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Sample records for acquired sensorineural hearing

  1. [Berger's disease and acquired sensorineural hearing loss].

    PubMed

    Ramírez Ponferrada, R; Gallardo Avilla, A; Solís García, E; Castilla, J M; Martínez, R; Rodríguez, B

    1997-03-01

    A relation between kidney and inner ear disease, specifically neurosensorial hearing loss, has been established. Likewise, the role of tonsillitis in certain glomerulonephritides is well known. A case of post-streptococcal mesangial glomerulonephritis with IgA deposit (Berger's disease) and neurosensorial hearing loss is reported. The absence of any relevant family or personal history suggests an immunological origin for both disorders.

  2. Eye color as a risk factor for acquired sensorineural hearing loss: a review.

    PubMed

    Mujica-Mota, Mario A; Schermbrucker, Jonah; Daniel, Sam J

    2015-02-01

    Eye color may be an indicator of inner ear melanin content and has been associated with hearing loss. There is controversy as to whether eye color has an effect on acquired causes of sensorineural hearing loss. This review was conducted to analyze the literature evaluating the relationship between eye color and causes of sensorineural hearing loss. Six databases were searched to identify eligible studies. Included articles were independently assessed for quality by two authors. Eighteen articles were eligible for review. Eye color was not found to have an effect in the non-exposed population or in presbycusis. In noise-induced sensorineural hearing loss, light-eyed patients had more significant loss following noise exposure, although the variability reported due to eye color was modest (r(2) = 0.01-0.14). Two out of three studies reported that dark eye color is associated with cisplatin ototoxicity. In one study, green-eyed patients were found to be at higher risk of radiation-induced hearing loss. Eye color does not appear to play a role in hearing loss in non-exposed individuals or presbycusis. It is possible that dark-eyed individuals, with greater inner ear melanin content, are better protected against noise-induced hearing loss. Evidence suggests that melanin can be protective against radiation-induced sensorineural hearing loss, but may predispose individuals to cisplatin ototoxicity. Future studies are required to support these conclusions.

  3. Sudden sensorineural hearing loss.

    PubMed

    Stew, B T; Fishpool, S J C; Williams, H

    2012-02-01

    Sudden onset sensorineural hearing loss is a medical emergency that continues to be poorly understood despite being recognized in the literature since 1944 (De Kleyn, 1944). A commonly used criterion to qualify for this diagnosis is a sensorineural hearing loss over three contiguous pure-tone frequencies of 30 dB or more that develops within 72 hours. The vast majority of cases are unilateral and the estimated annual incidence is 20 per 100 000 persons (Nosrati-Zarenoe et al, 2007). A cause for the hearing loss is only identified in up to 10% of cases but 50% of patients will improve spontaneously (Penido et al, 2009).

  4. Congenital sensorineural hearing loss

    SciTech Connect

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

    1984-02-01

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

  5. Sudden Sensorineural Hearing Loss

    PubMed Central

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

    2011-01-01

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

  6. Sudden sensorineural hearing loss.

    PubMed

    Schreiber, Benjamin E; Agrup, Charlotte; Haskard, Dorian O; Luxon, Linda M

    2010-04-03

    Sudden sensorineural hearing loss is usually unilateral and can be associated with tinnitus and vertigo. In most cases the cause is not identified, although various infective, vascular, and immune causes have been proposed. A careful examination is needed to exclude life threatening or treatable causes such as vascular events and malignant diseases, and patients should be referred urgently for further assessment. About half of patients completely recover, usually in about 2 weeks. Many treatments are used, including corticosteroids, antiviral drugs, and vasoactive and oxygen-based treatments. Although no treatment is proven, we recommend a short course of oral high-dose corticosteroids. There is much to learn about pathogenesis of sudden sensorineural hearing loss, and more clinical trials are needed to establish evidence-based management.

  7. Pediatric Sudden Sensorineural Hearing Loss.

    PubMed

    Kizilay, Ahmet; Koca, Çiğdem Firat

    2016-06-01

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

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

    PubMed

    Kujawa, Sharon G; Liberman, M Charles

    2015-12-01

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

  9. Evidence-based practice: management of adult sensorineural hearing loss.

    PubMed

    Chau, Justin K; Cho, John J W; Fritz, Dieter K

    2012-10-01

    Sensorineural hearing loss is a complex disease state influenced by genetics, age, noise, and many other factors. This article reviews our current knowledge regarding the causes of sensorineural hearing loss and reviews the more challenging clinical presentations of sensorineural hearing loss. We have reviewed the latest medical literature in an attempt to provide an evidence-based strategy for the assessment and management of sudden sensorineural hearing loss, rapidly progressive sensorineural hearing loss, and asymmetric/unilateral sensorineural hearing loss.

  10. Sudden sensorineural hearing loss after heroin injection.

    PubMed

    Schrock, Andreas; Jakob, Mark; Wirz, Stefan; Bootz, Friedrich

    2008-05-01

    Sudden sensorineural hearing loss is a symptom of cochlear injury. Potential aetiologies are vascular diseases, viral infections, allergic reactions, autoimmune disorders, and traumatic rupture of the intralabyrinthe membrane. Unlike in unilateral cases bilateral sensorineural hearing loss is often associated with specific disease entities. We report a case of sudden bilateral deafness after intravenous heroin abuse. The putative pathophysiological mechanisms are discussed.

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

    PubMed

    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.

  12. Chikungunya virus induced sudden sensorineural hearing loss.

    PubMed

    Bhavana, Kranti; Tyagi, Isha; Kapila, Rajeev Kumar

    2008-02-01

    The aim of this study is to demonstrate the association of Chikungunya virus and sudden sensorineural hearing loss. In the case report described we had a case which developed sudden unilateral sensorineural hearing loss following chikungunya fever. A 15-year-old female presented to us with the complains of unilateral sudden onset of hearing loss following an episode of fever, arthralgia and rashes 1 month ago. At the time of these symptoms there were many cases of chikungunya fever in the city, three being in her locality. Clinically Chikungunya fever was suspected and a positive serological test further confirmed our diagnosis. The hearing loss could thus be attributed to Chikungunya virus. Viruses have always been implicated in causing sudden sensorineural hearing loss but Chikungunya virus as a cause has not been documented earlier making this case report a unique one.

  13. Medulloblastoma manifesting as sudden sensorineural hearing loss.

    PubMed

    Terakawa, Yuzo; Tsuyuguchi, Naohiro; Takami, Toshihiro; Ohata, Kenji

    2011-07-01

    We present a rare case of medulloblastoma which presented with unilateral sudden sensorineural hearing loss as an initial symptom. A 19-year-old man was admitted to our hospital with a chief complaint of dizziness and facial numbness on the right side. His illness had begun two years previously with sudden hearing loss on the right side, for which he had been treated as an idiopathic sudden hearing loss. Magnetic resonance imaging demonstrated abnormal signals located mainly in the right middle cerebellar peduncle. We performed partial resection of the tumor by suboccipital craniotomy. The histopathological diagnosis was medulloblastoma. Intrinsic brain tumor is an extremely rare cause of sudden sensorineural hearing loss and is therefore easily overlooked as was in the present case. The present case highlights not only the need to evaluate patients with sudden sensorineural hearing loss by magnetic resonance imaging but also the importance of paying attention to intrinsic lesions involving the brainstem. Although this condition like the presented case might be rare, intrinsic brain tumor should be considered as a potential cause of sudden sensorineural hearing loss, as it may be easily missed leading to a delay in appropriate treatment.

  14. Vibrant Soundbridge rehabilitation of sensorineural hearing loss.

    PubMed

    Khan, Andleeb; Hillman, Todd; Chen, Douglas

    2014-12-01

    The Vibrant Soundbridge is a means to rehabilitate patients with sensorineural hearing loss. It differs from hearing aids in that it uses mechanical energy rather than acoustic sound to deliver better sound quality to the inner ear. The implant's crucial component is a floating mass transducer that is directly fixed to the incus to drive it, which is introduced into the middle ear through a facial recess approach. Although this is a newer technology, studies thus far have demonstrated better hearing results compared with hearing aids in terms of functional gain and speech intelligibility, and better outcomes on subjective assessments.

  15. Sudden sensorineural hearing loss associated with vardenafil.

    PubMed

    Snodgrass, Amanda J; Campbell, Heather M; Mace, David L; Faria, Victoria L; Swanson, Kathleen M; Holodniy, Mark

    2010-01-01

    The phosphodiesterase type 5 (PDE-5) inhibitors-sildenafil, vardenafil, and tadalafil-are used primarily in erectile dysfunction, but sildenafil is also indicated for pulmonary hypertension. Common adverse effects of vardenafil include headache, flushing, nasal congestion, dyspepsia, and nausea. Recently, PDE-5 inhibitors have been associated with adverse vision effects, and emerging evidence now indicates that they may also be responsible for hearing changes and hearing loss. We describe a patient who developed unilateral sudden sensorineural hearing loss possibly related to the use of vardenafil for erectile dysfunction. To our knowledge, only one other case of hearing loss related to this drug class has been published. Our patient was a 57-year-old man who came to the emergency department with right-sided mild-to-moderate hearing loss in the 500-3000-Hz range, confirmed by audiogram, that occurred after ingestion of vardenafil. The patient was hospitalized 2 days later for administration of intravenous dexamethasone, followed by oral prednisone. He reported that his hearing had improved on the fourth hospital day and was discharged 3 days later, continuing to taper the prednisone on an outpatient basis. A repeat audiogram after 10 days of corticosteroid therapy confirmed that his hearing in the 500-3000-Hz range was within normal limits. Use of the Naranjo adverse drug reaction probability scale indicated a possible (score of 3) adverse reaction of sudden sensorineural hearing loss associated with vardenafil consumption. We also performed an analysis of hearing loss cases related to PDE-5 inhibitors in the United States Food and Drug Administration's Adverse Event Reporting System database to compare the characteristics of our patient with those of other reported adverse event cases. Based on the temporal relation of the sudden sensorineural hearing loss to this patient's drug consumption, we propose that the vardenafil is a likely cause of the hearing loss

  16. Sudden Sensorineural Hearing Loss: Primary Care Update

    PubMed Central

    Leung, Marcia A; Flaherty, Anna; Zhang, Julia A; Hara, Jared; Barber, Wayne

    2016-01-01

    The primary care physician's role in recognizing sudden sensorineural hearing (SSNHL) loss and delivering initial treatment is critical in the management of the syndrome. This role involves recognizing its clinical symptoms, distinguishing it from conductive hearing loss with the Weber tuning fork or the Rauch hum test, and urgent administration of high dose oral corticosteroids. Diagnosis and treatment should not be delayed for audiometric testing or referral to otolaryngology. This paper provides an update on the initial evaluation and treatment of this syndrome based on the literature and clinical guideline recommendations. PMID:27413627

  17. Idiopathic Sudden Sensorineural Hearing Loss With Minimal Hearing Impairment

    PubMed Central

    Cho, Chin Saeng

    2015-01-01

    Objectives The aim of the study was to determine the characteristics of patients who did not match the audiometric criteria of idiopathic sudden sensorineural hearing loss (SSNHL) but complained of acute hearing loss. Methods By thorough medical chart reviews, historical cohort study was performed with consecutive data of 589 patients complaining of acute unilateral sensorineural hearing loss without identifiable causes between 2005 and 2013. Those patients demonstrating a hearing loss of at least 30 dB at three consecutive frequencies based on pure tone audiometry were classified as group I; the others were classified as group II. Patients' characteristics, final hearing, and hearing improvement rate (HIR) between the two groups were compared. Results Group II exhibited distinctive characteristics, including an early age of onset of the hearing loss (P<0.01), an absence of accompanying diabetes (P<0.01) and hypertension (P<0.01), and better unaffected hearing and final hearing compared with group I (P<0.001). However, the HIR of the patients in the two groups was not significantly different (P>0.05). Conclusion Patients who did not meet the audiological criteria of SSNHL exhibited distinctive characteristics compared to SSNHL patients. PMID:26622953

  18. Genetic Effects on Sensorineural Hearing Loss and Evidence-based Treatment for Sensorineural Hearing Loss.

    PubMed

    Yu, Yong-qiang; Yang, Huai-an; Xiao, Ming; Wang, Jing-wei; Huang, Dong-yan; Bhambhani, Yagesh; Sonnenberg, Lyn; Clark, Brenda; Jin, Yuan-zhe; Fu, Wei-neng; Zhang, Jie; Yu, Qian; Liang, Xue-ting; Zhang, Ming

    2015-09-01

    In this article, the mechanism of inheritance behind inherited hearing loss and genetic susceptibility in noise-induced hearing loss are reviewed. Conventional treatments for sensorineural hearing loss (SNHL), i.e. hearing aid and cochlear implant, are effective for some cases, but not without limitations. For example, they provide little benefit for patients of profound SNHL or neural hearing loss, especially when the hearing loss is in poor dynamic range and with low frequency resolution. We emphasize the most recent evidence-based treatment in this field, which includes gene therapy and allotransplantation of stem cells. Their promising results have shown that they might be options of treatment for profound SNHL and neural hearing loss. Although some treatments are still at the experimental stage, it is helpful to be aware of the novel therapies and endeavour to explore the feasibility of their clinical application.

  19. Laterality of sudden sensorineural hearing loss.

    PubMed

    Reiss, Michael; Reiss, Gilfe

    2014-08-01

    It is known that sudden sensorineural hearing loss and other otoneurologic diseases, such as tinnitus or Ménière disease, occur more frequently in the left ear than in the right. We studied lateralization of sudden deafness in 489 patients treated at Radebeul Hospital from January 2004 to December 2009. The male-to-female ratio was 1:1.24; we found a predominance of the left side only in female patients. The cause for this predominance is unclear. The slight asymmetry might indicate a greater vulnerability of the left inner ear in women, suggesting hormonal factors in the genesis of sudden deafness.

  20. Sensorineural hearing loss in Kawasaki disease.

    PubMed

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

    2016-01-01

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

  1. Sensorineural hearing loss and auditory perceptual organization

    NASA Astrophysics Data System (ADS)

    Hall, Joseph W.; Grose, John H.; Buss, Emily

    2004-05-01

    This talk will consider the implications of sensorineural hearing loss for auditory perceptual organization. In everyday environments, the listener is often faced with the difficulty of processing a target sound that intermingles acoustically with one or more extraneous sounds. Under such circumstances, several auditory processes enable the complex waveforms reaching the two ears to be interpreted in terms of putative auditory objects giving rise to the target and extraneous sounds. Such processes of perceptual organization depend upon the central analysis of cues that allow distributed spectral information to be either linked together or split apart on the basis of details related to such variables as synchrony of onset/modulation, harmonic relation, rhythm, and interaural differences. Efficient perceptual organization must depend not only upon such central auditory analyses but also upon the fidelity with which the peripheral auditory system encodes the spectral and temporal characteristics of sound. We will consider the implications of sensorineural hearing loss for perceptual organization in terms of both peripheral and central auditory processes.

  2. Prognostic Factors in Sudden Sensorineural Hearing Loss

    PubMed Central

    Atay, Gamze; Kayahan, Bahar; çınar, Betül çiçek; Saraç, Sarp; Sennaroğlu, Levent

    2016-01-01

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

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

    PubMed

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

    2016-05-01

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

  4. Idiopathic sensorineural hearing loss in the only hearing ear.

    PubMed

    Berrettini, S; De Vito, A; Bruschini, L; Fortunato, S; Forli, F

    2016-04-01

    A retrospective chart review was used for 31 patients with sudden, progressive or fluctuating sensorineural hearing loss (SHL) in the only hearing ear who had been consecutively evaluated at the ENT, Audiology and Phoniatrics Unit of the University of Pisa. The group of patients was evaluated with a complete history review, clinical evaluation, imaging exam (MRI, CT), audiologic tests (tone and speech audiometry, tympanometry, study of stapedial reflexes, ABR and otoacoustic emission) evaluation. In order to exclude genetic causes, patients were screened for CX 26 and CX30 mutations and for mitochondrial DNA mutation A1555G. Patients with sudden or rapidly progressive SHL in the only hearing ear were treated with osmotic diuretics and corticosteroids. In patients who did not respond to intravenous therapy we performed intratympanic injections of corticosteroid. Hearing aids were fitted when indicated and patients who developed severe to profound SHL were scheduled for cochlear implant surgery. The aim of this study is to report and discuss the epidemiology, aetiopathogenesis, therapy and clinical characteristic of patients affected by SHL in the only hearing hear and to discuss the issues related to the cochlear implant procedure in some of these patients, with regard to indications, choice of the ear to implant and results.

  5. Prognostic factors of profound idiopathic sudden sensorineural hearing loss.

    PubMed

    Wen, Yu-Hsuan; Chen, Peir-Rong; Wu, Hung-Pin

    2014-06-01

    Profound idiopathic sudden sensorineural hearing loss is thought to have a poor prognosis, but few studies have focused on this condition. We aimed to assess the impact of patient factors, audiologic parameters, and salvage intratympanic steroid injection therapy on the prognosis of profound idiopathic sudden sensorineural hearing loss. The demographic, clinical, and audiologic data, degree of hearing recovery, and efficacy of intratympanic steroid injection therapy in 576 patients with profound idiopathic sudden sensorineural hearing loss (mean age 56.2 ± 14.9 years) who had been admitted at four tertiary referral centers between 2000 and 2011 were retrospectively reviewed. The mean hearing level at the initial presentation was 108.1 ± 9.5 dB. Many patients experienced vertigo (52.1%) and tinnitus (77.4%). At the 2-month follow-up, 172 (29.8%) patients showed some degree of hearing recovery, but only 21 (3.6%) patients recovered normal hearing. Further, the 116 patients who had received salvage intratympanic steroid injections showed a better audiologic outcome (improvement, 26.1 ± 24.3 vs. 15.7 ± 22.1 dB; P = 0.000) than those who had not (n = 429). In conclusion, a higher degree of hearing loss at the initial presentation indicates a poorer prognosis. Salvage intratympanic steroid injection therapy may improve the hearing of patients with profound idiopathic sudden sensorineural hearing loss after the failure of systemic steroid therapy.

  6. Sudden sensorineural hearing loss in a multiple sclerosis case.

    PubMed

    Tekin, Muhammet; Acar, Gul Ozbilen; Cam, Osman Halit; Hanege, Fatih Mehmet

    2014-01-01

    Multiple sclerosis (MS) is the most common demyelinating disease of the central nervous system. MS involves different regions of the central nervous system in different periods, and causes demyelination. MS is a neuromotor disorder which progresses with remissions and relapses. Symptoms of MS may regress completely or heal after the relapses leaving sequelae. Sudden sensorinerural hearing loss (SSHL) is hearing loss of 30 dB or more over at least three contiguous audiometric frequencies that develops over a period of a few hours to 3 days. In 4-10 % of the MS patients, sensorineural hearing loss occurs between relapses or remissions. In this case, audiotory brainstem response (ABR) test is the most appropriate test for the diagnosis of sensorineural hearing loss in MS patients. In this article, we will discuss a patient diagnosed as MS who presented with sudden sensorineural hearing loss during the remission of the disease.

  7. [Sudden unilateral sensorineural hearing loss after carbon monoxide intoxication].

    PubMed

    Michalska-Piechowiak, Teresa; Miarzyńska, Maria; Perlik-Gattner, Irena

    2004-01-01

    A case of unilateral sensorineural hearing loss of the left ear after carbon monoxide intoxication was presented. The diagnosis was based upon an interview, medical examinations and audiometric investigations. Results of diagnostic evaluations, clinical presentation and treatment were discussed. Hearing improvement was obtained after 6 days of treatment and normal hearing returned after 14 days. Patients who suffer from CO intoxication are at risk of hearing impairment, therefore, there is a need for audiometric follow up in these patients.

  8. Sensorineural hearing loss in hemodialysis patients.

    PubMed

    Jakić, Marko; Mihaljević, Dubravka; Zibar, Lada; Jakić, Marijana; Kotromanović, Zeljko; Roguljić, Hrvoje

    2010-03-01

    Chronic renal failure affects all organ systems. Senses are not exception and hearing impairment is common, particularly sensorineural hearing loss (SNHL). The term SNOS of unknown origin or uremic deafness is related to only a smaller part of the cases with unclear etiology of the impairment. The study searched for SNOS in 66 chronic hemodialysis (HD) patients, mean age 51.50 +/- 12.70 years. They were treated by HD for 69.70 +/- 53.80 months. The relation between the severity of the impairment and the patients' age, duration of HD treatment (months) and a set of laboratory parameters typical for chronic HD patients was examined. The aim of the study was to detect potential causes of the impairment. The increased hearing threshold (HT) of above 20 dB for all frequencies was found in 42 patients (mean HT 26 +/- 10.50 dB), for speaking area frequencies in 22 patients (mean HT 19.70 +/- 8.80 dB), and in 56 patients for high frequencies (mean HT 41.70 +/- 19.70 dB). The significant positive correlation of HT was found only with the patients' age (r = 0.49, p < 0.01). The patients older than 45 years had higher mean HT than those younger, and those older than 65 also had higher HT than the younger ones. Patients with pathological value of HT were significantly more common among the older subgroup of patients, when divided according to the age at both cutoff values of 45 and of 60 years. Mean HT did not differ significantly according to the duration of HD treatment (subgroups A- no longer than 60 months, B- from 61 to 120 months, and C- longer than 120 months). The patients with pathological HT did not differ significantly in frequency among those subgroups, and the subgroups were not different according to the mean age (A--50.30 +/- 13.20 years; B--51.40 +/- 12.75 years; C--55.80 +/- 10.55 years). In conclusion, our results along with other authors'published data report on SNHL as very frequent finding among chronic HD patients and suggest multifactorial etiology

  9. Dichotic listening in adults with sensorineural hearing loss.

    PubMed

    Roeser, R J; Johns, D F; Price, L L

    1976-01-01

    Digits and consonant-vowel (CV) nonsense syllables were presented dichotically to 36 normal-hearing subjects and 36 subjects with bilaterally symmetrical sensorineural hearing loss. The normal-hearing subjects performed significantly better in the recall of both digits and CV nonsense syllables, and recall decreased significantly as the degree of hearing loss increased. The expected right ear advantage was observed for the normal-hearing subjects on both tests. As a group, the hearing-loss subjects showed no significant ear preference, and ear preference did not vary significantly with the degree of hearing loss. However, individual hearing-loss subjects showed marked ear asymmetry for the dichotic digits. For both dichotic tests, individual ear preference increased as the degree of hearing loss increased. Significant but low correlations were observed between better ear speech discrimination scores for the hearing-loss subjects and the preferred ear for dichotic CV nonsense syllables.

  10. [Aggravation after Diagnosis of Sudden Sensorineural Hearing Loss].

    PubMed

    Fukumoto, Ichiro; Nemoto, Toshimitsu; Tsukuda, Tomoko; Koshizuka, Keiichi

    2015-03-01

    Among 95 patients with sudden sensorineural hearing loss who received inpatient treatment at our hospital within the 27-month period between October 2009 and December 2011, those in whom hearing loss was aggravated after diagnosis were compared with a control group. Hearing loss aggravation was defined as a decrease by 10 dB or more in the mean hearing threshold at 5 frequencies from 250 to 4,000 Hz or decrease of 15 dB or more in the hearing threshold at 2 consecutive frequencies. Hearing loss was aggravated after diagnosis in 22 (23.2%) of the 95 patients, showing a similar tendency to that previously reported. Although the grades of hearing loss in these patients were higher than those in 73 control group patients, according to the sudden hearing loss severity classification, their outcomes were favorable. The hearing loss aggravation group consisted of those with steroid-dependent hearing loss (6) and those who had undergone perilymphatic fistula repair (4), in addition to a large number of patients with idiopathic hearing loss, including suspicious perilymphatic fistula (10). When hearing loss becomes aggravated after the diagnosis of sudden sensorineural hearing loss, it may be important to determine the most appropriate approach in each case, such as a careful and gradual decrease in the adenocortical steroid dose and the consideration of perilymphatic fistula repair.

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

    PubMed

    Page, Joshua Cody; Peters, Bob

    2015-01-01

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

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

    PubMed

    Turan, Mahfuz; Kalkan, Ferhat; Bozan, Nazım; Özçalimli, İsa; Zeki Erdem, Mehmet; Yalınkılıç, Abdülaziz; Garca, Mehmet Fatih

    2015-01-01

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

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

    PubMed Central

    Turan, Mahfuz; Kalkan, Ferhat; Bozan, Nazım; Özçalimli, İsa; Zeki Erdem, Mehmet; Yalınkılıç, Abdülaziz; Garca, Mehmet Fatih

    2015-01-01

    In most of the surviving patients after a lightning strike, audiovestibular abnormalities have been reported. The most frequently reported type of abnormalities is a tympanic membrane perforation with hearing loss and external ear canal burn. However a sensor neural hearing loss and mixed type hearing loss can also occur, but these occur rarely. A nineteen-year-old female patient had, after a lightning strike, serious burns on the left ear, behind the ear, and on the chest and neck. She also had in her left ear 108 dB hearing loss with irregular central perforation and in her right ear 52 dB sensorineural hearing loss. There was no hearing loss before the strike. A hearing aid was recommended for the right ear and good care and follow-up were recommended for the left ear. A lightning strike can cause serious audiological damage. Therefore, it is necessary to make a careful audiovestibular evaluation of the patients. Although there exist rarely healed cases from sensorineural hearing loss after lightning strike in literature, in our case hearing loss occurred bilaterally and then it healed unilaterally. This condition is quite rare in literature. PMID:26161278

  14. Lipid Profile among Patients with Sudden Sensorineural Hearing Loss.

    PubMed

    Mohammed, Ali A Muttalib

    2014-12-01

    Associations between hearing and blood lipids have been the focus of scientific inquiry for more than 50 years. The aim of the present study is to evaluate the association between hyperlipidaemia among patients presented with sudden sensorineural hearing loss compared to normal controls. A case control study concerned with 22 patients presented with sudden sensorineural hearing loss who underwent lipid profile evaluation. The lipid profile of these patients was compared with corresponding results of 55 age matched persons (volunteers) with normal hearing. These patients were collected from the Out Patient Department of ENT at Al-Jamhory Teaching Hospital, Mosul/Iraq and private clinic of the author for the period from February 2011 to July 2013. The average age of patients was 44.7 years with a range of 26-65 years. The peak age incidence was in the 5(th) decade of life. The study included 11 male patients (50 %) and 11 females (50 %). Meanwhile, the average age of the control group was 41.7 years with 25 (45.5 %) males and 30 (54.5 %) females. Statistical analysis showed that there was significant difference between the means of lipid profile and blood sugar of the patients and the control group apart from HDL where there was no significant difference. In conclusion, hyperlipidemia seems to be significantly associated with the occurrence of sudden sensorineural hearing loss according to this study.

  15. Pediatric vestibular evaluation: two children with sensorineural hearing loss.

    PubMed

    Valente, L Maureen; Goebel, Joel A; Sinks, Belinda

    2012-04-01

    These two cases illustrate several important areas of vestibular evaluation with children. The two case reports represent two children who display very different vestibular findings despite having significant sensorineural hearing loss. These case reports highlight that pediatric findings can differ significantly from adult findings, stressing the importance of comparing pediatric results with pediatric normative data. These two cases also highlight that vestibular techniques may successfully be adapted for use with hearing-impaired children. That is, rotary chair, computerized dynamic posturography, and vestibular evoked myogenic potentials can be adapted to use with children, including those who demonstrate significant sensorineural hearing loss. Although there is a paucity of research and clinical work in this area, some investigators (Eviatar and Eviatar, 1977; Buchman et al, 2004; Jacot et al, 2009) have reported very rapid recovery from pediatric vestibular deficits. However, it is important for audiologists to be aware that techniques may successfully be adapted for children and that many children should undergo thorough vestibular evaluation.

  16. Etiological diagnosis of bilateral, sensorineural hearing impairment in a pediatric Greek population.

    PubMed

    Riga, M; Psarommatis, I; Lyra, Ch; Douniadakis, D; Tsakanikos, M; Neou, P; Apostolopoulos, N

    2005-04-01

    Early diagnosis, evaluation and treatment of childhood deafness are essential for a child's normal growth. Etiological diagnosis of hearing loss makes prevention, family scheduling and more effective therapy feasible goals. Etiological assessment of sensorineural deafness still remains difficult although recently with the progress of genetics it has become more efficient. In this retrospective study, the etiology of bilateral, sensorineural hearing loss with indication for hearing aids has been studied in 153 hearing impaired children. Etiological diagnosis was based on family and patient record, physical, audiological and laboratory examinations. Among the 94 children who completed the diagnostic protocol etiological groups revealed the following distribution: non-hereditary acquired hearing impairment was present in 36 children (38%) and hereditary was present in 44 (47%) children. The etiology remained unknown in 14 (15%) children. Non-syndromic autosomal dominant type accounted for 13 (29% of hereditary hearing loss) children, non-syndromic autosomal recessive type for 21 (48%) children and syndromic deafness for 10 (23%) children. Modern diagnostic methods, such as genetic testing, help diminish the number of cases with hearing impairment of unknown etiology, for the benefit of children who receive early and appropriate medical, audiologic, genetic and educational counseling based on the etiology of their hearing loss.

  17. Head Position Comparison between Students with Normal Hearing and Students with Sensorineural Hearing Loss

    PubMed Central

    Melo, Renato de Souza; Amorim da Silva, Polyanna Waleska; Souza, Robson Arruda; Raposo, Maria Cristina Falcão; Ferraz, Karla Mônica

    2013-01-01

    Introduction Head sense position is coordinated by sensory activity of the vestibular system, located in the inner ear. Children with sensorineural hearing loss may show changes in the vestibular system as a result of injury to the inner ear, which can alter the sense of head position in this population. Aim Analyze the head alignment in students with normal hearing and students with sensorineural hearing loss and compare the data between groups. Methods This prospective cross-sectional study examined the head alignment of 96 students, 48 with normal hearing and 48 with sensorineural hearing loss, aged between 7 and 18 years. The analysis of head alignment occurred through postural assessment performed according to the criteria proposed by Kendall et al. For data analysis we used the chi-square test or Fisher exact test. Results The students with hearing loss had a higher occurrence of changes in the alignment of the head than normally hearing students (p < 0.001). Forward head posture was the type of postural change observed most, occurring in greater proportion in children with hearing loss (p < 0.001), followed by the side slope head posture (p < 0.001). Conclusion Children with sensorineural hearing loss showed more changes in the head posture compared with children with normal hearing. PMID:25992037

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

    PubMed Central

    Bal, Kemal Koray; Bucioglu, Helen; Vayısoğlu, Yusuf; Gorur, Kemal

    2016-01-01

    Sudden hearing loss in the only hearing ear cases are rarely published in the English literature; most of the cases are idiopathic. It is an otologic emergency needing urgent treatment. Delayed diagnosis can interfere with patient's social life with interrupting the verbal communication. In this case report we presented a 33-year-old female patient having sudden sensorineural hearing loss in the only hearing ear diagnosed as bilateral large vestibular aqueduct syndrome. PMID:28018692

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

    PubMed

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

    2015-12-15

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

  20. Bilateral sudden sensorineural hearing loss following unilateral temporal bone fracture.

    PubMed

    Hunchaisri, Niran

    2009-06-01

    Temporal bone fractures usually cause unilateral sensorineural hearing loss (SNHL) by fracture that violated otic capsule of that side. Bilateral SNHL from unilateral temporal bone fracture were rarely seen. Labyrinthine concussion was considered to be the pathogenesis in these cases. This article reports an additional case of bilateral SNHL from unilateral temporal bone fracture but in a different pattern of SNHL which may result from an occlusion of the internal auditory artery.

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

    PubMed

    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.

  2. Implications of Sensorineural Hearing Loss With Hydrocodone/Acetaminophen Abuse

    PubMed Central

    Novac, Andrei; Iosif, Anamaria M.; Groysman, Regina; Bota, Robert G.

    2015-01-01

    Sensorineural hearing loss is an infrequently recognized side effect of pain medication abuse. Chronic pain patients treated with opiates develop different degrees of tolerance to pain medications. In many cases, the tolerance becomes the gateway to a variety of cycles of overuse and unmasking of significant psychiatric morbidity and mortality. An individualized approach utilizing combined treatment modalities (including nonopiate pharmaceuticals) is expected to become the norm. Patients can now be provided with multidisciplinary care that addresses an individual’s psychiatric, social, and medical needs, which requires close cooperation between physicians of varying specialties. This report describes a patient who experienced hearing loss from hydrocodone/acetaminophen abuse. PMID:26835162

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

    PubMed

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

    2011-07-01

    We report a case of sudden unilateral sensorineural hearing loss of sudden onset during an aeroplane flight, which completely resolved during a roller coaster ride at Alton Towers theme park. A review of the literature concerning sudden idiopathic sensorineural hearing loss and spontaneous resolution are discussed. Initially, pure-tone audiometry showed a profound sensorineural hearing loss in the right ear and mild sensorineural hearing loss in the left ear (of note, the hearing was normal prior to the episode). Following resolution of the patient's symptoms during a roller coaster ride, pure-tone audiometry showed normal hearing thresholds in both ears. Sudden sensorineural hearing loss is a symptom of cochlear injury and the mechanism of the patient's symptoms was attributed to a patent cochlear aqueduct.

  4. [The audiological phenotype and the prevalence of GJB2-related sensorineural loss of hearing in the infants suffering acoustic disturbances].

    PubMed

    Lalaiants, M R; Markova, T G; Bakhshinian, V V; Bliznets, E A; Poliakov, A V; Tavartikiladze, G A

    2014-01-01

    The objective of the present work was to study specific features of the audiological phenotype and the prevalence of GJB2-related sensorineural hearing loss (SNHL) in the infants suffering acoustic disturbances. The study included 264 children with bilateral non-syndromic sensorineural loss of hearing diagnosed during the first year of life by means of detailed audiological examination that included tympanometry, registration of short-latency auditory action potentials (SLAAP), delayed evoked otoacoustic emission (DEOAE), distortion product-frequency otoacoustic emission (DPFOAE), and auditory brain-stem response (ABR). In addition, stationary acoustically evoked responses (SAER) were recorded in 38 children presenting with hearing impairment associated with GJB2-related sensorineural loss of hearing. The follow-up dynamic study involved 113 children subjected to repeated audiological examination. The study revealed the genotype with pathological mutations in 182 (69.0%) children including 171 (64.8%) ones with biallelic mutations and 11 (4.2%) with a single mutation (heterozygous genotype). Eighty two (31.0%) children had genotype without mutations. A total of 21 different mutations and 30 different genotypes were identified. Analysis of the family histories of the children showed that neither the absence of relatives suffering from hearing impairment nor the presence of risk factors of acquired hearing impairment excludes the possibility of GJB2-related sensorineural loss of hearing in the infants. Otoacoustic emission fails to be registered in the majority of the children with the altered genotype (87%) during the stay in the maternity house. Mutations in the GJB2 gene are most frequently diagnosed in the patients with the moderate, moderately severe, and severe loss of hearing. At the same time, almost half of the infants presenting with the mild loss of hearing were found to exhibit changes in the GJB2 gene. The thresholds of registration of short

  5. Sensorineural hearing loss associated with a factitious disorder.

    PubMed

    Maruyama, Ayako; Noguchi, Yoshihiro; Ito, Taku; Narushima, Kenji; Kitamura, Ken

    2015-12-01

    Factitious disorders are characterized by intentionally abnormal physical and/or psychological behavior, and affected patients often make up their symptoms and clinical histories. The most serious and chronic type of factitious disorder is Munchausen syndrome. We report the case of a 24-year-old woman with a 2-year history of sensorineural hearing loss (SNHL) who later confessed to feigning her hearing loss. She was eventually diagnosed with a factitious disorder. During those 2 years, she was able to induce her SNHL by exposing herself to excessive noise or high doses of aspirin. To the best of our knowledge, this is the first report describing an association between a factitious disorder and SNHL.

  6. Dissonance perception by listeners with sensorineural hearing loss

    NASA Astrophysics Data System (ADS)

    Tufts, Jennifer B.; Leek, Marjorie R.; Molis, Michelle R.

    2004-05-01

    The perceived dissonance of two simultaneous tones (a dyad) depends upon their frequency separation (in the case of two pure tones) or their fundamental frequency ratio (in the case of two harmonic complex tones). The purpose of this study was to determine whether the perceived dissonance of puretone and harmonic complex dyads is altered in the presence of sensorineural hearing (SNHL), and, if so, whether this can be explained by the reduced frequency selectivity typically associated with SNHL. Four normal-hearing and four hearing-impaired listeners evaluated the dissonance of puretone and harmonic complex dyads centered at 500 and 2000 Hz. Frequency selectivity was estimated at 500 and 2000 Hz for each listener. People with SNHL rated the dissonance of puretone dyads similarly to the normal-hearing listeners, although auditory sensitivity and frequency selectivity differed considerably between the groups. However, their ratings of harmonic complex dyads did not show the pronounced differences in dissonance as a function of fundamental frequency ratio that were observed in the normal-hearing group. The poorer frequency selectivity of these listeners may have allowed more extensive interactions to occur among harmonic components, resulting in a less clear separation of dissonance and consonance. [Work supported by NIH-NIDCD.

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

    PubMed Central

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

    2016-01-01

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

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

    PubMed Central

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

    2015-01-01

    Objective: To investigate the cerebral gray matter volume alterations in unilateral sudden sensorineural hearing loss patients within the acute period by the voxel-based morphometry method, and to determine if hearing impairment is associated with regional gray matter alterations in unilateral sudden sensorineural hearing loss patients. Study Design: Prospective case study. Setting: Tertiary class A teaching hospital. Patients: Thirty-nine patients with left-side unilateral sudden sensorineural hearing loss and 47 patients with right-side unilateral sudden sensorineural hearing loss. Intervention: Diagnostic. Main Outcome Measure: To compare the regional gray matter of unilateral sudden sensorineural hearing loss patients and healthy control participants. Results: Compared with control groups, patients with left side unilateral sudden sensorineural hearing loss had significant gray matter reductions in the right middle temporal gyrus and right superior temporal gyrus, whereas patients with right side unilateral sudden sensorineural hearing loss showed gray matter decreases in the left superior temporal gyrus and left middle temporal gyrus. A significant negative correlation with the duration of the sudden sensorineural hearing loss (R = −0.427, p = 0.012 for left-side unilateral SSNHL and R = −0.412, p = 0.013 for right-side unilateral SSNHL) was also found in these brain areas. There was no region with increased gray matter found in both groups of unilateral sudden sensorineural hearing loss patients. Conclusions: This study confirms that detectable decreased contralateral auditory cortical morphological changes have occurred in unilateral SSNHL patients within the acute period by voxel-based morphometry methods. The gray matter volumes of these brain areas also perform a negative correlation with the duration of the disease, which suggests a gradual brain structural impairment after the progression of the disease. PMID:26595717

  9. Association of TMTC2 With Human Nonsyndromic Sensorineural Hearing Loss

    PubMed Central

    Runge, Christina L.; Indap, Amit; Zhou, Yifan; Kent, Jack W.; King, Ericka; Erbe, Christy B.; Cole, Regina; Littrell, Jack; Merath, Kate; James, Roland; Rüschendorf, Franz; Kerschner, Joseph E.; Marth, Gabor; Hübner, Norbert; Göring, Harald H. H.; Friedland, David R.; Kwok, Wai-Meng; Olivier, Michael

    2016-01-01

    IMPORTANCE Sensorineural hearing loss (SNHL) is commonly caused by conditions that affect cochlear structures or the auditory nerve, and the genes identified as causing SNHL to date only explain a fraction of the overall genetic risk for this debilitating disorder. It is likely that other genes and mutations also cause SNHL. OBJECTIVE To identify a candidate gene that causes bilateral, symmetric, progressive SNHL in a large multigeneration family of Northern European descent. DESIGN, SETTING, AND PARTICIPANTS In this prospective genotype and phenotype study performed from January 1, 2006, through April 1, 2016, a 6-generation family of Northern European descent with 19 individuals having reported early-onset hearing loss suggestive of an autosomal dominant inheritance were studied at a tertiary academic medical center. In addition, 179 unrelated adult individuals with SNHL and 186 adult individuals reporting nondeafness were examined. MAIN OUTCOMES AND MEASURES Sensorineural hearing loss. RESULTS Nine family members (5 women [55.6%]) provided clinical audiometric and medical records that documented hearing loss. The hearing loss is characterized as bilateral, symmetric, progressive SNHL that reached severe to profound loss in childhood. Audiometric configurations demonstrated a characteristic dip at 1000 to 2000 Hz. All affected family members wear hearing aids or have undergone cochlear implantation. Exome sequencing and linkage and association analyses identified a fully penetrant sequence variant (rs35725509) on chromosome 12q21 (logarithm of odds, 3.3) in the TMTC2 gene region that segregates with SNHL in this family. This gene explains the SNHL occurrence in this family. The variant is also associated with SNHL in a cohort of 363 unrelated individuals (179 patients with confirmed SNHL and 184 controls, P = 7 × 10−4). CONCLUSIONS AND RELEVANCE A previously uncharacterized gene, TMTC2, has been identified as a candidate for causing progressive SNHL in humans

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

    PubMed Central

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

    2016-01-01

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

  11. Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss.

    PubMed

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

    2016-01-25

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

  12. Acute Wernicke encephalopathy and sensorineural hearing loss complicating bariatric surgery.

    PubMed

    Jethava, Ashif; Dasanu, Constantin A

    2012-01-01

    Health-care professionals must be aware of the mandatory vitamin supplementation in patients status post bariatric surgery. A recent increase in the number of gastric bypass surgeries in US has been associated with a proportional increase in Wernicke encephalopathy reports. Subtle or atypical neurologic features are not uncommon. Our report is of a female patient with acute Wernicke encephalopathy accompanied by sensorineural hearing loss six weeks after bariatric surgery. The patient had only a partial recovery of her neurologic symptoms eightweeks after vigorous therapy for this condition. Symptomatic thiamine (vitamin B1) and vitamin B12 deficiencies are particularly concerning effects of bariatric procedures, as neurologic and cognitive deficits may be long lasting or even permanent despite aggressive replacement therapy.

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

    PubMed Central

    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

  14. Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss

    PubMed Central

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

    2016-01-01

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

  15. A case of sudden unilateral sensorineural hearing loss with contralateral psychogenic hearing loss induced by gunshot noise.

    PubMed

    Hong, Young-Ho; Mun, Seog-Kyun

    2011-10-01

    The reasons behind sudden sensorineural hearing loss are mostly unknown, but viral infections, blood disorders, ototoxicity, noise trauma, autoimmune disease, acoustic tumor, and even mental stress may be related to the disease. In cases of hearing loss as a result of psychogenic factors, early diagnosis and adequate treatment under collaboration with the psychiatric department are crucial, since failure to take appropriate measures may result in permanent sequela. We report a case, with a review of the literature, of sudden unilateral sensorineural hearing loss with contralateral psychogenic hearing loss induced by gunshot noise.

  16. Sudden Sensorineural Hearing Loss after Orthopedic Surgery under Combined Spinal and Epidural Anesthesia

    PubMed Central

    Vilhena, Ditza; Pereira, Luís; Duarte, Delfim; Oliveira, Nuno

    2016-01-01

    Postoperative hearing loss following nonotologic surgery is rare. For patients undergoing subarachnoid anesthesia, the loss of cerebral spinal fluid and hence the drop in intracranial pressure can result in hearing loss and cranial nerve palsy. We report a case in which a patient sustained orthopedic surgery under combined spinal and epidural anesthesia complicated by severe and persistent sensorineural hearing loss. This report is a reminder that postoperative sudden sensorineural hearing loss is a poorly understood complication. A high index of suspicion is required for early diagnosis of this complication, although prompt treatment does not guarantee a good outcome. PMID:26904339

  17. [The active middle ear implant for the rehabilitation of sensorineural, mixed and conductive hearing losses].

    PubMed

    Sprinzl, G M; Wolf-Magele, A; Schnabl, J; Koci, V

    2011-09-01

    Active middle ear implants, such as the Vibrant Soundbridge, are used as an important part in the rehabilitation of sensorineural, conductive hearing, or mixed hearing loss. The attachment of the Vibrant Soundbridge at the round window and the usage of the Vibroplasty couplers strongly expanded the application of the Vibrant Soundbridge.The Vibrant Soundbridge is developed for patients who have an intolerance to hearing aids and a moderate to profound sensorineural hearing loss. The VSB also provides an optimal solution for patients with failed middle ear reconstructions or patients with atresia. To capture the improvement with the VSB Implant with different hearing losses a literature analysis was conducted. The functional gain was analyzed for 107 patients with conductive hearing loss and for 214 patients with sensorineural hearing loss out of 14 studies.Patients with conductive and mixed hearing loss resulted in a functional gain from 30 to 58 dB with the VSB. Patients with a pure sensorineural hearing loss showed a functional gain of 23-30 dB. The VSB bone conduction threshold shift was analyzed for all studies conducted in the years between 2000 and 2009. In 11 of the 16 studies there was no significant (p=0.05) change found. In 5 studies, the pre- to post-surgical bone conduction threshold shift was less than 10 dB. None of these studies measured a threshold shift of more than 10 dB.The flexible attachment at either the long process of the incus with sensorineural hearing loss, with an conductive hearing loss at the round window or the use of Vibroplasty couplers at the oval window, head of the stapes or round window makes the VSB an extremely versatile instrument. If patients can't wear conventional hearing aids, had failed middle ear reconstructions or atresia the VSB presents, due to the significant hearing improvement in any type of hearing loss, an ideal solution.

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

    PubMed

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

    2014-01-29

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

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

    PubMed Central

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

    2016-01-01

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

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

    PubMed

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

    2013-12-01

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

  1. Isolated contralateral sudden sensorineural hearing loss: an unusual manifestation of pontine infarct.

    PubMed

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

    2014-09-01

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

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

    ERIC Educational Resources Information Center

    Walton, Joseph P.; Hendricks-Munoz, Karen

    1991-01-01

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

  3. Profound Bilateral Sensorineural Hearing Loss in Nigerian Children: Any Shift in Etiology?

    ERIC Educational Resources Information Center

    Dunmade, A. D.; Segun-Busari, S.; Olajide, T. G.; Ologe, F. E.

    2007-01-01

    Deafness, profound hearing loss, is a global problem. However, the causes of, attitudes toward, and management options for deafness differ considerably from region to region. This study seeks to identify the present causes of profound sensorineural hearing loss in Nigeria, which in our environment is almost synonymous to a life sentence of silence…

  4. Prognostic effect of hyperbaric oxygen therapy starting time for sudden sensorineural hearing loss.

    PubMed

    Yıldırım, Erol; Murat Özcan, K; Palalı, Mehmet; Cetin, Mehmet Ali; Ensari, Serdar; Dere, Hüseyin

    2015-01-01

    Sudden sensorineural hearing loss is one of the otological emergencies whose pathogenesis is uncertain and associated with total or partial loss of hearing function. The aim of this study was to investigate whether the hyperbaric oxygen therapy starting time affects the management of sudden sensorineural hearing loss. Fifty-nine patients with sudden sensorineural hearing loss admitted to our clinic between 2008 and 2012 were retrospectively included in this study. All patients received hyperbaric oxygen therapy. In addition, each patient received intravenous piracetam and 37 patients received steroid therapy. Hyperbaric oxygen therapy was initiated between 1 and 7 days with 20 patients determined as Group A, between 8 and 14 days with 25 patients determined as Group B and between 15 and 28 days with 14 patients determined as Group C. Hearing gains of these three groups were statistically evaluated. Each of them showed statistically significant improvement. Lowest hearing gain was observed in Group C and the gain of this group was statistically less than the other two groups. There was no significant difference between the hearing gains of the Group A and Group B. Starting hyperbaric oxygen therapy in patients with sudden sensorineural hearing loss within the first 14 days has positive effect on the prognosis of the disease.

  5. Prediction model for hearing outcome in patients with idiopathic sudden sensorineural hearing loss.

    PubMed

    Suzuki, Hideaki; Mori, Takanori; Hashida, Koichi; Shibata, Minori; Nguyen, Khac-Hung; Wakasugi, Tetsuro; Hohchi, Nobusuke

    2011-04-01

    The aim of this study is to develop a regression model for predicting hearing outcome in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). A total of 174 consecutive patients with ISSNHL (average of the hearing levels at 250, 500, 1,000, 2,000, and 4,000 Hz was ≥40 dB; time from onset to treatment was ≤30 days) were retrospectively analyzed. They received steroid administration (400 mg/day of hydrocortisone sodium succinate followed by tapered doses) in combination with hyperbaric oxygen therapy. The hearing improvement rate compared to the unaffected contralateral ear was calculated. Correlations between the hearing improvement rate and four prognostic factors (patient's age, days from onset to treatment, initial hearing level, and the presence of vertigo) were examined by simple and multiple regression analyses. In the simple regression analysis, significant correlations were observed between the hearing improvement rate and all four prognostic factors. In the multiple regression analysis, the correlation was significant for patient's age, days from onset to treatment, and the presence of vertigo with partial correlation coefficients of -0.221, -0.324, and -0.329, respectively, but was not significant for the initial hearing level. We subsequently formulated a multiple regression equation for predicting the hearing improvement rate. The multiple correlation coefficient was 0.495 with a p value of 1.42 × 10(-9). Using this regression model, the hearing improvement rate is still difficult to predict with 95% probability, but is predictable with 70% probability.

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

    PubMed

    Lee, Eun Jung; Yoon, Yong Joo

    2012-01-01

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

  7. Early-onset sensorineural hearing loss in Lassa fever.

    PubMed

    Ibekwe, T S; Okokhere, P O; Asogun, D; Blackie, F F; Nwegbu, M M; Wahab, K W; Omilabu, S A; Akpede, G O

    2011-02-01

    Lassa fever (LF) is a viral hemorrhagic disease which affects one-fourth to two million people annually with the fatality rate of about 10,000. It is associated with sensorineural hearing loss (SNHL) usually at the convalescent stage. Recently, cases of SNHL at the acute phase have been reported. This study was done to further investigate the incidence and features of SNHL in acute phase of LF. It is a prospective case-control study of LF patients seen with acute SNHL conducted between July 2007 and April 2009 at Irrua Specialist Teaching Hospital Nigeria. The diagnosis of acute LF was based on the clinical features and detection of IgM antibodies and/or positive Lassa virus-specific reverse transcriptase-polymerase chain reaction using primers S36+ and LVS 339 while SNHL was diagnosed clinically and confirmed with PTA and speech discrimination tests. Patients with other acute febrile illnesses were used as control. Statistical analysis was done using SPSS version 11 and Fisher's exact test while level of significance was set at p < 0.05. Out of the 37 confirmed cases of LF, 5 (13.5%) and none (0%) of the control developed early-onset SNHL (p = 0.03). Forty percent of the cases studied had negative IgM. The audiograms showed involvement at all frequency groups with pure tone average 65-85 dB and the speech discrimination 20-40%. The overall case fatality rate was 27.0%, and for early SNHL cases 60.0% (p > 0.05). The incidence of SNHL in LF infection is about 13.5% and could be a reflection of a worse disease process. There is possibility of direct viral invasion aside immunological reaction as a causative mechanism.

