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Sample records for acquired sensorineural hearing

  1. 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.

  2. Prestin as a biochemical marker for early detection of acquired sensorineural hearing loss.

    PubMed

    Parham, Kourosh

    2015-08-01

    Acquired sensorineural hearing loss and tinnitus can come about through various etiologies such as exposure to excessively loud noise or drugs with ototoxic properties. As such, acquired hearing loss is a common source of morbidity which deleteriously affects the ability to communicate. At present our ability to detect acquired hearing loss and tinnitus at its earliest stages is limited and there are no adjuncts to audiometric evaluation. The earliest cellular targets of noise and ototoxins in the cochlea are the outer hair cells (OHC). I hypothesize that serum assays of OHC specific protein, prestin, will allow detection and quantification of OHC damage before audiometric testing can identify presence of hearing loss. At present, there are no data available to evaluate this hypothesis, but initial evaluation can readily be carried out using existing experimental animal models of ototoxicity and noise-induced hearing loss. Early detection of OHC damage is critical to adoption of measures aimed at ameliorating hearing loss and tinnitus, thus reducing permanent deficits and disability. PMID:25920562

  3. The Design and Screening of Drugs to Prevent Acquired Sensorineural Hearing Loss

    PubMed Central

    Mukherjea, Debashree; Rybak, Leonard P.; Sheehan, Kelly E; Kaur, Tejbeer; Ramkumar, Vickram; Jajoo, Sarvesh; Sheth, Sandeep

    2011-01-01

    Introduction Sensorineural hearing loss affects a high percentage of the population. Ototoxicity is a serious and pervasive problem in patients treated with cisplatin. Strategies to ameliorate ototoxicity without compromising on antitumor activity of treatments are urgently needed. Similar problems occur with aminoglycoside antibiotic therapy for infections. Noise-induced hearing loss affects a large number of people. The use of ear protection is not always possible or effective. The prevention of hearing loss with drug therapy would have a huge impact in reducing the number of persons with hearing loss from these major causes. Areas covered This review discusses significant research findings dealing with the use of protective agents against hearing loss caused by cisplatin, aminoglycoside antibiotics and noise trauma. The efficacy in animal studies and the application of these protective agents in clinical trials that are ongoing are presented. Expert opinion The reader will gain new insights into current and projected future strategies to prevent sensorineural hearing loss from cisplatin chemotherapy, aminoglycoside antibiotic therapy and noise exposure. The future appears to offer numerous agents to prevent hearing loss caused by cisplatin, aminoglycoside antibiotics and noise. Novel delivery systems will provide ways to guide these protective agents to the desired target areas in the inner ear and will circumvent problems with therapeutic interference of anti-tumor and antibiotics agents and will minimize undesired side effects. PMID:22646075

  4. 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).

  5. 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). PMID:22504750

  6. 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.

  7. 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

  8. 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.

  9. 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 .

  10. Methadone Induced Sensorineural Hearing Loss

    PubMed Central

    Saifan, Chadi; Barakat, Iskandar; El-Sayegh, Suzanne

    2013-01-01

    Background. Sudden sensorineural hearing loss (SSHL) caused by opiate abuse or overuse has been well documented in the medical literature. Most documented case reports have involved either heroin or hydrocodone/acetaminophen. Recently, case reposts of methadone induced SSHL have been published. Case Report. We present the case of a 31-year-old man who developed SSHL after a methadone overdose induced stupor. He was subsequently restarted on methadone at his regular dose. On follow-up audiometry exams, he displayed persistent moderately severe sensorineural hearing loss bilaterally. Discussion. This case is notable because unlike all but one previously reported case, the patient—who was restated on methadone—did not make a complete recovery. Conclusion. Methadone overuse in rare cases causes SSHL. PMID:23983704

  11. Sensorineural deafness

    MedlinePlus

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

  12. 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.

  13. Naproxen-associated sudden sensorineural hearing loss.

    PubMed

    McKinnon, B J; Lassen, L F

    1998-11-01

    Naproxen is a commonly used nonsteroidal anti-inflammatory drug (NSAID) whose side effects include tinnitus and transient hearing loss. Sudden sensorineural hearing loss has rarely been reported as a result of NSAID use. This usually occurs in patients taking other ototoxic medications, with poor renal function, or with autoimmune disease. This article reports the case of an otherwise healthy patient who experienced permanent sensorineural hearing loss after a brief course of naproxen and reviews the literature on NSAID-related permanent sensorineural hearing loss.

  14. Sensorineural hearing loss in chronic otitis media.

    PubMed

    MacAndie, C; O'Reilly, B F

    1999-06-01

    Although many studies have demonstrated an association between chronic otitis media (COM) and sensorineural hearing loss (SNHL), there still remains disagreement about the relationship. A retrospective study was conducted to examine the relationship between sensorineural hearing loss and chronic otitis media. Forty-one patients met the following criteria: unilateral COM and no history of head injury, meningitis or previous otological surgery. The differences in preoperative bone conduction threshold between diseased and control (contralateral normal) ear were statistically significant (P < 0.01) and varied from 5.24 to 9.02 dB across the frequency range. The effect of duration of disease on the degree of SNHL was also analysed but no correlation was found. The presence of cholesteatoma and/or ossicular erosion was not associated with a significantly increased risk of sensorineural hearing loss. PMID:10384849

  15. 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.

  16. Sudden Sensorineural Hearing Loss: Primary Care Update.

    PubMed

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

    2016-06-01

    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. 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

  18. Sudden Sensorineural Hearing Loss: Primary Care Update.

    PubMed

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

    2016-06-01

    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.

  19. 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

  20. 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.

  1. 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. PMID:27011703

  2. Sensorineural hearing loss in Kawasaki disease

    PubMed Central

    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 2nd 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. PMID:27011703

  3. 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

  4. 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

  5. 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

  6. 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.

  7. Progressive bilateral sensorineural hearing loss induced by an antithyroid drug.

    PubMed

    Sano, Masaki; Kitahara, Nobuo; Kunikata, Ryutarou

    2004-01-01

    We report a patient with antithyroid drug-induced progressive bilateral sensorineural hearing loss associated with myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA). While antithyroid drugs have been linked to MPO-ANCA-associated small-vessel vasculitis, sensorineural hearing loss rarely was noted. A 36-year-old man treated for hyperthyroidism with propylthiouracil (PTU) developed progressive bilateral sensorineural hearing loss accompanied by fever and arthritis. MPO-ANCA were demonstrated in serum. Distortion product otoacoustic emissions test results suggested dysfunction of outer hair cells of the organ of Corti. Inner ear blood flow impairment from ANCA-associated small-vessel vasculitis presumably caused cochlear dysfunction. PTU withdrawal and high-dose methylprednisolone administration greatly improved hearing on both sides.

  8. [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.

  9. Hyperbaric oxygen therapy for idiopathic sudden sensorineural hearing loss.

    PubMed

    Murphy-Lavoie, H; Piper, S; Moon, R E; Legros, T

    2012-01-01

    Idiopathic sudden sensorineural hearing loss (ISSHL) is the newest indication approved by the Undersea and Hyperbaric Medical Society's Hyperbaric Oxygen Therapy Committee. Idiopathic sudden sensorineural hearing loss appears to be characterized by hypoxia in the perilymph and therefore the scala tympani and the organ of Corti. A review of the literature reveals more than 100 publications evaluating the use of hyperbaric oxygen (HBO2) for the treatment of ISSHL, including eight randomized controlled trials. The best and most consistent results are obtained when HBO2 is initiated within two weeks of symptom onset and combined with corticosteroid treatment. The average hearing gain is 19.3 dB for moderate hearing loss and 37.7 dB for severe cases. This improvement brings hearing deficits from the moderate/severe range into the slight/no impairment range. This is a significant gain that can markedly improve a patient's quality of life, both clinically and functionally.

  10. 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.

  11. 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. PMID:26161278

  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. 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.

  14. 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

  15. Hereditary Non-Syndromic Sensorineural Hearing Loss

    PubMed Central

    Schrijver, Iris

    2004-01-01

    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. PMID:15507665

  16. Myeloperoxidase-antineutrophil cytoplasmic antibody-associated sensorineural hearing loss.

    PubMed

    Maguchi, S; Fukuda, S; Chida, E; Terayama, Y

    2001-05-01

    A 36-year-old female with hyperthyroidism that had been treated with propilthiouracil (PTU) complained of tinnitus and hearing loss in both ears. She was treated with steroid administration by an otolaryngologist; however, hearing continued to fluctuate when the steroids were tapered. Laboratory evaluation revealed a decreased complement level and elevated levels of immunoglobulin M (IgM) and myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA). With the withdrawal of PTU and high-dose methylprednisolone, she had excellent return of right-sided hearing. In recent years, there have been many reports about MPO-ANCA-associated small vessel vasculitis. Although any organ may be affected by this disease, there are no reports about MPO-ANCA-associated progressive hearing loss without any other organ involvement. The present case suggests the possibility that inner ear blood flow impairment due to ANCA-associated small vessel vasculitis induces the so-called autoimmune sensorineural hearing loss.

  17. Acquired Hearing Loss in Children.

    PubMed

    Kenna, Margaret A

    2015-12-01

    Hearing loss is the most common congenital sensory impairment. According to National Health and Nutrition Examination Survey data from 2001 to 2008, 20.3% of subjects aged greater than or equal to 12 had unilateral or bilateral hearing loss. The World Health Organization notes that, worldwide, there are 360 million people with disabling hearing loss, with 50% preventable. Although many hearing losses are acquired, many others are manifestations of preexisting conditions. The purpose of a pediatric hearing evaluation is to identify the degree and type of hearing loss and etiology and to outline a comprehensive strategy that supports language and social development and communication.

  18. Gene therapy for sensorineural hearing loss.

    PubMed

    Chien, Wade W; Monzack, Elyssa L; McDougald, Devin S; Cunningham, Lisa L

    2015-01-01

    Gene therapy is a promising treatment modality that is being explored for several inherited disorders. Multiple human gene therapy clinical trials are currently ongoing, but few are directed at hearing loss. Hearing loss is one of the most prevalent sensory disabilities in the world, and genetics play an important role in the pathophysiology of hearing loss. Gene therapy offers the possibility of restoring hearing by overcoming the functional deficits created by the underlying genetic mutations. In addition, gene therapy could potentially be used to induce hair cell regeneration by delivering genes that are critical to hair cell differentiation into the cochlea. In this review, we examine the promises and challenges of applying gene therapy to the cochlea. We also summarize recent studies that have applied gene therapy to animal models of hearing loss.

  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. Sudden onset unilateral sensorineural hearing loss after rabies vaccination.

    PubMed

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

    2015-01-01

    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. PMID:26670892

  1. 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

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

    PubMed

    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. 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.

  4. 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. PMID:22754856

  5. 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.

  6. Risk of Sudden Sensorineural Hearing Loss in Patients with Common Preexisting Sensorineural Hearing Impairment: A Population-Based Study in Taiwan

    PubMed Central

    Koo, Malcolm; Hwang, Juen-Haur

    2015-01-01

    Objective The role of preexisting sensorineural hearing impairment on the risk for sudden sensorineural hearing loss (SSHL) is still unclear. In this study, we aimed to assess the risk of SSHL in patients with common preexisting sensorineural hearing impairment using population-based data. Methods A population-based case-control study design was used to analyze claims data between January 2001 and December 2011 obtained from the Taiwan National Health Insurance Research Database. The cases consisted of 514 patients with SSHL and the controls were frequency matched to 2,570 cases by sex, 10-year age group, and year of index date. Common sensorineural hearing impairments were retrospectively assessed in the cases and controls. Associations between sensorineural hearing impairment and risk of SSHL were evaluated using unconditional univariate and multivariate logistic regression analyses. Results The mean age for the 3,084 study subjects was 53.1 years (standard deviation, S.D. = 15.6). Of the 514 cases, 49 (9.5%) had sensorineural hearing impairment while only 44 (1.7%) of the 2,570 controls had the same condition. Univariate logistic regression analyses indicated that preexisting sensorineural hearing impairment was significantly associated with SSHL (odds ratio, OR = 6.05, p < 0.001). Other comorbidities including hypertension, diabetes mellitus, and hyperlipidemia also showed significant associations with SSHL. Similar results were obtained when the association between SSHL and sensorineural hearing impairment was adjusted with either all the covariates (adjusted OR = 6.22, p < 0.001) or with only those selected using a backward elimination procedure (adjusted OR = 6.20, p < 0.001). Conclusions Results from this population-based case-control study revealed that common sensorineural hearing impairment might be a novel risk factor for SSHL. PMID:25815507

  7. Course of hearing recovery according to frequency in patients with acute acoustic sensorineural hearing loss.

    PubMed

    Harada, Hirofumi; Ichikawa, Daisuke; Imamura, Akihide

    2008-01-01

    Through pure-tone audiometry, we studied the course of hearing recovery in 24 ears of 20 men (ages 18-48 years) who had acute acoustic sensorineural hearing loss (ASHL). All subjects were members of the Japanese Self-Defense Force. The hearing level in 5 ears returned to normal, the hearing level of 13 ears recovered but was not within the normal range, and the hearing level of 6 ears was unchanged. The time from noise exposure to presentation was longer in patients with unchanged hearing than in other patients. Recovery of hearing was poorest at 4,000 Hz, followed by 8,000 and 2,000 Hz. We concluded that hearing in patients with acute ASHL is likely to return to normal when the hearing level at 4,000 Hz recovers gradually; partial recovery of hearing is expected when the hearing level at 4,000 Hz reaches an early plateau. PMID:18616091

  8. A bone-anchored hearing aid for patients with pure sensorineural hearing impairment: a pilot study.

    PubMed

    Stenfelt, S; Håkansson, B; Jönsson, R; Granström, G

    2000-01-01

    This pilot study assesses the potential benefits of an optimized bone-anchored hearing aid (BAHA) for patients with a mild to moderate pure sensorineural high frequency hearing impairment. The evaluation was conducted with eight first-time hearing aid users by means of psycho-acoustic sound field measurements and a questionnaire on subjective experience; all of the patients benefited from the BAHA. On average, the eight patients showed improvement in PTA threshold of 3.4 dB and in speech intelligibility in noise of 14%. Seven of the subjects, also fitted with present standard air conduction hearing aids (ACHA) found the ACHA thresholds to be improved more than the BAHA ones. In speech tests, the ACHA was only slightly better; these patients chose between their different hearing aids according to the sound environment. Although the BAHA was preferred for wearing and sound comfort, it cannot be used as the sole aid for patients with pure sensorineural impairment.

  9. Sensorineural hearing loss in patients with coronary artery bypass surgery

    PubMed Central

    Sadeghi, Mohsen Mirmohammad; Radman, Masoud; Bidaki, Reza; Sonbolestan, Mehdi

    2013-01-01

    Background: This study is planned to obtain a better understanding of the correlation between sudden sensorineural hearing loss and cardiopulmonary bypass. There are many causes for sudden hearing loss which include infectious, circulatory, inner ear problems like meniere's disease, neoplastic, traumatic, metabolic, neurologic, immunologic, toxic, cochlear, idiopathic (unknown cause) and other causes. One of the less common cause is surgery include cardiopulmonary bypass procedures. Materials and Methods: This study is a self controlled clinical trial on 105 patients that was carried out in chamran Hospital, Esfahan, Iran. Participants were including all those patients undergoing coronary artery bypass surgery in the hospital who fell under the criteria for inclusion. Patients underwent audiometric testing at our hospital on three or two different occasions during the course of this study, Initially before the procedure to test the baseline hearing capacity; then two week after the procedure to assess any changes in hearing ability following the surgery. Data analysis performed by co-variance analysis. Results: In our study the changes in the threshold of hearing in frequency of 1000 in right ear and in frequencies of 2000 and 4000 in left ear were significant, but this changes were about 2-3 db and were not noticeable. The difference in degree of SNHL, before and after surgery in different frequencies were been shown. Conclusion: As loss of the patients with symptomatic sensory neural hearing loss in this study, It isn't commanded the routin auditory assessment pre and post surgery was been done. PMID:23930250

  10. Autoimmune sensorineural hearing loss: the otology-rheumatology interface.

    PubMed

    Mijovic, Tamara; Zeitouni, Anthony; Colmegna, Inés

    2013-05-01

    Autoimmune sensorineural hearing loss (SNHL) is a rare clinical entity characterized by a progressive fluctuating bilateral asymmetric SNHL that develops over several weeks to months. Vestibular symptoms, tinnitus and aural fullness are present in up to 50% of patients. Due to the lack of specific diagnostic tests, both clinical suspicion and responsiveness to corticosteroids are the pillars for the diagnosis of autoimmune SNHL. The evaluation of patients in whom this condition is suspected should include a detailed history and physical examination, an audiogram, an MRI and a limited laboratory workup to exclude secondary causes of hearing loss. The low frequency of this condition, the heterogeneity in the designs of the available studies and the absence of randomized trials comparing treatment responses and assessing long-term outcomes are some of the factors accounting for the limited evidence to guide the clinician in the approach to the diagnosis and treatment of autoimmune SNHL.

  11. 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. PMID:26670765

  12. 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

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

    PubMed

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

    2016-04-20

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

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

    PubMed

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

    2016-04-20

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

  15. Temporary bilateral sensorineural hearing loss following cardiopulmonary bypass -A case report-.

    PubMed

    Son, Hyo Jung; Joh, Jung Hwa; Kim, Wook Jong; Chin, Ji Hyun; Choi, Dae Kee; Lee, Eun Ho; Sim, Ji Yeon; Choi, In-Cheol

    2011-08-01

    Sudden sensorineural hearing loss has been reported to occur following anesthesia and various non-otologic surgeries, mostly after procedures involving cardiopulmonary bypass. Unilateral sensorineural hearing loss resulting from microembolism is an infrequent complication of cardiopulmonary bypass surgery that has long been acknowledged. Moreover, there are few reports on the occurrence of bilateral sensorineural hearing loss without other neurologic deficits and its etiology has also not been determined. We describe here a rare case of bilateral hearing loss without other neurologic deficits in an otherwise healthy 27-year-old woman who underwent cardiopulmonary bypass surgery for repair of severe mitral valve stenosis. The patient suffered from profound sensorineural hearing loss in both ears that was recognized immediately upon extubation, and audiometry tests confirmed the diagnosis. Without any treatment, her hearing recovered almost completely by the time of her discharge one week after surgery.

  16. Temporary bilateral sensorineural hearing loss following cardiopulmonary bypass -A case report-

    PubMed Central

    Son, Hyo Jung; Joh, Jung Hwa; Kim, Wook Jong; Chin, Ji Hyun; Choi, Dae Kee; Lee, Eun Ho; Sim, Ji Yeon

    2011-01-01

    Sudden sensorineural hearing loss has been reported to occur following anesthesia and various non-otologic surgeries, mostly after procedures involving cardiopulmonary bypass. Unilateral sensorineural hearing loss resulting from microembolism is an infrequent complication of cardiopulmonary bypass surgery that has long been acknowledged. Moreover, there are few reports on the occurrence of bilateral sensorineural hearing loss without other neurologic deficits and its etiology has also not been determined. We describe here a rare case of bilateral hearing loss without other neurologic deficits in an otherwise healthy 27-year-old woman who underwent cardiopulmonary bypass surgery for repair of severe mitral valve stenosis. The patient suffered from profound sensorineural hearing loss in both ears that was recognized immediately upon extubation, and audiometry tests confirmed the diagnosis. Without any treatment, her hearing recovered almost completely by the time of her discharge one week after surgery. PMID:21927689

  17. 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

  18. 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

  19. Multiple-ASSR Interactions in Adults with Sensorineural Hearing Loss.

    PubMed

    Ishida, Ieda M; Stapells, David R

    2012-01-01

    The multiple auditory steady-state response (multiple-ASSR) technique, where thresholds for up to 8 frequencies (4 in each ear) are obtained simultaneously, is currently of great interest for audiometric assessment of infants. Although threshold estimates using the multiple-ASSR appear to be reasonably accurate, it is not currently known whether it is more efficient to use multiple stimuli or single stimuli when testing individuals with sensorineural hearing loss (SNHL). The current study investigated the effect of single versus multiple simultaneous stimuli on the 80- and 40-Hz ASSRs in adults with normal hearing or SNHL. Results showed significant interactions (i.e., decreased amplitudes) for both ASSRs going from single to multiple stimuli in one ear. Going from multiple one ear to multiple two ears did not further reduce the amplitude of the 80-Hz ASSR. At the 40-Hz rate, however, there was a further amplitude decrease going from one-ear multiple to two-ear multiple stimuli. Importantly, these interactions did not differ between the normal-hearing and SNHL groups. Although supportive of the multiple-ASSR technique, there are likely situations where it is more efficient to use single stimuli. Future studies are required to assess these interactions in infants with varying degrees and configurations of hearing loss. PMID:23049561

  20. 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.

  1. Strategies for developing novel therapeutics for sensorineural hearing loss

    PubMed Central

    Nakagawa, Takayuki

    2014-01-01

    Sensorineural hearing loss (SNHL) is a common disability in the world; however, at present, options for the pharmacological treatment of SNHL are very limited. Previous studies involving human temporal bone analyses have revealed that the degeneration of the cochlea is a common mechanism of SNHL. A major problem for the development of novel pharmacotherapy for SNHL has been the limited regeneration capacity in mammalian cochlear cells. However, recent progress in basic studies has led to several effective strategies for the induction of regeneration in the mammalian cochlea, in accordance with the stage of degeneration. In addition, recent advances in the identification of human deafness genes and their characterization in mouse models have elucidated cellular and/or molecular mechanisms of SNHL, which will contribute to clarify molecular targets of pharmacotherapy for treatment of SNHL. PMID:25278894

  2. Unilateral sensorineural hearing loss: analysis of 200 consecutive cases.

    PubMed

    Kumar, A; Maudelonde, C; Mafee, M

    1986-01-01

    The spectrum of diseases diagnosed and the protocol used in the investigation of 200 consecutive patients with unilateral sensorineural hearing loss (SNHL) is presented. The diagnostic tests found most useful in defining the site of the lesion were a combination of a well-masked pure tone audiogram, speech discrimination score (SDS), acoustic reflex test (ART), and a vestibular evaluation using photoelectric nystagmography (PENG) and the Torok Monothermal Differential Caloric Test. With this protocol, the site of the lesion was correctly defined in 94% of patients with unilateral progressive SNHL. Laboratory and radiological studies confirmed a variety of lesions other than acoustic tumors. It is, therefore, felt that the primary objective of investigations should be to define the site of the lesion rather than aim to diagnose a specific disease entity.

  3. Sudden sensorineural hearing loss during oral anticoagulant therapy.

    PubMed

    Mierzwa, Kathleen; Schneider, Gerlind; Müller, Andreas

    2004-11-01

    This study investigated the role of sudden sensorineural hearing loss (SSNHL) as a symptom in oral anticoagulant therapy with vitamin K antagonists (Phenprocoumon; Marcumar, Falithrom). Vascular compromise of the cochlea due to thrombosis, embolus, reduced blood flow or vasospasm is one of the four possible pathways that can lead to SSNHL. Oral anticoagulant therapy should prevent thrombosis; if it does not the question arises as to whether the anticoagulation is working, or the wrong hypothesis of vascular compromise has been made. Patients with SSNHL during oral anticoagulant therapy who were admitted to the ENT Department of the University Hospital in Jena from 1998 to 2001 were included. The pure-tone audiograms and the prothrombin time (PT) values before and after the event of the SSNHL were evaluated. The study found 10 patients with SSNHL during oral anticoagulant therapy. Although the audiograms showed some improvement in the majority of cases, three cases showed almost no improvement in hearing. On admission, half of the patients showed a PT-value higher than 30 per cent and in nine cases a PT-value >30 per cent could be demonstrated at least once during testing. It was not possible to demonstrate a relationship between the SSNHL and oral anticoagulation. Vascular compromise cannot be excluded as a cause for sudden hearing loss in patients undergoing oral anticoagulant therapy. It is possible that oral anticoagulants influence the viscosity of the plasma leading to interference with the microcirculation in the inner ear. Further research into this area is currently being conducted.

  4. 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

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

    PubMed

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

    2016-01-01

    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

  6. 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

  7. Speech Coding in the Midbrain: Effects of Sensorineural Hearing Loss.

    PubMed

    Carney, Laurel H; Kim, Duck O; Kuwada, Shigeyuki

    2016-01-01

    In response to voiced speech sounds, auditory-nerve (AN) fibres phase-lock to harmonics near best frequency (BF) and to the fundamental frequency (F0) of voiced sounds. Due to nonlinearities in the healthy ear, phase-locking in each frequency channel is dominated either by a single harmonic, for channels tuned near formants, or by F0, for channels between formants. The alternating dominance of these factors sets up a robust pattern of F0-synchronized rate across best frequency (BF). This profile of a temporally coded measure is transformed into a mean rate profile in the midbrain (inferior colliculus, IC), where neurons are sensitive to low-frequency fluctuations. In the impaired ear, the F0-synchronized rate profile is affected by several factors: Reduced synchrony capture decreases the dominance of a single harmonic near BF on the response. Elevated thresholds also reduce the effect of rate saturation, resulting in increased F0-synchrony. Wider peripheral tuning results in a wider-band envelope with reduced F0 amplitude. In general, sensorineural hearing loss reduces the contrast in AN F0-synchronized rates across BF. Computational models for AN and IC neurons illustrate how hearing loss would affect the F0-synchronized rate profiles set up in response to voiced speech sounds. PMID:27080684

  8. Vestibular schwannoma in patients with sudden sensorineural hearing loss.

    PubMed

    Lee, Jong Dae; Lee, Byung Don; Hwang, Sun Chul

    2011-03-01

    Sudden sensorineural hearing loss (SSNHL) has several etiologies. It may be a presenting symptom of vestibular schwannoma (VS). This study aimed to establish the incidence of VS in patients with SSNHL, and we report several unusual cases among these patients. We reviewed retrospectively the charts and magnetic resonance imaging (MRI) findings of all adult patients who presented with SSNHL between 2002 and 2008. We utilized three-dimensional fast imaging with steady-state acquisition temporal MRI as a screening method. Of the 295 patients with SSNHL, VS was found in 12 (4%). All patients had intrameatal or small to medium-sized tumors. There were three cases with SSNHL in one ear and an incidental finding of intracanalicular VS in the contralateral ear. There were four cases of VS that showed good recovery from SSNHL with corticosteroid treatment. There were two cases that mimicked labyrinthitis with hearing loss and vertigo. A greater number of cases than expected of VS were detected in patients with SSNHL, as a result of increasing widespread use of MRI. Various unusual findings in these patients were identified. MRI would seem to be mandatory in all cases of SSNHL.

  9. 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

  10. 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.

  11. 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. PMID:22128658

  12. Progressive hearing loss following acquired cytomegalovirus infection in an immunocompromised child.

    PubMed

    Kato, Ken; Otake, Hironao; Tagaya, Mitsuhiko; Takahashi, Yoshiyuki; Ito, Yoshinori; Hama, Asahito; Muramatsu, Hideki; Kojima, Seiji; Naganawa, Shinji; Nakashima, Tsutomu

    2013-01-01

    We report a rare case of progressive hearing loss after acquired CMV infection in a child with Langerhans cell histiocytosis (LCH). A 5-month-old female was diagnosed as having LCH. When she was 14 months old, she received an unrelated donor umbilical cord blood transfusion for the treatment of intractable LCH. CMV infection was confirmed after the blood transfusion. Because her own umbilical cord had no CMV, the CMV infection was not congenital. When she was 7 years old, mixed hearing loss was noted with bilateral otitis media with effusion. After that time, the sensorineural hearing loss progressed to bilateral profound hearing loss over 3 years. Three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging with gadolinium contrast enhancement revealed a high intensity area in the inner ear that suggested bilateral labyrinthitis. This case demonstrates the possibility that, under the immunodeficiency, the acquired CMV infection causes progressive sensorineural hearing loss.

  13. 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

  14. 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.

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

    PubMed Central

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

    2014-01-01

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

  16. 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.

  17. Gentamicin Exposure and Sensorineural Hearing Loss in Preterm Infants

    PubMed Central

    Fuchs, Aline; Zimmermann, Lara; Bickle Graz, Myriam; Cherpillod, Jacques; Tolsa, Jean-François; Buclin, Thierry; Giannoni, Eric

    2016-01-01

    Objective To evaluate the impact of gentamicin exposure on sensorineural hearing loss (SNHL) in very low birth weight (VLBW) infants. Methods Exposure to gentamicin was determined in infants born between 1993 and 2010 at a gestational age < 32 weeks and/or with a birthweight < 1500 g, who presented with SNHL during the first 5 years of life. For each case, we selected two controls matched for gender, gestational age, birthweight, and year of birth. Results We identified 25 infants affected by SNHL, leading to an incidence of SNHL of 1.58% in our population of VLBW infants. The proportion of infants treated with gentamicin was 76% in the study group and 70% in controls (p = 0.78). The total cumulated dose of gentamicin administered did not differ between the study group (median 10.2 mg/kg, Q1-Q3 1.6–13.2) and the control group (median 7.9 mg/kg, Q1-Q3 0–12.8, p = 0.47). The median duration of gentamicin treatment was 3 days both in the study group and the control group (p = 0.58). Maximum predicted trough serum levels of gentamicin, cumulative area under the curve and gentamicin clearance were not different between cases and controls. Conclusion The impact of gentamicin on SNHL can be minimized with treatments of short duration, monitoring of blood levels and dose adjustment. PMID:27390846

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

    PubMed

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

    2010-12-01

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

  19. 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.

  20. 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…

  1. 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…

  2. 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.

  3. Occupational noise exposure and sensorineural hearing loss among workers of a steel rolling mill.

    PubMed

    Ologe, Foluwasayo E; Akande, Tanimola M; Olajide, Toye G

    2006-07-01

    Hearing loss is one of the most common chronic health conditions and has important implications for the patient's quality of life. However, hearing loss is substantially underestimated and under treated. The purpose of this study was to determine the prevalence of sensorineural hearing loss among the workers in a steel rolling mill in Nigeria. Each of the 150 randomly selected subjects had a structured questionnaire administered to them, followed by a full otological examination. Of these, 116 had tympanometry and pure-tone audiometry. Also a noise mapping of their respective work units was done. The workers were exposed to noise levels varying from 49 to 93 dBA. About 28.2% of the 103 who had their audiogram analysed had mild to moderate sensorineural hearing loss in their better ear and 56.8% of them had mild to moderate sensorineural hearing loss in their worse ear. The pure-tone average and the average hearing thresholds at 4 kHz for the groups significantly increased with an increasing noise exposure level. The prevalence of sensorineural hearing loss among the study population is high; and noise exposure is at least contributory. Pre-employment and regular audiometry while on the job is highly recommended.

  4. Semi-implantable middle ear electromagnetic hearing device for sensorineural hearing loss.

    PubMed

    Maniglia, A J; Ko, W H; Garverick, S L; Abbass, H; Kane, M; Rosenbaum, M; Murray, G

    1997-05-01

    A semi-implantable middle ear electromagnetic hearing device (SIMEHD) is proposed for limited clinical trial in adult patients to evaluate the implantable hearing device for moderate to severe sensorineural hearing loss. Food and Drug Administration (FDA) investigational device exemption (IDE) approval has been granted (May 1996) for clinical trials. The implant unit has been evaluated acutely and chronically in animals (cats) with excellent results. Five cats undergoing chronic implantation were allowed to survive an average of 9.6 months, showing that the SIMEHD is biocompatible, functional and without untoward complications. All implant units recovered from the cats were functional, except for wire breakage of the internal antenna. A new antenna was redesigned for human implantation. The SIMEHD system consists of an external and internal unit. The external unit consists of a microphone, audio amplifier, modulator, radio frequency (RF) amplifier, antenna and battery. The internal unit is composed of a receiving antenna, hybrid electronic circuit, air core driving coil, and a target magnet cemented to the incus. All materials in contact with the body are biocompatible and expected to survive indefinitely. The implant unit is miniaturized and manufactured with existing fabrication technology by our industrial collaborator, Wilson Greatbatch, Ltd. The specific aims and major tasks of the proposed research are: a) to evaluate reliability, safety and efficacy of the SIMEHD system in a selected group of patients diagnosed with sensorineural hearing loss, due mainly to presbycusis or aging of the inner ear; and b) to obtain objective and subjective evaluation of audiologic and psychoacoustic performance as compared to the acoustic hearing aid. This paper describes the design, illustrates the actual device (newest prototype) and details the technique for surgical implantation in the attic and mastoid antrum in humans.

