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Sample records for acquired sensorineural hearing

  1. Pediatric sensorineural hearing loss, part 2: syndromic and acquired causes.

    PubMed

    Huang, B Y; Zdanski, C; Castillo, M

    2012-03-01

    This article is the second in a 2-part series reviewing neuroimaging in childhood SNHL. Previously, we discussed the clinical work-up of children with hearing impairment, the classification of inner ear malformations, and congenital nonsyndromic causes of hearing loss. Here, we review and illustrate the most common syndromic hereditary and acquired causes of childhood SNHL, with an emphasis on entities that demonstrate inner ear abnormalities on cross-sectional imaging. Syndromes discussed include BOR syndrome, CHARGE syndrome, Pendred syndrome, Waardenburg syndrome, and X-linked hearing loss with stapes gusher. We conclude the article with a review of acquired causes of childhood SNHL, including infections, trauma, and neoplasms. PMID:21596810

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

    PubMed

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

    2015-02-01

    Eye color may be an indicator of inner ear melanin content and has been associated with hearing loss. There is controversy as to whether eye color has an effect on acquired causes of sensorineural hearing loss. This review was conducted to analyze the literature evaluating the relationship between eye color and causes of sensorineural hearing loss. Six databases were searched to identify eligible studies. Included articles were independently assessed for quality by two authors. Eighteen articles were eligible for review. Eye color was not found to have an effect in the non-exposed population or in presbycusis. In noise-induced sensorineural hearing loss, light-eyed patients had more significant loss following noise exposure, although the variability reported due to eye color was modest (r(2) = 0.01-0.14). Two out of three studies reported that dark eye color is associated with cisplatin ototoxicity. In one study, green-eyed patients were found to be at higher risk of radiation-induced hearing loss. Eye color does not appear to play a role in hearing loss in non-exposed individuals or presbycusis. It is possible that dark-eyed individuals, with greater inner ear melanin content, are better protected against noise-induced hearing loss. Evidence suggests that melanin can be protective against radiation-induced sensorineural hearing loss, but may predispose individuals to cisplatin ototoxicity. Future studies are required to support these conclusions. PMID:25529530

  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. Congenital sensorineural hearing loss

    SciTech Connect

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

    1984-02-01

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

  5. Sudden Sensorineural Hearing Loss

    PubMed Central

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

    2011-01-01

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

  6. 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. PMID:27171971

  7. 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

  8. Fluctuating sensorineural hearing loss in children.

    PubMed

    Brookhouser, Patrick E

    2002-08-01

    Childhood sensorineural hearing loss (SNHL) that fluctuates or is progressive enhances parental concern and complicates medical management, hearing aid selection, and individualized educational planning for the affected child. Despite intensive multidisciplinary evaluation and intervention, continued threshold fluctuation or a gradual decline in auditory acuity may proceed unabated in a significant percentage of these youngsters. With the adoption of universal newborn hearing screening mandates by an increasing number of states, any challenges to the accurate determination of auditory thresholds must be addressed within the first few months of life. PMID:12487089

  9. 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

  10. 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

  11. Sudden Sensorineural Hearing Loss; Prognostic Factors

    PubMed Central

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

    2015-01-01

    Introduction: Sudden sensorineural hearing loss (SSNHL) is a frightening and frustrating symptom for the patient as well as the physician. Prognosis is affected by multiple factors including duration of hearing loss, presence of associated vertigo and tinnitus, and co-morbidities such as hypertension and diabetes. Materials and Methods: Forty subjects presenting to our department with features of sudden hearing loss were included in the study. Detailed otological history and examination, serial audiometric findings and course of disease were studied. Results: Subjects presenting late (in older age), having associated vertigo, hypertension and diabetes had a significantly lower rate of recovery. Conclusion: Only 60–65% of patients experiencing SSNHL recover within a period of 1 month; this rate is further affected by presence of multiple prognostic indicators. PMID:26568939

  12. 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

  13. 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

  14. 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

  15. Sensorineural hearing loss in otitis media.

    PubMed

    Paparella, M M; Morizono, T; Le, C T; Mancini, F; Sipilä, P; Choo, Y B; Lidén, G; Kim, C S

    1984-01-01

    Additional evidence is presented to support the hypothesis that both acute purulent otitis media (POM) and chronic suppurative otitis media (COM) can cause high frequency sensorineural hearing loss. In selected patients and in animals (chinchillas) in a pilot study using electrophysiological methods, both temporary threshold shifts and permanent threshold shifts of basal cochlear turn involvement were demonstrated in POM. Data of cochlear involvement in 475 ears with bilateral COM, 607 ears with unilateral COM, and 607 ears serving as controls were obtained from six centers in five countries. In group 1 (15 dB or greater), 43% of ears with unilateral COM and 42% of ears with bilateral COM showed losses, for a combined odds ratio eight times that in controls. In group 2 (30 dB or greater), 16% of ears with unilateral COM and 17% of ears with bilateral COM demonstrated, respectively, seven and ten times that in controls. These statistically significant findings influence clinical considerations. PMID:6508134

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

    PubMed Central

    2009-01-01

    Introduction Lassa fever is an acute arena viral haemorrhagic fever with varied neurological sequelae. Sensorineural hearing loss is one of the rare complications which occur usually during the convalescent stage of the infection. Case presentation The cases of two female patients aged 19 and 43 years old, respectively, with clinical features suggestive of Lassa fever and confirmed by immunoserological/Lassa-virus-specific reverse transcriptase polymerase chain reaction are presented. Both patients developed severe sensorineural hearing loss at acute phases of the infections. Conclusion Sensorineural hearing loss from Lassa fever infections can occur in both acute and convalescent stages and is probably induced by an immune response. PMID:19178735

  17. 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

  18. [Steroid-responsive sensorineural hearing loss with low tone loss].