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

    PubMed

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

    2016-09-01

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

  9. Risk of sudden sensorineural hearing loss in stroke patients

    PubMed Central

    Kuo, Chin-Lung; Shiao, An-Suey; Wang, Shuu-Jiun; Chang, Wei-Pin; Lin, Yung-Yang

    2016-01-01

    Abstract Poststroke sudden sensorineural hearing loss (SSNHL) can hinder communication between patients and healthcare professionals, thereby restricting participation in rehabilitation programs and limiting improvements in physical performance. However, the relationship between stroke and SSNHL remains unclear. This study employed a nationwide population-based dataset to investigate the relationship between stroke and SSNHL. The Taiwan Longitudinal Health Insurance Database was used to compile data from 11,115 stroke patients and a comparison cohort of 33,345 matched nonstroke enrollees. Each patient was followed for 5 years to identify new-onset SSNHL. Stratified Cox proportional-hazard regression analysis was used to examine the association of stroke with subsequent SSNHL. Among the 44,460 patients, 66 patients (55,378 person-years) from the stroke cohort and 105 patients (166,586 person-years) from the comparison cohort were diagnosed with SSNHL. The incidence of SSNHL was approximately twice as high among stroke patients than among nonstroke patients (1.19 and 0.63/1000 person-years, respectively). Stroke patients had a 71% increased risk of SSNHL, compared with nonstroke patients (adjusted hazard ratio [HR] 1.71, 95% confidence interval [CI] 1.24–2.36). We also observed a remarkable increase in risk of SSNHL in stroke patients within 1-year of follow-up (adjusted HR 5.65, 95% CI 3.07–10.41) or under steroid therapy during hospitalization (adjusted HR 5.14, 95% CI 2.08–12.75). Patients with stroke had a higher risk of subsequent SSNHL compared with patients without stroke. In particular, stroke patients within 1-year follow-up and those undergoing steroid therapy during hospitalization should be treated with the utmost caution, considering that the risk of SSNHL increases by more than 5-fold. PMID:27603402

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

    PubMed

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

    2016-11-01

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

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

    PubMed

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

    2011-09-01

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

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

    PubMed

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

    2015-04-01

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

  13. Can Mild Bilateral Sensorineural Hearing Loss Affect Developmental Abilities in Younger School-Age Children?

    ERIC Educational Resources Information Center

    Ðokovic, Sanja; Gligorovic, Milica; Ostojic, Sanja; Dimic, Nadežda; Radic-Šestic, Marina; Slavnic, Svetlana

    2014-01-01

    The research study was conducted for the purpose of examining the influence of mild bilateral sensorineural hearing loss (MBSNHL) on developmental abilities of younger school-age children. The sample encompassed 144 children with MBSNHL, aged 7.5-11 (M = 8.85). MBSNHL (20-40 dB HL) was identified by pure tone audiometry. The control group…

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

    ERIC Educational Resources Information Center

    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…

  15. Some Non-Occupational Aspects of Sensorineural Loss of Hearing Acuity - A Proposed Design for Study.

    DTIC Science & Technology

    1985-06-01

    last twelve months only one person was retired from the U.S. Air Force solely due to hearing loss ( Caraway , Per- sonal interview, 1985). Sensorineural...Loss in Teenage Boys, Scandinavian Audiological Journal 1981; 10 (2): 91-6. Caraway , Rebecca, Captain, USAF, Personal Interview, Division of Medical

  16. Intratympanic steroid therapy for treatment of idiopathic sudden sensorineural hearing loss.

    PubMed

    Bear, Zachary W; Mikulec, Anthony A

    2014-01-01

    Idiopathic sudden sensorineural hearing loss (ISSHL) is the sudden loss of unilateral hearing of unknown etiology. The standard treatment consist of a high dose oral steroid taper. This article serves to review the current literature on intratympanic steroid injections for ISSHL. Current literature suggested intratympanic steroids are equivalent to oral steroid therapy, primary combined therapy is superior to either alone and intratympanic steroids should be offered for salvage therapy in ISSHL.

  17. Perception of dissonance by people with normal hearing and sensorineural hearing loss.

    PubMed

    Tufts, Jennifer B; Molis, Michelle R; Leek, Marjorie R

    2005-08-01

    The purpose of this study was to determine whether the perceived sensory dissonance of pairs of pure tones (PT dyads) or pairs of harmonic complex tones (HC dyads) is altered due to sensorineural hearing loss. Four normal-hearing (NH) and four hearing-impaired (HI) listeners judged the sensory dissonance of PT dyads geometrically centered at 500 and 2000 Hz, and of HC dyads with fundamental frequencies geometrically centered at 500 Hz. The frequency separation of the members of the dyads varied from 0 Hz to just over an octave. In addition, frequency selectivity was assessed at 500 and 2000 Hz for each listener. Maximum dissonance was perceived at frequency separations smaller than the auditory filter bandwidth for both groups of listners, but maximum dissonance for HI listeners occurred at a greater proportion of their bandwidths at 500 Hz than at 2000 Hz. Further, their auditory filter bandwidths at 500 Hz were significantly wider than those of the NH listeners. For both the PT and HC dyads, curves displaying dissonance as a function of frequency separation were more compressed for the HI listeners, possibly reflecting less contrast between their perceptions of consonance and dissonance compared with the NH listeners.

  18. Perception of dissonance by people with normal hearing and sensorineural hearing loss

    NASA Astrophysics Data System (ADS)

    Tufts, Jennifer B.; Molis, Michelle R.; Leek, Marjorie R.

    2005-08-01

    The purpose of this study was to determine whether the perceived sensory dissonance of pairs of pure tones (PT dyads) or pairs of harmonic complex tones (HC dyads) is altered due to sensorineural hearing loss. Four normal-hearing (NH) and four hearing-impaired (HI) listeners judged the sensory dissonance of PT dyads geometrically centered at 500 and 2000 Hz, and of HC dyads with fundamental frequencies geometrically centered at 500 Hz. The frequency separation of the members of the dyads varied from 0 Hz to just over an octave. In addition, frequency selectivity was assessed at 500 and 2000 Hz for each listener. Maximum dissonance was perceived at frequency separations smaller than the auditory filter bandwidth for both groups of listners, but maximum dissonance for HI listeners occurred at a greater proportion of their bandwidths at 500 Hz than at 2000 Hz. Further, their auditory filter bandwidths at 500 Hz were significantly wider than those of the NH listeners. For both the PT and HC dyads, curves displaying dissonance as a function of frequency separation were more compressed for the HI listeners, possibly reflecting less contrast between their perceptions of consonance and dissonance compared with the NH listeners.

  19. Intratympanic injection of dexamethasone for treatment of tinnitus in patients with sudden sensorineural hearing loss.

    PubMed

    Yoshida, Tadao; Teranishi, Masaaki; Iwata, Tomoyuki; Otake, Hironao; Nakashima, Tsutomu

    2012-01-09

    The purpose of this study is to test the effectiveness of intratympanic dexamethasone injections as a treatment for severe tinnitus in idiopathic sudden sensorineural hearing loss (SNHL). We studied 37 patients who received intratympanic dexamethasone injections and 14 control patients who did not receive it, with severe tinnitus after onset of unilateral sudden SNHL. Hearing level did not change during this study in any patient. The relationship between the duration of tinnitus and effectiveness of treatment was investigated in sudden SNHL. We used a visual analogue scale to evaluate 51 patients with severe tinnitus at the stage of stable hearing level after idiopathic sudden sensorineural hearing loss. Forty-one per cent of patients showed significant improvement after treatment. The average period between onset of sudden sensorineural hearing loss and initiation of intratympanic dexamethasone injection was significantly shorter (207 days) in the improved group than in the unchanged group (482 days) (P<0.001). In control group, one of 14 patients presented significant improvement spontaneously. Intratympanic dexamethasone treatment may be effective in treatment of severe tinnitus after sudden SNHL at the stage of stable hearing level, and the shorter the period from onset of sudden deafness to the start of intratympanic dexamethasone treatment, the greater the improvement in tinnitus that can be expected.

  20. Intratympanic Steroid Treatment for Idiopathic Sudden Sensorineural Hearing Loss after Failure of Intravenous Therapy

    PubMed Central

    Ferri, Emanuele; Frisina, Antonio; Fasson, Anna Chiara; Armato, Enrico; Spinato, Giacomo; Amadori, Maurizio

    2012-01-01

    Purpose. The aim of this study is the investigation of the effectiveness of intratympanic steroids therapy (IST) in patients with idiopathic sudden sensorineural hearing loss (ISSHL) who had not responded to intravenous treatment, evaluating the overall hearing recovery and comparing the results with different variables. Materials and Methods. Our study consisted of 55 patients with refractory ISSHL who, at the end of 10 days of therapy with intravenous steroids, had puretone 4-frequency average (PTA) of worse than 30 dB. The patients received 0.5 mL of methylprednisolone by direct intratympanic injection. The procedure was carried out up to 7 times within a 20-days period. Statistical analysis was carried out. Results. Overall 29 patients (52.7%) showed improvement in PTA, 24 (43.8%) had no change in hearing, and 2 (3.5%) worsened. There was a significant statistical correlation between hearing recovery and time to onset of symptoms, severity of hearing loss and frequency of hearing loss. Conclusions. IST is an effective and safe therapy in sudden sensorineural hearing loss cases that are refractory to standard treatment. The earlier IST, the hearing losses less than 90 dB and the involvement of the low frequencies seem to influence positively the hearing recovery. PMID:23724270

  1. [Clinical diagnose and significance of congenital sensorineural hearing loss combined with BPES].

    PubMed

    Ao, Liying; Liu, Yongzhi

    2015-09-01

    To analyze congenital sensorineural hearing loss combined with blepharophimosis-ptosis-epicanthus inversus syndrome (BPES). For the case of cochlear implantation to child with congenital sensorineural deafness combined BPES, accomplish routine examination and assessment, combining with literature to analyze the clinical diagnosis of this disease and its significance. Sensorineural hearing loss is a common congenital diseases with neonatal incidence of 1 per thousand - 3 per thousand, 50%-70% of deafness is associated with genetic factors, the incidence of congenital sensorineural hearing loss combined with eye disease is about 40%-60%, mainly reflected in ametropia and retinopathy. BPES's main clinical manifestations is blepharophimosis, ptosis, epicanthus inversus, and telecanthus. BPES is a rare autosomal dominant disease caused by FOXL 2 gene mutation, sometimes associated with retarded growth, delayed development, congenital heart disease, and microcephaly. Suffering from both sensorineural hearing loss and BPES is rare in reported literature. This case is diagnosed by clinical examination, without visual impairment. Facial nerve dysplasia has been found during the surgery. For congenital deafness patients with eye disease or other diseases, timely and correct diagnosis has important clinical significance, which can improve the diagnostic rate and make it coming true to early intervention, and then, effectively improve the quality of the patients. There are few literature reports, of patients with two kinds of genetic diseases. Our inference is that the cases are rare or the patients has visited different departments and ignored the other systems' signs. Therefore, in such doubtful cases, we should do the professional comprehensive examination in daily clinical work in order to avoid missed diagnosis or delayed treatment and intervention. By analyzing this case, the patient may also suffer from facial nerve dysplasia. Preoperatively viewing CT scan and

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

    PubMed Central

    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

    2015-01-01

    ABSTRACT 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. IMPORTANCE 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. PMID:26719273

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

    PubMed Central

    Jalilvand, Hamid; Pourbakht, Akram; Jalaee, Shohreh

    2015-01-01

    Background: When fitting hearing aid as a compensatory device for an impaired cochlea in a patient with sensorineural hearing loss (HL), it is needed to the effective and efficient frequency response would be selected regarding providing the patient's perfect speech perception. There is not any research about the effects of frequency modifications on speech perception in patients with HL regarding the cochlear desensitization. The effect (s) of modifications in frequency response of hearing aid amplification on the results of acceptable noise level (ANL) test is the main aim of this study. Materials and Methods: The amounts of ANL in two conditions of linear amplification (high frequency emphasis [HFE] and mid frequency emphasis [MFE]) were measured. Thirty-two male subjects who participated in this study had the moderate to severe sensorineural HL. Results: There was not any significant difference between ANL in linear amplification of hearing aid with HFE frequency response and ANL in linear amplification of hearing aid with MFE frequency response. Conclusion: The gain modification of frequency response not only does not affect the patient's performance of speech intelligibility in ANL test. This indicates that we need to note to the cochlear desensitization phenomenon when fitting hearing aid as a compensatory device for an impaired cochlea in a patient. The cochlear desensitization has not been considered properly in hearing aid fitting formula which is needed to be explored more about the bio-mechanisms of impaired cochlea. PMID:26918238

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

    SciTech Connect

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

    1994-10-01

    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.

  5. Effects of stimulus material on the dichotic listening performance of patients with sensorineural hearing loss.

    PubMed

    Speaks, C; Niccum, N; Van Tasell, D

    1985-03-01

    Dichotic listening scores were obtained from 27 patients with sensorineural hearing loss in response to four dichotic speech tests: digits, vowel words, consonant words, and CV nonsense syllables. Monotic performance-intensity functions for each ear were defined with the CV syllables, and the four dichotic tests were administered at an intensity that produced asymptotic monotic performance for both ears with the CV syllables. Four blocks of 30 pairs of items per block were presented for each dichotic test, which produced 240 responses per test for each patient. Reliable differences among left-ear scores, right-ear scores, performance level, and the ear advantage were observed among the four tests. The digit test appeared to be most promising for assessing central auditory function when the patient had a sensorineural hearing loss because performance for the digits was only slightly affected by the peripheral loss.

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

    PubMed

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

    2015-08-01

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

  7. Risk factors for sensorineural hearing loss in children.

    PubMed

    Núñez-Batalla, Faustino; Trinidad-Ramos, Germán; Sequí-Canet, José Miguel; Alzina De Aguilar, Valentín; Jáudenes-Casaubón, Carmen

    2012-01-01

    In the last decade, tremendous progress has been made very rapidly in the development of Early Hearing Detection and Intervention (EHDI) systems as a major public health initiative. The percentage of infants screened annually in Spain has increased significantly since the EHDI systems have expanded to all autonomic regions. Historically, high risk indicators have been used for the identification of infants who should receive audiological evaluation but who live in geographic locations where universal hearing screening is not yet available, to help identify infants who pass neonatal screening but are at risk of developing delayed-onset hearing loss and to identify infants who may have passed neonatal screening but have mild forms of permanent hearing loss. In this review, the standard risk factors for hearing loss are analysed and the risk factors known to be associated with late onset or progressive hearing loss are identified. The recommendation for infants with a risk factor that may be considered as low risk is to perform at least one audiology assessment by 24-30 months. In contrast, for an infant with risk factors known to be associated with late onset or progressive hearing loss (such as cytomegalovirus infection or family history), early and more frequent assessment is appropriate. All infants should have an objective standardised screening of global development with a validated assessment tool at 9, 18 and 24-30 months of age or at any time if the health care professional or the family is concerned.

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

    PubMed

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

    2016-10-01

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

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

    PubMed Central

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

    2016-01-01

    We describe an imported case of Lassa fever with both encephalopathy and bilateral sensorineural hearing deficit. Absence of fever during hospitalization, initially nonspecific symptoms, and onset of hearing deficit in a late stage of disease probably contributed to delayed diagnosis (14 days after admittance to hospital). The pathogenesis of neurological manifestations of Lassa fever is poorly understood and no specific treatment was given. A total of 118 personnel had close contact with the patient, but no secondary cases occurred. This case highlights the importance of considering Lassa fever as a differential diagnosis in patients with recent travel to endemic areas. PMID:27975074

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

    PubMed

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

    2016-10-01

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

  11. [The revised version of the german guidelines "sudden idiopathic sensorineural hearing loss"].

    PubMed

    Michel, O

    2011-05-01

    The new revised version (expires 2012) for sudden, unilateral, sensorineural hearing loss stresses the urgence but not the emergency of diagnostics and therapy in this inner ear disfunction with still increasing incidence. Minimum diagnostics should comprise ENT examination with earmicroscopy, pure tone and tuning fork hearing tests, tympano- and vestibulometry. Classification by frequency loss seems of interest in regard to different underlying pathology, which is still obscure. Therefore from experience glucocorticoids and rheological therapy are recommended in the light of some favoring recent studies. The intratympanic appliCation of glucokorticoids is considered as spare therapy.

  12. Hyperbaric oxygen therapy in sudden sensorineural hearing loss following spinal anesthesia: case reports.

    PubMed

    Carneiro, Sandra N; Guerreiro, Ditza V; Cunha, Anita M; Camacho, Óscar F; Aguiar, Isabel C

    2016-01-01

    The management of sudden sensorineural hearing loss following spinal anesthesia is currently an open problem. Several strategies have been used with variable results and, to the best of our knowledge, there are no prior accounts in the literature on the use of hyperbaric oxygen therapy in the treatment of this complication. We report two cases of acute onset of unilateral hearing loss after spinal anesthesia, with significantly improved results after hyperbaric oxygen therapy. A hypothesis on the possible mechanism behind this complication is discussed. A relation is established between hyperbaric oxygen therapy and this hypothetical mechanism, in order to explain successful results in the reported cases.

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

    SciTech Connect

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

    1991-08-01

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

  14. Modeling the Effects of Sensorineural Hearing Loss on Sound Localization in the Median Plane

    PubMed Central

    Majdak, Piotr; Laback, Bernhard

    2016-01-01

    Listeners use monaural spectral cues to localize sound sources in sagittal planes (along the up-down and front-back directions). How sensorineural hearing loss affects the salience of monaural spectral cues is unclear. To simulate the effects of outer-hair-cell (OHC) dysfunction and the contribution of different auditory-nerve fiber types on localization performance, we incorporated a nonlinear model of the auditory periphery into a model of sagittal-plane sound localization for normal-hearing listeners. The localization model was first evaluated in its ability to predict the effects of spectral cue modifications for normal-hearing listeners. Then, we used it to simulate various degrees of OHC dysfunction applied to different types of auditory-nerve fibers. Predicted localization performance was hardly affected by mild OHC dysfunction but was strongly degraded in conditions involving severe and complete OHC dysfunction. These predictions resemble the usually observed degradation in localization performance induced by sensorineural hearing loss. Predicted localization performance was best when preserving fibers with medium spontaneous rates, which is particularly important in view of noise-induced hearing loss associated with degeneration of this fiber type. On average across listeners, predicted localization performance was strongly related to level discrimination sensitivity of auditory-nerve fibers, indicating an essential role of this coding property for localization accuracy in sagittal planes. PMID:27659486

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

    PubMed

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

    2015-09-14

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

  16. Traumatic Labyrinthine Concussion in a Patient with Sensorineural Hearing Loss

    PubMed Central

    Chiaramonte, R.; Bonfiglio, M.; D'Amore, A.; Viglianesi, A.; Cavallaro, T.; Chiaramonte, I.

    2013-01-01

    Blunt head trauma without any temporal bone fracture or longitudinal temporal bone fracture, with an associated fracture of the labyrinth may cause labyrinthine injury with sensor neural hearing loss and vertigo because of a concussive injury to the membranous labyrinth. Sudden sensory neural hearing loss is relatively frequent. In most cases, the etiology is not discovered. One of the possible causes for sudden deafness is inner labyrinth bleeding or concussion, which were difficult to diagnose before the advent of magnetic resonance imaging. Vertigo without a demonstrable fracture may also be the result of labyrinthine concussion, cupololithiasis and perilymphatic fistula. We describe the clinical case of a patient with acute traumatic hearing loss and vertigo, without skull base fracture detected on computed tomography. Magnetic resonance study was also performed. We have integrated the discussion with features that allow the differential diagnosis from other similar conditions. PMID:23859168

  17. Smartphone-based hearing test as an aid in the initial evaluation of unilateral sudden sensorineural hearing loss.

    PubMed

    Handzel, Ophir; Ben-Ari, Oded; Damian, Doris; Priel, Maayan M; Cohen, Jacob; Himmelfarb, Mordechai

    2013-01-01

    Sudden sensorineural hearing loss (SSNHL) can cause significant morbidity. Treatment with steroids can improve outcome. Delay in initiation of treatment reduces the chance to regain hearing. For this reason SSNHL is considered an emergency. Diagnosis is based on history, physical examination and a standard audiogram, the latter requiring specialized equipment and personnel. Standard audiogram may not be available at the time and place of patient presentation. A smartphone or tablet computer-based hearing test may aid in the decision to prescribe steroids in this setting. In this study the uHear™ hearing test application was utilized. The output of this ear-level air conduction hearing test is reported in hearing grades for 6 frequencies ranging from 250 to 6000 Hz. A total of 32 patients with unilateral SSNHL proven by a standard audiogram were tested. The results of standard and iPod hearing tests were compared. Based on the accepted criterion of SSNHL (at least 30 dB loss - or 2 hearing grades - in 3 consecutive frequencies) the test had a sensitivity of 0.76 and specificity of 0.91. Using a less stringent criterion of a loss of 2 hearing grades over at least 2 frequencies the sensitivity was 0.96 and specificity 0.86. The correlation coefficient for the comparison of the average hearing grade across the 6 measured frequencies of the study and standard audiogram was 0.83. uHear more accurately reflected hearing thresholds at mid and high tones. Similarly to previously published data, low frequency thresholds could be artificially elevated. In conclusion, uHear can be useful in the initial evaluation of patients with single-sided SSNHL by providing important information guiding the decision to initiate treatment before a standard audiogram is available.

  18. Congenital inner ear malformations without sensorineural hearing loss in children.

    PubMed

    Ozeki, Michio; Kato, Zenichiro; Sasai, Hideo; Kubota, Kazuo; Funato, Michinori; Orii, Kenji; Kaneko, Hideo; Fukao, Toshiyuki; Kondo, Naomi

    2009-10-01

    Inner ear malformations are frequently found in patients with congenital hearing loss. It has been reported that normal hearing is rare in patients with severe inner ear vestibular malformations. A 9-year-old boy had had complained of recurrent dizziness and disequilibrium for 2 months. Clinical and neuro-otological examinations showed peripheral involvement of the vestibular system, while audiological investigation was normal. High-resolution magnetic resonance imaging, with three-dimensional reconstruction, showed dysplasia of the bilateral lateral semicircular canals (LSCCs). Isolated vestibular malformation might not be as rare as previously thought, and should be examined by imaging of the temporal bone.

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

    DTIC Science & Technology

    2016-07-05

    distribution is unlimited. UNCLASSIFIED Title: Systemic polyarteritis nodosa as the cause of sudden-onset bilateral sensorineural hearing loss...tissues from infected primates revealed pathology consistent with systemic polyarterisis nodosa (PAN), an autoimmune disorder associated with sudden...etiology for LASV-associated sudden-onset hearing loss. One Sentence Summary: Systemic polyarteritis nodosa is the proposed underlying pathology

  20. [The algorithm for the medical maintenance of the aircraft personnel suffering from chronic sensorineural impairment of hearing].

    PubMed

    Pankova, V B; Skryabina, L Yu; Barkhatova, O A

    2016-01-01

    The present study was designed to systematize the causes underlying the development of chronic sensorineural impairment of hearing in the aircraft personnel engaged in commercial aviation of the Russian Federation. A detailed clinical and audiological picture of chronic sensorineural loss of hearing in the aircraft personnel is presented with special reference to the criteria accepted in the civil aviationfor the evaluation of professional suitability and occupational selection in terms of hearing conditions. The study has demonstrated the paramount importance of the aviation medical expertise for the flight safety control in civil aviation. We analyzed the results of the audiological examination of the aircraft personnel suffering from chronic sensorineural impairment of hearing and proposed the algorithm for the rehabilitation of such subjects taking into consideration the stage of the chronic process.

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

    ERIC Educational Resources Information Center

    Backous, Douglas D.; Niparko, John K.

    1997-01-01

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

  2. Value of genetic testing in the otological approach for sensorineural hearing loss.

    PubMed

    Matsunaga, Tatsuo

    2009-12-01

    Sensorineural hearing loss (SNHL) is one of the most common disabilities in human, and genetics is an important aspect for SNHL, especially in children. In recent 10 years, our knowledge in genetic causes of SNHL has made a significant advance, and now it is used for diagnosis and other clinical practices. Hereditary hearing loss can be classified into syndromic and nonsyndromic hearing loss. As the nonsyndromic deafness genes, more than 100 loci for deafness genes have been determined, and more than 40 genes were identified. Furthermore, more than 300 forms of syndromic hearing loss have been characterized, and each syndrome may have several causative genes. In childhood hearing loss, early educational intervention is required in addition to medical intervention for normal development of speech and language. In addition, even severe to profound hearing loss may be restored very effectively by hearing aids or cochlear implants. Because of these features of SNHL, genetic testing has exceptionally high value in the medical practice for hereditary hearing loss. Several strategies are used for genetic testing of SNHL for accurate and efficient identification of the genetic causes, and the results were used for explanation of the cause, prediction of auditory features, prevention of deafness, management of associated symptoms, determination of therapy, and genetic counseling. Identification of damaged cells in the inner ear and the underlying mechanism by genetic testing undoubtedly facilitates development and introduction of novel and specific therapies to distinct types of SNHL.

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

    PubMed Central

    2016-01-01

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

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

    PubMed

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

    2012-04-01

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

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

    PubMed

    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.

  6. Molecular and hereditary mechanisms of sensorineural hearing loss with focus on selected endocrinopathies.

    PubMed

    Masindova, I; Varga, L; Stanik, J; Valentinova, L; Profant, M; Klimes, I; Gasperikova, D

    2012-07-01

    Hearing loss is one of the most widespread sensory disorders. The incidence of deafness in general population is 1:1000 newborns. About one half of the cases of the congenital sensorineural hearing loss (SNHL) is inherited. Recessive mutations in the gap junction beta 2 (GJB2) gene are the most common genetic causes of the nonsyndromic SNHL. The GJB2 encodes a protein connexin 26 which forms a subunit of gap junction essential for the correct function of the inner ear. The syndromic SNHL is associated with a wide range of other symptoms, which encompass also dysfunctions of endocrine organs. The Pendred syndrome associated with the hearing impairment is characterized by a prelingual, bilateral sever to profound SNHL, goiter, and iodine organification defect. It is an autosomal recessive disorder, which develops due to mutations in pendrin, an anion channel encoded by SLC26A4 gene. Another important type of syndromic hearing loss is the Maternally Inherited Diabetes and Deafness syndrome, which is caused by several mitochondrial DNA mutations. These mutations are clinically manifested by a hearing impairment with development of the diabetes in the adult age. Hearing impairment occurs during puberty when sensation of high frequency tones is affected following with further progress to profound bilateral sensorineural hearing impairment in the whole frequency range. This review deals with the molecular mechanisms of common genetic causes of the hereditary SNHL along with the selected endocrinopathies emphasizing that the DNA analyses along with the functional studies significantly contribute to the early SNHL diagnosis followed by personalized therapy and genetic counseling.

  7. Muckle-Wells syndrome: a treatable cause of congenital sensorineural hearing loss.

    PubMed

    Stew, B T; Fishpool, S J C; Owens, D; Quine, S

    2013-01-01

    Muckle-Wells syndrome (MWS) is a rare autosomal dominant condition with variable expression. It is a subset of auto-inflammatory diseases characterised by recurrent inflammatory crises and is associated with chronic recurrent urticaria, sensorineural deafness, periodic arthritis and secondary amyloidosis. The diagnosis of MWS is a clinical one with sufferers classically presenting in childhood with a moderate fever and non-pruiginous urticaria. We describe a case of a six-year-old girl who was successfully diagnosed and treated with Anakinra. Muckle and Wells originally described this syndrome in 1962; however, only recently was it discovered to be genetically linked to chromosome 1q44 and subsequently to missense mutations in the CIAS1/NALP3/PYPAF1 gene. Since then, treatment has evolved and it remains one of few treatable causes of congenital profound sensorineural hearing loss.

  8. Multicenter audiometric results with the Vibrant Soundbridge, a semi-implantable hearing device for sensorineural hearing impairment.

    PubMed

    Snik, A F; Mylanus, E A; Cremers, C W; Dillier, N; Fisch, U; Gnadeberg, D; Lenarz, T; Mazolli, M; Babighian, G; Uziel, A S; Cooper, H R; O'Connor, A F; Fraysse, B; Charachon, R; Shehata-Dieler, W E

    2001-04-01

    The Vibrant Soundbridge, a semi-implantable hearing device for subjects with moderate to severe sensorineural hearing impairment was introduced commercially. First audiologic results are presented on 63 patients from 10 European implant centers. Hearing loss was at 0.5, 1, 2, and 4 kHz varying between 43 and 81 dB HL. The patients used the analogue audio processor, type 302. Measured sound-field gain was compared with NAL-R target values. For most patients an acceptable agreement was found. There was a subgroup of patients, however, with relatively low gain. The results suggest that this was related to the suboptimal positioning and fixation of the transducer to the incus.

  9. Congenital inner ear malformations without sensorineural hearing loss.

    PubMed

    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.

  10. [Hereditary sensorineural hearing impairment and macrothrombocytopenia: a rare MYH9 gene mutation].

    PubMed

    Böttcher, A; Knecht, R; Busch, C-J; Lörincz, B B; Dalchow, C V

    2013-02-01

    We report on a rare case of an exon 16 mutation of the MYH9 gene in a 23-year-old woman. This gene encodes for non-muscular myosin IIA, which acts as a cytoskeletal contractile protein in diverse cell types. This disorder led to sensorineural hearing loss, macrothrombocytopenia, and proteinuria. MYH9 gene mutation can lead to diverse organ manifestation like pre-senile cataract or renal failure which are progressive in course. Due to the current lack of causal treatment, diagnostic steps, advice for follow-up examinations and symptomatic therapy approaches are presented.

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

    PubMed Central

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

    2017-01-01

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

  12. Hereditary gingival fibromatosis and sensorineural hearing loss in a 42-year-old man with Jones syndrome.

    PubMed

    Kasaboğlu, O; Tümer, C; Balci, S

    2004-01-01

    Hereditary gingival fibromatosis and sensorineural hearing loss in a 42-year-old man with Jones syndrome: Gingival fibromatosis is a rare disease, which can be seen as an isolated condition or associated with some uncommon syndromes. This case report describes the evaluation and treatment of a 42-year-old male patient with hereditary gingival fibromatosis, sensorineural hearing loss, undescended testis and maxillary odontogenic cyst (Jones Syndrome). Six years follow up of the index patient after the surgery revealed no recurrence of the gingival fibromatosis. This report also describes the anamnestic data of the patient's family that showed progressive deafness and gingival enlargement in three generations.

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

    PubMed Central

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

    2016-01-01

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

  14. Does Herpes Virus Reactivation Affect Prognosis in Idiopathic Sudden Sensorineural Hearing Loss?

    PubMed Central

    Park, Sang Man; Han, Chun; Lee, Jae Woo; Kong, Tae Hoon; Seo, Young Joon

    2017-01-01

    Objectives Several etiologies have been proposed to underlie idiopathic sudden sensorineural hearing loss (ISSNHL), including viral infection, vascular disturbance, and immune-mediated mechanisms. However, none of these mechanisms are conclusive. Should ISSNHL be caused by reactivation of a viral infection, antiviral treatment would be an important option. Thus, in this study, we reported the prognosis according to serologic test results of herpes viruses in patients with ISSNHL. We also evaluated treatment response with acyclovir and corticosteroids versus corticosteroids alone in herpes simplex virus (HSV) seropositive ISSNHL patients. Methods We compared hematologic examinations and the results of audiometry testing in 232 patients with ISSNHL. Statistical analyses for initial hearing impairment, progression of hearing impairment, recovery of hearing loss, and laboratory results were performed in all patients. All statistical analyses were performed using SPSS software. Results The 232 ISSNHL patients were divided into two subgroups according to HSV immunoglobulin M (IgM) serologic results (seropositive or seronegative). When the seropositive group was compared to the seronegative group, age, gender, body mass index (BMI), white blood cell, absolute neutrophil count, absolute monocyte count, and platelet count were not significantly different. Initial hearing level, final recovery hearing level, and recovery time were also not significantly different between the two groups (P>0.05). Though there were no significant differences in age, gender, BMI, or viral HSV IgM titer, the two groups had similar initial hearing level, final recovery hearing level, and recovery time. The difference in hearing threshold before and after treatment was larger (18.7±37.1) in the group receiving antiviral medication than in the corticosteroids group (11.0±44.5), but this difference was not significant (P=0.619). Conclusion There was no significant difference of prognosis between

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

    PubMed

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

    2016-05-01

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

  16. Measurements of normal inner ear on computed tomography in children with congenital sensorineural hearing loss.

    PubMed

    Lan, Ming-Ying; Shiao, Jiun-Yih; Ho, Ching-Yin; Hung, Hao-Chun

    2009-09-01

    The objective of this study is to use standardized measurements of the inner ear to see whether there are subtle bony malformations in children with congenital sensorineural hearing loss (SNHL) whose temporal bone computed tomography (CT) are grossly normal. The study includes 45 ears with congenital SNHL and grossly normal temporal bone CT scans and 45 ears with normal inner ear structures and normal hearing. Standardized measurements of the inner ear structures were made on axial temporal bone CT scans. Student's t test was performed to compare the measurements of the two groups. There were significant differences in the measurements of the bony island width of the superior semicircular canal, bony island width of the lateral semicircular canal and maximal height of cochlea between two groups (P < 0.05). In conclusion, standardized measurements of bony labyrinth of inner ear on temporal bone CT can identify subtle abnormalities of inner ear in patients with congenital SNHL having grossly normal radiological images.

  17. Unilateral sensorineural hearing loss and facial nerve paralysis associated with low-voltage electrical shock.

    PubMed

    Ozkiris, Mahmut

    2014-02-01

    Electrical injuries can occur as a result of contact with low- or high-voltage electricity. Low-voltage injuries are more common, as they usually occur in the home, but reports in the literature are few. After exposure to electric current, almost every organ system in the body is affected. The severity of an injury depends on many factors, including the type of current, the duration of exposure, and the resistance of the tissue involved. Reported cases of hearing loss and facial nerve paralysis associated with low-voltage electrical shock are rare, and minimal information is available about this circumstance. In this article, the author describes a case of low-voltage electrical shock in a 20-year-old man. To the best of the author's knowledge, this is the first report in the literature of a resolution of unilateral sensorineural hearing loss and facial nerve paralysis caused by a low-voltage electrical shock.

  18. Acupuncture as a primary and independent treatment in the acute phases of sudden sensorineural hearing loss

    PubMed Central

    Jin, Yuanyuan; Lu, Ming

    2016-01-01

    Abstract Sudden sensorineural hearing loss (SSHL) is an otological emergency defined as a rapid hearing loss, seriously affects patient's social life. To data, no study has reported the treatment by acupuncture alone in the acute phase. In this report, Acupuncture and Moxibustion therapy of excitation-focus transfer is outlined. The patient was a 26-year-old young woman who had an SSHL coupled with ear fullness. The patient had no past medical history, but she had undergone variable emotions and had a history of excessive noise exposure. The patient refused to receive any medicine especially steroids and hyperbaric oxygen therapy. She just only received acupuncture treatment. Her symptoms and outcome measurements were improved every week and completely recovered after the last week. Even though the article presents a single case and is based on self-reports, there are very clear trends on how patients with SSHL responded to acupuncture treatments. PMID:27368045

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

    PubMed

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

    2016-01-28

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

  20. Clinical Features and Prognosis of Sudden Sensorineural Hearing Loss Secondary to Intralabyrinthine Hemorrhage

    PubMed Central

    Lee, Jae Woo; Park, Yoon Ah; Park, Sang Man; Kong, Tae Hoon; Park, Sang Yoo; Bong, Jeong Pyo; Park, Dong-Joon

    2016-01-01

    Background and Objectives A number of etiologies of idiopathic sudden sensorineural hearing loss (ISSNHL) have been proposed, including viral infection, vascular disturbance, and immune-mediated mechanisms. Intralabyrinthine hemorrhage (ILH) as a cause of SSNHL is extremely rare, and there have been no studies defining the characteristics of hearing impairment and prognosis in patients with ISSNHL due to ILH. This study aimed to investigate the difference in impaired hearing patterns and prognosis for hearing recovery between patients with ISSNHL due to ILH confirmed by magnetic resonance imaging (MRI) and sex- and age-matched patients with ISSNHL due to causes other than ILH. Subjects and Methods We compared the results of audiometry and MRI in 12 patients who had ILH on MRI (hemorrhage group) and in 23 sex- and age-matched controls without abnormal findings related to their hearing loss on MRI (non-hemorrhage group). Initial hearing impairment, progression, and recovery of hearing loss were compared between the two groups. Results A majority of patients (92%) in the hemorrhage group complained of dizziness. Initial hearing impairment was more frequent in the hemorrhage group than in the non-hemorrhage group (94.09±35.9 vs. 66.66±30.1, p-value=0.036). The final recovery threshold in the hemorrhage group was worse (78.19±46.26 vs. 37.17±31.96, p-value=0.014) than that in the non-hemorrhage group. In the hemorrhage group, hearing recovery seemed to occur less often at high frequencies (2,000, 4,000, and 8,000 Hz) than at low frequencies (250, 500, and 1,000 Hz). Conclusions The presence of ILH was associated with poor hearing prognosis and the occurrence of vertigo. The abrupt onset of hearing loss associated with vertigo and the presence of hyperresonance on fat-suppressed T1-weighted MRI images of labyrinthic fluid strongly suggests acute intralabyrinthine hemorrhage, and is predictive of considerable hearing impairment and poor prognosis. PMID:27144231

  1. Changes in the default mode networks of individuals with long-term unilateral sensorineural hearing loss.

    PubMed

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

    2015-01-29

    Hearing impairment contributes to cognitive dysfunction. Previous studies have found changes of functional connectivity in the default mode network (DMN) associated with cognitive processing in individuals with sensorineural hearing loss (SNHL). Whereas the changes in the DMN in patients with long-term unilateral SNHL (USNHL) is still not entirely clear. In this work, we analyzed resting-state functional magnetic resonance imaging (fMRI) data and neuropsychological test scores from normal hearing subjects (n = 11) and patients (n = 21) with long-term USNHL. Functional connectivity and nodal topological properties were computed for every brain region in the DMN. Analysis of covariance (ANCOVA) and post hoc analyses were conducted to identify differences between normal controls and patients for each measure. Results indicated that the left USNHL presented enhanced connectivity (p < 0.05, false discovery rate (FDR) corrected), and significant changes (p < 0.05, Bonferroni corrected) of the nodal topological properties in the DMN compared with the control. More changes in the DMN have been found in the left than right long-term USNHL (RUSNHL). However, the neuropsychological tests did not show significant differences between the USNHL and the control. These findings suggest that long-term USNHL contributes to changes in the DMN, and these changes might affect cognitive abilities in patients with long-term USNHL. Left hearing loss affects the DMN more than the right hearing loss does. The fMRI measures might be more sensitive for observing cognitive changes in patients with hearing loss than clinical neuropsychological tests. This study provides some insights into the mechanisms of the association between hearing loss and cognitive function.

  2. Short-Term Outcomes of Acute Low-Tone Sensorineural Hearing Loss According to Treatment Modality

    PubMed Central

    Chang, Jinkyung; Yum, Gunhwee; Im, Ha-Young; Jung, Jong Yoon; Rah, Yoon Chan

    2016-01-01

    Background and Objectives We compared improvements in hearing thresholds in acute low-tone sensorineural hearing loss (ALHL) patients after two different treatments: steroid alone and steroid and diuretic combined. We analyzed how the duration between the onset of symptoms and the initiation of treatment affected hearing loss improvement and investigated the relation between presence of vertigo in ALHL patients and ALHL progression to Ménière's disease (MD). Subjects and Methods We retrospectively analyzed the medical records of 47 ALHL patients aged 21 to 76 years. Patients received either orally administered steroid alone (n=12) or steroid and diuretic combined (n=35). We compared improvements in the two groups' hearing thresholds at three lower frequencies (125, 250, and 500 Hz) after participants had received one month of each respective treatment. Results Our two treatments did not show any statistical difference in hearing loss improvement after one month. Forty percent of ALHL patients with vertigo developed MD, which was a significantly higher rate than the 12.5% of ALHL patients without vertigo who developed MD. The shorter duration between the onset of symptoms and the initiation of treatment significantly increased improvement in the sum of lower frequency hearing threshold after one month. Conclusions The current study suggests that steroid and diuretic administered together and steroid alone similarly improve the hearing threshold in ALHL patients after one month. We concluded that patients should initiate ALHL treatment as soon as they experience symptoms. ALHL patients should also be notified of their higher risk of developing MD. PMID:27144234

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

    ERIC Educational Resources Information Center

    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…

  4. Sudden sensorineural hearing loss associated with internal carotid artery pseudoaneurysm: causal or incidental?

    PubMed

    Palma, Silvia; Soloperto, Davide; Casoni, Federica; Rovati, Raffaella; Galassi, Giuliana

    2013-03-01

    Sudden deafness is acute onset of impaired hearing which develops within hours to few days. The commonly accepted audiometric criterion is a decrease in hearing of ≥ 30 dB, affecting at least three consecutive frequencies. Hearing loss is thought to involve several causative factors, including internal ear circulatory disturbances. We report the case of a female with an internal carotid artery (ICA) pseudoaneurysm in the distal cervical tract and unilateral sudden sensorineural hearing loss (SSNHL). As putative risk vascular factor, the patient had history of migraine since youth. Extensive screenings for autoimmune, rheumatic diseases, virological, and microbiological infections were negative. The patient denied recent cervical trauma. Furosemide and oral prednisone were given with initial benefit and withdrawn in 3 weeks. The patient experienced short-lasting episodes of headache, tinnitus, vertigo. Five weeks after first onset, she underwent magnetic resonance imaging (MRI) angiogram which revealed fusiform dilatation of left ICA in the cervical tract. It can be proposed, but it remains to be proved, that the pseudoaneurysm of the cervical ICA plays a role in the patient SSNHL in relation to turbulent flow or thromboembolism of branches to the inner ear.

  5. Sensorineural hearing loss in patients with sickle cell anaemia in Kenya.

    PubMed

    Tsibulevskaya, G; Oburra, H; Aluoch, J R

    1996-07-01

    The auditory function of sixty two Kenyan sickle cell anaemia patients aged seven to thirty years was compared to age-matched fifty five healthy controls with haemoglobin AA. Sensorineural hearing loss of 30 db and above was demonstrated in 25 (40%) of sickle cell anaemia patients and in three (5.5%) control subjects. Both sexes were equally affected. Bilateral lesion registered in 16%. Hearing threshold level was normal in 97% of the sickle cell group. High frequencies were commonly affected with hearing loss of 30-40 db. Two cases with severe unilateral deafness at all frequencies had severe recruitment suggestive of cochlea lesion. There were no cases of acoustic reflex decay in all study patients. The hearing loss was of slow onset. The high risk of deafness in Kenyan sickle cell anaemia patients may be a reflection of the severe course of the disease due to specific Kenyan haematological profile (haplotype 20 with low Hb F level), the level of medical care available and the geographical distribution in the tropics together with other factors.

  6. Prognostic factors for profound sudden idiopathic sensorineural hearing loss: a multicenter retrospective study.

    PubMed

    Lee, Ho Yun; Kim, Dong-Kee; Park, Yong-Ho; Cha, Wang Woon; Kim, Geun Jeon; Lee, Seung Hun

    2017-01-01

    The aim of this study was to assess the outcomes of various treatment modalities for profound idiopathic sudden sensorineural hearing loss (ISSNHL) and confirm the prognostic factors. In total, 191 patients were enrolled after a thorough medical chart review of patients diagnosed with unilateral, profound ISSNHL (≥90 dB). Epidemiological profiles, therapeutic regimens, and the results of pure tone audiometry tests were recorded for all patients. Final recovery was assessed according to Siegel's criteria and by comparing the final hearing level of the affected ear with that of the unaffected ear. The mean follow-up duration and the final hearing level were 75 ± 54 days and 77 ± 24 dB, respectively. None of the evaluated prognostic factors were significantly associated with complete recovery (<25 dB). However, improved hearing in both ears, the absence of dizziness, the use of lipo-prostaglandin E1 (lipo-PGE1), and the use of plasma volume expanders were independently associated with a final hearing level of up to 45 dB (p < 0.05). Steroid dose reduction, worse initial hearing, and non-use of lipo-PGE1 increased the possibility of no recovery. Although the efficacy of oral steroid treatment for profound ISSNHL has been questioned, steroid dose reduction was significantly associated with no recovery. Therefore, adequate oral corticosteroid doses should be considered in the absence of contraindications. In addition, the use of lipo-PGE1 and/or a plasma volume expander seems preferable for better recovery, and their use for the management of profound ISSNHL should be considered.

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

    PubMed

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

    2014-03-01

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

  8. [Inner Ear Hearing Loss Part II: Sudden Sensorineural Hearing Loss, Therapeutic Options].

    PubMed

    Hesse, Gerhard

    2016-07-01

    The great majority of hearing disorders generates from pathologies in the inner ear, mainly the outer hair cells, as mentioned in the first part of this review. Very often, however, hearing loss appears suddenly and even without external causes like noise exposure. This sudden hearing loss is mostly unilateral, recovers very often spontaneously and should be treated, if persisting. Only in this acute stage there are therapeutic options available. If the inner ear hearing loss is chronic there is no curative therapy, an effective management of the hearing disorder is only possible through rehabilitation. This is due to the fact, that hair cells of all mammals, incl. humans, have no regenerative capacity and neither pharmaceutic agents nor other means can induce regeneration and recovery of hair cells. Even a gen-therapy is not available yet. In the second part of this review the main focus lies in sudden hearing loss and general therapeutic options for inner ear hearing loss.

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

    PubMed Central

    Smith, Sandra Nelson; Lucas, Laura

    2016-01-01

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

  10. Persistent Positional Vertigo in a Patient with Sudden Sensorineural Hearing Loss: A Case Report

    PubMed Central

    Kim, Yong Won; Shin, Jung Eun; Lee, Yong-Sik

    2015-01-01

    Because inner ear organs are interconnected through the endolymph and surrounding endolymphatic membrane, the patients with sudden sensorineural hearing loss (SSNHL) often complain of vertigo. In this study, we report a patient with SSNHL accompanied by persistent positional vertigo, and serial findings of head-roll tests are described. At acute stage, head-roll test showed persistent geotropic direction-changing positional nystagmus (DCPN), which led to a diagnosis of SSNHL and ipsilateral light cupula. Although vertigo symptom gradually improved, positional vertigo lasted for more than 3 weeks. At this chronic stage, persistent apogeotropic DCPN was observed in a head roll test, which led to a diagnosis of the heavy cupula. Although the mechanism for the conversion of nystagmus direction from geotropic to apogeotropic persistent DCPN is unclear, the change of specific gravity of the endolymph might be one of the plausible hypothetical explanations. PMID:26413578

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

    PubMed

    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.

  12. Fully implantable Otologics MET Carina™ device for the treatment of sensorineural hearing loss. Preliminary surgical and clinical results

    PubMed Central

    Bruschini, L; Forli, F; Santoro, A; Bruschini, P; Berrettini, S

    2009-01-01

    Summary Middle ear implants overcome some of the common problems of conventional hearing aid technology, such as feedback, signal distortion, ear canal occlusion and associated issues. The Otologics MET Carina™, Boulder, CO, USA, is a fully implantable hearing prosthesis designed to address the amplification needs of adults (> 18 years of age), with moderate to severe sensorineural hearing loss and normal middle ears, providing a mechanical direct stimulation of middle ear ossicles. Recently, it has been successfully used also in patients with conductive hearing loss. In the present report, personal surgical and clinical experience with the fully implantable Carina™ is described in 5 adults with moderate to severe sensorineural hearing loss, operated upon between November 2007 and May 2008 in the ENT Unit, University of Pisa. Mean follow-up was 10.2 months of device use (range 7-13). Surgery was performed under general anaesthesia, in ~3 hours, with no surgical complications in any of the patients. In these 5 cases, no significant post-operative variation was observed in hearing thresholds, either for air or bone conduction, indicating absence of surgical damage to the cochlea. All patients showed improvements in hearing thresholds, in free field and in speech perception abilities, with the device functioning, moreover, they reported subjective benefits. With regard to post-operative adverse effects, no cases of extrusion of the device, device failure, loss of external communication or increased charging times were observed. Problems of feedback noise occurred, which were resolved with minor fitting adjustments in 4 cases, while a second operation was required to change the microphone position in the other patient. The present results, in agreement with those reported in the literature, confirm that the Otologics MET Carina™ is viable treatment for moderate to severe sensorineural hearing loss and, in selected cases, may represent an alternative to

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

    PubMed

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

    2015-01-01

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

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

    PubMed Central

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

    2015-01-01

    Idiopathic sudden sensorineural hearing loss (ISSHL) is a common otologic emergency whose cause is still unclear. The importance of blood lipids in the pathogenesis of ISSHL is widely reported in literature. In fact elevated levels of low density lipoprotein cholesterol (LDL), total cholesterol (TC) and apolipoprotein B (Apo-B) have been proposed as risk factors for this pathology. No correlation has been described between serum lipid parameters and the prognosis of ISSHL. Aim of the present study was to identify prognostic factors associated with hearing recovery in a group of patients affected by ISSHL. Ninety-four patients with the diagnosis of ISSHL hospitalized between March 2013 and October 2014 were included in this study. Patients’ blood sampling and hearing assessments were carried out. Patients were divided into two groups as “recovered” and “unrecovered”, according to their response to the treatment. We found a statistically significant higher level of total cholesterol in the unrecovered group compared to the recovered one (p = 0.03). None of the other routine laboratory parameters have shown a statistically significant difference between the patients successfully treated and patients with poor outcomes. Total cholesterol concentrations may be a prognostic factor for recovery in ISSHL and should be assessed together with routine tests in patients with this condition. The other routine laboratory parameters seem to have no effect on the development and prognosis of this pathology. PMID:26208311

  15. GJB2 Gene Mutations in Syndromic Skin Diseases with Sensorineural Hearing Loss.

    PubMed

    Iossa, Sandra; Marciano, Elio; Franzé, Annamaria

    2011-11-01

    The GJB2 gene is located on chromosome 13q12 and it encodes the connexin 26, a transmembrane protein involved in cell-cell attachment of almost all tissues. GJB2 mutations cause autosomal recessive (DFNB1) and sometimes dominant (DFNA3) non-syndromic sensorineural hearing loss. Moreover, it has been demonstrated that connexins are involved in regulation of growth and differentiation of epidermis and, in fact, GJB2 mutations have also been identified in syndromic disorders with hearing loss associated with various skin disease phenotypes. GJB2 mutations associated with skin disease are, in general, transmitted with a dominant inheritance pattern. Nonsyndromic deafness is caused prevalently by a loss-of-function, while literature evidences suggest for syndromic deafness a mechanism based on gain-of-function. The spectrum of skin manifestations associated with some mutations seems to have a very high phenotypic variability. Why some mutations can lead to widely varying cutaneous manifestations is poorly understood and in particular, the reason why the skin disease-deafness phenotypes differ from each other thus remains unclear. This review provides an overview of recent findings concerning pathogenesis of syndromic deafness imputable to GJB2 mutations with an emphasis on relevant clinical genotype-phenotype correlations. After describing connexin 26 fundamental characteristics, the most relevant and recent information about its known mutations involved in the syndromic forms causing hearing loss and skin problems are summarized. The possible effects of the mutations on channel expression and function are discussed.