  5. Endothelial dysfunction in patients with sudden sensorineural hearing loss

    PubMed Central

    Berjis, Nezamoddin; Moeinimehr, Maryam; Hashemi, Seyyed Mostafa; Hashemi, Seyyed Mohammad; Bakhtiari, Elham Khosravi; Nasiri, Safoura

    2016-01-01

    Background: Endothelial dysfunction probably has a role in the etiology of sudden sensorineural hearing loss (SSNHL). The aim of this study was determining of the relationship between endothelial dysfunction and SSNHL. Materials and Methods: In a case–control study, 30 patients with SSNHL and 30 otherwise healthy age and sex-matched controls were studied. Demographic data gathered included age, gender, family history of SSNHL, and history of smoking, cardiovascular disease, hypertension, diabetes, and dyslipidemia. Laboratory data included measurement of hemoglobin, fasting blood sugar (FBS) and lipid profile. Endothelial function was assessed by measuring flow-mediated dilation (FMD). Results: The two groups were the same in age (47.9 ± 9.3 and 48.1 ± 9.6 years, P = 0.946) with female/male ratio of 1:1 in both groups. Diabetes and dyslipidemia were more frequent in patients than controls (20% vs. 0%, P = 0.024). Brachial artery diameter was greater in patients than controls before (4.24 ± 0.39 vs. 3.84 ± 0.23 mm, P < 0.001) and after ischemia (4.51 ± 0.43 vs. 4.28 ± 0.27 mm, P = 0.020), but FMD was lower in patients than controls (6.21 ± 3.0 vs. 11.52 ± 2.30%, P < 0.001). Binary logistic regression analysis showed that FMD was associated with SSNHL independent from FBS and lipid profile (odds ratio [95% confidence interval] =0.439 [0.260–0.740], P = 0.002). Conclusion: Endothelial dysfunction, among other cardiovascular risk factors, is associated with SSNHL. This association is independent from other cardiovascular risk factors including diabetes and dyslipidemia. PMID:26955626

  6. 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.

  7. 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

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

    PubMed

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

    2013-07-01

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

  9. Vertebral artery dissection: an unusual cause of transient ataxia, vertigo, and sensorineural hearing loss.

    PubMed

    Touil, Leila L; Watson, Glen James; Small, Michael

    2013-12-01

    We present the case of a 33-year-old man who was admitted with intermittent ataxia, vertigo, and sensorineural hearing loss as a result of a vertebral artery dissection following minor neck trauma. Our aim is to highlight the importance of obtaining magnetic resonance imaging, magnetic resonance angiography, and/or duplex color-flow imaging when presented with a case of fluctuating vertigo and sensorineural hearing loss with side-specific ataxia. Likewise, it is important to obtain the input of neurologists to optimize a patient's prognosis and minimize long-term sequelae.

  10. Reversible sensorineural hearing loss after non-otological surgery under general anaesthetic

    PubMed Central

    Pau, H; Selvadurai, D; Murty, G

    2000-01-01

    Acute sensorineural hearing loss can occur after both otolaryngological and non-otolaryngological procedures. The mechanism of such hearing loss remains unproved; but nitrous oxide has been implicated and where used, attendants should be aware of its potential damage to hearing. It is essential that patients with sudden hearing loss are identified as soon as possible as the recovery rate had been shown to be higher in those who presented early; and as our case demonstrates, complete recovery is possible. Anaesthetists, non-otolaryngological surgeons, and ward nurses must be aware of this early postoperative complication if appropriate treatment is to be instituted.


Keywords: surgery; sensorineural hearing loss PMID:10775288

  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. 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. PMID:26019394

  14. 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…

  15. 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…

  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. [The clinical aspects of occupational sensorineural impairment of hearing of the acoustic origin].

    PubMed

    Zinkin, V N; Sheshegov, P M; Chistov, S D

    2015-01-01

    The objective of the present study was to elucidate the specific clinical features of occupational sensorineural impairment of hearing (OSNHI) depending on the origin of this pathology, viz. noise, infrasound and/or their combination. The review of research concerning this problem made it possible to systematize variants of ODNHI based on the clinical signs taking into consideration the influence of these physical factors. Pathophysiological features of the exposure of the organs of hearing to infrasound are described. PMID:27006983

  18. 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

    2012-01-01

    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.

  19. Intratympanic steroid treatment for idiopathic sudden sensorineural hearing loss after failure of intravenous therapy.

    PubMed

    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.

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

    PubMed

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

    2016-07-01

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

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

    PubMed

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

    2016-07-01

    Idiopathic sudden sensorineural hearing loss (ISSHL) involves sudden loss of hearing from cochlear or retrocochlear origin of unknown cause. Systemic corticosteroids may be considered in the management of ISSNHL. However, an updated Cochrane systematic review concludes that "the value of steroids in the treatment of idiopathic sudden sensorineural hearing loss remains unclear since the evidence obtained from randomized controlled trials is contradictory in outcome." Therefore, a new clinical trial that addresses this question is mandatory. A first step in its design is to determine if placebo control would be ethically acceptable. We conclude that there is equipoise (uncertainty) about the use of corticosteroids for ISSHL. A new trial is justified-but with the inclusion of interim analyses to detect early imbalances on efficacy or safety and with the ability to stop the trial if needed. PMID:27371619

  3. Bilateral sudden sensorineural hearing loss caused by leptomeningeal carcinomatosis: case report and review.

    PubMed

    Öztürk, Murat; Ila, Kadri; Düzgöl, Cihan; Akansel, Gür; Almaç, Ahmet

    2014-01-01

    Leptomeningeal carcinomatosis is a rare condition characterized by diffuse infiltration of the meninges after the metastasis of the solid tumors. Bilateral sudden hearing loss is a rare initial symptom. In this article, we report a 44-year-old male patient with bilateral sudden hearing loss and dizziness. Magnetic resonance imaging showed involvement of the bilateral vestibulocochlear nerves. Malignant cells were detected in cerebrospinal fluid cytology. To the best of our knowledge, leptomeningeal carcinomatosis due to duodenum adenocarcinoma has not been reported before in the English literature. Leptomeningeal carcinomatosis should be kept in mind in patients who have bilateral sudden sensorineural hearing loss.

  4. Pleiotropy in Coffin-Lowry syndrome: sensorineural hearing deficit and premature tooth loss as early manifestations.

    PubMed

    Hartsfield, J K; Hall, B D; Grix, A W; Kousseff, B G; Salazar, J F; Haufe, S M

    1993-03-01

    We report on 7 patients (6 M, 1 F) with Coffin-Lowry syndrome who have a sensorineural hearing deficit in addition to developmental delay and characteristic facial changes. One of the patients also had a history of premature exfoliation of primary teeth. These are previously unappreciated clinical signs that may aid in the early diagnosis of Coffin-Lowry syndrome. Early diagnosis and recognition of a hearing deficit in the patient can lead to the use of hearing aids to help the patient achieve his or her full potential. These "new" clinical manifestations expand the phenotype of Coffin-Lowry syndrome and constitute an additional indication of pleiotropy.

  5. 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

  6. 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.

  7. 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

  8. Gestational vitamin A deficiency: a novel cause of sensorineural hearing loss in the developing world?

    PubMed

    Emmett, Susan D; West, Keith P

    2014-01-01

    Hearing loss is a substantial public health problem with profound social and economic consequences in the developing world. The World Health Organization (WHO) estimates that there are 360 million people living with disabling hearing loss globally, and 80% of these individuals are from low- and middle-income countries. The epidemiology of hearing impairment remains poorly defined in most impoverished societies. Middle ear infections in childhood are a key determinant; however, congenital anomalies may also comprise an important etiology and may arise from gestational malnutrition. While evidence exists that preventable vitamin A deficiency exacerbates the severity of ear infections and, consequently, hearing loss, antenatal vitamin A deficiency during sensitive periods of fetal development may represent an etiologically distinct and virtually unexplored causal pathway. Evidence from multiple animal systems clearly shows that fetal inner ear development requires adequate vitamin A nutriture to proceed normally. Inner ear malformations occur in experimentally imposed maternal vitamin A deficiency in multiple species in a dose-response manner. These anomalies are likely due to the loss of retinoic acid-dependent regulation of both hindbrain development and otic morphogenic processes. Based on in vivo evidence in experimental animals, we hypothesize that preventable gestational vitamin A deficiency, especially during early stages of fetal development, may predispose offspring to inner ear malformations and sensorineural hearing loss. As vitamin A deficiency affects an estimated 20 million pregnant women globally, we hypothesize that, in undernourished settings, routine provision of supplemental vitamin A at the recommended allowance throughout pregnancy may promote normal inner ear development and reduce risk of an as yet unknown fraction of sensorineural hearing loss. If our hypothesis proves correct, gestational vitamin A deficiency would represent a potentially

  9. Bilateral sudden sensorineural hearing loss caused by alcohol abuse and heroin sniffing.

    PubMed

    Antonopoulos, Stavros; Balatsouras, Dimitrios G; Kanakaki, Sofia; Dona, Athina; Spiliopoulou, Christina; Giannoulis, Grigoris

    2012-06-01

    Objective. Acute or chronic heroin abuse has been associated with various central neurologic pathologies and, occasionally, with peripheral nervous system damage. The effect of heroin on hearing has not been adequately documented, although several cases with sudden hearing loss owed to heroin abuse have been reported. We present a young male with bilateral sudden sensorineural hearing loss, following heroin sniffing and alcohol consumption. Methods. Our patient underwent a detailed clinical and audiological evaluation, including auditory brainstem responses and otoacoustic emission. Routine laboratory blood tests and imaging studies were performed. Results. The patient was treated with corticosteroids and magnesium, resulting in complete restoration of hearing after one month. Conclusion. Sudden hearing loss owed to heroin abuse is usually curable, following adequate treatment.

  10. 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).

  11. Sudden sensorineural hearing loss after varicose vein surgery under general anesthesia. Case report.

    PubMed

    Galanopoulos, G; Rapti, D; Nikolopoulos, I; Lambidis, C

    2011-01-01

    Sudden sensorineural hearing loss following non-otologic surgery, under general anesthesia, is an extremely rare complication. Furthermore, postoperative deafness has more commonly been associated with cardiac surgery. This acute dysfunction of the inner ear consists a controversial subject in terms of etiopathogenic mechanisms and treatment modalities. We present the case of a 60-year-old male patient who underwent varicose vein surgery under general anesthesia and presented immediately after the operation bilateral and profound impairment of his hearing acuity. A high index of suspicion is required to early diagnosis of this complication, although prompt treatment does not guarantee a good outcome.

  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. Superficial siderosis and sudden sensorineural hearing loss: a case report and review of the literature.

    PubMed

    Lee, Kirsti S V; Sritharan, Niranjan; Forrest, Allan

    2013-01-01

    This case report highlights an unusual case of sudden sensorineural hearing loss related to superficial siderosis (SS). Our patient had a craniotomy for medulloblastoma 23 years earlier, and this may represent a delayed complication related to this procedure. Magnetic resonance imaging (MRI) remains the key diagnostic investigation to illustrate the imaging features of superficial siderosis and exclude other pathologies. Increased awareness of progressive and sudden hearing complications caused by SS is important in the otolaryngologic community to expedite management and better counsel patients during the consent process.

  14. [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.

  15. Management of Children with Severe, Severe-profound, and Profound Sensorineural Hearing Loss.

    PubMed

    Iseli, Claire; Buchman, Craig A

    2015-12-01

    Management of children with severe, severe-profound, and profound sensorineural hearing loss is best achieved using a family-centered approach by a team of health professionals, including audiologists, speech pathologists, otolaryngologists, pediatricians, genetic counselors, and early intervention programs. Early diagnosis and intervention offers the best chance for speech and language acquisition. Although hearing aids can provide some of the needed information, they are often not sufficient for spoken language development and a cochlear implant is needed. This must be combined with a strong audiology and speech therapy rehabilitation program.

  16. 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.

  17. 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.

  18. 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

  19. Esteem® middle ear device versus conventional hearing aids for rehabilitation of bilateral sensorineural hearing loss.

    PubMed

    Monini, Simonetta; Biagini, Michela; Atturo, Francesca; Barbara, Maurizio

    2013-07-01

    This retrospective study aimed to evaluate the efficacy of the Esteem(®) middle ear implant in sensorineural hearing loss (SNHL) of different degree as well as to compare it with that obtained with conventional hearing aids. Fifteen out of 30 adults patients who received an Esteem(®) middle ear device for rehabilitation of sensorineural hearing loss met the primary eligibility criterion of prior, continuous use of conventional hearing aids. Study population included moderate-to-severe SNHL (8 patients) and severe-to-profound SNHL (7 patients). Audiometric measurements included free-field pure-tone and speech audiometry in Esteem(®)-aided, HA-aided, and baseline threshold. For speech audiometry, speech reception threshold (SRT) and word recognition score (WRS) were assessed. Subjective benefit was evaluated by Client Oriented Scale of Improvement (COSI) questionnaire. In all the subjects, SRT and WRS showed improvement both with conventional HA and Esteem(®) in respect to the unaided situation. Although not statistically significant, a slight prevalence of the Esteem(®) performances was recorded both audiometrically and as subjective satisfaction score. The Esteem(®) middle ear device demonstrated appreciable benefit for rehabilitation of SNHL of different degree, comparable to what can be achieved by conventional hearing aids. In addition, this rehabilitative process may enable also individuals presenting with severe-to-profound SNHL to achieve remarkable functional outcomes.

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

    PubMed Central

    Ashraf, Omer

    2005-01-01

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

  1. 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.

  2. 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…

  3. [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. PMID:27213653

  4. 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. PMID:25459416

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

    PubMed

    Mirvakili, Abbas; Dadgarnia, Mohammad Hossein; Baradaranfar, Mohammad Hossein; Atighechi, Saeid; Zand, Vahid; Ansari, Abdollah

    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.

  7. 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

  8. [Intrauterine infections as a risk factor of the development of sensorineural impairment of hearing ].

    PubMed

    Boboshko, M Yu; Vikhnin, S M; Savenko, I V

    2016-01-01

    Intrauterine infections are a crucial pathogenic factor exerting an appreciable influence on the development of the fetus. They can provoke intrauterine death, cause multiple lesions in the organs and tissues as well as long-term complications that manifest themselves at the later stages of the growth and development of the child. One of such complications is the sensorineural loss of hearing. The importance of hearing impairment arises from the high prevalence of tis condition and frequent incapacitation it causes in the patients. The present publication is focused on various mechanisms underlying the development of hearing impairment depending on the primary infection. Special attention is given to the methods of diagnostics and treatment of intrauterine infections. PMID:27351041

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

    PubMed

    Sandgren, Olof; Hansson, Kristina; Sahlén, Birgitta

    2015-01-01

    Whereas the language development of children with sensorineural hearing impairment (SNHI) has repeatedly been shown to differ from that of peers with normal hearing (NH), few studies have used an experimental approach to investigate the consequences on everyday communicative interaction. This mini review gives an overview of a range of studies on children with SNHI and NH exploring intra- and inter-individual cognitive and linguistic systems during communication. Over the last decade, our research group has studied the conversational strategies of Swedish speaking children and adolescents with SNHI and NH using referential communication, an experimental analog to problem-solving in the classroom. We have established verbal and non-verbal control and validation mechanisms, related to working memory capacity and phonological short term memory. We present main findings and future directions relevant for the field of cognitive hearing science and for the clinical and school-based management of children and adolescents with SNHI. PMID:25806012

  10. [The specific features of the vestibular function in the patients presenting with sensorineural hearing loss of vascular genesis].

    PubMed

    Kirichenko, I M; Popadyuk, V I; Tuzhilina, K V

    2016-01-01

    The authors consider the specific features of the vestibular function in the patients with sensorineural hearing loss of vascular genesis. The study included 60 patients at the age from 28 to 75 years presenting with sensorineural impairment of hearing of vascular genesis. All of them were examined with the use of the extended otoneurological method. The data obtained were compared with the structural changes and hemodynamic characteristics of vertebral arteries (VA) and internal carotid arteries (ICA) and with the results of magnetic resonance imaging (MRI) of the brain.

  11. 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

  12. 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

  13. Intratympanic steroid injection for sudden sensorineural hearing loss in a patient on hemodialysis.

    PubMed

    Wu, Rui-Xin; Chen, Chun-Chi; Wang, Chih-Hung; Chen, Hsin-Chien

    2014-01-01

    Sudden sensorineural hearing loss (SSNHL) is being described with increasing incidence among patients with end-stage renal disease (ESRD) undergoing hemodialysis (HD). There are no widely accepted guidelines in the medical literature for the appropriate management of this medical emergency. Administration of systemic steroids remains the mainstay of the management of SSNHL in conjunction with the supportive treatment, in this vulnerable group of patients, as well. However, encouraged by the evolving evidence on the efficacy of the intratympanic steroid injections (ITSI) in the treatment of SSNHL among patients without renal disease--we successfully treated SSNHL in an elderly diabetic with sepsis due to bilateral pneumonitis undergoing regular HD treatment with multiple ITSI and antibiotics resulting in complete recovery of hearing function within 3 months of onset of the first symptoms.

  14. Low Level Laser Effect in Treatment of Patients with Intractable Tinnitus Due To Sensorineural Hearing Loss

    PubMed Central

    Mirvakili, Abbas; Mehrparvar, Amirhoushang; Mostaghaci, Mehrdad; Mollasadeghi, Abolfazl; Mirvakili, Masud; Baradaranfar, Mohammadhosein; Davari, Mohammadhosein

    2014-01-01

    Introduction: Tinnitus is defined as a perception of sound without an external acoustic stimulus. Due to large number of causes and limited knowledge of its pathophysiology, tinnitus still remains an obscure symptom. Methods: This was a cross-sectional study on 120 patients with tinnitus and sensorineural hearing loss who were randomly divided into two groups; one group received low-level laser and the second group used the same instrument but off, for 20 sessions of 20 minutes. A tinnitus handicap inventory (THI) and Visual Analog Scale (VAS) were used to evaluate the severity of patients’ symptoms. Severity and frequency of tinnitus were also determined using Audiometric tests. Results: The average age of the 120 patients in the two groups of study were not statistically significantly different. The mean difference of severity of tinnitus between the two groups was statistically significant at the end of the study and 3 month after completion of treatment. The VAS and THI mean differences after the treatment were statistically significant between the two groups but not statistically significant after 3 months of completion the study. Conclusion: Low level laser radiation is effective for short-term treatment of Tinnitus caused by sensorineural hearing loss and its impact may be reduced over the time. PMID:25653802

  15. Secondary hyperbaric oxygen therapy for idiopathic sudden sensorineural hearing loss in the subacute and chronic phases.

    PubMed

    Ohno, Kazuchika; Noguchi, Yoshihiro; Kawashima, Yoshiyuki; Yagishita, Kazuyoshi; Kitamura, Ken

    2010-06-01

    This study investigated the efficacy of hyperbaric oxygen therapy (HBOT) as a secondary treatment for patients with idiopathic sudden sensorineural hearing loss (ISSNHL) in the subacute and chronic phases. Forty-eight ISSNHL patients (HBOT group) who had received primary conventional treatment within 4 weeks after onset and underwent HBOT between 4 and 20 weeks post-onset were retrospectively compared with 44 ISSNHL patients (control group) with primary conventional treatment alone. Mean hearing gain was slight, with gains of 5.2 +/- 8.9 dB in the HBOT group and 2.0 +/- 7.6 dB in the control group. However, no significant difference was recognized between the two groups. In the HBOT group, no significant difference was observed in hearing gain among patients with HBOT initial time at 4-7, 8-11, 12-15 or 16-20 weeks after onset. Meanwhile, hearing gain was significantly higher in patients with profound hearing loss than in the other patients. We conclude that the effectiveness of secondary HBOT for ISSNHL patients in either subacute or chronic phase remains unproven, and thus, the decision administer HBOT should be made with caution.

  16. The Effects of Sensorineural Hearing Impairment on Asynchronous Glimpsing of Speech

    PubMed Central

    Buss, Emily; Hall, Joseph W.

    2016-01-01

    In a previous study with normal-hearing listeners, we evaluated consonant identification masked by two or more spectrally contiguous bands of noise, with asynchronous square-wave modulation applied to neighboring bands. Speech recognition thresholds were 5.1–8.5 dB better when neighboring bands were presented to different ears (dichotic) than when all bands were presented to one ear (monaural), depending on the spectral width of the frequency bands. This dichotic advantage was interpreted as reflecting masking release from peripheral spread of masking from neighboring frequency bands. The present study evaluated this effect in listeners with sensorineural hearing loss, a population more susceptible to spread of masking. Speech perception (vowel-consonant-vowel stimuli, as in /aBa/) was measured in the presence of fluctuating noise that was either modulated synchronously across frequency or asynchronously. Hearing-impaired listeners (n = 9) and normal-hearing controls were tested at either the same intensity (n = 7) or same sensation level (n = 8). Hearing-impaired listeners had mild-to-moderate hearing loss and symmetrical, flat audiometric thresholds. While all groups of listeners performed better in the dichotic than monaural condition, this effect was smaller for the hearing-impaired (3.5 dB) and equivalent-sensation-level controls (3.3 dB) than controls tested at the same intensity (11.0 dB). The present study is consistent with the idea that dichotic presentation can improve speech-in-noise listening for hearing-impaired listeners, and may be enhanced when combined with amplification. PMID:27144601

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

    PubMed

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

    1989-09-01

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

  18. Uni- and multivariate models for investigating potential prognostic factors in idiopathic sudden sensorineural hearing loss.

    PubMed

    Lionello, Marco; Staffieri, Claudia; Breda, Stefano; Turato, Chiara; Giacomelli, Luciano; Magnavita, Paola; de Filippis, Cosimo; Staffieri, Alberto; Marioni, Gino

    2015-08-01

    With a worldwide incidence estimated at 8-15 per 100,000 population a year, idiopathic sudden sensorineural hearing loss (ISSHL) is a common clinical finding for otologists. There is a shortage of information on the clinical factors capable of predicting hearing recovery and response to therapy. The aim of the present study was to retrospectively investigate the prognostic value of clinical variables in relation to hearing recovery, in a cohort of 117 consecutive patients with ISSHL. Clinical parameters (signs, symptoms, comorbidities and treatments) and audiometric data were analyzed with univariate and multivariate statistical approaches for prognostic purposes to identify any correlation with hearing recovery, also expressed according to the Wilson criteria. Univariate analysis showed that age and hypertension were significantly related to hearing outcome (p = 0.004 and p = 0.015, respectively). Elderly patients and those with hypertension were at higher risk of experiencing no hearing recovery (OR = 3.25 and OR = 2.89, respectively). Age was an independent prognostic factor on multivariate analysis (p = 0.007). Tinnitus as a presenting symptom showed a trend towards an association with hearing recovery (p = 0.07). The treatment regimen, the time elapsing between the onset of symptoms and the start of therapy (p = 0.34), and the duration of the treatment (p = 0.83) were unrelated to recovery on univariate analysis. Among the parameters considered, only age was significantly and independently related to hearing outcome. There is a need for well-designed, randomized clinical trials to enable an evidence-based protocol to be developed for the treatment of ISSHL.

  19. 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

  20. Total implantation of the Implex TICA hearing amplifier implant for high frequency sensorineural hearing loss: the Tübingen University experience.

    PubMed

    Zenner, H P; Leysieffer, H

    2001-04-01

    Hearing devices may be classified as sound-producing hearing aids, electrically stimulating devices, and vibratory hearing aids. Because patients may lose physiologic cochlear amplification, hearing devices for the treatment of sensorineural hearing loss are used as signal amplifiers. The totally implantable communication assistance (TICA) device is a European-approved totally implantable vibratory amplifier implant. It picks up the sound signal transcutaneously from the external auditory canal near the eardrum, amplifies the signal, and transduces the signal into microvibrations that are delivered to the ossicular chain.

  1. 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…

  2. Prevalence and risk factors for sensorineural hearing loss: Western Sicily overview.

    PubMed

    Salvago, Pietro; Martines, Enrico; Martines, Francesco

    2013-11-01

    The objective of this work was to evaluate the prevalence of sensorineural hearing loss (SNHL) and distribution of the main risk factors associated to it focusing on their role in the development of deafness and their interaction. We performed a global audiological assessment (through TEOAE, tympanometry and ABR) in 508 infants at risk studying the main risk factors reported by Joint Committee on Infant Hearing (2007). Fifty-one infants (10.03 %) were diagnosed with SNHL (45 bilateral and 6 unilateral) with a mean hearing threshold of 87.39 ± 28.25 dB HL; family history of hearing impairment (HI) and TORCH infections indicated independent significant risk factors (P < 0.00001 and P = 0.024, respectively). High SNHL percentages were evidenced also in NICU babies, due to the various pathologies and risk factors presented by these infants, and among newborns who suffered from hyperbilirubinemia (11.97 and 9.52 %, respectively). The mean degree of hearing loss for children with family history of HI (>100 dB HL) emphasizes the necessity of an early diagnosis to avoid the consequences of auditory deprivation. Craniofacial abnormalities and syndromes associated to HI showed an important relationship (P < 0.00001) with conductive hearing loss. A progressive increase was evidenced in SNHL incidence as the number of risk factors rises (from 5.12 for 2 risk factors to 28.5 % for 5 or more) with a significant difference among the groups (P = 0.049); multiple risk factors showed an additional cofactor for HL (r (2) = 0.93). Considering the high SNHL prevalence (10.03 %) in infants at risk, this study highlights the necessity to implement a neonatal hearing screening program in Western Sicily.

  3. 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.

  4. 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

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

    PubMed Central

    Hosseinabadi, Reza; Jafarzadeh, Sadegh

    2014-01-01

    Background: The Auditory steady state response (ASSR) provides a frequency-specific and automatic assessment of hearing sensitivity and is used in infants and difficult-to-test adults. Objectives: The aim of this study was to compare the ASSR thresholds among various types (normal, conductive, and sensorineural), degree (normal, mild, and moderate), and configuration (flat and sloping) of hearing sensitivity, and measuring the cutoff point between normal condition and hearing loss for different frequencies. Patients and Methods: This clinical trial was performed in Iran and included patients who were referred from Ear, Nose, and Throat Department. A total of 54 adults (27 with sensorineural hearing loss, 17 with conductive hearing losses, and 10 with normal hearing) were randomly chosen to participate in our study. The type and degree of hearing loss were determined through testing by otoscopy, tympanometry, acoustic reflex, and pure tone audiometry. Then the ASSR was tested at carrier frequencies of 500, 1000, 2000, and 4000 Hz. Results: The ASSR accurately estimates the behavioral thresholds as well as flat and sloping configurations. There was no correlation between types of hearing loss and difference of behavioral and ASSR thresholds (P = 0.69). The difference between ASSR and behavioral thresholds decreased as severity of hearing loss increased. The 40, 35, 30, and 35 dB could be considered as cutoffs between normal hearing and hearing loss for 500, 1000, 2000, and 4000 Hz, respectively. Conclusions: The ASSR can accurately predict the degree and configuration of hearing loss and discriminate the normal hearing from mild or moderate hearing loss and mild from moderate hearing loss, except for 500 Hz. The Air-conducted ASSR could not define the type of hearing loss. PMID:25763263

  6. 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.

  7. Prognostic impact of standard laboratory values on outcome in patients with sudden sensorineural hearing loss

    PubMed Central

    2014-01-01

    Background Aim of the present study was to evaluate prognostic factors, in particular standard laboratory parameters, for better outcome after idiopathic sudden sensorineural hearing loss (SSNHL). Methods Using a retrospective review, 173 patients were included presenting between 2006 and 2009 with unilateral SSNHL, ≥30 dB bone conduction in three succeeding frequencies between 0.125 to 8 kHz in pure tone audiometry (PTA), and a time interval between first symptoms and diagnostics ≤ 4 weeks. Hearing gain of <10 dB versus ≥10 dB in the affected ear in 6PTA values was the primary outcome criterion. Univariate and multivariate statistical tests were used to analyze predictors for better outcome. Results The initial hearing loss was 50.6 ± 27.2 dB. The absolute hearing gain was 15.6 ± 20.1 dB. Eighty-one patients (47%) had a final hearing gain of ≥10 dB. Low-frequency hearing loss (p <0.0001); start of inpatient treatment <4 days after onset (p = 0.018); first SSNHL (versus recurrent SSNHL, p = 0.001); initial hearing loss ≥ 60 dB (p < 0.0001); an initial quick value lower than the reference values (p = 0.040); and a pretherapeutic hyperfibrinogenemia (p = 0.007) were significantly correlated to better outcome (≥10 dB absolute hearing gain). Multivariate analysis revealed that first SSNHL (p = 0.004), start of treatment <4 days after onset (p = 0.015), initial hearing loss ≥ 60 dB (p = 0.001), and hyperfibrinogenemia (p = 0.032) were independent prognostic factors for better hearing recovery. Conclusion Better hearing gain in patients with hyperfibrinogenemia might be explained by the rheological properties of the applied therapy and supports the hypothesis that SSNHL is caused in part by vascular factors. PMID:25028570

  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. PMID:27392187

  9. [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.

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

    PubMed Central

    de Andrade, Kelly Cristina Lira; de Lemos Menezes, Pedro; Carnaúba, Aline Tenório Lins; de Sousa Rodrigues, Renato Glauco; de Carvalho Leal, Mariana; Pereira, Liliane Desgualdo

    2013-01-01

    OBJECTIVE: The audibility thresholds for the sound frequency of 137 upward- and downward-sloping audiograms showing sensorineural hearing loss were selected and analyzed in conjunction with speech recognition thresholds obtained from individuals seen at a public otolaryngology clinic to determine which frequencies in slope audiograms best represent speech recognition thresholds. METHOD: The linear regression model and mean square error were used to determine the associations between the threshold values. RESULT: The mean square error identified larger errors when using thresholds of 500, 1000, and 2000 Hz than when using audibility thresholds of 500, 1000, 2000, and 4000 Hz. The linear regression model showed a higher correlation (91%) between the audiogram thresholds for frequencies of 500, 1000, 2000, and 4000 Hz than for the frequencies of 500, 1000, and 2000 Hz (88%). CONCLUSION: Frequencies of 500, 1000, 2000, and 4000 Hz were the most significant in predicting the speech recognition threshold. PMID:23778471

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

    PubMed

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

    2015-09-01

    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.

  12. 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

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

    PubMed Central

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

    2014-01-01

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

  14. 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.

  15. Objective measures of perceptual quality for predicting speech intelligibility in sensorineural hearing loss.

    PubMed

    Chiaramello, E; Moriconi, S; Tognola, G

    2015-08-01

    An objective method to predict speech intelligibility in sensorineural hearing loss of different types and increasing degrees of severity is proposed and validated with experimental data. The novel approach is based on the combined use of acoustic simulations of impaired perception and objective measures of perceptual speech quality (PESQ). Acoustic simulations were obtained after degradation of the original, non distorted, speech waveforms by spectral smearing, expansive nonlinearity, and level scaling. PESQ was used to measure perceptual quality of the acoustic simulations obtained by varying the degree of the simulated hearing loss. A logistic function was applied to transform PESQ scores into predicted intelligibility scores. A set of CV and VC syllables in /a/, /u/, and /i/ contexts was used as reference test material. The method was validated with subjective measures of intelligibility of the degraded speech obtained in a group of 10 normal hearing subjects. Overall, prediction of experimental speech intelligibility through the transformed PESQ measures was good (R(2)=0.7; RMSE=0.08) revealing that the proposed approach could be a valuable aid in real clinical applications. PMID:26737556

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

    PubMed Central

    Okano, Takayuki

    2014-01-01

    Sensorineural hearing loss (SNHL) is a common clinical condition resulting from dysfunction in one or more parts in the auditory pathway between the inner ear and auditory cortex. Despite the prevalence of SNHL, little is known about its etiopathology, although several mechanisms have been postulated including ischemia, viral infection or reactivation, and microtrauma. Immune-mediated inner ear disease has been introduced and accepted as one SNHL pathophysiology; it responds to immunosuppressive therapy and is one of the few reversible forms of bilateral SNHL. The concept of immune-mediated inner ear disease is straightforward and comprehensible, but criteria for clinical diagnosis and the precise mechanism of hearing loss have not been determined. Moreover, the therapeutic mechanisms of corticosteroids are unclear, leading to several misconceptions by both clinicians and investigators concerning corticosteroid therapy. This review addresses our current understanding of the immune system in the inner ear and its involvement in the pathophysiology in SNHL. Treatment of SNHL, including immune-mediated inner ear disorder, will be discussed with a focus on the immune mechanism and immunocompetent cells as therapeutic targets. Finally, possible interventions modulating the immune system in the inner ear to repair the tissue organization and improve hearing in patients with SNHL will be discussed. Tissue macrophages in the inner ear appear to be a potential target for modulating the immune response in the inner ear in the pathophysiology of SNHL. PMID:25228882

  17. 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

  18. 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

  19. 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.

  20. 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

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

    PubMed

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

    2010-02-01

    The auditory system is capable of perceptually restoring inaudible portions of speech. This restoration may be compromised as a result of hearing impairment, particularly if it is combined with advanced age, because of degradations in the bottom-up and top-down processes. To test this hypothesis, phonemic restoration was quantitatively measured with hearing-impaired listeners of varying ages and degrees of hearing impairment, as well as with a normal hearing control group. The results showed that the restoration benefit was negatively correlated with both hearing impairment and age, supporting the original hypothesis. Group data showed that listeners with mild hearing loss were able to perceptually restore the missing speech segments as well as listeners with normal hearing. By contrast, the moderately-impaired listeners showed no evidence of perceptual restoration. Further analysis using the articulation index showed that listeners with mild hearing loss were able to increase phonemic restoration with audibility. Moderately-impaired listeners, on the other hand, were unable to do so, even when the articulation index was high. The overall findings suggest that, in addition to insufficient audibility, degradations in the bottom-up and/or top-down mechanisms as a result of hearing loss may limit or entirely prevent phonemic restoration. PMID:19922784

  2. The noise exposed factory workers: the prevalence of sensori-neural hearing loss and their use of personal hearing protection devices.