    PubMed

    Toriya, R; Yamashita, H; Hisashi, K; Komune, S; Komiyama, S

    1995-11-01

    Five cases of sensorineural hearing loss of sudden onset were reviewed. They were not responsive to administration of ATP and Vit. B12, but very responsive to steroid administration. All the patients were male and showed hearing loss in low frequencies in pure tone audiogram. Administration of steroid recovered hearing impairment immediately. However, cessation of steroid aggravated the recovered hearing. Serological and immunological examinations did not show any abnormal findings on all the patients. It was considered that these five patients had characteristics of both steroid-sensitive and low tone-loss type sensorineural hearing losses. PMID:8566929

  19. 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

  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. 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

  4. 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

  5. Sensorineural hearing loss associated with a factitious disorder.

    PubMed

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

    2015-12-01

    Factitious disorders are characterized by intentionally abnormal physical and/or psychological behavior, and affected patients often make up their symptoms and clinical histories. The most serious and chronic type of factitious disorder is Munchausen syndrome. We report the case of a 24-year-old woman with a 2-year history of sensorineural hearing loss (SNHL) who later confessed to feigning her hearing loss. She was eventually diagnosed with a factitious disorder. During those 2 years, she was able to induce her SNHL by exposing herself to excessive noise or high doses of aspirin. To the best of our knowledge, this is the first report describing an association between a factitious disorder and SNHL. PMID:26670765

  6. 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

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

    PubMed Central

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

    2016-01-01

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

  8. 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

  9. 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

  10. 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

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

    PubMed

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

    2016-01-01

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

  12. 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

  13. Sensorineural hearing loss in patients with Kawasaki disease

    PubMed Central

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

    2015-01-01

    Purpose Kawasaki disease involves acute febrile systemic vasculitis that can cause a variety of symptoms by affecting various organs. Here, we aimed to evaluate the prevalence, causes, and prognosis of sensorineural hearing loss (SNHL) occurring in children with Kawasaki disease. Methods Patients who were diagnosed with Kawasaki disease and received inpatient treatment in the Pediatrics Department at one of three university hospitals in Daegu city from February 2012 to September 2012 were enrolled in the study. The clinical features, hematological results, echocardiography results, audiometry results, and aspirin and salicylic acid serum levels of the patients were evaluated. Results Of the 59 children enrolled in the study, three showed mild bilateral SNHL on audiometry tests conducted after 48 hours of defervescence; these patients demonstrated normal patterns of recovery on follow-up tests 8 weeks later. Aspirin serum levels were significantly higher in the SNHL group after 48 hours of afebrile condition with high dose aspirin intake (P=0.034). However, no significant differences were found in other laboratory tests or for fever duration (P>0.05). Upon echocardiography, coronary artery abnormality was observed in 9 cases, but none of these patients showed hearing loss. Conclusion The results indicate that SNHL in children with Kawasaki disease might occur during treatment of the acute phase; this SNHL usually involves mild bilateral hearing loss and recovers naturally. However, this study suggests that determination of the causes and clinical implications of hearing loss in Kawasaki disease requires long-term follow-up studies with more cases. PMID:26692879

  14. 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

  15. Sudden post-traumatic sensorineural hearing loss reverted to normal by sneezing

    PubMed Central

    2014-01-01

    An 11-year-old child with sudden post-traumatic sensorineural hearing loss regained his hearing functions after sneezing. This case report is a first in medical literature in describing recovery from hearing loss by sneezing. The therapeutic implications of this rare case deserve further investigation. PMID:27489670

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

    PubMed Central

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

    2016-01-01

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

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

    PubMed

    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

  18. [The experience with the use of neuromedin preparations for the treatment of sensorineural impairment of hearing].

    PubMed

    Tambovtseva, O V; Platonova, I S; Vankova, S N; Ushkov, E V; Vorozheĭkina, N G; Turkina, T A

    2014-01-01

    The objective of the present study was to evaluate the effectiveness of the application of neuromedin for the treatment of sensorineural loss of hearing of different etiology. A total of 230 patients at the age varying from 20 to 60 years presenting with sensorineural impairment of hearing were available for the observation. Neuromedin was administered intramuscularly at 15 mg/day (1.5% solution at a dose of 1.0 ml) for 10 day; thereafter, the patients took 20 mg neuromedin tablets thrice daily during one month. The majority of the patients reported an improvement of hearing and speech intelligibility. Complete restoration of hearing was documented in certain patients presenting with acute sensorineural impairment of hearing. PMID:25588490

  19. 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

    2014-01-01

    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

  20. 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

  1. 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

  2. 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

  3. Spontaneous improvement in sensorineural hearing loss developed as a complication of neonatal hyperbilirubinemia.

    PubMed

    Khalid, Sameen; Qadir, Maqbool; Salat, Muhammad Sohail

    2015-09-01

    Icterus neonatorum, or neonatal jaundice, is defined as a total serum bilirubin level above 5mg/dl. Acute bilirubin encephalopathy and kernicterus are known to be the two major complications associated with it, resulting in neurotoxic effects, including sensorineural hearing loss, hypotonia, delayed motor skills and intellectual deficits. We report two similar cases of neonatal jaundice requiring exchange transfusion that subsequently developed sensorineural hearing loss. Follow-up at the end of 1year revealed spontaneous recovery of hearing with normal speech. This report aims at increasing awareness of this condition among physicians to allow early detection of risk factors and prompt management and follow-up. PMID:26338755

  4. 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…

  5. 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…

  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. PMID:26521189

  7. Sensorineural Hearing Loss – A Changing Paradigm for Its Evaluation

    PubMed Central

    Smith, Richard J.H.