  16. Sudden sensorineural hearing loss as prodromal symptom of anterior inferior cerebellar artery infarction.

    PubMed

    Martines, Francesco; Dispenza, Francesco; Gagliardo, Cesare; Martines, Enrico; Bentivegna, Daniela

    2011-01-01

    Sudden sensorineural hearing loss is a clinical condition characterized by a sudden onset of unilateral or bilateral hearing loss. In recent years sudden deafness has been frequently described in association with anterior inferior cerebellar artery (AICA) infarction generally presenting along with other brainstem and cerebellar signs such as ataxia, dysmetria and peripheral facial palsy. The authors report a rare clinical case of a 53-year-old man who suddenly developed hearing loss and tinnitus without any brainstem or cerebellar signs. Computed tomography of his brain was normal, and the audiological results localized the lesion causing deafness to the inner ear. Surprisingly, magnetic resonance imaging showed an ischemic infarct in the right AICA territory. This case represents the fifth in the literature to date but it confirms that AICA occlusion can cause sudden deafness even without brainstem or cerebellar signs. Therefore, we recommend submitting the patient for neuroimaging, as an emergency, in order to exclude infarction of the AICA territory. By doing this, it may be possible to limit the extent of the lesion by commencing early therapy.

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

    PubMed

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

    2015-04-01

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

  18. GJB2 Gene Mutations in Syndromic Skin Diseases with Sensorineural Hearing Loss.

    PubMed Central

    Iossa, Sandra; Marciano, Elio; Franzé, Annamaria

    2011-01-01

    The GJB2 gene is located on chromosome 13q12 and it encodes the connexin 26, a transmembrane protein involved in cell-cell attachment of almost all tissues. GJB2 mutations cause autosomal recessive (DFNB1) and sometimes dominant (DFNA3) non-syndromic sensorineural hearing loss. Moreover, it has been demonstrated that connexins are involved in regulation of growth and differentiation of epidermis and, in fact, GJB2 mutations have also been identified in syndromic disorders with hearing loss associated with various skin disease phenotypes. GJB2 mutations associated with skin disease are, in general, transmitted with a dominant inheritance pattern. Nonsyndromic deafness is caused prevalently by a loss-of-function, while literature evidences suggest for syndromic deafness a mechanism based on gain-of-function. The spectrum of skin manifestations associated with some mutations seems to have a very high phenotypic variability. Why some mutations can lead to widely varying cutaneous manifestations is poorly understood and in particular, the reason why the skin disease-deafness phenotypes differ from each other thus remains unclear. This review provides an overview of recent findings concerning pathogenesis of syndromic deafness imputable to GJB2 mutations with an emphasis on relevant clinical genotype-phenotype correlations. After describing connexin 26 fundamental characteristics, the most relevant and recent information about its known mutations involved in the syndromic forms causing hearing loss and skin problems are summarized. The possible effects of the mutations on channel expression and function are discussed. PMID:22547955

  19. Is Intratympanic Dexamathasone Injection Effective for the Treatment of Idiopathic Sudden Sensorineural Hearing Loss?

    PubMed Central

    Lee, Kyu Ho; Ryu, Sung Hoon; Lee, Hyung Min; Park, Su Kyoung; Kim, Hyung-Jong

    2015-01-01

    Background and Objectives The purpose of this study is to identify the effectiveness of intratympanic (IT) steroids when used as an initial therapy in sudden sensorineural hearing loss (SSNHL), and when it is used as a salvage therapy in a refractory SSNHL. Subjects and Methods This retrospective study included 122 patients with SSNHL. First, patients were divided into three groups according to the initial treatment; IT steroid, systemic steroid and combined (systemic and IT steroid) groups. Second, patients were divided into two groups according to the salvage treatment in a refractory SSNHL; IT steroid and the control (non IT group). Fifty eight patients who did not respond to initial therapy were included in the second analysis. Hearing was assessed immediately before the treatment and 2 weeks, 4 weeks and 3 months after the treatments. Hearing recovery was defined as an improvement of >15 dB and the final hearing of 25-45 dB in the audiogram. Results When we analyzed the hearing recovery in initial treatment, the comparison of audiogram among three groups did not result in significantly different outcomes. There were no differences in the recovery rate in 2 and 4 weeks throughout all the frequencies. Also, the analysis of the salvage treatment demonstrated that neither IT steroid group nor control group was significantly effective in treating the refractory SSNHL. Conclusions The results suggest that initial treatment of SSNHL with IT steroid alone is as effective as systemic steroid alone or a combination therapy. Also, salvage IT steroids for refractory SSNHL did not have any additional beneficial effects. PMID:26771014

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

    PubMed

    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<0.05) and the overall response rate(χ(2)=12.379, P<0.01)between the first and the second treatments. For the patients with 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

  1. Contribution of High Frequencies to Speech Recognition in Quiet and Noise in Listeners with Varying Degrees of High-Frequency Sensorineural Hearing Loss

    ERIC Educational Resources Information Center

    Amos, Nathan E.; Humes, Larry E.

    2007-01-01

    Purpose: The contribution of audible high-frequency information to speech-understanding performance in listeners with varying degrees of high-frequency sensorineural hearing loss was examined. Method: Thirty-six elderly hearing-impaired (EHI) and 24 young normal-hearing (YNH) listeners were tested in quiet (+20 dB speech-to-noise ratio [SNR]) and…

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

    PubMed Central

    Berjis, Nezamoddin; Soheilipour, Saeed; Musavi, Alireza; Hashemi, Seyed Mostafa

    2016-01-01

    Background: During the past years various drugs have been used for sudden sensorineural hearing loss (SSNHL) treatment including steroids that are shown to be beneficial. Directed delivery of high doses of steroids into the inner ear is suggested for its potential and known as intratympanic steroids therapy (IST). Despite the use of dexamethasone and methylprednisolone as the traditional treatments, there are still debates about the optimal dosage, preferred drug, and the route of administration. Materials and Methods: We performed a randomized clinical trial study in which 50 patients suffering from SSNHL and resistant to standard therapy were employed. Each patient took 0.5 ml methylprednisolone (40 mg/mg) along with bicarbonate or dexamethasone (4 mg/mL) through direct intratympanic injection. This method was performed and scheduled once every 2 days for three times only for the dexamethasone receiving group. Hearing test was carried out and the results were analyzed according to a four-frequency (0.5, 1.0, 2.0, 3.0 kHz) pure tone average (PTA) and Siegel's criteria. Results: According to Siegel's criteria, three out of 25 (12%) dexamethasone receiving patients were healed in 1 and 4 (16%), 9 (32%) were respectively recovered in Siegel's criteria 2, 3, and 9 (32%) showed no recovery. In the group receiving methylprednisolone, recovery was found in 6 (24%), 8 (32%), 7 (28%) patients in the Siegel's criteria 1, 2, 3, respectively, and in 4 (16%) patients no recovery was recorded. In methylprednisolone group, hearing was significantly improved compared to the dexamethasone group (P < 0.05). The general hearing improvement rate was 84% in methylprednisolone receiving patients showing a significantly higher improvement than 64% in the dexamethasone group. Conclusions: Topical intratympanic treatment with methylprednisolone is safe and an effective treatment approach for those SSNHL cases that are refractory to the common therapies by Dexamethasone. PMID:27403406

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

    PubMed

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

    2015-07-01

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

  4. First audiometric results with the Vibrant soundbridge, a semi-implantable hearing device for sensorineural hearing loss.

    PubMed

    Snik, A F; Cremers, C W

    1999-01-01

    The Vibrant soundbridge is a semi-implantable hearing device. The implanted electromagnetic transducer is attached to the incus and it is linked by telemetry to the externally worn audio processor. In Nijmegen, this device has been applied to seven patients with moderate or severe sensorineural hearing loss (PTA between 43 and 71 dB HL) who could not tolerate ear moulds. As the amplification of the device depends on the input level (amplifier with wide dynamic range compression), loudness scaling measurements were performed. The gain as a function of input level was determined from aided and unaided loudness growth curves. The mean gain was 21 dB at an input level of 40 dB SPL. The mean gain decreased to 5 dB at an input level of 90 dB SPL. Measured gain values were lower than target values prescribed by the FIG6 method, mainly however for the low-frequency range and for low-level sounds. It was concluded that this device is very promising for patients who cannot tolerate an ear mould.

  5. Preoperative imaging of sensorineural hearing loss in pediatric candidates for cochlear implantation.

    PubMed

    Young, Joseph Y; Ryan, Maura E; Young, Nancy M

    2014-01-01

    Cochlear implantation is the only U.S. Food and Drug Administration-approved treatment for children with marked bilateral sensorineural hearing loss. It provides auditory benefits that range from simple sound detection to substantial word understanding. Improved hearing through cochlear implantation has been demonstrated to enhance the rate of language acquisition, enable development of spoken language, and advance literacy in deaf children. Magnetic resonance imaging and computed tomography both have roles in the preoperative assessment of inner-ear abnormalities, cochlear nerve deficiency, and variant anatomy that may affect the decision to implant and the prognosis for auditory improvement and increase the risk for complications. Most cochlear abnormalities may be successfully treated with cochlear implantation, but the presence of a cochlear malformation may increase the risk for intraoperative cerebrospinal fluid leakage and postoperative bacterial meningitis. Eighth-nerve deficiency correlates with poor auditory outcomes and may affect eligibility for cochlear implantation. Another important consideration for implantation is the presence of labyrinthitis ossificans in some children with deafness resulting from bacterial meningitis, which may cause obstruction that limits electrode insertion. Anatomic variations of the facial nerve or middle-ear cavity, which are more common in syndromic patients, may also affect the surgical approach and make implantation difficult.

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

    PubMed Central

    Iwasa, Yoh-ichiro; Nishio, Shin-ya; Usami, Shin-ichi

    2016-01-01

    Background In general, autosomal dominant inherited hearing loss does not have a founder mutation, with the causative mutation different in each family. For this reason, there has been a strong need for efficient diagnosis methods for autosomal dominant sensorineural hearing loss (ADSNHL) patients. This study sought to verify the effectiveness of our analysis algorithm for the screening of ADSNHL patients as well as the usefulness of the massively parallel DNA sequencing (MPS). Subjects and Methods Seventy-five Japanese ADSNHL patients from 53 ENT departments nationwide participated in this study. We conducted genetic analysis of 75 ADSNHL patients using the Invader assay, TaqMan genotyping assay and MPS-based genetic screening. Results A total of 46 (61.3%) ADSNHL patients were found to have at least one candidate gene variant. Conclusion We were able to achieve a high mutation detection rate through the combination of the Invader assay, TaqMan genotyping assay and MPS. MPS could be used to successfully identify mutations in rare deafness genes. PMID:27911912

  7. Non-Mendelian mitochondrial inheritance as a cause of progressive genetic sensorineural hearing loss.

    PubMed

    Gold, M; Rapin, I

    1994-08-01

    Awareness of non-Mendelian mitochondrial inheritance and of its role as an agent of genetic sensorineural hearing loss (SNHL) is recent. Mitochondria are passed on exclusively from the ovum to all the offspring of both sexes, a novel pattern of inheritance. Owing to the critical role of mitochondria in cellular energy metabolism, deletions or point mutations of the mitochondrial DNA often cause progressive SNHL and a variety of disorders in other organ systems (mitochondrial cytopathies). The clinical expression of mitochondrial diseases varies and depends on the proportion of mutated mitochondria in various body tissues, as well as the nature of the mutation or deletion. In order to determine how often SNHL occurs in mitochondrial diseases and what is its presenting symptom, and also whether SNHL is a marker for particular phenotypes, we carried out a review of published case reports of patients with an established diagnosis of mitochondrial disease. The review indicates that SNHL occurs at all ages and in virtually all variants of mitochondrial diseases. It is not clear whether SNHL is a marker for a more severe and more rapid course of disease; the lower prevalence of SNHL in descriptions of live patients than of those who had died may be an artifact of case selection reported in the literature. Mitochondrial disease needs to be considered in progressive hearing loss and better longitudinal audiometric study of established cases will be required to answer these questions.

  8. Meteorological Conditions Related to the Onset of Idiopathic Sudden Sensorineural Hearing Loss

    PubMed Central

    Seo, Jae-Hyun; Park, Yong-Soo; Kim, JunHyun; Chang, Ki-Hong; Yeo, Sang-Won

    2014-01-01

    Purpose The objective of this study was to evaluate the effect of meteorological factors on the onset of idiopathic sudden sensorineural hearing loss (ISSHL). Materials and Methods Meteorological data from 2005 to 2011 were obtained from the web-based "Monthly Weather Reports of the Meteorological Administration" database. Patients with ISSHL who visited our hospital during this same period and presented the precise day on which hearing loss developed were included in this retrospective study. Twelve meteorological factors were analyzed between the days when ISSHL onset was observed as well as the days when ISSHL did not occur. The weather conditions occurring 1-7 days before ISSHL onset were also analyzed to assess any possible delayed effects of meteorological factors on the onset of ISSHL. Results During the study period, 607 patients were included for the study. Although mean and maximal wind velocities were higher for the days when ISSHL occurred than the days without ISSHL onset, after adjusting the value for multiple comparisons, we cannot find any significant relationship between any of meteorological factors and the onset of ISSHL. However, in analysis of time lag effect of the weather conditions, we found that there was still a significant difference in maximum wind speed on 5 days before ISSHL onset even after applying Bonferroni correction. Conclusion The result of this study suggests that stronger wind speed may be related to the occurrence of ISSHL. PMID:25323908

  9. Steroid-dependent sensorineural hearing loss in a patient with Charcot-Marie-Tooth disease showing auditory neuropathy.

    PubMed

    Maeda, Yukihide; Kataoka, Yuko; Sugaya, Akiko; Kariya, Shin; Kobayashi, Katsuhiro; Nishizaki, Kazunori

    2015-06-01

    Charcot-Marie-Tooth disease (CMT) is the most common form of hereditary sensorimotor neuropathy and sometimes involves disorders of the peripheral auditory system. We present a case of steroid-dependent auditory neuropathy associated with CMT, in which the patient experienced 3 episodes of acute exacerbation of hearing loss and successful rescue of hearing by prednisolone. An 8-year-old boy was referred to the otolaryngology department at the University Hospital. He had been diagnosed with CMT type 1 (demyelinating type) at the Child Neurology Department and was suffering from mild hearing loss due to auditory neuropathy. An audiological diagnosis of auditory neuropathy was confirmed by auditory brainstem response and distortion-product otoacoustic emissions. At 9 years and 0 months old, 9 years and 2 months old, and 10 years and 0 months old, he had experienced acute exacerbations of hearing loss, each of which was successfully rescued by intravenous or oral prednisolone within 2 weeks. Steroid-responsive cases of CMT have been reported, but this is the first case report of steroid-responsive sensorineural hearing loss in CMT. The present case may have implications for the mechanisms of action of glucocorticoids in the treatment of sensorineural hearing loss.

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

    PubMed

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

    2016-09-01

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

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

    PubMed

    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.

  12. Sensorineural hearing loss: a complication of acute otitis media in adults.

    PubMed

    Park, Joo Hyun; Park, Sung Joon; Kim, Young Ho; Park, Min-Hyun

    2014-07-01

    We aim to evaluate the incidence and clinical manifestations of sensorineural hearing loss (SNHL) in adult patients with acute otitis media (AOM). Seventy-five patients (age > 18 years; 83 ears) diagnosed with AOM between January 2008 and March 2011 at our clinic were enroled and retrospectively reviewed. We detected audiometrically confirmed SNHL during the course of AOM in eight patients. The clinical course, treatment, and audiometric final outcome of each case were reviewed. SNHL was associated with AOM in 8 out of 83 ears (9.3%). The mean age of patients was 57.5 years, and the mean follow-up period was 21.1 months (range 0.6-46.3 months). The most common symptom was tinnitus. Mean bone conduction hearing threshold was 39.5 dB in pure tone audiometry. All patients showed high-frequency HL, and three showed pan-frequency HL. All patients were treated with oral antibiotics at the initial visit. Seven ears were treated with a combination of oral steroids. Myringotomy was also performed. Seven of eight patients showed improvement; however, 8 kHz thresholds were not improved. This suggested that the inflammation spread through the round window. The mean duration of recovery was 18.6 days. SNHL associated with AOM in adult patients occurs during the early phases of the disease course. High-frequency hearing was commonly affected and was well treated with oral antibiotics, myringotomy, and steroid therapy. Audiometry can be helpful for treating adult patients with AOM. Active treatment, including myringotomy, should be performed during the early phase, if SNHL is suspected.

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

    PubMed

    Manning, Candice; Mermagen, Timothy; Scharine, Angelique

    2016-10-27

    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.

  14. Role of worry in patients with chronic tinnitus and sensorineural hearing loss: a preliminary study.

    PubMed

    Caldirola, Daniela; Teggi, Roberto; Daccò, Silvia; Sangiorgio, Erika; Bussi, Mario; Perna, Giampaolo

    2016-12-01

    Tinnitus-related distress appears to be more strongly associated with multiple psychological factors than with any perceptual properties of tinnitus. Prior studies have not investigated the role of worry in tinnitus sufferers. Worry is a dispositional cognitive trait that involves a pervasive, non-specific, future-oriented proneness to fretting, which can foster negative affective states and catastrophic thinking about a specific trouble when the trouble is actual and present. We examined the relationship between worry and self-perceived anxiety and depressive symptoms and handicap in 54 outpatients with chronic tinnitus and sensorineural hearing loss who had been previously recruited for a randomized double-blind study on the efficacy of transmeatal low-level laser therapy for tinnitus. We measured the current anxiety and depressive symptoms with the State-Trait Anxiety Inventory Form Y-1/Self-evaluation Depression Scale, the handicap with the Tinnitus Handicap Inventory, and the proneness to worry with the Penn State Worry Questionnaire. For the psychoacoustic tinnitus measures, we considered the loudness match and the minimum masking level. We found that tinnitus-related anxiety and depressive symptoms and handicap were significantly associated with proneness to worry (linear regression models, p < 0.01), whereas no associations were found with the psychoacoustic measures. This suggests the usefulness of worry assessment when managing chronic tinnitus in clinical practice. Early therapeutic interventions for reducing proneness to worry may facilitate better adaptation to tinnitus.

  15. Abnormal Magnetic Resonance Imaging Findings in Patients With Sudden Sensorineural Hearing Loss

    PubMed Central

    Jeong, Kyung-Hwa; Choi, Jin Woo; Shin, Jung Eun; Kim, Chang-Hee

    2016-01-01

    Abstract The etiology of sudden sensorineural hearing loss (SSNHL) remains unclear in most cases. This study aimed to assess abnormal magnetic resonance imaging (MRI) findings in patients with SSNHL and evaluate the value of MRI in identifying the cause of SSNHL. A retrospective analysis of the charts and MRI findings of 291 patients with SSNHL was performed. In 291 patients, MRI abnormality, which was considered a cause of SSNHL, was detected in 13 patients. Vestibular schwannoma involving the internal auditory canal (IAC) and/or cerebellopontine angle was observed in 9 patients. All 9 patients had intrameatal tumors, and 6 of the 9 patients displayed extrameatal extension of their tumors. The tumor was small (<1 cm) or medium-sized (1.1–2.9 cm) in these 6 patients. Intralabyrinthine schwannoma, labyrinthine hemorrhage, IAC metastasis, and a ruptured dermoid cyst were each observed in 1 patient. The most commonly observed MRI abnormality in patients with SSNHL was vestibular schwannoma, and all of the lesions were small or medium-sized tumors involving the IAC. PMID:27124066

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

    PubMed Central

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

    2015-01-01

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

  17. Transplantation of human umbilical cord mesenchymal stem cells in cochlea to repair sensorineural hearing

    PubMed Central

    Ma, Yueying; Guo, Weiwei; Yi, Haijin; Ren, Lili; Zhao, Lidong; Zhang, Yue; Yuan, Shuolong; Liu, Riyuan; Xu, Liangwei; Cong, Tao; EK, Oghagbon; Zhai, Suoqiang; Yang, Shiming

    2016-01-01

    To examine if transplantation of human umbilical cord mesenchymal stem cells (UMSC) into cochlea can be used to repair sensorineural hearing. Here we transplanted the fifth and sixth generations of UMSCs through the subarachnoid cavity of congenital deaf albino pigs. Auditory brainstem responses (ABR) were measured before and after UMSC transplantation. Cochlear samples were collected at 2 hrs, 3 days, 1, 2, 3, 4 and 8 weeks after transplantation. Immunohistochemistry was used to detect the proliferated cell nuclear antigen (PCNA). The UMSCs were found in different regions of the cochlea, including the stria vascularis, the basal membrane and the spiral ganglions, 3 days to 4 weeks after the transplantation. UMSCs and their DNA were found also in the areas of the brain, the heart, the liver, the kidney and the lung. ABR tests displayed a new waveform in the congenital deaf albino pigs after the UMSCs transplantation. We conclude that human UMSCs injected into the subarachnoid space can migrate into the inner ear, the central nervous system and the periphery organs. The presence of UMSCs in the cochlea maybe associated with changes of ABR waveforms in the congenital deaf albino pigs. PMID:28077998

  18. Phonology Matters: A Comprehensive Investigation of Reading and Spelling Skills of School-Age Children with Mild to Moderate Sensorineural Hearing Loss

    ERIC Educational Resources Information Center

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

    2013-01-01

    The investigators measured 7 literacy skills in a group of 21 school-age children with mild to moderate sensorineural hearing loss (MSNH group), and compared the scores to those of 2 age-matched groups: children with dyslexia (DYS group) and, as a control, typically developing hearing children (CA group). The MSNH group performed consistently…

  19. [The expertise of health status dynamics in the members of locomotive crews presenting with occupational sensorineural impairment of hearing].

    PubMed

    Pankova, V B; Bulatskaia, T V; Merkulova, E P

    2013-01-01

    The objective of the present study was to estimate the diagnostic value of the characteristics of somatic pathology included in the algorithm for early diagnostics of occupational sensorineural impairment of hearing (OSNIH). The relationship between the development of the main nosological forms of somatic pathology as possible markers of internal ear diseases and professional sensorineural impairment of hearing was elucidated among the members of locomotive crews (n=224). The parameters of interest were evaluated 4 years before, within 1 year, and 4 years after the establishment of diagnosis of OSNIH. It was shown that the members of locomotive crews most frequently suffer atherosclerosis (20.0%), gastrointestinal disorders (34.8%), arterial hypertension (25.0%), osteochondrosis (largely vertebrogenic lumbodynia (21.3%)), and microcirculatory disturbances in the form of vascular spasm in the fundus of the eye (32.9%). 97.6% of the subjects above 35 years of age presented with hypercholesterolemia. Comparative analysis of the occurrence of these nosological forms among the members of locomotive crews with hearing problems on the one hand and the normally functioning organ of hearing on the other hand has demonstrated that none of these pathologies can be regarded as a marker for early diagnosis of OSNIH. At the same time, overweight even in the subjects under 35 years of age should be taken into account when planning and implementing preventive measures.

  20. [Effect of cochlear implantation on sound localization for patients with unilateral sensorineural hearing loss].

    PubMed

    Liu, J F; Dai, J S; Wang, N Y

    2016-08-07

    The aim of this review was to examine the current literature regarding application of cochlear implantation on patients with unilateral sensorineural hearing loss (USNHL) for improvement on sound localization. The literature were searched in the PubMed database with 'cochlear implantation AND single-sided deafness' or 'cochlear implantation AND unilateral deafness' as keywords. The publication date of the articles was up to 2015-2-12. A total of 12 articles were included. The results show that the ability of sound localization for most of the USNHL subjects (90%) with cochlear implantation was significantly improved than that without CI, which suggests that CI is a superior auditory rehabilitation treatment than BAHA and CROS hearing aids for patients with USNHL, because of the re-establishment of the benefits of binaural hearing. In addition, the benefit of CI for USNHL requires a period of auditory experience or training. About 30% subjects showed significantly improvement on sound localization ability after CI worked for three months. For most of the patients (90%), the sound localization ability improved after CI worked for six months. When CI worked for nine months, all the subjects would show improvement on sound localization ability. Sound localization of the USNHL subjects with a CI is based primarily on interaural level differences (ILD) while interaural time differences (ITD) provide little advantage or probably not perceptible at all. The younger subject suffers from USNHL, the stronger the plasticity of the auditory center shows, which results in more obvious degeneration of the affected side and adaptive enhancement of the contralateral side of the auditory pathway. Similarly, the longer duration of USNHL lead to more obvious degeneration of the affected side and adaptive enhancement of the contralateral side. An adaptive enhancement of auditory pathway corresponding to the healthy ear will rely more on the monaural spatial cues that available to the

  1. Sensorineural hearing loss in insulin-like growth factor I-null mice: a new model of human deafness.

    PubMed

    Cediel, R; Riquelme, R; Contreras, J; Díaz, A; Varela-Nieto, I

    2006-01-01

    It has been reported that mutations in the gene encoding human insulin-like growth factor-I (IGF-I) cause syndromic hearing loss. To study the precise role of IGF-I in auditory function and to hypothesize the possible morphological and electrophysiological changes that may occur in the human inner ear, we have analysed the auditory brainstem response in a mouse model of IGF-I deficiency. We show here that homozygous Igf-1(-/-) mice present an all-frequency involved bilateral sensorineural hearing loss. Igf-1(-/-) mice also present a delayed response to acoustic stimuli; this increases along the auditory pathway, indicating a contribution of the central nervous system to the hearing loss in Igf-1(-/-) mice. These results support the use of the Igf-1(-/-) mouse as a new model for the study of human syndromic deafness.

  2. [Efficacy of intratympanic steroid treatment for idiopathic sudden sensorineural hearing loss after failure of intravenous steroid treatment].

    PubMed

    Kawano, Toshiro; Matsuura, Masaki; Ishitoya, Junichi; Oridate, Nobuhiko

    2014-06-01

    This study investigated the efficacy of intratympanic steroid (ITS) therapy as a salvage treatment for idiopathic sudden sensorineural hearing loss after failure of intravenous steroid (IVS) therapy. Systemic steroid therapy is the only standard drug therapy. However, ethically, we could not simply compare ITS with IVS. Conventionally, we have treated idiopahic sudden sensorineural hearing loss patients after failure of systemic steroid therapy with the double combined therapy IVS and hyperbaric oxygen (HBO), as the salvage modality. We examined the effect of ITS by adding it to the double combined therapy with IVS and HBO. Retrospectively, we clinically examined the effect of double combined therapy with IVS and HBO (A group) for 31 patients (12 men and 19 women) (median age: 54 years) with sudden hearing loss after failure of systemic steroid therapy between June, 2003 and July, 2010. Prospectively, we also examined clinically the effect of triple combined therapy with IVS and HBO, ITS (B group) for 29 patients (17 men and 12 women) (median age: 51 years) with sudden hearing loss after failure of systemic steroid therapy between August, 2010 and April, 2012. In the examination of patients treated within 30 days from the onset, one patient (3.2%) demonstrated remarkable recovery, 6 patients (19.4%) demonstrated mild recovery, while no change was noted in 24 patients (77.4%) in the A group. In the B group, 5 patients (17.2%) demonstrated complete recovery, 3 patients (10.3%) demonstrated remarkable recovery, mild recovery was seen in 14 patients (48.3%), and the remaining 7 patients (24.1%) showed no change. There was a significant difference (p < 0.05) between the A group and the B group. Furthermore, the hearing improvement in group B in five pure tone average was significantly better than in the group A (p < 0.05). We concluded that the B group demonstrated better hearing improvement than the A group. Therefore, ITS could be effective for idiopathic sudden

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

    PubMed

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

    2015-10-01

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

  4. Sensorineural Hearing Loss After Treatment of Nasopharyngeal Carcinoma: A Longitudinal Analysis

    SciTech Connect

    Chan, S.H. Ng, W.T.; Kam, K.L.; Lee, Michael C.H.; Choi, C.W.; Yau, T.K.; Lee, Anne W.M.; Chow, S.K.

    2009-04-01

    Purpose: To analyze the effects of radiotherapy (RT) and chemotherapy in relation to sensorineural hearing loss (SNHL) after contemporary treatment of nasopharyngeal carcinoma. Methods and Materials: A total of 87 nasopharyngeal carcinoma patients were treated with RT or chemoradiotherapy using either three-dimensional conformal RT or intensity-modulated RT between 2004 and 2005. Tympanometry and pure-tone audiogram assessments were performed before treatment and then serially at 6-month intervals. The dose-volume data of the cochlea were analyzed. The effects of cisplatin administered in concurrent and nonconcurrent phases was explored. Results: Of the 170 eligible ears, RT (n = 30) and chemoradiotherapy (n = 140) resulted in 40% (n = 12) and 56.4% (n = 79) persistent SNHL ({>=}15 dB loss), respectively, after a median follow-up of 2 years. SNHL at a high frequency was more frequent statistically in the chemoradiotherapy group than in the RT-alone group (55% vs. 33.3%, p < 0.01), but not at a low frequency (7.9% vs. 16.7%, p = 0.14). Within the chemoradiotherapy group, the mean cochlea dose and concurrent cisplatin dose were important determinants of high-frequency SNHL, with an odds ratio of 1.07/Gy increase (p = 0.01) and an odds ratio of 1.008/mg/m{sup 2} increase (p < 0.01), respectively. Age, gender, and nonconcurrent cisplatin dose were not statistically significant factors. A mean radiation dose to the cochlea of <47 Gy would result in <15% of patients developing severe ({>=}30 dB) high-frequency SNHL. Conclusion: The results of our study have shown that high-frequency SNHL is significantly related to the mean cochlea dose and the concurrent cisplatin dose. A mean dose constraint of 47 Gy to the cochlea is recommended to minimize SNHL after chemoradiotherapy.

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

    PubMed Central

    Lee, Chung; Kim, So Young; Kim, Nayoung K. D.; Chang, Mun Young; Rhee, Jihye; Park, Mi-Hyun; Koo, Soo Kyung; Kim, Min Young; Han, Jin Hee; Oh, Seung-ha; Park, Woong-Yang; Choi, Byung Yoon

    2016-01-01

    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. PMID:27792758

  6. Sensorineural hearing loss and status epilepticus associated with ulcerative colitis: Is there enough evidence to support immune-related mechanisms?

    PubMed Central

    Yazici, Sinem; Benbir, Gulcin; Ince, Birsen

    2015-01-01

    Ulcerative colitis (UC) is characterized by an inflammatory disorder of the gastrointestinal tract. Immune-mediated extraintestinal manifestations of UC have increasingly attracted attention in the literature recently, for which UC is now considered as a systemic disease. Neurologic involvement associated with UC is probably under-reported because of the unawareness of many physicians, although early recognition and treatment are crucial in preventing major morbidity and sequel. In this case report is presented a patient newly diagnosed as UC, who developed both sensorineural hearing loss and intractable status epilepticus that we suggest to have resulted from immune-mediated mechanisms. PMID:25552855

  7. Prevalence of GBJ2 mutations in patients with severe to profound congenital nonsyndromic sensorineural hearing loss in Bulgarian population.

    PubMed

    Popova, Diana P; Kaneva, Radka; Varbanova, Sonya; Popov, Todor M

    2012-06-01

    Objective of the study is to assess the prevalence of Connexin 26 (GJB2) mutation in patients with congenital nonsyndromic sensorineural hearing loss in Bulgarian population. Study design is done prospectively. Patient inclusion criteria for this study were diagnosis of congenital nonsyndromic hearing loss, and absence of potential sibling relationships between patients included in the study (anamnestic pedigree for at least three generations). Patients were excluded from the study group if one of the following conditions were present: secondary hearing loss (cytomegalovirus, rubella, meningo-encephalitis, mastoiditis, other infections, posterior fossa tumors, etc.), exposure to drugs or other prenatal or perinatal etiology of deafness, and congenital syndromic hearing loss. Genomic DNA samples from whole blood were tested with sequence analysis for mutations in the coding region of the GJB2. Results state that 51 patients were analyzed for GJB2 mutations. Twenty of the patients (39%) with mutant alleles were homozygous for the c.35delG mutation (c.35delG/c.35delG) and four patients (8%) presented as heterozygotes (c.35delG/WT). In one patient, who carried a heterozygous mutation c.35delG, a second mutation was found-312del114. Additionally, in two other patients were discovered the mutations Trp24X (W24X) and, respectively, Arg127His(R127H), both in heterozygous states. From the whole study group there was only one patient with compound heterozygous genotype-p.Leu90Pro(L90P)/p.Ile121Asn. The latter one has never been reported in the literature so far. In conclusion, this study determines the importance of connexin 26 mutations in Bulgarian children with severe to profound congenital nonsyndromic sensorineural hearing loss, the prevalence of the different mutation variants and their relationship with the ethnical background of the patients. In addition, we report for the first time a novel mutation in the GJB2 gene.

  8. A Novel De Novo Dominant Mutation in GJB2 Gene Associated with a Sporadic Case of Nonsyndromic Sensorineural Hearing Loss.

    PubMed

    Onsori, Habib; Rahmati, Mohammad; Fazli, Davood

    2014-12-01

    Mutations in the GJB2 gene are the most common known cause of hereditary congenital hearing loss. Rapid genomic DNA extraction (RGDE) method was used for genomic DNA extraction. After amplification of coding region of CX26 gene with specific primers, expected PCR products with 724bp length were subjected to direct sequencing in both directions. We describe here a novel heterozygous -T to -C transition at codon 202 (TGC→CGC) of the GJB2 gene in a patient, 40-year-old Iranian woman, which replaces a cysteine with an arginine residue (C202R). The dominant mutation C202R associated with non-syndromic sensorineural hearing loss. This mutation has not previously been described in affected or control samples from other populations investigated for GJB2 mutations, indicating that it is a rare substitution. This dominant mutation was recorded in NCBI GenBank with accession number KF 638275.

  9. Complete recovery following hyperbaric oxygen therapy in idiopathic sudden sensorineural hearing loss--a report of two cases.

    PubMed

    Agrawal, Swati; Sharma, Nishi

    2016-01-01

    Idiopathic sudden sensorineural hearing loss (ISSHL), a common otologic emergency, presents mostly as an abrupt onset unilateral hearing loss, aural fullness, often with vertigo and tinnitus, usually upon awakening in the morning. Its etiopathogenesis is multifactorial, so a number of different therapeutic regimens are in practice, hyperbaric oxygen (HBO2) therapy being an effective yet underutilized one. Not all cases recover completely even after treatment. Here we describe two cases of ISSHL, diagnosed on the basis of clinical examination and pure-tone audiometry, who had a complete recovery following administration of HBO2 therapy in addition to medical treatment. These cases are reported to highlight the effectiveness of this modality in a physician's armamentarium.

  10. Identification of a novel mutation in the SLC26A4 gene in an Italian with fluctuating sensorineural hearing loss.

    PubMed

    Cama, Elona; Alemanno, Maria Stella; Bellacchio, Emanuele; Santarelli, Rosamaria; Carella, Massimo; Zelante, Leopoldo; Palladino, Teresa; Inches, Ingrid; di Paola, Francesco; Arslan, Edoardo; Melchionda, Salvatore

    2009-10-01

    Pendred syndrome is an autosomal recessive disorder characterized by congenital sensorineural deafness, goitre and defective iodide organification. Congenital and profound hearing loss is the hallmark of the syndrome, while goitre and thyroid dysfunction are highly variable even within the same family. Clinical features are due to altered formation of pendrin, a chloride/iodide transporter protein expressed in the inner ear, thyroid gland and kidney. A novel substitution was found in exon 7 of the pendrin encoding gene (SLC26A4) that leads to a stop codon, S314X. The new variation was found in compound heterozygosity with L445W mutation in a hearing impaired patient with bilateral Mondini's dysplasia and goitre.

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

    PubMed

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

    2016-01-01

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

  12. The mitochondrion: a perpetrator of acquired hearing loss.

    PubMed

    Böttger, Erik C; Schacht, Jochen

    2013-09-01

    Age, drugs, and noise are major causes of acquired hearing loss. The involvement of reactive oxygen species (ROS) in hair cell death has long been discussed, but there is considerably less information available as to the mechanisms underlying ROS formation. Most cellular ROS arise in mitochondria and this review will evaluate evidence for mitochondrial pathology in general and dysfunction of the mitochondrial respiratory chain in particular in acquired hearing loss. We will discuss evidence that different pathways can lead to the generation of ROS and that oxidative stress might not necessarily be causal to all three pathologies. Finally, we will detail recent advances in exploiting knowledge of aminoglycoside-mitochondria interactions for the development of non-ototoxic antibacterials. This article is part of a Special Issue entitled "Annual Reviews 2013".

  13. Neural Alterations in Acquired Age-Related Hearing Loss.

    PubMed

    Mudar, Raksha A; Husain, Fatima T

    2016-01-01

    Hearing loss is one of the most prevalent chronic health conditions in older adults. Growing evidence suggests that hearing loss is associated with reduced cognitive functioning and incident dementia. In this mini-review, we briefly examine literature on anatomical and functional alterations in the brains of adults with acquired age-associated hearing loss, which may underlie the cognitive consequences observed in this population, focusing on studies that have used structural and functional magnetic resonance imaging, diffusion tensor imaging, and event-related electroencephalography. We discuss structural and functional alterations observed in the temporal and frontal cortices and the limbic system. These neural alterations are discussed in the context of common cause, information-degradation, and sensory-deprivation hypotheses, and we suggest possible rehabilitation strategies. Although, we are beginning to learn more about changes in neural architecture and functionality related to age-associated hearing loss, much work remains to be done. Understanding the neural alterations will provide objective markers for early identification of neural consequences of age-associated hearing loss and for evaluating benefits of intervention approaches.

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

    PubMed Central

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

    2017-01-01

    OBJECTIVES: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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. PMID:28226026

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

    PubMed Central

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

    2017-01-01

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

  16. Combined intratympanic and systemic use of steroids for idiopathic sudden sensorineural hearing loss: a meta-analysis.

    PubMed

    Gao, Yang; Liu, Dong

    2016-11-01

    The main objective of the meta-analysis was to investigate whether intratympanic steroid injections in combination with systemic steroids would provide an additional advantage over systemic steroid therapy (SST) alone in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). The results will provide a meaningful suggestion in clinical therapy of ISSNHL. The electronic database search was based on the database in OVID Medline, Embase and PubMed up to December 15, 2015 with the goal of identifying all available observational studies examining the effects of combination therapy and SST in ISSNHL patients. Observational studies that compared the pure tone average (PTA) improvement and recovery rate between combination therapy and SST group in ISSNHL patients were selected. Finally we have identified eight eligible studies that focused on comparing the combination therapy and SST in ISSNHL from designated researches. In the PTA improvement group, seven studies have been analyzed to compare the pooled mean differences between two therapy modalities and subgroups based on initial hearing loss and treatment delay. In the recovery rate group, six studies were calculated for pooled risk ratios and subgroup analysis was also conducted. Through our meta-analysis, we have reached the conclusion that combination therapy exhibited better outcomes in PTA improvement than SST alone, especially in severe-profound initial hearing loss cases. Combination therapy also showed advantages in recovery rate. Whether time of treatment delay would influence the PTA improvement and recovery rate requires further researches.

  17. Sensorineural hearing loss with brainstem auditory evoked responses changes in homozygote and heterozygote sickle cell patients in Guadeloupe (France).

    PubMed

    Jovanovic-Bateman, L; Hedreville, R

    2006-08-01

    This prospective study involved 79 homozygote and heterozygote sickle cell anaemia patients (16 to 50 years old) and a control group of 40 people.All patients underwent ENT, audiological and brainstem auditory evoked responses (BSER) examinations in order to evaluate the incidence of sensorineural hearing loss (SNHL), to identify the changes at the level of the cochlear nerve and the central pathways, and to determine the most vulnerable group, in order to intervene with early prevention and rehabilitation for this condition.A hearing loss of greater than 20 dB at two or more frequencies was found in 36 (45.57 per cent) sickle cell patients (19 (47.22 per cent) HbSC patients and 17 (43.59 per cent) HbSS patients) and three (7.5 per cent) members of the control group. Homozygote and heterozygote patients, as well as both sexes, were equally affected. Bilateral hearing loss occurred in 19 (52.78 per cent) patients, unilateral right-sided hearing loss in five (13.89 per cent) patients and unilateral left-sided hearing loss in 12 (33.33 per cent) patients. Brainstem auditory evoked potential demonstrated a prolonged I-V (III-V) interpeak latency in 13 (25.35 per cent) sickle cell patients (11 men (eight with HbSS) and two women). The hearing loss in HbSS patients was neural in nature and of earlier onset; the hearing loss in HbSC patients was usually cochlear in nature and of later onset. Despite high medical standards and 100 per cent social security cover, the high incidence of SNHL in our sickle cell affected patients (the majority with the Benin haplotype) was probably due to their specific haematological profile and to the original geographical distribution of the disease in the tropics. Our results highlight the necessity for early and regular hearing assessment of sickle cell patients, including BSER examination, especially in male patients with SNHL.

  18. Corticosteroid treatment of idiopathic sudden sensorineural hearing loss: analysis of an RCT and material drawn from the Swedish national database.

    PubMed

    Hultcrantz, Elisabeth; Nosrati-Zarenoe, Ramesh

    2015-11-01

    A randomized placebo-controlled study has demonstrated no effect of prednisolone in customary dosage on idiopathic sudden sensorineural hearing loss (ISSNHL). The aim of the present paper is to analyse a larger patient group by meta-analysis of data from the RCT together with a corresponding material drawn from the Swedish national database for ISSNHL. Data from 192 patients, 18-80 years with ISSNHL, were available. All had an acute hearing loss of at least 30 dB measured as PTA in the three most affected contiguous frequencies. All patients had been enrolled within one week after onset and evaluated by audiograms after 3 months. 45/99 (RCT) and 54/99 (the database) had been treated with prednisolone in tapering doses from 60 mg daily and 42/93 with placebo (RCT) or 51/93 with no treatment (the database). Primary outcome was the mean hearing improvement on day 90 for the different groups. A mean difference of >10 dB improvement was required to demonstrate a treatment effect for prednisolone compared to placebo/no treatment. No significant difference was seen between the prednisolone group and placebo/no treatment (p = 0.06). Total recovery was 38% in prednisolone group, 40% in the placebo and 14% in the no treatment group. Vertigo at the onset of hearing loss and age at onset had an equal negative prognostic value in all groups and signs of inflammation had a positive effect. Prednisolone in customary dosage does not influence recovery after ISSNHL.

  19. A common variant in CLDN14 causes precipitous, prelingual sensorineural hearing loss in multiple families due to founder effect.

    PubMed

    Pater, Justin A; Benteau, Tammy; Griffin, Anne; Penney, Cindy; Stanton, Susan G; Predham, Sarah; Kielley, Bernadine; Squires, Jessica; Zhou, Jiayi; Li, Quan; Abdelfatah, Nelly; O'Rielly, Darren D; Young, Terry-Lynn

    2017-01-01

    Genetic isolates provide unprecedented opportunities to identify pathogenic mutations and explore the full natural history of clinically heterogeneous phenotypes such as hearing loss. We noticed a unique audioprofile, characterized by prelingual and rapid deterioration of hearing thresholds at frequencies >0.5 kHz in several adults from unrelated families from the island population of Newfoundland. Targeted serial Sanger sequencing of probands for deafness alleles (n = 23) that we previously identified in this founder population was negative. Whole exome sequencing in four members of the largest family (R2010) identified a CLDN14 (DFNB29) variant [c.488C>T; p. (Ala163Val)], likely pathogenic, sensorineural hearing loss, autosomal recessive. Although not associated with deafness or disease, CLDN14 p.(Ala163Val) has been previously reported as a variant of uncertain significance (VUS). Targeted sequencing of 169 deafness probands identified one homozygote and one heterozygous carrier. Genealogical studies, cascade sequencing and haplotype analysis across four unrelated families showed all subjects with the unique audioprofile (n = 12) were also homozygous for p.(Ala163Val) and shared a 1.4 Mb DFNB29-associated haplotype on chromosome 21. Most significantly, sequencing 175 population controls revealed 1% of the population are heterozygous for CLDN14 p.(Ala163Val), consistent with a major founder effect in Newfoundland. The youngest CLDN14 [c.488C>T; p.(Ala163Val)] homozygote passed newborn screening and had normal hearing thresholds up to 3 years of age, which then deteriorated to a precipitous loss >1 kHz during the first decade. Our study suggests that genetic testing may be necessary to identify at-risk children in time to prevent speech, language and developmental delay.

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

    SciTech Connect

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

    2009-03-01

    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{sup 2} weekly cisplatin, 13 received 100 mg/m{sup 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{sup 2} or 40 mg/m{sup 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{sup 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.

  1. [Diagnosis of sensorineural hearing loss by ipsilateral masking of brain stem auditory evoked potentials].

    PubMed

    Motta, G; Tranchino, G; Lombardo, N; Ripa, G; Motta, S

    1991-01-01

    The Authors show their original technique for the differential diagnosis of cochlear and retrocochlear hearing loss which employs ipsilateral masking of the A.B.R. in order to define the difference in V wave latency between a standard technique (clicks at 21 pps without noise) and a sensitized technique (clicks at 21 pps with noise at S/N of +40). This difference in latency has been named Sensitizing Latency Difference (SLD). Sixty normal subjects, 85 patients with cochlear hearing loss and 6 with retrocochlear hearing loss, were studied. The obtained data showed a SLD value: in normal hearing of: a) 0.31 msec. (+/- 0.14 SD) from 18 to 40 years old; b) 0.36 msec. (+/- 0.26 SD) from 41 to 60 years old; c) 0.48 msec. (+/- 0.21 SD) over 60 years old; in patients with cochlear hearing loss 0.12 msec. (+/- 0.14 SD) in 2 subjects affected by retrocochlear hearing loss a value superior to 1.5 msec. in the other 4 cases the V waves disappeared during recording of the sensitized test. A differential value of 1 msec. between cochlear and retrocochlear hearing loss was established by the Authors who with this value correctly identified all the patients with retrocochlear hearing loss and 97.8% of the subjects with cochlear hearing loss. An incorrect diagnosis of the hearing loss origin (false positives) was made in 2.2% of the patients with cochlear hearing loss. In conclusion, the Authors feel that the SLD evaluation, in consideration of its large clinical utility and of the high reliability of obtained data, is a particularly useful test in making a differential diagnosis between cochlear and retrocochlear hearing loss.

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

    PubMed

    Cabrera, Sonia; Sanchez, Elena; Requena, Teresa; Martinez-Bueno, Manuel; Benitez, Jesus; Perez, Nicolas; Trinidad, Gabriel; Soto-Varela, Andrés; Santos-Perez, Sofía; Martin-Sanz, Eduardo; Fraile, Jesus; Perez, Paz; Alarcon-Riquelme, Marta E; Batuecas, Angel; Espinosa-Sanchez, Juan M; Aran, Ismael; Lopez-Escamez, Jose A

    2014-01-01

    Meniere's disease is an episodic vestibular syndrome associated with sensorineural hearing loss (SNHL) and tinnitus. Patients with MD have an elevated prevalence of several autoimmune diseases (rheumatoid arthritis, systemic lupus erythematosus, ankylosing spondylitis and psoriasis), which suggests a shared autoimmune background. Functional variants of several genes involved in the NF-κB pathway, such as REL, TNFAIP3, NFKB1 and TNIP1, have been associated with two or more immune-mediated diseases and allelic variations in the TLR10 gene may influence bilateral affectation and clinical course in MD. We have genotyped 716 cases of MD and 1628 controls by using the ImmunoChip, a high-density genotyping array containing 186 autoimmune loci, to explore the association of immune system related-loci with sporadic MD. Although no single nucleotide polymorphism (SNP) reached a genome-wide significant association (p<10(-8)), we selected allelic variants in the NF-kB pathway for further analyses to evaluate the impact of these SNPs in the clinical outcome of MD in our cohort. None of the selected SNPs increased susceptibility for MD in patients with uni or bilateral SNHL. However, two potential regulatory variants in the NFKB1 gene (rs3774937 and rs4648011) were associated with a faster hearing loss progression in patients with unilateral SNHL. So, individuals with unilateral MD carrying the C allele in rs3774937 or G allele in rs4648011 had a shorter mean time to reach hearing stage 3 (>40 dB HL) (log-rank test, corrected p values were p = 0.009 for rs3774937 and p = 0.003 for rs4648011, respectively). No variants influenced hearing in bilateral MD. Our data support that the allelic variants rs3774937 and rs4648011 can modify hearing outcome in patients with MD and unilateral SNHL.

  3. Intronic Variants in the NFKB1 Gene May Influence Hearing Forecast in Patients with Unilateral Sensorineural Hearing Loss in Meniere's Disease

    PubMed Central

    Cabrera, Sonia; Sanchez, Elena; Requena, Teresa; Martinez-Bueno, Manuel; Benitez, Jesus; Perez, Nicolas; Trinidad, Gabriel; Soto-Varela, Andrés; Santos-Perez, Sofía; Martin-Sanz, Eduardo; Fraile, Jesus; Perez, Paz; Alarcon-Riquelme, Marta E.; Batuecas, Angel; Espinosa-Sanchez, Juan M.; Aran, Ismael; Lopez-Escamez, Jose A.