    PubMed

    Maisarah, S Z; Said, H

    1993-09-01

    A total of 524 industrial workers were studied. They consisted of 442 noise exposed and 82 non-noise exposed workers. The purpose was to compare the prevalence of sensori-neural hearing loss among the noise exposed and the non-noise exposed workers, to study their knowledge on the hazard of noise to hearing and the workers' attitude towards the hearing protection devices. The prevalence of sensori-neural hearing loss was significantly higher among the noise exposed workers, i.e., 83% versus 31.7% (p < 0.01). However, the prevalence of hearing impairment was much lower for both groups, being 30.1% for the noise exposed and 3.7% for the non-noise exposed group. Although hearing protection devices were provided to 80.5% of the workers, only 5.1% were wearing them regularly. The possibility of developing hearing loss due to exposure to excessive noise was only known by 35.5% of the noise exposed workers. This awareness was found to have a positive correlation with the workers' compliance to the hearing protection devices. Our findings highlight the need for workers to be educated on the hazards of excessive noise exposure to hearing.

  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. Intratympanic steroids as a salvage treatment for sudden sensorineural hearing loss? A meta-analysis.

    PubMed

    Ng, Jia Hui; Ho, Roger Chun Man; Cheong, Crystal Shuk Jin; Ng, Adele; Yuen, Heng Wai; Ngo, Raymond Yeow Seng

    2015-10-01

    Sudden sensorineural hearing loss is typically treated with systemic steroids. The aim of this meta-analysis was to evaluate the efficacy of salvage intratympanic steroid treatment in patients who have initial treatment failure with systemic steroids. A MEDLINE literature search was performed, supported by searches of Web of Science, Biosis, and Science Direct. Articles of all languages were included. Selection of relevant publications was conducted independently by three authors. Only randomized controlled trials were considered. In one arm of the studies, the patients received salvage intratympanic steroids. In the other arm, patients did not receive further treatment. The standard difference in mean (SDM) amount of improvement in hearing threshold between patients who did and did not receive salvage intratympanic steroids was calculated. From an initial 184 studies found via the search strategy, 5 studies met inclusion criteria and were included. There was a statistically significant greater reduction in hearing threshold on pure-tone audiometry in patients who received salvage intratympanic steroids than in those who did not (SDM = -0.401, p = 0.005). Subgroup analysis showed that administration by intratympanic injection (SDM = -0.375, p = 0.013) rather than a round window catheter (SDM = -0.629, p = 0.160) yielded significant improvement in outcome. The usage of dexamethasone yielded better outcomes (SDM = -0.379, p = 0.039) than the use of methylprednisolone (SDM = -0.459, p = 0.187). No serious side effect of treatment was reported. In patients who have failed initial treatment with systemic steroids, additional treatment with salvage intratympanic dexamethasone injections demonstrate a statistically significant reduction in the hearing thresholds as compared to controls.

  5. 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

  6. Sleep Disturbance and Altered Expression of Circadian Clock Genes in Patients With Sudden Sensorineural Hearing Loss.

    PubMed

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

    2015-07-01

    The cause of sudden sensorineural hearing loss (SSNHL) remains unclear and therefore it is often considered as idiopathic. Sleep disturbance has been linked to SSNHL and circadian rhythm disruption, but the link between circadian rhythm disruption and SSNHL has never been investigated.In this study, we surveyed the sleep quality of 38 patients with SSNHL using a simple insomnia sleep questionnaire. The expression of circadian clock genes in peripheral blood (PB) leukocytes from 38 patients with SSNHL and 71 healthy subjects was accessed using real-time quantitative reverse transcriptase-polymerase chain reaction and validated using immunocytochemical staining.We found that 61.8% of patients with SSNHL suffered from insomnia before the insult of hearing loss. Besides, significantly decreased expression of PER1, CRY1, CRY2, CLOCK, BMAL1, and CKlε was found in PB leukocytes of patients with SSNHL when compared with healthy subjects. SSNHL patients with vertigo had significantly lower expression of CRY1 and CKlε than patients without vertigo symptoms. Our results imply the association of sleep disturbance and disrupted circadian rhythm in SSNHL.

  7. 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.

  8. Pharmacological influence on inner ear endothelial cells in relation to the pathogenesis of sensorineural hearing loss.

    PubMed

    Gloddek, B; Lamm, K; Arnold, W

    2002-01-01

    Despite an increasing incidence of acute sensorineural hearing loss, the pathogenesis of this disease remains uncertain. While viral infection of the stria vascularis, organ of Corti or spiral ganglion cells is discussed in the American literature, a vascular genesis with resulting impaired perfusion of the inner ear is favoured by European investigators. Although both hypotheses are supported by different therapeutic strategies to regain normal hearing, the influence of spontaneous remission remains unclear. This study aims at combining these seemingly opposing concepts with the assumption of an immunologically mediated vasculitis with consequent cochlear hypoperfusion. We already know from other organs that during viral vasculitis circulating immunoglobulins are deposited perivascularly, which leads to a local decrease in perfusion and tissue hypoxia. Also in autoimmune diseases, perivasculitis is common with the endothelium playing a major role at the initial stages of the disease. These endothelial cells promote vasculitis by secreting pro-inflammatory cytokines like IL-1, IL-6 or TNF-alpha in addition to the expression of adhesion molecules. Due to the persistence of these immunopathological mechanisms stenosis or atresia with ischaemic necrosis results. To examine whether this pathomechanism is also important in inner ear dysfunction, the immunological response after stimulation of the cochlear endothelium of guinea pigs was determined. In addition, the influence of corticosteroids on this immune cascade was examined.

  9. [A national database could solve the issue of sudden sensorineural hearing loss].

    PubMed

    Hultcrantz, Elisabeth; Nosrati-Zarenoe, Ramesh; Arlinger, Stig

    2003-09-25

    A national database is being organised for the disease Sudden Sensorineural Hearing-loss, SSH (often called "Sudden deafness"). The cause of SSH is unknown. Many theories have given rise to many treatment regimens: the vascular theory, assuming a reduced blood flow to the inner ear, the infection theory, assuming bacteria or virus being the etiological agent, and the auto-immune theory, where also blood vessels may be involved. Still another theory concerns ruptured membranes, either towards the middle ear, leaking perilymph, or intra-cochlear ruptures, resulting in perilymph and endolymph mixing and giving rise to the hearing loss. Many cases recover spontaneously: up to one third completely and another one third significantly improved. The most common therapy today is high-dose corticosteroids. Collecting data on a large number of patients in a database will allow the assessment of different background factors and the possible effects of therapy. We hope to obtain data on 400 patients per year during a 4-year period in this multi-centre project. A controlled study is planned to assess the effect of cortico-steroid treatment.

  10. Repetitive transcranial magnetic stimulation improves both hearing function and tinnitus perception in sudden sensorineural hearing loss patients

    PubMed Central

    Zhang, Dai; Ma, Yuewen

    2015-01-01

    The occurrence of sudden sensorineural hearing loss (SSHL) affects not only cochlear activity but also neural activity in the central auditory system. Repetitive transcranial magnetic stimulation (rTMS) above the auditory cortex has been reported to improve auditory processing and to reduce the perception of tinnitus, which results from network dysfunction involving both auditory and non-auditory brain regions. SSHL patients who were refractory to standard corticosteroid therapy (SCT) and hyperbaric oxygen (HBO) therapy received 20 sessions of 1 Hz rTMS to the temporoparietal junction ipsilateral to the symptomatic ear (rTMS group). RTMS therapy administered in addition to SCT and HBO therapy resulted in significantly greater recovery of hearing function and improvement of tinnitus perception compared SCT and HBO therapy without rTMS therapy. Additionally, the single photon emission computed tomography (SPECT) measurements obtained in a subgroup of patients suggested that the rTMS therapy could have alleviated the decrease in regional cerebral brain flow (rCBF) in SSHL patients. RTMS appears to be an effective, practical, and safe treatment strategy for SSHL. PMID:26463446

  11. 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.

  12. Factors influencing the outcome of idiopathic sudden sensorineural hearing loss treated with hyperbaric oxygen therapy.

    PubMed

    Körpinar, Sefika; Alkan, Zeynep; Yiğit, Ozgür; Gör, Ayşe Pelin; Toklu, Akin Savaş; Cakir, Burak; Soyuyüce, Ozlem Gedik; Ozkul, Haluk

    2011-01-01

    Idiopathic sudden sensorineural hearing loss (ISSNHL) is an otologic emergency with an incidence of about 5-20 per 100,000 of the population per year. There is no universally accepted standard protocol for the treatment of patients with ISSNHL. Hyperbaric oxygen therapy (HBOT), was first reported to improve the outcome following acute inner ear disorders during the late 1960s by both French and German authors. The increase in perilymph oxygenation produced by HBOT provides logical basis for the use of this treatment modality in ISSNHL. We reviewed the records of 97 cases that received HBOT for SSNHL to identify the factors that may affect the treatment outcomes. The effects of age, gender, affected ear, status of the contralateral ear, symptoms associated with hearing loss, presence of a cardiovascular disease, dyslipidemia, history of diabetes mellitus, seasonal factor, smoking, degree of hearing loss, audiogram type, medical treatments provided prior to HBOT, onset time, and number of HBOT sessions were evaluated. The mean hearing gain in all cases after the HBOT was 29.5 dB. The gains were statistically significant in the following cases: early onset of HBOT (p = 0.016), higher number of HBOT sessions (p < 0.01), steroid usage (p = 0.009), low frequency-ascending and total audiogram configuration (p < 0.01) and profound hearing loss (p = 0.011). The success rate was significantly lower in cases with high frequency-descending audiogram configuration (p < 0.001). The most important factor affected the prognosis favorably was found as steroid therapy. This retrospective study and our clinical experience suggest that HBOT has beneficial effects when administered in the early phase of the disease together with steroids. HBOT is a safe practice when used properly by an experienced hyperbaric team. In the treatment of ISSNHL, 20 sessions of HBOT at 2.5 ATA can be tolerated well besides some minor side effects. HBOT should be considered for the cases

  13. 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.

  14. 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.

  15. High-frequency sensorineural hearing loss and its underlying genetics (Hfhl1 and Hfhl2) in NIH Swiss mice.

    PubMed

    Keller, James M; Neely, Harold R; Latoche, Joseph R; Noben-Trauth, Konrad

    2011-10-01

    Studies using inbred strains of mice have been invaluable for identifying alleles that adversely affect hearing. However, the efficacy of those studies is limited by the phenotypes that these strains express and the alleles that they segregate. Here, by selectively breeding phenotypically and genetically heterogeneous NIH Swiss mice, we generated two lines-the all-frequency hearing loss (AFHL) line and the high-frequency hearing loss (HFHL) line-with differential hearing loss. The AFHL line exhibited characteristics typical of severe, early-onset, sensorineural hearing impairment. In contrast, the HFHL line expressed a novel early-onset, mildly progressive, and frequency-specific sensorineural hearing loss. By quantitative trait loci (QTLs) analyses in these two lines, we identified QTLs on chromosomes 7, 8, and 10 that significantly affected hearing function. The loci on chromosomes 7 and 8 (Hfhl1 and Hfhl2, respectively) are novel and appear to adversely affect only high frequencies (≥30 kHz). Mice homozygous for NIH Swiss alleles at either Hfhl1 or Hfhl2 have 32-kHz auditory-evoked brain stem response thresholds that are 8-14 dB SPL higher than the corresponding heterozygotes. DNA sequence analyses suggest that both the Cdh23(ahl) and Gipc3(ahl5) variants contribute to the chromosome 10 QTL detected in the AFHL line. The frequency-specific hearing loss indicates that the Hfhl1 and Hfhl2 alleles may affect tonotopic development. In addition, dissecting the underlying complex genetics of high-frequency hearing loss may prove relevant in identifying less severe and common forms of hearing impairment in the human population.

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

    PubMed Central

    Fereczkowski, Michal; Zaar, Johannes; Santurette, Sébastien; Dau, Torsten

    2016-01-01

    Physiological studies have shown that noise-induced sensorineural hearing loss (SNHL) enhances the amplitude of envelope coding in auditory-nerve fibers. As pitch coding of unresolved complex tones is assumed to rely on temporal envelope coding mechanisms, this study investigated pitch-discrimination performance in listeners with SNHL. Pitch-discrimination thresholds were obtained for 14 normal-hearing (NH) and 10 hearing-impaired (HI) listeners for sine-phase (SP) and random-phase (RP) complex tones. When all harmonics were unresolved, the HI listeners performed, on average, worse than NH listeners in the RP condition but similarly to NH listeners in the SP condition. The increase in pitch-discrimination performance for the SP relative to the RP condition (F0DL ratio) was significantly larger in the HI as compared with the NH listeners. Cochlear compression and auditory-filter bandwidths were estimated in the same listeners. The estimated reduction of cochlear compression was significantly correlated with the increase in the F0DL ratio, while no correlation was found with filter bandwidth. The effects of degraded frequency selectivity and loss of compression were considered in a simplified peripheral model as potential factors in envelope enhancement. The model revealed that reducing cochlear compression significantly enhanced the envelope of an unresolved SP complex tone, while not affecting the envelope of a RP complex tone. This envelope enhancement in the SP condition was significantly correlated with the increased pitch-discrimination performance for the SP relative to the RP condition in the HI listeners. PMID:27604780

  17. 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. PMID:23990061

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

    PubMed

    Bianchi, Federica; Fereczkowski, Michal; Zaar, Johannes; Santurette, Sébastien; Dau, Torsten

    2016-01-01

    Physiological studies have shown that noise-induced sensorineural hearing loss (SNHL) enhances the amplitude of envelope coding in auditory-nerve fibers. As pitch coding of unresolved complex tones is assumed to rely on temporal envelope coding mechanisms, this study investigated pitch-discrimination performance in listeners with SNHL. Pitch-discrimination thresholds were obtained for 14 normal-hearing (NH) and 10 hearing-impaired (HI) listeners for sine-phase (SP) and random-phase (RP) complex tones. When all harmonics were unresolved, the HI listeners performed, on average, worse than NH listeners in the RP condition but similarly to NH listeners in the SP condition. The increase in pitch-discrimination performance for the SP relative to the RP condition (F0DL ratio) was significantly larger in the HI as compared with the NH listeners. Cochlear compression and auditory-filter bandwidths were estimated in the same listeners. The estimated reduction of cochlear compression was significantly correlated with the increase in the F0DL ratio, while no correlation was found with filter bandwidth. The effects of degraded frequency selectivity and loss of compression were considered in a simplified peripheral model as potential factors in envelope enhancement. The model revealed that reducing cochlear compression significantly enhanced the envelope of an unresolved SP complex tone, while not affecting the envelope of a RP complex tone. This envelope enhancement in the SP condition was significantly correlated with the increased pitch-discrimination performance for the SP relative to the RP condition in the HI listeners. PMID:27604780

  19. Protection and treatment of sensorineural hearing disorders caused by exogenous factors: experimental findings and potential clinical application.

    PubMed

    Duan, Mao Li; Ulfendahl, Mats; Laurell, Göran; Counter, S A; Counter, Allen S; Pyykkö, Ilmari; Borg, Erik; Rosenhall, Ulf

    2002-07-01

    During the last decade, there have been numerous interesting findings regarding the roles of neurotrophins, nitric oxide, reactive oxygen species, glutamate receptors, and shock protein in the auditory system. These findings have provided a scientific basis for the development of techniques to protect the auditory system against trauma as well as for the treatment of peripheral hearing disorders. This review focuses on recent advances in experimental prevention and treatment of hearing impairment which are expected to be of clinical value in the near future. Viral vector and non-viral vector gene therapy and transplantation of stem cells are discussed as potential treatments of irreversible sensorineural inner ear damage.

  20. 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

  1. 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

  2. Association of Chiari I malformation and cerebellar ectopia with sensorineural hearing loss.

    PubMed

    Haktanir, Alpay; Yücedağ, Fatih; Kaçar, Emre; Ulu, Sahin; Gültekin, Mehmet Ali; Ünlü, Ebru; Bucak, Abdülkadir; Ayçiçek, Abdullah

    2013-07-01

    We aimed to examine the prevalence of cerebellar tonsil ectopia and Chiari 1 malformation in sensorineural hearing loss (SHL) that has, to the best of our knowledge, not been studied previously. Magnetic resonance imaging records of 166 subjects with SHL and 50 controls without known otologic disturbances were included in the study. A tonsils descent more than 2 mm was assumed as cerebellar ectopia, and a descent equal to or more than 5 mm was assumed as Chiari 1 malformation. A tonsil descent group was also formed by summation of both groups. Transverse diameters of bilateral intracranial vertebral arteries and transverse sinuses were also measured, and all parameters were analyzed using appropriate statistics. A significant difference of frequencies of Chiari 1, ectopia, and tonsil descent was detected between patients and controls. In comparison of cerebellar ectopia and Chiari 1 groups, SHL did not show any significant difference. The left lateral sinus diameter showed positive correlation with tonsil descent. There was no significant correlation for the diameters of other vessels. A powerful correlation was detected between SHL and age. In addition, right and vertebral artery diameters showed positive correlations with age. Chiari 1 malformation and cerebellar ectopia showed an association with SHL. These patients should also be evaluated for otologic disturbances. Further high-resolution magnetic resonance imaging studies to explain the exact cause of this currently unknown association seems required.

  3. Proinflammatory and proadhesive activation of lymphocytes and macrophages in sudden sensorineural hearing loss.

    PubMed

    Kassner, Stefan S; Schöttler, Sarah; Bonaterra, Gabriel A; Stern-Sträter, Jens; Sommer, Ulrich; Hormann, Karl; Kinscherf, Ralf; Gössler, Ulrich R

    2011-01-01

    Even though sudden sensorineural hearing loss (SHL) is a quite frequent disease, the pathogenetic processes leading to it are widely unknown. There is increasing evidence that immunomodulatory cells, especially T lymphocytes, might be involved. Twelve patients with acute SHL and 12 healthy, age-matched individuals were included in this study. In addition to routine blood parameters, plasma levels of tumor necrosis factor alpha (TNF-α), soluble CD40 (sCD40) and sCD40 ligand (sCD40L) were determined by ELISA. Moreover, peripheral blood mononuclear cells were isolated by Ficoll density gradient. Afterwards, in subpopulations--identified by CD14 (monocytes), CD68 (macrophages), CD3 (T lymphocytes) or CD19 (B lymphocytes) immunoreactivity--proinflammatory (CD40, TNF-α or cyclooxygenase-2) and proadhesive (CD38) proteins were measured by 2-color fluorescence-activated cell sorter analyses. In comparison with healthy individuals, patients with acute SHL revealed elevated plasma levels of sCD40 and sCD40L and a significantly decreased percentage (36%) of lymphocytes, especially of T lymphocytes (28%). Additionally, in patients with acute SHL the percentage of proinflammatory CD40, TNF-α, cyclooxygenase-2 or CD38-positive T or B lymphocytes was significantly increased. Our data suggest an enhanced extravasation of proadhesive and proinflammatory lymphocytes from the peripheral circulation, which may contribute to SHL disease induction as well as progression and, thus, may be suggested as a novel therapeutical target.

  4. Cytomegalovirus-induced sensorineural hearing loss with persistent cochlear inflammation in neonatal mice.

    PubMed

    Schachtele, Scott J; Mutnal, Manohar B; Schleiss, Mark R; Lokensgard, James R

    2011-06-01

    Congenital cytomegalovirus (CMV) infection is the leading cause of sensorineural hearing loss (SNHL) in children. During murine (M)CMV-induced encephalitis, the immune response is important for both the control of viral dissemination and the clearance of virus from the brain. While the importance of CMV-induced SNHL has been described, the mechanisms surrounding its pathogenesis and the role of inflammatory responses remain unclear. This study presents a neonatal mouse model of profound SNHL in which MCMV preferentially infected both cochlear perilymphatic epithelial cells and spiral ganglion neurons. Interestingly, MCMV infection induced cochlear hair cell death by 21 days post-infection, despite a clear lack of direct infection of hair cells and the complete clearance of the virus from the cochlea by 14 dpi. Flow cytometric, immunohistochemical, and quantitative PCR analysis of MCMV-infected cochlea revealed a robust and chronic inflammatory response, including a prolonged increase in reactive oxygen species production by infiltrating macrophages. These data support a pivotal role for inflammation during MCMV-induced SNHL.

  5. Sudden sensorineural hearing loss associated with iron-deficiency anemia: a population-based study.

    PubMed

    Chung, Shiu-Dong; Chen, Po-Yueh; Lin, Herng-Ching; Hung, Shih-Han

    2014-05-01

    IMPORTANCE Vascular events play a big part in the development of sudden sensorineural hearing loss (SSNHL), but only those associated with sickle-cell anemia have been previously associated with SSNHL. This study demonstrates an association between SSNHL and prior iron-deficiency anemia (IDA).OBJECTIVE To evaluate the association between IDA and SSNHL using a nationwide population-based database.DESIGN, SETTING, AND PARTICIPANTS In this case-control study in Taiwan, participants with SSNHL (n = 4004) were identified, and controls (n = 12 012) were randomly selected.MAIN OUTCOMES AND MEASURES Conditional logistic regression was used to calculate the ORs (95%CIs) for IDA in participants with SSNHL vs controls.RESULTS Of the 16 016 sampled participants, 533 (3.3%) had previously been diagnosed with IDA, including 172 (4.3%) participants with SSNHL and 361 (3.0%) controls. The χ2 test revealed a significant difference (P < .001) in the prevalence of prior IDA between participants with SSNHL and controls. By conditional logistic regression, we found that the OR for previous IDA among the participants with SSNHL was 1.34 (95%CI, 1.11-1.61) (P < .01)after adjusting for monthly income, geographic region, urbanization level, and comorbidities(ie, hypertension, diabetes, hyperlipidemia, renal disease, and coronary heart disease). The significant relationship between SSNHL and prior IDA was most pronounced among those 44 years or younger (adjusted OR, 1.91; 95%CI, 1.35-2.72) (P < .001) for the participants with SSNHL compared with controls, and the strength of this relationship decreased with age.CONCLUSIONS AND RELEVANCE There is an association between SSNHL and prior IDA.Patients with IDA, especially those younger than 60 years, should be more aggressively surveyed and managed to reduce hearing-related morbidities.

  6. Sudden sensorineural hearing loss and polymorphisms in iron homeostasis genes: new insights from a case-control study.

    PubMed

    Castiglione, Alessandro; Ciorba, Andrea; Aimoni, Claudia; Orioli, Elisa; Zeri, Giulia; Vigliano, Marco; Gemmati, Donato

    2015-01-01

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

  7. Comparison of simultaneous systemic steroid and hyperbaric oxygen treatment versus only steroid in idiopathic sudden sensorineural hearing loss.

    PubMed

    Ersoy Callioglu, Elif; Tuzuner, Arzu; Demirci, Sule; Cengiz, Ceyhun; Caylan, Refik

    2015-01-01

    A retrospective chart review to assess the effectiveness of hyperbaric oxygen treatment in sudden sensorineural hearing loss. 44 patients aged between 17-67 years diagnosed with idiopathic sensorineural hearing loss less than 30 days were admitted to our clinic Patients were treated with systemic steroid alone or systemic steroid plus hyperbaric oxygen therapy. In the comparison of two groups, there was no statistically significant difference of audiometric evaluation (P>0.0028) found in hearing improvement for each frequency on 5th day of the treatment and post treatment. Age (≤45 and >45) and initial hearing level (≤60 dB. And >60 dB.) does not seem to be an influential factor according to the results of the study (P>0.0007). The present study did not show more superior healing effect of hyperbaric oxygen therapy added to systemic steroid therapy than steroid infusion alone. The results are consistent with those of some papers. However there are also conflicting data that support significant effect of hyperbaric oxygen therapy. Routine administration of this therapy seems to be unnecessary in view of these results.

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

    PubMed

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

    2016-08-01

    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

  9. Genetic characteristics of the couple with non-syndromic sensorineural hearing loss and fertility guidance

    PubMed Central

    Liu, Ri-Ming; Liu, Hong-Jie; Cong, Jiang-Lin; Sun, Ai-Ling; Du, Jiang-Dong; Sun, Cheng-Ming

    2015-01-01

    Purpose: We aim to report a genetic testing and fertility guidance for the deaf through analyzing pedigree and molecular genetic characteristics of the couple who have non-syndromic sensorineural hearing loss (NSHL). Methods: One of hospitalized congenial deaf couple and family members were included in this study. The wife was twin pregnant woman and her gestational age was 31+5 pregnant weeks. The DNA was extracted from peripheral blood and umbilical vein blood, respectively. Mutation screening of common deafness genes was performed in pregnant women and other family members. Nine common mutations in four major deafness genes, GJB2 (35delG, 176del16, 235delC, 299delAT), GjB3 (C538T), SLC26A4 (IVS7-2A>G, A2168G) and Mitochondrial 12S rRNA (A1555G, C1494T), were detected simultaneously with a microarray based method. SLC26A4 whole genome sequencing was carried out for the results of the DNA microarray. According to the test results, the couple chose abortion termination of pregnancy twins, and after one year obtained singleton pregnancy by artificial insemination by donor (AID). In week 16 of pregnancy, amniocentesis had been done to collect fetal somatic cell and extract DNA, and then the above tests had been repeated. Results: The couple had SLC26A4 combined heterozygous mutation. Both parents had SLC26A4 single heterozygous mutation. Twin fetuses had SLC26A4 combined heterozygous mutation. The probability of naturally being pregnant and bearing deaf children for the pregnant women was 100%. Fetus obtained by AID had SLC26A4 single heterozygous mutation. After the birth of the baby, her hearing has been normal. Conclusions: To reduce children with congenital deafness, screening high mutation sites by microarray, combined with pedigree analysis and gene sequencing is effective, and should be used as a routine inspection item for the deaf before marriage and pregnancy. On the basis of genetic testing for the couple with hearing loss, human assisted reproductive

  10. A comparison of phonological processing skills of children with mild to moderate sensorineural hearing loss and children with dyslexia.

    PubMed

    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 phonological deficits that were restricted to phonological awareness tasks (elision/blending) and a phonological memory task (nonword repetition), yet exhibited unimpaired rapid naming ability. Children with dyslexia showed deficits in all 3 phonological constructs. Finally, both degree of hearing loss and age at which hearing loss was identified in the MSNH group were related to the children's phonological processing skills. Because of their deteriorated phonological skills, children with MSNH may be at risk of starting school with weaknesses in early literacy skills. Implications for practice aimed at improving phonological and literacy skills of these children are described. PMID:22978204

  11. [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

  12. 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

  13. 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.

  14. 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.

  15. A novel nonsense CDK5RAP2 mutation in a Somali child with primary microcephaly and sensorineural hearing loss.

    PubMed

    Pagnamenta, Alistair T; Murray, Jennie E; Yoon, Grace; Sadighi Akha, Elham; Harrison, Victoria; Bicknell, Louise S; Ajilogba, Kaseem; Stewart, Helen; Kini, Usha; Taylor, Jenny C; Keays, David A; Jackson, Andrew P; Knight, Samantha J L

    2012-10-01

    Primary microcephaly is a genetically heterogeneous condition characterized by reduced head circumference (-3 SDS or more) and mild-to-moderate learning disability. Here, we describe clinical and molecular investigations of a microcephalic child with sensorineural hearing loss. Although consanguinity was unreported initially, detection of 13.7 Mb of copy neutral loss of heterozygosity (cnLOH) on chromosome 9 implicated the CDK5RAP2 gene. Targeted sequencing identified a homozygous E234X mutation, only the third mutation to be described in CDK5RAP2, the first in an individual of non-Pakistani descent. Sensorineural hearing loss is not generally considered to be consistent with autosomal recessive microcephaly and therefore it seems likely that the deafness in this individual is caused by the co-occurrence of a further gene mutation, independent of CDK5RAP2. Nevertheless, further detailed clinical descriptions of rare CDK5RAP2 patients, including hearing assessments will be needed to resolve fully the phenotypic range associated with mutations in this gene. This study also highlights the utility of SNP-array testing to guide disease gene identification where an autosomal recessive condition is plausible. PMID:22887808

  16. Evaluation of a dual-channel full dynamic range compression system for people with sensorineural hearing loss.

    PubMed

    Moore, B C; Johnson, J S; Clark, T M; Pluvinage, V

    1992-10-01

    This article describes an evaluation of an in the ear hearing aid, which applies fast-acting full dynamic range compression independently in two frequency bands. This can compensate for the loudness recruitment typically associated with sensorineural hearing loss. The crossover frequency between the two bands and the gain and compression ratio in each band are programmable to suit the individual patient. Twenty subjects with moderate sensorineural hearing loss were tested in a counterbalanced order using the aid programmed as a linear amplifier (condition L) and as a two-band compressor (condition C). All subjects were fitted binaurally. Subjects were also tested without hearing aids (condition U) and using the hearing aids that they normally wore (condition Own). Speech intelligibility was measured in quiet at three sound levels (50, 65, and 80 dB SPL), and speech reception thresholds (SRTs) in 12-talker babble were measured under monaurally and binaurally aided conditions, with the speech and babble both coincident and spatially separated. In condition C, speech intelligibility in quiet was high at all sound levels. Speech intelligibility at the two lower levels decreased in condition L, and decreased still further in conditions Own and U. Condition C gave, on average, better speech intelligibility in babble (lower SRTs) than conditions L, Own, or U. The advantage of condition C over condition L varied across subjects and was correlated with the dynamic range for tones at high frequencies; small dynamic ranges were associated with greater benefit from compression. A significant advantage for binaural aiding was found both when the speech and noise were spatially separated and when they were coincident. The binaural advantage was similar for the C and L conditions, indicating that the independent compression at the two ears did not adversely affect the use of binaural cues. Questionnaires on the subjects' experiences with the aids in everyday life indicated that

  17. Combination Therapy with Systemic Steroids, an Antiviral Agent, Anticoagulants, and Stellate Ganglion Block for Treatment of Sudden Sensorineural Hearing Loss

    PubMed Central

    Lee, Chi-Kyou; Lee, Jong Dae; Park, Moo Kyun; Lee, Byung Don

    2012-01-01

    Background and Objectives Sudden sensorineural hearing loss (SSNHL) is commonly defined as a loss of at least 30 dB in three contiguous frequencies occurring within 3 days. Systemic steroid administration has become the most widely accepted treatment option for SSNHL. Since viral infection and vascular compromise are considered specific causes of SSNHL, antiviral agents, anticoagulants, and stellate ganglion block have been used for its treatment, although the evidence of their effectiveness is weak. The present study evaluated the hearing recovery rate in the combination therapy group (systemic steroids, antiviral agent, anticoagulants, and stellate ganglion block) in comparison with patients treated with systemic steroids alone. Subjects and Methods A total of 85 patients diagnosed with SSNHL were treated with combination therapy (group A, 46 patients) or systemic steroids only (group B, 39 patients). Hearing improvement was defined as a hearing gain of more than slight improvement using Siegel's criteria. All patients were treated with a 10-day course of systemic steroids (10-mg dexamethasone for 5 days, followed by tapering for 5 days). Acyclovir, heparin, and stellate ganglion block were included in the group A treatment regimen. Results The overall rate of hearing improvement was 60.9% (28/46 patients) in group A, which was significantly higher than that (38.5%, 15/39 patients) in group B. The distribution of prognostic factors was not significantly different between the two groups with the exception of the degree of initial hearing loss, which was more severe in group A. Upon analysis according to prognostic factors, group A showed a better hearing improvement recovery rate than group B in patients with hearing loss >70 dB, age >41 years, dizziness, and early treatment (<1 week). Conclusions Thus SSNHL patients treated with combination therapy have a higher likelihood of hearing improvement than those treated with systemic steroids alone. PMID:24653874

  18. Plasticity in the Developing Auditory Cortex: Evidence from Children with Sensorineural Hearing Loss and Auditory Neuropathy Spectrum Disorder

    PubMed Central

    Cardon, Garrett; Campbell, Julia; Sharma, Anu

    2013-01-01

    The developing auditory cortex is highly plastic. As such, the cortex is both primed to mature normally and at risk for re-organizing abnormally, depending upon numerous factors that determine central maturation. From a clinical perspective, at least two major components of development can be manipulated: 1) input to the cortex and 2) the timing of cortical input. Children with sensorineural hearing loss (SNHL) and auditory neuropathy spectrum disorder (ANSD) have provided a model of early deprivation of sensory input to the cortex, and demonstrated the resulting plasticity and development that can occur upon introduction of stimulation. In this article, we review several fundamental principles of cortical development and plasticity and discuss the clinical applications in children with SNHL and ANSD who receive intervention with hearing aids and/or cochlear implants. PMID:22668761

  19. 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.

  20. Treatment of sudden sensorineural hearing loss with transtympanic injection of steroids as single therapy: a randomized clinical study.

    PubMed

    Dispenza, Francesco; Amodio, Emanuele; De Stefano, Alessandro; Gallina, Salvatore; Marchese, Donatella; Mathur, Navneet; Riggio, Francesco

    2011-09-01

    The aim of this study was to verify the efficacy and the safety of transtympanic dexamethasone to treat sudden sensorineural hearing loss as first and single drug method. Considering ethical implication of performing a mininvasive procedure on middle ear, we matched such proposed treatment with systemic prednisone administration that represents the widest adopted protocol. Randomized prospective study was conducted. The inclusion criterion was a sudden sensorineural hearing loss of at least 30 dB across three contiguous frequencies over a period of 24 h. Group A received transtympanic steroid injections; Group B received oral administration of steroids. 25 patients were treated with transtympanic therapy whereas 21 underwent systemic treatment. The mean of initial PTA was 59 dB for the whole series: 65 dB for group A and 51 dB for group B. The recovery better than 10 dB was obtained in 80% of patients of group A and in 17 81% of patients of group B, with a total of 80.5%. The mean relative gain in PTA was 41.16% in the group A and 44.7% in the group B. In the frequencies tested (0.5, 1, 2, and 4 kHz) PTA improvements after transtympanic treatment were higher than after systemic treatment, but these differences were not statistically significant (P = 0.61). Both transtympanic and systemic treatment had similar clinical recovery times. This prospective randomized clinical study showed good result in terms of hearing recovery, better than the expected results of the simple observation without treatment. We can consider transtympanic administration as a first line treatment, because of the statistical analysis confirmed similar results with systemic therapy, reducing possible side effects of systemic drug administration. The delay of treatment does not influence the outcome, allowing treating patients within 10 days of onset.