    2016-01-01

    Objective 1) Determine how practicing clinicians evaluate patients with sensorineural hearing loss (SNHL), and 2) analyze the cost-effectiveness of current algorithms in the evaluation of these patients. Study Design/Setting An interactive online survey allowing respondents to order diagnostic testing in the evaluation of four simulated patients with SNHL across two testing encounters per patient. Subjects and Methods The survey was distributed to clinician members of the American Society of Pediatric Otolaryngology and the American Society of Human Genetics between May and August 2014. Statistical tests included Chi-square and non-parametric testing with Mann-Whitney U test. Results Otolaryngologists were significantly more likely to order repeat audiometric testing and significantly less likely to order genetic testing than other clinicians. Respondents who completed training more recently were significantly more likely to order MRI and EKG. On average, respondents spent $4,756 in the evaluation of a single patient, with otolaryngologists spending significantly more than other clinicians. CT of the temporal bone (40%), ophthalmology consultation (39%), and genetics consultation (37%) were ordered most frequently in the first encounter. Comprehensive genetic testing was ordered least frequently on the first encounter (20%) but was the most frequently ordered test on the second encounter (30%). Conclusion Recent guidelines advocate comprehensive genetic testing in the evaluation of patients with SNHL as early genetic testing can prevent uninformative additional tests which otherwise increase healthcare expenditures. Results from this survey indicate that comprehensive genetic testing is now frequently, but not uniformly, included in evaluation of patients with SNHL. PMID:26216887

  8. 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

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

    PubMed

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

    2011-02-01

    Lassa fever (LF) is a viral hemorrhagic disease which affects one-fourth to two million people annually with the fatality rate of about 10,000. It is associated with sensorineural hearing loss (SNHL) usually at the convalescent stage. Recently, cases of SNHL at the acute phase have been reported. This study was done to further investigate the incidence and features of SNHL in acute phase of LF. It is a prospective case-control study of LF patients seen with acute SNHL conducted between July 2007 and April 2009 at Irrua Specialist Teaching Hospital Nigeria. The diagnosis of acute LF was based on the clinical features and detection of IgM antibodies and/or positive Lassa virus-specific reverse transcriptase-polymerase chain reaction using primers S36+ and LVS 339 while SNHL was diagnosed clinically and confirmed with PTA and speech discrimination tests. Patients with other acute febrile illnesses were used as control. Statistical analysis was done using SPSS version 11 and Fisher's exact test while level of significance was set at p < 0.05. Out of the 37 confirmed cases of LF, 5 (13.5%) and none (0%) of the control developed early-onset SNHL (p = 0.03). Forty percent of the cases studied had negative IgM. The audiograms showed involvement at all frequency groups with pure tone average 65-85 dB and the speech discrimination 20-40%. The overall case fatality rate was 27.0%, and for early SNHL cases 60.0% (p > 0.05). The incidence of SNHL in LF infection is about 13.5% and could be a reflection of a worse disease process. There is possibility of direct viral invasion aside immunological reaction as a causative mechanism. PMID:20809263

  10. 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…

  11. 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…

  12. [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

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

    PubMed

    Ahmmed, Ansar; Brockbank, Christopher; Adshead, June

    2008-08-01

    The diagnostic dilemma surrounding the presence of cochlear microphonics (CM) coupled with significantly elevated auditory brainstem response (ABR) thresholds in babies failing the newborn hearing screening is highlighted. A case report is presented where initial electo-diagnostic assessment could not help in differentiating between Auditory Neuropathy/Auditory Dys-synchrony (AN/AD) and sensorineural hearing loss (SNHL). In line with the protocol and guidelines provided by the national Newborn Hearing Screening Programme in the UK (NHSP) AN/AD was suspected in a baby due to the presence of CM at 85 dBnHL along with click evoked ABR thresholds of 95 dBnHL in one ear and 100 dBnHL in the other ear. Significantly elevated thresholds for 0.5 and 1kHz tone pip ABR fulfilled the audiological diagnostic criteria for AN/AD. However, the possibility of a SNHL could not be ruled out as the 85 dBnHL stimuli presented through inserts for the CM would have been significantly enhanced in the ear canals of the young baby to exceed the threshold level of the ABR that was carried out using headphones. SNHL was eventually diagnosed through clinical and family history, physical examination and imaging that showed enlarged vestibular aqueducts. Presence of CM in the presence of very high click ABR thresholds only suggests a pattern of test results and in such cases measuring thresholds for 0.5 and 1 kHz tone pip ABR may not be adequate to differentiate between SNHL and other conditions associated with AN/AD. There is a need for reviewing the existing AN/AD protocol from NHSP in the UK and new research to establish parameters for CM to assist in the differential diagnosis. A holistic audiological and medical approach is essential to manage babies who fail the newborn hearing screening. PMID:18571245

  14. [The specific clinical features of acute sensorineural loss of hearing associated with vertigo].