    2014-01-01

    Meniere's disease is an episodic vestibular syndrome associated with sensorineural hearing loss (SNHL) and tinnitus. Patients with MD have an elevated prevalence of several autoimmune diseases (rheumatoid arthritis, systemic lupus erythematosus, ankylosing spondylitis and psoriasis), which suggests a shared autoimmune background. Functional variants of several genes involved in the NF-κB pathway, such as REL, TNFAIP3, NFKB1 and TNIP1, have been associated with two or more immune-mediated diseases and allelic variations in the TLR10 gene may influence bilateral affectation and clinical course in MD. We have genotyped 716 cases of MD and 1628 controls by using the ImmunoChip, a high-density genotyping array containing 186 autoimmune loci, to explore the association of immune system related-loci with sporadic MD. Although no single nucleotide polymorphism (SNP) reached a genome-wide significant association (p<10−8), we selected allelic variants in the NF-kB pathway for further analyses to evaluate the impact of these SNPs in the clinical outcome of MD in our cohort. None of the selected SNPs increased susceptibility for MD in patients with uni or bilateral SNHL. However, two potential regulatory variants in the NFKB1 gene (rs3774937 and rs4648011) were associated with a faster hearing loss progression in patients with unilateral SNHL. So, individuals with unilateral MD carrying the C allele in rs3774937 or G allele in rs4648011 had a shorter mean time to reach hearing stage 3 (>40 dB HL) (log-rank test, corrected p values were p = 0.009 for rs3774937 and p = 0.003 for rs4648011, respectively). No variants influenced hearing in bilateral MD. Our data support that the allelic variants rs3774937 and rs4648011 can modify hearing outcome in patients with MD and unilateral SNHL. PMID:25397881

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

    PubMed

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

    2016-10-15

    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.

  5. The Prevalence and Characteristics of Tinnitus with Profound Sensori-Neural Hearing Impairment.

    ERIC Educational Resources Information Center

    Drukier, Gale S.

    1989-01-01

    Of 331 children (aged 6-18) with profound hearing impairment, 96 were found to have tinnitus. More females than males reported tinnitus. Most of the children with tinnitus were bothered to some degree by it and indicated that the noises adversely affected their ability to hear voices. (JDD)

  6. Hereditary non-syndromic sensorineural hearing loss: transforming silence to sound.

    PubMed

    Schrijver, Iris

    2004-11-01

    Tremendous progress has been made in our understanding of the molecular basis of hearing and hearing loss. Through recent advances, we have begun to understand the fascinating biology of the auditory system and unveiled new molecular mechanisms of hearing impairment. Changes in the diagnostic impact of genetic testing have occurred, as well as exciting developments in therapeutic options. Molecular diagnosis, which is already a reality for several hearing-associated genes, will doubtlessly continue to increase in the near future, both in terms of the number of mutations tested and the spectrum of genes. Genetic analysis for hearing loss is mostly used for diagnosis and treatment, and relatively rarely for reproductive decisions, in contrast to other inherited disorders. Inherited hearing loss, however, is characterized by impressive genetic heterogeneity. An abundance of genes carry a large number of mutations, but specific mutations in a single gene may lead to syndromic or non-syndromic hearing loss. Some mutations predominate in individual ethnic groups. For clinical and laboratory diagnosticians, it is challenging to keep abreast of the unfolding discoveries. This review aims to provide the framework pertinent to diagnosticians and a practical approach to mutation analysis in the hearing impaired.

  7. Development of the stria vascularis and potassium regulation in the human fetal cochlea: Insights into hereditary sensorineural hearing loss

    PubMed Central

    de Groot, John C.M.J.; van Iperen, Liesbeth; Huisman, Margriet A.; Frijns, Johan H.M.

    2015-01-01

    ABSTRACT Sensorineural hearing loss (SNHL) is one of the most common congenital disorders in humans, afflicting one in every thousand newborns. The majority is of heritable origin and can be divided in syndromic and nonsyndromic forms. Knowledge of the expression profile of affected genes in the human fetal cochlea is limited, and as many of the gene mutations causing SNHL likely affect the stria vascularis or cochlear potassium homeostasis (both essential to hearing), a better insight into the embryological development of this organ is needed to understand SNHL etiologies. We present an investigation on the development of the stria vascularis in the human fetal cochlea between 9 and 18 weeks of gestation (W9–W18) and show the cochlear expression dynamics of key potassium‐regulating proteins. At W12, MITF+/SOX10+/KIT+ neural‐crest‐derived melanocytes migrated into the cochlea and penetrated the basement membrane of the lateral wall epithelium, developing into the intermediate cells of the stria vascularis. These melanocytes tightly integrated with Na+/K+‐ATPase‐positive marginal cells, which started to express KCNQ1 in their apical membrane at W16. At W18, KCNJ10 and gap junction proteins GJB2/CX26 and GJB6/CX30 were expressed in the cells in the outer sulcus, but not in the spiral ligament. Finally, we investigated GJA1/CX43 and GJE1/CX23 expression, and suggest that GJE1 presents a potential new SNHL associated locus. Our study helps to better understand human cochlear development, provides more insight into multiple forms of hereditary SNHL, and suggests that human hearing does not commence before the third trimester of pregnancy. © 2015 Wiley Periodicals, Inc. Develop Neurobiol 75: 1219–1240, 2015 PMID:25663387

  8. Sensorineural hearing loss of noise in members of aviation club of Larissa (Greece)

    PubMed Central

    Gerostergiou, E; Tsitiridis, I; Batzakakis, D; Limpanovnou, G; Vathilakis, I; Sandris, V

    2008-01-01

    Objective: To evaluate the clinical and audiometric characteristics of noise by an one-engine Cessna (1300-1750 RPM), by ultra light aircrafts and different types of air modeling products which induce hearing loss in air modeling members of Aviation club of Larissa in Greece. Material-Methods: During the past 2 years members in the aviation club were referred for clinical and audiological examination in the ENT Department of hearing and speech and underwent a personal interview with a specific questionnaire. No one of the examined members was using individual protection systems during aviation activities. Results: From all members examined, a 30% had an audiogram with a slope in high frequencies, not attributed to presbyacousis and had experienced at least one symptom associated with hearing disturbance. Conclusions: Air modeling members in aviation club that are exposed to noise during aviation activities without protection are at high risk of noise induced hearing loss. PMID:19048094

  9. [Universal screening program and early intervention (USPEI) in congenital bilateral sensorineural hearing loss in Chile].

    PubMed

    Albertz, Nicolás; Cardemil, Felipe; Rahal, Maritza; Mansilla, Francisca; Cárdenas, Rodrigo; Zitko, Pedro

    2013-08-01

    Congenital hearing loss is the total or partial inability to hear sounds through the ears. It is the most common disability in newborns in Chile and worldwide, and is a permanent condition. The direct impact on children who are not adequately diagnosed is the alteration in acquisition of language and cognitive skills and a decline in their social and school insertion, jeopardizing their professional and potentially productive life. Universal screening programs for hearing loss are essential for the diagnosis, since 50% of infants with hearing loss have no known risk factor. Screening before one month of age, confirmation before 3 months, and effective intervention before 6 months, allows the development of these children as if they had normal hearing. In Chile there is a selective program of screening for infants aged less than 32 weeks or 1,500 grams, as part of Explicit Health Guarantees, but it covers only 0.9% of newborns per year. Therefore, a large majority of children remain without diagnosis. The aim of this review is to compare the situation in Chile with other countries, raising the need to move towards a universal neonatal hearing loss screening program, and propose necessary conditions in terms of justification and implementation of a universal screening public policy.

  10. A novel mutation in the mitochondrial tRNA(Ser(UCN)) gene in a family with non-syndromic sensorineural hearing impairment.

    PubMed

    Hutchin, T P; Parker, M J; Young, I D; Davis, A C; Pulleyn, L J; Deeble, J; Lench, N J; Markham, A F; Mueller, R F

    2000-09-01

    We describe a family with non-syndromic sensorineural hearing impairment inherited in a manner consistent with maternal transmission. Affected members were found to have a novel heteroplasmic mtDNA mutation, T7510C, in the tRNA(Ser(UCN)) gene. This mutation was not found in 661 controls, is well conserved between species, and disrupts base pairing in the acceptor stem of the tRNA, making it the probable cause of hearing impairment in this family. Sequencing of the other mitochondrial tRNA genes did not show any other pathogenic mutations. Four other mutations causing hearing impairment have been reported in the tRNA(Ser(UCN)) gene, two having been shown to affect tRNA(Ser(UCN)) levels. With increasing numbers of reports of mtDNA mutations causing hearing impairment, screening for such mutations should be considered in all cases unless mitochondrial inheritance can be excluded for certain.

  11. Sensorineural hearing loss in high school teenagers in Mexico City and its relationship with recreational noise.

    PubMed

    Martínez-Wbaldo, María del Consuelo; Soto-Vázquez, Claudia; Ferre-Calacich, Ivonne; Zambrano-Sánchez, Elizabeth; Noguez-Trejo, Lucía; Poblano, Adrián

    2009-12-01

    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.

  12. A Sound Therapy-Based Intervention to Expand the Auditory Dynamic Range for Loudness among Persons with Sensorineural Hearing Losses: Case Evidence Showcasing Treatment Efficacy.

    PubMed

    Formby, Craig; Sherlock, LaGuinn P; Hawley, Monica L; Gold, Susan L

    2017-02-01

    Case evidence is presented that highlights the clinical relevance and significance of a novel sound therapy-based treatment. This intervention has been shown to be efficacious in a randomized controlled trial for promoting expansion of the dynamic range for loudness and increased sound tolerance among persons with sensorineural hearing losses. Prior to treatment, these individuals were unable to use aided sound effectively because of their limited dynamic ranges. These promising treatment effects are shown in this article to be functionally significant, giving rise to improved speech understanding and enhanced hearing aid benefit and satisfaction, and, in turn, to enhanced quality of life posttreatment. These posttreatment sound therapy effects also are shown to be sustained, in whole or part, with aided environmental sound and to be dependent on specialized counseling to maximize treatment benefit. Importantly, the treatment appears to be efficacious for hearing-impaired persons with primary hyperacusis (i.e., abnormally reduced loudness discomfort levels [LDLs]) and for persons with loudness recruitment (i.e., LDLs within the typical range), which suggests the intervention should generalize across most individuals with reduced dynamic ranges owing to sensorineural hearing loss. An exception presented in this article is for a person describing the perceptual experience of pronounced loudness adaptation, which apparently rendered the sound therapy inaudible and ineffectual for this individual. Ultimately, these case examples showcase the enormous potential of a surprisingly simple sound therapy intervention, which has utility for virtually all audiologists to master and empower the adaptive plasticity of the auditory system to achieve remarkable treatment benefits for large numbers of individuals with sensorineural hearing losses.

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

    PubMed

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

    2011-12-01

    Idiopathic sudden sensorineural hearing loss is a rare disorder of unknown pathogenesis in which hearing is lost partially or totally. About 60 treatment modalities have been described. We aimed to compare the efficacy of hyperbaric oxygen, oral steroid, intratympanic steroid therapy and their combinations in idiopathic sudden sensorineural hearing loss patients. Files of patients who were followed up between 2004 and 2010 in our clinic were examined retrospectively. Patients were divided into four groups according to the therapy received: Oral steroid, oral steroid + hyperbaric oxygen, intratympanic steroid and hyperbaric oxygen. Treatment success was assessed by Siegel criteria and mean gains using pre-treatment and post-treatment audiograms. 217 patients and 219 ears were examined. The proportion of patients responding to therapy was the highest in the oral steroid + hyperbaric oxygen group with 86.88% (53/61) followed by the oral steroid group with 63.79% (37/58), the intratympanic steroid group with 46,51% (20/43) and the hyperbaric oxygen group with 43.85% (25/57). The proportion of patients who had complete recovery was the highest in the oral steroid + hyperbaric oxygen group with 42.6% (26/61) followed by the oral steroid group with 19.0% (11/58), the hyperbaric oxygen group with 17.5% (10/57) and the intratympanic steroid group with 11.6% (5/43). The oral steroid + hyperbaric oxygen group has the highest mean hearing gain among all groups (p < 0.05). Idiopathic sudden sensorineural hearing loss patients receiving oral steroid + hyperbaric oxygen combination therapy have a higher likelihood of recovery than patients receiving oral steroids, hyperbaric oxygen or intratympanic steroids alone.

  14. CT and MR imaging of the inner ear and brain in children with congenital sensorineural hearing loss.

    PubMed

    Joshi, Varsha M; Navlekar, Shantanu K; Kishore, G Ravi; Reddy, K Jitender; Kumar, E C Vinay

    2012-01-01

    Imaging plays an important role in the evaluation of congenital sensorineural hearing loss. In children who are candidates for cochlear implantation surgery, it provides vital preoperative information about the inner ear, the vestibulocochlear nerve, and the brain. High-resolution computed tomography (CT) and magnetic resonance (MR) imaging provide excellent delineation of the intricate anatomy of the inner ear: CT depicts the minute details of osseous structures, and MR imaging allows visualization of the fluid-filled spaces and the vestibulocochlear nerve. Together, these complementary modalities can aid decision making about the best management strategy by facilitating the identification and characterization of inner ear malformations and any associated neurologic abnormalities. It is important that the radiologist be familiar with the key imaging features when interpreting CT and MR images obtained in this patient group. A broad spectrum of inner ear malformations have been described and linked to developmental insults at different stages of embryogenesis, and various systems have been proposed for classifying them. In this article, these malformations are described by using classification systems used by otolaryngologists for ease of interpretation. The relevant normal anatomy and development of the inner ear are briefly surveyed, standard imaging protocols for studying the inner ear are reviewed, and the imaging appearances of frequently observed inner ear malformations are described and illustrated. The impact of the identification of these malformations and commonly associated brain abnormalities on clinical management and prognosis also is discussed.

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

    PubMed

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

    2015-12-01

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

  16. Sensorineural hearing loss: potential therapies and gene targets for drug development.

    PubMed

    Tang, Louisa S; Montemayor, Celina; Pereira, Fred A

    2006-09-01

    Recent advances in the developmental biology, genetics and cell biology of the inner ear are guiding research to novel therapeutic modalities - a market currently estimated to be at least US Dollars 10 billion. This article highlights prospects to manipulate the mammalian hearing organ with gene and stem cell delivery to the inner ear to protect, repair or regenerate the hair cells, supporting cells and associated nerves.

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

    PubMed

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

    2013-01-01

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

  18. The role of allergy in the etiopathogenesis of idiopathic sudden sensorineural hearing loss.

    PubMed

    Keleş, Erol; Sapmaz, Emrah; Gödekmerdan, Ahmet

    2013-05-01

    To evaluate the role of allergy in the etiopathogenesis of sudden hearing loss using cytokine profile, allergic parameters, and lymphocyte subgroups, a total of 31 patients aged between 30 and 62 years (mean age: 42.48 ± 15.86 years) with the diagnosis of sudden hearing loss were enrolled in the study between 2008 and 2011. The control group comprised 30 age-matched adult subjects accepted as clinically healthy individuals living in the same region with comparable socioeconomic parameters. The patient and the control group were questioned for history of allergy. Lymphocyte subgroups in the peripheral blood were evaluated using Becton-Dickinson monoclonal CD4, CD8, CD19, and CD23 antibodies. In the patient and control groups, the levels of IFN- γ, IL4, IL10, and total IgE were measured and compared between the groups. Skin test was used for detection of inhaler allergens in the patient and the control groups, and specific IgE tests were performed using the RAST method for food allergens. The Spearman's rho correlation, the unpaired t test, the Mann-Whitney U test, and the Shapiro-Wilk test were used to determine the differences between the groups. The limit for statistical significance was p < 0.05. When the patient and the control group were compared, the differences between CD4, CD23, IL4, IL10, and IgE were statistically significant. On analyses of the patients' serum samples, strongly positive correlations were determined between IgE and CD23, IL10, IL4; IL4 and IL10, CD23, CD4; IL10 and CD23, CD4 (Spearman rho, p = 0.000), while a weakly positive correlation was found between IgE and CD4 (Spearman rho, p = 0.020). A correlation between IFN-γ and CD4 was weakly negative (Spearman rho, p = 0.040). The total IgE levels were estimated to be above the normal reference ranges (0-100 IU/ml) in 25.8 % of the cases in the patient group. History of allergy was found in 61.9 % of the patients. The results of this study substantiate the role of allergy in the

  19. Sensorineural hearing loss and ischemic injury: Development of animal models to assess vascular and oxidative effects.

    PubMed

    Olivetto, E; Simoni, E; Guaran, V; Astolfi, L; Martini, A

    2015-09-01

    Hearing loss may be genetic, associated with aging or exposure to noise or ototoxic substances. Its aetiology can be attributed to vascular injury, trauma, tumours, infections or autoimmune response. All these factors could be related to alterations in cochlear microcirculation resulting in hypoxia, which in turn may damage cochlear hair cells and neurons, leading to deafness. Hypoxia could underlie the aetiology of deafness, but very few data about it are presently available. The aim of this work is to develop animal models of hypoxia and ischemia suitable for study of cochlear vascular damage, characterizing them by electrophysiology and gene/protein expression analyses. The effects of hypoxia in infarction were mimicked in rat by partial permanent occlusion of the left coronary artery, and those of ischemia in thrombosis by complete temporary carotid occlusion. In our models both hypoxia and ischemia caused a small but significant hearing loss, localized at the cochlear apex. A slight induction of the coagulation cascade and of oxidative stress pathways was detected as cell survival mechanism, and cell damages were found on the cuticular plate of outer hair cells only after carotid ischemia. Based on these data, the two developed models appear suitable for in vivo studies of cochlear vascular damage.

  20. Association between the methylenetetrahydrofolate reductase gene C677T polymorphism and sudden sensorineural hearing loss: a meta-analysis.

    PubMed

    Shu, Jingcheng; Yin, Shihua; Tan, An-Zhou; He, Meirong

    2015-09-01

    A variety of epidemiological studies have evaluated the association between methylenetetrahydrofolate reductase (MTHFR) gene C677T polymorphism and sudden sensorineural hearing loss (SSNHL), but the results were inconsistent. The aim of this meta-analysis was to clarify more accurately the association of this polymorphism with SSNHL. A systematic literature search of the associated studies up to May 1, 2014, was conducted using the following electronic databases: PubMed, Embase, Medline, and the China National Knowledge Infrastructure. Statistical analyses were performed by STATA12.0 software, with odds ratios (ORs) and their 95 % confidence intervals (CIs). Six eligible studies including covering 1,271 objects were identified. A pooled analysis of these studies showed no significant association between C677T polymorphism and risk of SSNHL: T vs. C (OR = 1.334, POR = 0.105); TT vs. CC (OR = 1.580, POR = 0.231); CT vs. CC (OR = 1.500, POR = 0.123); TT vs. CC + CT (OR = 1.326, POR = 0.293); and TT + CT vs. CC (OR = 1.540, POR = 0.102). But in subgroup analysis, a significant association was found in European populations (T vs. C, OR = 1.542, 95 % CI 1.008-2.359, P = 0.046; TT vs. CT + CC, OR = 1.856, 95 % CI 1.245-2.767, P = 0.002). There was no significant association in any model in the Asian populations. The present meta-analysis suggests that MTHFR gene C677T polymorphism is significantly associated with increased risk of SSNHL disease in European populations, but no statistically significant association was found between the MTHFR C677T gene mutation and SSNHL in Asian. Further large and well-designed studies are needed to confirm this association.

  1. Impact of methionine synthase gene and methylenetetrahydrofolate reductase gene polymorphisms on the risk of sudden sensorineural hearing loss.

    PubMed

    Gross, Menachem; Friedman, Gideon; Eliashar, Ron; Koren-Morag, Nira; Goldschmidt, Neta; Atta, Iman Abou; Ben-Yehuda, Arie

    2006-01-01

    Idiopathic sudden sensorineural hearing loss (SSNHL) represents a frequently encountered otological disease of unknown etiology. In recent years, several inherited risk factors have been found in the pathogenesis of vascular diseases. In the present study, we determined whether specific polymorphism or the combination of polymorphisms in folate-dependent homocysteine metabolism genes can act as predisposing inherited vascular risk factors in the development of SSNHL. We conducted a prospective case-control study using DNA samples extracted from 81 patients diagnosed as suffering from SSNHL and 264 healthy control subjects. Three functional polymorphisms were analyzed by polymerase chain reaction amplification, restriction enzyme digestion, and DNA fragment separation by electrophoresis: methylenetetrahydrofolate reductase (MTHFR) C677T, MTHFR A1298C, and methionine synthase (MTR) A2756G polymorphisms. The prevalence of the homozygous genotype of MTR 2756GG in the SSNHL patients (9%) was significantly higher than in the control group (4%) (p = 0.011). The allelic frequency of the G allele of the MTR A2756G polymorphism among SSNHL patients (12.5%) was also significantly higher than in the control group (5%) (p = 0.033). The prevalence of patients possessing two polymorphisms (31%) and three polymorphisms (17%) in the SSNHL group was significantly higher than in the control group (23 and 9%, respectively; p = 0.019). The frequency of patients with a very high rank risk (double homozygous) was significantly higher in the SSNHL group, MTHFR 677TT/MTR 2675GG--7%, than the frequency of patients in the control group, MTHFR 677TT/MTR 2675GG--3% (p = 0.030). Certain polymorphisms in genes encoding enzymes in the folate-dependent homocysteine metabolism are associated with SSNHL. In our case-control study, a significant association between MTR 2756GG genotype and SSNHL was found which may represent an inherited vascular risk factor in the pathogenesis of SSNHL.

  2. Acupuncture Therapy for Sudden Sensorineural Hearing Loss: A Systematic Review and Meta-Analysis of Randomized Controlled Trials

    PubMed Central

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

    2015-01-01

    Objective 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. Methods 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. Results 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. Conclusion 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

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

    PubMed

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

    2017-03-01

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

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

    PubMed Central

    Swaminathan, Jayaganesh; Heinz, Michael G.

    2011-01-01

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

  5. Hearing Loss in Adults.

    ERIC Educational Resources Information Center

    House, John W.

    1997-01-01

    This article discusses hearing loss in adults. It begins with an explanation of the anatomy of the ear and then explains the three types of hearing loss: conductive hearing loss, sensorineural hearing loss, and mixed conductive-sensorineural hearing loss. Tinnitus, hearing aids, and cochlear implants are also addressed. (CR)

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

    PubMed Central

    Liu, Yongzhi; Ao, Liying; Ding, Haitao; Zhang, Dongli

    2016-01-01

    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. PMID:27727359

  7. Genetics Home Reference: sensorineural deafness and male infertility

    MedlinePlus

    ... deafness and male infertility sensorineural deafness and male infertility Enable Javascript to view the expand/collapse boxes. ... All Close All Description Sensorineural deafness and male infertility is a condition characterized by hearing loss and ...

  8. Mitochondrial DNA variant m.15218A > G in Finnish epilepsy patients who have maternal relatives with epilepsy, sensorineural hearing impairment or diabetes mellitus

    PubMed Central

    2013-01-01

    Background Mitochondrial diseases caused by mutations in mitochondrial DNA (mtDNA) affect tissues with high energy demand. Epilepsy is one of the manifestations of mitochondrial dysfunction when the brain is affected. We have studied here 79 Finnish patients with epilepsy and who have maternal first- or second-degree relatives with epilepsy, sensorineural hearing impairment or diabetes mellitus. Methods The entire mtDNA was studied by using conformation sensitive gel electrophoresis and PCR fragments that differed in mobility were directly sequenced. Results We found a common nonsynonymous variant m.15218A > G (p.T158A, MTCYB) that occurs in haplogroup U5a1 to be more frequent in patients with epilepsy. The m.15218A > G variant was present in five patients with epilepsy and in four out of 403 population controls (p = 0.0077). This variant was present in two branches in the phylogenetic network constructed on the basis of mtDNA variation among the patients. Three algorithms predicted that m.15218A > G is damaging in effect. Conclusions We suggest that the m.15218A > G variant is mildly deleterious and that mtDNA involvement should be considered in patients with epilepsy and who have a maternal history of epilepsy, sensorineural hearing impairment or diabetes mellitus. PMID:23870133

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

    PubMed Central

    Doobe, G.; Ernst, A.; Ramalingam, R.; Mittmann, P.; Todt, I.

    2015-01-01

    Objective. To investigate the treatment outcome of a simultaneous labyrinthectomy and cochlear implantation in patients with single-sided Ménière's disease and profound sensorineural hearing loss. Study Design. Prospective study. Method. Five patients with single-sided Ménière's disease with active vertigo and functional deafness were included. In all cases, simultaneous cochlear implantation combined with labyrinthectomy surgery was performed. The outcome has been evaluated by the Dizziness Handicap Inventory (DHI) and speech recognition. Results. The combined labyrinthectomy and cochlear implantation led in all patients to a highly significant reduction of dizziness up to a restitutio ad integrum. After activation of the cochlear implant and rehabilitation, a mean monosyllabic speech understanding of 69% at 65 dB was observed. Conclusion. For patients with single-sided Ménière's disease and profound sensorineural hearing loss the simultaneous labyrinthectomy and cochlear implantation are efficient method for the treatment of vertigo as well as the rehabilitation of the auditory system. PMID:26380275

  10. Sensorineural hearing loss after otitis media with effusion and subacute mastoiditis after viral infections of the upper respiratory tract: A comparative study of conservative and surgical treatment.

    PubMed

    Wilhelm, Thomas; Stelzer, Tim; Hagen, Rudolf

    2016-09-01

    Involvement of the middle ear after viral infections of the upper airways may lead to serous otitis media with effusion in the absence of bacterial infection. This can be accompanied by a concomitant shading of the mastoid air cells, which could manifest as a reduced opacity on computed tomography (CT) in the absence of a history of chronic mastoiditis or acute inflammatory signs. This can lead to a subsequent impairment of inner ear function. CT scans reveal an extended pneumatization of the temporal bones in affected patients. Inner ear hearing impairment can probably be attributed to a concomitant labyrinthine reaction-the so-called toxic inner ear lesion. If no remission occurs within 5 days after initial conservative treatment (paracentesis or hemorrheologic infusions), surgical treatment with a mastoidectomy can accelerate hearing restoration. We conducted a retrospective, nonrandomized study of short- and long-term hearing outcomes in patients with a toxic inner ear lesion who had been treated with conservative measures alone (CONS group) or with surgery (SURG group) in a tertiary care referral center. Our study group was made up of 52 consecutively presenting patients (57 ears) who had been seen over a 10-year period; there were 20 patients (21 ears) in the CONS group and 32 patients (36 ears) in the SURG group. Initially, 15 CONS patients (75%) and 18 SURG patients (56%) complained of dizziness or a balance disorder. The initial averaged sensorineural hearing loss (over 0.5, 1.0, 2.0, and 3.0 kHz) was 32.4 ± 15.6 dB in the CONS group and 35.4 ± 12.0 dB in the SURG group. At follow-up (mean: 31.7 mo), the SURG group experienced a significantly greater improvement in hearing (p = 0.025). We conclude that patients with viral otitis media and concomitant noninflammatory mastoiditis with impairment of inner ear function (sensorineural hearing loss) experience a better hearing outcome when a mastoidectomy is performed during primary treatment.

  11. Concurrent posterior semicircular canal benign paroxysmal positional vertigo in patients with ipsilateral sudden sensorineural hearing loss: is it caused by otolith particles?

    PubMed

    Kim, Chang-Hee; Shin, Jung Eun; Park, Hong Ju; Koo, Ja-Won; Lee, Jun Ho

    2014-04-01

    The etiology of benign paroxysmal positional vertigo (BPPV) is still elusive even though detached otolith particles from the utricular macula are generally thought to be responsible for the pathogenesis of BPPV. Sudden sensorineural hearing loss (SSNHL), of which the etiology is also idiopathic in most cases, may accompany concurrent BPPV. This uncommon condition of concurrent BPPV with SSNHL has been assumptively explained as selective damage of the cochlea and the utricle due to viral neurolabyrinthitis. Recently, radiological evidences that inner ear hemorrhage is observed in patients with SSNHL accompanied by severe vertigo have been reported. The basic hypothesis for this study is that blood debris in the endolymphatic fluid due to inner ear hemorrhage is one of the causes of concurrent posterior semicircular canal (PSCC) BPPV in patient with ipsilateral SSNHL. In this report, we will outline the clinical findings of 4 patients with PSCC BPPV with SSNHL, and present an experimental results using whole blood in artificial endolymph to evaluate the hypothesis.

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

    PubMed

    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.

  13. Age-related hearing impairment and the triad of acquired hearing loss

    PubMed Central

    Yang, Chao-Hui; Schrepfer, Thomas; Schacht, Jochen

    2015-01-01

    Understanding underlying pathological mechanisms is prerequisite for a sensible design of protective therapies against hearing loss. The triad of age-related, noise-generated, and drug-induced hearing loss displays intriguing similarities in some cellular responses of cochlear sensory cells such as a potential involvement of reactive oxygen species (ROS) and apoptotic and necrotic cell death. On the other hand, detailed studies have revealed that molecular pathways are considerably complex and, importantly, it has become clear that pharmacological protection successful against one form of hearing loss will not necessarily protect against another. This review will summarize pathological and pathophysiological features of age-related hearing impairment (ARHI) in human and animal models and address selected aspects of the commonality (or lack thereof) of cellular responses in ARHI to drugs and noise. PMID:26283913

  14. 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.

    PubMed

    Formby, Craig; Hawley, Monica L; Sherlock, LaGuinn P; Gold, Susan; Payne, JoAnne; Brooks, Rebecca; Parton, Jason M; Juneau, Roger; Desporte, Edward J; Siegle, Gregory R

    2015-05-01

    The primary aim of this research was to evaluate the validity, efficacy, and generalization of principles underlying a sound therapy-based treatment for promoting expansion of the auditory dynamic range (DR) for loudness. The basic sound therapy principles, originally devised for treatment of hyperacusis among patients with tinnitus, were evaluated in this study in a target sample of unsuccessfully fit and/or problematic prospective hearing aid users with diminished DRs (owing to their elevated audiometric thresholds and reduced sound tolerance). Secondary aims included: (1) delineation of the treatment contributions from the counseling and sound therapy components to the full-treatment protocol and, in turn, the isolated treatment effects from each of these individual components to intervention success; and (2) characterization of the respective dynamics for full, partial, and control treatments. Thirty-six participants with bilateral sensorineural hearing losses and reduced DRs, which affected their actual or perceived ability to use hearing aids, were enrolled in and completed a placebo-controlled (for sound therapy) randomized clinical trial. The 2 × 2 factorial trial design was implemented with or without various assignments of counseling and sound therapy. Specifically, participants were assigned randomly to one of four treatment groups (nine participants per group), including: (1) group 1-full treatment achieved with scripted counseling plus sound therapy implemented with binaural sound generators; (2) group 2-partial treatment achieved with counseling and placebo sound generators (PSGs); (3) group 3-partial treatment achieved with binaural sound generators alone; and (4) group 4-a neutral control treatment implemented with the PSGs alone. Repeated measurements of categorical loudness judgments served as the primary outcome measure. The full-treatment categorical-loudness judgments for group 1, measured at treatment termination, were significantly

  15. 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

    PubMed Central

    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

  16. Polymorphisms in genes involved in oxidative stress response in patients with sudden sensorineural hearing loss and Ménière's disease in a Japanese population.

    PubMed

    Teranishi, Masaaki; Uchida, Yasue; Nishio, Naoki; Kato, Ken; Otake, Hironao; Yoshida, Tadao; Suzuki, Hirokazu; Sone, Michihiko; Sugiura, Saiko; Ando, Fujiko; Shimokata, Hiroshi; Nakashima, Tsutomu

    2012-10-01

    The etiologies of idiopathic sudden sensorineural hearing loss (SSNHL) and Ménière's disease remain unclear. Recently, accumulating evidence has demonstrated that oxidative stress is related to the pathology of inner ear disease. Because genetic factors may contribute partly to the etiologies of SSNHL and Ménière's disease, we investigated the associations between genetic polymorphisms located in oxidative stress response genes and susceptibility to SSNHL and Ménière's disease. We compared 84 patients affected by SSNHL, 82 patients affected by Ménière's disease, and 2107 adults (1056 men and 1051 women; mean age, 59.2 years; range, 40-79 years) who participated in the National Institute for Longevity Sciences, Longitudinal Study of Aging. Multiple logistic regression was used to calculate odds ratios for SSNHL and Ménière's disease in individuals with polymorphisms in the genes glutathione peroxidase 1 (GPX1) (Pro198Leu, rs1050450), paraoxonase 1 (PON1) (Gln192Arg, rs662; and Met55Leu, rs854560), PON2 (Ser311Cys, rs7493), and superoxide dismutase 2 (SOD2) (Val16Ala, rs4880), with adjustment for age and gender. No significant differences in the distribution of the genotypes at these polymorphisms were observed among individuals with SSNHL and Ménière's disease and controls. No significant risk for SSNHL and Ménière's disease was observed in the additive genetic model, regardless of moderating variables. The C allele of SOD2 (rs4880) was more frequent in Ménière's disease cases with a hearing level over 50 dB compared with cases with a hearing level below 50 dB, suggesting that this polymorphism is associated with progression of a hearing loss in Ménière's disease. In conclusion, no significant associations between the polymorphisms of GPX1, PON1, PON2, and SOD2 and risk of SSNHL and Ménière's disease were observed in this Japanese case-control study.

  17. Abnormal Magnetic Resonance Imaging Findings in Patients With Sudden Sensorineural Hearing Loss: Vestibular Schwannoma as the Most Common Cause of MRI Abnormality.

    PubMed

    Jeong, Kyung-Hwa; Choi, Jin Woo; Shin, Jung Eun; Kim, Chang-Hee

    2016-04-01

    The etiology of sudden sensorineural hearing loss (SSNHL) remains unclear in most cases. This study aimed to assess abnormal magnetic resonance imaging (MRI) findings in patients with SSNHL and evaluate the value of MRI in identifying the cause of SSNHL.A retrospective analysis of the charts and MRI findings of 291 patients with SSNHL was performed.In 291 patients, MRI abnormality, which was considered a cause of SSNHL, was detected in 13 patients. Vestibular schwannoma involving the internal auditory canal (IAC) and/or cerebellopontine angle was observed in 9 patients. All 9 patients had intrameatal tumors, and 6 of the 9 patients displayed extrameatal extension of their tumors. The tumor was small (<1 cm) or medium-sized (1.1-2.9 cm) in these 6 patients. Intralabyrinthine schwannoma, labyrinthine hemorrhage, IAC metastasis, and a ruptured dermoid cyst were each observed in 1 patient.The most commonly observed MRI abnormality in patients with SSNHL was vestibular schwannoma, and all of the lesions were small or medium-sized tumors involving the IAC.

  18. The efficacy and safety of systemic injection of Ginkgo biloba extract, EGb761, in idiopathic sudden sensorineural hearing loss: a randomized placebo-controlled clinical trial.

    PubMed

    Koo, Ja-Won; Chang, Mun Young; Yun, Sung-Cheol; Kim, Tae Su; Kong, Soo-Keun; Chung, Jong Woo; Goh, Eui-Kyung

    2016-09-01

    Steroids are currently the most frequently accepted agents for idiopathic sudden sensorineural hearing loss (ISSNHL). However, the therapeutic effect of steroids is not always satisfactory. In this pilot study, we evaluated whether systemic treatment with Ginkgo biloba extract (EGb761) has an additive therapeutic effect in patients receiving a systemic steroid due to ISSNHL. A multicenter, randomized, double-blind clinical trial was performed. Fifty-six patients with ISSNHL were allocated to either EGb761 or placebo. In both groups, methylprednisolone was administered for 14 days. EGb761 was infused intravenously for 5 days in the EGb761 group, while the same amount of normal saline was infused in the placebo group. For the efficacy evaluation, pure-tone audiometry, speech audiometry, tinnitus handicap inventory (THI) and short form-36 health (SF-36) survey outcomes were obtained before administration and on days 3, 5, 14 and 28 of administration. Twenty-four patients in each group completed the study protocol. There was no difference in hearing loss between the two groups before treatment. At day 28, air conduction threshold values in the placebo and EGb761 groups were 34.63 ± 28.90 and 23.84 ± 25.42 dB, respectively (p = 0.082). Speech discrimination scores in the placebo and EGb761 groups were 69.17 ± 40.89 and 87.48 ± 28.65 %, respectively (p = 0.050). THI and SF-36 scores in the placebo and EGb761 groups were similar. Although a combination of steroid and EGb761 for initial treatment did not show better pure tone threshold, compared with steroid alone, speech discrimination was significantly improved in combination therapy. Further studies will be needed to know if addition of EGb761 actually improves the outcome of ISSNHL treatment.

  19. Establishment of a Flexible Real-Time Polymerase Chain Reaction-Based Platform for Detecting Prevalent Deafness Mutations Associated with Variable Degree of Sensorineural Hearing Loss in Koreans

    PubMed Central

    Kim, Min Young; Ahn, Soyeon; Oh, Seung-Ha; Song, Ju Hun; Choi, Byung Yoon

    2016-01-01

    Many cutting-edge technologies based on next-generation sequencing (NGS) have been employed to identify candidate variants responsible for sensorineural hearing loss (SNHL). However, these methods have limitations preventing their wide clinical use for primary screening, in that they remain costly and it is not always suitable to analyze massive amounts of data. Several different DNA chips have been developed for screening prevalent mutations at a lower cost. However, most of these platforms do not offer the flexibility to add or remove target mutations, thereby limiting their wider use in a field that requires frequent updates. Therefore, we aimed to establish a simpler and more flexible molecular diagnostic platform based on ethnicity-specific mutation spectrums of SNHL, which would enable bypassing unnecessary filtering steps in a substantial portion of cases. In addition, we expanded the screening platform to cover varying degrees of SNHL. With this aim, we selected 11 variants of 5 genes (GJB2, SLC26A4, MTRNR1, TMPRSS3, and CDH23) showing high prevalence with varying degrees in Koreans and developed the U-TOP™ HL Genotyping Kit, a real-time PCR-based method using the MeltingArray technique and peptide nucleic acid probes. The results of 271 DNA samples with wild type sequences or mutations in homo- or heterozygote form were compared between the U-TOP™ HL Genotyping Kit and Sanger sequencing. The positive and negative predictive values were 100%, and this method showed perfect agreement with Sanger sequencing, with a Kappa value of 1.00. The U-TOP™ HL Genotyping Kit showed excellent performance in detecting varying degrees and phenotypes of SNHL mutations in both homozygote and heterozygote forms, which are highly prevalent in the Korean population. This platform will serve as a useful and cost-effective first-line screening tool for varying degrees of genetic SNHL and facilitate genome-based personalized hearing rehabilitation for the Korean population

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

    PubMed Central

    Lacerda, Clara Fonseca; Silva, Luciana Oliveira e; de Tavares Canto, Roberto Sérgio; Cheik, Nadia Carla

    2012-01-01

    Summary 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. PMID:25991930

  1. Homozygosity for the V37I GJB2 mutation in fifteen probands with mild to moderate sensorineural hearing impairment: further confirmation of pathogenicity and haplotype analysis in Asian populations.

    PubMed

    Gallant, Emily; Francey, Lauren; Tsai, Ellen A; Berman, Micah; Zhao, Yaru; Fetting, Heather; Kaur, Maninder; Deardorff, Matthew A; Wilkens, Alisha; Clark, Dinah; Hakonarson, Hakon; Rehm, Heidi L; Krantz, Ian D

    2013-09-01

    Hearing impairment affects 1 in 650 newborns, making it the most common congenital sensory impairment. Autosomal recessive nonsyndromic sensorineural hearing impairment (ARNSHI) comprises 80% of familial hearing impairment cases. Mutations in GJB2 account for a significant number of ARNSHI (and up to 50% of documented recessive (e.g., more than 1 affected sibling) hearing impairment in some populations). Mutations in the GJB2 gene are amongst the most common causes of hearing impairment in populations of various ethnic backgrounds. Two mutations of this gene, 35delG and 167delT, account for the majority of reported mutations in Caucasian populations, especially those of Mediterranean and Ashkenazi Jewish background. The 235delC mutation is most prevalent in East Asian populations. Some mutations are of less well-characterized significance. The V37I missense mutation, common in Asian populations, was initially described as a polymorphism and later as a potentially pathogenic mutation. We report here on 15 unrelated individuals with ARNSHI and homozygosity for the V37I GJB2 missense mutation. Nine individuals are of Chinese ancestry, two are of unspecified Asian descent, one is of Japanese descent, one individual is of Vietnamese ancestry, one of Philippine background and one of Italian and Cuban/Caucasian background. Homozygosity for the V37I GJB2 mutation may be a more common pathogenic missense mutation in Asian populations, resulting in mild to moderate sensorineural hearing impairment. We report a presumed haplotype block specific to East Asian individuals with the V37I mutation encompassing the GJB2 gene that may account for the high prevalence in East Asian populations.

  2. Fibrinogen is not a prognostic factor for response to HELP-apheresis in sudden sensorineural hearing loss (SSHL).

    PubMed

    Berger, T; Kaiser, T; Scholz, M; Bachmann, A; Ceglarek, U; Hesse, G; Hagemeyer, B; Stumvoll, M; Thiery, J; Dietz, A

    2015-12-01

    Higher levels of fibrinogen or cholesterol were associated with improved hearing recovery in SSHL patients after treatment with HELP-apheresis (Heparin-induced extracorporeal LDL precipitation apheresis). The present trial was performed to demonstrate HELP-related effects on relevant metabolic and inflammatory parameters in the context of SSHL treatment. In the framework of a single arm non-controlled trial, we investigated the variation of metabolic and inflammatory parameters using HELP-apheresis for a defined group of 100 patients with SSHL. Based on cut off inclusion criteria (Serum LDL-cholesterol >1.6 g/l and/or fibrinogen >2.0 g/l, SSHL in minimum three frequencies more than 30 dB, time after event not longer than 6 days), the protocol followed a strict time line with one single shot HELP-apheresis and follow-up monitoring including laboratory parameters at six defined time points. If HELP-apheresis could not effect improvement of hearing on day 5, additional corticosteroid treatment was applied. Concentration of anti-inflammatory IL-10 increased while other proinflammatory parameters declined. Serum levels of all measured sterols and apolipoproteins decreased significantly. None of the investigated parameters were suitable to predict hearing improvement of the patients. Levels of fibrinogen and LDL-cholesterol were not prognostic for outcome after HELP-apheresis. A significant (p < 0.001) increase of anti-inflammatory IL-10 after apheresis was notable, while most of the proinflammatory parameters declined. Despite the limited validity of a single arm non-controlled trial, these alterations on immune modulating factors indicate possible secondary pleiotropic effects caused by HELP-apheresis.

  3. Polymorphisms in genes involved in the free-radical process in patients with sudden sensorineural hearing loss and Ménière's disease.

    PubMed

    Teranishi, M; Uchida, Y; Nishio, N; Kato, K; Otake, H; Yoshida, T; Suzuki, H; Sone, M; Sugiura, S; Ando, F; Shimokata, H; Nakashima, T

    2013-07-01

    The etiologies of idiopathic sudden sensorineural hearing loss (SSNHL) and Ménière's disease remain unclear. Recently, accumulating evidence has demonstrated that free radicals are related to the pathology of inner ear disease. Because genetic factors may contribute partly to the etiologies of SSNHL and Ménière's disease, we investigated the association between genetic polymorphisms located in genes related to the free-radical process and susceptibility to SSNHL and Ménière's disease. We compared 83 patients affected by SSNHL and 83 patients affected by Ménière's disease with 2048 adults (for SSNHL) and 1946 adults (for Ménière's disease) who participated in the National Institute for Longevity Sciences, Longitudinal Study of Aging. Multiple logistic regression was used to calculate odds ratios (ORs) for SSNHL and Ménière's disease in individuals with polymorphisms in the genes: methionine synthase (MTR; rs1805087); methionine-synthase reductase (MTRR; rs1801394); nitric oxide synthase 3 (NOS3; rs1799983); caveolin 1 (Cav1; rs3840634); melatonin receptor 1B (MTNR1B; rs1387153); NAD(P)H oxidase p22(phox) subunit (NADH/NADPHp22phox; rs4673); and mitochondria 5178 (MT5178; rs28357984). The NOS3 polymorphism was significantly associated with a risk of SSNHL; in addition, the OR for the NOS3 polymorphism and SSNHL risk was 2.108 (CI, 1.343-3.309) with adjustment for age and sex. The Cav1 polymorphism was significantly associated with a risk of Ménière's disease; moreover, the OR for the Cav1 polymorphism and Ménière's disease risk was 1.849 (CI, 1.033-3.310) with adjustment for age and sex. In conclusion, the NOS3 and Cav1 polymorphisms were significantly associated with the risk of SSNHL and Ménière's disease, respectively.

  4. Temporal bone histopathology in a case of sensorineural hearing loss caused by superficial siderosis of the central nervous system and treated by cochlear implantation

    PubMed Central

    Nadol, Joseph B.; Adams, Joe C.; O'Malley, Jennifer T.

    2011-01-01

    Objectives To evaluate the histopathology of the temporal bones of a patient with documented superficial siderosis of the central nervous system who underwent right cochlear implantation six years before death. Background Superficial siderosis of the central nervous system is due to chronic or repeated subarachnoid hemorrhage and results in sensorineural deafness in 95% of affected individuals in addition to other neurologic findings. The deposition of hemosiderin in the meninges and around cranial nerves is thought to be causative. There have been no previous reports of temporal bone pathology in this disorder. This 57 year old man developed progressive, bilateral hearing loss starting in his 30's with loss of pure tone thresholds and word recognition. He underwent a right cochlear implant at age 51 with full insertion of the device. Methods The temporal bones and brainstem were fixed in formalin and prepared for histologic study by standard techniques. Special stains, including Gomori stain for iron were performed on sections of the temporal bones and cochlear nucleus. Results There was severe bilateral degeneration of the organ of Corti, spiral ligament, stria vascularis, and spiral ganglion cells. Gomori stain revealed iron deposits within the spiral ligament, stria vascularis and in the subepithelial mesenchymal tissue of the maculae of the vestibular system. Evaluation of the cochlear nucleus revealed iron deposits within glial cells and larger cells, probably macrophages, near the CSF surface. On the right side, the track created by the cochlear implant entered the scala tympani and continued to mm17, as measured from the round window. Discussion and Conclusions This is the first known case of superficial siderosis with documented temporal bone histopathology. Hearing loss was likely caused by severe degeneration of spiral ganglion cells in both ears, despite the presence of remaining hair cells in the middle and apical turns. This was consistent with

  5. A comparison of inner ear imaging features at different time points of sudden sensorineural hearing loss with three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging.

    PubMed

    Zhu, Honglei; Ou, Yongkang; Fu, Jia; Zhang, Ya; Xiong, Hao; Xu, Yaodong

    2015-10-01

    It has been reported that about half of patients with sudden sensorineural hearing loss (SSNHL) show high signals in the affected inner ear on three-dimensional, fluid-attenuated inversion recovery magnetic resonance imaging (3D-FLAIR MRI). These signals may reflect minor hemorrhage or an increased concentration of protein in the inner ear, which has passed through blood vessels with increased permeability. Our objective was to compare the positive ratio of the high signal in affected inner ears at different time points to determine the suitable imaging time point for 3D-FLAIR MRI in SSNHL. 3D-FLAIR MRI images were taken at three times, precontrast and approximately 10 min and 4 h after intravenous injection of a single dose of gadodiamide (Gd) (0.1 mmol/kg), in 46 patients with SNHL. We compared the positive findings of the high signals in the inner ear of patients with SNHL as well as the signal intensity ratio (SIR) between the affected cochleae and unaffected cochleae at three time points. The positive ratios of the high signals in the affected inner ear at the time points of precontrast and 10 min and 4 h after the intravenous Gd injection were 26.1, 32.6, and 41.3%, respectively. The high signal intensity ratios of affected inner ears at the three time points were 1.28, 1.31, and 1.48, respectively. The difference between the positive ratios precontrast and at 10 min after the intravenous Gd injection was statistically significant (P = 0.006); the differences between the positive ratios at 4 h after the intravenous Gd injection and precontrast and between the ratios at 4 h and 10 min after the intravenous Gd injection were not statistically significant. The time effects of the median value of SIR were not significant (P = 0.064). We do not recommend 4 h after intravenous Gd injection as a time point to image the inner ear in SNHL. We believe that imaging precontrast and at 10 min after the intravenous Gd injection are suitable time points.

  6. Viral causes of hearing loss: a review for hearing health professionals.

    PubMed

    Cohen, Brandon E; Durstenfeld, Anne; Roehm, Pamela C

    2014-07-29

    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.

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

    PubMed Central

    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

  8. Hearing Loss: Hearing Augmentation.

    PubMed

    Atcherson, Samuel R; Moreland, Christopher; Zazove, Philip; McKee, Michael M

    2015-07-01

    Etiologies of hearing loss vary. When hearing loss is diagnosed, referral to an otology subspecialist, audiology subspecialist, or hearing aid dispenser to discuss treatment options is appropriate. Conventional hearing aids provide increased sound pressure in the ear canal for detection of sounds that might otherwise be soft or inaudible. Hearing aids can be used for sensorineural, conductive, or mixed hearing loss by patients with a wide range of hearing loss severity. The most common type of hearing loss is high-frequency, which affects audibility and perception of speech consonants, but not vowels. As the severity of hearing loss increases, the benefit of hearing aids for speech perception decreases. Implantable devices such as cochlear implants, middle ear implants, and bone-anchored implants can benefit specific patient groups. Hearing assistive technology devices provide auditory, visual, or tactile information to augment hearing and increase environmental awareness of sounds. Hearing assistive devices include wireless assistive listening device systems, closed captioning, hearing aid-compatible telephones, and other devices. For some patients, financial barriers and health insurance issues limit acquisition of hearing aids, implantable devices, and hearing assistive devices. Physicians should be aware that for some patients and families, hearing augmentation may not be desired for cultural reasons.

  9. Sensorineural Organs Dysfunction and Cognitive Decline: A Review Article

    PubMed Central

    Wongrakpanich, Supakanya; Petchlorlian, Aisawan; Rosenzweig, Andrew

    2016-01-01

    Vision, hearing, olfaction, and cognitive function are essential components of healthy and successful aging. Multiple studies demonstrate relationship between these conditions with cognitive function. The present article focuses on hearing loss, visual impairment, olfactory loss, and dual sensory impairments in relation to cognitive declination and neurodegenerative disorders. Sensorineural organ impairment is a predictive factor for mild cognitive impairment and neurodegenerative disorders in the elderly. We recommend early detection of sensorineural dysfunction by history, physical examination, and screening tests. Assisted device and early cognitive rehabilitation may be beneficial. Future research is warranted in order to explore advanced treatment options and method to slow progression for cognitive declination and sensorineural organ impairment. PMID:28053826

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

    PubMed

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

    2008-01-01

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

  11. Hearing disorders in cats.

    PubMed

    Strain, George M

    2017-03-01

    Practical relevance: Auditory function is a sense that is central to life for cats - being important in situational awareness of potential predators, pursuit of prey, and for communication with conspecifics, humans and other species. Deafness in cats is most frequently the result of a genetic disorder, strongly associated with white fur and blue eyes, but may also result from acquired causes such as advancing age, ototoxic drugs, infection, environmental noise and physical trauma. Deafness can be sensorineural, where there is loss of cochlear hair cells, or conductive, where sound is muffled on its way to the inner ear. Clinical challenges: Establishing whether a cat is deaf can be difficult as behavioral testing of hearing is subjective and does not reliably detect unilateral deafness. Brainstem auditory evoked response testing is an objective measure but is limited in its availability. Currently, sensorineural deafness is irreversible because no treatments are available to restore lost hair cells. Conductive hearing loss can usually be treated, although full hearing recovery following otitis media may take weeks as the body clears the middle ear of debris. Evidence base: The author draws on the published literature and his extensive research on clinical aspects and molecular genetics of deafness, principally in companion animals, to review types and forms of deafness in cats. He also discusses current diagnostic approaches and provides brief advice for managing cats with hearing loss.