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

    PubMed Central

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

    2012-01-01

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

  2. Distortion product otoacoustic emissions as a prognostic factor for idiopathic sudden sensorineural hearing loss.

    PubMed

    Chao, Ting-Kuang; Chen, Tony Hsiu-Hsi

    2006-01-01

    We investigated distortion product otoacoustic emissions (DPOAE) as a prognostic factor in idiopathic sudden sensorineural hearing loss (ISSHL) patients with the time-dependent Cox proportional-hazards model. We also compared the importance of the prognostic factors that are reported in the literature. 108 patients with ISSHL were included. Both DPOAE and pure tone audiometry were performed everyday for a maximum of 7 days during admission and followed every other week or monthly after discharge. All DPOAE amplitudes were analyzed at 2f1-f2--namely 1093, 1375, 1750, 2187, 2781, 3500, 4375, and 5500 Hz. The average of two series of DPOAE intensity corrected for the noise level in eight frequencies was coded as dichotomous at > or =6 or <6 dB. We selected the most updated DPOAE at 3 days or more before the assessment of recovery into the analysis. The potential confounders including age, sex, history of vertigo at onset, history of hypertension, diabetes, coronary arterial disease, and stroke, configuration and severity of initial pure tone audiometry, the duration from onset to treatment, auditory brainstem response (ABR), vestibular evoked myogenic potential (VEMP), and plasma triglyceride and cholesterol levels, hemoglobin level, and erythrocyte sedimentation rate were collected for evaluation. The results showed that a better DPOAE amplitude was a significantly good prognostic indicator both in univariate analysis (recovery rate ratio = 3.626, 95% CI = 2.119-6.205, p < 0.0001) and multivariate analysis (recovery rate ratio = 2.94, 95% CI = 1.537-5.624, p = 0.0011). The Kaplan-Meier estimates showed that the younger age group (< or =40 years) with better initial pure tone audiometry (<65 dB) represented a better prognosis that was compatible with previous literature (log-rank test, p = 0.0297 and p = 0.0019 respectively). In the univariate analysis, normal ABR and VEMP waveforms were associated with a better prognosis (ABR: recovery rate ratio = 2.984, 95% CI = 1

  3. The efficiency of intratympanic dexamethasone injection as a sequential treatment after initial systemic steroid therapy for sudden sensorineural hearing loss.

    PubMed

    Lee, Jong Bin; Choi, Seong Jun; Park, Keehyun; Park, Hun Yi; Choo, Oak-Sung; Choung, Yun-Hoon

    2011-06-01

    The effect of intratympanic steroid injection is controversial as salvage or initial treatment option for sudden sensorineural hearing loss (SSNHL) and almost unknown if it is consecutively to use after initial systemic steroids. This study aimed to analyze the efficiency of intratympanic dexamethasone injection (ITDI) as a sequential treatment in the patients who failed initial systemic steroid treatments for SSNHL. Forty-six patients with SSNHL who did not respond to initial systemic steroids were prospectively included in the study. The patients were randomly classified into two groups; the ITDI group (21 patients) did not take four sequential ITDI within 2 weeks after systemic steroids, and the control group (25 patients) took any more medications. Hearing improvement was defined as a 10 dB or more decrease in the pure tone average (PTA) of the four-frequencies (0.5, 1, 2, and 3 kHz). Hearing improvement was observed in 10 (47.6%) of 21 ITDI patients and in 4 (16.0%) of 25 control patients (P = 0.027). An improvement of the mean PTA was 11.4 dB in the ITDI group and 1.7 dB in the control group (P = 0.004). The ITDI group showed significant hearing improvement at low frequency (500 Hz) than the control group. The patients with 70 or more dB in PTA before ITDI showed significant hearing improvement than the other patients with better PTAs (P = 0.038). The sequential ITDI, which is performed immediately after initial systemic steroid therapy, may be a simple, effective second-line treatment of choice for the patients who show poor response to initial treatments for SSNHL.

  4. 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".

  5. Neural Alterations in Acquired Age-Related Hearing Loss

    PubMed Central

    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. PMID:27313556

  6. 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.

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

    PubMed Central

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

    2015-01-01

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

  8. The Relationship between Serum Lipids and Sudden Sensorineural Hearing Loss: A Systematic Review and Meta-Analysis

    PubMed Central

    Chang, I Jen; Kang, Chung Jan; Yueh, Chen Yu; Fang, Ku Hao; Yeh, Re Ming; Tsai, Yao Te

    2015-01-01

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

  9. 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.

  10. Sudden sensorineural hearing loss in multiple sclerosis: clinical course and possible pathogenesis.

    PubMed

    Hellmann, M A; Steiner, I; Mosberg-Galili, R

    2011-10-01

    OBJECTIVE - To assess the symptom of sudden hearing loss in multiple sclerosis (MS). METHOD - We reviewed patient files in our MS clinic between January 2004 and November 2009 for symptoms of sudden hearing loss. RESULTS - We were able to identify 11 of 253 patients (4.35%) with sudden hearing loss. In seven patients, the hearing decline was the presenting symptom of MS and in all 11 patients, it appeared early in the course of the disease. There was no residual hearing deficit in 9/11 patients. In no patient was the condition bilateral and in none did it recur. CONCLUSION - Episodes of hearing loss are not uncommon in MS and have a good chance of complete recovery.

  11. A preliminary study on the role of inherited prothrombotic risk factors in Taiwanese patients with sudden sensorineural hearing loss.

    PubMed

    Lan, Ming-Ying; Shiao, Jiun-Yih; Hsu, Yen-Bin; Lin, Feng-Yi; Lin, Jin-Ching

    2011-06-01

    Sudden sensorineural hearing loss (SSHL) is a disease with unknown etiology. Recently, several studies revealed that some inherited prothrombotic risk factors are associated with SSHL in western populations. The objective of this study was to investigate the roles of the two most common genetic prothrombotic factors, the factor V Leiden G1691A and prothrombin G20210A in Taiwanese patients with SSHL. Twenty-four patients diagnosed with SSHL of more than 30 dB on average pure tone audiometry (PTA) and thirty-six healthy subjects without a history of hearing loss were enrolled in this study. Genomic DNA was isolated from peripheral blood leukocytes and the single nucleotide polymorphisms (SNPs) genotyping of factor V Leiden G1691A and prothrombin G20210A were analyzed using the TaqMan genotyping assays. Neither factor V Leiden G1691A nor prothrombin G20210A was detected in SSHL patients or in the control subjects. Both the patient group and the control group exhibited wild-type V Leiden 1691GG and wild-type prothrombin 20210GG. In conclusion, the factors V Leiden G1691A and prothrombin G20210A do not seem to play any role in Taiwanese patients with SSHL. Further studies with a large series of patients are needed to identify other possible candidate genes in order to elucidate the pathogenesis of SSHL.

  12. Impaired CD4+CD25+ regulatory T cell activity in the peripheral blood of patients with autoimmune sensorineural hearing loss.

    PubMed

    Xia, Ming; Zhang, Han Bing; Liu, Fang; Yin, Hai Ying; Xu, An Ting

    2008-09-01

    CD4+CD25+ regulatory T cells exert an immune regulatory function and thus play an important role in the control of self-reactivity in the pathogenesis of autoimmune inflammatory conditions. The aim of the study presented here is to perform a quantitative and functional analyses of these cells in patients with autoimmune sensorineural hearing loss (ASNHL). T cell subsets (CD4+CD25+, CD4+CD25(high), CD4+, and CD8+) from the peripheral blood of 17 patients with ASNHL, 16 patients with noise induced hearing loss (NHL), and 100 normal controls were analyzed by flow cytometry. The CD4/CD8 ratio was also analyzed. In addition, the suppressive capability of CD4+CD25+ T cells was tested in vitro by measuring their ability to suppress the proliferation and IFN-gamma secretion of CD4+CD25- T cells. No significant difference was found in the T cell subsets of ASNHL patients compared to normal controls or NHL patients, except that the proportion of CD4+ T cells was elevated in ASNHL patients. However, we did observe defective regulatory function of CD4+CD25+ T cells in patients with ASNHL. Our data supported the idea that CD4+CD25+ regulatory T cells played an immunosuppressive function in the periphery. The impaired suppressive activity of these cells may be an important factor in the pathogenesis of ASNHL.

  13. 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.

  14. 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)

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

    PubMed

    Locher, Heiko; de Groot, John C M J; van Iperen, Liesbeth; Huisman, Margriet A; Frijns, Johan H M; Chuva de Sousa Lopes, Susana M

    2015-11-01

    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. PMID:25663387

  16. 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

  17. Acute Sensorineural Hearing Loss After Abuse of an Inhaled, Crushed Oxymorphone Extended-Release Tablet.

    PubMed

    MacDonald, Lisa E; Onsrud, Jennifer E; Mullins-Hodgin, Rita

    2015-07-01

    Oxymorphone, a semisynthetic μ-opioid receptor agonist, is the major active metabolite of oxycodone. It is a highly potent narcotic analgesic due to its high lipid solubility, which allows it to readily cross the blood-brain barrier and enter the central nervous system. It is available as both an immediate-release and extended-release (ER) formulation. Oxymorphone can be abused by injection or inhalation of crushed tablets; thus, in 2011, the manufacturer of ER oxymorphone reformulated the drug with crush-resistant technology to deter its misuse and abuse. We describe the case of a previously healthy, 24-year-old male who experienced reproducible acute subjective bilateral temporary hearing loss that occurred after inhalation of oxymorphone. He presented to the emergency department complaining of acute bilateral hearing loss after he reported snorting a crushed oxymorphone ER 30-mg tablet. Emergency department evaluation revealed obvious bilateral hearing loss as well as coincidental aspiration pneumonia. The patient's medical history revealed that he had experienced a similar episode of hearing loss after a previous episode of oxymorphone inhalation. His hearing loss began to improve 3 hours after presentation to the emergency department and was completely resolved by the following day. Use of the Naranjo adverse drug reaction probability scale revealed oxymorphone to be a probable cause of this patient's acute hearing loss (score of 6). The mechanism of action of opioid-associated hearing loss (OAHL) is not completely understood, but it is thought to be due to disturbances within the cochlea, such as cochlear ischemia. To our knowledge, this is only the second published case report of acute reversible hearing loss following oxymorphone inhalation and the first published case report of reproducible OAHL. Since opioid misuse continues to be prevalent despite attempts at reformulations to make the drugs crush resistant, a high degree of clinical suspicion is needed

  18. 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.

  19. Genetic linkage analysis of a novel syndrome comprising North Carolina-like macular dystrophy and progressive sensorineural hearing loss

    PubMed Central

    Francis, P J; Johnson, S; Edmunds, B; Kelsell, R E; Sheridan, E; Garrett, C; Holder, G E; Hunt, D M; Moore, A T

    2003-01-01

    Aim: To characterise the phenotype and identify the underlying genetic defect in a family with deafness segregating with a North Carolina-like macular dystrophy (NCMD). Methods: Details of the family were obtained from the Moorfields Eye Hospital genetic clinic database and comprised eight affected, four unaffected members, and two spouses. Pedigree data were collated and leucocyte DNA extracted from venous blood. Positional candidate gene and genetic linkage strategies utilising polymerase chain reaction (PCR) based microsatellite marker genotyping were performed to identify the disease locus. Results: The non-progressive ocular phenotype shared similarities with North Carolina macular dystrophy. Electro-oculography and full field electroretinography were normal. Progressive sensorineural deafness was also present in all affected individuals over the age of 20 years. Hearing was normal in all unaffected relatives. Haplotype analysis indicated that this family is unrelated to previously reported families with NCMD. Genotyping excluded linkage to the MCDR1 locus and suggested a potential novel disease locus on chromosome 14q (Z=2.92 at θ=0 for marker D14S261). Conclusion: The combination of anomalies segregating in this family represents a novel phenotype. This molecular analysis indicates the disease is genetically distinct from NCMD. PMID:12812894

  20. Novel, Compound Heterozygous, Single Nucleotide Variants in MARS2 Associated with Developmental Delay, Poor Growth, and Sensorineural Hearing Loss

    PubMed Central

    Webb, Bryn D.; Wheeler, Patricia G.; Hagen, Jacob J.; Cohen, Ninette; Linderman, Michael D.; Diaz, George A.; Naidich, Thomas P.; Rodenburg, Richard J.; Houten, Sander M.; Schadt, Eric E.

    2015-01-01

    Novel, single nucleotide mutations were identified in the mitochondrial methionyl amino-acyl-tRNA synthetase gene (MARS2) via whole exome sequencing in two affected siblings with developmental delay, poor growth, and sensorineural hearing loss. We show that compound heterozygous mutations c.550C>T:p.Gln184* and c.424C>T:p.Arg142Trp in MARS2 lead to decreased MARS2 protein levels in patient lymphoblasts. Analysis of respiratory complex (RC) enzyme activities in patient fibroblasts revealed decreased Complex I and IV activities. Immunoblotting of patient fibroblast and lymphoblast samples revealed reduced protein levels of NDUFB8 and COXII, representing Complex I and IV respectively. Additionally, overexpression of wild-type MARS2 in patient fibroblasts increased NDUFB8 and COXII protein levels. These findings suggest that recessive single nucleotide mutations in MARS2 are causative for a new mitochondrial translation deficiency disorder with a primary phenotype including developmental delay and hypotonia. Identification of additional patients with single nucleotide mutations in MARS2 is necessary to determine if pectus carinatum is also a consistent feature of this syndrome. PMID:25754315

  1. Gallstone is correlated with an increased risk of idiopathic sudden sensorineural hearing loss: a retrospective cohort study

    PubMed Central

    Chiu, Chong-Chi; Lee, Kuan-Ji; Weng, Shih-Feng; Yang, Yuan-Ming; Lin, Yung-Song

    2015-01-01

    Objectives This study aims to test the hypothesis that gallstone disease (GSD) is a risk factor for the development of idiopathic sudden sensorineural hearing loss (ISSNHL). Research has shown risks of cardiovascular and cerebrovascular events in patients with GSD; however, well-conducted English studies on the association between GSD and the development of ISSNHL are lacking. Design and setting Retrospective cohort study using the Taiwan Longitudinal Health Insurance Database. Participants We compared 26 449 patients diagnosed with GSD between 1 January 2001 and 31 December 2007, with 52 898 age-matched, gender-matched and comorbidities propensity scores-matched controls. Outcome measured We followed each patient until the end of 2011 and evaluated the incidence of ISSNHL for at least 4 years after the initial GSD diagnosis. Results The incidence of ISSNHL was 1.42 times higher in the GS cohort than in the non-GS cohort (9.27 vs 6.52/10 000 person-years). Using Cox proportional hazard regressions, the adjusted HR was 1.44 (95% CI 1.19 to 1.74). In the cohort of patients with GSD who needed a cholecystectomy, 37 patients suffered from ISSNHL. Among those patients, 31 (83.7%) patients sustained ISSNHL before cholecystectomy and 6 (16.2%) patients sustained ISSNHL after cholecystectomy. Conclusions A diagnosis of GSD may be an independent risk for ISSNHL. This finding suggests that an underlying vascular and inflammatory mechanism may contribute to the development of ISSNHL. Physicians may want to counsel patients with GSD to seek medical attention if they have hearing impairments, because patients may be at an increased risk of developing ISSNHL. PMID:26419682

  2. Risk of sudden sensorineural hearing loss in patients with systemic lupus erythematosus: a population-based cohort study.

    PubMed

    Lin, Charlene; Lin, Shih-Wei; Weng, Shih-Feng; Lin, Yung-Song

    2013-01-01

    Sudden sensorineural hearing loss (SSHL) may be a manifestation of systemic vascular involvement in systemic lupus erythematosus (SLE) and may have an important impact on the health of patients with SLE. To investigate the risk of developing SSHL in patients with SLE, we conducted a population-based, retrospective cohort study from the Taiwan National Health Insurance Research Database. A total of 7168 patients diagnosed with SLE and 35840 control subjects without SLE were selected from claims made from 2001 to 2006. The incidence of SSHL was assessed and determined at the end of 2010. The incidence of SSHL was 2.22-fold higher in the SLE group than in the non-SLE group (6.52 vs. 2.93 per 10000 person-years), with an adjusted hazard ratio (HR) of 2.253 (95% confidence interval, CI=1.407-3.608) calculated using a Cox proportional hazard regression model. Age was an independent risk factor for SSHL, with adjusted HRs of 2.103 for individuals aged≥35 years compared with those 0-34 years. In the 0-34 age range, the incidence of developing SSHL was 4.27-fold (95% CI=2.11-8.67) higher in the SLE group compared with the non-SLE group. In female patients, the incidence of developing SSHL was 2.19-fold (95% CI=1.73-3.50) higher in the SLE group than in the non-SLE group. Systemic lupus erythematosus was significantly associated with an increased risk of developing SSHL. Scheduled auditory examinations for patients with SLE to assess the presence of chronic hearing impairment are advised to enable the early detection of SSHL.

  3. 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.

  4. In-flight speech intelligibility evaluation of a service member with sensorineural hearing loss: case report.

    PubMed

    Casto, Kristen L; Cho, Timothy H

    2012-09-01

    This case report describes the in-flight speech intelligibility evaluation of an aircraft crewmember with pure tone audiometric thresholds that exceed the U.S. Army's flight standards. Results of in-flight speech intelligibility testing highlight the inability to predict functional auditory abilities from pure tone audiometry and underscore the importance of conducting validated functional hearing evaluations to determine aviation fitness-for-duty. PMID:23025145

  5. A missense variant of the ATP1A2 gene is associated with a novel phenotype of progressive sensorineural hearing loss associated with migraine.

    PubMed

    Oh, Se-Kyung; Baek, Jeong-In; Weigand, Karl M; Venselaar, Hanka; Swarts, Herman G P; Park, Seong-Hyun; Hashim Raza, Muhammad; Jung, Da Jung; Choi, Soo-Young; Lee, Sang-Heun; Friedrich, Thomas; Vriend, Gert; Koenderink, Jan B; Kim, Un-Kyung; Lee, Kyu-Yup

    2015-05-01

    Hereditary sensorineural hearing loss is an extremely clinical and genetic heterogeneous disorder in humans. Especially, syndromic hearing loss is subdivided by combinations of various phenotypes, and each subtype is related to different genes. We present a new form of progressive hearing loss with migraine found to be associated with a variant in the ATP1A2 gene. The ATP1A2 gene has been reported as the major genetic cause of familial migraine by several previous studies. A Korean family presenting progressive hearing loss with migraine was ascertained. The affected members did not show any aura or other neurologic symptoms during migraine attacks, indicating on a novel phenotype of syndromic hearing loss. To identify the causative gene, linkage analysis and whole-exome sequencing were performed. A novel missense variant, c.571G>A (p.(Val191Met)), was identified in the ATP1A2 gene that showed co-segregation with the phenotype in the family. In silico studies suggest that this variant causes a change in hydrophobic interactions and thereby slightly destabilize the A-domain of Na(+)/K(+)-ATPase. However, functional studies failed to show any effect of the p.(Val191Met) substitution on the catalytic rate of this enzyme. We describe a new phenotype of progressive hearing loss with migraine associated with a variant in the ATP1A2 gene. This study suggests that a variant in Na(+)/K(+)-ATPase can be involved in both migraine and hearing loss.

  6. MTHFR 677T Is a Strong Determinant of the Degree of Hearing Loss Among Polish Males with Postlingual Sensorineural Hearing Impairment

    PubMed Central

    Pollak, Agnieszka; Mueller-Malesinska, Malgorzata; Lechowicz, Urszula; Skorka, Agata; Korniszewski, Lech; Sobczyk-Kopciol, Agnieszka; Waskiewicz, Anna; Broda, Grazyna; Iwanicka-Pronicka, Katarzyna; Oldak, Monika; Skarzynski, Henryk

    2012-01-01

    Hearing impairment (HI) is the most common sensory handicap. Congenital HI often has a genetic basis, whereas the etiology of nonsyndromic postlingual HI (npHI) usually remains unidentified. Our purpose was to test whether the MTHFR C677T (rs1801133) polymorphism affecting folate metabolism is associated with the occurrence or severity of npHI. We studied rs1801133 genotypes in 647 npHI patients (age <40, sudden sensorineural loss excluded, HI characterized as mean of better ear hearing thresholds for 0.5–8 kHz) and 3273 adult controls from the background population. Genotype distribution among patients and controls was similar, but among male cases (n=302) we found a dose-dependent correlation of MTHFR 677T with the degree of HI (mean thresholds in dB: 38.8, 44.9, and 53.3, for CC, CT, and TT genotypes, respectively; p=0.0013, pcor.=0.017). Among male patients rs1801133 TT significantly increased the risk of severe/profound HI (odds ratio=4.88, p=0.001). Among controls the known effect of MTHFR 677T on plasma total homocysteine was more pronounced in men than in women (p<0.00004 for genotype-sex interaction) suggesting that in Poland folate deficiency is more prevalent in males. In conclusion, we report a novel strong effect of MTHFR 677T among males with npHI. The functional significance of rs1801133 suggests that these patients may benefit from folate supplementation—an intervention which is simple, cheap, and devoid of side effects. PMID:22424391

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

    PubMed Central

    Parker, Mark A.

    2011-01-01

    Purpose To provide an overview of the methodologies involved in the field of hair cell regeneration. First, a tutorial on the biotechnological foundations of this field will be provided in order to assist the reader in the comprehension and interpretation of the research involved in hair cell regeneration. Next, a review of stem cell and gene therapy will be presented and a critical appraisal of their application to hair cell regeneration will be provided. The methodologies used in these approaches will be highlighted. Method Narrative review of the fields of cellular, molecular, and developmental biology, tissue engineering, and stem cell and gene therapy using the PubMed database. Results The use of biotechnological approaches to the treatment of hearing loss, such as stem cell and gene therapy, has led to new methods of regenerating cochlear hair cells in mammals. Conclusions There have been incredible strides made in assembling important pieces of the puzzle that comprise hair cell regeneration. However, mammalian hair cell regeneration using stem cell and gene therapy are years if not decades away from being clinically feasible. If the goals of the biological approaches are met, these therapies may represent the future treatments for hearing loss. PMID:21386039

  8. 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.

  9. Sensorineural Hearing Loss after Combined Intensity Modulated Radiation Therapy and Cisplatin-Based Chemotherapy for Nasopharyngeal Carcinoma12

    PubMed Central

    Wang, Jin; Chen, Yuan-Yuan; Tai, An; Chen, Xue-Lin; Huang, Shao-Ming; Yang, Cungen; Bao, Yong; Li, Ning-Wei; Deng, Xiao-Wu; Zhao, Chong; Chen, Ming; Li, X. Allen

    2015-01-01

    PURPOSE: The incidence of sensorineural hearing loss (SNHL) after treatment with combination of intensity-modulated radiation therapy (IMRT) and cisplatin-based chemotherapy in nasopharyngeal carcinoma (NPC) patients was evaluated, and relationships of SNHL with host factors, treatment-related factors, and radiation dosimetric parameters were investigated. METHODS: Fifty-one NPC patients treated with IMRT from 2004 to 2009 were analyzed. All patients received neoadjuvant, concurrent, or adjuvant use of cisplatin. Pure tone audiometry was performed during the follow-up period with a median time of 60 months, ranging from 28 to 84 months. Correlation of SNHL at low frequencies (pure tone average, 0.5-2 kHz) with a series of factors was analyzed. RESULTS: Among 102 ears, 12.7% had low-frequency SNHL and 42.2% had high-frequency (4 kHz) SNHL. The incidence of low-frequency SNHL was greater in patients with age > 40, with T-stage 4, or who received cumulative cisplatin dose (CCD) > 200 mg/m2 (P = .034, .011, and .003, respectively) and in ears with secretory otitis media (SOM) (P = .002). Several dosimetric parameters were found to be correlated with SNHL. Univariate analysis showed that the minimum radiation dose to 0.1 ml highest dose volume (D0.1 ml) of the cochlea was the best radiation-related predictive parameter. Multivariate analysis indicated that CCD, SOM, and D0.1 ml of cochlea (P = .035, .012, and .022, respectively) were the factors associated with SNHL. CONCLUSION: For NPC patients treated with IMRT and chemotherapy, the incidence of treatment-related SNHL was associated with CCD, D0.1 ml of cochlea, and SOM. PMID:26692526

  10. Risk of sudden sensorineural hearing loss in stroke patients: A 5-year nationwide investigation of 44,460 patients.

    PubMed

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

    2016-09-01

    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

  11. 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.

  12. Risk of sudden sensorineural hearing loss in stroke patients: A 5-year nationwide investigation of 44,460 patients.

    PubMed

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

    2016-09-01

    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.

  13. Retinitis pigmentosa and progressive sensorineural hearing loss caused by a C12258A mutation in the mitochondrial MTTS2 gene.

    PubMed Central

    Mansergh, F C; Millington-Ward, S; Kennan, A; Kiang, A S; Humphries, M; Farrar, G J; Humphries, P; Kenna, P F

    1999-01-01

    Family ZMK is a large Irish kindred that segregates progressive sensorineural hearing loss and retinitis pigmentosa. The symptoms in the family are almost identical to those observed in Usher syndrome type III. Unlike that in Usher syndrome type III, the inheritance pattern in this family is compatible with dominant, X-linked dominant, or maternal inheritance. Prior linkage studies had resulted in exclusion of most candidate loci and >90% of the genome. A tentative location for a causative nuclear gene had been established on 9q; however, it is notable that no markers were found at zero recombination with respect to the disease gene. The marked variability in symptoms, together with the observation of subclinical muscle abnormalities in a single muscle biopsy, stimulated sequencing of the entire mtDNA in affected and unaffected individuals. This revealed a number of previously reported polymorphisms and/or silent substitutions. However, a C-->A transversion at position 12258 in the gene encoding the second mitochondrial serine tRNA, MTTS2, was heteroplasmic and was found in family members only. This sequence change was not present in 270 normal individuals from the same ethnic background. The consensus C at this position is highly conserved and is present in species as divergent from Homo sapiens as vulture and platypus. The mutation probably disrupts the amino acid-acceptor stem of the tRNA molecule, affecting aminoacylation of the tRNA and thereby reducing the efficiency and accuracy of mitochondrial translation. In summary, the data presented provide substantial evidence that the C12258A mtDNA mutation is causative of the disease phenotype in family ZMK. PMID:10090882

  14. 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

  15. 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

  16. 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 ...

  17. 38 CFR 17.149 - Sensori-neural aids.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

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

  18. 38 CFR 17.149 - Sensori-neural aids.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

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

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

    PubMed

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

    2013-01-01

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

  20. 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

  1. 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

  2. Insulin Receptor Substrate 2 (IRS2)-Deficient Mice Show Sensorineural Hearing Loss That Is Delayed by Concomitant Protein Tyrosine Phosphatase 1B (PTP1B) Loss of Function

    PubMed Central

    Murillo-Cuesta, Silvia; Camarero, Guadalupe; González-Rodríguez, Águeda; de la Rosa, Lourdes Rodríguez; Burks, Deborah J; Avendaño, Carlos; Valverde, Ángela M; Varela-Nieto, Isabel

    2012-01-01

    The insulin receptor substrate (IRS) proteins are key mediators of insulin and insulinlike growth factor 1 (IGF-1) signaling. Protein tyrosine phosphatase (PTP)-1B dephosphorylates and inactivates both insulin and IGF-1 receptors. IRS2-deficient mice present altered hepatic insulin signaling and β-cell failure and develop type 2–like diabetes. In addition, IRS2 deficiency leads to developmental defects in the nervous system. IGF1 gene mutations cause syndromic sensorineural hearing loss in humans and mice. However, the involvement of IRS2 and PTP1B, two IGF-1 downstream signaling mediators, in hearing onset and loss has not been studied. Our objective was to study the hearing function and cochlear morphology of Irs2-null mice and the impact of PTP1B deficiency. We have studied the auditory brainstem responses and the cochlear morphology of systemic Irs2−/−Ptpn1+/+, Irs2+/+Ptpn1−/−and Irs2−/−Ptpn1−/− mice at different postnatal ages. The results indicated that Irs2−/−Ptpn1+/+ mice present a profound congenital sensorineural deafness before the onset of diabetes and altered cochlear morphology with hypoinnervation of the cochlear ganglion and aberrant stria vascularis, compared with wild-type mice. Simultaneous PTP1B deficiency in Irs2−/−Ptpn1−/− mice delays the onset of deafness. We show for the first time that IRS2 is essential for hearing and that PTP1B inhibition may be useful for treating deafness associated with hyperglycemia and type 2 diabetes. PMID:22160220

  3. Intratympanic steroids as primary initial treatment of idiopathic sudden sensorineural hearing loss. The Hospital Universitario Ramón y Cajal experience and review of the literature.

    PubMed

    Labatut, Tomás; Daza, María José; Alonso, Antonio

    2013-11-01

    The objective of this study was to determine the effectiveness, dosage, safety and comfort of intratympanic steroids as primary initial treatment of idiopathic sudden sensorineural hearing loss (ISSHL). Prospective nonrandomized clinical practice study was performed in Tertiary referral center university hospital. A total of 35 consecutive patients suffering from newly diagnosed sudden sensorineural hearing loss with mean pure-tone average thresholds of 81 ± 21 dB were treated. No previous therapy had been undertaken. Intratympanic steroid injection as primary initial treatment was administered during 2 weeks on a twice a week schedule, between 2010 and 2011. Treatment was started on average within 2 days of symptoms onset. Pre and post-treatment audiometric evaluations were analyzed on follow-up as well as tolerance of the procedure and possible adverse effects. 66-85 % of patients achieved successful treatment according to the different outcome criteria used to evaluate hearing improvement (Furuhashi criteria/improvement of ≥10 dB in pure-tone average). Mean post-treatment improvement regarding pure-tone average was 34 ± 21 dB. A 48 ± 43 % improvement in speech discrimination score was observed. Patients received an average of 18 mg of methylprednisolone per injection and a total dose of 72 mg per treatment cycle. No serious adverse effects were noted. Intratympanic steroid injection is an effective, safe and well-tolerated office based-procedure for the treatment of ISSHL as primary initial treatment that can avoid the potential adverse effects of systemic steroids. A higher dose, schedule of administration as well as standardization of hearing recovery criteria still need to be established.

  4. Even in the era of congenital hypothyroidism screening mild and subclinical sensorineural hearing loss remains a relatively common complication of severe congenital hypothyroidism.

    PubMed

    Bruno, Rocco; Aversa, Tommaso; Catena, Mariaausilia; Valenzise, Mariella; Lombardo, Fortunato; De Luca, Filippo; Wasniewska, Malgorzata

    2015-09-01

    Only few studies have focused on neurosensory hearing function of patients with congenital hypothyroidism (CH) identified by CH screening programs and treated early and, therefore, this issue remains still controversial. The aim of this study was to ascertain whether an early and adequate replacement treatment may be able to prevent sensorineural hearing loss in 32 screened children with CH and no associated risk factors for neuro-otologic alterations. These patients were recruited according to highly selective criteria aiming to preliminarily exclude the negative interference of both treatment variables and other underlying risk factors. All the selected patients underwent, at a median age of 15.4 years, an audiologic investigation, which evidenced a mild and subclinical hearing loss in 25% of them. The poorest hearing scores were recorded in the individuals with athyreosis and in those with absence of distal femur bony nucleus at CH diagnosis. The prevalence of hearing impairment was significantly higher in CH patients than in 32 age-matched control subjects with no CH (χ(2) = 6.3, p < 0.025). In light of these findings, we concluded that: a) 25% of CH patients detected by CH screening may show, at a median age of 15.4 years, a mild and subclinical hearing impairment, despite early and adequate replacement treatment; b) the risk of hearing loss is higher in CH young patients than in age-matched control subjects without CH; c) the risk of hearing loss is closely associated with the severity of CH; d) this risk is particularly relevant in the children with pre-natal onset of hypothyroidism.

