have mainly been designed to optimize the intelligibility of speech. Little attention has been paid to the effectiveness of hearing aids for listening to music. Perhaps as a consequence, many hearing-aid users complain that they are not satisfied with their hearing aids when listening to music. This issue inspired the Internet-based survey presented here. The survey was designed to identify the nature and prevalence of problems associated with listening to live and reproduced music with hearing aids. Responses from 523 hearing-aid users to 21 multiple-choice questions are presented and analyzed, and the relationships between responses to questions regarding music and questions concerned with information about the respondents, their hearing aids, and their hearing loss are described. Large proportions of the respondents reported that they found their hearing aids to be helpful for listening to both live and reproduced music, although less so for the former. The survey also identified problems such as distortion, acoustic feedback, insufficient or excessive gain, unbalanced frequency response, and reduced tone quality. The results indicate that the enjoyment of listening to music with hearing aids could be improved by an increase of the input and output dynamic range, extension of the low-frequency response, and improvement of feedback cancellation and automatic gain control systems.

  12. Electromagnetic interference of bone-anchored hearing aids by cellular phones.

    Science.gov (United States)

    Kompis, M; Negri, S; Häusler, R

    2000-10-01

    We report a case of electromagnetic interference between a bone-anchored hearing aid (BAHA) and a cellular phone. A 54-year-old women was successfully treated for severe mixed conductive and sensorineural hearing loss with a BAHA. Five years after implantation, the patient experienced a sudden feeling of dizziness, accompanied by a loud buzzing sound and by a sensation of head pressure while examining a digital mobile phone. During a subsequent experiment, the buzzing sound could be reproduced and was identified as electromagnetic interference between the BAHA and digital cellular phones. Seventeen adult BAHA users from our clinic participated in a subsequent survey. Of the 13 patients with some experience of digital cellular phones, 11 reported hearing annoying noises elicited by these devices. However, no other sensation, such as dizziness, was described. Owing to the increasing number of users of both hearing aids and cellular phones, the incidence of electromagnetic interference must be expected to increase as well. Although to date there is no evidence that such interference may be harmful or dangerous to users of conventional or bone-anchored hearing aids, unexpected interference can be a frightening experience.

  13. A Targeted Approach for Congenital Cytomegalovirus Screening Within Newborn Hearing Screening.

    Science.gov (United States)

    Fowler, Karen B; McCollister, Faye P; Sabo, Diane L; Shoup, Angela G; Owen, Kris E; Woodruff, Julie L; Cox, Edith; Mohamed, Lisa S; Choo, Daniel I; Boppana, Suresh B

    2017-02-01

    Congenital cytomegalovirus (cCMV) infection remains a leading cause of childhood hearing loss. Currently universal CMV screening at birth does not exist in the United States. An alternative approach could be testing infants who do not pass their newborn hearing screening (NHS) for cCMV. This study was undertaken to evaluate whether a targeted approach will identify infants with CMV-related sensorineural hearing loss (SNHL). Infants born at 7 US medical centers received NHS and were also screened for cCMV while in the newborn nursery. Infants who tested positive for CMV received further diagnostic audiologic evaluations to identify or confirm hearing loss. Between 2007 and 2012, 99 945 newborns were screened for both hearing impairment and cCMV. Overall, 7.0% of CMV-positive infants did not pass NHS compared with 0.9% of CMV-negative infants (P CMV-infected infants who passed their NHS had SNHL confirmed by further evaluation during early infancy. NHS in this cohort identified 57% of all CMV-related SNHL that occurred in the neonatal period. A targeted CMV approach that tests newborns who fail their NHS identified the majority of infants with CMV-related SNHL at birth. However, 43% of the infants with CMV-related SNHL in the neonatal period and cCMV infants who are at risk for late onset SNHL were not identified by NHS. Copyright © 2017 by the American Academy of Pediatrics.

  14. Assessment of senses of hearing and balance in chronic suppurative otitis media

    Directory of Open Access Journals (Sweden)

    Babić Borivoj

    2008-01-01

    Full Text Available Chronic suppurative otitis media is among the most frequent illnesses treated in ENT wards. To establish the diagnosis, otomicroscopy alone usually suffice. Assessing the sense of hearing, often the sense of balance, too, is mandatory. Assessment of hearing will show the extent of conductive and sensorineural hearing loss. Apart from this, sometimes, when the finding is not obvious, it is necessary to conduct audiological investigation in more detail in order to establish whether the diagnosis of chronic suppurative otitis media is correct. Existence and extent of conductive hearing loss or confIrmation of tympanic membrane perforation by tympanometry can help a great deal. Also, some new results about the site of perforation and the middle ear volume influence on conductive hearing loss may help have a better insight into chronic suppurative otitis media. Assessing the sense of balance may show dysfunctions with not yet necessarily permanent damage: perilymphatic fistula and benign paroxysmal positional vertigo (BPPV. Unilateral or bilateral damage may be diagnosed with appropriate tests of vestibuloocular reflex (VOR: spontaneous nystagmus, head impulse test, head