  12. Congenital sensorineural deafness in dogs: a molecular genetic approach toward unravelling the responsible genes.

    PubMed

    Rak, Simone G; Distl, Ottmar

    2005-03-01

    Deafness is often diagnosed in different dog breeds and has been identified as a significant problem for breeders, owners and clinicians. The aetiology can be inherited or acquired, and a distinction must be made between sensorineural and conductive forms of deafness. This paper provides a brief overview of the varieties of findings in different dog breeds and in one breed in particular including prevalence, phenotypic and gender associations, histology, modes of inheritance and the number of contributing genes in congenital sensorineural deafness. We have also described molecular genetic approaches to canine hearing loss and discuss how comparative genomics could help reduce the prevalence of deafness in affected breeds leading to new insights into the molecular mechanisms of auditory function in both dogs and humans.

  13. Contribution of monaural and binaural cues to sound localization in listeners with acquired unilateral conductive hearing loss: improved directional hearing with a bone-conduction device.

    PubMed

    Agterberg, Martijn J H; Snik, Ad F M; Hol, Myrthe K S; Van Wanrooij, Marc M; Van Opstal, A John

    2012-04-01

    Sound localization in the horizontal (azimuth) plane relies mainly on interaural time differences (ITDs) and interaural level differences (ILDs). Both are distorted in listeners with acquired unilateral conductive hearing loss (UCHL), reducing their ability to localize sound. Several studies demonstrated that UCHL listeners had some ability to localize sound in azimuth. To test whether listeners with acquired UCHL use strongly perturbed binaural difference cues, we measured localization while they listened with a sound-attenuating earmuff over their impaired ear. We also tested the potential use of monaural pinna-induced spectral-shape cues for localization in azimuth and elevation, by filling the cavities of the pinna of their better-hearing ear with a mould. These conditions were tested while a bone-conduction device (BCD), fitted to all UCHL listeners in order to provide hearing from the impaired side, was turned off. We varied stimulus presentation levels to investigate whether UCHL listeners were using sound level as an azimuth cue. Furthermore, we examined whether horizontal sound-localization abilities improved when listeners used their BCD. Ten control listeners without hearing loss demonstrated a significant decrease in their localization abilities when they listened with a monaural plug and muff. In 4/13 UCHL listeners we observed good horizontal localization of 65 dB SPL broadband noises with their BCD turned off. Localization was strongly impaired when the impaired ear was covered with the muff. The mould in the good ear of listeners with UCHL deteriorated the localization of broadband sounds presented at 45 dB SPL. This demonstrates that they used pinna cues to localize sounds presented at low levels. Our data demonstrate that UCHL listeners have learned to adapt their localization strategies under a wide variety of hearing conditions and that sound-localization abilities improved with their BCD turned on.

  14. [Auto-immune sensorineural deafness: physiopathology and therapeutic approach].

    PubMed

    Hervier, B; Bordure, P; Masseau, A; Calais, C; Agard, C; Hamidou, M

    2010-03-01

    Sensorineural hearing loss may be due to an autoimmune mechanism. The mechanisms that could induce autoimmune inner ear damage are now better understood, but are not exclusive. Moreover, there is no specific immunologic test available for the diagnosis of autoimmune sensorineural hearing loss, which could also complicate the disease course of other autoimmune systemic diseases. Thus, the incidence of sensorineural autoimmune hearing loss is probably underestimated. The aim of this study was to review the experimental immunologic data in favour of an autoimmune mechanism in this subgroup of sensorineural hearing loss: humoral specific response against inner ear (autoantibodies against a transmembrane transporter) and also cellular response (against cochlin: one of the major proteins expressed in the inner ear). The aim of this review was also to focus on clinical and epidemiological human data that provide evidence for an autoimmune etiopathogeny of some sensorineural hearing loss. Therapeutic options such as immunosuppressive treatments (oral corticosteroids and other immunosuppressive drugs, such as methotrexate and anti-TNFalpha) are also discussed.

  15. Musicians' ability to judge the risk of acquiring noise induced hearing loss.

    PubMed

    Hagerman, Björn

    2013-01-01

    The objective of this research was to study musicians' abilities to estimate the risk to obtain a hearing loss. Twenty-two professional musicians mainly playing classical music wore dosimeters during 2 working weeks. They also wrote a diary describing all their musical activities and tried to judge the percentage of time that every activity was harmful to their hearing. Half of the musicians seemed to be capable to reasonably judge the harmfulness of the music that they were exposed to. They started to judge the levels to be risky at 80 dB(A) and regarded themselves as slightly more susceptible to noise induced hearing loss than normal.

  16. The Effects of Hearing Protectors on Speech Communication and the Perception of Warning Signals

    DTIC Science & Technology

    1989-06-01

    sensorineural hearing loss (S) . While the...Figure 1. Effect of earmuffs on the audibility of a signal in noise by a subject with normal hearing (N) and one with sensorineural hearing loss (S). Upper...investigated the psychological and social effects of " sudden hearing loss " by occluding the ears of normal- hearing individuals. Subjects wore earplugs

  17. Persons with acquired profound hearing loss (APHL): how do they and their families adapt to the challenge?

    PubMed

    Hallam, Richard; Ashton, Paul; Sherbourne, Katerina; Gailey, Lorraine

    2008-07-01

    The study examined the impact of acquired profound hearing loss (APHL) on the relationship between the hearing impaired person and their normally hearing close family member, usually a partner, and identified the kinds of adjustment leading to maintenance or deterioration of the relationship. The participants were 25 people with APHL and 25 family members, interviewed separately in their own home. Analysis of the interview transcripts adopted a grounded theory methodology. The different levels of analysis were linked in terms of a core category based on the social construction of a committed relationship. The conceptual codes were grouped as: (a) aural impairments giving rise to the need for adjustment; (b) pragmatic adjustments to spoken communication and family activities; (c) managing the adjustments without negative consequences; (d) adjustments leading to negative interaction. The results suggest that APHL places considerable strain on relationships and increases their vulnerability to failure, consistent with previous research. They highlight the need for professional support and suggest that a systemic conceptual framework is needed that includes the public response to profound hearing impairment.

  18. [Genotype--phenotype correlation limits in sensorineural hearing loss: case report of a three-year-old child with a bilateral cochleovestibular impairment and a molecular variant of the COCH gene].

    PubMed

    Montava, M; Roman, S; Sigaudy, S; Marlin, S; Nicollas, R; Triglia, J M

    2012-01-01

    Mutations of the COCH gene inherited in an autosomal dominant mode are responsible for late-onset cochleovestibular impairment on both sides. Our objective is to report the youngest patient (3 years) associating a molecular variant of the COCH gene and a cochleovestibular impairment on both sides. The clinical sequence has started with a vestibular dysfunction in a two-year-old child: recurrent rotatory dizziness during 12 months. At the age of 3, a sensorineural hearing loss on both sides has occured associated with spontaneous variation during 6 months. The lack of mutation of the connexin 26, connexin 30 and pendrin genes has reorientated the genetic investigation. A molecular variant of the COCH gene was found in the vWFA2 domain. It was an in-frame deletion predicting the synthesis of an abnormal protein in which 21 aminoacid were missing. Others family members with mutation were asymptomatics. In this isolated case report, the study was in favor of a non pathogenic molecular variant of the COCH gene. For all that, mutations of the COCH gene could be searched in progressive cochleovestibular dysfunctions on both sides in children, even without family affect.

  19. Inflectional morphology in German hearing-impaired children.

    PubMed

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

    2016-01-01

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

  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.

    PubMed

    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.).

  1. Hearing

    ERIC Educational Resources Information Center

    Koehlinger, Keegan M.; Van Horne, Amanda J. Owen; Moeller, Mary Pat

    2013-01-01

    Purpose: Spoken language skills of 3- and 6-year-old children who are hard of hearing (HH) were compared with those of children with normal hearing (NH). Method: Language skills were measured via mean length of utterance in words (MLUw) and percent correct use of finite verb morphology in obligatory contexts based on spontaneous conversational…

  2. An Evaluation of the BKB-SIN, HINT, QuickSIN, and WIN Materials on Listeners with Normal Hearing and Listeners with Hearing Loss

    ERIC Educational Resources Information Center

    Wilson, Richard H.; McArdle, Rachel A.; Smith, Sherri L.

    2007-01-01

    Purpose: The purpose of this study was to examine in listeners with normal hearing and listeners with sensorineural hearing loss the within- and between-group differences obtained with 4 commonly available speech-in-noise protocols. Method: Recognition performances by 24 listeners with normal hearing and 72 listeners with sensorineural hearing…

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

    PubMed

    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.

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

    PubMed

    Desir, Julie; Abramowicz, Marc

    2008-10-15

    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 Harboyan syndrome) should

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

    PubMed

    Ma, Jing; Wan, Lang; Xu, Fen

    2015-09-01

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

  6. Genes and Syndromic Hearing Loss.

    ERIC Educational Resources Information Center

    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…

  7. Ranking Hearing Aid Input-Output Functions for Understanding Low-, Conversational-, and High-Level Speech in Multitalker Babble

    ERIC Educational Resources Information Center

    Chung, King; Killion, Mead C.; Christensen, Laurel A.

    2007-01-01

    Purpose: To determine the rankings of 6 input-output functions for understanding low-level, conversational, and high-level speech in multitalker babble without manipulating volume control for listeners with normal hearing, flat sensorineural hearing loss, and mildly sloping sensorineural hearing loss. Method: Peak clipping, compression limiting,…

  8. Renal diagnosis without renal biopsy. Nephritis and sensorineural deafness.

    PubMed

    Richardson, D; Shires, M; Davison, A M

    2001-06-01

    Two examples of hereditary nephropathy within the context of clinical syndromes are described. Emphasis is put on the ability to make a renal diagnosis without renal biopsy and the benefits of screening relatives once a diagnosis is achieved. A variant of Alport's syndrome with associated macrothrombocytic thrombocytopenia, known as Epstein's syndrome, is reported. In addition siblings with Alström's syndrome characterized by pigmentary retinal degeneration (causing blindness in early childhood), progressive sensorineural hearing loss, and progressive renal failure are reported. Both cases had previously presented for non-renal pathology in advance of the onset of symptomatic renal failure and may have benefited from appropriate screening.

  9. Niikawa-Kuroki (Kabuki) syndrome with congenital sensorineural deafness: evidence for a wide spectrum of inner ear abnormalities.

    PubMed

    Tekin, Mustafa; Fitoz, Suat; Arici, Serap; Cetinkaya, Ergun; Incesulu, Armagan

    2006-05-01

    Hearing loss, mainly due to recurrent otitis media, has been reported in approximately 40% of individuals with Niikawa-Kuroki (Kabuki) syndrome (NKS). Sensorineural hearing loss leading to congenital or prelingual deafness has been described rarely. We have identified two unrelated individuals with Niikawa-Kuroki syndrome among 535 probands who have severe to profound sensorineural deafness. Bilateral absence of the cochlea with dilated dysplastic vestibule and unilateral enlarged vestibule were demonstrated in these two individuals. In conclusion, Niikawa-Kuroki syndrome should be kept in mind when evaluating an individual with congenital deafness and a wide spectrum of inner ear abnormalities occurs in this syndrome.

  10. Sudden hearing loss in children.

    PubMed

    Ječmenica, Jovana; Bajec-Opančina, Aleksandra

    2014-08-01

    Sudden sensorineural hearing loss (SSHL) is defined as a unilateral or bilateral sensorineural hearing loss with at least 30 dB decrease in threshold in 3 contiguous test frequencies occurring over 72 hours or less. It is very rare in children. Sudden hearing loss is a symptom that suggests that there is a problem in the inner ear, surrounding structures, or the whole organism. The etiology and development of this disorder are still not fully understood. The literature contains numerous models of the pathogenesis of SSHL, with childhood SSHL having certain peculiarities. In practical terms, the multifactorial nature of SSHL is important in the choice of diagnostic methods and treatment methods. It is important to determine the cause and effect relationship between the underlying disease and hearing loss.

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

    PubMed

    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.

  12. [Hereditary sensorineural deafness].

    PubMed

    Denoyelle, F; Marlin, S; Petit, C; Garabédian, E N

    2000-01-15

    Deafness is the most common sensory defect. The investigation of the cause of deafness is critical for genetic counselling, and sometimes for appropriate management of associated pathologies. About two thirds of cases of congenital deafness are genetic forms, and the proportion is probably similar concerning the forms of deafness that appears during childhood. Some of the genetic forms are syndromic and the associated signs are sometimes inapparent or may appear during childhood. Consequently, a systematic search for the most frequent syndromes is necessary in each deaf individual. In the majority of genetic cases, deafness is the sole defect (non-syndromic deafness) and the major mode of transmission is autosomal recessive. The DFNB1 form of deafness, due to connexin 26 gene mutations, underlies half of the cases of non syndromic congenital deafness cases. The hearing loss has a prelingual onset, and it is most frequently severe or profound. There is no associated pathologies or radiological anomalies of the inner ear, and the vestibular tests are normal. The possibility of offering molecular diagnosis of connexin 26 gene defects is profoundly modifying daily medical practice in the investigation of the cause of deafness.

  13. Acquired Immune Deficiency Syndrome (AIDS) and the Veterans' Administration. Hearing before the Subcommittee on Hospitals and Health Care of the Committee on Veterans' Affairs. House of Representatives, One Hundredth Congress, First Session.

    ERIC Educational Resources Information Center

    Congress of the U.S., Washington, DC. House Committee on Veterans' Affairs.

    This document presents witness testimony and prepared statements from the Congressional hearing called to examine the issue of acquired immune deficiency syndrome (AIDS) and the role of the Veterans' Administration (VA) in combating AIDS. Opening statements are included from Representatives G. V. Montgomery, J. Roy Rowland, Joseph P. Kennedy, II,…

  14. Sudden hearing loss following non-petrous craniotomy.

    PubMed

    Ryan, J C; Bird, P A; Bonkowski, J A

    2010-01-01

    We present two patients with known otosclerosis undergoing craniotomy for conditions unrelated to the temporal bone who experienced sudden sensorineural hearing loss. In both patients, the hearing loss was noted immediately post-operatively and there was no subsequent recovery. Sudden hearing loss is a rare complication of non-otologic, non-cardiopulmonary bypass surgery. To our knowledge it has not been described in patients with otosclerosis undergoing craniotomy. This is a rare event that may occur in patients with vulnerable ears, such as those with otosclerosis or pre-existing sensorineural hearing loss.

  15. [Hearing disorders in young subjects].

    PubMed

    Kunel'skaia, N L; Skriabina, L Iu

    2014-01-01

    The objective of the present work was to study the state of the hearing function and the prevalence of ear pathologies in the applicants and students of a higher education institution. A total of 44.525 subjects at the age varying from 15 to 30 years were available for the examination of whom 42.829 ones were involved in the routine medical examination programs for the applicants and students; 1696 persons presenting with acute ear diseases and impairment of hearing passed the primary medical examination in the University polyclinic. The hearing function was evaluated with the help of the speech and tuning fork tests; in part of the patients it was evaluated by tonal threshold audiometry in both the standard and the extended frequency ranges, tympanometry, registration of the ipsilateral acoustic reflex, and a questionnaire study. Chronic ear pathology is known to occur in 1.77-2.09% of the young people; it is the third most frequent condition after diseases of throat, nose, and paranasal sinuses in the structure of chronic ENT morbidity. The structure of chronic ear diseases is dominated by Eustachian tube pathology (0.99-1.4%) followed by chronic middle and inner ear diseases (0.35-0.62% and 0.15-0.26% respectively). The commonest chronic disease of the middle ear is adhesive otitis media that is accompanied by the conductive impairment of hearing in 53.5% of the cases. Next in importance is chronic suppurative otitis media associated with the conductive or mixed-type loss of hearing in 91.7% of the patients. The subclinical form of sensorineural hearing loss was found in 11.7-15.1% of the young subjects Including minimal sensorineural hearing loss (enhancement of the hearing threshold by 1-3 frequencies) in 11.7-12.4% of the patients. A frequent cause of impaired hearing in the case of chronic sensorineural hearing loss is a single or repeated acoustic trauma.

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

    PubMed

    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.

  17. AIDS Federal Policy Act of 1987. Hearings on S. 1575: To Amend the Public Health Service Act To Establish a Grant Program To Provide for Counseling and Testing Services Relating to Acquired Immune Deficiency Syndrome and To Establish Certain Prohibitions for the Purpose of Protecting Individuals with Acquired Immune Deficiency Syndrome or Related Conditions. Committee on Labor and Human Resources. United States Senate, One Hundredth Congress, First Session.

    ERIC Educational Resources Information Center

    Congress of the U.S., Washington, DC. Senate Committee on Labor and Human Resources.

    This document presents the text from two Senate hearings on the AIDS Federal Policy Act of 1987 which concerns voluntary testing for AIDS virus, education and counseling to stop the spread of AIDS (Acquired Immune Deficiency Syndrome), and confidentiality and discrimination against AIDS victims. In the first hearing, opening statements are…

  18. The Nonfunctioning Hearing Aid: A Case of Double Jeopardy.

    ERIC Educational Resources Information Center

    Smedley, Thayne; Plapinger, Donald

    1988-01-01

    Insertion of a dead hearing aid in 15 otologically normal children (ages 4-6) resulted in a mean hearing loss of 25-30 dB. Results suggest that sensorineural hearing losses of 50 dB may increase to 80 dB with nonfunctioning aids in place, compounding a hearing-impaired child's existing communication and educational difficulties. (Author/VW)

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

    PubMed

    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).

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

    PubMed

    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.

  1. Audiometric comparison of Lassa fever hearing loss and idiopathic sudden hearing loss: evidence for viral cause.

    PubMed

    Liao, B S; Byl, F M; Adour, K K

    1992-03-01

    A recently published prospective study on acute sensorineural deafness in Lassa fever among a West African population showed the audiometric pattern of a known virally induced hearing loss. Using the audiometric data from the patients with Lassa fever in that study, we analyzed and classified the initial hearing loss and final recovery into three groups by pure-tone average values and then did the same for 222 patients with idiopathic sudden hearing loss (SHL) in our study. Statistical analyses of the severity of initial hearing loss and the hearing recovery pattern indicate that the clinical course of our 222 patients with idiopathic SHL showed no statistically significant differences from the clinical course of the patients with Lassa fever. We found a marked difference in age, however, and a clinically significant difference in the incidence of bilateral hearing loss. In reviewing the literature on sudden sensorineural hearing loss, we found no apparent relation between severity of viral illness and initial hearing loss or subsequent recovery. Cummins et al. suggest that virally induced hearing loss in Lassa fever is linked to the host's immune response and not to the viremia. We thus propose a virally induced immune response mechanism for idiopathic sensorineural SHL. Further prospective studies are needed for verification.

  2. Audiovisual spoken word training can promote or impede auditory-only perceptual learning: prelingually deafened adults with late-acquired cochlear implants versus normal hearing adults

    PubMed Central

    Bernstein, Lynne E.; Eberhardt, Silvio P.; Auer, Edward T.

    2014-01-01

    Training with audiovisual (AV) speech has been shown to promote auditory perceptual learning of vocoded acoustic speech by adults with normal hearing. In Experiment 1, we investigated whether AV speech promotes auditory-only (AO) perceptual learning in prelingually deafened adults with late-acquired cochlear implants. Participants were assigned to learn associations between spoken disyllabic C(=consonant)V(=vowel)CVC non-sense words and non-sense pictures (fribbles), under AV and then AO (AV-AO; or counter-balanced AO then AV, AO-AV, during Periods 1 then 2) training conditions. After training on each list of paired-associates (PA), testing was carried out AO. Across all training, AO PA test scores improved (7.2 percentage points) as did identification of consonants in new untrained CVCVC stimuli (3.5 percentage points). However, there was evidence that AV training impeded immediate AO perceptual learning: During Period-1, training scores across AV and AO conditions were not different, but AO test scores were dramatically lower in the AV-trained participants. During Period-2 AO training, the AV-AO participants obtained significantly higher AO test scores, demonstrating their ability to learn the auditory speech. Across both orders of training, whenever training was AV, AO test scores were significantly lower than training scores. Experiment 2 repeated the procedures with vocoded speech and 43 normal-hearing adults. Following AV training, their AO test scores were as high as or higher than following AO training. Also, their CVCVC identification scores patterned differently than those of the cochlear implant users. In Experiment 1, initial consonants were most accurate, and in Experiment 2, medial consonants were most accurate. We suggest that our results are consistent with a multisensory reverse hierarchy theory, which predicts that, whenever possible, perceivers carry out perceptual tasks immediately based on the experience and biases they bring to the task. We

  3. Timbre discrimination in normal-hearing and hearing-impaired listeners under different noise conditions.

    PubMed

    Emiroglu, Suzan; Kollmeier, Birger

    2008-07-18

    In an attempt to quantify differences in object separation and timbre discrimination between normal-hearing and hearing-impaired listeners with a moderate sensorineural hearing loss of two different configurations, psychoacoustic measurements were performed with a total of 50 listeners. The experiments determined just noticeable differences (JND) of timbre in normal-hearing and hearing-impaired subjects along continua of "morphed" musical instruments and investigated the variance of JND in silence and different background noise conditions and on different sound levels. The results show that timbre JNDs of subjects with a steep hearing loss are significantly higher than of normal-hearing subjects, both in silence and noise, whereas timbre JNDs of flat/diagonal hearing-impaired subjects are similar to JNDs of normal-hearing subjects for signal levels above 55 dB (plus appropriate amplification for hearing-impaired). In noise (SNR=+10 dB) timbre JNDs of all subject groups are significantly higher than in silence. In the condition testing, transferability from silence to noise (i.e., the ability to imagine how the stimulus heard in silence would sound in noise), no significant JND differences across listener groups were found. The results can be explained by primary factors involved in sensorineural hearing loss and contradict the hypothesis that hearing-impaired people generally have more problems in object discrimination than normal-hearing people.

  4. Temporal Intraspeech Masking of Plosive Bursts: Effects of Hearing Loss and Frequency Shaping

    ERIC Educational Resources Information Center

    Mackersie, Carol L.

    2007-01-01

    Purpose: The purposes were (a) to compare masking of consonant bursts by adjacent vowels for listeners with and without hearing loss and (b) to determine the extent to which the temporal intraspeech masking can be reduced by a simulated hearing-aid frequency-response shaping. Method: Fourteen adults with sensorineural hearing loss and 10 with…

  5. The Effects of Blast Trauma (Impulse Noise) on Hearing: A Parametric Study

    DTIC Science & Technology

    1991-05-01

    pathological cochleas using physiological and psychoacoustic methods. In humans with sensorineural hearing losses , psychophysical TCs also show a...15 B. Relation Between Hearing Loss and Impulse Noise Parameters. . 16 1. Intensity-Duration Tradeoff ..... ................ ... 16 2. Impulse... loss and the last section presents some background on the role of tuning curves in the assessment of normal and pathological hearing function. These

  6. Bilateral sensorineural deafness, partial agenesis of the corpus callosum, and arachnoid cysts in two sisters.

    PubMed

    Hendriks, Y M; Laan, L A; Vielvoye, G J; van Haeringen, A

    1999-09-10

    We describe two sisters (ages 10 and 3 years, respectively) with a normal development and a combination of congenital sensorineural hearing loss, partial agenesis of the corpus callosum, arachnoid cyst, and hydrocephalus. Neither girl has distinctive physical anomalies. In the oldest girl, there was a hearing loss of 80 dB bilaterally, and the most severe loss on audiogram was seen at 2,000-4,000 Hz. In the youngest girl, there was a hearing loss of 100 dB bilaterally. Above 2,000 Hz no neural reactions were seen. Cerebral magnetic resonance imaging in one girl and computed tomography in the other showed a partial agenesis of the corpus callosum and a cyst in the pineal region, causing an aqueduct stenosis by compression and consequent hydrocephalus. The parents have normal hearing, and brain magnetic resonance imaging showed no abnormalities. They are nonconsanguineous but from the same small village. This is the first report of a combination of congenital sensorineural hearing loss, partial agenesis of the corpus callosum, and an arachnoid cyst. The pattern of inheritance is probably autosomal recessive.

  7. Hearing Loss, Control, and Demographic Factors Influencing Hearing Aid Use among Older Adults.

    ERIC Educational Resources Information Center

    Garstecki, Dean C.; Erler, Susan F.

    1998-01-01

    Older adults (N=131) with hearing loss completed measures of hearing, hearing handicap, psychological control, depression, and ego strength. Older adults who accepted advice from hearing professionals to acquire and use hearing aids differed from those not accepting such advice on measures of hearing sensitivity, psychological control, and…

  8. Noise induced hearing loss impairs spatial learning/memory and hippocampal neurogenesis in mice

    PubMed Central

    Liu, Lijie; Shen, Pei; He, Tingting; Chang, Ying; Shi, Lijuan; Tao, Shan; Li, Xiaowei; Xun, Qingying; Guo, Xiaojing; Yu, Zhiping; Wang, Jian

    2016-01-01

    Hearing loss has been associated with cognitive decline in the elderly and is considered to be an independent risk factor for dementia. One of the most common causes for acquired sensorineural hearing loss is exposure to excessive noise, which has been found to impair learning ability and cognitive performance in human subjects and animal models. Noise exposure has also been found to depress neurogenesis in the hippocampus. However, the effect is mainly attributed to the oxidant stress of noise on the cognitive brain. In the present study, young adult CBA/CAJ mice (between 1.5 and 2 months of age) were briefly exposed a high sound level to produce moderate-to-severe hearing loss. In both the blood and hippocampus, only transient oxidative stress was observed after noise exposure. However, a deficit in spatial learning/memory was revealed 3 months after noise exposure. Moreover, the deficit was correlated with the degree of hearing loss and was associated with a decrease in neurogenesis in the hippocampus. We believe that the observed effects were likely due to hearing loss rather than the initial oxidant stress, which only lasted for a short period of time. PMID:26842803

  9. Screening of Connexin 26 in Nonsyndromic Hearing Loss

    PubMed Central

    Moreira, Danielle; Silva, Daniela da; Lopez, Priscila; Mantovani, Jair Cortez

    2014-01-01

    Introduction The first locus for nonsyndromic autosomal recessive hearing loss is on chromosome 13q11–22. The 35delG mutation is present in 80% of cases in which GJB2 is involved, which makes the study of this mutation very important. The viability and benefits of screening for mutations in the connexin 26 gene are now beginning to change the diagnostic evaluation and identification of the etiology of hearing loss. Objective To investigate the occurrence of the 35delG mutation in patients with nonsyndromic sensorineural hearing loss and their first degree relatives. Methods This transversal study included 72 patients from the local hospital. The patients were divided into three groups: group A, sensorineural hearing loss (n = 58); group B, first-degree relatives of group A with sensorineural hearing loss (n = 09); and group C, first-degree relatives of patients from group A without hearing loss (n = 05). All patients had audiological evaluation and genetic testing of the 35delG mutation. Results The 35delG mutation was found in four heterozygous mutations (three of them found in the same family). The other heterozygous mutation was found in a female patient with bilateral, moderate, prelingual, sensorineural hearing loss. A single homozygous mutation was found in a male patient, with severe sensorineural hearing loss in his right ear and profound hearing loss in the left ear. Conclusions The 35delG mutation was found in 7% of the cases. The test is easy to perform and inexpensive, but it is necessary to investigate other genes related to hearing loss. PMID:25992148

  10. [Clinical features associated with sudden hearing loss in children].

    PubMed

    Taiji, Hidenobu; Morimoto, Noriko

    2012-07-01

    Sudden sensorineural hearing loss is usually unilateral, and the cause is not identified in most adult cases. However, a specific cause has frequently been found in the case of children, in whom idiopathic sudden sensorineural hearing loss (ISHL) is comparatively rare. We investigated 20 cases of acute unilateral sensorineural hearing loss in children associated with a certain disease, but which was first diagnosed as ISHL. Thirteen patients aged 6 to 16 years old were diagnosed as having psychogenic (functional) hearing loss. Discrepancies in behavioral and objective tests are most valuable when functional hearing loss is suspected. Elevated pure-tone thresholds associated with normal distortion product otoacoustic emissions (DPOAEs) enable prompt further investigation. There are several conditions that may mimic functional hearing loss, so auditory brainstem response (ABR) testing is necessary to verify the actual cause. The unilateral profound hearing loss in 2 patients aged 7 and 11 years old was due to asymptomatic mumps proven by detecting the mumps IgM antibody. Total hearing recovery in the 125-1000 Hz frequencies occurred in one case. In 5 patients aged 6 to 12 years old with acute hearing loss and vertigo, high resolution CT imaging showed an abnormally enlarged vestibular aqueduct on the affected side. Large vestibular aqueduct syndrome should be considered in acute high-frequency sloping hearing loss with an A-B gap at low frequencies.

  11. Radiation Therapy and Hearing Loss

    SciTech Connect

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

    2010-03-01

    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.

  12. Current aspects of hearing loss from occupational and leisure noise

    PubMed Central

    Plontke, S.; Zenner, H.-P.

    2004-01-01

    Hearing loss from occupational and leisure noise numbers amongst the most frequent causes of an acquired sensorineural hearing loss. Here we present a review of up-to-date findings on the pathophysiology of acoustic injury to the inner ear, with special attention being paid to its molecular-biological and genetic aspects. Epidemiological aspects shall also be dealt with, as shall the roles of lacking recovery from occupational noise due to additional exposure by leisure noise and the combined exposure of noise and chemicals. Based on the epidemiological and pathophysiological findings and against the background of published animal-experimental, pre-clinical and clinical findings, the various approaches for prevention, protection and therapeutic intervention with acoustic trauma are discussed. Pharmacological strategies involving anti-oxidative, anti-excitotoxic and anti-apoptotic substances as well as non-pharmacological strategies like "sound conditioning" are given attention. Furthermore, systemic and local substance application as well as the therapy of acute acoustic trauma and chronic hearing problems (including modern therapy forms for comorbidities such as tinnitus) shall be delved into. PMID:22073048

  13. Middle Ear Surgery in Only Hearing Ears and Postoperative Hearing Rehabilitation

    PubMed Central

    Yoo, Myung Hoon; Kang, Byung Chul; Park, Hong Ju

    2014-01-01

    Background and Objectives The aim of this study was to evaluate surgical interventions and hearing rehabilitation in patients with chronic middle ear disease of only hearing ears. Subjects and Methods Thirty-one patients with chronic middle ear disease of only hearing ears were enrolled in this retrospective study. Patients were classified into three groups according to the hearing level: groups A [pure tone audiometry (PTA)<40], B (40≤PTA<70), and C (PTA≥70). We evaluated hearing results and patterns of auditory rehabilitation. Results The main consideration for a surgical procedure was the presence of recurrent otorrhea and structural destruction. The reasons for surgical intervention in only hearing ears were otorrhea caused by chronic otitis media (68%), cholesteatoma (29%), and cholesterol granuloma (3%). The causes of contralateral deaf ears were chronic otitis media (81%) and sensorineural hearing loss (19%). Although there was hearing deterioration in some patients with severe hearing loss (PTA≥70), all patients achieved dry ears after surgery and functional hearing using auditory rehabilitation. Hearing aids were used in most patients with moderate to moderately severe hearing loss and cochlear implants were used for auditory rehabilitation in patients with severe to profound hearing loss. Conclusions Proper evaluation and indications for surgery in only hearing ears are important for successful eradication of inflammation and hearing preservation. Surgical interventions can achieve dry ear and enable further auditory rehabilitations using hearing aids and cochlear implantation. PMID:25279226

  14. Active Duty-U.S. Army Noise Induced Hearing Injury Quarterly Surveillance Q3 2011 thru Q4 2013

    DTIC Science & Technology

    2014-06-30

    incident case rates for sensorineural hearing loss significant threshold shift, tinnitus , and Noise-Induced Hearing Loss. RECOMMENDATIONS: Commanders...2013 A-1 APPENDIX A REFERENCES Humes LE, Jollenbeck LM, Durch JS: Noise and military service: Implications for hearing loss and tinnitus . Washington...FUNCTION STUDIES TINN Tinnitus 38830 TINNITUS UNSPECIFIED TINN Tinnitus 38831 SUBJECTIVE TINNITUS TINN Tinnitus 38832 OBJECTIVE TINNITUS CPT Codes

  15. Prevalence of minimal hearing loss in South Korea

    PubMed Central

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

    2017-01-01

    This study evaluated the prevalence of minimal hearing loss (MHL) in South Korea based on the 2010 to 2012 Korea National Health and Nutrition Examination Survey. A total of 16,630 representative individuals (older than 12 years) who completed ear examinations and structured questionnaires were analyzed. Only participants who had normal tympanic membranes were included. MHL was categorized into the following three groups: 1) unilateral sensorineural hearing loss (USHL, pure-tone average (PTA) ≥ 15 dB in the affected ear), 2) bilateral sensorineural hearing loss (BSHL, 15 dB ≤ PTA < 40 dB in both ears), and 3) high-frequency sensorineural hearing loss (HFSHL, two or more high-frequency thresholds > 25 dB in either ear). To evaluate clinical symptoms, subjective hearing status, tinnitus, and quality of life of each MHL group were compared to those of normal-hearing listeners. The use of hearing aids (HAs) was also investigated in the MHL population. The prevalence of normal hearing and MHL were 58.4% and 37.4%, respectively. In univariate analyses, the prevalence of MHL increased with age. It was significantly increased in males. Regarding clinical symptoms, 13.0% and 92.1% of participants with MHL reported difficulties with hearing and annoying tinnitus, respectively. In multivariate analyses, these proportions were significantly higher in the MHL groups than in normal-hearing listeners. Participants with MHL also showed significantly lower Euro Qol-5D index scores than did normal-hearing listeners. Regarding hearing rehabilitation, among minimally hearing impaired participants with subjective hearing loss, only 0.47% of individuals used HAs. Our results reveal that MHL is common in South Korea. It is associated with significant subjective hearing loss, tinnitus, and poor quality of life. Therefore, clinicians need to pay attention to this special group and provide proper counselling and rehabilitative management. PMID:28196098

  16. 38 CFR 17.149 - Sensori-neural aids.

    Code of Federal Regulations, 2010 CFR

    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...

  17. 38 CFR 17.149 - Sensori-neural aids.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2011-07-01 2011-07-01 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...

  18. 38 CFR 17.149 - Sensori-neural aids.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2012-07-01 2012-07-01 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...

  19. 38 CFR 17.149 - Sensori-neural aids.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2013-07-01 2013-07-01 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...

  20. 38 CFR 17.149 - Sensori-neural aids.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2014-07-01 2014-07-01 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...

  1. Rituximab Not Effective for Hearing Loss in Cogan's Syndrome

    PubMed Central

    Kerr, Leslie Dubin

    2016-01-01

    Importance. Rituximab was not effective in ameliorating the hearing loss in a patient with atypical Cogan's syndrome. Observations. We report the case of a patient who developed acute bilateral uveitis and sensorineural hearing loss. A diagnosis of atypical Cogan's syndrome was made. The patient's hearing loss did not improve despite high dose steroids and azathioprine. Rituximab was administered given a recent report of its efficacy in a patient with refractory disease; however, our patient's hearing loss did not improve. Conclusion. Hearing loss in Cogan's syndrome is difficult to treat. Though rituximab was ineffective in our case, earlier administration in the disease course could be effective for future patients. PMID:27843668

  2. Attention Interchanges at Story-Time: A Case Study from a Deaf and Hearing Twin Pair Acquiring Swedish Sign Language in Their Deaf Family

    ERIC Educational Resources Information Center

    Cramer-Wolrath, Emelie

    2012-01-01

    This case study longitudinally analyzes and describes the changes of attentional expressions in interchanges between a pair of fraternal twins, 1 deaf and 1 hearing, from the age of 10-40 months, and their Deaf family members. The video-observed attentional expressions of initiating and reestablishing interchange were grouped in 5 functional…

  3. Effects of a Cognitive Behavioral Self-Help Program on Emotional Problems for People with Acquired Hearing Loss: A Randomized Controlled Trial

    ERIC Educational Resources Information Center

    Garnefski, Nadia; Kraaij, Vivian

    2012-01-01

    The aim of the study was to examine whether a cognitive-behavioral self-help program was effective in improving depressed mood and anxiety in people with acquired deafness. Participants were 45 persons with acquired deafness, randomly allocated to the Cognitive-Behavioral Self-help (CBS) group or the Waiting List Control (WLC) group. Depression…

  4. Sudden bilateral hearing loss after organophosphate inhalation.

    PubMed

    Dundar, Mehmet Akif; Derin, Serhan; Aricigil, Mitat; Eryilmaz, Mehmet Akif

    2016-12-01

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

  5. Auditory Sequential Organization among Children with and without a Hearing Loss.

    ERIC Educational Resources Information Center

    Jutras, Benoit; Gagne, Jean-Pierre

    1999-01-01

    Forty-eight children, either with or without a sensorineural hearing loss and either young (6 and 7 years old) or older (9 and 10 years old) reproduced sequences of acoustic stimuli that varied in number, temporal spacing, and type. Results suggested that the poorer performance of the hearing-impaired children was due to auditory processing…

  6. Language Development in a Hearing and a Deaf Twin with Simultaneous Bilateral Cochlear Implants

    ERIC Educational Resources Information Center

    Ruggirello, Caterina; Mayer, Connie

    2010-01-01

    This case study is an examination of the language development of a single pair of fraternal twins--one with a profound, sensorineural hearing loss who received simultaneous bilateral cochlear implants at 1 year of age and the other with normal hearing. The purpose of the study was to compare the twins' language development over time from 6 months…

  7. Venturing beyond the Sentence Level: Narrative Skills in Children with Hearing Loss

    ERIC Educational Resources Information Center

    Reuterskiold, Christina; Ibertsson, Tina; Sahlen, Birgitta

    2010-01-01

    This study explores the differences in oral narrative skills between school-age children with mild-to-moderate sensorineural hearing loss (HL) and children who have typical hearing and language development. Narrative samples were collected following a picture-elicited storytelling task. Language samples were transcribed and coded for a number of…

  8. Hearing Aids

    MedlinePlus

    ... Health Info » Hearing, Ear Infections, and Deafness Hearing Aids On this page: What is a hearing aid? ... the ear through a speaker. How can hearing aids help? Hearing aids are primarily useful in improving ...

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

    PubMed

    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. Vibrant soundbridge: a new implantable alternative to conventional hearing AIDS in children.

    PubMed

    Sia, K J; Chai, C K; Tang, I P; Prepageran, N

    2012-12-01

    The Vibrant Soundbridge is a new middle ear implantable hearing device. It was first introduced for adult patients with moderate to severe sensorineural hearing loss. With the innovation of the surgical techniques, its usage had been broadened for children and those patients with conductive and mixed hearing loss. We report first two cases of monoaural Vibrant Soundbridge implantation in Malaysia. They were children with bilateral conductive hearing loss who had failed to benefit from previous hearing aids. Floating mass transducers were attached in oval window and long process of incus respectively. Remarkable hearing yield was observed without surgical complication.

  11. Unilateral sudden hearing loss: a rare symptom of Moyamoya disease.

    PubMed

    Gül, Fatih; Berçin, Sami; Müderris, Togay; Yalçıner, Gökhan; Ünal, Özkan; Kırış, Muzaffer

    2016-01-01

    A 38-year-old female patient experienced a sudden onset of unilateral sensorineural hearing loss due to Moyamoya disease. A detailed summary of audiological and neurological findings indicated that the sudden hearing loss might be due to Moyamoya disease resulting in occlusion of posterior and middle cerebral arteries. Intravenous prednisolone and trimetazidine dihydrochloride may improve hearing thresholds and speech understanding. To our knowledge, this is the first article in the literature reporting a case of sudden hearing loss as the first manifestation of Moyamoya disease in a young adult.

  12. Identifying hearing loss by means of iridology.

    PubMed

    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 < 0.05). Although statistically significant sensitivity and specificity rates for identifying hearing loss by iridology were not comparable to those of traditional audiological screening procedures.

  13. Chiari Type I malformation presenting with bilateral hearing loss.

    PubMed

    Dolgun, Habibullah; Turkoglu, Erhan; Kertmen, Hayri; Yilmaz, Erdal R; Sekerci, Zeki

    2009-09-01

    Chiari Type I malformations can present with several clinical signs and symptoms. We describe a 44-year-old female patient presenting with bilateral hearing loss with hydrocephalus coexisting with Chiari Type I malformation and a unilateral arachnoid cyst. Thus, sensorineural hearing loss may be caused by hydrocephalus with Chiari Type I malformation. The placement of a ventriculoperitoneal shunt without a posterior fossa decompression is an effective treatment.

  14. Homozygous mutation in PTRH2 gene causes progressive sensorineural deafness and peripheral neuropathy.

    PubMed

    Sharkia, Rajech; Shalev, Stavit A; Zalan, Abdelnaser; Marom-David, Milit; Watemberg, Nathan; Urquhart, Jill E; Daly, Sarah B; Bhaskar, Sanjeev S; Williams, Simon G; Newman, William G; Spiegel, Ronen; Azem, Abdussalam; Elpeleg, Orly; Mahajnah, Muhammad

    2017-04-01

    PTRH2 is an evolutionarily highly conserved mitochondrial protein that belongs to a family of peptidyl-tRNA hydrolases. Recently, patients from two consanguineous families with mutations in the PTRH2 gene were reported. Global developmental delay associated with microcephaly, growth retardation, progressive ataxia, distal muscle weakness with ankle contractures, demyelinating sensorimotor neuropathy, and sensorineural hearing loss were present in all patients, while facial dysmorphism with widely spaced eyes, exotropia, thin upper lip, proximally placed thumbs, and deformities of the fingers and toes were present in some individuals. Here, we report a new family with three siblings affected by sensorineural hearing loss and peripheral neuropathy. Autozygosity mapping followed by exome sequencing identified a previously reported homozygous missense mutation in PTRH2 (c.254A>C; p.(Gln85Pro)). Sanger sequencing confirmed that the variant segregated with the phenotype. In contrast to the previously reported patient, the affected siblings had normal intelligence, milder microcephaly, delayed puberty, myopia, and moderate insensitivity to pain. Our findings expand the clinical phenotype and further demonstrate the clinical heterogeneity related to PTRH2 variants.

  15. Active Duty - U.S. Army Noise Induced Hearing Injury Surveillance, Calendar Years 2007-2011

    DTIC Science & Technology

    2013-01-01

    summary for calendar years (CY) 2007-2011 showed increasing incidence rates for sensorineural hearing loss (SNHL), tinnitus , and significant threshold...induced hearing loss (NIHL) and tinnitus among U.S. military members from World War II through 2005. The report’s authors concluded that military hearing...epidemiological data to reliably estimate the incidence of NIHL and tinnitus in the U.S. Armed Forces. In response to the IOM report, military audiologists

  16. Hearing Aids

    MedlinePlus

    ... Surgery? A Week of Healthy Breakfasts Shyness Hearing Aids KidsHealth > For Teens > Hearing Aids Print A A ... with certain types of hearing loss. How Hearing Aids Help So you went to audiologist and found ...

  17. Subjective and Objective Effects of Fast and Slow Compression on the Perception of Reverberant Speech in Listeners with Hearing Loss

    ERIC Educational Resources Information Center

    Shi, Lu-Feng; Doherty, Karen A.

    2008-01-01

    Purpose: The purpose of the current study was to assess the effect of fast and slow attack/release times (ATs/RTs) on aided perception of reverberant speech in quiet. Method: Thirty listeners with mild-to-moderate sensorineural hearing loss were tested monaurally with a commercial hearing aid programmed in 3 AT/RT settings: linear, fast (AT = 9…

  18. Impact of Early Intervention on Expressive and Receptive Language Development among Young Children with Permanent Hearing Loss

    ERIC Educational Resources Information Center

    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…

  19. A Review of the Literature on Large Vestibular Aqueduct Syndrome for Teachers of the Deaf and Hard of Hearing.

    ERIC Educational Resources Information Center

    Vause, Kellie; Beattie, R. G.

    1997-01-01

    This review describes the history, clinical features, methods of diagnosis, and treatment of (1) large vestibular aqueduct syndrome (LVAS), a condition involving congenital hearing loss that is purely sensorineural or mixed in nature, and (2) progressive stepwise hearing losses following minor head trauma. Emphasis is on prevention of further…

  20. [The application of implantable hearing aids using the Vibrant Soundbridge as an example].

    PubMed

    Strenger, T; Stark, T

    2012-02-01

    Over the last decade, bone conducting hearing aids, cochlear implants and implantable hearing aids have come to represent additional treatment options in clinical routine-alongside conventional hearing aids-for hearing impaired patients. Thanks to experience gained in recent years with implantable hearing aids and the consistent evaluation of functional results, the original spectrum of indications has been progressively extended. Today, implantable hearing aids are available for the hearing (re)habilitation of various forms of middle ear pathology as well as sensorineural hearing loss within the audiological criteria. With CE certification for children, the treatment of younger patients with implantable hearing aids has also become possible. Using the Vibrant Soundbridge as an example, the function, indications and contraindications of implantable hearing aids are described and the surgical procedure and post-operative care discussed.

  1. Auditory nerve synapses persist in ventral cochlear nucleus long after loss of acoustic input in mice with early-onset progressive hearing loss.

    PubMed

    McGuire, Brian; Fiorillo, Benjamin; Ryugo, David K; Lauer, Amanda M

    2015-04-24

    Perceptual performance in persons with hearing loss, especially those using devices to restore hearing, is not fully predicted by traditional audiometric measurements designed to evaluate the status of peripheral function. The integrity of auditory brainstem synapses may vary with different forms of hearing loss, and differential effects on the auditory nerve-brain interface may have particularly profound consequences for the transfer of sound from ear to brain. Loss of auditory nerve synapses in ventral cochlear nucleus (VCN) has been reported after acoustic trauma, ablation of the organ of Corti, and administration of ototoxic compounds. The effects of gradually acquired forms deafness on these synapses are less well understood. We investigated VCN gross morphology and auditory nerve synapse integrity in DBA/2J mice with early-onset progressive sensorineural hearing loss. Hearing status was confirmed using auditory brainstem response audiometry and acoustic startle responses. We found no change in VCN volume, number of macroneurons, or number of VGLUT1-positive auditory nerve terminals between young adult and older, deaf DBA/2J. Cell-type specific analysis revealed no difference in the number of VGLUT1 puncta contacting bushy and multipolar cell body profiles, but the terminals were smaller in deaf DBA/2J mice. Transmission electron microscopy confirmed the presence of numerous healthy, vesicle-filled auditory nerve synapses in older, deaf DBA/2J mice. The present results suggest that synapses can be preserved over a relatively long time-course in gradually acquired deafness. Elucidating the mechanisms supporting survival of central auditory nerve synapses in models of acquired deafness may reveal new opportunities for therapeutic intervention.

  2. Novel PTPRQ mutations identified in three congenital hearing loss patients with various types of hearing loss

    PubMed Central

    Sakuma, Naoko; Moteki, Hideaki; Azaiez, Hela; Booth, Kevin T; Takahashi, Masahiro; Arai, Yasuhiro; Shearer, A Eliot; Sloan, Christina M; Nishio, Shin-ya; Kolbe, Diana L; Iwasaki, Satoshi; Oridate, Nobuhiko; Smith, Richard J H; Usami, Shin-ichi

    2015-01-01

    Objective We present three patients with congenital sensorineural hearing loss (SNHL) caused by the novel PTPRQ mutations, including clinical manifestations and phenotypic features. Methods Two hundred and twenty (220) Japanese subjects with sensorineural hearing loss from unrelated and non-consanguineous families were enrolled in the study. Targeted genomic enrichment with massively parallel sequencing of all known non-syndromic hearing loss genes was performed to identify the genetic cause of hearing loss. Results Four novel causative PTPRQ mutations were identified in three cases. Case 1 had progressive profound SNHL with homozygous nonsense mutation. Case 2 had non-progressive profound SNHL with compound heterozygous mutation (nonsense and missense mutation). Case 3 had non-progressive moderate SNHL with compound heterozygous mutation (missense and splice site mutation). Caloric test and vestibular evoked myogenic potential (VEMP) test showed vestibular dysfunction in Case 1. Conclusion Hearing loss levels and progression among the present cases were varied, and there seem to be no obvious correlation between genotypes and the phenotypic features of their hearing loss. The PTPRQ mutation appeared to be responsible for the vestibular dysfunction. PMID:25788564

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

    PubMed

    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.

  4. Analysis of caloric test responses in sudden hearing loss.

    PubMed

    Shih, Cheng-Ping; Chou, Yu-Ching; Chen, Hsin-Chien; Lee, Jih-Chin; Chu, Yueng-Hsiang; Wang, Chih-Hung

    2017-02-01

    Sudden sensorineural hearing loss is characterized by a rapid-onset hearing loss that develops within 3 days. Vertigo may also be present. We conducted a retrospective study to investigate whether the severity of a loss of caloric function is associated with the initial hearing loss and with hearing recovery. Our study population was made up of 135 patients-67 men and 68 women, aged 25 to 71 years (mean: 50.9)-with sudden sensorineural hearing loss who had undergone bithermal caloric testing. We compared various patient factors according to patients' hearing level and their response to caloric testing. We also analyzed the canal paresis (CP) value in patients with an abnormal caloric response according to three factors: disease severity, vertigo, and hearing recovery, and we evaluated the correlation between the loss of caloric function and hearing outcomes. We found that an abnormal caloric response was significantly associated with a profound hearing loss at presentation, the presence of vertigo, and poor hearing recovery. Among patients with an abnormal caloric response, the CP value was significantly correlated with hearing recovery (r = 0.503, p = 0.001). Poor hearing recovery was seen in 80% of patients with a CP value of ≥40% but in only 25% of patients with a value of <40%; in addition, the degree of hearing recovery was worse in the patients with a CP value of ≥40% (p = 0.002). We conclude that a CP value of ≥40% is a significant prognostic factor for an unfavorable treatment outcome.

  5. The Hearing Outcomes of Cochlear Implantation in Waardenburg Syndrome

    PubMed Central

    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. PMID:27376080

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

    PubMed

    Stritzel, S; Wöhlke, A; Distl, O

    2009-02-01

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

  7. Hearing Aids

    MedlinePlus

    ... more in both quiet and noisy situations. Hearing aids help people who have hearing loss from damage ... your doctor. There are different kinds of hearing aids. They differ by size, their placement on or ...