  5. 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. PMID:27657322

  6. 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)

  7. Diffusion Tensor Imaging of the Auditory Neural Pathway for Clinical Outcome of Cochlear Implantation in Pediatric Congenital Sensorineural Hearing Loss Patients

    PubMed Central

    Huang, Lexing; Zheng, Wenbin; Wu, Chunxiao; Wei, Xiaoqin; Wu, Xianguang; Wang, Yanting; Zheng, Hongyi

    2015-01-01

    Although conventional structural MRI provides vital information in the evaluation of congenital sensorineural hearing loss (SNHL), it is relatively insensitive to white matter microstructure. Our objective was to evaluate possible changes in microstructure of the auditory pathway in children with congenital sensorineural hearing loss (SNHL), and the possible distinction between good and poor outcome of cochlear implantation (CI) patients by using diffusion tensor imaging (DTI). Twenty-four patients with congenital SNHL and 20 healthy controls underwent conventional MRI and DTI examination using a 1.5T MR scanner. The DTI metrics of fractional anisotropy (FA) and mean diffusivity (MD) of six regions of interest (ROIs) positioned along the auditory pathway—the trapezoid body, superior olivary nucleus, inferior colliculus, medial geniculate body, auditory radiation and white matter of Heschl's gyrus—was measured in all subjects. Among the 24 patients, 8 patients with a categorie of auditory performance (CAP) score over 6 were classified into the good outcome group, and 16 patients with a CAP score below 6 were classified into the poor outcome group. A significant decrease was observed in FA values while MD values remained unchanged at the six ROIs of SNHL patients compared with healthy controls. Compared to good outcome subjects, poor outcome subjects displayed decreased FA values at all of the ROIs. No changes were observed in MD values. Correlation analyses only revealed strong correlations between FA values and CAP scores, and strong correlations between CAP scores and age at implant were also found. No correlations of FA values with age at implant were observed. Our results show that preoperative DTI can be used to evaluate microstructural alterations in the auditory pathway that are not detectable by conventional MR imaging, and may play an important role in evaluating the outcome of CI. Early cochlear implantation might be more effectively to restore hearing

  8. Predictors of Rehabilitation Intervention Decisions in Adults with Acquired Hearing Impairment

    ERIC Educational Resources Information Center

    Laplante-Levesque, Ariane; Hickson, Louise; Worrall, Linda

    2011-01-01

    Purpose: This study investigated the predictors of rehabilitation intervention decisions in middle-age and older adults with acquired hearing impairment seeking help for the first time. Method: Using shared decision making, 139 participants were offered intervention options: hearing aids, communication programs (group or individual), and no…

  9. [The influence of dead lymphocytes from middle ear secretion on the development of sensorineural hearing impairment associated with acute otitis media].

    PubMed

    Yashan, A I; Khoruzhiĭ, I V

    2015-01-01

    The objective of the present work was to estimate the number of lymphocytes in the middle ear secretion that had died as a result of apoptosis or necrosis in the patients presenting with acute otitis media and to elucidate the relationship between this phenomenon and the development of sensorineural impairment of hearing. The study included a total of 106 patients suffering from acute middle otitis allocated two groups. Group 1 was comprised of 75 (70.8%) patients with hearing loss of the conductive type alone while group 2 contained 31 (29.2%) patients with the combined type of hearing loss. The contents of the tympanic cavity was obtained by means of tympanic puncture, the lymphocytes isolated from middle ear secretion were studied with the use of a flow cytometer in order to determine the number of dead cells. In the patients of group 1, 15.2±0.6% and 10.6±0.5% of all lymphocytes underwent apoptosis and necrosis respectively. In the patients of group, lymphocyte apoptosis was observed in 24.7±1.% of the cases (p<0.05) and necrosis in 14.0±0.5% ones (p<0.05). It was shown that liberation of intracellular proteolytic enzymes and cytokines taking place largely in the course of necrosis activates phagocytosis, stimulates the inflammatory reaction, and thereby promotes resolution of the pathological processes in the middle ear due to the prevention of their extension into the labyrinth.

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

    PubMed Central

    Nash-Kille, Amy; Sharma, Anu

    2014-01-01

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

  11. Exogenous IL-4-expressing bone marrow mesenchymal stem cells for the treatment of autoimmune sensorineural hearing loss in a guinea pig model.

    PubMed

    Tan, Chang-qiang; Gao, Xia; Guo, Lang; Huang, He

    2014-01-01

    Bone marrow mesenchymal stem cells (BMSCs) expressing recombinant IL-4 have the potential to remediate inflammatory diseases. We thus investigated whether BMSCs expressing exogenous IL-4 could alleviate autoimmune sensorineural hearing loss. BMSCs isolated from guinea pigs were transfected with recombinant lentivirus expressing IL-4. A total of 33 animals were divided into three groups. Group A received scala tympani injection of IL-4-expressing BMSCs, and Group B received control vector-expressing BMSCs, and Group C received phosphate-buffered saline. The distribution of implanted BMSCs in the inner ears was assessed by immunohistochemistry and fluorescence microscopy. Auditory brain-stem response (ABR) was monitored to evaluate the auditory changes. Following BMSCs transplantation, the threshold levels of ABR wave III decreased in Groups A and B and significant differences were observed between these two groups (P < 0.05). Transplanted BMSCs distributed in the scala tympani and scala vestibuli. In some ears with hearing loss, there was a decrease in the number of spiral ganglion cells and varying degrees of endolymphatic hydrops or floccule. Following transplantation, the lentivirus-infected BMSCs migrated to the inner ear and produced IL-4. Our results demonstrate that, upon transplantation, BMSCs and BMSCs expressing recombinant IL-4 have the ability to remediate the inflammatory injury in autoimmune inner ear diseases.

  12. Mechanisms of sensorineural cell damage, death and survival in the cochlea

    PubMed Central

    Wong, Ann C. Y.; Ryan, Allen F.

    2015-01-01

    The majority of acquired hearing loss, including presbycusis, is caused by irreversible damage to the sensorineural tissues of the cochlea. This article reviews the intracellular mechanisms that contribute to sensorineural damage in the cochlea, as well as the survival signaling pathways that can provide endogenous protection and tissue rescue. These data have primarily been generated in hearing loss not directly related to age. However, there is evidence that similar mechanisms operate in presbycusis. Moreover, accumulation of damage from other causes can contribute to age-related hearing loss (ARHL). Potential therapeutic interventions to balance opposing but interconnected cell damage and survival pathways, such as antioxidants, anti-apoptotics, and pro-inflammatory cytokine inhibitors, are also discussed. PMID:25954196

  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 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

  15. Association between the V Leiden G1691A mutation and sudden sensorineural hearing loss in Italian population: a meta-analysis.

    PubMed

    Shu, Jingcheng; Si, Yongfeng; Yin, Shihua; He, Meirong

    2016-09-01

    Epidemiological studies have reported inconsistent findings on the association between the V Leiden G1691A mutation and sudden sensorineural hearing loss (SSNHL) in Italian population. The aim of this meta-analysis was to clarify this association. PubMed, Embase, and the China National Knowledge Infrastructure (CNKI) were searched up to April 1, 2015. We used STATA12.0 to calculate summary odds ratios (ORs) with 95 % confidence intervals (CIs). Four studies including 958 patients were identified. Pooled data showed no significant association between V Leiden G1691A mutation and risk of SSNHL in Italian population: A vs. G (OR = 1.660, 95 % CI 0.428-6.446, P OR = 0.464) and AG vs. GG (OR = 1.680, 95 % CI 0.422-6.688, P OR = 0.462). The present meta-analysis suggests that V Leiden G1691A mutation is not significantly associated with increased risk of SSNHL disease in Italian population. Further large and well-designed studies are needed to confirm this association.

  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 Central

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

    2012-01-01

    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. PMID:22877234

  17. 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

    Han, Kyu-Hee; Kim, Ah Reum; 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

  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. 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.

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

    PubMed

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

    2009-12-01

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

  3. Bi-allelic Truncating Mutations in CEP78, Encoding Centrosomal Protein 78, Cause Cone-Rod Degeneration with Sensorineural Hearing Loss.

    PubMed

    Namburi, Prasanthi; Ratnapriya, Rinki; Khateb, Samer; Lazar, Csilla H; Kinarty, Yael; Obolensky, Alexey; Erdinest, Inbar; Marks-Ohana, Devorah; Pras, Eran; Ben-Yosef, Tamar; Newman, Hadas; Gross, Menachem; Swaroop, Anand; Banin, Eyal; Sharon, Dror

    2016-09-01

    Inherited retinal diseases (IRDs) are a diverse group of genetically and clinically heterogeneous retinal abnormalities. The present study was designed to identify genetic defects in individuals with an uncommon combination of autosomal recessive progressive cone-rod degeneration accompanied by sensorineural hearing loss (arCRD-SNHL). Homozygosity mapping followed by whole-exome sequencing (WES) and founder mutation screening revealed two truncating rare variants (c.893-1G>A and c.534delT) in CEP78, which encodes centrosomal protein 78, in six individuals of Jewish ancestry with CRD and SNHL. RT-PCR analysis of CEP78 in blood leukocytes of affected individuals revealed that the c.893-1G>A mutation causes exon 7 skipping leading to deletion of 65bp, predicted to result in a frameshift and therefore a truncated protein (p.Asp298Valfs(∗)17). RT-PCR analysis of 17 human tissues demonstrated ubiquitous expression of different CEP78 transcripts. RNA-seq analysis revealed three transcripts in the human retina and relatively higher expression in S-cone-like photoreceptors of Nrl-knockout retina compared to rods. Immunohistochemistry studies in the human retina showed intense labeling of cone inner segments compared to rods. CEP78 was reported previously to interact with c-nap1, encoded by CEP250 that we reported earlier to cause atypical Usher syndrome. We conclude that truncating mutations in CEP78 result in a phenotype involving both the visual and auditory systems but different from typical Usher syndrome. PMID:27588452

  4. 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.

  5. [Hearing implants].

    PubMed

    Stokroos, Robert J; George, Erwin L J

    2013-01-01

    In the Netherlands, more than 1.5 million people suffer from sensorineural hearing loss or deafness. However, fitting conventional hearing aids does not provide a solution for everyone. In recent decades, developments in medical technology have produced implantable and other devices that restore both sensorineural and conductive hearing losses. These hearing devices can be categorized into bone conductive devices, implantable middle ear prostheses, cochlear implants and auditory brainstem implants. Furthermore, new implants aimed at treating tinnitus and loss of vestibular function have recently been developed.

  6. Mutational screening in patients with profound sensorineural hearing loss and neurodevelopmental delay: Description of a novel m.3861A > C mitochondrial mutation in the MT-ND1 gene.

    PubMed

    Ammar, Marwa; Tabebi, Mouna; Sfaihi, Lamia; Alila-Fersi, Olfa; Maalej, Marwa; Felhi, Rahma; Chabchoub, Imen; Keskes, Leila; Hachicha, Mongia; Fakhfakh, Faiza; Mkaouar-Rebai, Emna

    2016-06-10

    Mitochondrial diseases caused by mitochondrial dysfunction are a clinically and genetically, heterogeneous group of disorders involving multiple organs, particularly tissues with high-energy demand. Hearing loss is a recognized symptom of a number of mitochondrial diseases and can result from neuronal or cochlear dysfunction. The tissue affected in this pathology is most probably the cochlear hair cells, which are essential for hearing function since they are responsible for maintaining the ionic gradients necessary for sound signal transduction. Several mitochondrial DNA mutations have been associated with hearing loss and since mitochondria are crucial for the cellular energy supply in many tissues, most of these mtDNA mutations affect several tissues and will cause syndromic hearing loss. In the present study, we described 2 patients with sensorineural hearing loss and neurodevelopmental delay in whom we tested mitochondrial genes described to be associated with syndromic hearing loss. One of these patients showed a novel heteroplasmic mitochondrial mutation m.3861A > C (W185C) which lead to a loss of stability of the ND1 protein since it created a new hydrogen bund between the unique created cystein C185 and the A182 residue. In the second patient, we detected two novel heteroplasmic variations m.12350C > A (T5N) and m.14351T > C (E108G) respectively in the MT-ND5 and the MT-ND6 genes. The TopPred II prediction for the E108G variation revealed a decrease of the hydrophobicity in the mutated MT-ND6. PMID:27155156

  7. 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.

  8. 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

  9. Dilemmatic group memberships of hard-of-hearing employees during the process of acquiring and adapting to the use of hearing aids.

    PubMed

    Koskela, Inka; Ruusuvuori, Johanna; Juvonen-Posti, Pirjo; Nevala, Nina; Husman, Päivi; Aaltonen, Tarja; Lonka, Eila; Laakso, Minna

    2016-09-01

    We describe how hard-of-hearing (HOH) employees renegotiate both their existing and new group memberships when they acquire and begin to use hearing aids (HAs). Our research setting was longitudinal and we carried out a theory-informed qualitative analysis of multiple qualitative data. When an individual discovers that they have a hearing problem and acquire a HA, their group memberships undergo change. First, HOH employees need to start negotiating their relationship with the HOH group. Second, they need to consider whether they see themselves as members of the disabled or the nondisabled employee group. This negotiation tends to be context-bound, situational, and nonlinear as a process, involving a back-and-forth movement in the way in which HOH employees value different group memberships. The dilemmatic negotiation of new group memberships and the other social aspects involved in HA rehabilitation tend to remain invisible to rehabilitation professionals, occupational healthcare, and employers. PMID:27128825

  10. The impact of acquired hearing impairment on intimate relationships: implications for rehabilitation.

    PubMed

    Hétu, R; Jones, L; Getty, L

    1993-01-01

    Hearing disabilities, due to their interactive nature, strongly affect intimate relationships. This paper reviews the literature on this dimension of the experience of hearing impairment. The effects of progressive hearing loss and of the coping process within the couple are examined borrowing concepts from symbolic interaction theory. A summary of the interactive dimensions of the effects of hearing impairment on the couple is drawn from the relevant studies. Specific needs have been identified for each partner in terms of information, support and communication facilitation. Implications are drawn for the audiological rehabilitation of hearing-impaired adults. PMID:8267527

  11. 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.

  12. 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.

  13. Genetic Basis of Nonsyndromic Sensorineural Hearing Loss in the Sub-Saharan African Island Population of São Tomé and Príncipe: The Role of the DFNB1 Locus?

    PubMed

    Caroça, Cristina; de Matos, Tiago Morim; Ribeiro, Diogo; Lourenço, Vera; Martins, Tânia; Campelo, Paula; Fialho, Graça; Silva, Susana Nunes; Paço, João; Caria, Helena

    2016-08-01

    Hearing loss (HL) is a common condition with both genetic and environmental causes, and it greatly impacts global health. The prevalence of HL is reportedly higher in developing countries such as the Sub-Saharan African island of São Tomé and Príncipe, where the deaf community is estimated to be less than 1% of the population. We investigated the role of the DFNB1 locus (GJB2 and GJB6 genes) in the etiology of nonsyndromic sensorineural hearing loss (NSSHL) in São Tomé and Príncipe. A sample of 316 individuals, comprising 136 NSSHL patients (92 bilateral, 44 unilateral) and 180 controls, underwent a clinical and audiological examination. Sequencing of the GJB2 coding region and testing for the (GJB6-D13S1830) and del(GJB6-D13S1854) GJB6 deletions were performed. A total of 311 out of 316 individuals were successfully analyzed regarding the GJB2 and GJB6 genetic variations, respectively. The frequency of the GJB2 coding mutations in patients and controls was low. Some of those coding mutations are the most commonly found in Eurasian and Mediterranean populations and have also been identified in Portugal. None of the GJB6 deletions was present. The presence of certain coding variants in São Tomé and Príncipe suggests a non-Sub-Saharan genetic influx and supports the previously reported genetic influx from European (mainly Portuguese) ancestors. In summary, DFNB1 locus does not appear to be a major contributor to NSSHL in São Tomé and Príncipe. However, the presence of both pathogenic and likely pathogenic mutations in GJB2 suggests that GJB2-related NSSHL might still occur in this population, warranting further research on GJB2 testing in NSSHL cases. PMID:27501294

  14. PECONPI: a novel software for uncovering pathogenic copy number variations in non-syndromic sensorineural hearing loss and other genetically heterogeneous disorders.

    PubMed

    Tsai, Ellen A; Berman, Micah A; Conlin, Laura K; Rehm, Heidi L; Francey, Lauren J; Deardorff, Matthew A; Holst, Jenelle; Kaur, Maninder; Gallant, Emily; Clark, Dinah M; Glessner, Joseph T; Jensen, Shane T; Grant, Struan F A; Gruber, Peter J; Hakonarson, Hakon; Spinner, Nancy B; Krantz, Ian D

    2013-09-01

    This report describes an algorithm developed to predict the pathogenicity of copy number variants (CNVs) in large sample cohorts. CNVs (genomic deletions and duplications) are found in healthy individuals and in individuals with genetic diagnoses, and differentiation of these two classes of CNVs can be challenging and usually requires extensive manual curation. We have developed PECONPI, an algorithm to assess the pathogenicity of CNVs based on gene content and CNV frequency. This software was applied to a large cohort of patients with genetically heterogeneous non-syndromic hearing loss to score and rank each CNV based on its relative pathogenicity. Of 636 individuals tested, we identified the likely underlying etiology of the hearing loss in 14 (2%) of the patients (1 with a homozygous deletion, 7 with a deletion of a known hearing loss gene and a point mutation on the trans allele and 6 with a deletion larger than 1 Mb). We also identified two probands with smaller deletions encompassing genes that may be functionally related to their hearing loss. The ability of PECONPI to determine the pathogenicity of CNVs was tested on a second genetically heterogeneous cohort with congenital heart defects (CHDs). It successfully identified a likely etiology in 6 of 355 individuals (2%). We believe this tool is useful for researchers with large genetically heterogeneous cohorts to help identify known pathogenic causes and novel disease genes.

  15. Progressive Hearing Impairment in Children with Congenital Cytomegalovirus Infection.

    ERIC Educational Resources Information Center

    Dahle, Arthur J.; And Others

    1979-01-01

    Audiological assessment of 86 children (mean age 38 months at last evaluation time) with congenital cytomegalovirus infection revealed progressive hearing loss in four of 12 Ss with sensorineural hearing impairments. Case descriptions documented the progression of the hearing loss. (Author)

  16. [New recurrent extended deletion, including GJB2 and GJB6 genes, results in isolated sensorineural hearing impairment with autosomal recessive type of inheritance].

    PubMed

    Bliznets, E A; Makienko, O N; Okuneva, E G; Markova, T G; Poliakov, A V

    2014-04-01

    Hereditary hearing loss with the autosomal recessive type of inheritance of the DFNB 1 genetic type, caused by mutations in the GJB2 gene, is the main reason of innate non-syndromal hearing impairment in most developed countries of the world (including Russia). Intragenic point mutations prevail among the GJB2 gene defectors; however, extended deletions in the DFNB1 locus are also found with considerable frequency in some populations (for example, Spain, Great Britain, France, United States, and Brazil). Among the four known extended deletions, only one deletion affects directly the GJB2 gene sequence and was described in a single family. A new extended deletion in the GJB2 and GJB6 gene sequences (approximately 101 kb in size; NC_000013.10:g.20,757,021_20,858,394del), detected in three unrelated Russian patients, was described and characterized. Ingush origin of this mutation is assumed. If the new deletion is frequent, its detection is very important for the genetic consulting of families with hereditary hearing impairment. PMID:25715449

  17. Can low frequency sound stimulation during posturography help diagnosing possible perilymphatic fistula in patients with sensorineural hearing loss and/or vertigo?

    PubMed

    Selmani, Z; Ishizaki, H; Pyykkö, I

    2004-03-01

    Perilymphatic fistula (PLF) is often difficult to diagnose because of the similar symptomatology, such as vertigo, tinnitus and hearing loss, which is found in several inner ear diseases. We attempted to correlate a positive result of low frequency sound (LFS) stimulation tests in posturography with the presence or absence of a PLF confirmed by transtympanic endoscopy in 209 patients with various inner ear diseases (Meniere's disease ( n=128), vestibulopathy ( n=41), cochleopathy ( n=28) and sudden deafness ( n=12). LFS provoked unsteadiness in posturography without PLF in 24 patients with Meniere's disease, in 5 patients with vestibulopathy, in 3 patients with cochleopathy and in 2 patients with sudden deafness. In one patient, tympanoscopy revealed fistula in the round window membrane that was covered with a fibrinous layer. In four cases there was abnormal light reflex in the round window but without PLF. In eight cases, Hennebert's sign was present with nystagmus, without PLF. We conclude that pathological responses to the LFS test in posturography can also be encountered in other inner ear diseases without PLF.

  18. 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…

  19. 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…

  20. The Syndrome of Familial Hypoparathyroidism, Sensorineural Deafness and Renal Dysplasia.

    PubMed

    Meena, Ratti Lal; Maloo, Sudheer Kumar; Samar, Neera; Ruhela, Asim; Saini, Subhash

    2015-06-01

    The syndrome of familial hypoparathyroidism, sensorineural deafness, and renal dysplasia (HDR syndrome) is inherited as an autosomal dominant trait, caused by haploinsufficiency of the GATA3 gene in chromosome 10p. Although first described years ago, but the disease is considered to be very rare. Patients usually present with hypocalcemia, tetany, or afebrile convulsions at any age. Hearing loss is usually bilateral, range from mild to profound impairment. Renal disease includes dysplasia, hypoplasia or aplasia.

  1. [Hereditary hearing loss: genetic counselling].

    PubMed

    Cabanillas Farpón, Rubén; Cadiñanos Bañales, Juan

    2012-01-01

    The aim of this review is to provide an updated overview of hereditary hearing loss, with special attention to the etiological diagnosis of sensorineural hearing loss, the genes most frequently mutated in our environment, the techniques available for their analysis and the clinical implications of genetic diagnosis. More than 60% of childhood sensorineural hearing loss is genetic. In adults, the percentage of hereditary hearing loss is unknown. Genetic testing is the highest yielding test for evaluating patients with sensorineural hearing loss. The process of genetic counselling is intended to inform patients and their families of the medical, psychological and familial implications of genetic diseases, as well as the risks, benefits and limitations of genetic testing. The implementation of any genetic analysis must be always preceded by an appropriate genetic counselling process.

  2. [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.

  3. Hearing loss in Australian divers.

    PubMed

    Edmonds, C; Freeman, P

    1985-11-11

    Permanent hearing loss of the sensorineural type has been demonstrated to be an occupational hazard of professional SCUBA divers. An audiometric survey was performed on a group of professional abalone divers, all of whom had experienced excessive exposure to dysbaric conditions. The results of this survey revealed that, even allowing for the very liberal requirements of the Australian Standard for divers, over 60% had unacceptable sensorineural, high frequency deafness. In half these cases deafness was unilateral, and in half bilateral. Making allowance for age, two-thirds had hearing loss to a degree which is compensable, according to the method of the National Acoustic Laboratories (1974) for determining proportional loss of hearing.

  4. 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,…

  5. 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…

  6. [Presbycusis - Age Related Hearing Loss].

    PubMed

    Fischer, N; Weber, B; Riechelmann, H

    2016-07-01

    Presbycusis or age related hearing loss can be defined as a progressive, bilateral and symmetrical sensorineural hearing loss due to age related degeneration of inner ear structures. It can be considered a multifactorial complex disorder with environmental and genetic factors. The molecular, electrophysiological and histological damage at different levels of the inner ear cause a progressive hearing loss, which usually affects the high frequencies of hearing. The resulting poor speech recognition has a negative impact on cognitive, emotional and social function in older adults. Recent investigations revealed an association between hearing impairment and social isolation, anxiety, depression and cognitive decline in elderly. These findings emphasize the importance of diagnosis and treating hearing loss in the elderly population. Hearing aids are the most commonly used devices for treating presbycusis. The technical progress of implantable hearing devices allows an effective hearing rehabilitation even in elderly with severe hearing loss. However, most people with hearing impairments are not treated adequately. PMID:27392191

  7. [An update on bone anchored hearing aids].

    PubMed

    Fries, S; Maire, R; Grosjean, P; George, M; Simon, C; Zaugg, Y

    2014-10-01

    Hearing loss represents a hidden handicapwith various repercussions on development and social life. In the majority of cases, classical hearing aids address most hearing losses. However, the enhancement required for severe deafness frequently involves sound distortions which are very uncomfortable for patients. With the advent of bone anchored hearing aids, conductive hearing losses as well as mixed hearing losses are now better rehabilitated. Recently their indications have been expanded to profound to severe sensorineural hearing loss. The emergence of new materials as well as subcutaneous implants has lead to lessen skin complications and has diminished the aesthetic discomfort of this type of hearing devices.

  8. Hearing loss and the Mayer-Rokitansky-Küster-Hauser syndrome.

    PubMed

    Strübbe, E H; Cremers, C W; Dikkers, F G; Willemsen, W N

    1994-05-01

    The hearing of 51 female patients with the Mayer-Rokitansky-Küster-Hauser syndrome was examined using otoscopy and standard audiometry. A unilateral or bilateral hearing loss of more than 15 dB Fletcher index was found in 13 of 51 (25.5%). Four of these 13 patients had a hearing loss of less than 20 dB in the worst ear. The remainder had a hearing loss of at least 30 dB in the worst ear. Five of the 13 patients had pure conductive hearing loss; in four of these five, a congenital origin was accepted. Two of the 13 had mixed hearing loss that was a residual symptom from previous otitis media; six had sensorineural hearing loss. A congenital cause was found in one of these six, based on the fact that she had been deaf and dumb since birth. In one other patient, noise-related deafness was likely (i.e., an acquired cause). In the other four cases in this group, the cause was unknown. The results of this study show that hearing loss is a characteristic associated with the Mayer-Rokitansky-Küster-Hauser syndrome. PMID:8579156

  9. 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.

  10. Perception of Suprasegmental Features of Speech by Children with Cochlear Implants and Children with Hearing Aids

    ERIC Educational Resources Information Center

    Most, Tova; Peled, Miriam

    2007-01-01

    This study assessed perception of suprasegmental features of speech by 30 prelingual children with sensorineural hearing loss. Ten children had cochlear implants (CIs), and 20 children wore hearing aids (HA): 10 with severe hearing loss and 10 with profound hearing loss. Perception of intonation, syllable stress, word emphasis, and word pattern…

  11. Hearing loss in older adults.

    PubMed

    Walling, Anne D; Dickson, Gretchen M

    2012-06-15

    Hearing loss affects approximately one-third of adults 61 to 70 years of age and more than 80 percent of those older than 85 years. Men usually experience greater hearing loss and have earlier onset compared with women. The most common type is age-related hearing loss; however, many conditions can interfere with the conduction of sound vibrations to the inner ear and their conversion to electrical impulses for conduction to the brain. Screening for hearing loss is recommended in adults older than 50 to 60 years. Office screening tests include the whispered voice test and audioscopy. Older patients who admit to having difficulty hearing may be referred directly for audiometry. The history can identify risk factors for hearing loss, especially noise exposure and use of ototoxic medications. Examination of the auditory canal and tympanic membrane can identify causes of conductive hearing loss. Audiometric testing is required to confirm hearing loss. Adults presenting with idiopathic sudden sensorineural hearing loss should be referred for urgent assessment. Management of hearing loss is based on addressing underlying causes, especially obstructions (including cerumen) and ototoxic medications. Residual hearing should be optimized by use of hearing aids, assistive listening devices, and rehabilitation programs. Surgical implants are indicated for selected patients. Major barriers to improved hearing in older adults include lack of recognition of hearing loss; perception that hearing loss is a normal part of aging or is not amenable to treatment; and patient nonadherence with hearing aids because of stigma, cost, inconvenience, disappointing initial results, or other factors.

  12. A Comparison of Psychometric Functions for Detection in Normal-Hearing and Hearing-Impaired Listeners.

    ERIC Educational Resources Information Center

    Arehart, Kathryn Hoberg; And Others

    1990-01-01

    Psychometric functions (PFD) for the detection of pure tones were obtained from 10 adolescent and young adult listeners with normal hearing and 10 adult listeners with sensorineural impairments of presumed cochlear origin. The slopes of the PFDs were abnormally steep in some of the hearing-impaired listeners but were statistically significant only…

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

    PubMed Central

    Ahmed, Mohi; Ura, Kiyoe; Streit, Andrea

    2015-01-01

    ABSTRACT WHSC1 is a histone methyltransferase (HMT) that catalyses the addition of methyl groups to lysine 36 on histone 3. In humans, WHSC1 haploinsufficiency is associated with all known cases of Wolf-Hirschhorn syndrome (WHS). The cardinal feature of WHS is a craniofacial dysmorphism, which is accompanied by sensorineural hearing loss in 15% of individuals with WHS. Here, we show that WHSC1-deficient mice display craniofacial defects that overlap with WHS, including cochlea anomalies. Although auditory hair cells are specified normally, their stereocilia hair bundles required for sound perception fail to develop the appropriate morphology. Furthermore, the orientation and cellular organisation of cochlear hair cells and their innervation are defective. These findings identify, for the first time, the likely cause of sensorineural hearing loss in individuals with WHS. PMID:26092122

  15. Anosmia associated with hearing loss and benign positional vertigo after head trauma.

    PubMed

    Ottaviano, G; Marioni, G; Marchese-Ragona, R; Trevisan, C P; De Filippis, C; Staffieri, A

    2009-10-01

    It is well known that head trauma may cause hearing loss, which can be either conductive or sensorineural. Benign paroxysmal positional vertigo and olfactory dysfunction due to head trauma are also well known. The association between sensorineural hearing loss and anosmia, following head trauma, is extremely rare. Two rare cases of post-traumatic occurrence of hearing loss, olfactory dysfunction and benign positional vertigo are reported and the pathophysiology of the association between sensorineural hearing loss, anosmia and benign paroxysmal positional vertigo, after head injury, are briefly discussed. ENT specialists should, in the authors' opinion, be aware of the possible association between anosmia, sensorineural hearing loss and benign paroxysmal positional vertigo after head injury, even in the absence of skull fracture.

  16. 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…

  17. Sensorineural deafness due to compression chamber noise.

    PubMed

    Hughes, K B

    1976-05-01

    A case of unilateral sensorineural deafness following exposure to compression chamber noise is described. A review of the current literature concerning the otological hazards of compression chambers is made. The possible pathological basis is discussed.

  18. Bilateral sudden hearing loss following habitual abortion: a case report and review of literature.

    PubMed

    Yin, Tuanfang; Huang, Fengying; Ren, Jihao; Liu, Wei; Chen, Xing; Li, Lihua; Xie, Dinghua; Lu, Yongde

    2013-01-01

    Sudden sensorineural hearing loss (SSNHL) is usually unilateral and can be associated with tinnitus and vertigo. The most common causes of this disease are known to be the vascular and viral agents, but immune disorders are involved in the development of sudden deafness. The antiphospholipid syndrome (APS) is an acquired autoimmune system disorder, which is defined as the presence of antiphospholipid antibodies (APA) in the patient's blood, then cause venous and/or arterial thrombosis in various organs of the body, for example, thrombosis can occur in the placenta and/or the inner ear. As a result, it can cause abortion and/or sudden deafness. Bilateral SSNHL following habitual abortion is a rare clinical event. Here, we report a case of 32-year-old woman who presented with bilateral sudden hearing loss following recurrent pregnancy loss (RPL) as the first manifestation of primary antiphospholipid syndrome. Combine the literature, the diagnosis, clinical implication and treatment are discussed.

  19. Iodine deficiency, thyroid function and hearing deficit: a review.

    PubMed

    Melse-Boonstra, Alida; Mackenzie, Ian

    2013-12-01

    Iodine deficiency affects an estimated 241 million school-aged children in the world. Little is known about iodine deficiency in relation to auditory function, except for the fact that deaf-mutism is one of the features of cretinism. In the present review, we documented the scientific knowledge on the role of iodine and hypothyroidism in the auditory system. We found that ear development and hearing function depend on thyroid hormones. Multiple pathways are involved in this, including both inner ear morphology as well as neurological processes. Conductive as well as sensorineural hearing loss is found in studies with animals that were rendered hypothyroidic. In humans, auditory impairment is reported frequently in relation to hypothyroidism, ranging from mild disturbances to severe handicap. Auditory impairment has been related more explicitly to congenital hypothyroidism than to acquired hypothyroidism. The critical period for thyroid function-related hearing maturation is the first and second trimesters of pregnancy. Although only a limited number of studies have directly investigated the relationship between iodine deficiency and auditory function, most studies point toward an association. However, evidence from good randomised controlled trials is lacking. Inclusion of auditory outcomes in iodine supplementation studies is therefore to be recommended, especially for trials in pregnancy. Hearing deficit is an invisible abnormality, but has major consequences for educational and social skills if not detected. In view of this, auditory impairment should be mapped in iodine-deficient areas in order to realistically estimate the magnitude of the problem.

  20. Hear, Hear!

    ERIC Educational Resources Information Center

    Rittner-Heir, Robbin

    2000-01-01

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

  1. 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).

  2. [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.

  3. 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

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

    ERIC Educational Resources Information Center

    Kahn, Gerri; Goldman, Ellen

    1991-01-01

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

  5. Temporary Threshold Shift Caused by Hearing Aid Use.

    ERIC Educational Resources Information Center

    Macrae, John H.

    1993-01-01

    Temporary threshold shift over a wide range of frequencies was found after 4 hours of hearing aid use by a 15-year-old student with severe sensorineural hearing loss who was using real-ear insertion gains 10 to 20 decibels greater than those recommended by current standards. Less gain was recommended at frequencies from 500 to 1500 hertz.…

  6. A Young Man With Progressive Vision and Hearing Loss.

    PubMed

    Kung, Nathan H; Bucelli, Robert C; Van Stavern, Renee B; Goebel, Joel A; Van Stavern, Gregory P

    2016-07-01

    A 37-year-old man with a history of progressive bilateral sensorineural hearing loss presented to a neuro-ophthalmology clinic with an acute left homonymous hemianopsia. In this article, we discuss the clinical approach and differential diagnosis of progressive combined vision and hearing loss and guide the reader to discover the patient's ultimate diagnosis. PMID:27213952

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

    ERIC Educational Resources Information Center

    Alexander, Joshua M.; Kluender, Keith R.

    2009-01-01

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

  8. Speech Recognition Threshold in Noise: Effects of Hearing Loss, Frequency Response, and Speech Materials.

    ERIC Educational Resources Information Center

    Van Tasell, Dianne J.; Yanz, Jerry L.