    PubMed

    Pal'chun, V T; Guseva, A L; Levina, Yu V; Chistov, S D

    2016-01-01

    The objective of the present study was to estimate the prevalence of vertigo and to determine the type of the lesion of the vestibular analyzer in the patients presenting with acute sensorineural loss of hearing (ASNLH). The secondary objective was to evaluate the possibility of the restoration of the auditory thresholds. The results of the examination and treatment of 94 patients suffering from ASNLH are presented. It was shown that the development of acute sensorineural loss of hearing was accompanied by unilateral peripheral vestibulopathy in 22.3% of the patients. In 5.3% of these cases, dizziness could be attributed to benign paroxysmal positional vertigo and canalolythias is of the posterior semicircular canal on the side of hearing impairment. It is concluded that the presence of the clinical signs of lesions of the vestibular analyzer and peripheral vestibular dysfunction in the form of latent spontaneous nystagmus without gaze fixation and/or asymmetric nystagmus with unilateral weakness in the caloric test is a negative prognostic factor for the restoration of the auditory thresholds in the patients presenting with acute sensorineural loss of hearing. PMID:26977560

  15. Mutations in OTOGL, Encoding the Inner Ear Protein Otogelin-like, Cause Moderate Sensorineural Hearing Loss

    PubMed Central

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

    2012-01-01

    Hereditary hearing loss is characterized by a high degree of genetic heterogeneity. Here we present OTOGL mutations, a homozygous one base pair deletion (c.1430 delT) causing a frameshift (p.Val477Glufs∗25) in a large consanguineous family and two compound heterozygous mutations, c.547C>T (p.Arg183∗) and c.5238+5G>A, in a nonconsanguineous family with moderate nonsyndromic sensorineural hearing loss. OTOGL maps to the DFNB84 locus at 12q21.31 and encodes otogelin-like, which has structural similarities to the epithelial-secreted mucin protein family. We demonstrate that Otogl is expressed in the inner ear of vertebrates with a transcription level that is high in embryonic, lower in neonatal, and much lower in adult stages. Otogelin-like is localized to the acellular membranes of the cochlea and the vestibular system and to a variety of inner ear cells located underneath these membranes. Knocking down of otogl with morpholinos in zebrafish leads to sensorineural hearing loss and anatomical changes in the inner ear, supporting that otogelin-like is essential for normal inner ear function. We propose that OTOGL mutations affect the production and/or function of acellular structures of the inner ear, which ultimately leads to sensorineural hearing loss. PMID:23122586

  16. 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.

  17. 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

  18. 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

  19. Sensorineural deafness

    MedlinePlus

    ... at birth (congenital) is most often due to: Genetic syndromes Infections that the mother passes to her baby in the womb ( toxoplasmosis , rubella , herpes ) Sensorineural hearing loss may develop ...

  20. 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. PMID:27265992

  1. 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.

  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. [Clinical observations of acute low-tone sensorineural hearing loss considered as cochlear hydrops].

    PubMed

    Yamasoba, T; Sugasawa, T; Yagi, M; Harada, T; Futaki, T

    1990-02-01

    Acute low-tone sensorineural hearing loss (ALHL) has the following three criteria; obscure origin, acute onset and sensorineural hearing loss limited to low frequencies. Sixteen cases of ALHL which were considered as cochlear hydrops using glycerol test and electrocochleogram were studied. All patients visited our department within two weeks after onset and were followed up for one year or more after initial examination. The subjective symptoms, the character of vestibular and hearing impairment and prognosis of 16 cases with ALHL were also investigated. The results were as follows. 1. Patients complained of ear fullness rather than hearing impairment. Four patients were unaware of hearing loss. 2. Recruitment phenomenon was found in all of 15 cases examined. Vestibular findings were mostly normal, except that spontaneous nystagmus was found in two cases and head-shaking nystagmus in one. 3. Recurrence and fluctuation of hearing impairment occurred in 14 cases. Three cases had an attack of rotatory vertigo once and two has diagnosed as Meniere's disease later. 4. During three months prior to last examination, hearing was stabilized in nine cases and continued to fluctuate in seven cases. In the former, hearing was improved in four cases, unchanged in three, and worsened in two. 5. Two patients underwent an endolymphatic sac operation and have had a good prognosis. 6. The authors suggest that most of ALHL should be considered as transient cochlear hydrops because the subjective symptom and audiological and vestibular findings of our cases are similar to those of cases which were reported as ALHL by other investigators. According to the findings of glycerol test and electrocochleogram, endolymphatic hydrops in ALHL is considered to exist in all turns of cochlea. PMID:2348281

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

    PubMed

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

    2012-04-01

    We report a case of infarction of the anterior inferior cerebellar artery (AICA) with peripheral facial palsy following vertigo and acute sensorineural hearing loss. A 39-year-old female presented with vertigo and sudden hearing loss, tinnitus, and aural fullness of the right ear. An audiogram revealed a severe hearing loss at all tested frequencies in the right ear. Spontaneous nystagmus toward the left side was also observed. Otoneurological examinations showed sensorineural hearing loss of the right ear and horizontal and rotatory gaze nystagmus toward the left side, and a caloric reflex test demonstrated canal paresis. Initially, we diagnosed the patient for sudden deafness with vertigo. However, right peripheral facial palsy appeared 2 days later. An eye tracking test (ETT) and optokinetic pattern test (OKP) showed centralis abnormality. The patient's brain was examined by magnetic resonance imaging (MRI) and magnetic resonance angioglaphy (MRA) and showed an infarction localized in the pons and cerebellum. MRI and MRA revealed infarction of the right cerebellar hemisphere indicating occlusion of the AICA. Consequently, the patient was diagnosed with AICA syndrome but demonstrated regression following steroid and edaravone treatment. We suggest that performing MRI and MRA in the early stage of AICA syndrome is important for distinguishing cerebellar infarction resulting from vestibular disease. PMID:21862260