  8. Hearing Aids

    MedlinePlus

    ... and Consumer Devices Consumer Products Hearing Aids Hearing Aids Share Tweet Linkedin Pin it More sharing options ... to restrict your daily activities. Properly fitted hearing aids and aural rehabilitation (techniques used to identify and ...

  9. [Digital vs. analog hearing aids for children. Is there a method for making an objective comparison possible?].

    PubMed

    Prinz, I; Nubel, K; Gross, M

    2002-09-01

    Until now, the assumed benefits of digital hearing aids are reflected only in subjective descriptions by patients with hearing aids, but cannot be documented adequately by routine diagnostic methods. Seventeen schoolchildren with moderate severe bilateral symmetrical sensorineural hearing loss were examined in a double-blinded crossover study. Differences in performance between a fully digital hearing aid (DigiFocus compact/Oticon) and an analogous digitally programmable two-channel hearing aid were evaluated. Of the 17 children, 13 choose the digital and 4 the analogous hearing aid. In contrast to the clear subjective preferences for the fully digital hearing aid, we could not obtain any significant results with routine diagnostic methods. Using the "virtual hearing aid," a subjective comparison and speech recognition performance task yielded significant differences. The virtual hearing aid proved to be suitable for a direct comparison of different hearing aids and can be used for double-blind testing in a pediatric population.

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

    PubMed Central

    Tambs, Kristian; Engdahl, Bo

    2015-01-01

    Purpose This study examined the association between time of onset of hearing loss (childhood vs. adulthood) and self-reported hearing handicap in adults. Methods This is a population-based cohort study of 2,024 adults (mean = 48 years) with hearing loss (binaural pure-tone average 0.5–4 kHz ≥ 20 dB HL) who completed a hearing handicap questionnaire. In childhood, the same persons (N = 2,024) underwent audiometry in a school investigation (at ages 7, 10, and 13 years), in which 129 were diagnosed with sensorineural hearing loss (binaural pure-tone average 0.5–4 kHz ≥ 20 dB HL), whereas 1,895 had normal hearing thresholds. Results Hearing handicap was measured in adulthood as the sum-score of various speech perception and social impairment items (15 items). The sum-score increased with adult hearing threshold level (p < .001). After adjustment for adult hearing threshold level, hearing aid use, adult age, sex, and socioeconomic status, there was no significant difference in hearing handicap sum-score between the group with childhood-onset hearing loss (n = 129) and the group with adult-onset hearing loss (n = 1,895; p = .882). Conclusion Self-reported hearing handicap in adults increased with hearing threshold level. After adjustment for adult hearing threshold level, this cohort study revealed no significant association between time of onset of hearing loss (childhood vs. adulthood) and self-reported hearing handicap. PMID:26649831

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

    PubMed

    Peces, R

    2000-11-01

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

  12. Level of User Satisfaction with Hearing Aids and Environment: The International Outcome Inventory for Hearing Aids

    PubMed Central

    Kozlowski, Lorena; Almeida, Gleide; Ribas, Angela

    2014-01-01

    Introduction The main function of hearing is to enable oral communication. Hearing loss impairs communication skills. Objective To evaluate the level of user satisfaction with hearing aids. Methods This is a cross-sectional group study comprising 108 subjects (56% men and 44% women). The average age of the subjects was 77 years. These subjects had been recently fitted with their hearing aids and showed sensorineural (90%) and mixed (10%) hearing loss as determined via the Questionnaire International Outcome Inventory for Hearing Aids Outcome Inventory (IOI-HA), which determined the benefit and satisfaction obtained by sound amplification. Results The hearing aids improved the quality of life of 52.78% of the patients, which was revealed by their high scores (mean = 27.3). The relationship of the user with the environment was significantly better (p < 0.001) than that of the user with the hearing aid. Conclusion IOI-HA is a simple and easy-to-use tool. Based on the results of this study, we can show a high degree of satisfaction with their hearing aids in the majority of the participants, which improved the quality of life. PMID:25992097

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

    PubMed

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

    2016-04-01

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

  14. Hearing and Hearing Protection

    DTIC Science & Technology

    2005-06-01

    of Saint-Louis (ISL) 5 rue du Général Cassagnou 68301 Saint-Louis, France Summary We study the origin of the Noise-Induced Hearing Losses ( NIHL ...hair cells in order to be able to prevent Noise-Induced Hearing Loss ( NIHL ) and to treat the acoustic trauma (see below). - Functional consequences...Financial consequences The NIHL are responsible for many expenses. Soldiers suffering large PTS can be definitively withdrawn from front line

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

    PubMed Central

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

    2016-01-01

    Introduction 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. Methods 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. Results 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. Conclusion 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. PMID:27800104

  16. Acute-onset unilateral psychogenic hearing loss in adults: report of six cases and diagnostic pitfalls.

    PubMed

    Oishi, Naoki; Kanzaki, Sho; Kataoka, Chinatsu; Tazoe, Mami; Takei, Yasuhiko; Nagai, Keiichi; Kohno, Naoyuki; Ogawa, Kaoru

    2009-01-01

    We encountered 6 rare cases of acute-onset unilateral psychogenic hearing loss in adults. All were women in their 20s and 30s. Three cases had severe hearing impairment characterized by hearing loss at every frequency; 2 cases had profound hearing impairment, and 1 case had low-frequency hearing impairment. Of the 6 cases, 3 had a history of hearing loss, and 1 had a history of psychogenic visual disturbance. All 6 cases were initially diagnosed as having idiopathic sudden sensorineural hearing loss; all subsequently received steroid therapy. Three cases were not diagnosed as being psychogenic in origin until otoacoustic emissions and auditory brain responses were performed. Although the presence of distinctive clinical features (age, gender, and past history) is important for suspecting psychogenic hearing loss, objective audiological tests such as otoacoustic emissions are essential for diagnosing some cases. Compared to the existing reports of similar cases, our cases had a poorer prognosis (only 2 cases were cured).

  17. Formal auditory training in adult hearing aid users

    PubMed Central

    Gil, Daniela; Iorio, Maria Cecília Martinelli

    2010-01-01

    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. PMID:20186300

  18. Hearing Screening

    ERIC Educational Resources Information Center

    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.

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

    PubMed

    Pankova, V B; Bushmanov, A Y U

    2014-01-01

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

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

    PubMed

    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 < 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.

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

    ERIC Educational Resources Information Center

    Cotanche, Douglas A.

    2008-01-01

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

  2. An Across-Frequency Processing Deficit in Listeners with Hearing Impairment Is Supported by Acoustic Correlation

    ERIC Educational Resources Information Center

    Healy, Eric W.; Kannabiran, Anand; Bacon, Sid P.

    2005-01-01

    It has been recently suggested that listeners having a sensorineural hearing impairment (HI) may possess a deficit in their ability to integrate speech information across different frequencies. When presented with a task that required across-frequency integration of speech patterns, listeners with HI performed more poorly than their normal-hearing…

  3. Hearing impairment in Stickler syndrome: a systematic review

    PubMed Central

    2012-01-01

    Background Stickler syndrome is a connective tissue disorder characterized by ocular, skeletal, orofacial and auditory defects. It is caused by mutations in different collagen genes, namely COL2A1, COL11A1 and COL11A2 (autosomal dominant inheritance), and COL9A1 and COL9A2 (autosomal recessive inheritance). The auditory phenotype in Stickler syndrome is inconsistently reported. Therefore we performed a systematic review of the literature to give an up-to-date overview of hearing loss in Stickler syndrome, and correlated it with the genotype. Methods English-language literature was reviewed through searches of PubMed and Web of Science, in order to find relevant articles describing auditory features in Stickler patients, along with genotype. Prevalences of hearing loss are calculated and correlated with the different affected genes and type of mutation. Results 313 patients (102 families) individually described in 46 articles were included. Hearing loss was found in 62.9%, mostly mild to moderate when reported. Hearing impairment was predominantly sensorineural (67.8%). Conductive (14.1%) and mixed (18.1%) hearing loss was primarily found in young patients or patients with a palatal defect. Overall, mutations in COL11A1 (82.5%) and COL11A2 (94.1%) seem to be more frequently associated with hearing impairment than mutations in COL2A1 (52.2%). Conclusions Hearing impairment in patients with Stickler syndrome is common. Sensorineural hearing loss predominates, but also conductive hearing loss, especially in children and patients with a palatal defect, may occur. The distinct disease-causing collagen genes are associated with a different prevalence of hearing impairment, but still large phenotypic variation exists. Regular auditory follow-up is strongly advised, particularly because many Stickler patients are visually impaired. PMID:23110709

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

    PubMed

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

    PubMed Central

    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

  6. A novel locus for non-syndromic sensorineural deafness (DFN6) maps to chromosome Xp22.

    PubMed

    del Castillo, I; Villamar, M; Sarduy, M; Romero, L; Herraiz, C; Hernández, F J; Rodríguez, M; Borrás, I; Montero, A; Bellón, J; Tapia, M C; Moreno, F

    1996-09-01

    Non-syndromic X-linked deafness is highly heterogeneous. At least five different clinical forms have been described, but only two loci have been mapped. Here we report a Spanish family affected by a previously undescribed X-linked form of hearing impairment. Deafness is non-syndromic, sensorineural, and progressive. In affected males, the auditory impairment is first detected at school age, affecting mainly the high frequencies. Later it evolves to become severe to profound, involving all frequencies for adulthood. Carrier females manifest a moderate hearing impairment in the high frequencies, with the onset delayed to the fourth decade of life. Deafness was assumed to be X-linked dominant, with incomplete penetrance and variable expressivity in carrier females. The family was genotyped for a set of microsatellite markers evenly spaced at intervals of about 10 cM. We found evidence of linkage to markers in the Xp22 region (maximum lod score of 5.30 at theta = 0.000 for DXS8036 and for DXS8022). The position of the novel deafness locus (DFN6) was refined by haplotype analysis. Mapping of the breakpoints in two critical recombinants allowed us to define an interval for DFN6, delimited by DXS7108 on the distal side and by DXS7105 on the proximal side, and spanning a genetic distance of about 15 cM.

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

    PubMed

    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.

  8. Hearing Loss in Patients with Shunt-Treated Hydrocephalus.

    PubMed

    Panova, Margarita V; Geneva, Ina E; Madjarova, Kalina I; Bosheva, Miroslava N

    2015-01-01

    Hearing loss is a common manifestation of the long-term complications in patients with shunt treated hydrocephalus along with motor development disturbance, cognitive and visual impairment, epilepsy and endocrine disorders. The aim of the present study was to investigate the alterations of hearing in patients with shunt treated hydrocephalus of non-tumor etiology and at least one year after implantation of ventriculo-peritoneal shunt, as well as their impact on the quality of life of patients. The study included 70 patients (age range 1.25 years - 21.25 years) with shunted non-tumor hydrocephalus and at least one year after placement of the shunt system. Hearing alterations were proved by measuring the brainstem auditory evoked potentials (BAEP) for children up to 5 years of age and children with mental retardation; audiograms was used for children older than 5 years with normal neuro-psychological development (NPD). Of the 70 studied patients 17 (24%) had hearing loss (10 bilateral and 7-unilateral) and all of them had sensorineural hearing loss, which is associated with low weight at birth, posthemorrhagic hydrocephalus and brainstem symptoms at the time of diagnosis of hydrocephalus. Hearing pathology was found more often in shunt-treated patients with NPD retardation, poor functional status and low quality of life. Children with shunt-treated hydrocephalus have hearing loss of sensorineural type. Children with brain stem symptomatology at diagnosing hydrocephalus and children with post-hemorrhagic hydrocephalus show higher risk of hearing loss. Children with shunted hydrocephalus and hearing loss show lower NPD, lower quality of life and lower functional status.

  9. The benefit of amplification on auditory working memory function in middle-aged and young-older hearing impaired adults.

    PubMed

    Doherty, Karen A; Desjardins, Jamie L

    2015-01-01

    Untreated hearing loss can interfere with an individual's cognitive abilities and intellectual function. Specifically, hearing loss has been shown to negatively impact working memory function, which is important for speech understanding, especially in difficult or noisy listening conditions. The purpose of the present study was to assess the effect of hearing aid use on auditory working memory function in middle-aged and young-older adults with mild to moderate sensorineural hearing loss. Participants completed two objective measures of auditory working memory in aided and unaided listening conditions. An aged matched control group followed the same experimental protocol except they were not fit with hearing aids. All participants' aided scores on the auditory working memory tests were significantly improved while wearing hearing aids. Thus, hearing aids worn during the early stages of an age-related hearing loss can improve a person's performance on auditory working memory tests.

  10. [Non-syndromic hereditary hearing impairment].

    PubMed

    Birkenhäger, R; Aschendorff, A; Schipper, J; Laszig, R

    2007-04-01

    Hearing impairment is the most common sensorineural disorder in humans. Approximately one of thousand new-borns is affected by severe to profound deafness at birth or during early childhood. Genetic causes account for around half of these cases of prelingual hearing impairment and the remainder are attributed to environmental factors. Genetic causes of hearing impairment in combination with a syndrome as Usher, Pendred are distinguished from non-syndromic hearing impairment. In the last years a tremendous growth in the localisation and identification of genes for non-syndromic hereditary hearing impairment has evolved. It has become clear that these conditions are genetically extremely heterogeneous. Approximately 120 different gene loci associated with non syndromic hearing impairment have been identified. Presently 54 gene loci associated with autosomal dominant mode of inheritance and 67 gene loci with autosomal recessive mode of inheritance have been identified; 7 are X-chromosome linked and 4 mitochondrial. Of these, 19 genes have been characterised for autosomal dominant (DFNA), 20 for autosomal recessive (DFNB), and 2 for X-linked (DFN) disorders. These genes encode proteins of diverse functions, including transcription factors, cytoskeletal and extracellular matrix components, and ion channels. Despite this heterogeneity, up to 50 % of prelingual recessive non-syndromic deafness can be attributed to mutations in the GJB2 gene (Connexin-26, gap-junction protein). However, the diversity of genes and genetic loci implicated in hearing loss illustrates the complexity of the genetic basis of hearing. Knowing the gene and the function of its products helps understanding the mechanisms of hearing.

  11. Acquired hyperpigmentations*

    PubMed Central

    Cestari, Tania Ferreira; Dantas, Lia Pinheiro; Boza, Juliana Catucci

    2014-01-01

    Cutaneous hyperpigmentations are frequent complaints, motivating around 8.5% of all dermatological consultations in our country. They can be congenital, with different patterns of inheritance, or acquired in consequence of skin problems, systemic diseases or secondary to environmental factors. The vast majority of them are linked to alterations on the pigment melanin, induced by different mechanisms. This review will focus on the major acquired hyperpigmentations associated with increased melanin, reviewing their mechanisms of action and possible preventive measures. Particularly prominent aspects of diagnosis and therapy will be emphasized, with focus on melasma, post-inflammatory hyperpigmentation, periorbital pigmentation, dermatosis papulosa nigra, phytophotodermatoses, flagellate dermatosis, erythema dyschromicum perstans, cervical poikiloderma (Poikiloderma of Civatte), acanthosis nigricans, cutaneous amyloidosis and reticulated confluent dermatitis PMID:24626644

  12. Active Duty-U.S. Army Noise Induced Hearing Injury Quarterly Surveillance Q3 2007 thru Q4 2009

    DTIC Science & Technology

    2014-05-11

    years (CY) Q3 2007-Q4 2009 shows incident case rates for sensorineural hearing loss (SNHL), significant threshold shift (STS), tinnitus , and Noise-Induced...Prev Med. 2010;38(1S):S71-S77. Humes LE, Jollenbeck LM, Durch JS. Noise and military service: Implications for hearing loss and tinnitus . Washington...threshold shift 79415 NONSPECIFIC ABNORMAL AUDITORY FUNCTION STUDIES TINN Tinnitus 38830 TINNITUS UNSPECIFIED TINN Tinnitus 38831 SUBJECTIVE TINNITUS

  13. Persistent bilateral hearing loss after shunt placement for hydrocephalus. Case report.

    PubMed

    Stoeckli, S J; Böhmer, A

    1999-04-01

    Transient hearing decrease following loss of cerebrospinal fluid (CSF) has been reported in patients undergoing lumbar puncture, spinal anesthesia, myelography, and/or different neurosurgical interventions. The authors present the first well-documented case of a patient with persistent bilateral low-frequency sensorineural hearing loss after shunt placement for hydrocephalus and discuss the possible pathophysiological mechanisms including the role of the cochlear aqueduct. These findings challenge the opinion that hearing decreases after loss of CSF are always transient. The authors provide a suggestion for treatment.

  14. Organization and administration of the NIDCD/VA Hearing Aid Clinical Trial.

    PubMed

    Henderson, William G; Larson, Vernon D; Williams, David; Leuthke, Lynn

    2002-08-01

    This article describes the organization and administration of the NIDCD/VA Hearing Aid Clinical Trial. The trial involved a total of 360 patients with bilateral, sensorineural hearing loss from eight VA Medical Centers to study three different hearing aid circuits in a three-period, three-treatment crossover design. Strong central coordination of such a complex multi-center clinical trial is essential to its success. The trial took more than 5 years to design, implement, and complete. This timeline is also described.

  15. Genetics of Nonsyndromic Congenital Hearing Loss

    PubMed Central

    Egilmez, Oguz Kadir; Kalcioglu, M. Tayyar

    2016-01-01

    Congenital hearing impairment affects nearly 1 in every 1000 live births and is the most frequent birth defect in developed societies. Hereditary types of hearing loss account for more than 50% of all congenital sensorineural hearing loss cases and are caused by genetic mutations. HL can be either nonsyndromic, which is restricted to the inner ear, or syndromic, a part of multiple anomalies affecting the body. Nonsyndromic HL can be categorised by mode of inheritance, such as autosomal dominant (called DFNA), autosomal recessive (DFNB), mitochondrial, and X-linked (DFN). To date, 125 deafness loci have been reported in the literature: 58 DFNA loci, 63 DFNB loci, and 4 X-linked loci. Mutations in genes that control the adhesion of hair cells, intracellular transport, neurotransmitter release, ionic hemeostasis, and cytoskeleton of hair cells can lead to malfunctions of the cochlea and inner ear. In recent years, with the increase in studies about genes involved in congenital hearing loss, genetic counselling and treatment options have emerged and increased in availability. This paper presents an overview of the currently known genes associated with nonsyndromic congenital hearing loss and mutations in the inner ear. PMID:26989561

  16. Consumer Preferences for Hearing Aid Attributes

    PubMed Central

    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

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

    PubMed

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

    2012-05-01

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

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

    PubMed

    Zenner, H P; Leysieffer, H

    1997-10-01

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

  19. Vowel Perception in Listeners With Normal Hearing and in Listeners With Hearing Loss: A Preliminary Study

    PubMed Central

    Charles, Lauren; Street, Nicole Drakopoulos

    2015-01-01

    Objectives To determine the influence of hearing loss on perception of vowel slices. Methods Fourteen listeners aged 20-27 participated; ten (6 males) had hearing within normal limits and four (3 males) had moderate-severe sensorineural hearing loss (SNHL). Stimuli were six naturally-produced words consisting of the vowels /i a u æ ɛ ʌ/ in a /b V b/ context. Each word was presented as a whole and in eight slices: the initial transition, one half and one fourth of initial transition, full central vowel, one-half central vowel, ending transition, one half and one fourth of ending transition. Each of the 54 stimuli was presented 10 times at 70 dB SPL (sound press level); listeners were asked to identify the word. Stimuli were shaped using signal processing software for the listeners with SNHL to mimic gain provided by an appropriately-fitting hearing aid. Results Listeners with SNHL had a steeper rate of decreasing vowel identification with decreasing slice duration as compared to listeners with normal hearing, and the listeners with SNHL showed different patterns of vowel identification across vowels when compared to listeners with normal hearing. Conclusion Abnormal temporal integration is likely affecting vowel identification for listeners with SNHL, which in turn affects vowel internal representation at different levels of the auditory system. PMID:25729492

  20. Is Sudden Hearing Loss Associated with Atherosclerosis?

    PubMed Central

    Rajati, Mohsen; Azarpajooh, Mahmoud Reza; Mouhebati, Mohsen; Nasrollahi, Mostafa; Salehi, Maryam; Khadivi, Ehsan; Nourizadeh, Navid; Hashemi, Firoozeh; Bakhshaee, Mehdi

    2016-01-01

    Introduction: Sudden sensorineural hearing-loss (SSNHL) patients constitute approximately 2–3% of referrals to ear, nose and throat (ENT) clinics. Several predisposing factors have been proposed for this condition; one of which is vascular disorders and perfusion compromise. In this research the atherosclerotic changes and their known risk factors are studied in SSNHL patients. Materials and Methods: Thirty SSNHL patients and 30 controls were evaluated with regard to cardiovascular risks including history, heart examination, blood pressure, body mass index, waist circumference, electrocardiogram, blood sugar, triglycerides, cholesterol, high-sensitivity C-reactive protein (HSCRP); also, carotid artery color Doppler study was undertaken to measure intima media thickness(IMT). Results: IMT and HSCRP showed an increased risk in the case group compared with the controls (P= 0.005 & P=0.001). However, waist circumference, history of smoking, fasting blood sugar, lipid profile, and electrocardiogram revealed no significant difference between the two groups. Interestingly, blood pressure and body mass index were higher in the controls in this study. Conclusion: Sudden sensorineural hearing loss may be associated with subclinical atherosclerosis. PMID:27429947

  1. Vowel perception by noise masked normal-hearing young adults

    NASA Astrophysics Data System (ADS)

    Richie, Carolyn; Kewley-Port, Diane; Coughlin, Maureen

    2005-08-01

    This study examined vowel perception by young normal-hearing (YNH) adults, in various listening conditions designed to simulate mild-to-moderate sloping sensorineural hearing loss. YNH listeners were individually age- and gender-matched to young hearing-impaired (YHI) listeners tested in a previous study [Richie et al., J. Acoust. Soc. Am. 114, 2923-2933 (2003)]. YNH listeners were tested in three conditions designed to create equal audibility with the YHI listeners; a low signal level with and without a simulated hearing loss, and a high signal level with a simulated hearing loss. Listeners discriminated changes in synthetic vowel tokens /smcapi e ɛ invv æ/ when F1 or F2 varied in frequency. Comparison of YNH with YHI results failed to reveal significant differences between groups in terms of performance on vowel discrimination, in conditions of similar audibility by using both noise masking to elevate the hearing thresholds of the YNH and applying frequency-specific gain to the YHI listeners. Further, analysis of learning curves suggests that while the YHI listeners completed an average of 46% more test blocks than YNH listeners, the YHI achieved a level of discrimination similar to that of the YNH within the same number of blocks. Apparently, when age and gender are closely matched between young hearing-impaired and normal-hearing adults, performance on vowel tasks may be explained by audibility alone.

  2. Types of Hearing Aids

    MedlinePlus

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

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

    PubMed

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

    2015-01-01

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

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

    PubMed Central

    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

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

    PubMed

    Pottackal Mathai, Jijo; Mohammed, Hasheem

    2017-02-01

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

  6. Spatial release from masking in normally hearing and hearing-impaired listeners as a function of the temporal overlap of competing talkersa

    PubMed Central

    Best, Virginia; Mason, Christine R.; Kidd, Gerald

    2011-01-01

    Listeners with sensorineural hearing loss are poorer than listeners with normal hearing at understanding one talker in the presence of another. This deficit is more pronounced when competing talkers are spatially separated, implying a reduced “spatial benefit” in hearing-impaired listeners. This study tested the hypothesis that this deficit is due to increased masking specifically during the simultaneous portions of competing speech signals. Monosyllabic words were compressed to a uniform duration and concatenated to create target and masker sentences with three levels of temporal overlap: 0% (non-overlapping in time), 50% (partially overlapping), or 100% (completely overlapping). Listeners with hearing loss performed particularly poorly in the 100% overlap condition, consistent with the idea that simultaneous speech sounds are most problematic for these listeners. However, spatial release from masking was reduced in all overlap conditions, suggesting that increased masking during periods of temporal overlap is only one factor limiting spatial unmasking in hearing-impaired listeners. PMID:21428524

  7. Do Hearing Protectors Protect Hearing?

    PubMed Central

    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

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

    PubMed Central

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

    2016-01-01

    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 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. Conclusion We found that the developed SHSE had valuable internal consistency, test–retest reliability, and convergent and construct

  9. Endocranial anatomy of lambeosaurine hadrosaurids (Dinosauria: Ornithischia): a sensorineural perspective on cranial crest function.

    PubMed

    Evans, David C; Ridgely, Ryan; Witmer, Lawrence M

    2009-09-01

    Brain and nasal cavity endocasts of four corythosaurian lambeosaurines (Dinosauria: Ornithischia) were investigated to test hypotheses of cranial crest function related to sensorineural systems. Endocasts were generated through computed tomography and three-dimensional rendering and visualization software. The sample comprises a range of ontogenetic stages from the taxa Lambeosaurus, Corythosaurus, and Hypacrosaurus. Results show that the morphology of brain endocasts differs little from that of hadrosaurines. The strikingly convoluted nasal vestibule of Hypacrosaurus altispinus, when interpreted in the context of lambeosaurine phylogeny, suggests selective pressure for nasal cavity function independent from changes in the external shape of the crest and associated visual display function. The plesiomorphically small olfactory bulbs and apparently small olfactory region of the nasal cavity argues against the hypothesis that increased olfactory acuity played a causal role in crest evolution. The elongate cochlea of the inner ear reveals that hearing in lambeosaurines emphasized low frequencies consistent with the hypothesized low-frequency calls made by the crests under the resonation model of crest function. The brain is relatively large in lambeosaurines compared with many other large dinosaurs, and the cerebrum is relatively larger than that of all non-hadrosaurian ornithischians and large theropods, but compares favorably with hadrosaurine hadrosaurids as well as some maniraptoran theropods. It is concluded that the large brains of lambeosaurines are consistent with the range of social behaviors inferred when the crest is interpreted as an intraspecific signaling structure.

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

    PubMed

    Kluth, Susanne; Distl, Ottmar

    2013-01-01

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

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

    PubMed

    Blum, Meike; Distl, Ottmar

    2014-01-01

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

  12. The novel c.247_249delTTC (p.F83del) GJB2 mutation in a family with prelingual sensorineural deafness.

    PubMed

    Petersen, Michael B; Grigoriadou, Maria; Koutroumpe, Maria; Kokotas, Haris

    2012-07-01

    Non-syndromic hearing loss is one of the most common hereditary determined diseases in human, and the disease is a genetically heterogeneous disorder. Mutations in the GJB2 gene, encoding connexin 26 (Cx26), are a major cause of non-syndromic recessive hearing impairment in many countries and are largely dependent on ethnic groups. Due to the high frequency of the c.35delG GJB2 mutation in the Greek population, we have previously suggested that Greek patients with sensorineural, non-syndromic deafness should be tested for the c.35delG mutation and the coding region of the GJB2 gene should be sequenced in c.35delG heterozygotes. Here we present on the clinical and molecular genetic evaluation of a family suffering from prelingual, sensorineural, non-syndromic deafness. A novel c.247_249delTTC (p.F83del) GJB2 mutation was detected in compound heterozygosity with the c.35delG GJB2 mutation in the proband and was later confirmed in the father, while the mother was homozygous for the c.35delG GJB2 mutation. We conclude that compound heterozygosity of the novel c.247_249delTTC (p.F83del) and the c.35delG mutations in the GJB2 gene was the cause of deafness in the proband and his father.

  13. Prevalence of p.V37I variant of GJB2 among Chinese infants with mild or moderate hearing loss.

    PubMed

    Huang, Yue; Yang, Xiao-Lin; Chen, Wen-Xia; Duan, Bo; Lu, Ping; Wang, Yan; Xu, Zheng-Min

    2015-01-01

    GJB2 accounts for more than 80% of recessive forms of hereditary hearing loss (HL); however, the correlation between the p.V37I variant of GJB2 and hearing phenotype is controversial. This study aimed to investigate the clinical and epidemiological characteristics of the p.V37I variant in sensorineural hearing loss in Chinese infants (0-3 months). Hearing and gene tests were conducted in 300 infants (aged 0-3 months) with sensorineural hearing impairment and 484 normal infants (aged 0-3 months). Among the 300 hearing-impaired infants, 16 (5.33%) exhibited homozygous p.V37I variation and 7 (2.34%) showed a compound-heterozygous p.V37I variation, whereas no homozygous p.V37I (0%) or compound-heterozygous p.V37I (0%) condition was found among the 484 normal infants. The hearing impairment ranged from mild to profound in all patients exhibiting the homozygous p.V37I or the compound-heterozygous p.V37I condition, although most patients (61.54%) exhibit mild or moderate HL. Our results indicated that the p.V37I variation of GJB2 mutation is mainly associated with mild or moderate hearing impairment. Therefore, otolaryngologists should also screen the p.V37I variant of GJB2 in patients with mild or moderate HL.

  14. Degree of satisfaction among hearing aid users

    PubMed Central

    Mondelli, Maria Fernanda Capoani Garcia; Rocha, Andressa Vital; Honório, Heitor Marques

    2013-01-01

    Summary Introduction: Hearing loss (HL) is defined as the complete or partial loss of hearing ability. Aims: To characterize (1) the degree of satisfaction among adult and elderly hearing aid (HA) users who were treated by a public hearing health service and (2) the relationship between satisfaction and the variables of gender, age, degree of HL, and type of HA. Method: The clinical and experimental study included the administration of the Satisfaction with Amplification in Daily Life (SADL) questionnaire to 110 patients who had used HAs for more than 3 months and were 18 years of age or older. Results: Test patients were sex-balanced (48% were women) and had a mean age of 67 years. A relatively high incidence of sensorineural moderate HL was detected in the study patients (66%) and device B was the most commonly used HA type (48%). No significant differences were evident between HA satisfaction and sex. The importance placed on services/costs and personal image varied between age groups. Correlation was evident at all levels between user satisfaction and amplification. Decreased satisfaction was observed in individuals with severe and/or profound HL. The type of HA used yielded statistically significant differences in the positive effects referring. Conclusion: No correlations were evident between the different factors proposed. HA users exhibited high levels of satisfaction in all SADL areas. PMID:25991994

  15. Small fenestra stapedectomy technique: reducing risk and improving hearing.

    PubMed

    Bailey, H A; Pappas, J J; Graham, S S

    1983-10-01

    During the past 25 years many variations have emerged in stapedectomy, most of which centered around either a change in the prosthesis itself or in the type of oval window seal. The small fenestra stapedectomy technique (SFT) represents a change in surgical procedure rather than in prosthetic design. This technique offers the opportunity to improve hearing results while reducing risks in stapedectomy surgery. Four areas of significant improvement are seen in patients in whom the SFT was used: (1) improved hearing in the high frequencies of 2000, 4000, and 8000 Hz, (2) improved speech discrimination scores, (3) a significant reduction in the number of reported vestibular complaints, and (4) a reduction in the number of serious postoperative sensorineural hearing losses.

  16. The Effect of Smoking on the Hearing Status–A Hospital Based Study

    PubMed Central

    Kumar, Adesh; Gulati, Rajiv; Singhal, Sangeeta; Hasan, Abrar; Khan, Asif

    2013-01-01

    Background: Tobacco smoking has been known to affect the human physiology and among the various damaging effects of tobacco, it has been linked with its effect on the sense of hearing. Aim and Objective: This study was designed with the aim of finding the relationship between smoking and hearing loss in various age groups. Materials and Methods: This study included 148 subjects among which 108 were smokers and 40 were age matched non smokers. The smoking history of all the subjects whose ages ranged from 20 to 60 years was taken in detail and their audiometric thresholds were recorded in a sound proof room by a professional audiometrist. The data was analyzed by using appropriate statistical tests. Observation and Results: Smoking was found to be signifi- cantly associated with hearing loss. Also, the hearing loss was mainly of the sensorineural type, with the mild type (26-40 dB) of hearing loss being the most common among the smokers. PMID:23542766

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

    PubMed Central

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

    2013-01-01

    Summary 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. PMID:25992015

  18. The Neural Representation of Consonant-Vowel Transitions in Adults Who Wear Hearing Aids

    PubMed Central

    Tremblay, Kelly L.; Kalstein, Laura; Billings, Cuttis J.; Souza, Pamela E.

    2006-01-01

    Hearing aids help compensate for disorders of the ear by amplifying sound; however, their effectiveness also depends on the central auditory system's ability to represent and integrate spectral and temporal information delivered by the hearing aid. The authors report that the neural detection of time-varying acoustic cues contained in speech can be recorded in adult hearing aid users using the acoustic change complex (ACC). Seven adults (50–76 years) with mild to severe sensorineural hearing participated in the study. When presented with 2 identifiable consonant-vowel (CV) syllables (“shee” and “see”), the neural detection of CV transitions (as indicated by the presence of a P1-N1-P2 response) was different for each speech sound. More specifically, the latency of the evoked neural response coincided in time with the onset of the vowel, similar to the latency patterns the authors previously reported in normal-hearing listeners. PMID:16959736

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

    PubMed

    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.

  20. Comparing Loudness Tolerance and Acceptable Noise Level in Listeners With Hearing Loss.

    PubMed

    Franklin, Clifford A; White, Letitia J; Franklin, Thomas C; Livengood, Lindsay G

    2016-08-01

    The present study was a follow-up investigation to a previous study exploring the relationship between listeners' loudness tolerance and listeners' acceptable noise level among normally hearing adults. The present study compared the same two measures, but data were obtained from listeners with hearing loss; 12 adults with sensorineural hearing loss participated in a loudness tolerance measure using a scaling technique initially established for setting hearing aid output limits, in addition to an acceptable noise level measure. The acceptable noise level procedure used in this study quantified the listeners' acceptance of background noise while listening to speech. As with the research involving listeners with normal hearing, the Pearson correlation procedure indicated a lack of any statistically significant correlation between the two measures.

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

    PubMed

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

    2010-05-15

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

  2. Progressive hereditary hearing impairment caused by a MYO6 mutation resembles presbyacusis.

    PubMed

    Oonk, A M M; Leijendeckers, J M; Lammers, E M; Weegerink, N J D; Oostrik, J; Beynon, A J; Huygen, P L M; Kunst, H P M; Kremer, H; Snik, A F M; Pennings, R J E

    2013-05-01

    Since deafness is the most common sensorineural disorder in humans, better understanding of the underlying causes is necessary to improve counseling and rehabilitation. A Dutch family with autosomal dominantly inherited sensorineural hearing loss was clinically and genetically assessed. The MYO6 gene was selected to be sequenced because of similarities with other, previously described DFNA22 phenotypes and a pathogenic c.3610C > T (p.R1204W) mutation was found to co-segregate with the disease. This missense mutation results in a flat configured audiogram with a mild hearing loss, which becomes severe to profound and gently to steeply downsloping later in life. The age-related typical audiograms (ARTA) constructed for this family resemble presbyacusis. Speech audiometry and results of loudness scaling support the hypothesis that the phenotype of this specific MYO6 mutation mimics presbyacusis.

  3. Clinical aspects of hereditary hearing loss.

    PubMed

    Kochhar, Amit; Hildebrand, Michael S; Smith, Richard J H

    2007-07-01

    Hearing loss is an etiologically diverse condition with many disease-related complications and major clinical, social, and quality of life implications. As the rate of acquired hearing loss secondary to environmental causes decreases and improvements in the diagnosis of abnormalities occur, the significance of genetic factors that lead to deafness increases. Advancements in molecular biology have led to improved detection and earlier intervention in patients with hearing loss. Subsequently, earlier implementation of educational services and cochlear implant technology in patients with profound hearing loss now results in superior communication skills and enhanced language development. The aim of this review is to provide a comprehensive framework underlying the causes of hearing impairment and to detail the clinical management for patients with hereditary hearing loss.

  4. Effect of Early Intervention on Language Development in Hearing-Impaired Children

    PubMed Central

    Shojaei, Elahe; Jafari, Zahra; Gholami, Maryam

    2016-01-01

    Introduction: Hearing loss from birth up to the age of 3 years has a negative effect on speech/language development and results in sensory, cognitive, emotional, and academic defects in adulthood by causing delayed development of communicative-linguistic abilities. The present study was performed in order to assess the effect of early intervention on language development in Persian children aged 6-7 years with severe sensorineural hearing loss. Materials and Methods: Thirty boys and girls aged 6-7 years participated in this study, all of them had severe congenital sensorineural hearing loss in both ears. All children were using bilateral behind-the-ear hearing aid, and had similar economic/socio-cultural backgrounds. Subjects were categorized into two groups based on the age of identification/intervention of hearing loss (3-6 and 12-15 months of age). The Persian TOLD-P3 test was used to evaluate language development in all subjects. Data collection was accomplished by observation, completion of questionnaires, and speech recording. Results: There was a significant difference in language development in 11 sub-tests and five lingual gains on the Persian TOLD-P3 test between early (3-6 months of age) and late identified/intervened (12-15 months of age) hearing-impaired children (P<0.05). Early identified/intervened hearing-impaired children had a notable preference in all assessed sub-tests and lingual gains. Conclusion: Early identification/intervention of hearing loss before the age of 6 months has a significant positive effect on a child’s language development in terms of picture/relational/oral vocabulary, grammatical comprehension, sentence combining, grammatical completion, phonologic analysis, word differentiation, word production, semantics, and syntax. Moreover, early identification/ intervention of hearing loss develops the hearing-impaired child’s lingual gains in visual vocabulary, grammatical completion, word differentiation, phonologic analysis, and

  5. Hearing Aids

    MedlinePlus

    ... hair cells (outer and inner rows). When the vibrations move through this fluid, the tiny outer hair ... ear to the brain. Hearing aids intensify sound vibrations that the damaged outer hair cells have trouble ...

  6. Hearing Impairment

    MedlinePlus

    ... up of invisible waves of energy, causes these vibrations. Hearing begins when sound waves that travel through ... When the eardrum vibrates, the ossicles amplify these vibrations and carry them to the inner ear. The ...

  7. Hearing Loss

    MedlinePlus

    ... topic was provided by the National Institute on Deafness and Other Communication Disorders Topic last reviewed: December ... a total loss of hearing. It can be hereditary or it can result from disease, trauma, certain ...

  8. Hearing Loss

    MedlinePlus

    ... and heart screening. If your baby doesn’t pass his newborn hearing screening, it doesn’t always ... be screened again. If your baby doesn’t pass a second time, it’s very important that he ...

  9. Hearing Loss

    MedlinePlus

    ... turn the volume higher when you listen to music, the radio or television Some causes of hearing ... levels include snowmobiling, motorcycling or listening to loud music. Some medications. Drugs, such as the antibiotic gentamicin ...

  10. Hearing Impairment

    MedlinePlus

    ... known as noise-induced hearing loss (NIHL) . Personal music players are among the chief culprits of NIHL ... exposure to high noise levels (such as loud music) over time can cause permanent damage to the ...

  11. About Hearing

    MedlinePlus

    ... Technology Cochlear Implants Deaf Culture Deaf Education Deaf Students with Disabilities Early Intervention, Early Childhood Education Family ... Technology Cochlear Implants Deaf Culture Deaf Education Deaf Students with Disabilities Early Intervention Family Resources Hearing Aids ...

  12. Fish Hearing.

    ERIC Educational Resources Information Center

    Blaxter, J. H. S.

    1980-01-01

    Provides related information about hearing in fish, including the sensory stimulus of sound in the underwater environment, mechanoreceptors in fish, pressure perception and the swimbladder, specializations in sound conduction peculiar to certain fish families. Includes numerous figures. (CS)

  13. Sudden hearing loss and vertigo after tooth extraction successfully treated with combined therapy including HBO2: a case report.

    PubMed

    Yilmaz, Huseyin Baki; Erdogan, Raziye Banu Atalay; Paksoy, Mustafa; Sanli, Arif

    2015-01-01

    Sudden sensorineural hearing loss (SSNHL) is a decrease in hearing of at least 30 dB that occurs within three days and which affects at least three consecutive frequencies in either ear or both ears. This case report describes a woman who had sudden hearing loss and vertigo in the right ear after tooth extraction. As the first-line therapy, systemic and intratympanic steroid injections were used this led to a slight improvement; however, the majority of improvement in hearing was not observed until hyperbaric oxygen (HBO2) therapy was instituted on the 20th day of hearing loss. Sudden hearing loss and vertigo after tooth extraction is an otologic emergency and early evaluation and treatment are effective. HBO2, although employed beyond the Undersea and Hyperbaric Medical Society's recommended initial 14 days of symptom onset, very was effective for this particular case.

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

    PubMed Central

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

    2016-01-01

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

  15. Active Duty - U.S. Army Noise Induced Hearing Injury Surveillance, Calendar Years 2008-2012

    DTIC Science & Technology

    2014-03-03

    summary for calendar years 2008–2012 shows incident case rates for sensorineural hearing loss, significant threshold shift, tinnitus , and Noise-Induced...and tinnitus . Appendix B also presents Current Procedural Terminology (CPT) codes and DOD occupation codes used in this data summary. Appendix C...STS, NIHL, and Tinnitus ) was analyzed using a regression model to determine the trend of incidence rates from 2007 to the current year. Statistical

  16. Musicians change their tune: how hearing loss alters the neural code.

    PubMed

    Parbery-Clark, Alexandra; Anderson, Samira; Kraus, Nina

    2013-08-01

    Individuals with sensorineural hearing loss have difficulty understanding speech, especially in background noise. This deficit remains even when audibility is restored through amplification, suggesting that mechanisms beyond a reduction in peripheral sensitivity contribute to the perceptual difficulties associated with hearing loss. Given that normal-hearing musicians have enhanced auditory perceptual skills, including speech-in-noise perception, coupled with heightened subcortical responses to speech, we aimed to determine whether similar advantages could be observed in middle-aged adults with hearing loss. Results indicate that musicians with hearing loss, despite self-perceptions of average performance for understanding speech in noise, have a greater ability to hear in noise relative to nonmusicians. This is accompanied by more robust subcortical encoding of sound (e.g., stimulus-to-response correlations and response consistency) as well as more resilient neural responses to speech in the presence of background noise (e.g., neural timing). Musicians with hearing loss also demonstrate unique neural signatures of spectral encoding relative to nonmusicians: enhanced neural encoding of the speech-sound's fundamental frequency but not of its upper harmonics. This stands in contrast to previous outcomes in normal-hearing musicians, who have enhanced encoding of the harmonics but not the fundamental frequency. Taken together, our data suggest that although hearing loss modifies a musician's spectral encoding of speech, the musician advantage for perceiving speech in noise persists in a hearing-impaired population by adaptively strengthening underlying neural mechanisms for speech-in-noise perception.

  17. [A clinical study on 106 infant cases who received detailed hearing tests after newborn hearing screening].

    PubMed

    Okano, Takayuki; Iwai, Noriko; Taniguchi, Mirei; Ito, Juichi

    2014-10-01

    Newborn hearing screening (NHS) has been conducted widely in Japan in the last decade, however, there seems to be some confusion regarding the significance of NHS or management of the results obtained from NHS among clinics and practitioners. The system of NHS in Japan should be improved and refined through continuous evaluation of NHS, in terms of cost effectiveness in particular, so that NHS can be conducted more efficiently and effectively. To achieve this goal, the authors thought it important to clarify the current status and roles of our department as a facility for infants with congenital hearing impairment. In the present study, we studied 106 infant cases who were referred to the Department of Otolaryngology in Kyoto University Hospital after NHS before the age of twelve months in a period of seven years from 2006 to 2012 via retrospective chart reviewing. 79.2% of 96 infants who were qualified as referred either unilaterally or bilaterally following NHS were diagnosed as having hearing impairment in any form, either unilateral or bilateral, or conductive and/or sensorineural. The positive agreement rate was 88.7% in 53 cases who were qualified as referred bilaterally in NHS, demonstrating a high reliability of the NHS system. Twenty-four cases were diagnosed as having the need for hearing aids and were assigned to treatment and education. All the infants who underwent cochlear implantation in our department had severe bilateral hearing impairment of more than 105 dBnHL in both ears at the first examination. Moreover, a number of infants who were qualified as having passed in both ears in NHS or who had failed to receive NHS at birth were revealed as having hearing impairment and needed treatment later in the first year of their life, suggesting that NHS should be conducted in combination with periodical health checkups by family practitioners in order to identify infants with hearing impairment earlier in their life with higher efficacy.

  18. Cochlear implantation: a biomechanical prosthesis for hearing loss

    PubMed Central

    Yawn, Robert; Hunter, Jacob B.; Sweeney, Alex D.

    2015-01-01

    Cochlear implants are a medical prosthesis used to treat sensorineural deafness, and one of the greatest advances in modern medicine. The following article is an overview of cochlear implant technology. The history of cochlear implantation and the development of modern implant technology will be discussed, as well as current surgical techniques. Research regarding expansion of candidacy, hearing preservation cochlear implantation, and implantation for unilateral deafness are described. Lastly, innovative technology is discussed, including the hybrid cochlear implant and the totally implantable cochlear implant. PMID:26097718

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

    PubMed

    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.

  20. Two large French pedigrees with non syndromic sensorineural deafness and the mitochondrial DNA T7511C mutation: evidence for a modulatory factor.

    PubMed

    Chapiro, Elise; Feldmann, Delphine; Denoyelle, Françoise; Sternberg, Damien; Jardel, Claude; Eliot, Marie-Madeleine; Bouccara, Didier; Weil, Dominique; Garabédian, Eréa-Noel; Couderc, Rémy; Petit, Christine; Marlin, Sandrine

    2002-12-01

    Hearing impairment is the most frequent sensory defect in children, with a genetic basis in about 50% of cases. Several point mutations and deletions in mitochondrial DNA (mtDNA) have been identified in non-syndromic sensorineural hearing loss (NSSNHL). Beside the frequent A1555G mutation, a number of mutations in tRNAs have been reported recently, but their incidence remains unknown. We identified the T7511C mutation in the tRNASer(UCN) gene in two French families with isolated deafness. Maternal transmission was obvious in both. The 15 patients with hearing impairment exhibited a variable disease phenotype in terms of onset, severity, and progression. T7511C was present in all the patients screened. Homoplasmic and heteroplasmic levels were observed and did not correlate with the severity of the disease. T7511C was also present in 12 hearing offspring of the oldest deaf mothers, confirming the existence of modulatory factors. Our data suggest that this mtDNA mutation should be screened for in all cases of familial NSSNHL compatible with maternal transmission.

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

    SciTech Connect

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

    2008-11-01

    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.

  2. Cochlear implants as a treatment option for unilateral hearing loss, severe tinnitus and hyperacusis.

    PubMed

    Ramos Macías, Angel; Falcón González, Juan Carlos; Manrique, Manuel; Morera, Constantino; García-Ibáñez, Luis; Cenjor, Carlos; Coudert-Koall, Chrystellel; Killian, Matthijs

    2015-01-01

    Tinnitus is an incapacitating condition commonly affecting cochlear implant (CI) candidates. The aim of this clinical study is to assess the long-term effects of CI treatment in patients with severe-to-profound, sensorineural, unilateral hearing loss (UHL) and incapacitating tinnitus. We performed a prospective Cochlear™ company-sponsored multicentre study in five Spanish centres. Sixteen patients with UHL and incapacitating tinnitus, which was indicated by a Tinnitus Handicap Inventory (THI) score >58%, received a Nucleus® CI in their deaf ear. The study design includes repeated within-subject measures on hearing, tinnitus, hyperacusis and quality of life up to 12 months after initial CI fitting. In addition to hearing loss and tinnitus, all patients suffered from hyperacusis. Most patients had a sudden hearing loss and received a CI within 2 years after their hearing loss. Preliminary 6-month, post-CI activation data of 13 subjects showed that the majority of patients perceived a subjective benefit from CI treatment, which was assessed using the THI, a Visual Analogue Scale of tinnitus loudness/annoyance and the Speech, Spatial and Qualities of Hearing Scale. Preliminary 12-month data of 7 subjects showed that most patients also perceived a degree of relief from their hyperacusis. One patient showed no improvements in any of the applied scales, which could be explained by partial insertion of the electrode due to obstruction of the cochlea by otosclerosis. In conclusion, CI can successfully be used in the treatment of UHL patients with accompanying severe tinnitus and hyperacusis. Implantation resulted in hearing benefits and a durable relief from tinnitus and hyperacusis in the majority of patients. These findings support the hypothesis that pathophysiological mechanisms after peripheral sensorineural hearing loss are at least partly reversible when hearing is restored with a CI.

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

    NASA Astrophysics Data System (ADS)

    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.

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

    PubMed Central

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

    2014-01-01

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

  5. Hereditary hearing loss with thyroid abnormalities.

    PubMed

    Choi, Byung Yoon; Muskett, Julie; King, Kelly A; Zalewski, Christopher K; Shawker, Thomas; Reynolds, James C; Butman, John A; Brewer, Carmen C; Stewart, Andrew K; Alper, Seth L; Griffith, Andrew J

    2011-01-01

    Mutations in SLC26A4 can cause deafness and goiter in Pendred syndrome (PDS) or isolated non-syndromic enlargement of the vestibular aqueduct (NSEVA). PDS is one of the most common hereditary causes of deafness. It is characterized by autosomal-recessive inheritance of sensorineural hearing loss, enlarged vestibular aqueducts (EVA), and an iodide organification defect with or without goiter. The diagnosis is confirmed by detection of two mutant alleles of SLC26A4 in a patient with EVA. The perchlorate discharge test can detect the underlying thyroid biochemical defect and is useful for the evaluation of goiter or for the clinical diagnosis of PDS in a patient with a non-diagnostic SLC26A4 genotype. SLC26A4 encodes the pendrin polypeptide, an anion exchanger that, in recombinant expression systems, transports chloride, bicarbonate, and iodide. Investigation of pendrin function in the inner ear has been facilitated by the Slc26a4(Δ) (knockout) mouse model, but the exact mechanism of its hearing loss remains unclear, as does pendrin's principal transport function in the inner ear. Treatment of PDS is focused upon rehabilitation of hearing loss, and surveillance and management of goiter and, less commonly, hypothyroidism.

  6. Acquired Cystic Kidney Disease

    MedlinePlus

    ... They Work Kidney Disease A-Z Acquired Cystic Kidney Disease What is acquired cystic kidney disease? Acquired cystic kidney disease happens when a ... cysts. What are the differences between acquired cystic kidney disease and polycystic kidney disease? Acquired cystic kidney ...

  7. 'Ecstasy' enhances noise-induced hearing loss.