    1987-01-01

    Speech recognition threshold (SRT) was measured in quiet and in noise for normal hearing subjects and subjects with high frequency sensorineural hearing loss. The speed, reliability, and apparent sensitivity of the SRT to frequency response characteristics make it a potentially useful tool for hearing aid evaluation. (Author/DB)

  9. [Acute unilateral deafness and contralateral hearing loss following inguinal hernia repair under controlled anesthesia].

    PubMed

    Constantinidis, J; Mertzlufft, F; Steinhart, H

    1999-10-01

    Acute hearing loss following non-otologic surgery and general anesthesia is a rare occurrence. Deafness following anesthesia has more commonly been associated with spinal anesthesia or cardiopulmonary bypass surgical procedures. We present a case with unilateral cochlear dysfunction and sensorineural hearing loss after inguinal hernia operation. The literature is reviewed and the mechanisms causing hearing loss during anesthesia are discussed.

  10. 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…

  11. Congenital and acquired perilymph fistula: review of the literature.

    PubMed

    Roman, S; Bourliere-Najean, B; Triglia, J M

    1998-08-01

    Perilymph fistula is caused by an abnormal communication between the perilymph space and the middle ear. The etiology is either congenital or acquired. The congenital fistula can be associated or not with clinical symptoms or radiologically detectable abnormalities of the temporal bone. In patients presenting congenital fistula without symptoms or radiologically detectable abnormalities, little malformations of the middle ear may be detected during surgery. The acquired fistula can be caused by iatrogenic trauma, physical injury or erosion. As far as therapy is concerned, surgical treatment can be performed and the perilymph fistula thus represents one of the few causes of sensorineural hearing loss that can be treated surgically. However, the main challenge is the identification of those patients that need to undergo an exploratory tympanotomy, since there are no clinical-audiologic symptoms or radiographic indicators that can be considered pathognomonic of perilymph fistula. The aim of this review of the literature is to define the guidelines for preoperative diagnosis to indicate exploratory tympanotomy both in children and in adults. On the basis of our results, exploratory tympanotomy should be performed in patients with vertigo and/or progressive, sudden or fluctuating hearing loss in association with one or more than one of the following elements: a history of cranial trauma, radiographically detectable abnormalities of the inner ear, congenital malformations of the head, recurring meningitis, positive fistula test. The surgical treatment consists in placing a graft of temporalis fascia or tragal perichondrium and it usually results in a significant improvement of vestibular symptoms and sometimes of the hearing function as well.

  12. 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...

  13. 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...

  14. 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...

  15. 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

  16. 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

  17. 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.

  18. [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.

  19. 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

  20. Hearing loss in older adults.

    PubMed

    Walling, Anne D; Dickson, Gretchen M

    2012-06-15

    Hearing loss affects approximately one-third of adults 61 to 70 years of age and more than 80 percent of those older than 85 years. Men usually experience greater hearing loss and have earlier onset compared with women. The most common type is age-related hearing loss; however, many conditions can interfere with the conduction of sound vibrations to the inner ear and their conversion to electrical impulses for conduction to the brain. Screening for hearing loss is recommended in adults older than 50 to 60 years. Office screening tests include the whispered voice test and audioscopy. Older patients who admit to having difficulty hearing may be referred directly for audiometry. The history can identify risk factors for hearing loss, especially noise exposure and use of ototoxic medications. Examination of the auditory canal and tympanic membrane can identify causes of conductive hearing loss. Audiometric testing is required to confirm hearing loss. Adults presenting with idiopathic sudden sensorineural hearing loss should be referred for urgent assessment. Management of hearing loss is based on addressing underlying causes, especially obstructions (including cerumen) and ototoxic medications. Residual hearing should be optimized by use of hearing aids, assistive listening devices, and rehabilitation programs. Surgical implants are indicated for selected patients. Major barriers to improved hearing in older adults include lack of recognition of hearing loss; perception that hearing loss is a normal part of aging or is not amenable to treatment; and patient nonadherence with hearing aids because of stigma, cost, inconvenience, disappointing initial results, or other factors. PMID:22962895

  1. Vici syndrome associated with sensorineural hearing loss and laryngomalacia.

    PubMed

    Ozkale, Murat; Erol, Ilknur; Gümüş, Ayten; Ozkale, Yasemin; Alehan, Füsun

    2012-11-01

    The phenotypically heterogeneous, autosomal recessive Vici syndrome was first described in 1988 in a sister and brother with oculocutaneous albinism, agenesis of the corpus callosum, cataract, cardiomyopathy, cleft lip, and immunodeficiency. Only 14 cases of Vici syndrome have yet been reported, several involving morphologic and functional defects in addition to those described in the initial case. We report on a 3-month-old Turkish girl with Vici syndrome associated with laryngomalacia, further expanding the clinical spectrum. We also review clinical features in all 15 Vici syndrome patients, to distinguish general from less common signs. To the best of our knowledge, this report is the first of a Turkish patient with Vici syndrome. PMID:23044023

  2. A model of peripherally developing hearing loss and tinnitus based on the role of hypoxia and ischemia.

    PubMed

    Mazurek, Birgit; Haupt, Heidemarie; Georgiewa, Petra; Klapp, Burghard F; Reisshauer, Anett

    2006-01-01

    The incidence of sensorineural hearing loss often caused by direct damage to the cochlear hair cells is by far more frequent and more serious than disorders affecting the external ear or the middle ear. Mechanisms that are discussed to be relevant for the genesis of tinnitus and acquired hearing impairment are hair cell loss, signal transduction disturbances in the region of the outer and inner hair cells and the spiral ganglion, impairment of cochlear blood flow, mechanical disturbance, and hypoxia and ischemia. The present model surveys the possible cellular and molecular biological causes of peripherally developing hearing loss and tinnitus. In particular, the paper discusses the roles of hypoxia and ischemia in the cochlea and in the etiology of the neurosensory types of tinnitus. Peripheral origins of hearing disturbances and tinnitus may be: (a) damage to the stereocilia and the tip links, (b) dysfunction of potassium channels or (c) modification of the glutamate release. Moreover, the hypoxia inducible factor-1 may have an important role to play as a key transcription factor in the cells' adaptation to hypoxia and ischemia. An impairment of the cochlear blood flow may be induced by the expression of target genes like nitrogen monoxide synthase and endothelin-1 resulting in tinnitus. The paper discusses consequences resulting from the present model for the medical treatment of peripherally developing tinnitus and hearing loss.

  3. 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…

  4. The Effects of High-Frequency Amplification on the Objective and Subjective Performance of Hearing Instrument Users with Varying Degrees of High-Frequency Hearing Loss

    ERIC Educational Resources Information Center

    Plyler, Patrick N.; Fleck, Erica L.

    2006-01-01

    Purpose: The purpose of the present study was to determine if amplifying beyond 2 kHz affected the objective and subjective performance of hearing instrument users with varying degrees of mild-to-severe high-frequency sensorineural hearing loss. Method: Twenty participants were fitted binaurally with digital completely-in-the-canal devices with…

  5. Hearing loss in diving--a study amongst Navy divers.

    PubMed

    Zulkaflay, A R; Saim, L; Said, H; Mukari, S Z; Esa, R

    1996-03-01

    Despite the commonly observed condition of middle and inner ear barotrauma among divers, there is little evidence of insidious and permanent development of sensorineural hearing loss associated with diving. An audiometric survey was performed on a group of 120 divers and 166 non divers from the Royal Malaysian Naval Base in Lumut, West Malaysia between July to December 1991. The results of this survey revealed that insidious development of high frequency sensorineural hearing loss may be associated with diving. At frequencies 4000, 6000 and 8000 Hz the divers had higher mean hearing levels than non divers and their hearing at those frequencies seemed to deteriorate faster. The etiology of this insidious hearing loss is multifactorial and may be related to inner ear barotrauma, decompression sickness or noise-induced deafness.

  6. 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…

  7. Audiometry and other hearing tests.

    PubMed

    Davies, R A

    2016-01-01

    Hearing tests of the peripheral auditory system are well established and the pure-tone audiogram is generally regarded as the screening test of choice in adults. It allows the distinction to be made between conductive, i.e., outer- and middle-ear, and sensorineural, i.e., cochlear, hearing loss, and also to describe the configuration of the hearing thresholds in terms of severity and the frequency affected. Electrophysiologic testing with auditory potentials, e.g., the auditory brainstem response, can identify sites of lesion in the eighth nerve, brainstem, and more centrally. However, it is only in the last two decades that a battery of central auditory tests has been established that can probe the central pathways in more details, i.e., when the pure-tone audiogram may be normal, and yet the patient still has symptoms of hearing dysfunction. PMID:27638069

  8. The Envoy Esteem implantable hearing system.

    PubMed

    Marzo, Sam J; Sappington, Joshua M; Shohet, Jack A

    2014-12-01

    This article discusses the Envoy Esteem implantable hearing system, a completely implantable hearing device. The device is indicated for patients older than 18 years with stable moderate to severe sensorineural hearing loss and good speech discrimination. The device is placed through an intact canal wall tympanomastoidectomy with a wide facial recess approach. The implant is typically activated at 6 to 8 weeks postoperatively and usually requires several adjustments for optimal performance. The sound processor/battery lasts 4.5 to 9.0 years and can be replaced through a minor outpatient procedure.

  9. [Acute perceptive hearing loss. Importance of tuning fork test in primary care].

    PubMed

    Verburg, A F E Arianne; Alkhateeb, W H F Waiel; Merkus, Paul

    2011-01-01

    A 56-year-old woman presented with acute right-sided hearing loss. At first presentation she was diagnosed as having otitis media with effusion. No tuning fork test was performed. After four weeks she was finally correctly diagnosed as having a right-sided sensorineural hearing loss of 90 dB. As a result of the delay no treatment was started. Her hearing loss did not show any improvement after three months. Sensorineural hearing loss is a rare, potentially invalidating condition with a considerable psychological impact. The treatment consists of systemic steroids, which may only be useful if started within 14 days after symptoms start. Some patients are initially treated for conductive hearing loss. Routine performance of the tuning fork test helps in differentiating between conductive and perceptive hearing loss. In cases of acute perceptive hearing loss, patients should be referred to the otorhinolaryngologist to exclude possible causes and start treatment and guidance.

  10. Analysis of a Recorded Test for the Measurement of Hearing in Children.

    ERIC Educational Resources Information Center

    Matkin, Noel D.

    To study the feasibility of using filtered environmental sounds as test stimuli to determine the auditory sensitivity of young children, a tape recorded test was prepared using environmental sounds which retain their identity when filtered. Twenty normal-hearing preschoolers and 40 hearing impaired children (20 with flat sensori-neural hearing…

  11. The Reliability and Validity of the Comfort Level Method of Setting Hearing Aid Gain

    ERIC Educational Resources Information Center

    Walden, Brian E.; And Others

    1977-01-01

    Investigated in a series of experiments with 40 adults (20- to 70-years-old) having bilateral sensorineural hearing impairments was the test-retest reliability of the comfort level method for setting the acoustic gain of hearing aids, and the relationship between the comfort settings utilized in more realistic daily listening situations.…

  12. 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…

  13. Hearing Loss

    MedlinePlus

    ... version of this page please turn Javascript on. Hearing Loss What is Hearing Loss? Hearing loss is a common problem caused by ... sec Click to watch this video Types of Hearing Loss Hearing loss comes in many forms. It can ...

  14. Otoacoustic Emissions in an Adult with Severe Hearing Loss.

    ERIC Educational Resources Information Center

    Prieve, Beth A.; And Others

    1991-01-01

    The paper describes the unexpected finding of evoked otoacoustic emissions from one ear of a subject with severe-to-profound bilateral sensorineural hearing loss. It is suggested that the subject may have a group of surviving outer hair cells in some regions of the left cochlea with corresponding inner hair cell or neural damage. (Author/DB)

  15. Vocabulary and Working Memory in Children Fit with Hearing Aids

    ERIC Educational Resources Information Center

    Stiles, Derek J.; McGregor, Karla K.; Bentler, Ruth A.

    2012-01-01

    Purpose: To determine whether children with mild-to-moderately severe sensorineural hearing loss (CHL) present with disturbances in working memory and whether these disturbances relate to the size of their receptive vocabularies. Method: Children 6 to 9 years of age participated. Aspects of working memory were tapped by articulation rate, forward…

  16. 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.

  17. Sudden hearing loss after dental treatment.

    PubMed

    Kansu, Leyla; Yilmaz, Ismail

    2013-08-01

    A 66-year-old man presented with impaired balance, tinnitus, sensation of blockage, and hearing loss in his left ear, which developed after dental treatment for dental pain 4 days previously. Treatment of the carious left upper second molar tooth had included pulp extirpation, canal expansion, and tooth filling under local anesthesia with articaine and epinephrine. Impaired balance decreased spontaneously within 3 days of dental treatment, but tinnitus and hearing loss persisted. Pure tone audiogram showed profound sensorineural hearing loss in the left ear, with a downslope from 40 to 100 dB, and an abnormal speech discrimination score (50%). Treatment included intravenous prednisolone, intratympanic dexamethasone, and oral betahistine and trimetazidine. The patient had improved hearing and resolution of tinnitus. Sudden hearing loss is rare after dental treatment, and awareness of this complication may prompt early referral for treatment and may improve recovery and prognosis.

  18. Sudden hearing loss after dental treatment.

    PubMed

    Kansu, Leyla; Yilmaz, Ismail

    2013-08-01

    A 66-year-old man presented with impaired balance, tinnitus, sensation of blockage, and hearing loss in his left ear, which developed after dental treatment for dental pain 4 days previously. Treatment of the carious left upper second molar tooth had included pulp extirpation, canal expansion, and tooth filling under local anesthesia with articaine and epinephrine. Impaired balance decreased spontaneously within 3 days of dental treatment, but tinnitus and hearing loss persisted. Pure tone audiogram showed profound sensorineural hearing loss in the left ear, with a downslope from 40 to 100 dB, and an abnormal speech discrimination score (50%). Treatment included intravenous prednisolone, intratympanic dexamethasone, and oral betahistine and trimetazidine. The patient had improved hearing and resolution of tinnitus. Sudden hearing loss is rare after dental treatment, and awareness of this complication may prompt early referral for treatment and may improve recovery and prognosis. PMID:23642550

  19. A case of bilateral sudden hearing loss and tinnitus after salicylate intoxication.

    PubMed

    Kim, Sang Min; Jo, Joon-Man; Baek, Moo Jin; Jung, Kyu Hwan

    2013-04-01

    Salicylate, the active ingredient of aspirin can cause sensorineural hearing loss and tinnitus when plasma concentrations reach a critical level. The ototoxic mechanisms of salicylate remain unclear but hearing and tinnitus usually recovers a few days after intoxication. There have been few reports of salicylate-induced ototoxicity in Korea, and the majority is caused by a low dose of aspirin. Herein, we report a case of sudden hearing loss and tinnitus after acute salicylate intoxication and review recent updates on salicylate ototoxicity.

  20. 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…

  1. 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…

  2. Baha solutions for patients with severe mixed hearing loss.

    PubMed

    Flynn, Mark C; Sadeghi, Andre; Halvarsson, Glenn

    2009-01-01

    Patients with a mixed hearing loss present special challenges. The amplification demands of mixed hearing loss can drive powerful digital hearing aids to their limits and introduce distortion through saturation. Conversely, the Baha System effectively bypasses the conductive component and focuses on compensating for the sensorineural component of the hearing loss. Ten patients with a mixed hearing loss participated in the present study. Results indicate that Baha provided significant benefits (p < 0.01) over conventional air conduction hearing instruments across the dimensions of audibility, speech understanding and sound quality. Given the increased output force of the latest Baha instruments, once the conductive component of a severe mixed hearing loss becomes greater than 30 dB, a Baha should be considered and evaluated on audiological grounds alone to provide optimal amplification. PMID:19195004

  3. Can self assessment of communication predict hearing loss?

    PubMed

    Vij, S; Nagarkar, A N; Jindal, P

    2007-05-01

    A total of 120 subjects with hearing loss (75 men, 45 women), within the age range 18-70 years (mean, 38 years), and 15 normal subjects were administered a modified Hindi adaptation of the 'self assessment of communication' hearing loss inventory. The study aimed to determine whether there was any correlation between subjects' average pure tone thresholds and their inventory scores. Data was analysed using the Pearson coefficient of correlation and regression analysis. A negative correlation was obtained stating that the greater the hearing loss, the lower the inventory score. An equation could also be derived for the bilateral symmetrical sensorineural hearing loss group and the bilateral symmetrical conductive hearing loss group to enable calculation of patients' average hearing loss from their inventory scores, in the absence of an audiogram. This could aid rehabilitation in cases with either type of hearing loss (in which no medical intervention was required) when pure tone audiometry is not possible.

  4. 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

  5. Connexin 26 mutations in cases of sensorineural deafness in eastern Austria.

    PubMed

    Frei, Klemens; Szuhai, Károly; Lucas, Trevor; Weipoltshammer, Klara; Schöfer, Christian; Ramsebner, Reinhard; Baumgartner, Wolf-Dieter; Raap, Anton K; Bittner, Reginald; Wachtler, Franz J; Kirschhofer, Karin

    2002-07-01

    Mutations in the connexin 26 (Cx26) gene (GJB2) are associated with autosomal nonsyndromic sensorineural hearing loss. This study describes mutations in the Cx26 gene in cases of familial and sporadic hearing loss (HL) by gene sequencing and identifies the allelic frequency of the most common mutation leading to HL (35delG) in the population of eastern Austria. For this purpose we have developed and applied a molecular beacon based real-time mutation detection assay. Mutation frequencies in the Cx26 gene of individuals from affected families (14 out of 46) and sporadic cases (11 out of 40) were 30.4% and 27.5%, respectively. In addition to known disease related alterations, a novel mutation 262 G-->T (A88S) was also identified. 35delG accounted for almost 77% of all Cx26 mutations detected and displayed an allelic frequency in the normal hearing population of 1.7% (2 out of 120). The high prevalence of the 35delG mutation in eastern Austria would therefore allow screening of individuals and family members with Cx26 dependent deafness by a highly specific and semi-automated method.

  6. 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

  7. [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. PMID:27038034

  8. [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.

  9. The Esteem System: a totally implantable hearing device.

    PubMed

    Maurer, J; Savvas, E

    2010-01-01

    The Esteem totally implantable active middle ear implant is a new technology to augment hearing in patients suffering from moderate-to-severe and severe sensorineural hearing loss. In contrast to conventional (acoustic) hearing aids, the system uses two piezoelectric transducers (PZTs). PZTs are used as the sensor and driver to replace the function of the middle ear. Sound is received via a PZT sensor that picks up eardrum vibrations, following the piezoelectric principle, and transforms them into an electric signal. This signal is filtered, modified, amplified and transferred to a PZT driver, which mechanically drives the stapes and thereby the inner ear. The sound processor also contains a power source, which is an implantable lithium iodide battery. All components of the hearing restoration system are totally implantable to offer good sound fidelity and reduce hearing aid stigma caused by the visibility of conventional and semi-implantable hearing systems. Our experience shows that this system can provide considerable benefit to patients with sensorineural hearing loss.

  10. Effects of hearing loss on the voice in children.

    PubMed

    Bolfan-Stosic, Natalija; Simunjak, Boris

    2007-04-01

    The object of this paper is to report on preliminary acoustic characteristics obtained from a group of 10 to 12 year old males from special institution from Zagreb with more than mild sensorineural hearing losses. The study was structured as an investigation of voice and resonance characteristics of Croatian children with and without sensorineural hearing loss, using sustained phonation of the vowel /a/ which was recorded using a high-quality tape recorder carried out by two voice clinicians. The samples were digitized and analyzed for frequency and spectral characteristics by EZVoice and Bruel & Kjaer Real-time Frequency Analyzer and high quality sound level meter (mouth-to-microphone distance = 30 cm). Differences were observed in perturbation measures; F0 variability; vocal intensity. Spectral deviations were also observed. Discussion focuses on application of these findings by Croatian speech and hearing specialists with the hearing impaired population. Results indicated the following: measures of jitter were significantly elevated in the hearing loss group as compared to the normal controls. A similar result was observed for measures of shimmer. Lack of voice professional's awareness of importance for making pleasant voice quality of hearing-impaired individuals was the initial idea of this study. Patients with hearing losses have been reported to show a wide variety of voice disturbances.

  11. Hearing Problems

    MedlinePlus

    ... This flow chart will help direct you if hearing loss is a problem for you or a family ... may damage the inner ear. This kind of hearing loss is called OCCUPATIONAL. Prevent occupational hearing loss by ...

  12. 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 ...

  13. The Right Not To Hear: The Ethics of Parental Refusal of Hearing Rehabilitation

    PubMed Central

    Byrd, Serena; Shuman, Andrew G.; Kileny, Sharon; Kileny, Paul R.

    2015-01-01

    Objective To explore the ethics of parental refusal of auditory-oral hearing rehabilitation. Study Design Case study with medical ethical discussion and review. Methods Two young brothers present with severe-to-profound congenital sensorineural hearing loss. The parents, both of whom have normal hearing and work as sign language interpreters, have decided to raise their children with American Sign Language as their only form of communication. They have chosen not to pursue cochlear implantation nor support the use of hearing aids. Discussion This case raises significant questions concerning whether hearing rehabilitation should be mandated, and if there are circumstances in which parental preferences should be questioned or overridden with regard to this issue. In addition, legal concerns may be raised regarding the possible need to file a report with child protective services. Although similar cases involving the Deaf community have historically favored parental rights to forego hearing rehabilitation with either cochlear implantation or hearing aids, we explore whether conclusions should be different because the parents in this case are not hearing impaired. Conclusions The ethics of parental rights to refuse hearing augmentation are complex and strikingly context-dependent. A comprehensive appreciation of the medical, practical and legal issues is crucial prior to intervening in such challenging situations. PMID:21792972

  14. 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

  15. 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…

  16. [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. PMID:25734302

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

    PubMed

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

    2016-04-01

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

  18. [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.

  19. 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. PMID:24953884

  20. Acquired lymphangiectasis.

    PubMed

    Celis, A V; Gaughf, C N; Sangueza, O P; Gourdin, F W

    1999-01-01

    Acquired lymphangiectasis is a dilatation of lymphatic vessels that can result as a complication of surgical intervention and radiation therapy for malignancy. Acquired lymphangiectasis shares clinical and histologic features with the congenital lesion, lymphangioma circumscriptum. Diagnosis and treatment of these vesiculo-bullous lesions is important because they may be associated with pain, chronic drainage, and cellulitis. We describe two patients who had these lesions after treatment for cancer and review the pertinent literature. Although a number of treatment options are available, we have found CO2 laser ablation particularly effective. PMID:9932832

  1. [Application of the middle ear implant in case of high frequency hearing loss--case study].

    PubMed

    Skarzyński, Henryk; Obrycka, Anita; Piotrowska, Anna; Lorens, Artur

    2008-01-01

    People who suffer from hearing impairment complain mainly of a problem with communication. Wearing hearing aids can often compensate some problems related with moderate to serve sensorineural hearing loss. Many of hearing aids users complain of some problems associated with plugging the ear canal, such as feedback, unpleasant sound while eating, unnatural sound of their own voice, physical discomfort. The alternative method for some group of patients with sensorineural loss is the middle ear implant. In the middle ear implant the sound is converted into mechanical vibrations that directly drive the ossicular chain inside the middle ear. It leaves the ear canal open and ear drum undisturbed. The sound quality is improved and residual hearing is preserved. Additionally feedback is reduced while comfort improved. 53-year-old man with bilateral sensorineural hearing loss (mild at low frequencies up to 1000 Hz in combination with serve degree at high frequencies) was implanted with Vibrant Soundbridge system. Tests of speech comprehension in quiet were performed using monosyllabic word lists. Improvement in patienfs score was observed while testing with Vibrant Soundbridge. Patient also reports significant improvement in subjective assessment. Application of the Floating Mass Transducer for sound processing results in significant improvement in sound quality. Results obtained indicate a high level of benefits with the Yibrant Soundbridge.

  2. Cochlear implantation: a biomechanical prosthesis for hearing loss.

    PubMed

    Yawn, Robert; Hunter, Jacob B; Sweeney, Alex D; Bennett, Marc L

    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.

  3. Fluctuating hearing loss, episodic headache, and stroke with platelet hyperaggregability: coexistence of auditory neuropathy and cochlear hearing loss.

    PubMed

    Nobutoki, Tatsuro; Sasaki, Masayuki; Fukumizu, Michio; Hanaoka, Shigeru; Sugai, Kenji; Anzai, Yuki; Kaga, Makiko

    2006-01-01

    We encountered a 10-year-old girl with fluctuating sensorineural hearing loss, episodic headache, and white matter stroke. Strenuous exercise, febrile illness, and general anesthesia all temporarily worsened hearing. Audiologic findings were asymmetric: left-sided retrocochlear dysfunction consistent with auditory neuropathy contrasted with cochlear hearing loss in the right ear. Platelets obtained during a headache-free period showed excessive responsiveness to collagen in vitro, while episodic elevations of thromboxane B(2) and thrombin-antithrombin III complex were noted in blood sampled during headache. Treatment of hyperaggregability of platelets with aspirin and antioxidant vitamins relieved headache, while adenosine triphosphate administration improved hearing thresholds. In this patient, hearing impairment and white matter strokes appeared to respectively related to impaired blood flow to the cochlea and white matter caused by platelet dysfunction triggered by physiologic stresses.

  4. 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.

  5. 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

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

    PubMed

    John, Andrew B; Kreisman, Brian M; Pallett, Stephen

    2012-01-01

    Worker's compensation for hearing loss caused by occupational noise exposure is calculated by varying methods, from state to state within the United States (US), with many employing arithmetic formulas based on the pure-tone audiogram, to quantify hearing loss. Several assumptions unsupported or weakly supported by empirical data underlie these formulas. The present study evaluated the ability of various arithmetic hearing impairment calculations to predict a self-reported hearing handicap in a sample of presenting with sensorineural hearing loss. 204 adults (127 male, 77 female) ranging in age from 18 to 94 served as participants. The sample was selected to exclude patients who had been referred for hearing testing for a medicolegal examination or a hearing conservation appointment. A hearing handicap was measured by the Hearing Handicap Inventory for Adults/for the Elderly (HHIA/E). The covariance analysis of linear structural equations was used to assess the relative strength of correlation with the HHIA/E score among the six formulas and various forms of pure-tone average. The results revealed that all the hearing impairment calculations examined were significantly, but weakly, correlated with the self-reported hearing impairment scores. No significant differences among the predictive abilities of the impairment calculations were evident; however, the average binaural impairment assigned differed significantly among the six calculations examined. Individuals who demonstrated 0% impairment had significantly lower (i.e., better) HHIA/E scores compared to those with non-zero impairment for each formula. These results supported the idea that audiometric data provided an insufficient explanation for real-world hearing difficulties. PMID:22387708

  7. 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. PMID:26611684

  8. 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

  9. Hearing loss with frequent diving (deaf divers).

    PubMed

    Edmonds, C

    1985-09-01

    An audiometric survey was performed on a group of professional abalone divers, all of whom had experienced a great deal of exposure to dysbaric conditions. The results of this survey revealed that, even allowing for the very liberal requirements of the Australian standards for divers, over 60% had an unacceptable sensorineural, high frequency deafness. In half of these cases it was unilateral, and half bilateral. Making allowance for age, over 70% had evidence of hearing loss to a degree considered by the National Acoustic Laboratories to be compensatable.

  10. Towards understanding the specifics of cochlear hearing loss: a modelling approach.

    PubMed

    Stenfelt, Stefan

    2008-11-01

    It is well known that two patients suffering from a sensorineural hearing loss with similar audiograms can benefit significantly differently from amplified hearing even if the same settings of the hearing aids are used. The origin of this problem is complex but one part can be caused by the diagnosis itself; all inner-ear hearing losses are assumed similar. Such hypothesis is a simplification that probably leads to suboptimal hearing-aid fitting. For a better understanding of the signal degeneration caused by a cochlear lesion a model layout of the signal transmission in the peripheral hearing organ is presented. This model differentiates between processes in the inner ear caused by the outer hair cells, the inner hair cells, and the endocochlear potential driving the system. The model is intended to predict alteration of the signal caused by different types of cochlear lesions. Ultimately, the model may lead to improved hearing aids and fittings.

  11. Better-ear glimpsing in hearing-impaired listeners

    PubMed Central

    Best, Virginia; Mason, Christine R.; Kidd, Gerald; Iyer, Nandini; Brungart, Douglas S.

    2015-01-01

    When competing speech sounds are spatially separated, listeners can make use of the ear with the better target-to-masker ratio. Recent studies showed that listeners with normal hearing are able to efficiently make use of this “better-ear,” even when it alternates between left and right ears at different times in different frequency bands, which may contribute to the ability to listen in spatialized speech mixtures. In the present study, better-ear glimpsing in listeners with bilateral sensorineural hearing impairment, who perform poorly in spatialized speech mixtures, was investigated. The results suggest that this deficit is not related to better-ear glimpsing. PMID:25698053

  12. Optimization of speech in noise with three signal processing algorithms for normal-hearing and hearing-impaired subjects

    NASA Astrophysics Data System (ADS)

    Franck, Bas A. M.; Dreschler, Wouter A.; Lyzenga, Johannes

    2002-05-01

    In this study a three-dimensional Simplex procedure was applied to optimize speech in noise by a combination of signal processing algorithms for different acoustic conditions and hearing losses. The algorithms used to span the three dimensions are noise reduction, spectral tilting, and spectral enhancement, respectively. Additionally, we studied the algorithms for their main effects and interaction effects within the optimization process. The subjects were asked to evaluate two consecutive, differently processed sentences on listening comfort. Three different noise types and two signal-to-noise ratios (S/N) were used. Three groups of subjects participated: normal hearing, normal hearing with simulated impaired auditory filtering (by spectral smearing), and sensorineurally hearing-impaired subjects. For the normal-hearing group we applied S/N=0 dB. For the hearing-impaired and the simulated hearing-impaired subjects we applied S/N=5 dB. We will discuss the similarities and differences in the response patterns of the three groups. Also, the individual preferences will be related to the hearing capacity, and to the type of interfering noise. Finally, we will discuss differences in the perceptual features that are used to judge listening comfort of the fragments by normal-hearing and hearing-impaired subjects.

  13. 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.

  14. 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. PMID:27180348

  15. 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

  16. Evolution of a universal infant hearing screening program in an inner city hospital.

    PubMed

    Cox, L C; Toro, M R

    2001-06-01

    This study reports the evolution of a newborn hearing screening program which began in 1988. Data are reported from the period of time that universal newborn hearing screening was initiated, i.e. April 1996 to December 2000 (total screened=7128 babies). From 1996 to the present, the program has developed to the current form. During 2000, 1713 infants in the well-baby nursery and neonatal intensive care unit were screened at a cost of 18.44 dollars per child. Thirty (1.7%) infants failed the screen, of which 26 (86%) returned for follow-up testing. Fifteen infants were documented with hearing loss, 10 with conductive and five with sensorineural losses. The false positive rate was 0.96% and the overall sensorineural impairment rate was 1/343.

  17. [Mapping of gene underlying autosomal dominant non-syndromic hearing loss(DFNA)].

    PubMed

    Sun, Han-Jun; Tao, Ran; Cheng, Jing; Yang, Shu-Zhi; Cao, Ju-Yang; Yu, Li-Ming; Hong, Meng-Di; Feng, Guo-Yin; Dai, Pu; Yuan, Hui-Jun; Han, Dong-Yi; He, Lin

    2006-12-01

    Hereditary non-syndromic sensorineural hearing loss is a genetically highly heterogeneous group of disorders. To date, at least 50 loci for autosomal dominant non-syndromic sensorineural hearing loss (DFNA) have been identified by linkage analysis. Here we report a huge family with late onset autosomal dominant hereditary non-syndromic hearing loss. In this family, 73 of 170 family members have been conducted physical examination, pure-tone audiometry, immittance testing and auditory brainstem response testing (ABR). The results indicated that 39 of 73 tested family members have sensorineural hearing loss in various degrees. No associated visible abnormalities in other systems were found in this family. After exclusion of the 14 known DFNA loci with markers from the Hereditary Hearing Loss Homepage (URL: http://dnalab-www.uia.ac.be/dnalab/hhh), a genome wide scan was carried out using 382 highly informative microsatellite markers at approximately 9.2 cM intervals throughout the genome. Linkage analysis was carried out under a fully penetrant autosomal dominant mode of inheritance with no phenocopies. A maximum two-point LOD score of 6.69 at theta=0 was obtained for marker D14S1040. Haplotype analysis placed the locus within a 7.6 cM genetic interval defined by marker D14S1021 and D14S70, overlapping with the DFNA9 locus. PMID:17138532

  18. [Migraine-associated vertigo with hearing loss and recurrent vertigo attack].