  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. PMID:25459416

  6. 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

  7. [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

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

    PubMed

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

    2013-01-01

    Sudden sensorineural hearing loss (SSNHL) can cause significant morbidity. Treatment with steroids can improve outcome. Delay in initiation of treatment reduces the chance to regain hearing. For this reason SSNHL is considered an emergency. Diagnosis is based on history, physical examination and a standard audiogram, the latter requiring specialized equipment and personnel. Standard audiogram may not be available at the time and place of patient presentation. A smartphone or tablet computer-based hearing test may aid in the decision to prescribe steroids in this setting. In this study the uHear™ hearing test application was utilized. The output of this ear-level air conduction hearing test is reported in hearing grades for 6 frequencies ranging from 250 to 6000 Hz. A total of 32 patients with unilateral SSNHL proven by a standard audiogram were tested. The results of standard and iPod hearing tests were compared. Based on the accepted criterion of SSNHL (at least 30 dB loss - or 2 hearing grades - in 3 consecutive frequencies) the test had a sensitivity of 0.76 and specificity of 0.91. Using a less stringent criterion of a loss of 2 hearing grades over at least 2 frequencies the sensitivity was 0.96 and specificity 0.86. The correlation coefficient for the comparison of the average hearing grade across the 6 measured frequencies of the study and standard audiogram was 0.83. uHear more accurately reflected hearing thresholds at mid and high tones. Similarly to previously published data, low frequency thresholds could be artificially elevated. In conclusion, uHear can be useful in the initial evaluation of patients with single-sided SSNHL by providing important information guiding the decision to initiate treatment before a standard audiogram is available. PMID:23689282

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

    PubMed

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

    2009-03-01

    This study asked whether or not listeners with sensorineural hearing loss have an impaired ability to use top-down attention to enhance speech intelligibility in the presence of interfering talkers. Listeners were presented with a target string of spoken digits embedded in a mixture of five spatially separated speech streams. The benefit of providing simple visual cues indicating when and/or where the target would occur was measured in listeners with hearing loss, listeners with normal hearing, and a control group of listeners with normal hearing who were tested at a lower target-to-masker ratio to equate their baseline (no cue) performance with the hearing-loss group. All groups received robust benefits from the visual cues. The magnitude of the spatial-cue benefit, however, was significantly smaller in listeners with hearing loss. Results suggest that reduced utility of selective attention for resolving competition between simultaneous sounds contributes to the communication difficulties experienced by listeners with hearing loss in everyday listening situations. PMID:19009321

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

    PubMed Central

    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

  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. Loss of osteoprotegerin expression in the inner ear causes degeneration of the cochlear nerve and sensorineural hearing loss.

    PubMed

    Kao, Shyan-Yuan; Kempfle, Judith S; Jensen, Jane B; Perez-Fernandez, Deborah; Lysaght, Andrew C; Edge, Albert S; Stankovic, Konstantina M

    2013-08-01

    Osteoprotegerin (OPG) is a key regulator of bone remodeling. Mutations and variations in the OPG gene cause many human diseases that are characterized by not only skeletal abnormalities but also poorly understood hearing loss: Paget's disease, osteoporosis, and celiac disease. To gain insight into the mechanisms of hearing loss in OPG deficiency, we studied OPG knockout (Opg(-/-)) mice. We show that they develop sensorineural hearing loss, in addition to conductive hearing loss due to abnormal middle-ear bones. OPG deficiency caused demyelination and degeneration of the cochlear nerve in vivo. It also activated ERK, sensitized spiral ganglion cells (SGC) to apoptosis, and inhibited proliferation and survival of cochlear stem cells in vitro, which could be rescued by treatment with exogenous OPG, an ERK inhibitor, or bisphosphonate. Our results demonstrate a novel role for OPG in the regulation of SGC survival, and suggest a mechanism for sensorineural hearing loss in OPG deficiency. PMID:23607938

  13. Risk factors in the genesis of sensorineural hearing loss in Finnish forestry workers.

    PubMed Central

    Pyykkö, I; Koskimies, K; Starck, J; Pekkarinen, J; Färkkilä, M; Inaba, R

    1989-01-01

    A detailed analysis of risk factors for the development of sensorineural hearing loss (SNHL) was carried out in 199 forest workers. The hearing threshold of both ears at 4000 Hz was measured, and the effect of age, exposure to noise, blood pressure, presence of vibration induced white finger (VWF), tobacco smoking, plasma LDL-cholesterol concentration, and consumption of drugs were evaluated by multiple linear regression analysis. Aging was the major risk factor, followed by exposure to occupational noise and the presence of VWF. Plasma LDL-cholesterol concentration and the use of antihypertensive drugs also correlated significantly with SNHL. These main factors were able to explain about 28% of the SNHL variance. Additional factors in the analysis, including smoking, systolic and diastolic blood pressure, and consumption of salicylates did not significantly contribute to the genesis of SNHL. PMID:2765417