    PubMed

    Church, Michael W; Zhang, Jinsheng S; Langford, Megan M; Perrine, Shane A

    2013-08-01

    'Ecstasy' or 3,4-methylenedioxy-N-methamphetamine (MDMA) is an amphetamine abused for its euphoric, empathogenic, hallucinatory, and stimulant effects. It is also used to treat certain psychiatric disorders. Common settings for Ecstasy use are nightclubs and "rave" parties where participants consume MDMA and dance to loud music. One concern with the club setting is that exposure to loud sounds can cause permanent sensorineural hearing loss. Another concern is that consumption of MDMA may enhance such hearing loss. Whereas this latter possibility has not been investigated, this study tested the hypothesis that MDMA enhances noise-induced hearing loss (NIHL) by exposing rats to either MDMA, noise trauma, both MDMA and noise, or neither treatment. MDMA was given in a binge pattern of 5 mg/kg per intraperitoneal injections every 2 h for a total of four injections to animals in the two MDMA-treated groups (MDMA-only and Noise + MDMA). Saline injections were given to the animals in the two non-MDMA groups (Control and Noise-only). Following the final injection, noise trauma was induced by a 10 kHz tone at 120 dB SPL for 1 h to animals in the two noise trauma-treated groups (Noise-only and Noise + MDMA). Hearing loss was assessed by the auditory brainstem response (ABR) and cochlear histology. Results showed that MDMA enhanced NIHL compared to Noise-only and that MDMA alone caused no hearing loss. This implies that "clubbers" and "rave-goers" are exacerbating the amount of NIHL when they consume MDMA and listen to loud sounds. In contrast to earlier reports, the present study found that MDMA by itself caused no changes in the click-evoked ABR's wave latencies or amplitudes.

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

    ERIC Educational Resources Information Center

    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…

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

    SciTech Connect

    Brown, K.A.; Nobel, A.; Markham, A.F.

    1994-09-01

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

  10. Frequency Discrimination and Literacy Skills in Children with Mild to Moderate Sensorineural Hearing Loss

    ERIC Educational Resources Information Center

    Halliday, L. F.; Bishop, D. V. M.

    2005-01-01

    It has been suggested that specific reading disability (SRD) may be attributable to an impaired ability to perceive spectral differences between sounds that leads to a deficit in frequency discrimination and subsequent problems with language and literacy. The objective of the present study was threefold. We aimed to (a) determine whether children…

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

    NASA Astrophysics Data System (ADS)

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

    2002-05-01

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

  12. Sudden hearing loss due to oxaliplatin use in a patient with colon cancer.

    PubMed

    Güvenç, M Güven; Dizdar, Denizhan; Dizdar, Senem Kurt; Okutur, Sadi Kerem; Demir, Gökhan

    2016-08-01

    Oxaliplatin is used to treat advanced colorectal cancer. Platinum-containing chemotherapeutic agents are known to be ototoxic. However, ototoxicity is rare with newer generation platinum-derived agents, such as oxaliplatin. This case report presents a rare case of sudden unilateral sensorineural hearing loss following intravenous (IV) infusion of oxaliplatin in a 64-year-old woman with advanced colon cancer. The hearing loss was severe and did not respond to treatment. To the best of our knowledge, this is the fifth reported case of oxaliplatin ototoxicity. Although oxaliplatin ototoxicity is rare, physicians must be aware of this important adverse effect, and an audiometric evaluation must be performed when necessary. Patients treated with oxaliplatin should be followed closely for early signs and symptoms of hearing loss, and if hearing loss is detected, treatment should be stopped immediately.

  13. Hearing Problems

    MedlinePlus

    ... guns, driving a truck or listening to loud music for long periods of time?YesNoAre you experiencing ... CareSee your doctor.Start OverDiagnosisYou may have MENIERE'S DISEASE or a more serious TUMOR on the hearing ...

  14. Distortion product otoacoustic emissions: an objective technique for the screening of hearing loss in children treated with platin derivatives.

    PubMed

    Dhooge, Ingeborg; Dhooge, Catharina; Geukens, Sven; De Clerck, Bieke; De Vel, Eddy; Vinck, Bart M

    2006-06-01

    In order to develop a sensitive audiometric protocol for identifying ototoxicity in children, a retrospective study of 16 children treated with cisplatin and/or carboplatin was performed. Audiometric testing was done by means of pure-tone threshold audiometry (PTA), high-frequency audiometry (HFA), and distortion product otoacoustic emissions (DPOAEs). Cisplatin caused a sensorineural high-frequency hearing loss in the study group compared to the controls (p < 0.01). Sixty-six percent of the cisplatin patients had a grade 2 or 3 ototoxicity. However, ototoxicity was not found in the patients treated with carboplatin. An excellent correlation was found between DPOAE levels and results obtained by audiometry (r = 0.82). Patients exposed to cisplatin are at significant risk for the development of drug-induced sensorineural hearing loss. Because of the several advantages of DPOAEs (noninvasive, objective, rapid, easy to use, sensitive) this method should be added in the audiological follow-up in infants and toddlers.

  15. Neural tracking of attended versus ignored speech is differentially affected by hearing loss.

    PubMed

    Petersen, Eline Borch; Wöstmann, Malte; Obleser, Jonas; Lunner, Thomas

    2017-01-01

    Hearing loss manifests as a reduced ability to understand speech, particularly in multitalker situations. In these situations, younger normal-hearing listeners' brains are known to track attended speech through phase-locking of neural activity to the slow-varying envelope of the speech. This study investigates how hearing loss, compensated by hearing aids, affects the neural tracking of the speech-onset envelope in elderly participants with varying degree of hearing loss (n = 27, 62-86 yr; hearing thresholds 11-73 dB hearing level). In an active listening task, a to-be-attended audiobook (signal) was presented either in quiet or against a competing to-be-ignored audiobook (noise) presented at three individualized signal-to-noise ratios (SNRs). The neural tracking of the to-be-attended and to-be-ignored speech was quantified through the cross-correlation of the electroencephalogram (EEG) and the temporal envelope of speech. We primarily investigated the effects of hearing loss and SNR on the neural envelope tracking. First, we found that elderly hearing-impaired listeners' neural responses reliably track the envelope of to-be-attended speech more than to-be-ignored speech. Second, hearing loss relates to the neural tracking of to-be-ignored speech, resulting in a weaker differential neural tracking of to-be-attended vs. to-be-ignored speech in listeners with worse hearing. Third, neural tracking of to-be-attended speech increased with decreasing background noise. Critically, the beneficial effect of reduced noise on neural speech tracking decreased with stronger hearing loss. In sum, our results show that a common sensorineural processing deficit, i.e., hearing loss, interacts with central attention mechanisms and reduces the differential tracking of attended and ignored speech.

  16. Improvement of sudden bilateral hearing loss after vertebral artery stenting

    PubMed Central

    Kim, Ji Hwa; Roh, Kyung Jin; Suh, Sang Hyun; Lee, Kyung-Yul

    2015-01-01

    Bilateral deafness is a rare but possible symptom of vertebrobasilar ischemia. We report a case of sudden bilateral sensorineural hearing loss caused by bilateral vertebral artery (VA) occlusion which dramatically improved after stenting. A 54-year-old man was admitted with sudden onset of bilateral deafness, vertigo, and drowsy mental status. Brain diffusion-weighted MRI showed acute infarction involving both the posterior inferior cerebellar artery and left posterior cerebral artery territory. Cerebral angiography showed bilateral distal VA occlusion, and emergency intracranial stenting was performed in the left VA. After reperfusion therapy his symptoms gradually improved, including hearing impairment. Endovascular stenting may be helpful in a patient with sudden deafness caused by bilateral VA occlusion. PMID:25697296

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

    PubMed Central

    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

  18. Frequency selectivity in canaries with a hereditary hearing loss

    NASA Astrophysics Data System (ADS)

    Lauer, Amanda M.; Dooling, Robert J.

    2002-05-01

    Sensorineural hearing loss is associated with reduced frequency selectivity due to the broadening of the auditory filters in mammals. In European starlings, hearing loss caused by the ototoxic drug kanamycin results in the widening of auditory filters at 5 kHz [Marean et al. (1998)]. In the present study, we examine frequency selectivity in a bird with a permanent hereditary hearing impairment, the Belgian Waterslager (BWS) canary. This strain of canary has long been bred for its loud, low-frequency song, and has been shown to have a hearing loss primarily at higher frequencies (2 kHz and above). Using operant conditioning and the method of constant stimuli, thresholds for detecting pure tones in flat-spectrum broadband noise were measured in BWS and non-BWS canaries. Critical ratios were calculated for comparison with other species of birds. At higher frequencies, critical ratios for BWS canaries were much larger than those of non-BWS canaries and other birds, suggesting reduced frequency selectivity in the region of the birds' hearing loss. [Work supported by NIDCD R01DC001372 to RJD and Brenda M. Ryals.

  19. Evaluation of hearing functions in patients with euthyroid Hashimoto's thyroiditis.

    PubMed

    Arduc, Ayse; Isık, Serhat; Allusoglu, Serpil; Iriz, Ayse; Dogan, Bercem Aycicek; Gocer, Celil; Tuna, Mazhar Muslim; Berker, Dilek; Guler, Serdar

    2015-12-01

    Sensorineural hearing loss has been reported in various autoimmune diseases. The relationship between Hashimoto's thyroiditis (HT) and the auditory system has not been previously evaluated. In this study, we investigated the effect of euthyroid HT on the hearing ability of adult patients. The study included 30 patients with newly diagnosed euthyroid HT and 30 age- and gender-matched healthy controls. All subjects had a normal otoscopic examination and tympanometry, and they were negative for rheumatoid factor, antinuclear, anti-smooth muscle, antimitochondrial, antineutrophilcytoplasmic, and antigliadin antibodies. Pure tone audiometry exams at 250, 500, 1000, 2000, 4000, 6000, and 8000 Hertz (Hz) were performed in both groups. Thyroid peroxidase antibody and thyroglobulin antibody (anti-Tg) levels were higher in HT group while TSH, free T4, free T3, plasma electrolytes, glucose, lipid profile, vitamin B12, and blood pressure measurements were similar between the two groups. Higher audiometric thresholds and a higher prevalence of hearing loss at 250, 500, and 6000 Hz were detected in the HT patients than in the healthy controls (P < 0.05). Hearing levels at 250 and 500 Hz correlated positively with anti-Tg levels (ρ = 0.650, P = 0.002; ρ = 0.719, P < 0.001, respectively), and this association remained significant in linear regression analysis. Anti-Tg-positive HT patients had higher hearing thresholds at 250 and 500 Hz than anti-Tg-negative HT patients. Hearing thresholds were similar between anti-Tg-negative HT patients and the control subjects. This study demonstrated that hearing functions are impaired in HT patients. Thyroid autoimmunity seems to have an important impact on a decreased hearing ability, particularly at lower frequencies, in this population of patients.

  20. Association between idiopathic hearing loss and mitochondrial DNA mutations: A study on 169 hearing-impaired subjects

    PubMed Central

    GUARAN, VALERIA; ASTOLFI, LAURA; CASTIGLIONE, ALESSANDRO; SIMONI, EDI; OLIVETTO, ELENA; GALASSO, MARCO; TREVISI, PATRIZIA; BUSI, MICOL; VOLINIA, STEFANO; MARTINI, ALESSANDRO

    2013-01-01

    Mutations in mitochondrial DNA (mtDNA) have been shown to be an important cause of sensorineural hearing loss (SNHL). In this study, we performed a clinical and genetic analysis of 169 hearing-impaired patients and some of their relatives suffering from idiopathic SNHL, both familial and sporadic. The analysis of four fragments of their mtDNA identified several polymorphisms, the well known pathogenic mutation, A1555G, and some novel mutations in different genes, implying changes in the aminoacidic sequence. A novel sporadic mutation in 12S rRNA (MT-RNR1), not previously reported in the literature, was found in a case of possible aminoglycoside-induced progressive deafness. PMID:23969527

  1. Buying a Hearing Aid

    MedlinePlus

    ... aids typically cannot be custom-fit. What are costs and styles of hearing aids? Hearing aids vary ... and for improvement in hearing tones. Real ear measurements may also be done, which determine how much ...

  2. Informational Masking in Normal-Hearing and Hearing-Impaired Listeners Measured in a Nonspeech Pattern Identification Task.

    PubMed

    Roverud, Elin; Best, Virginia; Mason, Christine R; Swaminathan, Jayaganesh; Kidd, Gerald

    2016-04-08

    Individuals with sensorineural hearing loss (SNHL) often experience more difficulty with listening in multisource environments than do normal-hearing (NH) listeners. While the peripheral effects of sensorineural hearing loss certainly contribute to this difficulty, differences in central processing of auditory information may also contribute. To explore this issue, it is important to account for peripheral differences between NH and these hearing-impaired (HI) listeners so that central effects in multisource listening can be examined. In the present study, NH and HI listeners performed a tonal pattern identification task at two distant center frequencies (CFs), 850 and 3500 Hz. In an attempt to control for differences in the peripheral representations of the stimuli, the patterns were presented at the same sensation level (15 dB SL), and the frequency deviation of the tones comprising the patterns was adjusted to obtain equal quiet pattern identification performance across all listeners at both CFs. Tonal sequences were then presented at both CFs simultaneously (informational masking conditions), and listeners were asked either to selectively attend to a source (CF) or to divide attention between CFs and identify the pattern at a CF designated after each trial. There were large differences between groups in the frequency deviations necessary to perform the pattern identification task. After compensating for these differences, there were small differences between NH and HI listeners in the informational masking conditions. HI listeners showed slightly greater performance asymmetry between the low and high CFs than did NH listeners, possibly due to central differences in frequency weighting between groups.

  3. Informational Masking in Normal-Hearing and Hearing-Impaired Listeners Measured in a Nonspeech Pattern Identification Task

    PubMed Central

    Best, Virginia; Mason, Christine R.; Swaminathan, Jayaganesh; Kidd, Gerald

    2016-01-01

    Individuals with sensorineural hearing loss (SNHL) often experience more difficulty with listening in multisource environments than do normal-hearing (NH) listeners. While the peripheral effects of sensorineural hearing loss certainly contribute to this difficulty, differences in central processing of auditory information may also contribute. To explore this issue, it is important to account for peripheral differences between NH and these hearing-impaired (HI) listeners so that central effects in multisource listening can be examined. In the present study, NH and HI listeners performed a tonal pattern identification task at two distant center frequencies (CFs), 850 and 3500 Hz. In an attempt to control for differences in the peripheral representations of the stimuli, the patterns were presented at the same sensation level (15 dB SL), and the frequency deviation of the tones comprising the patterns was adjusted to obtain equal quiet pattern identification performance across all listeners at both CFs. Tonal sequences were then presented at both CFs simultaneously (informational masking conditions), and listeners were asked either to selectively attend to a source (CF) or to divide attention between CFs and identify the pattern at a CF designated after each trial. There were large differences between groups in the frequency deviations necessary to perform the pattern identification task. After compensating for these differences, there were small differences between NH and HI listeners in the informational masking conditions. HI listeners showed slightly greater performance asymmetry between the low and high CFs than did NH listeners, possibly due to central differences in frequency weighting between groups. PMID:27059627

  4. Hearing rehabilitation with single-stage bilateral vibroplasty in a child with Franceschetti syndrome.

    PubMed

    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.

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

    PubMed Central

    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 mg twice daily. Audiometry test showed moderate to severe neuronal hearing loss and reduced speech recognition in both ears of the patient. With suspicion of hydroxychloroquine-induced hearing loss, this drug was discontinued. After 2 months of hydroxychloroquine discontinuation, his audiometry findings were improved. Discussion A few cases of hydroxychloroquine-induced hearing loss have been reported. All of the cases were non-HIV positive individuals. Irreversible hearing loss was developed following long-term therapy with hydroxychloroquine. The present case was a HIV-positive man who developed hearing loss following short course (one month) hydroxychloroquine therapy and his problem was resolved following discontinuation of hydroxychloroquine and continuation of prednisolone. Conclusions Hydroxychloroquine-induced hearing loss may reversibly occur following short term therapy in HIV patients. PMID:24450358

  6. How to Get Hearing Aids

    MedlinePlus

    ... Consumer Products Hearing Aids How to get Hearing Aids Share Tweet Linkedin Pin it More sharing options ... my hearing aids? How do I get hearing aids? Before getting a hearing aid, you should consider ...

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

    PubMed

    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.

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

    PubMed Central

    Pathak, Shresh

    2016-01-01

    Objectives Autoinflammatory diseases are a family of immune‐mediated, rare diseases, some of which, exhibit sensorineural hearing loss (SNHL), suggesting potentially similar mechanisms of molecular pathogenesis between autoinflammatory‐mediated hearing loss and autoimmune inner ear disease (AIED) may exist. The purpose of this review is to compare the clinical features of autoimmune and autoinflammatory diseases that affect hearing, discuss the limitations of our knowledge, and highlight potential new disease mechanisms and therapeutics. Data sources Pubmed Literature Review; Google Scholar Literature review. Review methods A focused comparison of AIED with a number of autoinflammatory diseases that manifest with sensorineural hearing loss was performed. The pathogenesis of these diseases is reviewed in the context of the innate and adaptive immune system, cytokine expression and genetic polymorphisms. Results AIED, since first described by Cogan and Lehnhardt and first clinically characterized by McCabe, has remained an enigmatic disease, with limited advances in both new diagnostics and new therapeutics. Since the discovery of autoinflammatory diseases, a number of systemic autoimmune diseases have either been re‐classed as autoinflammatory diseases or identified to have features of autoinflammatory disease. Conclusion AIED has clinical features of both autoimmune and autoinflammatory disease. It is critical that autoinflammatory diseases be correctly identified, as failure to do so may result in systemic amyloidosis and kidney damage. PMID:27917401

  9. Preliminary evaluation of a light based Contact Hearing Device for the hearing impaired

    PubMed Central

    Fay, Jonathan P.; Perkins, Rodney; Levy, Suzanne Carr; Nilsson, Michael; Puria, Sunil

    2013-01-01

    Objective To assess the safety, stability, and performance of the broad spectrum, light based Contact Hearing Device (CHD) on listeners with hearing impairment. Study Design Feasibility study. Setting Single-Site Research and Development Facility. Subjects Thirteen subjects with symmetric mild to severe sensorineural hearing impairment had the CHD placed bilaterally. Intervention A custom-molded light activated Tympanic Contact Actuator (TCA) was placed into each ear by a physician, where it stayed in contact with the umbo and a portion of the medial wall of the ear canal for four months. Each CHD was calibrated and programmed to provide appropriate broad-spectrum amplification. Main Outcome Measures Safety was determined through routine otologic examinations. Aided and pre-TCA-insertion unaided audiometric thresholds, Reception Threshold for Sentences (RTS), and Abbreviated Profile of Hearing Aid Benefit (APHAB) measurements were made to characterize system performance as well as the benefits of amplification via the CHD. Results The TCAs remained on subjects’ ears for an average total of 122 days, without causing signs of inflammation or infection, and there were no serious device-related adverse events. Measured average maximum output of 90–110 dB SPL in the 0.25–10 kHz range, average maximum gain before feedback of 40 dB, and functional gain through 10 kHz show extended bandwidth broad spectrum output and gain. RTS results showed significant aided improvements of up to 2.8 dB, and APHAB results showed clinically significant aided benefits in 11/12 (92%) subjects. Conclusion The safety, stability, and performance demonstrated in this initial 4-month study suggest that the CHD may offer a feasible way of providing broad-spectrum amplification appropriate to treat listeners with mild to severe hearing impairment. PMID:23524632

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

    PubMed

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

    2015-01-01

    This study aimed to investigate the p19 expression in cisplatin-treated rats and the role of p19 in the degeneration of inner ear cells. It also searched for p19 gene alterations in patients with profound sensorineural deafness. P19ink4d is essential for the postmitotic maintenance of hair cells. It is presumed that a mutation in the functional homolog of p19 or a disturbance in its regulated expression can be the underlying cause of hearing loss. Experiments were conducted on male and female Sprague-Dawley rats (aged 6-7 weeks, 280-320 g) with thresholds of auditory brainstem responses <30 dB in the sound pressure level, and signs of middle ear infection were used for the experiment. For clinical evaluation, 400 children (age less than 13 years) from unrelated families with severe or profound sensorineural hearing loss (SNHL) were recruited at the second Xiangya Hospital of Central South University between 2005 and 2013, and genomic DNA for deafness gene analysis was obtained from peripheral blood samples of the patients and their lineal relatives. It was found that the p19 expression increased over time in the inner ear cells after cisplatin administration, but the p19 mRNA and protein levels significantly decreased in rats with manifested hearing loss induced by cisplatin. However, no mutation existed within the coding exons of p19 in the patients with profound sensorineural deafness. To conclude, the results support the concept that p19 may play an important role in the ototoxic effects of cisplatin and is probably involved in the pathogenesis of hearing loss.

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

    SciTech Connect

    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.

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

    PubMed

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

    2014-12-01

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

  13. Hearing Dysfunction in Xpa-Deficient Mice

    PubMed Central

    Shinomiya, Hitomi; Yamashita, Daisuke; Fujita, Takeshi; Nakano, Eiji; Inokuchi, Go; Hasegawa, Shingo; Otsuki, Naoki; Nishigori, Chikako; Nibu, Ken-ichi

    2017-01-01

    Xeroderma pigmentosum (XP) is a rare recessive heredity disease caused by DNA repair impairment characterized by photosensitivity and neurologic symptoms in half of the cases. There are eight subtypes of XP: XP-A–XP-G and XP variant. Among eight subtypes, XP complementation group A (XP-A) display the lowest DNA repair ability and the severest cutaneous and neurologic symptoms. While its pathogenesis of skin symptoms have been well-studied, that of neurological symptoms, including sensorineural hearing loss (SNHL) remains unknown. Basic studies have suggested that SNHL may be caused by inner ear damage, including damage to the spiral ganglion neurons and organ of Corti, and that the XP-A is associated with most severe form of SNHL in humans. Here, we report the occurrence of SNHL in Xpa-deficient mice. Xpa-deficient mice and wild-type mice underwent measurements for auditory brainstem response, and the results revealed that Xpa-deficient mice exhibited significantly greater (p < 0.01) ABR thresholds at 4, 8, and 16 kHz than the wild-type mice. Furthermore, the number of spiral ganglion neurons was reduced in Xpa-deficient mice compared with that in wild-type mice, indicating that hearing loss may be related to spiral ganglion neuron deficiency, consistent with the few reports published in human patients with XP. These results provide important insights into the pathogenesis of SNHL in patients with XP-A. PMID:28239347

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

    PubMed

    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

  15. New Program Builds Bridges for Hearing Impaired.

    ERIC Educational Resources Information Center

    Johnson, Jeannie

    1998-01-01

    Describes a new approach to teaching English-as-a-Second-Language to New Mexico elementary school students who have hearing impairments and whose dominant language is American Sign Language (ASL). These students must first acquire ASL as a bridge to learning English. Then, using the bilingual approach, they are able to focus on English literacy.…

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

    PubMed

    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.

  17. Relationship between Speech Perception and Level of Satisfaction of Hearing Aid Users

    PubMed Central

    Mantello, Erika Barioni; Silva, Carla Dias da; Massuda, Eduardo Tanaka; Hyppolito, Miguel Angelo; Reis, Ana Cláudia Mirândola Barbosa dos

    2015-01-01

    Introduction Hearing difficulties can be minimized by the use of hearing aids. Objective The objective of this study is to assess the speech perception and satisfaction of hearing aids users before and after aid adaptation and to determine whether these measures are correlated. Methods The study was conducted on 65 individuals, 54% females and 46% males aged 63 years on average, after the systematic use of hearing aids for at least three months. We characterized subjectś personal identification data, the degree, and configuration of hearing loss, as well as aspects related to adaptation. We then applied a satisfaction questionnaire and a speech perception test (words and sentences), with and without the use of the hearing aids. Results Mean speech recognition with words and sentences was 69% and 79%, respectively, with hearing aids use; whereas, without hearing aids use the figures were 43% and 53%. Mean questionnaire score was 30.1 points. Regarding hearing loss characteristics, 78.5% of the subjects had a sensorineural loss, 20% a mixed loss, and 1.5% a conductive loss. Hearing loss of moderate degree was present in 60.5% of cases, loss of descending configuration in 47%, and plain loss in 37.5%. There was no correlation between individual satisfaction and the percentages of the speech perception tests applied. Conclusion Word and sentence recognition was significantly better with the use of the hearing aids. The users showed a high degree of satisfaction. In the present study, there was no correlation observed between the levels of speech perception and levels of user satisfaction measured with the questionnaire. PMID:27746833

  18. A longitudinal study on postoperative hearing thresholds with the Vibrant Soundbridge device.

    PubMed

    Vincent, C; Fraysse, B; Lavieille, J-P; Truy, E; Sterkers, O; Vaneecloo, F-M

    2004-10-01

    The Vibrant Soundbridge is a semi-implantable middle ear hearing device used in the rehabilitation of adults with sensorineural hearing loss. In order to evaluate the long-term effects of the implanted part of the device, audiological data from 39 patients implanted over several implant sites across France were collected and analyzed retrospectively. The mean follow-up time was 16 months; 25 patients had a follow-up period of over 1 year. Surgery was uneventful in all cases. The present study of the 39 implanted patients with a mid- to long-term follow-up found a statistically significant modification of hearing thresholds (pre- versus postoperative) for frequencies of 0.5 and 4 kHz. However, the shift of threshold was rather limited (2.79 and 3.34 dB, respectively), and this variation was not statistically different from the evolution of the opposite non-operated ear.

  19. Evaluation of the noise reduction system in a commercial digital hearing aid.

    PubMed

    Alcántara, José L; Moore, Brian C J; Kühnel, Volker; Launer, Stefan

    2003-01-01

    We evaluated the effectiveness of a noise reduction system implemented in a commercial digital multichannel compression hearing aid. Eight experienced hearing aid wearers with moderate sensorineural hearing loss were fitted bilaterally according to the manufacturer's fitting guidelines. After a 3-month period of regular use of two programs, one with and one without the noise reduction system, speech recognition thresholds (SRTs) were measured in four types of background noise, including steady noise, and noises with spectral and/or temporal dips. SRTs were very similar with and without the noise reduction system; in both cases, SRTs were markedly lower than for unaided listening. SRTs were lower for the noises with dips than for the steady noise, especially for the aided conditions, indicating that amplification can help to 'listen in the dips'. Ratings of sound quality and listening comfort in the aided conditions were uniformly high and very similar with and without the noise reduction system.

  20. Maternally inherited hearing impairment in a family with the mitochondrial DNA A7445G mutation.

    PubMed

    Hutchin, T P; Lench, N J; Arbuzova, S; Markham, A F; Mueller, R F

    2001-01-01

    Despite the increasing number of reports of families with hearing impairment and mitochondrial DNA (mtDNA) mutations, the frequency of these mutations as causes of non-syndromic sensorineural hearing impairment (NSSHI) remains unknown. Mutations such as A1555G, A7445G and 7472insC have been found in several unrelated families implying they are more frequent than initially thought. We describe a family with NSSHI due to the presence of the homoplasmic mtDNA A7445G mutation in the tRNASer(UCN) gene. This is the fourth such family described with this mutation, all of different genetic backgrounds. Our study also demonstrates the difficulties sometimes encountered in establishing mitochondrial inheritance of hearing impairment in some families.

  1. The Acoustic Change Complex in Young Children with Hearing Loss: A Preliminary Study.

    PubMed

    Martinez, Amy S; Eisenberg, Laurie S; Boothroyd, Arthur

    2013-01-01

    The acoustic change complex (ACC) is a cortical auditory evoked potential elicited in response to a change in an ongoing sound. The ACC may have promise for assessing speech perception in infants and toddlers. In this preliminary study, the ACC was elicited in adults and young children in response to changes in speech stimuli representing vowel height /u/-/a/ and vowel place /u/-/i/ contrasts. The participants were adults with normal hearing (n = 3), children with normal hearing (n = 5), and children with mild to moderately severe bilateral sensorineural hearing loss (n = 5). The children with hearing loss were hearing aid users. The ages ranged from 2 years 3 months to 6 years 3months for the children and 44 to 55 years for the adults. Robust P1-N1-P2 responses were present for the adults and P1-N2 responses were present for all but the youngest child with hearing loss. The ACC response for the vowel place contrast was less robust than that for the vowel height contrast in one child with substantial hearing loss. The findings from this preliminary study support the conclusion that the ACC can be used successfully to assess auditory resolution in most young children.

  2. Speech-recognition performance after long-term hearing aid use.

    PubMed

    Shanks, Janet E; Wilson, Richard H; Stelmachowicz, Patricia; Bratt, Gene W; Williams, David W

    2007-04-01

    Larson et al (2000) reported the findings of a multicenter, NIDCDNA clinical trial that compared hearing aid performance for three output limiting circuits in 360 adults with symmetrical sensorineural hearing loss. The current study was undertaken to examine long-term hearing aid benefit in this same group of participants following five to six years of hearing aid use. The speech-recognition portion of the follow-up study enrolled 108 participants from the original study, 85% of whom were current hearing aid users and 15% of whom had not worn hearing aids during the past month (nonusers). Recognition performance in sound field on the NU-6 (quiet at 62 dB SPL) and the CST (quiet at 74 dB SPL and with -3 and 3 dB signal-to-babble ratios [S/B] at 62 and 74 dB SPL) was measured unaided and aided whenever possible. Speech-recognition abilities decreased significantly since the original study. Speech-recognition decrements were observed regardless of the speech materials (NU-6 and CST), test condition (quiet and noise), S/B (-3 and 3 dB), or stimulus level (62 and 74 dB SPL). Despite decreases in speech recognition, hearing aid benefit remained largely unchanged since the original study; aided performance exceeded unaided performance regardless of presentation level or noise condition. As in the original study, the relations among stimulus level, S/B, and speech-recognition performance were complex.

  3. Evaluation of hearing loss in juvenile insulin dependent patients with diabetes mellitus

    PubMed Central

    Okhovat, Sayyed Ahmadreza; Moaddab, Mohammad Hassan; Okhovat, Sayyed Hanif; Al-Azab, Anwar Abdullah Ali; Saleh, Fadhl Ali Ahmad; Oshaghi, Samira; Abdeyazdan, Zahra

    2011-01-01

    BACKGROUND: Diabetes mellitus is one of the most important epidemics of our era. Complications of this disease are diverse and include retinopathy, nephropathy and neuropathy. This study has been designed to evaluate hearing loss patterns in young children suffering from IDDM and define risk factors for this complication. METHODS: This descriptive analytic study includes 200 youngsters divided into two groups: 100 patients in diabetic group and 100 healthy individual in control group. Hearing thresholds are determined in 250, 500, 1000, 2000, 4000 and 8000 Hz and metabolic controls are evaluated as average of one year HbA1C, dividing diabetic group into well control and poor control subgroups. RESULTS: Twenty one out of 100 patients in diabetic group showed significant hearing loss. Hearing loss is correlated with metabolic control, showing less loss in patients with HbA1C less than 7.5%. Results showed that hearing loss is not related to sex of patients but duration of disease (more or less than 5 years) affects degree of hearing loss in some frequencies. CONCLUSIONS: Hearing loss in children suffering from IDDM is sensorineural, bilateral and symmetrical and is related to the duration of disease and state of metabolic control (HbA1C). PMID:22091228

  4. The Master Hearing Aid

    PubMed Central

    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

  5. Effects of hearing aid amplification on voice F0 variability in speakers with prelingual hearing loss.

    PubMed

    Lee, Guo-She; Liu, Chialin; Lee, Shao-Hsuan

    2013-08-01

    To investigate the audio-vocal feedback responses of (F0) to hearing amplification in severe-to-profound prelingual hearing loss (SPHL) using power spectral analysis of F0 contour of sustained vowels. Sustained phonations of vowel/a/of seventeen participants with SPHL were acquired with and without hearing-aid amplifications. The vocal intensity was visually fed back to the participants to help controlling the vocal intensity at 65-75 dBA and 85-95 dBA. The F0 contour of the phonations was extracted and submitted to spectral analysis to measure the extent of F0 fluctuations at different frequency ranges. The results showed that both high vocal intensity and hearing-aid amplification significantly improved voice F0 control by reducing the low-frequency fluctuations (low-frequency power, LFP, 0.2-3 Hz) in F0 spectrum. However, the enhanced feedback from higher vocal intensity and/or hearing amplification was not adequate to reduce the LFP to the level of a normal hearing person. Moreover, we found significant and negative correlations between LFP and supra-threshold feedback intensity (phonation intensity - hearing threshold level) for the frequencies of 500-2000 Hz. Increased vocal intensity, as well as hearing-aid amplification, improved voice F0 control by reducing the LFP of F0 spectrum, and the subtle changes in voices could be well explored using spectral analysis of F0.

  6. Canine hearing loss management.

    PubMed

    Scheifele, Lesa; Clark, John Greer; Scheifele, Peter M

    2012-11-01

    Dog owners and handlers are naturally concerned when suspicion of hearing loss arises for their dogs. Questions frequently asked of the veterinarian center on warning signs of canine hearing loss and what can be done for the dog if hearing loss is confirmed. This article addresses warning signs of canine hearing loss, communication training and safety awareness issues, and the feasibility of hearing aid amplification for dogs.

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

    PubMed

    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.

  8. [Hearing disorders in obliteration of the carotid artery. 2. Contribution to hearing loss in the aged].

    PubMed

    Böhme, G

    1989-07-01

    Otologic-audiologic examination was carried out in 75 patients (between 42 and 86 years of age; average age: 65 years) with confirmed internal angiologic obliteration of the carotid artery, either unilateral or bilateral. There were 51 unilateral and 24 bilateral stenoses/occlusions. Diseases of the ear were excluded clinically and audiologically. - The mean hearing loss shows a sensorineural high tone loss in the tone audiogram. The range of scatter of high tone loss increases proportionally to the increase of frequency. - Compared with the physiological examination of geriatric patients, the total word comprehension and especially the minimal discrimination loss point towards a pathologic impairment of hearing. The total word comprehension amounts to 250.79% in the 51-60 age group, 237.79% in the 61-70 age group, 175.83% in persons aged 71-80 years, and 118.33% for those over 80 years of age. The minimal discrimination loss comprises 5.83% in the 51-60 age group, 9.79% in the 61-70 age group, 22.50% in patients between 71 and 80 years, and 48.00% in those over 80 years. - No connection between the extent of loss of hearing and the stage of obliteration of the carotid artery can be shown. However, the decompensation of total word comprehension and especially the minimal discrimination loss is a very important sign. - These findings contribute towards a differentiation of physiologic and pathologic hearing diseases in old age with particulas reference to the underlying arteriosclerotic disease.

  9. Hearing complaints and the audiological profile of the users of an academic health center in the western region of São Paulo

    PubMed Central

    Samelli, Alessandra Giannella; de Andrade, Camila Quintiliano; Pereira, Marília Barbieri; Matas, Carla Gentile

    2013-01-01

    Summary Introduction: Few population-based studies have quantified hearing levels in Brazil; additional studies on this subject are needed. Purpose: The purpose of this study was to characterize hearing complaints and the audiological profile of the population served by the Clinical Audiology Service of an Academic Health Center in the western region of São Paulo, Brazil, between 2003 and 2008. An additional aim was to check whether there is a positive association between the signs/symptoms and type of hearing loss. Methods: This was a retrospective study of the records of 2,145 patients. The health history, tonal and vocal audiometry, and imitanciometry findings were analyzed. Results: The mean age of the patients was 20.6 years. The majority of the subjects had normal hearing thresholds, and the prevalence of hearing loss was approximately 35%. As the patient's age increased, the frequency of conductive hearing loss decreased and that of sensorineural hearing loss increased. There was a tendency toward hearing loss worsening with age. Conclusion: Hearing complaints can predict the type of hearing loss; therefore, they should always be valued because they can be used as a form of screening and thus help to determine the diagnostic hypothesis. This could help to reduce the gap between the patient's perception of the complaints and the audiological assessment and thus improve the prognosis. PMID:25992004

  10. [Hearing loss in adults].

    PubMed

    Eshraghi, Adrien A; Frachet, Bruno; Van De Water, Tom R; Eter, Elias

    2009-05-20

    The management of hearing loss in adults depends of etiology and its severity. It can be as simple as treating an external otitis, removing an impacted cerumen or a more complex one such as a surgery for otosclerosis. The hearing loss is managed mainly by new advances in hearing aids technology and implantable hearing devices which include BAHA, middle ear implant and cochlear implants. The research is focused on developing new molecules for intracochlear drug therapy to treat noise induced hearing loss, drug ototoxicity as well as hearing loss related to cochlear implant insertion trauma. Antioxidant molecules, molecules against apoptosis are at this time the most promising molecules than need further investigations.

  11. Acquired Idiopathic Generalized Anhidrosis.

    PubMed

    Gangadharan, Geethu; Criton, Sebastian; Surendran, Divya

    2015-01-01

    Acquired idiopathic generalized anhidrosis is a rare condition, where the exact pathomechanism is unknown. We report a case of acquired idiopathic generalized anhidrosis in a patient who later developed lichen planus. Here an autoimmune-mediated destruction of sweat glands may be the probable pathomechanism.

  12. LABORATORY-ACQUIRED MYCOSES

    DTIC Science & Technology

    laboratory- acquired mycoses . Insofar as possible, the etiological fungus, type of laboratory, classification of personnel, type of work conducted, and other...pertinent data have been listed in this study. More than 288 laboratory- acquired mycoses are described here, including 108 cases of

  13. NRF2 Is a Key Target for Prevention of Noise-Induced Hearing Loss by Reducing Oxidative Damage of Cochlea

    PubMed Central

    Honkura, Yohei; Matsuo, Hirotaka; Murakami, Shohei; Sakiyama, Masayuki; Mizutari, Kunio; Shiotani, Akihiro; Yamamoto, Masayuki; Morita, Ichiro; Shinomiya, Nariyoshi; Kawase, Tetsuaki; Katori, Yukio; Motohashi, Hozumi

    2016-01-01

    Noise-induced hearing loss (NIHL) is one of the most common sensorineural hearing deficits. Recent studies have demonstrated that the pathogenesis of NIHL is closely related to ischemia-reperfusion injury of cochlea, which is caused by blood flow decrease and free radical production due to excessive noise. This suggests that protecting the cochlea from oxidative stress is an effective therapeutic approach for NIHL. NRF2 is a transcriptional activator playing an essential role in the defense mechanism against oxidative stress. To clarify the contribution of NRF2 to cochlear protection, we examined Nrf2–/– mice for susceptibility to NIHL. Threshold shifts of the auditory brainstem response at 7 days post-exposure were significantly larger in Nrf2–/– mice than wild-type mice. Treatment with CDDO-Im, a potent NRF2-activating drug, before but not after the noise exposure preserved the integrity of hair cells and improved post-exposure hearing levels in wild-type mice, but not in Nrf2–/– mice. Therefore, NRF2 activation is effective for NIHL prevention. Consistently, a human NRF2 SNP was significantly associated with impaired sensorineural hearing levels in a cohort subjected to occupational noise exposure. Thus, high NRF2 activity is advantageous for cochlear protection from noise-induced injury, and NRF2 is a promising target for NIHL prevention. PMID:26776972

  14. NRF2 Is a Key Target for Prevention of Noise-Induced Hearing Loss by Reducing Oxidative Damage of Cochlea.

    PubMed

    Honkura, Yohei; Matsuo, Hirotaka; Murakami, Shohei; Sakiyama, Masayuki; Mizutari, Kunio; Shiotani, Akihiro; Yamamoto, Masayuki; Morita, Ichiro; Shinomiya, Nariyoshi; Kawase, Tetsuaki; Katori, Yukio; Motohashi, Hozumi

    2016-01-18

    Noise-induced hearing loss (NIHL) is one of the most common sensorineural hearing deficits. Recent studies have demonstrated that the pathogenesis of NIHL is closely related to ischemia-reperfusion injury of cochlea, which is caused by blood flow decrease and free radical production due to excessive noise. This suggests that protecting the cochlea from oxidative stress is an effective therapeutic approach for NIHL. NRF2 is a transcriptional activator playing an essential role in the defense mechanism against oxidative stress. To clarify the contribution of NRF2 to cochlear protection, we examined Nrf2(-/-) mice for susceptibility to NIHL. Threshold shifts of the auditory brainstem response at 7 days post-exposure were significantly larger in Nrf2(-/-) mice than wild-type mice. Treatment with CDDO-Im, a potent NRF2-activating drug, before but not after the noise exposure preserved the integrity of hair cells and improved post-exposure hearing levels in wild-type mice, but not in Nrf2(-/-) mice. Therefore, NRF2 activation is effective for NIHL prevention. Consistently, a human NRF2 SNP was significantly associated with impaired sensorineural hearing levels in a cohort subjected to occupational noise exposure. Thus, high NRF2 activity is advantageous for cochlear protection from noise-induced injury, and NRF2 is a promising target for NIHL prevention.

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

    PubMed

    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.

  16. Characterization of Hearing Thresholds from 500 to 16,000 Hz in Dentists: A Comparative Study

    PubMed Central

    Gonçalves, Claudia Giglio de Oliveira; Santos, Luciana; Lobato, Diolen; Ribas, Angela; Lacerda, Adriana Bender Moreira; Marques, Jair

    2014-01-01

    Introduction High-level noise exposure in dentists' workplaces may cause damages to the auditory systems. High-frequency audiometry is an important tool in the investigation in the early diagnosis of hearing loss. Objectives To analyze the auditory thresholds at frequencies from 500 to 16,000 Hz of dentists in the city of Curitiba. Methods This historic cohort study retrospectively tested hearing thresholds from 500 to 16,000 Hz with a group of dentists from Curitiba, in the state of Paraná, Brazil. Eighty subjects participated in the study, separated into a dentist group and a control group, with the same age range and gender across groups but with no history of occupational exposure to high levels of sound pressure in the control group. Subjects were tested with conventional audiometry and high-frequency audiometry and answered a questionnaire about exposure to noise. Results Results showed that 81% of dentists did not receive any information regarding noise at university; 6 (15%) dentists had sensorineural hearing impairment; significant differences were observed between the groups only at frequencies of 500 Hz and 1,000, 6,000 and 8,000 Hz in the right ear. There was no significant difference between the groups after analysis of mean hearing thresholds of high frequencies with the average hearing thresholds in conventional frequencies; subjects who had been working as dentists for longer than 10 years had worse tonal hearing thresholds at high frequencies. Conclusions In this study, we observed that dentists are at risk for the development of sensorineural hearing loss especially after 10 years of service. PMID:25992172

  17. Characterization of hearing thresholds from 500 to 16,000 hz in dentists: a comparative study.

    PubMed

    Gonçalves, Claudia Giglio de Oliveira; Santos, Luciana; Lobato, Diolen; Ribas, Angela; Lacerda, Adriana Bender Moreira; Marques, Jair

    2015-04-01

    Introduction High-level noise exposure in dentists' workplaces may cause damages to the auditory systems. High-frequency audiometry is an important tool in the investigation in the early diagnosis of hearing loss. Objectives To analyze the auditory thresholds at frequencies from 500 to 16,000 Hz of dentists in the city of Curitiba. Methods This historic cohort study retrospectively tested hearing thresholds from 500 to 16,000 Hz with a group of dentists from Curitiba, in the state of Paraná, Brazil. Eighty subjects participated in the study, separated into a dentist group and a control group, with the same age range and gender across groups but with no history of occupational exposure to high levels of sound pressure in the control group. Subjects were tested with conventional audiometry and high-frequency audiometry and answered a questionnaire about exposure to noise. Results Results showed that 81% of dentists did not receive any information regarding noise at university; 6 (15%) dentists had sensorineural hearing impairment; significant differences were observed between the groups only at frequencies of 500 Hz and 1,000, 6,000 and 8,000 Hz in the right ear. There was no significant difference between the groups after analysis of mean hearing thresholds of high frequencies with the average hearing thresholds in conventional frequencies; subjects who had been working as dentists for longer than 10 years had worse tonal hearing thresholds at high frequencies. Conclusions In this study, we observed that dentists are at risk for the development of sensorineural hearing loss especially after 10 years of service.

  18. Discrimination and identification of vowels by young, hearing-impaired adults

    NASA Astrophysics Data System (ADS)

    Richie, Carolyn; Kewley-Port, Diane; Coughlin, Maureen

    2003-11-01

    This study examined the effects of mild-to-moderate sensorineural hearing loss on vowel perception abilities of young, hearing-impaired (YHI) adults. Stimuli were presented at a low conversational level with a flat frequency response (approximately 60 dB SPL), and in two gain conditions: (a) high level gain with a flat frequency response (95 dB SPL), and (b) frequency-specific gain shaped according to each listener's hearing loss (designed to simulate the frequency response provided by a linear hearing aid to an input signal of 60 dB SPL). Listeners discriminated changes in the vowels /smcapi ee eh invv æ/ when F1 or F2 varied, and later categorized the vowels. YHI listeners performed better in the two gain conditions than in the conversational level condition. Performances in the two gain conditions were similar, suggesting that upward spread of masking was not seen at these signal levels for these tasks. Results were compared with those from a group of elderly, hearing-impaired (EHI) listeners, reported in Coughlin, Kewley-Port, and Humes [J. Acoust. Soc. Am. 104, 3597-3607 (1998)]. Comparisons revealed no significant differences between the EHI and YHI groups, suggesting that hearing impairment, not age, is the primary contributor to decreased vowel perception in these listeners.

  19. Noise-induced hearing loss alters the temporal dynamics of auditory-nerve responses.

    PubMed

    Scheidt, Ryan E; Kale, Sushrut; Heinz, Michael G

    2010-10-01

    Auditory-nerve fibers demonstrate dynamic response properties in that they adapt to rapid changes in sound level, both at the onset and offset of a sound. These dynamic response properties affect temporal coding of stimulus modulations that are perceptually relevant for many sounds such as speech and music. Temporal dynamics have been well characterized in auditory-nerve fibers from normal-hearing animals, but little is known about the effects of sensorineural hearing loss on these dynamics. This study examined the effects of noise-induced hearing loss on the temporal dynamics in auditory-nerve fiber responses from anesthetized chinchillas. Post-stimulus-time histograms were computed from responses to 50-ms tones presented at characteristic frequency and 30 dB above fiber threshold. Several response metrics related to temporal dynamics were computed from post-stimulus-time histograms and were compared between normal-hearing and noise-exposed animals. Results indicate that noise-exposed auditory-nerve fibers show significantly reduced response latency, increased onset response and percent adaptation, faster adaptation after onset, and slower recovery after offset. The decrease in response latency only occurred in noise-exposed fibers with significantly reduced frequency selectivity. These changes in temporal dynamics have important implications for temporal envelope coding in hearing-impaired ears, as well as for the design of dynamic compression algorithms for hearing aids.

  20. Mutations in LOXHD1 gene cause various types and severities of hearing loss

    PubMed Central

    Mori, Kentaro; Moteki, Hideaki; Kobayashi, Yumiko; Azaiez, Hela; Booth, Kevin T; Nishio, Shin-ya; Sato, Hiroaki; Smith, Richard J H; Usami, Shin-ichi

    2015-01-01

    Objective We present two families that were identified with novel mutations in LOXHD1, as a cause of non-progressive hearing loss. Methods One thousand three hundred fourteen (1,314) Japanese subjects with sensorineural hearing loss from unrelated families were enrolled in the study. Targeted genomic enrichment and massively parallel sequencing of all known non-syndromic hearing loss genes were performed to identify the genetic cause of hearing loss. Results Two patients in one family affected with homozygous mutation; c.879+1G>A in LOXHD1, showed profound congenital hearing loss, whereas two patients in the other family with compound heterozygous mutations; c.5869G>T (p.E1957X) and c.4480C>T (p.R1494X) showed moderate to severe hearing loss. Conclusion Mutations in LOXHD1 are extremely rare, and these cases are the first identified in a Japanese population. The genotype-phenotype correlation in LOXHD1 is still unclear. The differences of phenotypes in each patient might be the result of the nature of the mutations, or the location at the gene, or be influenced by genetic modifier. PMID:25792669

  1. JS-X syndrome: A multiple congenital malformation with vocal cord paralysis, ear deformity, hearing loss, shoulder musculature underdevelopment, and X-linked recessive inheritance.

    PubMed

    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.

  2. Living with hearing loss

    MedlinePlus

    ... medlineplus.gov/ency/patientinstructions/000360.htm Living with hearing loss To use the sharing features on this page, please enable JavaScript. If you are living with hearing loss, you know that it takes extra effort to ...

  3. Genetics of Hearing Loss

    MedlinePlus

    ... in Latin America Information For... Media Policy Makers Genetics of Hearing Loss Language: English Español (Spanish) Recommend ... of hearing loss in babies is due to genetic causes. There are also a number of things ...

  4. What's Hearing Loss?

    MedlinePlus

    ... pathologists to help them develop their hearing and speaking skills. Some people with hearing loss may need to use special techniques like these to communicate: speechreading (also ... without speaking. Remember: American Sign Language (ASL) is different from ...

  5. Managing Hearing Loss

    MedlinePlus

    ... a total loss of hearing. It can be hereditary or it can result from disease, trauma, certain ... build-up, fluid, or a punctured eardrum. Medical treatment or surgery can usually restore conductive hearing loss. ...

  6. Evaluation of hearing impairment.

    PubMed

    Zadeh, M H; Selesnick, S H

    2001-01-01

    Hearing impairment is among the most common medical condition presenting to health care professionals. Ear anatomy, physiology, and pathology resulting in hearing loss are discussed. A systematic approach to evaluation, diagnosis, and treatment is presented.

  7. Hearing Aid Assembly

    NASA Technical Reports Server (NTRS)

    Grugel, Richard N. (Inventor)

    2002-01-01

    Progress in hearing aids has come a long way. Yet despite such progress hearing aids are not the perfect answer to many hearing problems. Some adult ears cannot accommodate tightly fitting hearing aids. Mouth movements such as chewing, talking, and athletic or other active endeavors also lead to loosely fitting ear molds. It is well accepted that loosely fitting hearing aids are the cause of feedback noise. Since feedback noise is the most common complaint of hearing aid wearers it has been the subject of various patents. Herein a hearing aid assembly is provided eliminating feedback noise. The assembly includes the combination of a hearing aid with a headset developed to constrict feedback noise.