    PubMed

    Goto, Fumiyuki; Tsutsumi, Tomoko; Ogawa, Kaoru

    2013-05-01

    The clinical features of Ménière's disease and migraine-associated vertigo are quite similar. Both disorders are characterized by repeated vertigo spells. Several diagnostic criteria are used to diagnose migraine-associated vertigo. None of these criteria has been internationally defined, although the criteria proposed by Neuhauser are wieldy accepted. Hearing impairment is believed to be a key factor for diagnosing Ménière's disease. We report herein on a case of repeated vertigo spells with sensorineural hearing loss in the right ear. Initially, the condition was diagnosed as Ménière's disease. Treatment for improving endolymphatic hydrops did not have an effect on the vertigo spells. On careful questioning, we noted the coexistence of migraines without any aura. Treatment with Ca antagonists to prevent the migraine attacks successfully stopped the patient's vertigo spells. On the basis of this clinical course, it is safe to assume that the patient had migraine-associated vertigo with sensorineural hearing loss rather than Ménière's disease. For a patient experiencing migraines together with sensorineural hearing loss, an accurate diagnosis requires careful evaluation. The coexistence of migraines should be carefully ruled out, even if Ménière's disease with hearing loss is strongly suspected.

  19. About Hearing

    MedlinePlus

    ... ability to hear and understand. The duration and nature of a conductive loss will influence a student's ... nih.gov/health/hearing/neuropathy.asp . Implications: The nature and extent to which the hair cells in ...

  20. [Local drug therapy for inner ear hearing loss].

    PubMed

    Liebau, A; Plontke, S K

    2015-06-01

    The indications for local drug therapy of inner ear hearing loss include sudden sensorineural hearing loss, Menière's disease, autoimmune-associated hearing loss, ototoxicity as a side effect of other therapies, acute acoustic trauma and improvement of the safety and performance of cochlear implants. Various drugs are currently being used and tested for local treatment of inner ear hearing loss, including glucocorticoids, growth factors, apoptosis inhibitors, antioxidants, TNF-α inhibitors and antibodies. To further a better understanding of pharmacokinetics and the development of rational pharmacotherapy of the inner ear, the"liberation, absorption, distribution, metabolism, elimination" (LADME) principle can be applied to local therapy of the inner ear. Local application strategies can be differentiated into intratympanic applications to the middle ear cavity and direct intralabyrinthine or intracochlear applications.

  1. Hearing Aids

    MedlinePlus

    ... type and degree of loss. Are there different styles of hearing aids? Styles of hearing aids Source: NIH/NIDCD Behind-the- ... the ear canal and are available in two styles. The in-the-canal (ITC) hearing aid is ...

  2. Hearing loss in speech-language delayed children.

    PubMed

    Psarommatis, I M; Goritsa, E; Douniadakis, D; Tsakanikos, M; Kontrogianni, A D; Apostolopoulos, N

    2001-05-11

    An infant begins to communicate with his/her environment from the first months of life. However, true words do not appear until the age of 12-15 months, following a rather predictable sequence. Delay or failure of normal language development is not a rare situation in childhood and may be due to a variety of reasons. Among these, hearing undoubtedly plays a leading part in the language acquisition process. The purpose of this study was to assess the percentage of hearing-impaired children in a group of phenotypically healthy children presenting with speech-language delay. Between March 1993 and March 1999, 726 speech-language delayed children were examined in our department. In 72 of them, various diseases or syndromes had already been diagnosed and so they were excluded from the study. The remaining 654 apparently healthy children entered the study and underwent a thorough audiological assessment for determination of their hearing thresholds. Eighty-seven children (13.3%) showed various degrees of hearing loss. Most of them (55 children, 8.4%) suffered from sensorineural hearing impairment, while in 32 children (4.9%) a conductive hearing loss was discovered. The increased prevalence of hearing impairment found in our population mandates a thorough hearing evaluation for every case of speech-language delay, even for those children who show no evidence of other handicaps. This will help in the early diagnosis of hearing loss, allowing proper management to be instituted as early as possible. PMID:11335007

  3. [Temporal bone histopathology of noise-induced hearing loss].

    PubMed

    Nakamoto, Yoshinori; Iino, Yukiko; Kodera, Kazuoki

    2005-02-01

    To examine the relationship between hearing and changes in the inner ear, we investigated human temporal bone specimens from 2 patients with noise-induced hearing loss and prepared audio-cytocochleograms as described by Schuknecht et al. Patient 1 was a 50-year-old male who died of thyroid cancer and had worked at a printing house for 38 years. Patient 2 was a 58-year old male who died of maxillary sinus cancer and had worked in construction for 22 years. A pure-tone audiogram showed high-tone sensorineural hearing loss with c5-dip-type hearing disorder in both ears in Patient 1, and a high-tone abrupt form of sensorineural hearing loss in Patient 2. Pathological examination of the temporal bone revealed degeneration and disappearance of the organ of Corti at the basal turn and disappearance of cochlear neurons in both patients. Audio-cytocochleograms revealed hearing disorder consistent with the changes in the inner ear in both patients. Marked degeneration and disappearance of the organ of Corti and stria vascularis were present in patient 1. It is generally known that disorders of the organ of Corti for a long period is involved in the etiology of noise-induced hearing loss. This degeneration of the organ of Corti is produced at a basilar membrane with the maximum amplitude related to exposure to noise according to a physical and mechanical factors. Moreover, animal experiments have shown that exposure to noise decrease cochlear blood flow. In Patient 1 both the organ of Corti and the stria vascularis exhibited degeneration, suggesting that not only physical and mechanical factors but a cochlear circulatory disorder related to exposure to noise was involved in the etiology of the pathological changes in the temporal bone related to noise-induced hearing loss.

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

    PubMed

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

    2013-01-01

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

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

    PubMed Central

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

    2012-01-01

    Wireless synchronization of the digital signal processing (DSP) features between two hearing aids in a bilateral hearing aid fitting is a fairly new technology. This technology is expected to preserve the differences in time and intensity between the two ears by co-ordinating the bilateral DSP features such as multichannel compression, noise reduction, and adaptive directionality. The purpose of this study was to evaluate the benefits of wireless communication as implemented in two commercially available hearing aids. More specifically, this study measured speech intelligibility and sound localization abilities of normal hearing and hearing impaired listeners using bilateral hearing aids with wireless synchronization of multichannel Wide Dynamic Range Compression (WDRC). Twenty subjects participated; 8 had normal hearing and 12 had bilaterally symmetrical sensorineural hearing loss. Each individual completed the Hearing in Noise Test (HINT) and a sound localization test with two types of stimuli. No specific benefit from wireless WDRC synchronization was observed for the HINT; however, hearing impaired listeners had better localization with the wireless synchronization. Binaural wireless technology in hearing aids may improve localization abilities although the possible effect appears to be small at the initial fitting. With adaptation, the hearing aids with synchronized signal processing may lead to an improvement in localization and speech intelligibility. Further research is required to demonstrate the effect of adaptation to the hearing aids with synchronized signal processing on different aspects of auditory performance. PMID:26557339

  6. Permanent sensorineural deafness in a patient with chronic myelogenous leukemia secondary to intracranial hemorrhage.

    PubMed

    Kapur, Sakshi; Wax, Michael; Miles, Levin; Hussain, Adnan

    2013-01-01

    A 52-year-old male presented with tinnitus and fullness in left ear for one day. Workup revealed a white blood cell count of 685 × 10(3)/μL with marked increase in granulocyte series and myeloid precursors on peripheral smear. The initial impression was chronic myelogenous leukemia with hyperleukocytosis, and patient was started on hydration, hydroxyurea, and allopurinol. Patient tolerated bone marrow biopsy well but continued to bleed excessively from the biopsy site. Results confirmed Philadelphia chromosome positive chronic myelogenous leukemia (chronic phase). On day three of hospitalization, patient developed sudden slurred speech along with shaking movements involving extremities. Magnetic resonance imaging revealed multiple hemorrhages throughout the brain. Hydroxyurea was continued until insurance coverage for nilotinib was getting approved. On day nine of hospitalization, patient developed sudden bilateral sensorineural deafness. Repeat magnetic resonance imaging revealed multiple new hemorrhages throughout the brain. Computer tomography of the temporal bones showed inflammatory changes in right and left mastoid cells. Nilotinib was started on day eleven of hospitalization. Patient's white blood cell count continued to decrease, but there was no improvement in hearing. Four months later, patient was treated with bilateral transmastoid cochlear implants. This case highlights permanent deafness as a hemorrhagic complication secondary to chronic myelogenous leukemia.

  7. Permanent Sensorineural Deafness in a Patient with Chronic Myelogenous Leukemia Secondary to Intracranial Hemorrhage

    PubMed Central

    Wax, Michael; Miles, Levin

    2013-01-01

    A 52-year-old male presented with tinnitus and fullness in left ear for one day. Workup revealed a white blood cell count of 685 × 103/μL with marked increase in granulocyte series and myeloid precursors on peripheral smear. The initial impression was chronic myelogenous leukemia with hyperleukocytosis, and patient was started on hydration, hydroxyurea, and allopurinol. Patient tolerated bone marrow biopsy well but continued to bleed excessively from the biopsy site. Results confirmed Philadelphia chromosome positive chronic myelogenous leukemia (chronic phase). On day three of hospitalization, patient developed sudden slurred speech along with shaking movements involving extremities. Magnetic resonance imaging revealed multiple hemorrhages throughout the brain. Hydroxyurea was continued until insurance coverage for nilotinib was getting approved. On day nine of hospitalization, patient developed sudden bilateral sensorineural deafness. Repeat magnetic resonance imaging revealed multiple new hemorrhages throughout the brain. Computer tomography of the temporal bones showed inflammatory changes in right and left mastoid cells. Nilotinib was started on day eleven of hospitalization. Patient's white blood cell count continued to decrease, but there was no improvement in hearing. Four months later, patient was treated with bilateral transmastoid cochlear implants. This case highlights permanent deafness as a hemorrhagic complication secondary to chronic myelogenous leukemia. PMID:24367730

  8. [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.

  9. 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

  10. [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.

  11. Hearing: Noise-Induced Hearing Loss

    MedlinePlus

    MENU Return to Web version Hearing: Noise-Induced Hearing Loss Hearing: Noise-Induced Hearing Loss The importance of hearing Hearing allows you to ... surround the soft tissue of the inner ear. Hearing loss occurs when the inner ear is damaged. What ...

  12. The effects of repetitive vibration on sensorineural function: biomarkers of sensorineural injury in an animal model of metabolic syndrome

    PubMed Central

    Kiedrowski, Megan; Waugh, Stacey; Miller, Roger; Johnson, Claud; Krajnak, Kristine

    2016-01-01

    Exposure to hand-transmitted vibration in the work-place can result in the loss of sensation and pain in workers. These effects may be exacerbated by pre-existing conditions such as diabetes or the presence of primary Raynaud's phenomena. The goal of these studies was to use an established model of vibration-induced injury in Zucker rats. Lean Zucker rats have a normal metabolic profile, while obese Zucker rats display symptoms of metabolic disorder or Type II diabetes. This study examined the effects of vibration in obese and lean rats. Zucker rats were exposed to 4 h of vibration for 10 consecutive days at a frequency of 125 Hz and acceleration of 49 m/s2 for 10 consecutive days. Sensory function was checked using transcutaneous electrical stimulation on days 1, 5 and 9 of the exposure. Once the study was complete the ventral tail nerves, dorsal root ganglia and spinal cord were dissected, and levels of various transcripts involved in sensorineural dysfunction were measured. Sensorineural dysfunction was assessed using transcutaneous electrical stimulation. Obese Zucker rats displayed very few changes in sensorineural function. However they did display significant changes in transcript levels for factors involved in synapse formation, peripheral nerve remodeling, and inflammation. The changes in transcript levels suggested that obese Zucker rats had some level of sensory nerve injury prior to exposure, and that exposure to vibration activated pathways involved in injury and re-innervation. PMID:26433044

  13. The effects of repetitive vibration on sensorineural function: biomarkers of sensorineural injury in an animal model of metabolic syndrome.

    PubMed

    Kiedrowski, Megan; Waugh, Stacey; Miller, Roger; Johnson, Claud; Krajnak, Kristine

    2015-11-19

    Exposure to hand-transmitted vibration in the work-place can result in the loss of sensation and pain in workers. These effects may be exacerbated by pre-existing conditions such as diabetes or the presence of primary Raynaud's phenomena. The goal of these studies was to use an established model of vibration-induced injury in Zucker rats. Lean Zucker rats have a normal metabolic profile, while obese Zucker rats display symptoms of metabolic disorder or Type II diabetes. This study examined the effects of vibration in obese and lean rats. Zucker rats were exposed to 4h of vibration for 10 consecutive days at a frequency of 125 Hz and acceleration of 49 m/s(2) for 10 consecutive days. Sensory function was checked using transcutaneous electrical stimulation on days 1, 5 and 9 of the exposure. Once the study was complete the ventral tail nerves, dorsal root ganglia and spinal cord were dissected, and levels of various transcripts involved in sensorineural dysfunction were measured. Sensorineural dysfunction was assessed using transcutaneous electrical stimulation. Obese Zucker rats displayed very few changes in sensorineural function. However they did display significant changes in transcript levels for factors involved in synapse formation, peripheral nerve remodeling, and inflammation. The changes in transcript levels suggested that obese Zucker rats had some level of sensory nerve injury prior to exposure, and that exposure to vibration activated pathways involved in injury and re-innervation.

  14. Genetics of Nonsyndromic Congenital Hearing Loss.

    PubMed

    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

  15. 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.

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

    PubMed

    Carey, John C; Palumbos, Janice C

    2016-10-01

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

  17. Hearing thresholds in sickle cell anemia patients: emerging new trends?

    PubMed Central

    Aderibigbe, Ademola; Ologe, Foluwasayo E.; Oyejola, Benjamin A.

    2005-01-01

    PURPOSE OF STUDY: Advances in medicine resulting in better understanding of sickle cell disease and general improvement of the well-being of the sufferers even in the developing countries have positively affected the dreadful outlook of this disease with resultant increase in the population of sickle cell disease patients reaching adulthood, and less severe complications. We therefore set out to evaluate the presence and severity of sensorineural hearing loss in sickle cell anemia (SCA) patients in the light of the overall improvement in the morbidity and mortality. METHODS: A prospective case control study of SCA patients attending our adult SCA clinic and control subjects from homozygous hemoglobin AA patients attending the staff clinic of the hospital for routine medical tests. Tympanometry and diagnostic audiometry were performed on each patient. MAIN FINDINGS: Forty-six SCA patients (21 males, 45.7%) aged 16-48 years with a mean age of 22.9 years +/- 6.45 and 42 controls (24 males, 57.1%) aged 15-39 years with a mean age of 23.7 years +/- 5.69 were included in this study. The average hearing thresholds of SCA patients were consistently higher than controls in all frequencies tested in both right and left ears. Of the 92 ears of SCA patients tested, 95.7% exhibited hearing thresholds within normal limits, and 4.3% had mild hearing loss. The controls had thresholds within normal limits. CONCLUSION: The incidence of significant sensorineural hearing loss in SCA seems to have reduced in line with the general improvement and survival of SCA patients. The hearing loss is worse in the right ear and has a female preponderance. We hope that more aggressive primary and secondary prevention and adequate treatment of sickle cell crisis would reduce if not eliminate the hearing loss found in SCA. PMID:16173329

  18. Hearing Assistive Technology

    MedlinePlus

    ... for the Public / Hearing and Balance Hearing Assistive Technology Hearing Assistive Technology: FM Systems | Infrared Systems | Induction ... Assistive Technology Systems Solutions What are hearing assistive technology systems (HATS)? Hearing assistive technology systems (HATS) are ...

  19. Identifying congenital hearing impairment. Personal experience based on selective hearing screening.

    PubMed

    Molini, E; Ricci, G; Baroni, S; Ciorba, A; Bellocci, A; Simoncelli, C

    2004-06-01

    If all degrees of permanent uni- or bilateral hypoacusis are taken into consideration, hearing impairment is the most common congenital disease. Early detection of permanent infantile hearing impairment has become extremely important in preventive medicine, since steps can be taken with hearing aids and rehabilitation to ensure better development of language and higher cognitive functions. Aim of this study is to provide a critical review of the time of diagnosis of hypoacusis at our audiology laboratory, where two methods were used to screen hearing of children with/without risk indicators. Results of approximately 10 years' work were re-examined during which time outpatient screening was conducted on children referred by colleagues in neonatology and paediatrics. All were carriers of congenital risk indicators associated with sensorineural and/or conductive hearing loss, based on the Joint Committee on Infant Hearing findings, or were suspected of being hypoacusic even if they had no known congenital risk factors. Hearing screening was conducted in hospital on newborns with no risk factors, within the first few days of birth. Results of the present study showed that when selective hearing screening was performed, the mean age of high-risk patients diagnosed with hypoacusis was slightly higher than that in international guidelines. Moreover, these patients represent approximately half the hypoacusic population identified in the study period. The other half of congenital hypoacusic subjects identified had no risk indicators and there was a significant delay in diagnosis due to later manifestation of symptoms indicating hypoacusis, and thus, in turn, delayed referral for hearing tests. In contrast, subjects without risk indicators who underwent in-hospital hearing screening and proved to be hypoacusic, were diagnosed early. In our experience, however, universal screening has considerable disadvantages, such as difficulty in covering the entire population, difficulty

  20. 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

  1. 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.

  2. [Advances in hereditary hearing loss caused by TMC1 mutations].

    PubMed

    Wu, Kaiwen; Wang, Hongyang; Wang, Qiuju

    2016-03-01

    Hearing loss is the most frequent sensorineural disorder worldwild, among which about 50% are caused by genetic factors. TMC1 is one of the common genes causing hereditary hearing loss. TMC1 mutations can cause pre-lingual profound/severe autosomal recessive (DFNB7/11) and post-lingual progressive autosomal dominant (DFNA36) non-syndromic hearing loss. Murine models studies show that TMC1, 2 are expressed in cochlea inner and outer hair cells and maintain normal mechanoelectrical transduction (MET) functions of the hair cells. A growing number of evidence indicate that TMC1, 2 are components of the MET complex. It is necessary to definite the precise distribution and exact function of TMC1, 2, because it is important to understand the regulating mechanism of auditory function. PMID:27033582

  3. Better-ear glimpsing at low frequencies in normal-hearing and hearing-impaired listeners.

    PubMed

    Rana, Baljeet; Buchholz, Jörg M

    2016-08-01

    Better-ear glimpsing is an auditory process that takes advantage of short-term interaural level differences (ILDs) to improve the understanding of speech in spatial fluctuating noise. Since ILDs are mainly present at high frequencies, where most hearing-impaired (HI) listeners have the strongest hearing loss, HI individuals cannot fully utilize ILDs for better-ear glimpsing, which may lead to poorer understanding of speech in noise. This problem may be alleviated by hearing aids that artificially generate ILDs at low frequencies where hearing is typically less impaired. The present study therefore investigated the spatial benefit in speech intelligibility that is provided by better-ear glimpsing with low-frequency extended ILDs in a symmetric two-distractor speech background. Speech reception thresholds were measured in a spatially co-located and separated condition as a function of frequency region in ten normal-hearing (NH) and ten mild-to-moderate sensorineural HI subjects. In both groups the extended ILDs provided a substantial spatial advantage on top of the advantage already provided by natural ILDs. Moreover, the spatial advantage was largely independent of frequency region, suggesting that both NH and HI subjects can utilize low-frequency ILDs for improving speech understanding in noise. Overall performance as well as spatial advantage was reduced in the HI group. PMID:27586748

  4. An allograft mouse model for the study of hearing loss secondary to vestibular schwannoma growth.

    PubMed

    Bonne, Nicolas-Xavier; Vitte, Jérémie; Chareyre, Fabrice; Karapetyan, Gevorg; Khankaldyyan, Vazgen; Tanaka, Karo; Moats, Rex A; Giovannini, Marco

    2016-08-01

    Vestibular schwannoma is a benign neoplasm arising from the Schwann cell sheath of the auditory-vestibular nerve. It most commonly affects both sides in the genetic condition Neurofibromatosis type 2, causing progressive high frequency sensorineural hearing loss. Here, we describe a microsurgical technique and stereotactic coordinates for schwannoma cell grafting in the vestibular nerve region that recapitulates local tumor growth in the cerebellopontine angle and inner auditory canal with resulting hearing loss. Tumor growth was monitored by bioluminescence and MRI in vivo imaging, and hearing assessed by auditory brainstem responses. These techniques, by potentially enabling orthotopic grafting of a variety of cell lines will allow studies on the pathogenesis of tumor-related hearing loss and preclinical drug evaluation, including hearing endpoints, for NF2-related and sporadic schwannomas. PMID:27177628

  5. 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

  6. 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

  7. 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

  8. 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.

  9. 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…

  10. 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.

  11. Determinants of sensorineural hearing loss in chronic middle-ear disease.

    PubMed

    Kasliwal, Neeraj; Joshi, Sanjeev; Pareek, S M

    2004-10-01

    A statistical study was carried out on SNHL in CSOM. The study group consisted of 1,828 patients suffering from CSOM who underwent surgery at our centre from 1982 to 2001, out of these 510 cases with unilateral CSOM were selected for this study by a strict selection criteria so as to eliminate covariables such as exposure to acoustic trauma, head injury, previous ear surgery and hereditary causes. The healthy ear served as control. We determined the average SNHL in relation to the age of onset, duration of disease, examining it in relation to other eventual aural complications such as cholesteatoma, ossicular chain erosion und otorrhea.On the basis of data obtained we observed consistent co-relation between severity of SNHL and duration of the disease, presence of cholesteatoma, ossicular erosion, attic and subtotal perforations. These findings suggest that more severe middle ear disease may result in SNHL and thus early intervention in cases of chronic suppurative Otitis media is desired. PMID:23120094

  12. [Radionuclide tests of cerebral circulation in the assessment of treatment of sensorineural hearing loss].

    PubMed

    Karalkin, A V; Petukhova, N A

    2001-01-01

    Examination and treatment results are available for 59 patients with acute and chronic neurosensory hypoacusis. Cerebral circulation was investigated at radionuclide carotid arteriography and radionuclide single-photon emission computed tomography. Arterial flow to the brain, microcirculation of the gray matter and venous outflow from the cranial cavity were studied before and after the course of quantum hemotherapy.

  13. 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

  14. 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 ...

  15. Hearing Aid

    MedlinePlus

    ... and Food and Drug Administration Staff FDA permits marketing of new laser-based hearing aid with potential ... feeds Follow FDA on Twitter Follow FDA on Facebook View FDA videos on YouTube View FDA photos ...

  16. 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)

  17. Hearing assessment data in HIV-infected and uninfected children of Cape Town, South Africa.

    PubMed

    Torre, Peter; Cook, Alyssa; Elliott, Haley; Dawood, Gouwa; Laughton, Barbara

    2015-01-01

    Researchers are showing that the rate of hearing loss in children with perinatal HIV infection (PHIV) is higher than in HIV-unexposed, uninfected children. These data, however, have been collected mostly in the USA; extensive hearing data from low- and middle-income countries are lacking. The purpose of this study was to collect audiometric data in PHIV and HIV-uninfected children living in Cape Town, South Africa. Questionnaire data along with distortion product otoacoustic emissions (DPOAEs) and pure-tone testing were completed. Hearing loss was determined using the pure-tone thresholds defined as a pure-tone average (PTA) of 500, 1000, 2000, and 4000 Hz of >15 dB HL in the poorer ear. All data were compared between PHIV and HIV-uninfected children. Sixty-one (37 PHIV and 24 HIV-uninfected) children had hearing data. HIV status was not significantly associated with DPOAEs. The rate of conductive hearing loss was 11.5%; five PHIV and two HIV-uninfected children. The rate of any hearing loss was higher in PHIV children, but this difference was not statistically significant. PHIV children had a significantly higher mean PTA in the poorer ear than HIV-uninfected children. Conductive type of hearing loss was more common than sensorineural hearing loss. The underlying cause of hearing loss in the present study therefore remains unclear. Future research should include an examination of auditory neural function in an effort to determine the possible reason for differences in hearing. PMID:25760238

  18. 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.

  19. Hearing assessment data in HIV-infected and uninfected children of Cape Town, South Africa.

    PubMed

    Torre, Peter; Cook, Alyssa; Elliott, Haley; Dawood, Gouwa; Laughton, Barbara

    2015-01-01

    Researchers are showing that the rate of hearing loss in children with perinatal HIV infection (PHIV) is higher than in HIV-unexposed, uninfected children. These data, however, have been collected mostly in the USA; extensive hearing data from low- and middle-income countries are lacking. The purpose of this study was to collect audiometric data in PHIV and HIV-uninfected children living in Cape Town, South Africa. Questionnaire data along with distortion product otoacoustic emissions (DPOAEs) and pure-tone testing were completed. Hearing loss was determined using the pure-tone thresholds defined as a pure-tone average (PTA) of 500, 1000, 2000, and 4000 Hz of >15 dB HL in the poorer ear. All data were compared between PHIV and HIV-uninfected children. Sixty-one (37 PHIV and 24 HIV-uninfected) children had hearing data. HIV status was not significantly associated with DPOAEs. The rate of conductive hearing loss was 11.5%; five PHIV and two HIV-uninfected children. The rate of any hearing loss was higher in PHIV children, but this difference was not statistically significant. PHIV children had a significantly higher mean PTA in the poorer ear than HIV-uninfected children. Conductive type of hearing loss was more common than sensorineural hearing loss. The underlying cause of hearing loss in the present study therefore remains unclear. Future research should include an examination of auditory neural function in an effort to determine the possible reason for differences in hearing.

  20. 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.

  1. 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

    2016-01-01

    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. PMID:27585248

  2. [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.

  3. Using Speech Recall in Hearing Aid Fitting and Outcome Evaluation Under Ecological Test Conditions.

    PubMed

    Lunner, Thomas; Rudner, Mary; Rosenbom, Tove; Ågren, Jessica; Ng, Elaine Hoi Ning

    2016-01-01

    In adaptive Speech Reception Threshold (SRT) tests used in the audiological clinic, speech is presented at signal to noise ratios (SNRs) that are lower than those generally encountered in real-life communication situations. At higher, ecologically valid SNRs, however, SRTs are insensitive to changes in hearing aid signal processing that may be of benefit to listeners who are hard of hearing. Previous studies conducted in Swedish using the Sentence-final Word Identification and Recall test (SWIR) have indicated that at such SNRs, the ability to recall spoken words may be a more informative measure. In the present study, a Danish version of SWIR, known as the Sentence-final Word Identification and Recall Test in a New Language (SWIRL) was introduced and evaluated in two experiments. The objective of experiment 1 was to determine if the Swedish results demonstrating benefit from noise reduction signal processing for hearing aid wearers could be replicated in 25 Danish participants with mild to moderate symmetrical sensorineural hearing loss. The objective of experiment 2 was to compare direct-drive and skin-drive transmission in 16 Danish users of bone-anchored hearing aids with conductive hearing loss or mixed sensorineural and conductive hearing loss. In experiment 1, performance on SWIRL improved when hearing aid noise reduction was used, replicating the Swedish results and generalizing them across languages. In experiment 2, performance on SWIRL was better for direct-drive compared with skin-drive transmission conditions. These findings indicate that spoken word recall can be used to identify benefits from hearing aid signal processing at ecologically valid, positive SNRs where SRTs are insensitive.

  4. 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.

  5. Using Speech Recall in Hearing Aid Fitting and Outcome Evaluation Under Ecological Test Conditions.

    PubMed

    Lunner, Thomas; Rudner, Mary; Rosenbom, Tove; Ågren, Jessica; Ng, Elaine Hoi Ning

    2016-01-01

    In adaptive Speech Reception Threshold (SRT) tests used in the audiological clinic, speech is presented at signal to noise ratios (SNRs) that are lower than those generally encountered in real-life communication situations. At higher, ecologically valid SNRs, however, SRTs are insensitive to changes in hearing aid signal processing that may be of benefit to listeners who are hard of hearing. Previous studies conducted in Swedish using the Sentence-final Word Identification and Recall test (SWIR) have indicated that at such SNRs, the ability to recall spoken words may be a more informative measure. In the present study, a Danish version of SWIR, known as the Sentence-final Word Identification and Recall Test in a New Language (SWIRL) was introduced and evaluated in two experiments. The objective of experiment 1 was to determine if the Swedish results demonstrating benefit from noise reduction signal processing for hearing aid wearers could be replicated in 25 Danish participants with mild to moderate symmetrical sensorineural hearing loss. The objective of experiment 2 was to compare direct-drive and skin-drive transmission in 16 Danish users of bone-anchored hearing aids with conductive hearing loss or mixed sensorineural and conductive hearing loss. In experiment 1, performance on SWIRL improved when hearing aid noise reduction was used, replicating the Swedish results and generalizing them across languages. In experiment 2, performance on SWIRL was better for direct-drive compared with skin-drive transmission conditions. These findings indicate that spoken word recall can be used to identify benefits from hearing aid signal processing at ecologically valid, positive SNRs where SRTs are insensitive. PMID:27355764

  6. [Fibrinogen/LDL apheresis for treatment of sudden hearing loss: an observational study on 152 patients].

    PubMed

    Canis, M; Heigl, F; Hettich, R; Osterkorn, D; Osterkorn, K; Suckfuell, M

    2008-09-01

    Disturbances of cochlear microcirculation are among the most discussed causes of sudden sensorineural hearing loss. Increased levels of cholesterol and fibrinogen seem to act as risk factors for inner ear disorders. Fibrinogen/LDL apheresis greatly reduces the concentration of plasma fibrinogen thus leading to improved cochlear blood flow. In a retrospective case series remission rates of 152 patients suffering from sudden sensorineural hearing loss and resistant to former treatment were investigated after treatment with a single apheresis. Complete remission was reported in 11% of patients, partial remission in 43%. 37% had no change of hearing threshold and 2% reported a decrease in hearing. Rates of complete remissions decreased from 22% within the first 2 weeks after onset of hearing loss to 14% after 6 weeks. In the same period of time rates of partial remissions decreased from 33% to 13%. The present study shows that apheresis achieved complete or partial remission in 54% of patients even after unsuccessful treatment with another therapy and the therapeutic window lies by approximately 6 weeks.

  7. Cochlear Implantation in Adults with Asymmetric Hearing Loss

    PubMed Central

    Firszt, Jill B.; Holden, Laura K.; Reeder, Ruth M.; Cowdrey, Lisa; King, Sarah

    2012-01-01

    Objective Bilateral severe-to-profound sensorineural hearing loss is a standard criterion for cochlear implantation. Increasingly, patients are implanted in one ear and continue to use a hearing aid in the non-implanted ear to improve abilities such as sound localization and speech understanding in noise. Patients with severe-to-profound hearing loss in one ear and a more moderate hearing loss in the other ear (i.e., asymmetric hearing) are not typically considered candidates for cochlear implantation. Amplification in the poorer ear is often unsuccessful due to limited benefit, restricting the patient to unilateral listening from the better ear alone. The purpose of this study was to determine if patients with asymmetric hearing loss could benefit from cochlear implantation in the poorer ear with continued use of a hearing aid in the better ear. Design Ten adults with asymmetric hearing between ears participated. In the poorer ear, all participants met cochlear implant candidacy guidelines; seven had postlingual onset and three had pre/perilingual onset of severe-to-profound hearing loss. All had open-set speech recognition in the better hearing ear. Assessment measures included word and sentence recognition in quiet, sentence recognition in fixed noise (four-talker babble) and in diffuse restaurant noise using an adaptive procedure, localization of word stimuli and a hearing handicap scale. Participants were evaluated pre-implant with hearing aids and post-implant with the implant alone, the hearing aid alone in the better ear and bimodally (the implant and hearing aid in combination). Postlingual participants were evaluated at six months post-implant and pre/perilingual participants were evaluated at six and 12 months post-implant. Data analysis compared results 1) of the poorer hearing ear pre-implant (with hearing aid) and post-implant (with cochlear implant), 2) with the device(s) used for everyday listening pre- and post-implant and, 3) between the hearing

  8. Hearing deficits in the older patient: "I didn't notice anything".

    PubMed

    Pacala, James T; Yueh, Bevan

    2012-03-21

    Hearing loss is common in older adults. Patients, clinicians, and health care staff often do not recognize hearing loss, particularly in its early stages, and it is undertreated. Age-related hearing loss or presbycusis, the most common type of hearing loss in older adults, is a multifactorial sensorineural loss that frequently includes a component of impaired speech discrimination. Simple office-based screening and evaluation procedures can identify potential hearing disorders, which should prompt audiologic referral to confirm the diagnosis with audiometric testing. The mainstay of treatment is amplification. For many older adults, accepting the need for amplification, selecting and purchasing a hearing aid, and getting accustomed to its use is a daunting and often frustrating process. There are numerous barriers to hearing aid use, the most common of which is dissatisfaction with its performance across a range of sonic environments. Newer digital hearing aids have many features that improve performance, making them potentially more acceptable to users, but they are expensive and are not covered by Medicare. Hearing aids have been demonstrated to improve hearing function and hearing-related quality of life (QOL), but evidence is less robust for improving overall QOL. Depending upon the etiology of the hearing loss, other medical and surgical procedures, including cochlear implantation, may benefit older adults. Older adults with multiple morbidities and who are frail pose specific challenges for the management of hearing loss. These patients may require integration of hearing assessment and treatment as part of functional assessment in an interdisciplinary, team-based approach to care.