  14. The dichotic lead effect of CV syllables in sensorineural hearing loss.

    PubMed

    Cattey, T J

    1981-01-01

    This study investigated central processing of dichotic CV syllables on 10 patients with sensorineural hearing losses. Porter's Dichotic Lag Index (DLI) was established for each S at asynchronies of alignment of 30, 60, 90, and 150 msec by presenting the lagging syllable first to the R and then to the L ear. Ss were required to record a response to both CV syllables. Patients exhibited a dichotic lead advantage while 10 normal-hearing control Ss exhibited a dichotic lag advantage. A R ear advantage was not abolished when the lagging syllable was presented to the L ear and was not enhanced when the lagging syllable was presented to the R ear. The lead advantage of the patients may be attributed to the distortion of acoustic encoding for speech, causing a reduction in the special processing requirements of CV syllables. The reduction in special processing may then be responsible for the shift in temporal advantage. PMID:7349869

  15. 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

  16. 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

  17. 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

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

    PubMed

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

    2016-01-28

    Previous studies often report that early auditory deprivation or congenital deafness contributes to cross-modal reorganization in the auditory-deprived cortex, and this cross-modal reorganization limits clinical benefit from cochlear prosthetics. However, there are inconsistencies among study results on cortical reorganization in those subjects with long-term unilateral sensorineural hearing loss (USNHL). It is also unclear whether there exists a similar cross-modal plasticity of the auditory cortex for acquired monaural deafness and early or congenital deafness. To address this issue, we constructed the directional brain functional networks based on entropy connectivity of resting-state functional MRI and researched changes of the networks. Thirty-four long-term USNHL individuals and seventeen normally hearing individuals participated in the test, and all USNHL patients had acquired deafness. We found that certain brain regions of the sensorimotor and visual networks presented enhanced synchronous output entropy connectivity with the left primary auditory cortex in the left long-term USNHL individuals as compared with normally hearing individuals. Especially, the left USNHL showed more significant changes of entropy connectivity than the right USNHL. No significant plastic changes were observed in the right USNHL. Our results indicate that the left primary auditory cortex (non-auditory-deprived cortex) in patients with left USNHL has been reorganized by visual and sensorimotor modalities through cross-modal plasticity. Furthermore, the cross-modal reorganization also alters the directional brain functional networks. The auditory deprivation from the left or right side generates different influences on the human brain. PMID:26621123

  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. 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

  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. Concurrent hyperbaric oxygen therapy and intratympanic steroid application as salvage therapy after severe sudden sensorineural hearing loss.

    PubMed

    Lamm, Hans; Müller-Kortkamp, Claus; Warnecke, Athanasia; Pohl, Friederike; Paasche, Gerrit; Lenarz, Thomas; Stolle, Stefan R O

    2016-03-01

    Concurrent hyperbaric oxygen therapy (HBOT) and intratympanic steroid application (ITS) are beneficial as salvage therapy for therapy-refractory sudden sensorineural hearing loss (SSNHL). The findings encourage further research on the treatment of noise-induced and idiopathic SSNHL with concurrent use of HBOT and ITS respecting also patients with long-term or therapy-refractory SSNHL. PMID:27014454

  3. Strategy for the customized mass screening of genetic sensorineural hearing loss in koreans.

    PubMed

    Chang, Mun Young; Choi, Byung Yoon

    2014-09-01

    Hearing loss is one of the most common sensorineural disorder. More than half of congenital bilateral profound deafness cases have been estimated to be attributed to genetic cause. Identification of genetic cause can provide valuable information. We developed new diagnostic strategy combining phenotype-driven candidate gene approach and targeted exome sequencing to find out the causative mutation of hearing loss. The causative mutation detection rates of this strategy were 78.1% and 54.8% in Korean multiplex families and sporadic severe to profound hearing loss families, respectively. The most frequent causative genes of Korean multiplex families were SLC26A4 and POU3F4. The other causative genes were MRNR1, WFS1, COCH, TECTA, MYO6, COL11A2, EYA4, GJB3, OTOF, STRC, MYO3A, and GJB2. The most frequent causative gene of Korean sporadic severe to profound hearing loss families was SLC26A4 followed by GJB2, CHD7, and CDH23. Based upon the results, the value of this strategy as a diagnostic tool seems to be promising. Although whole genome and exome sequencing have advanced as the development of next-generation sequencing, this new strategy could be a good screening and diagnostic tool to find the causative mutations. PMID:25279224

  4. Strategy for the Customized Mass Screening of Genetic Sensorineural Hearing Loss in Koreans

    PubMed Central

    Chang, Mun Young

    2014-01-01

    Hearing loss is one of the most common sensorineural disorder. More than half of congenital bilateral profound deafness cases have been estimated to be attributed to genetic cause. Identification of genetic cause can provide valuable information. We developed new diagnostic strategy combining phenotype-driven candidate gene approach and targeted exome sequencing to find out the causative mutation of hearing loss. The causative mutation detection rates of this strategy were 78.1% and 54.8% in Korean multiplex families and sporadic severe to profound hearing loss families, respectively. The most frequent causative genes of Korean multiplex families were SLC26A4 and POU3F4. The other causative genes were MRNR1, WFS1, COCH, TECTA, MYO6, COL11A2, EYA4, GJB3, OTOF, STRC, MYO3A, and GJB2. The most frequent causative gene of Korean sporadic severe to profound hearing loss families was SLC26A4 followed by GJB2, CHD7, and CDH23. Based upon the results, the value of this strategy as a diagnostic tool seems to be promising. Although whole genome and exome sequencing have advanced as the development of next-generation sequencing, this new strategy could be a good screening and diagnostic tool to find the causative mutations. PMID:25279224

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

    PubMed

    Psillas, G; Daniilidis, M; Gerofotis, A; Veros, K; Vasilaki, A; Vital, I; Markou, K