  8. Hearing Profile in Patients with Dilated and Hypertrophic Cardiomyopathies

    PubMed Central

    El-Zarea, Gehan Abd El-Rahman; Hassan, Yasser Elsayed Mohamed; Mahmoud, Ahmed Mohamed Ahmed

    2016-01-01

    Introduction Cardiomyopathy may cause disruptions in the micro-vascular system of the stria vascularis in the cochlea, and, subsequently, may result in cochlear degeneration. Degeneration in the stria vascularis affects the physical and chemical processes in the organ of Corti, thereby causing a possible hearing impairment. The objective of this study was to assess the hearing profiles of patients with dilated and hypertrophic cardiomyopathies to determine the relationship between the degree of hearing loss and the degree and duration of the disease and to compare the dilated and hypertrophic cardiomyopathies as regards hearing profile. Methods In this case control study, we studied 21 patients (cases/study group/group 1) and 15 healthy individuals (controls/group 2). Six patients (group 1a) had hypertrophic cardiomyopathy (HCM), and 15 patients (group 1b) had dilated cardiomyopathy (DCM). The data were analyzed using the t-test, chi-squared test, Kruskal-Wallis test, and the Multiple Mann-Whitney test. Results The results of this study showed that 80% of those patients with DCM (group 1b) had bilateral sensorineural hearing loss (SNHL), and 100% of the patients with HCM (group 1a) had mild to severe bilateral sloping SNHL. Distortion Product Otoacoustic Emissions (DPOAEs) were present in 14% of the study group and in 100 % of the control group. The results of the measurements of auditory brainstem response (ABR) showed that 50% of the study group had abnormal latencies compared to the control group, and there was no correlation between the duration of the disease and the degree of hearing loss or DPOAE. Fifty percent of the patients with HCM and 35% of the patients with DCM had positive family histories of similar conditions, and 35% of those with HCM had a positive family history of sudden death. Conclusion The results of this study suggested that the link between heart disease and hearing loss and early identification of hearing loss in patients with

  9. Acquired inflammatory demyelinating neuropathies.

    PubMed

    Ensrud, E R; Krivickas, L S

    2001-05-01

    The acquired demyelinating neuropathies can be divided into those with an acute onset and course and those with a more chronic course. The acute neuropathies present as Guillain-Barré syndrome and include acute inflammatory demyelinating polyradiculoneuropathy (AIDP), Miller Fisher syndrome, acute motor axonal neuropathy (AMAN), acute motor and sensory axonal neuropathy (AMSAN), and acute pandysautonomia. The chronic neuropathies are collectively known as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and include MADSAM (multifocal acquired demyelinating sensory and motor neuropathy, also know as Lewis-Sumner syndrome) and DADS (distal acquired demyelinating symmetric neuropathy) as variants. The clinical features, pathology, pathogenesis, diagnosis, treatment, rehabilitation, and prognosis of these neuropathies are discussed.

  10. Rehabilitation of Hearing.

    ERIC Educational Resources Information Center

    World Federation of the Deaf, Rome (Italy).

    Rehabilitation of hearing is considered in five conference papers. Two papers come from Poland: "Rehabilitation of Hearing in Children 'Deaf' in First 5 Years of Age" by D. Borkowska-Gaertig and others and "Possibilities of Hearing Improvement in Adults with Conservative Methods" by T. Bystrzanowska. Also included are…

  11. Deafness and Hearing Loss.

    ERIC Educational Resources Information Center

    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…

  12. Hearing Conservation Medical Program

    NASA Technical Reports Server (NTRS)

    1993-01-01

    Background on hearing impairment is presented including causes and criteria for safe noise levels. The purpose of the Hearing Conservation Program at LeRC is outlined, and the specifics of the Medical Surveillance Program for Hearing Impairment at LeRC are discussed.

  13. Hearing loss - infants

    MedlinePlus

    ... loss. Two common tests are used to screen newborn infants for hearing loss: Auditory brain stem response (ABR) ... Over 30 states in the United States require newborn hearing screenings. Treating hearing loss early can allow many infants to develop normal language skills without delay. In ...

  14. RFX transcription factors are essential for hearing in mice

    PubMed Central

    Elkon, Ran; Milon, Beatrice; Morrison, Laura; Shah, Manan; Vijayakumar, Sarath; Racherla, Manoj; Leitch, Carmen C.; Silipino, Lorna; Hadi, Shadan; Weiss-Gayet, Michèle; Barras, Emmanuèle; Schmid, Christoph D.; Ait-Lounis, Aouatef; Barnes, Ashley; Song, Yang; Eisenman, David J.; Eliyahu, Efrat; Frolenkov, Gregory I.; Strome, Scott E.; Durand, Bénédicte; Zaghloul, Norann A.; Jones, Sherri M.; Reith, Walter; Hertzano, Ronna

    2015-01-01

    Sensorineural hearing loss is a common and currently irreversible disorder, because mammalian hair cells (HCs) do not regenerate and current stem cell and gene delivery protocols result only in immature HC-like cells. Importantly, although the transcriptional regulators of embryonic HC development have been described, little is known about the postnatal regulators of maturating HCs. Here we apply a cell type-specific functional genomic analysis to the transcriptomes of auditory and vestibular sensory epithelia from early postnatal mice. We identify RFX transcription factors as essential and evolutionarily conserved regulators of the HC-specific transcriptomes, and detect Rfx1,2,3,5 and 7 in the developing HCs. To understand the role of RFX in hearing, we generate Rfx1/3 conditional knockout mice. We show that these mice are deaf secondary to rapid loss of initially well-formed outer HCs. These data identify an essential role for RFX in hearing and survival of the terminally differentiating outer HCs. PMID:26469318

  15. Paired comparisons of nonlinear frequency compression, extended bandwidth, and restricted bandwidth hearing-aid processing for children and adults with hearing loss

    PubMed Central

    Brennan, Marc A.; McCreery, Ryan; Kopun, Judy; Hoover, Brenda; Alexander, Joshua; Lewis, Dawna; Stelmachowicz, Patricia G.

    2014-01-01

    Background Preference for speech and music processed with nonlinear frequency compression and two controls (restricted and extended bandwidth hearing-aid processing) was examined in adults and children with hearing loss. Purpose Determine if stimulus type (music, sentences), age (children, adults) and degree of hearing loss influence listener preference for nonlinear frequency compression, restricted bandwidth and extended bandwidth. Research Design Within-subject, quasi-experimental study. Using a round-robin procedure, participants listened to amplified stimuli that were 1) frequency-lowered using nonlinear frequency compression, 2) low-pass filtered at 5 kHz to simulate the restricted bandwidth of conventional hearing aid processing, or 3) low-pass filtered at 11 kHz to simulate extended bandwidth amplification. The examiner and participants were blinded to the type of processing. Using a two-alternative forced-choice task, participants selected the preferred music or sentence passage. Study Sample Sixteen children (8–16 years) and 16 adults (19–65 years) with mild-to-severe sensorineural hearing loss. Intervention All subjects listened to speech and music processed using a hearing-aid simulator fit to the Desired Sensation Level algorithm v.5.0a (Scollie et al, 2005). Results Children and adults did not differ in their preferences. For speech, participants preferred extended bandwidth to both nonlinear frequency compression and restricted bandwidth. Participants also preferred nonlinear frequency compression to restricted bandwidth. Preference was not related to degree of hearing loss. For music, listeners did not show a preference. However, participants with greater hearing loss preferred nonlinear frequency compression to restricted bandwidth more than participants with less hearing loss. Conversely, participants with greater hearing loss were less likely to prefer extended bandwidth to restricted bandwidth. Conclusion Both age groups preferred access to

  16. Acquired color vision deficiency.

    PubMed

    Simunovic, Matthew P

    2016-01-01

    Acquired color vision deficiency occurs as the result of ocular, neurologic, or systemic disease. A wide array of conditions may affect color vision, ranging from diseases of the ocular media through to pathology of the visual cortex. Traditionally, acquired color vision deficiency is considered a separate entity from congenital color vision deficiency, although emerging clinical and molecular genetic data would suggest a degree of overlap. We review the pathophysiology of acquired color vision deficiency, the data on its prevalence, theories for the preponderance of acquired S-mechanism (or tritan) deficiency, and discuss tests of color vision. We also briefly review the types of color vision deficiencies encountered in ocular disease, with an emphasis placed on larger or more detailed clinical investigations.

  17. Hearing impairment and language delay in infants: Diagnostics and genetics

    PubMed Central

    Lang-Roth, Ruth

    2014-01-01

    This overview study provides information on important phoniatric and audiological aspects of early childhood hearing and language development with the aim of presenting diagnostic and therapeutic approaches. The article first addresses the universal newborn hearing screening that has been implemented in Germany for all infants since January 2009. The process of newborn hearing screening from the maternity ward to confirmation diagnostics is presented in accordance with a decision by the Federal Joint Committee (G-BA). The second topic is pediatric audiology diagnostics. Following confirmation of a permanent early childhood hearing disorder, the search for the cause plays an important role. Hereditary hearing disorders and intrauterine cytomegalovirus (CMV) infection, probably the most common cause of an acquired hearing disorder, are discussed and compared with the most common temporary hearing disorder, otitis media with effusion, which in some cases is severe enough to be relevant for hearing and language development and therefore requires treatment. The third topic covered in this article is speech and language development in the first 3 years of life, which is known today to be crucial for later language development and learning to read and write. There is a short overview and introduction to modern terminology, followed by the abnormalities and diagnostics of early speech and language development. Only some aspects of early hearing and language development are addressed here. Important areas such as the indication for a cochlear implant in the first year of life or because of unilateral deafness are not included due to their complexity. PMID:25587365

  18. Hearing impairment and language delay in infants: Diagnostics and genetics.

    PubMed

    Lang-Roth, Ruth

    2014-01-01

    This overview study provides information on important phoniatric and audiological aspects of early childhood hearing and language development with the aim of presenting diagnostic and therapeutic approaches. The article first addresses the universal newborn hearing screening that has been implemented in Germany for all infants since January 2009. The process of newborn hearing screening from the maternity ward to confirmation diagnostics is presented in accordance with a decision by the Federal Joint Committee (G-BA). The second topic is pediatric audiology diagnostics. Following confirmation of a permanent early childhood hearing disorder, the search for the cause plays an important role. Hereditary hearing disorders and intrauterine cytomegalovirus (CMV) infection, probably the most common cause of an acquired hearing disorder, are discussed and compared with the most common temporary hearing disorder, otitis media with effusion, which in some cases is severe enough to be relevant for hearing and language development and therefore requires treatment. The third topic covered in this article is speech and language development in the first 3 years of life, which is known today to be crucial for later language development and learning to read and write. There is a short overview and introduction to modern terminology, followed by the abnormalities and diagnostics of early speech and language development. Only some aspects of early hearing and language development are addressed here. Important areas such as the indication for a cochlear implant in the first year of life or because of unilateral deafness are not included due to their complexity.

  19. ‘Ecstasy’ Enhances Noise-Induced Hearing Loss

    PubMed Central

    Church, Michael W.; Zhang, Jinsheng S.; Langford, Megan M.; Perrine, Shane A.

    2013-01-01

    ‘Ecstasy’ or 3,4-methylenedioxy-N-methamphetamine (MDMA) is an amphetamine abused for its euphoric, empathogenic, hallucinatory, and stimulant effects. It is also used to treat certain psychiatric disorders. Common settings for Ecstasy use are nightclubs and “rave” parties where participants consume MDMA and dance to loud music. One concern with the club setting is that exposure to loud sounds can cause permanent sensorineural hearing loss. Another concern is that consumption of MDMA may enhance such hearing loss. Whereas this latter possibility has not been investigated, this study tested the hypothesis that MDMA enhances noise-induced hearing loss (NIHL) by exposing rats to either MDMA, noise trauma, both MDMA and noise, or neither treatment. MDMA was given in a binge pattern of 5 mg/kg per intraperitoneal injections every 2 h for a total of four injections to animals in the two MDMA-treated groups (MDMA-only and Noise+MDMA). Saline injections were given to the animals in the two non-MDMA groups (Control and Noise-only). Following the final injection, noise trauma was induced by a 10 kHz tone at 120 dB SPL for 1 h to animals in the two noise trauma-treated groups (Noise-only and Noise+MDMA). Hearing loss was assessed by the auditory brainstem response (ABR) and cochlear histology. Results showed that MDMA enhanced NIHL compared to Noise-only and that MDMA alone caused no hearing loss. This implies that “clubbers” and “rave-goers” are exacerbating the amount of NIHL when they consume MDMA and listen to loud sounds. In contrast to earlier reports, the present study found that MDMA by itself caused no changes in the click-evoked ABR’s wave latencies or amplitudes. PMID:23711768

  20. A novel missense mutation in ACTG1 causes dominant deafness in a Norwegian DFNA20/26 family, but ACTG1 mutations are not frequent among families with hereditary hearing impairment.

    PubMed

    Rendtorff, Nanna D; Zhu, Mei; Fagerheim, Toril; Antal, Torben L; Jones, MaryPat; Teslovich, Tanya M; Gillanders, Elizabeth M; Barmada, Michael; Teig, Erik; Trent, Jeffrey M; Friderici, Karen H; Stephan, Dietrich A; Tranebjaerg, Lisbeth

    2006-10-01

    The gamma-actin gene (ACTG1) encodes a major cytoskeletal protein of the sensory hair cells of the cochlea. Recently, mutations in ACTG1 were found to cause autosomal dominant, progressive, sensorineural hearing impairment linked to the DFNA20/26 locus on chromosome 17q25.3 in four American families and in one Dutch family. We report here the linkage of autosomal dominant, progressive, sensorineural hearing impairment in a large Norwegian family to the DFNA20/26 locus. Sequencing of ACTG1 identified a novel missense mutation (c.1109T>C; p.V370A) segregating with the hearing loss. Functional analysis in yeast showed that the p.V370A mutation restricts cell growth at elevated temperature or under hyperosmolar stress. Molecular modelling suggested that the p.V370A mutation modestly alters a site for protein-protein interaction in gamma-actin and thereby modestly alters gamma-actin-based cytoskeletal structures. Nineteen Norwegian and Danish families with autosomal, dominant hearing impairment were analyzed for mutations in ACTG1 by sequencing, but no disease-associated mutations were identified. Finally, a long-term follow-up of the hearing loss progression associated with the p.V370A mutation in ACTG1 is provided. The present study expands our understanding of the genotype-phenotype relationship of this deafness gene and provides a sensitive and simple functional assay for missense mutations in this gene, which may assist future molecular diagnosis of autosomal-dominant hearing impairment. Finally, the present results do not indicate that mutations in ACTG1 are a frequent cause of autosomal-dominant postlingual sensorineural hearing impairment in Norway nor Denmark.

  1. Contemporary issues in audiology: a hearing scientist's perspective.

    PubMed

    Parker, David J

    2002-01-01

    Audiology has developed significantly over the last 30 years leading to better identification and assessment of hearing loss and better habilitation services for both children and adults with congenital or acquired deafness. Advancement in the profession and its services has been largely dependent on the technological development of key methodologies such as the auditory brainstem response and otoacoustic emissions. These methodologies have been used for the identification and assessment of the severity of hearing loss. As a result, these methodologies have underpinned the emergence of service development initiatives such as hearing screening and provision of hearing aids, particularly for newborn babies and young children. This review identifies, describes and evaluates the key methodologies and services involved and presents a hearing scientist's perspective on the developments to date. The aim is to provide state-of-the-art information to those working with children and adults with communication disorders, particularly speech and language therapists.

  2. Hearing loss in space

    NASA Technical Reports Server (NTRS)

    Buckey, J. C. Jr; Musiek, F. E.; Kline-Schoder, R.; Clark, J. C.; Hart, S.; Havelka, J.

    2001-01-01

    BACKGROUND: Temporary and, in some cases, permanent hearing loss has been documented after long-duration spaceflights. METHODS: We examined all existing published data on hearing loss after space missions to characterize the losses. RESULTS: Data from Russian missions suggest that the hearing loss, when it occurs, affects mainly mid to high frequencies and that using hearing protection often might prevent the loss. Several significant questions remain about hearing loss in space. While the hearing loss has been presumed to be noise-induced, no clear link has been established between noise exposure and hearing loss during spaceflight. In one documented case of temporary hearing loss from the Shuttle-Mir program, the pattern of loss was atypical for a noise-induced loss. Continuous noise levels that have been measured on the Mir and previous space stations, while above engineering standards, are not at levels usually associated with hearing loss in ground-based studies (which have usually been limited to 8-10 h exposure periods). Attempts to measure hearing in space using threshold-based audiograms have been unsuccessful in both the American and Russian programs due to noise interference with the measurements. CONCLUSIONS: The existing data highlight the need for reliable monitoring of both hearing and noise in long-duration spaceflight.

  3. The A1555G mutation in the 12S rRNA gene of human mtDNA: recurrent origins and founder events in families affected by sensorineural deafness.

    PubMed

    Torroni, A; Cruciani, F; Rengo, C; Sellitto, D; López-Bigas, N; Rabionet, R; Govea, N; López De Munain, A; Sarduy, M; Romero, L; Villamar, M; del Castillo, I; Moreno, F; Estivill, X; Scozzari, R

    1999-11-01

    The mtDNA variation of 50 Spanish and 4 Cuban families affected by nonsyndromic sensorineural deafness due to the A1555G mutation in the 12S rRNA gene was studied by high-resolution RFLP analysis and sequencing of the control region. Phylogenetic analyses of haplotypes and detailed survey of population controls revealed that the A1555G mutation can be attributed to >/=30 independent mutational events among the 50 Spanish families and that it occurs on mtDNA haplogroups that are common in all European populations. This indicates that the relatively high detection rate of this mutation in Spain is not due to sampling biases or to a single major founder event. Moreover, the distribution of these mutational events on different haplogroups is compatible with a random occurrence of the A1555G mutation and tends to support the conclusion that mtDNA backgrounds do not play a significant role in the expression of the mutation. Overall, these findings appear to indicate that the rare detection of this mutation in other populations is most likely due to inadequacy in patient ascertainment and molecular screening. This probable lack of identification of the A1555G mutation in subjects affected by sensorineural hearing loss implies that their maternally related relatives are not benefiting from presymptomatic detection and information concerning their increased risk of ototoxicity due to aminoglycoside treatments.

  4. The A1555G Mutation in the 12S rRNA Gene of Human mtDNA: Recurrent Origins and Founder Events in Families Affected by Sensorineural Deafness

    PubMed Central

    Torroni, Antonio; Cruciani, Fulvio; Rengo, Chiara; Sellitto, Daniele; López-Bigas, Núria; Rabionet, Raquel; Govea, Nancy; López de Munain, Adolfo; Sarduy, Maritza; Romero, Lourdes; Villamar, Manuela; del Castillo, Ignacio; Moreno, Felipe; Estivill, Xavier; Scozzari, Rosaria

    1999-01-01

    Summary The mtDNA variation of 50 Spanish and 4 Cuban families affected by nonsyndromic sensorineural deafness due to the A1555G mutation in the 12S rRNA gene was studied by high-resolution RFLP analysis and sequencing of the control region. Phylogenetic analyses of haplotypes and detailed survey of population controls revealed that the A1555G mutation can be attributed to ⩾30 independent mutational events among the 50 Spanish families and that it occurs on mtDNA haplogroups that are common in all European populations. This indicates that the relatively high detection rate of this mutation in Spain is not due to sampling biases or to a single major founder event. Moreover, the distribution of these mutational events on different haplogroups is compatible with a random occurrence of the A1555G mutation and tends to support the conclusion that mtDNA backgrounds do not play a significant role in the expression of the mutation. Overall, these findings appear to indicate that the rare detection of this mutation in other populations is most likely due to inadequacy in patient ascertainment and molecular screening. This probable lack of identification of the A1555G mutation in subjects affected by sensorineural hearing loss implies that their maternally related relatives are not benefiting from presymptomatic detection and information concerning their increased risk of ototoxicity due to aminoglycoside treatments. PMID:10521300

  5. Vancomycin during pregnancy: does it cause hearing loss or nephrotoxicity in the infant?

    PubMed

    Reyes, M P; Ostrea, E M; Cabinian, A E; Schmitt, C; Rintelmann, W

    1989-10-01

    Vancomycin was administered intravenously to 10 pregnant women for the treatment of methicillin-resistant Staphylococcus aureus infections. Auditory brainstem response testing and renal function studies were performed on the 10 babies in the experimental group and 10 babies in each of two control groups to determine the safety of vancomycin use during pregnancy. Auditory brainstem responses were not normal at birth in six infants from the three different groups studied (N = 30) but were normal at 3 months in five. The sixth infant had conductive hearing loss unrelated to vancomycin use that spontaneously disappeared at 12 months of age. Renal function was normal in all infants. Vancomycin was detected in cord blood in two patients and in breast milk in one. Adequate serum levels were achieved with routine doses in eight mothers tested; no adverse reactions occurred. It appears that vancomycin use during the second and third trimesters of pregnancy does not produce sensorineural hearing loss or nephrotoxicity in the infant.

  6. The Envoy® Totally Implantable Hearing System, St. Croix Medical

    PubMed Central

    Kroll, Kai; Grant, Iain L.; Javel, Eric

    2002-01-01

    The Totally Implantable Envoy® System is currently undergoing clinical trials in both the United States and Europe. The fully implantable hearing device is intended for use in patients with sensorineural hearing loss. The device employs piezoelectric transducers to sense ossicle motion and drive the stapes. Programmable signal processing parameters include amplification, compression, and variable frequency response. The fully implantable attribute allows users to take advantage of normal external ear resonances and head-related transfer functions, while avoiding undesirable earmold effects. The high sensitivity, low power consumption, and high fidelity attributes of piezoelectric transducers minimize acoustic feedback and maximize battery life (Gyo, 1996; Yanagihara, (1987) and 2001). The surgical procedure to install the device has been accurately defined and implantation is reversible. PMID:25425915

  7. Music and hearing aids.

    PubMed

    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.

  8. Music and Hearing Aids

    PubMed Central

    Moore, Brian C. J.

    2014-01-01

    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. PMID:25361601

  9. Congenital hearing loss.

    PubMed

    Korver, Anna M H; Smith, Richard J H; Van Camp, Guy; Schleiss, Mark R; Bitner-Glindzicz, Maria A K; Lustig, Lawrence R; Usami, Shin-Ichi; Boudewyns, An N

    2017-01-12

    Congenital hearing loss (hearing loss that is present at birth) is one of the most prevalent chronic conditions in children. In the majority of developed countries, neonatal hearing screening programmes enable early detection; early intervention will prevent delays in speech and language development and has long-lasting beneficial effects on social and emotional development and quality of life. A diagnosis of hearing loss is usually followed by a search for an underlying aetiology. Congenital hearing loss might be attributed to environmental and prenatal factors, which prevail in low-income settings; congenital infections, particularly cytomegalovirus infection, are also a common risk factor for hearing loss. Genetic causes probably account for the majority of cases in developed countries; mutations can affect any component of the hearing pathway, in particular, inner ear homeostasis (endolymph production and maintenance) and mechano-electrical transduction (the conversion of a mechanical stimulus into electrochemical activity). Once the underlying cause of hearing loss is established, it might direct therapeutic decision making and guide prevention and (genetic) counselling. Management options include specific antimicrobial therapies, surgical treatment of craniofacial abnormalities and implantable or non-implantable hearing devices. An improved understanding of the pathophysiology and molecular mechanisms that underlie hearing loss and increased awareness of recent advances in genetic testing will promote the development of new treatment and screening strategies.

  10. Acquired hypofibrinogenemia: current perspectives

    PubMed Central

    Besser, Martin W; MacDonald, Stephen G

    2016-01-01

    Acquired hypofibrinogenemia is most frequently caused by hemodilution and consumption of clotting factors. The aggressive replacement of fibrinogen has become one of the core principles of modern management of massive hemorrhage. The best method for determining the patient’s fibrinogen level remains controversial, and particularly in acquired dysfibrinogenemia, could have major therapeutic implications depending on which quantification method is chosen. This review introduces the available laboratory and point-of-care methods and discusses the relative advantages and limitations. It also discusses current strategies for the correction of hypofibrinogenemia. PMID:27713652

  11. Community-acquired pneumonia.

    PubMed

    Falguera, M; Ramírez, M F

    2015-11-01

    This article not only reviews the essential aspects of community-acquired pneumonia for daily clinical practice, but also highlights the controversial issues and provides the newest available information. Community-acquired pneumonia is considered in a broad sense, without excluding certain variants that, in recent years, a number of authors have managed to delineate, such as healthcare-associated pneumonia. The latter form is nothing more than the same disease that affects more frail patients, with a greater number of risk factors, both sharing an overall common approach.

  12. Analysis of the Prevalence of and Factors Associated with Hearing Loss in Korean Adolescents

    PubMed Central

    Hong, Seok Min; Park, Il-Seok; Kim, Yong Bok; Hong, Seok Jin; Lee, Byungho

    2016-01-01

    Background Hearing loss can lead to a number of disabilities, subsequently reducing the quality of life. In general, hearing thresholds of adolescents are better than adults and the elderly. However, occasionally, adolescents acquire hearing loss for a number of reasons. In this study, our goal was to estimate the prevalence of hearing loss in the Korean population and to investigate the factors related to hearing thresholds in adolescents. Methods A cross-sectional study was conducted using data from the Korean National Health and Nutrition Examination Survey (KNHANES) between 2010 and 2012. We enrolled a total of 1,658 participants, ages 13 to 18 years. We investigated the prevalence of hearing loss and the factors associated with hearing thresholds at various frequencies (0.5, 1, 2, 3, 4, and 6 KHz). Results Weighted prevalence of unilateral and bilateral hearing loss in Korean adolescents was 2.2% and 0.4%, respectively. Weighted prevalence of hearing thresholds ≥ 20 dB at speech and high frequencies were 3.1% and 5.0%, respectively, for unilateral hearing loss and 0.7% and 1.9%, respectively, for bilateral. Age group, tympanometric data, and household income were significantly related to unilateral or bilateral hearing thresholds ≥ 20 dB at speech frequencies. Earphone use in noisy places was associated with bilateral hearing thresholds ≥ 20 dB at high frequencies. Conclusions The prevalence of hearing loss in Korean adolescents was 2.6% using the general standard threshold associated with hearing loss. However, the prevalence of hearing thresholds ≥ 20 dB for speech and high frequencies was much higher. The results from this study provide an estimate of hearing loss in adolescents and suggest the need for early detection and hearing preservation programs among adolescents. PMID:27513659

  13. Developmental hearing loss impairs signal detection in noise: putative central mechanisms

    PubMed Central

    Gay, Jennifer D.; Voytenko, Sergiy V.; Galazyuk, Alexander V.; Rosen, Merri J.

    2014-01-01

    Listeners with hearing loss have difficulty processing sounds in noisy environments. This is most noticeable for speech perception, but is reflected in a basic auditory processing task: detecting a tonal signal in a noise background, i.e., simultaneous masking. It is unresolved whether the mechanisms underlying simultaneous masking arise from the auditory periphery or from the central auditory system. Poor detection in listeners with sensorineural hearing loss (SNHL) is attributed to cochlear hair cell damage. However, hearing loss alters neural processing in the central auditory system. Additionally, both psychophysical and neurophysiological data from normally hearing and impaired listeners suggest that there are additional contributions to simultaneous masking that arise centrally. With SNHL, it is difficult to separate peripheral from central contributions to signal detection deficits. We have thus excluded peripheral contributions by using an animal model of early conductive hearing loss (CHL) that provides auditory deprivation but does not induce cochlear damage. When tested as adults, animals raised with CHL had increased thresholds for detecting tones in simultaneous noise. Furthermore, intracellular in vivo recordings in control animals revealed a cortical correlate of simultaneous masking: local cortical processing reduced tone-evoked responses in the presence of noise. This raises the possibility that altered cortical responses which occur with early CHL can influence even simple signal detection in noise. PMID:25249949

  14. Auditory steady state response in hearing assessment in infants with cytomegalovirus

    PubMed Central

    Silva, Daniela Polo C.; Lopez, Priscila Suman; Montovani, Jair Cortez

    2013-01-01

    OBJECTIVE: To report an infant with congenital cytomegalovirus and progressive sensorineural hearing loss, who was assessed by three methods of hearing evaluation. CASE DESCRIPTION: In the first audiometry, at four months of age, the infant showed abnormal response in Otoacoustic Emissions and normal Auditory Brainstem Response (ABR), with electrophysiological threshold in 30dBnHL, in both ears. With six months of age, he showed bilateral absence of the ABR at 100dBnHL. The behavioral observational audiometry was impaired due to the delay in neuropsychomotor development. At eight months of age, he was submitted to Auditory Steady State Response (ASSR) and the thresholds were 50, 70, absent in 110 and in 100dB, respectively for 500, 1,000, 2,000 and 4,000Hz in the right ear, and 70, 90, 90 and absent in 100dB, respectively for 500, 1,000, 2,000 and 4,000Hz in the left ear. COMMENTS: In the first evaluation, the infant had abnormal Otoacoustic Emission and normal ABR, which became altered at six months of age. The hearing loss severity could be identified only by the ASSR, which allowed the best procedure for hearing aids adaptation. The case description highlights the importance of the hearing status follow-up for children with congenital cytomegalovirus. PMID:24473963

  15. Self-reported hearing loss among workers potentially exposed to industrial noise-United States

    SciTech Connect

    Not Available

    1988-04-15

    Noise-induced loss of hearing has been recognized as an occupational health problem since the 18th century. Occupational deafness is an irreversible, sensorineural condition that results from damage to the nerve cells of the inner ear. Recent estimates from surveys indicate that between 7.4 and 10.2 million people work at sites where the level of noise presents an increased risk of hearing loss (85 decibels (dBA) or higher). During the period of 1978-1987, an estimated $835 million was paid in workers' compensation claims for occupationally induced hearing impairment. To assess the prevalence of hearing-loss symptoms among adult workers in the United States, investigators from the National Institute of Occupational Safety and Health (NIOSH) recently analyzed data collected during the 1971 and 1977 National Health Interview Surveys (NHIS) conducted by the National Center for Health Statistics (NCHS). For this study, the prevalence of self-reported hearing loss was obtained for all persons over 17 years of age who were in the labor force at the time of interview. Data from the 1972-1974 National Occupational Hazard Survey (NOHS) were used to classify worksites by noise level. NOHS was conducted by NIOSH from 1972 to 1974 on a probability sample of approximately 5000 workplaces across the United States. The survey provides information on potential exposures of workers to chemical and physical agents. These data identified industries and occupations in which employees are exposed to continuous noise.

  16. Hearing characterization in oculoauriculovertebral spectrum: A prospective study with 10 patients.

    PubMed

    Goetze, Thayse Bienert; Sleifer, Pricila; Rosa, Rafael Fabiano Machado; da Silva, Alessandra Pawelec; Graziadio, Carla; Zen, Paulo Ricardo Gazzola

    2017-02-01

    Oculoauriculovertebral spectrum (OAVS), also known as Goldenhar syndrome, is considered a condition associated to failing of embryogenesis involving the first and second branchial arches, leading to structural abnormalities arising from it. The aim of this study is to verify the hearing features presented by patients with OAVS and provide additional information that may contribute to improvement of speech therapy. The sample consisted of 10 individuals diagnosed with OAVS and cared for by the Clinical Genetics Service. All patients underwent objective assessment of auditory function through tonal and vocal audiometry. This evaluation was completed using TOAE and BERA. The patient's age ranged from 1 year and 9 months to 27 years and 4 months. At physical examination it was found that 10 had microtia, 7 preauricular tags, 6 low-set ears, 6 ear canal atresia, and 2 preauricular pits. Among the patients, five presented with abnormal hearing. Three patients had conductive hearing loss ranging from mild to moderate, and two patients had sensorineural hearing loss from mild to profound. Three patients had hearing loss in both ears. Speech-language disorders are common in children with OAVS. Thus, the referral to the audiologist and speech pathologist is indicated as soon as possible. Early recognition and detailed understanding of aspects related to the etiology, clinical features, and outcome of patients with OAVS are essential for their proper management. © 2016 Wiley Periodicals, Inc.

  17. Hearing: Noise-Induced Hearing Loss

    MedlinePlus

    ... a cochlear implant. A cochlear implant is an electronic hearing device that replaces the damaged inner ear ... by listening to parents, teachers, television, and radio. Music, the sounds of nature, and the voices of ...

  18. Consumer preferences for hearing aid attributes: a comparison of rating and conjoint analysis methods.

    PubMed

    Bridges, John F P; Lataille, Angela T; Buttorff, Christine; White, Sharon; Niparko, John K

    2012-03-01

    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.

  19. Use of Hearing Aids by Adults with Hearing Loss

    MedlinePlus

    ... Health Info » Statistics and Epidemiology Use of Hearing Aids by Adults with Hearing Loss [text version] Note: ... displays time trends in the use of hearing aids for adults (20–69 years) and older adults ( ...

  20. Hearing impairment in patients with rheumatoid arthritis: association with anti-citrullinated protein antibodies.

    PubMed

    Lobo, Fabrício Silva; Dossi, Mario Orlando; Batista, Lígia; Shinzato, Márcia Midori

    2016-09-01

    It has been suggested that hearing impairment (HI) is one of the extra-articular features of rheumatoid arthritis (RA). Nevertheless, the prevalence and nature of HI in RA is still uncertain. The objectives were to study hearing function in patients with RA using audiometric tests and to examine whether HI correlates with autoantibodies. Hearing functions were investigated in 43 consecutive RA patients and 23 control subjects (less than 60 years old). Their sera were evaluated for the presence of rheumatoid factor (RF), anti-cyclic citrullinated peptide (anti-CCP), and anti-mutated citrullinated vimentin (anti-MCV) antibodies. HI was observed in 46.5 % of RA patients and in 30.4 % of control subjects, p = 0.32. HI was characterized as sensorineural in 80 and 85.7 % of RA patients and control subjects with HI, respectively, p = 1.00. RA patients had a worse hearing threshold for air conduction at 6 kHz in the right ear (p = 0.019) and had a decreased amplitude of otoacoustic emissions (OAEs) at 2 kHz bilaterally (p = 0.04) compared with control subjects. In the RA group, patients with and without HI were 80 and 34.78 % anti-CCP positive, respectively, p = 0.008. RA patients with and without HI were 85 and 43.48 % anti-MCV positive, respectively, p = 0.013. HI in RA patients was mainly sensorineural and was associated with anti-CCP and anti-MCV antibodies.

  1. In silico modeling of the pore region of a KCNQ4 missense mutant from a patient with hearing loss

    PubMed Central

    2012-01-01

    Background Mutation of the voltage-gated potassium channel KCNQ4 causes DFNA2-type nonsyndromic autosomal dominant sensorineural hearing loss. KCNQ4 is expressed predominantly in the auditory sensory outer hair cells, which are critical for sound amplification. Results We sequenced KCNQ4 from Japanese patients with sensorineural hearing loss, and identified a novel missense mutation encoding a Tyr270His located at the N-terminus of the highly conserved pore helix sequence. As this patient was not accessible to us and information about them was limited, we used molecular modeling to investigate whether this novel mutation is hypothetically pathogenic. A careful examination of an in silico structural model of the KCNQ4 pore region revealed that the Tyr270His mutation caused an alteration in the electrostatic surface potential of the pore helix. Conclusion We propose two possible means by which the Tyr270His mutation causes hearing loss: a positively charged His270 side chain might enhance the helix dipole moment of the pore helix, thereby destabilizing the helix and/or the pore region, or it might disturb transport of K+ through the channel by electrostatic repulsion. PMID:22420747

  2. [Consensus on diagnosis and treatment of sudden hearing loss. Asociación Madrileña de ORL].

    PubMed

    Plaza, Guillermo; Durio, Enrique; Herráiz, Carlos; Rivera, Teresa; García-Berrocal, José Ramón

    2011-01-01

    Idiopathic sudden sensorineural hearing loss is an unexplained unilateral hearing loss with onset over a period of less than 72 hours, without other known otological diseases. We present a consensus on the diagnosis, treatment and follow-up of this disease, designed by AMORL, after a systematic review of the literature from 1966 to June 2010. Diagnosis of sudden sensorineural hearing loss is based on mandatory otoscopy, acoumetry, tonal audiometry, speech audiometry, and tympanometry. After clinical diagnosis is settled, and before treatment is started, a full analysis should be done and an MRI should be requested later. Treatment is based on systemic corticosteroids (orally in most cases), helped by intratympanic doses as rescue after treatment failures. Follow-up should be done at day 7, with tonal and speech audiometries, and regularly at 15, 30, and 90 days after start of therapy, and after 12 months. By consensus, results after treatment should be reported as absolute dBs recovered in pure tonal audiometry, as improvement in the recovery rate in unilateral cases, and as improvement in speech audiometry.

  3. Simultaneous screening of multiple mutations by invader assay improves molecular diagnosis of hereditary hearing loss: a multicenter study.

    PubMed

    Usami, Shin-ichi; Nishio, Shin-ya; Nagano, Makoto; Abe, Satoko; Yamaguchi, Toshikazu

    2012-01-01

    Although etiological studies have shown genetic disorders to be a common cause of congenital/early-onset sensorineural hearing loss, there have been no detailed multicenter studies based on genetic testing. In the present report, 264 Japanese patients with bilateral sensorineural hearing loss from 33 ENT departments nationwide participated. For these patients, we first applied the Invader assay for screening 47 known mutations of 13 known deafness genes, followed by direct sequencing as necessary. A total of 78 (29.5%) subjects had at least one deafness gene mutation. Mutations were more frequently found in the patients with congenital or early-onset hearing loss, i.e., in those with an awareness age of 0-6 years, mutations were significantly higher (41.8%) than in patients with an older age of awareness (16.0%). Among the 13 genes, mutations in GJB2 and SLC26A4 were mainly found in congenital or early-onset patients, in contrast with mitochondrial mutations (12S rRNA m.1555A>G, tRNA(Leu(UUR)) m.3243A>G), which were predominantly found in older-onset patients. The present method of simultaneous screening of multiple deafness mutations by Invader assay followed by direct sequencing will enable us to detect deafness mutations in an efficient and practical manner for clinical use.

  4. Acquired Brain Injury Program.

    ERIC Educational Resources Information Center

    Schwartz, Stacey Hunter

    This paper reviews the Acquired Brain Injury (ABI) Program at Coastline Community College (California). The ABI Program is a two-year, for-credit educational curriculum designed to provide structured cognitive retraining for adults who have sustained an ABI due to traumatic (such as motor vehicle accident or fall) or non-traumatic(such as…

  5. Using Repeated Reading and Explicit Instruction to Teach Vocabulary to Preschoolers with Hearing Loss

    ERIC Educational Resources Information Center

    Bobzien, Jonna L.; Richels, Corrin; Schwartz, Kathryn; Raver, Sharon A.; Hester, Peggy; Morin, Lisa

    2015-01-01

    Children with hearing loss often experience communication and language delays that result in difficulties acquiring novel vocabulary and literacy skills. This research examined the effectiveness of using repeated storybook reading paired with explicit teacher instruction to teach novel vocabulary to young children with hearing loss who were…

  6. Comparing Deaf and Hearing Dutch Infants: Changes in the Vowel Space in the First 2 Years

    ERIC Educational Resources Information Center

    van der Stelt, Jeannette M.; Wempe, Ton G.; Pols, Louis C. W.

    2008-01-01

    The influence of the mother tongue on vowel productions in infancy is different for deaf and hearing babies. Audio material of five hearing and five deaf infants acquiring Dutch was collected monthly from month 5-18, and at 24 months. Fifty unlabelled utterances were digitized for each recording. This study focused on developmental paths in vowel…

  7. Hearing Lost, Hearing Gained. Hearing Aids Make a Difference. Tune in to Sound.

    ERIC Educational Resources Information Center

    Vandal, Ronda

    This illustrated booklet is intended to assist special education consultants, teachers, and parents to monitor hearing aid use by children with hearing impairments in the Northwest Territories (Canada). The first section presents basic information on what hearing aids are, types of personal hearing aids, and FM (frequency modulation) hearing aid…

  8. [Inner Ear Hearing Loss].

    PubMed

    Hesse, G

    2016-06-01

    Hearing loss is one of the most dominant handicaps in modern societies, which additionally very often is not realized or not admitted. About one quarter of the general population suffers from inner ear hearing loss and is therefore restricted in communicational skills. Demographic factors like increasing age play an important role as well as environmental influences and an increasing sound and noise exposure especially in leisure activities. Thus borders between a "classical" presbyacusis - if it ever existed - and envirionmentally induced hearing loss disappear. Today restrictions in hearing ability develop earlier in age but at the same time they are detected and diagnosed earlier. This paper can eventually enlighten the wide field of inner ear hearing loss only fragmentarily; therefore mainly new research, findings and developments are reviewed. The first part discusses new aspects of diagnostics of inner ear hearing loss and different etiologies.

  9. Towards a comprehensive approach for managing transitions of older workers with hearing loss.

    PubMed

    Fok, Daniel; Shaw, Lynn; Jennings, Mary Beth; Cheesman, Margaret

    2009-01-01

    Demographic and legislative trends suggest that many older workers may remain at work past the traditional retirement age. This extended work trajectory poses new challenges and opportunities for workers with acquired hearing loss as they age. Workplaces require a new approach to enable transitions of older workers with hearing loss to remain safe and productive. A review of the literature on older workers, those with hearing loss, and strategies used to accommodate them suggests that individualized and piecemeal approaches are predominant. While universal design represents a fresh ideology that may help create more accessible and usable products and environments, its application to improve workplaces for older workers with hearing loss is limited. This paper proposes that occupational science be integrated with knowledge in hearing sciences, accessibility, and usability to assist with the transitions faced by older workers with hearing loss. A more comprehensive approach including the following three key components will be posited to examine the nexus of aging, hearing loss and work: (1) the use of an occupational perspective, along with concepts in hearing sciences to examine hearing demands and improve hearing access; (2) the use of contextual processes to promote physical and social change, and (3) the inclusion of Universal Design for Hearing (UDH) considerations as stakeholders develop more hearing friendly workplaces.

  10. Hearing or speech impairment - resources

    MedlinePlus

    Resources - hearing or speech impairment ... The following organizations are good resources for information on hearing impairment or speech impairment: Alexander Graham Bell Association for the Deaf and Hard of Hearing -- www.agbell. ...

  11. Hearing Conservation Live #2430

    SciTech Connect

    Chochoms, Michael

    2016-08-09

    Occupational hearing loss is one of the most common work-related illnesses in the United States (US). From 22 to 30 million US workers are exposed to hazardous noise levels at work, and 25% of these workers will develop permanent hearing loss. Hearing loss from noise is slow and painless, and you can have a disability before you notice it. This course presents the hazards associated with workplace noise, the purpose and elements of the Los Alamos National Laboratory (LANL) Hearing Conservation Program (HCP), and controls that are available to reduce your exposure to hazardous levels of noise.

  12. Hearing protection for miners

    SciTech Connect

    Schulz, T.

    2008-10-15

    A NIOSH analysis showed that at age 50 approximately 90% of coal miners have a hearing impairment, yet noise included hearing loss is 100% preventable. The article discusses requirements of the MSHA regulations, 30 CFR Part 62 - occupational noise exposure (2000) and a 2008-MSHA document describing technologically achievable and promising controls for several types of mining machinery. Hearing protection is still required for exposure to greater than 90 dBA. These are now commercially available ways to determine how much attenuation an individual gets from a given hearing protector, known as 'fit testing'. 3 refs., 1 fig., 1 tab., 1 photo.

  13. Dentinogenesis imperfecta associated with short stature, hearing loss and mental retardation: a new syndrome with autosomal recessive inheritance?

    PubMed

    Cauwels, R G E C; De Coster, P J; Mortier, G R; Marks, L A M; Martens, L C

    2005-08-01

    The follow-up history and oral findings in two brothers from consanguineous parents suggest that the association of dentinogenesis imperfecta (DI), delayed tooth eruption, mild mental retardation, proportionate short stature, sensorineural hearing loss and dysmorphic facies may represent a new syndrome with autosomal recessive inheritance. Histological examination of the dentin matrix of a permanent molar from one of the siblings reveals morphological similarities with defective dentinogenesis as presenting in patients affected with Osteogenesis Imperfecta (OI), a condition caused by deficiency of type I collagen. A number of radiographic and histological characteristics, however, are inconsistent with classical features of DI. These findings suggest that DI may imply greater genetical heterogeneity than currently assumed.

  14. Hospital-acquired thrombocytopenia.

    PubMed

    McMahon, Christine M; Cuker, Adam

    2014-10-01

    The development of thrombocytopenia is common in hospitalized patients and is associated with increased mortality. Frequent and important causes of thrombocytopenia in hospitalized patients include etiologies related to the underlying illness for which the patient is admitted, such as infection and disseminated intravascular coagulation, and iatrogenic etiologies such as drug-induced immune thrombocytopenia, heparin-induced thrombocytopenia, posttransfusion purpura, hemodilution, major surgery, and extracorporeal circuitry. This review presents a brief discussion of the pathophysiology, distinguishing clinical features, and management of these etiologies, and provides a diagnostic approach to hospital-acquired thrombocytopenia that considers the timing and severity of the platelet count fall, the presence of hemorrhage or thrombosis, the clinical context, and the peripheral blood smear. This approach may offer guidance to clinicians in distinguishing among the various causes of hospital-acquired thrombocytopenia and providing management appropriate to the etiology.

  15. Desmosomes in acquired disease

    PubMed Central

    Stahley, Sara N.; Kowalczyk, Andrew P.

    2015-01-01

    Desmosomes are cell-cell junctions that mediate adhesion and couple the intermediate filament cytoskeleton to sites of cell-cell contact. This architectural arrangement functions to integrate adhesion and cytoskeletal elements of adjacent cells. The importance of this robust adhesion system is evident in numerous human diseases, both inherited and acquired, that occur when desmosome function is compromised. This review focuses on autoimmune and infectious diseases that impair desmosome function. In addition, we discuss emerging evidence that desmosomal genes are often misregulated in cancer. The emphasis of our discussion is placed on how human diseases inform our understanding of basic desmosome biology, and in turn, how fundamental advances in the cell biology of desmosomes may lead to new treatments for acquired diseases of the desmosome. PMID:25795143

  16. Desmosomes in acquired disease.

    PubMed

    Stahley, Sara N; Kowalczyk, Andrew P

    2015-06-01

    Desmosomes are cell-cell junctions that mediate adhesion and couple the intermediate filament cytoskeleton to sites of cell-cell contact. This architectural arrangement integrates adhesion and cytoskeletal elements of adjacent cells. The importance of this robust adhesion system is evident in numerous human diseases, both inherited and acquired, which occur when desmosome function is compromised. This review focuses on autoimmune and infectious diseases that impair desmosome function. In addition, we discuss emerging evidence that desmosomal genes are often misregulated in cancer. The emphasis of our discussion is placed on the way in which human diseases can inform our understanding of basic desmosome biology and in turn, the means by which fundamental advances in the cell biology of desmosomes might lead to new treatments for acquired diseases of the desmosome.

  17. Effects of noise reduction on speech intelligibility, perceived listening effort, and personal preference in hearing-impaired listeners.

    PubMed

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

    2014-10-13

    This study evaluates the perceptual effects of single-microphone noise reduction in hearing aids. Twenty subjects with moderate sensorineural hearing loss listened to speech in babble noise processed via noise reduction from three different linearly fitted hearing aids. Subjects performed (a) speech-intelligibility tests, (b) listening-effort ratings, and (c) paired-comparison ratings on noise annoyance, speech naturalness, and overall preference. The perceptual effects of noise reduction differ between hearing aids. The results agree well with those of normal-hearing listeners in a previous study. None of the noise-reduction algorithms improved speech intelligibility, but all reduced the annoyance of noise. The noise reduction that scored best with respect to noise annoyance and preference had the worst intelligibility scores. The trade-off between intelligibility and listening comfort shows that preference measurements might be useful in addition to intelligibility measurements in the selection of noise reduction. Additionally, this trade-off should be taken into consideration to create realistic expectations in hearing-aid users.

  18. Identification of a novel pathogenic OTOF variant causative of nonsyndromic hearing loss with high frequency in the Ashkenazi Jewish population

    PubMed Central

    Fedick, Anastasia M; Jalas, Chaim; Swaroop, Ananya; Smouha, Eric E; Webb, Bryn D

    2016-01-01

    Mutations in the OTOF gene have previously been shown to cause nonsyndromic prelingual deafness (DFNB9, OMIM 601071) as well as auditory neuropathy/dys-synchrony. In this study, the OTOF NM_194248.2 c.5332G>T, p.Val1778Phe variant was identified in a large Ashkenazi Jewish family as the causative variant in four siblings with hearing loss. Our analysis reveals a carrier frequency of the OTOF c.5332G>T, p.Val1778Phe variant of 1.27% in the Ashkenazi Jewish population, suggesting that this variant may be a significant contributor to nonsyndromic sensorineural hearing loss and should be considered for inclusion in targeted hearing loss panels for this population. Of note, the degree of hearing loss associated with this phenotype ranged from mild to moderately severe, with two of the four siblings not known to have hearing loss until they were genotyped and underwent pure tone audiometry and auditory brainstem response testing. The phenotypic variability along with the auditory neuropathy/dys-synchrony, which allows for the production of otoacoustic emissions, supports that nonsyndromic hearing loss caused by OTOF mutations may be much more common in the Ashkenazi Jewish population than currently appreciated due to a lack of diagnosis. PMID:27621663

  19. Underwater hearing: A review

    NASA Astrophysics Data System (ADS)

    Al-Masri, M.; Martin, A.; Nedwell, J.

    1993-05-01

    In view of the prevalence of hearing loss among commercial divers and the absence of widely accepted noise exposure limits for occupational underwater use, a review of studies of underwater hearing thresholds and hearing mechanisms was undertaken with the ultimate aim of developing noise exposure limits. Previous studies of underwater hearing thresholds appear to show that the ear underwater is less sensitive than compared with air. However, a surprisingly wide range of values for underwater hearing thresholds was reported, for example 35-90 dB SPL(re 20 MuPa) at 0.25 kHz and 30-80 dB at 1 kHz. No representative single threshold curve can be extracted with any validity. Possible reasons for such a wide scatter of results include high underwater ambient noise levels which may have masked the subjects underwater hearing thresholds, ill defined stimuli and underwater sound fields, and variable and informal audiometric methodology. Previous authors have proposed three somewhat interlinked theories to explain how sound is transmitted from water to the cochlea. These involve: the 'auricular' conduction pathway, the bone conduction pathway, and the dual conduction pathway. Up to this day, no one pathway has been shown to predominate, and all of them have been poorly evaluated. It is also possible that the presence of air bubbles in the ear canal and increased water depth may have significant effects on underwater hearing thresholds. These effects may be dependent on the underwater hearing mechanism. Again, the studies reviewed give conflicting results and no valid conclusion can be drawn. It is apparent that further experimental studies are required to establish underwater hearing thresholds and to provide an understanding of the mechanisms of hearing underwater. These should be based upon suitable facilities and methodologies for testing hearing thresholds underwater following modern and scientific audio metric practice.

  20. Evaluation of the occurrence of canine congenital sensorineural deafness in puppies of predisposed dog breeds using the brainstem auditory evoked response.

    PubMed

    Płonek, Marta; Giza, Elżbieta; Niedźwiedź, Artur; Kubiak, Krzysztof; Nicpoń, Józef; Wrzosek, Marcin

    2016-12-01

    Canine congenital sensorineural deafness (CCSD) affects predisposed breeds of dogs and is primarily caused by an atrophy of the stria vascularis of the organ of Corti. The analysis of the brainstem auditory evoked response (BAER) is a reliable method for the evaluation of hearing in animals