  9. Hearing deficits in the older patient: "I didn't notice anything".

    PubMed

    Pacala, James T; Yueh, Bevan

    2012-03-21

    Hearing loss is common in older adults. Patients, clinicians, and health care staff often do not recognize hearing loss, particularly in its early stages, and it is undertreated. Age-related hearing loss or presbycusis, the most common type of hearing loss in older adults, is a multifactorial sensorineural loss that frequently includes a component of impaired speech discrimination. Simple office-based screening and evaluation procedures can identify potential hearing disorders, which should prompt audiologic referral to confirm the diagnosis with audiometric testing. The mainstay of treatment is amplification. For many older adults, accepting the need for amplification, selecting and purchasing a hearing aid, and getting accustomed to its use is a daunting and often frustrating process. There are numerous barriers to hearing aid use, the most common of which is dissatisfaction with its performance across a range of sonic environments. Newer digital hearing aids have many features that improve performance, making them potentially more acceptable to users, but they are expensive and are not covered by Medicare. Hearing aids have been demonstrated to improve hearing function and hearing-related quality of life (QOL), but evidence is less robust for improving overall QOL. Depending upon the etiology of the hearing loss, other medical and surgical procedures, including cochlear implantation, may benefit older adults. Older adults with multiple morbidities and who are frail pose specific challenges for the management of hearing loss. These patients may require integration of hearing assessment and treatment as part of functional assessment in an interdisciplinary, team-based approach to care. PMID:22436959

  10. [A fully-implantable active hearing device in congenital auricular atresia].

    PubMed

    Siegert, R; Neumann, C

    2014-07-01

    Active implantable hearing devices were primarily developed for sensorineural hearing loss. The vibrator coupling mechanisms were oriented towards normal middle ear anatomy and function. The aim of this project was to modify the only fully implantable hearing device with an implantable microphone for application in congenital auricular atresia, Carina™, and to introduce the modified device into the clinic. A special prosthesis was developed for the transducer and its individual coupling achieved by a special cramping system. The system was implanted in 5 patients with congenital auricular atresia. Audiological results were good; with patients' hearing gain exceeding 30 dB HL. Anatomic limits to the system's indications and technical drawbacks are also discussed. PMID:25056646

  11. Targeted massive parallel sequencing: the effective detection of novel causative mutations associated with hearing loss in small families

    PubMed Central

    2012-01-01

    Background Hereditary hearing loss is one of the most common heterogeneous disorders, and genetic variants that can cause hearing loss have been identified in over sixty genes. Most of these hearing loss genes have been detected using classical genetic methods, typically starting with linkage analysis in large families with hereditary hearing loss. However, these classical strategies are not well suited for mutation analysis in smaller families who have insufficient genetic information. Methods Eighty known hearing loss genes were selected and simultaneously sequenced by targeted next-generation sequencing (NGS) in 8 Korean families with autosomal dominant non-syndromic sensorineural hearing loss. Results Five mutations in known hearing loss genes, including 1 nonsense and 4 missense mutations, were identified in 5 different genes (ACTG1, MYO1F, DIAPH1, POU4F3 and EYA4), and the genotypes for these mutations were consistent with the autosomal dominant inheritance pattern of hearing loss in each family. No mutational hot-spots were revealed in these Korean families. Conclusion Targeted NGS allowed for the detection of pathogenic mutations in affected individuals who were not candidates for classical genetic studies. This report is the first documenting the effective use of an NGS technique to detect pathogenic mutations that underlie hearing loss in an East Asian population. Using this NGS technique to establish a database of common mutations in Korean patients with hearing loss and further data accumulation will contribute to the early diagnosis and fundamental therapies for hereditary hearing loss. PMID:22938506

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

    PubMed Central

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

    2011-01-01

    OBJECTIVES: A lack of attention has been given to hearing health in primary care in developing countries. A strategy involving low-cost screening tools may fill the current gap in hearing health care provided to children. Therefore, it is necessary to establish and adopt lower-cost procedures that are accessible to underserved areas that lack other physical or human resources that would enable the identification of groups at risk for hearing loss. The aim of this study was to develop and analyze the efficacy of a low-cost screening tool to identify and classify hearing loss in children. METHODS: A total of 214 2-to-10 year-old children participated in this study. The study was conducted by providing a questionnaire to the parents and comparing the answers with the results of a complete audiological assessment. Receiver operating characteristic (ROC) curves were constructed, and discriminant analysis techniques were used to classify each child based on the total score. RESULTS: We found conductive hearing loss in 39.3% of children, sensorineural hearing loss in 7.4% and normal hearing in 53.3%. The discriminant analysis technique provided the following classification rule for the total score on the questionnaire: 0 to 4 points – normal hearing; 5 to 7 points – conductive hearing loss; over 7 points – sensorineural hearing loss. CONCLUSION: Our results suggest that the questionnaire could be used as a screening tool to classify children with normal hearing or hearing loss and according to the type of hearing loss based on the total questionnaire score. PMID:22086526

  13. [A comparative analysis of the neurophysiological mechanisms of hearing disorders of different origins by the indices of the background and evoked bioelectrical activity of the brain].

    PubMed

    Kozak, N S

    1999-07-01

    The hearing function was studied as was the bioelectrical activity of the brain in workers exposed to noise in their occupations and in those persons coming into contact with ionizing radiation with normal and disordered hearing. The studies made showed that those persons under exposure to ionizing radiation exhibited more pronounced disturbances in the brain stem structures of the acoustic analyzer than those exposed to noise even if they were early in the course of the ear disorder. We consider it expedient to undertake studies on bioelectrical activity of the brain to gain further insights into the mechanisms of sensorineural hearing loss.

  14. 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

  15. 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

  16. Otitis media with effusion and hearing loss in children with Cornelia de Lange syndrome.

    PubMed

    Marchisio, Paola; Selicorni, Angelo; Pignataro, Lorenzo; Milani, Donatella; Baggi, Elena; Lambertini, Lara; Dusi, Elisa; Villa, Laura; Capaccio, Pasquale; Cerutti, Marta; Esposito, Susanna; Principi, Nicola

    2008-02-15

    The development of children who have syndromes with mental retardation and/or language delay can be worsened by sensorineural or conductive hearing loss (HL). Given the existing scarcity of data, we investigated the prevalence of otitis media with effusion and/or HL in 50 children with Cornelia de Lange syndrome (CdLS) aged 1-18 years, and its impact on the children's performance. The children underwent otological and audiological examinations in order to ascertain the relative frequencies of otitis media with effusion and/or hearing impairment; their demographic and clinical data were obtained by questionnaires and from information in their medical charts. Otitis media with effusion was diagnosed in 94%, and its prevalence was similar in all age groups; HL was detected in 40 children (80%). Conductive HL due to middle ear effusion was the main cause of hearing impairment alone (60%) or in combination with sensorineural deficit (20%). HL had a negative impact on performance regardless of the type. A history of routine audiological and/or otological assessments was reported by a minority of parents. Our findings indicate that otitis media with effusion and/or HL is an important feature of children with CdLS and may negatively affect their performance. Careful follow-up throughout childhood is necessary to detect and treat any hearing loss in children with CdLS in order to minimize its impact on performance.

  17. 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.

  18. 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.

  19. Role of p19ink4d in the pathogenesis of hearing loss

    PubMed Central

    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. PMID:26722409

  20. 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

  1. Noise and Hearing Protection

    MedlinePlus

    ... particularly because such exposure is avoidable. What causes hearing loss? The ear has three main parts: the outer, ... can I tell if my hearing is damaged? Hearing loss usually develops over a period of several years. ...

  2. Help with Hearing

    MedlinePlus

    ... hearing. This problem can make it more diffi- cult to learn speech sounds and language correctly. Take ... how your child is hearing. See how diffi- cult it is to hear words correctly? Some children ...

  3. Hearing Loss and Older Adults

    MedlinePlus

    ... Home » Health Info » Hearing, Ear Infections, and Deafness Hearing Loss and Older Adults On this page: What is ... about hearing loss and older adults? What is hearing loss? Hearing loss is a sudden or gradual decrease ...

  4. [A model of healthcare for children with hearing disorders].

    PubMed

    Głowacka, Maria Danuta; Swidziński, Piotr; Mojs, Ewa; Frankowska, Anna

    2009-01-01

    Hearing disorders have risen today to the status of a civilization disease. Hearing loss during development or the prelingual age is an enormous problem for the child. Undiagnosed in time, hearing loss may lead to serious limitations in intellectual, cognitive, and emotional development of the child, and to difficulties in speaking, writing, reading, and memorising. This paper presents current structural solutions regarding healthcare for children with hearing disorders. The system of early detection of hearing disorders (mainly inborn) is coherent for neonates and small children but lacks a well-coordinated healthcare model for older hearing-impaired children, especially in the context of disclosing acquired hearing defects. A model of healthcare for children with hearing disorders covering every stage of development (age) is presented, aiming at improvements in the system of early detection of inborn and acquired hearing defects. The model provides for systematic monitoring of patients with hearing defects and offers the opportunity of early intervention. Furthermore, the model serves to follow the developmental dynamics of the communication process. PMID:20698186

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

    PubMed Central

    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

  6. 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

  7. Sudden hearing loss subsequent to diarrhea: what is the missing link?

    PubMed

    Jafari, Gholamali; Hosseini, Seyed Mohammadreza; Akhondzadeh, Shahin

    2014-01-08

    Sudden sensorineural hearing loss (SSNHL) is a debilitating condition with an incidence of nearly 20 per 100,000 in populations. Metronidazole-induced ototoxicity is an extremely rare etiology of SSNHL. In this report, we describe a young female with bilateral SSNHL due to oral use of metronidazole. A 23 years old female presented to the emergency department with acute bilateral hearing loss. We found out that her hearing loss had started 4 days after initiation of metronidazole which was administered for treatment of diarrhea. This case report shows that physicians should be aware of the uncommon side effects while prescribing metronidazole to patients in order to manage the possible adverse events on time.

  8. 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

    2014-01-01

    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. PMID:25421088

  9. 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.

  10. Dynamic Relation Between Working Memory Capacity and Speech Recognition in Noise During the First 6 Months of Hearing Aid Use

    PubMed Central

    Classon, Elisabet; Larsby, Birgitta; Arlinger, Stig; Lunner, Thomas; Rudner, Mary; Rönnberg, Jerker

    2014-01-01

    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. PMID:25421088

  11. 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

  12. Total implantation of the active hearing implant TICA for middle ear disease: a temporal bone study.

    PubMed

    Maassen, M M; Lehner, R; Leysieffer, H; Baumann, I; Zenner, H P

    2001-10-01

    A subpopulation of hearing-impaired patients has conductive hearing loss that cannot be improved by classic tympanoplasty. Other patients have a mixed hearing loss and cannot be helped by present forms of ear surgery or by hearing aids. Possible help for some patients may come from current implantable hearing devices if these are modified for the patient's specific anatomic situation. The TICA LZ 3001 is a hearing implant for total implantation used to treat moderate to severe sensorineural hearing loss. Most patients who use it have a normal ossicular chain that allows coupling of the implant to the incus. The present temporal bone study demonstrates that the TICA can also be used in patients with an interrupted ossicular chain. If the incus long process shows a defect, the TICA may be coupled to the incus body, and connection between the stapes and the long process of the incus can be achieved with a commercially available titanium-angle prosthesis or liquid ionomeric cement. In cases of an absent incus, the coupling axis of the transducer may be coupled to the stapes head via a modified coupling element. With an absent stapes, the coupling axis may be coupled directly to the perilymph by a coupling element similar to a gold stapes prosthesis.

  13. Deletion of the entire POU4F3 gene in a familial case of autosomal dominant non-syndromic hearing loss.

    PubMed

    Freitas, Érika L; Oiticica, Jeanne; Silva, Amanda G; Bittar, Roseli S M; Rosenberg, Carla; Mingroni-Netto, Regina C

    2014-03-01

    In 20% of cases, hereditary non-syndromic hearing loss has an autosomal dominant inheritance (ADNSHL). To date, more than 50 loci for ADNSHL have been mapped to different chromosomal regions. In order to verify whether genomic alterations contribute to the hearing loss etiology and to search for novel deafness candidate loci, we investigated probands from families with ADNSHL by oligonucleotide array-CGH. A deletion in the 5q32 region encompassing only one gene, POU4F3, which corresponds to DFNA15, was detected in one family. POU4F3 protein has an important role in the maturation, differentiation and survival of cochlear hair cells. Defects in these cells may therefore explain sensorineural hearing loss. Mutations in this gene have already been associated with autosomal dominant hearing loss but this is the first description of a germline POUF4F3 deletion associated with hearing impairment.

  14. 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.

  15. [Hearing preservation: Better hearing with advanced technology].

    PubMed

    Rader, T; Helbig, S; Stöver, T; Baumann, U

    2014-05-01

    Preservation of residual hearing after cochlear implantation allows patients the synergetic use of electric and acoustic stimulation (EAS). The application of specific surgical and therapeutic techniques enables the reduction of inner ear trauma, which leads otherwise to complete hearing loss. Due to simultaneous electric and acoustic stimulation, speech understanding is improved especially in noise. EAS is a well-accepted therapeutic treatment for subjects with profound hearing loss in the higher frequencies and no or mild hearing loss in the low frequencies. Several Manufacturers offer individual soft electrodes specially designed for hearing preservation as well as combined electric-acoustic audio processors. PMID:24782208

  16. 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

  17. Overexpression of the mitochondrial methyltransferase TFB1M in the mouse does not impact mitoribosomal methylation status or hearing.

    PubMed

    Lee, Seungmin; Rose, Simon; Metodiev, Metodi D; Becker, Lore; Vernaleken, Alexandra; Klopstock, Thomas; Gailus-Durner, Valerie; Fuchs, Helmut; Hrabě De Angelis, Martin; Douthwaite, Stephen; Larsson, Nils-Göran

    2015-12-20

    Mitochondrial dysfunction is a well-established cause of sensorineural deafness, but the pathophysiological events are poorly understood. Non-syndromic deafness and predisposition to aminoglycoside-induced deafness can be caused by specific mutations in the 12S rRNA gene of mtDNA and are thus maternally inherited traits. The pathophysiology induced by mtDNA mutations has traditionally been attributed to deficient oxidative phosphorylation, which causes energy crisis with functional impairment of multiple cellular processes. In contrast, it was recently reported that signaling induced by 'hypermethylation' of two conserved adenosines of 12S rRNA in the mitoribosome is of key pathophysiological importance in sensorineural deafness. In support for this concept, it was reported that overexpression of the essential mitochondrial methyltransferase TFB1M in the mouse was sufficient to induce mitoribosomal hypermethylation and deafness. At variance with this model, we show here that 12S rRNA is near fully methylated in vivo in the mouse and thus cannot be further methylated to any significant extent. Furthermore, bacterial artificial chromosome transgenic mice overexpressing TFB1M have no increase of 12S rRNA methylation levels and hear normally. We thus conclude that therapies directed against mitoribosomal methylation are unlikely to be beneficial to patients with sensorineural hearing loss or other types of mitochondrial disease.

  18. 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.

  19. 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. PMID:23648550

  20. [Universal newborn hearing screening--a silent revolution].

    PubMed

    Schmuziger, N; Veraguth, D; Probst, R

    2008-09-24

    Significant hearing loss is one of the most frequent congenital diseases and present at birth in approximately one in 1000 newborns. During the first years of life, this number of children with permanent hearing loss rises by approximately the same amount. Hearing impairment is not an obvious condition in newborns in most cases and produces very few signs in infancy. Such hearing loss threatens normal speech and personality development. The longer the child is without auditory stimulation by speech, the more difficult it is to acquire the missed linguistic skills. Therefore, early detection and rehabilitation of such hearing loss is crucial. Universal newborn hearing screening is the most effective method to detect such impairment and is based on objective audiometric test methods that were recently developed and adapted for screening requirements. Useful tests are otoacoustic emissions and the auditory brainstem response. This article reviews this new screening tests in general and with special regard to the situation in Switzerland.

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

    PubMed Central

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

    2015-01-01

    Using whole-exome sequencing, we have identified in ten families 14 individuals with microcephaly, developmental delay, intellectual disability, hypotonia, spasticity, seizures, sensorineural hearing loss, cortical visual impairment, and rare autosomal-recessive predicted pathogenic variants in spermatogenesis-associated protein 5 (SPATA5). SPATA5 encodes a ubiquitously expressed member of the ATPase associated with diverse activities (AAA) protein family and is involved in mitochondrial morphogenesis during early spermatogenesis. It might also play a role in post-translational modification during cell differentiation in neuronal development. Mutations in SPATA5 might affect brain development and function, resulting in microcephaly, developmental delay, and intellectual disability. PMID:26299366

  2. 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

  3. 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.

  4. 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.

  5. Hospital-acquired pneumonia

    MedlinePlus

    ... tends to be more serious than other lung infections because: People in the hospital are often very sick and cannot fight off ... prevent pneumonia. Most hospitals have programs to prevent hospital-acquired infections.

  6. Acquired Cerebral Trauma: Epilogue.

    ERIC Educational Resources Information Center

    Bigler, Erin D., Ed.

    1988-01-01

    The article summarizes a series of articles concerning acquired cerebral trauma. Reviewed are technological advances, treatment, assessment, potential innovative therapies, long-term outcome, family impact of chronic brain injury, and prevention. (DB)

  7. 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

  8. 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.

  9. [Acquired haemophilia (acquired factor VIII inhibitor)].

    PubMed

    Ceresetto, José M; Duboscq, Cristina; Fondevila, Carlos; Tezanos Pinto, Miguel

    2015-01-01

    Acquired haemophilia is a rare disorder. The clinical picture ranges from mild ecchymosis and anaemia to life threatening bleeding in up to 20% of patients. The disease is produced by an antibody against Factor VIII and it usually occurs in the elderly, with no previous history of a bleeding disorder. It can be associated to an underlying condition such as cancer, autoimmune disorders, drugs or pregnancy. It has a typical laboratory pattern with isolated prolonged activated partial thromboplastin time (aPTT) that fails to correct upon mixing tests with normal plasma and low levels of factor VIII. Treatment recommendations are based on controlling the acute bleeding episodes with either bypassing agent, recombinant activated factor VII or activated prothrombin complex concentrate, and eradication of the antibody with immunosuppressive therapy.

  10. 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

  11. Improved treatment of sudden hearing loss by specific fibrinogen aphaeresis.

    PubMed

    Ullrich, Heidrun; Kleinjung, Tobias; Steffens, Thomas; Jacob, Peter; Schmitz, Gerd; Strutz, Jürgen

    2004-01-01

    The etiology of sudden sensorineural hearing loss is still unclear and is thought to result from disturbances of microcirculation, infectious causes, or autoimmune disorders. So far standard therapy did not show clear improvement over spontaneous remission rate, which is assumed to be about 50% [Nakashima et al., Acta. Otolaryngol. Stockh. 514:14-16, 1994; Schuknecht and Donovan, Arch. Otorhinolaryngol. 243:1-15, 1986; Harris and Sharp, Laryngoscope 100:516-524, 1990; Mayot et al., Clin. Immunol. Immunopath. 68:41-45, 1993; Gussen, Ann. Otol. Rhinol. Laryngol. 85:94-100, 1976]. Elevated blood viscosity due to high fibrinogen levels is supposed to cause decreased cochlear blood flow and thus initiate sudden hearing loss. The specific lowering of fibrinogen immediately decreases plasma viscosity exactly to the desired extent and should lead to improved cochlear blood flow [Suckfüll et al., Acta. Otolaryngol 119:763-766, 1999; Suckfüll, Lancet 360:1811-1817, 2002; Walch et al., Laryngol. Rhino. Otol. 75:641-645, 1996; Suckfüll et al., Otol. Neurotol. 23:309-311, 2002]. In a prospective uncontrolled pilot study on 36 patients with unilateral sudden onset sensorineural hearing loss (SHL) we tried to establish that 1-3 specific fibrinogen aphaereses alone improve recovery of hearing and that it is possible to lower fibrinogen to the target of 80-100 mg/dl without important side effects. Pure tone audiometry was carried out immediately before and after each aphaeresis as well as at 2 and 4 weeks and 6 months after treatment. Sixteen patients recovered spontaneously before undergoing fibrinogen adsorption. All 20 aphaeresis patients improved during immunoadsorption; in 60% of patients auditory thresholds returned to normal after the first immunoadsorption and treatment could be discontinued, in another 20% of patients complete recovery was reached after 4 weeks. The mean plasma fibrinogen concentration of the 20 patients before the first aphaeresis session was 308

  12. Excitatory, inhibitory and facilitatory frequency response areas in the inferior colliculus of hearing impaired mice.

    PubMed

    Felix, Richard A; Portfors, Christine V

    2007-06-01

    Individuals with age-related hearing loss often have difficulty understanding complex sounds such as basic speech. The C57BL/6 mouse suffers from progressive sensorineural hearing loss and thus is an effective tool for dissecting the neural mechanisms underlying changes in complex sound processing observed in humans. Neural mechanisms important for processing complex sounds include multiple tuning and combination sensitivity, and these responses are common in the inferior colliculus (IC) of normal hearing mice. We examined neural responses in the IC of C57Bl/6 mice to single and combinations of tones to examine the extent of spectral integration in the IC after age-related high frequency hearing loss. Ten percent of the neurons were tuned to multiple frequency bands and an additional 10% displayed non-linear facilitation to the combination of two different tones (combination sensitivity). No combination-sensitive inhibition was observed. By comparing these findings to spectral integration properties in the IC of normal hearing CBA/CaJ mice, we suggest that high frequency hearing loss affects some of the neural mechanisms in the IC that underlie the processing of complex sounds. The loss of spectral integration properties in the IC during aging likely impairs the central auditory system's ability to process complex sounds such as speech. PMID:17412539

  13. Interactions between amplitude modulation and frequency modulation processing: Effects of age and hearing loss.

    PubMed

    Paraouty, Nihaad; Ewert, Stephan D; Wallaert, Nicolas; Lorenzi, Christian

    2016-07-01

    Frequency modulation (FM) and amplitude modulation (AM) detection thresholds were measured for a 500-Hz carrier frequency and a 5-Hz modulation rate. For AM detection, FM at the same rate as the AM was superimposed with varying FM depth. For FM detection, AM at the same rate was superimposed with varying AM depth. The target stimuli always contained both amplitude and frequency modulations, while the standard stimuli only contained the interfering modulation. Young and older normal-hearing listeners, as well as older listeners with mild-to-moderate sensorineural hearing loss were tested. For all groups, AM and FM detection thresholds were degraded in the presence of the interfering modulation. AM detection with and without interfering FM was hardly affected by either age or hearing loss. While aging had an overall detrimental effect on FM detection with and without interfering AM, there was a trend that hearing loss further impaired FM detection in the presence of AM. Several models using optimal combination of temporal-envelope cues at the outputs of off-frequency filters were tested. The interfering effects could only be predicted for hearing-impaired listeners. This indirectly supports the idea that, in addition to envelope cues resulting from FM-to-AM conversion, normal-hearing listeners use temporal fine-structure cues for FM detection.

  14. Auditory brainstem response screening for hearing loss in high risk neonates.

    PubMed

    Watson, D R; McClelland, R J; Adams, D A

    1996-07-01

    The present paper reports the findings of a 7 year study evaluating the use of the auditory brainstem response (ABR) as the basis of a hearing screening procedure in a group of newborns at increased risk of hearing impairment. A Special Care Baby Unit (SCBU) population of 417 infants with diverse clinical backgrounds and treatment histories was tested for hearing impairment at birth using ABR audiometry. Some 332 passed the original screen at 30 dBnHL test level in both ears. Of the failure group, 18 did not survive and 32 had some degree of hearing impairment confirmed, nine of which were sensorineural in origin. An increased incidence of persistent middle ear disease was also noted in the failure group. A detailed operational analysis demonstrates that provided appropriate pass/fail criteria are adopted, the ABR technique offers excellent sensitivity and specificity for the detection of significant hearing loss in the test population. Furthermore, the study establishes that implementation of an ABR-based screening programme could reduce the average age at detection of permanent hearing loss by 7 months. A cost assessment shows that the introduction of such a targetted screening procedure could be done at a reasonable outlay. PMID:8818761

  15. Noise-Induced Hearing Loss

    MedlinePlus

    ... Info » Hearing, Ear Infections, and Deafness Noise-Induced Hearing Loss On this page: What is noise-induced hearing ... additional information about NIHL? What is noise-induced hearing loss? Every day, we experience sound in our environment, ...

  16. Working memory training for adult hearing aid users: study protocol for a double-blind randomized active controlled trial

    PubMed Central

    2013-01-01

    Background One in ten people aged between 55 to 74 years have a significant hearing impairment in their better hearing ear (as defined by audiometric hearing thresholds). However, it is becoming increasingly clear that the challenges faced by older listeners cannot be explained by the audiogram alone. The ability for people with hearing loss to use cognition to support speech perception allows for compensation of the degraded auditory input. This in turn offers promise for new cognitive-based rehabilitative interventions. Working memory is known to be highly associated with language comprehension and recent evidence has shown significant generalization of learning from trained working memory tasks to improvements in sentence-repetition skills of children with severe to profound hearing loss. This evidence offers support for further investigation into the potential benefits of working memory training to improve speech perception abilities in other hearing impaired populations. Methods/Design This is a double-blind randomized active controlled trial aiming to assess whether a program of working memory training results in improvements in untrained measures of cognition, speech perception and self-reported hearing abilities in adult hearing aid users (aged 50 to 74 years) with mild-to-moderate hearing loss, compared with an active control group who receive a placebo version of the working memory training program. Discussion The present study aims to generate high-quality preliminary evidence for the efficacy of working memory training for adults with mild-to-moderate sensorineural hearing loss who are existing hearing aid users. This trial addresses a number of gaps in the published literature assessing training interventions for people with hearing loss, and in the general literature surrounding working memory training, such as the inclusion of an active control group, participant and tester blinding, and increased transparency in reporting. Trial registration

  17. 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

  18. 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

  19. 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. PMID:26186969

  20. 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

  1. 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.

  2. 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 ...

  3. Living with Hearing Loss

    MedlinePlus

    ... Issues Special Section: Focus on Communication Living with Hearing Loss Past Issues / Fall 2008 Table of Contents ... family, including dad Bob, have adapted to her hearing impairment. Photo courtesy of Stefan Radtke, www.stefanradtke. ...

  4. 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.

  5. Can Baby Hear?

    MedlinePlus

    ... 000 children born in the United States are deaf or hard-of-hearing. Research shows that early ... to this, the average age of identification for deaf and hearing impaired children was close to three ...

  6. Genes and Hearing Loss

    MedlinePlus

    ... Meeting Calendar Find an ENT Doctor Near You Genes and Hearing Loss Genes and Hearing Loss Patient ... mutation may only have dystopia canthorum. How Do Genes Work? Genes are a road map for the ...

  7. Hearing Disorders and Deafness

    MedlinePlus

    ... impossible, to hear. They can often be helped. Deafness can keep you from hearing sound at all. ... certain medicines, and surgery. NIH: National Institute on Deafness and Other Communication Disorders

  8. Auditory Spatial Discrimination and the Mismatch Negativity Response in Hearing-Impaired Individuals

    PubMed Central

    Cai, Yuexin; Zheng, Yiqing; Liang, Maojin; Zhao, Fei; Yu, Guangzheng; Liu, Yu; Chen, Yuebo; Chen, Guisheng

    2015-01-01

    The aims of the present study were to investigate the ability of hearing-impaired (HI) individuals with different binaural hearing conditions to discriminate spatial auditory-sources at the midline and lateral positions, and to explore the possible central processing mechanisms by measuring the minimal audible angle (MAA) and mismatch negativity (MMN) response. To measure MAA at the left/right 0°, 45° and 90° positions, 12 normal-hearing (NH) participants and 36 patients with sensorineural hearing loss, which included 12 patients with symmetrical hearing loss (SHL) and 24 patients with asymmetrical hearing loss (AHL) [12 with unilateral hearing loss on the left (UHLL) and 12 with unilateral hearing loss on the right (UHLR)] were recruited. In addition, 128-electrode electroencephalography was used to record the MMN response in a separate group of 60 patients (20 UHLL, 20 UHLR and 20 SHL patients) and 20 NH participants. The results showed MAA thresholds of the NH participants to be significantly lower than the HI participants. Also, a significantly smaller MAA threshold was obtained at the midline position than at the lateral position in both NH and SHL groups. However, in the AHL group, MAA threshold for the 90° position on the affected side was significantly smaller than the MMA thresholds obtained at other positions. Significantly reduced amplitudes and prolonged latencies of the MMN were found in the HI groups compared to the NH group. In addition, contralateral activation was found in the UHL group for sounds emanating from the 90° position on the affected side and in the NH group. These findings suggest that the abilities of spatial discrimination at the midline and lateral positions vary significantly in different hearing conditions. A reduced MMN amplitude and prolonged latency together with bilaterally symmetrical cortical activations over the auditory hemispheres indicate possible cortical compensatory changes associated with poor behavioral spatial

  9. Human adenylate kinase 2 deficiency causes a profound haematopoietic defect associated with sensorineural deafness

    PubMed Central

    Lagresle-Peyrou, Chantal; Six, Emmanuelle M.; Picard, Capucine; Rieux-Laucat, Frédéric; Michel, Vincent; Ditadi, Andrea; Chappedelaine, Corinne Demerens-de; Morillon, Estelle; Valensi, Françoise; Simon-Stoos, Karen L.; Mullikin, James C.; Noroski, Lenora M.; Besse, Céline; Wulffraat, Nicolas M.; Ferster, Alina; Abecasis, Manuel M.; Calvo, Fabien; Petit, Christine; Candotti, Fabio; Abel, Laurent; Fischer, Alain; Cavazzana-Calvo, Marina

    2008-01-01

    Reticular dysgenesis (RD) is an autosomal recessive form of human Severe Combined Immunodeficiency, characterized by an early differentiation arrest in the myeloid lineage and impaired lymphoid maturation. In addition, affected newborns have bilateral sensorineural deafness. We have identified biallelic mutations in the adenylate kinase 2 (AK2) gene in seven patients affected with RD. These mutations resulted in the absence or a strong decrease in protein expression. We then demonstrated that restoration of AK2 expression in the bone marrow cells of RD patients overcomes the neutrophil differentiation arrest underlining its specific requirement in the development of a restricted set of haematopoietic lineages. Lastly, we established that AK2 is specifically expressed in the stria vascularis region of the inner ear, which provides an explanation to the sensorineural deafness. These results suggest a novel mechanism regulating haematopoetic cell differentiation, and involved in one of the most severe human immunodeficiency syndromes. PMID:19043416

  10. 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

  11. 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…

  12. The Hearing Mechanism

    ERIC Educational Resources Information Center

    Sherbon, James W.

    1978-01-01

    An examination of the hearing mechanism and some of the factors involved in helping problems may offer encouragement for a regular schedule of hearing maintenance. It may also help music educators to become more aware and understanding of their own and students' hearing as it affects musical behavior. (Author)

  13. 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.

  14. Hearing-aid tester

    NASA Technical Reports Server (NTRS)

    Kessinger, R.; Polhemus, J. T.; Waring, J. G.

    1977-01-01

    Hearing aids are automatically checked by circuit that applies half-second test signal every thirty minutes. If hearing-aid output is distorted, too small, or if battery is too low, a warning lamp is activated. Test circuit is incorporated directly into hearing-aid package.

  15. 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…

  16. Implementing Hearing Safety

    ERIC Educational Resources Information Center

    Cliffe, Roger

    1978-01-01

    Hearing damage from noise exposure and approaches to implementing hearing safety in school industrial laboratories through noise reduction and protective equipment are discussed. Although all states have not adopted the Occupational Safety and Health Act, teachers should be aware of noise hazards and act to protect hearing. (MF)

  17. 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 "Re-Education and…

  18. Hearing Problems in Children

    MedlinePlus

    ... before they are a month old. If your child has a hearing loss, it is important to consider the use of hearing devices and other communication options by age 6 months. That's because children start learning speech and language long before they talk. Hearing ...

  19. An ENU-Induced Mutation of Cdh23 Causes Congenital Hearing Loss, but No Vestibular Dysfunction, in Mice

    PubMed Central

    Manji, Shehnaaz S.M.; Miller, Kerry A.; Williams, Louise H.; Andreasen, Lotte; Siboe, Maria; Rose, Elizabeth; Bahlo, Melanie; Kuiper, Michael; Dahl, Hans-Henrik M.

    2011-01-01

    Mutations in the human cadherin 23 (CDH23) gene cause deafness, neurosensory, autosomal recessive 12 (DFNB12) nonsyndromic hearing loss or Usher syndrome, type 1D (characterized by hearing impairment, vestibular dysfunction, and visual impairment). Reported waltzer mouse strains each harbor a Cdh23-null mutation and present with hearing loss and vestibular dysfunction. Two additional Cdh23 mouse mutants, salsa and erlong, each carry a homozygous Cdh23 missense mutation and have progressive hearing loss. We report the identification of a novel mouse strain, jera, with inherited hearing loss caused by an N-ethyl-N-nitrosourea–induced c.7079T>A mutation in the Cdh23 gene. The mutation generates a missense change, p.V2360E, in Cdh23. Affected mice have profound sensorineural deafness, with no vestibular dysfunction. The p.V2360E mutation is semidominant because heterozygous mice have milder and more progressive hearing loss in advanced age. The mutation affects a highly conserved Ca2+-binding motif in extracellular domain 22, thought to be important for Cdh23 structure and dimerization. Molecular modeling suggests that the Cdh23V2360E/V2360E mutation alters the structural conformation of the protein and affects Ca2+-binding properties. Similar to salsa mice, but in contrast to waltzer mice, hair bundle development is normal in jera and hearing loss appears to be due to the loss of tip links. Thus, jera is a novel mouse model for DFNB12. PMID:21689626

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

    NASA Astrophysics Data System (ADS)

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

    2007-03-01

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