    2013-01-01

    Sudden sensorineural hearing loss may be present as a symptom in systemic autoimmune diseases or may occur as a primary disorder without another organ involvement (autoimmune inner ear disease). The diagnosis of autoimmune inner ear disease is still predicated on clinical features, and to date specific diagnostic tests are not available. We report a case of bilateral sudden hearing loss, tinnitus, intense rotatory vertigo, and nausea in a female patient in which the clinical manifestations, in addition to raised levels of circulating immune complexes, antithyroglobulin antibodies, and the presence of the HLA A1-B8-DR3 haplotype, allowed us to hypothesize an autoimmune inner ear disease. Cyclosporine-A immunosuppressive treatment in addition to steroids helped in hearing recovery that occurred progressively with normalization of the hearing function after a five-month treatment. Cyclosporine-A could be proposed as a therapeutic option in case of autoimmune inner ear disease allowing the suspension of corticosteroids that, at high dose, expose patients to potentially serious adverse events. PMID:24106629

  6. Mutation in the transcriptional coactivator EYA4 causes dilated cardiomyopathy and sensorineural hearing loss.

    PubMed

    Schönberger, Jost; Wang, Libin; Shin, Jordan T; Kim, Sang Do; Depreux, Frederic F S; Zhu, Hao; Zon, Leonard; Pizard, Anne; Kim, Jae B; Macrae, Calum A; Mungall, Andy J; Seidman, J G; Seidman, Christine E

    2005-04-01

    We identified a human mutation that causes dilated cardiomyopathy and heart failure preceded by sensorineural hearing loss (SNHL). Unlike previously described mutations causing dilated cardiomyopathy that affect structural proteins, this mutation deletes 4,846 bp of the human transcriptional coactivator gene EYA4. To elucidate the roles of eya4 in heart function, we studied zebrafish embryos injected with antisense morpholino oligonucleotides. Attenuated eya4 transcript levels produced morphologic and hemodynamic features of heart failure. To determine why previously described mutated EYA4 alleles cause SNHL without heart disease, we examined biochemical interactions of mutant Eya4 peptides. Eya4 peptides associated with SNHL, but not the shortened 193-amino acid peptide associated with dilated cardiomyopathy and SNHL, bound wild-type Eya4 and associated with Six proteins. These data define unrecognized and crucial roles for Eya4-Six-mediated transcriptional regulation in normal heart function. PMID:15735644

  7. [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

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

    PubMed

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

    2006-01-01

    Haploinsufficiency of the zinc finger transcription factor GATA3 causes the triad of hypoparathyroidism, deafness and renal dysplasia, known by its acronym HDR syndrome. The purpose of the current study was to describe in detail the auditory phenotype in human HDR patients and compare these to audiometrical and histological data previously described in a mouse model of this disease. Pure tone audiometry, speech audiometry, speech in noise, auditory brainstem responses and transiently evoked otoacoustic emissions were measured in 2 patients affected by HDR syndrome. Both patients were affected by a moderate-to-severe sensorineural hearing loss. Speech reception thresholds were shifted and speech recognition in noise was disturbed. No otoacoustic emissions could be generated in either patient. Auditory brainstem response interpeak intervals were normal. The human and murine audiological phenotypes seem to correspond well. Hearing loss in HDR syndrome is moderate to severe, seems to be slightly worse at the higher end of the frequency spectrum and may be progressive with age. The absence of otoacoustic emissions and the loss of frequency selectivity suggest an important role for outer hair cells in causing the hearing loss. PMID:16988501

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

    PubMed Central

    Smith, Sandra Nelson; Lucas, Laura

    2016-01-01

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

  10. 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. PMID:25217083

  11. 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. PMID:25318685

  12. 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

  13. [A case of steroid psychosis caused by treatment for acute sensorineural hearing loss].

    PubMed

    Sato, Yuki; Masuda, Masatsugu; Kohno, Naoyuki

    2013-08-01

    A 36 y/o female presented with the chief complaint of diarrhea and vomiting which had lasted for four days, and with a family history of suicide. The first general examination showed severe dehydration with hyponatremia. After admission, she was diagnosed as having isolated adrenocorticotropic hormone (ACTH) deficiency and mixed connective tissue disease, and the steroid replacement therapy was started with the dose equivalent to 7.5 mg/day of prednisolone (PSL). Three days later, she had right sensorineural hearing loss (SNHL). She was given 40 mg/day PSL in addition to the steroid replacement therapy. On the next day, she developed a persecutory type of paranoid disorder, and then was given psychiatric medication. After tapering off PSL for SNHL, the delusion began to improve with psychiatric medication. Three weeks after the onset of SNHL, her hearing level had partially recovered. Ten months later, she did not show any psychic instability. A family history of psychosis and the present history of malnutrition and connective tissue disease are risk factors of steroid psychosis. It can develop even with 5 mg PSL if the patient has a risk factor. Careful medical history taking and knowledge about the steroid psychosis will prevent the severe side effects associated with steroid treatment. PMID:24044173

  14. 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. PMID:24519034

  15. 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

  16. 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

  17. 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

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

    PubMed

    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

  19. 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. PMID:25440412

  20. Polygenic inheritance of sensorineural hearing loss (Snhl2, -3, and -4) and organ of Corti patterning defect in the ALR/LtJ mouse strain.

    PubMed

    Latoche, Joseph R; Neely, Harold R; Noben-Trauth, Konrad

    2011-05-01

    Progressive sensorineural hearing loss in humans is a common and debilitating impairment. Sensorineural deafness in inbred strains of mice is a similarly common and genetically diverse phenotype providing experimental models to study the underlying genetics and the biological effects of the risk factors. Here, we report that ALR/LtJ mice develop early-onset profound sensorineural hearing loss as evidenced by high-to-low frequency hearing threshold shifts, absent distortion-product otoacoustic emissions, and normal endocochlear potentials. Linkage analyses of a segregating backcross revealed three novel quantitative trait loci named sensorineural hearing loss (Snhl) -2, -3, and -4. The QTLs achieved very high LOD scores with markers on chromosome 1 (Snhl2, LOD: 12), chromosome 6 (Snhl3, LOD: 24) and chromosome 10 (Snhl4, LOD: 11). Together, they explained 90% of the phenotypic variance. While Snhl2 and Snhl3 affected hearing thresholds across a broad range of test frequencies, Snhl4 caused primarily high-frequency hearing loss. The hearing impairment is accompanied by an organ of Corti patterning defect that is characterized by the ectopic expression of supernumerary outer hair cells organized in rows along the abneural site of the sensory epithelium in the presence of unaltered planar polarity and otherwise normal cochlear duct morphology. Cloning the Snhl2, -3, and -4 genes in the ALR/LtJ mice may provide important genetic and mechanistic insights into the pathology of human progressive sensorineural deafness. PMID:21185929

  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. [A case of ipsilateral ageusia, sensorineural hearing loss and facial sensorimotor disturbance due to pontine lesion].

    PubMed

    Sato, K; Nitta, E

    2000-05-01

    We report a 58-year-old woman with pontine lesion presented with subacute onset of unilateral gustatory disturbance accompanied by facial numbness, and hearing loss. Neurologic examination revealed superficial hypesthesia and paresthesia on the right side of the face, right peripheral type facial paresis, ageusia on the right half of the tongue and right sensorineural deafness. No other neurologic signs were observed, and laboratory data were all normal. Brain MRI revealed a small lesion in the right dorsolateral tegmentum of the middle pons. Electrogustometry showed marked reduction in the sense of taste on the right half of the tongue. ABR showed diminished amplitude in the IV-V wave of the right side, while SEP and VEP were normal. The clinical diagnosis was demyelinating lesion and intravenous methylprednisolone (1 g/day) was administered for 3 consecutive days, resulting in prompt improvement in the symptoms. The lesion was suspected of affecting ipsilateral side of the spinal trigeminal nerve tract and the nucleus, the intraaxial infranuclear facial nerve fiber, the lateral lemniscus adjacent to the superior olivary nucleus and the central gustatory tract. Our case suggests that the central gustatory pathway projecting from the nucleus of the solitary tract to the parabrachial nucleus, presumed to be pontine taste area, ascends ipsilaterally and is located laterally from the medial lemniscus. PMID:11002734

  3. 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. PMID:21416394

  4. 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

  5. Study of 100 patients with bilateral sensorineural hearing loss for lipid abnormalities.

    PubMed

    Lowry, L D; Isaacson, S R

    1978-01-01

    One hundred persons were selected from our audiology records who showed at least 20 dB bilateral sensorineural hearing loss. The number 100 was picked for an adequate statistical analysis. The patients were taken from the chart files which are filed in chronological order. Letters were mailed with instructions for fasting 14 hours prior to testing except for the consumption of water. On presentation, a history was taken for diabetes, height and weight, and the blood pressure was recorded. The blood samples were drawn in clot tubes and taken to a commercial laboratory for overnight refrigeration and testing of the serum. Testing included observation of serum for massive chylomicronemia, triglycerides, cholesterol and lipoprotein electrophoresis (by the cellulose acetate method). Lipoprotein testing results showed 12 patients with Type II A or II B and 8 patients with Type IV abnormalities. No Types I, III or V were found. These 20 patients represent a lower number of hyperlipoproteinemic patients than would be expected in the general population. PMID:207213

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

    PubMed Central

    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. PMID:26309671

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

    PubMed Central

    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. PMID:25789325

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

    ERIC Educational Resources Information Center

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

    2013-01-01

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

  11. 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.

  12. Application of Massively Parallel Sequencing to Genetic Diagnosis in Multiplex Families with Idiopathic Sensorineural Hearing Impairment

    PubMed Central

    Wu, Chen-Chi; Lin, Yin-Hung; Lu, Ying-Chang; Chen, Pei-Jer; Yang, Wei-Shiung; Hsu, Chuan-Jen; Chen, Pei-Lung

    2013-01-01

    Despite the clinical utility of genetic diagnosis to address idiopathic sensorineural hearing impairment (SNHI), the current strategy for screening mutations via Sanger sequencing suffers from the limitation that only a limited number of DNA fragments associated with common deafness mutations can be genotyped. Consequently, a definitive genetic diagnosis cannot be achieved in many families with discernible family history. To investigate the diagnostic utility of massively parallel sequencing (MPS), we applied the MPS technique to 12 multiplex families with idiopathic SNHI in which common deafness mutations had previously been ruled out. NimbleGen sequence capture array was designed to target all protein coding sequences (CDSs) and 100 bp of the flanking sequence of 80 common deafness genes. We performed MPS on the Illumina HiSeq2000, and applied BWA, SAMtools, Picard, GATK, Variant Tools, ANNOVAR, and IGV for bioinformatics analyses. Initial data filtering with allele frequencies (<5% in the 1000 Genomes Project and 5400 NHLBI exomes) and PolyPhen2/SIFT scores (>0.95) prioritized 5 indels (insertions/deletions) and 36 missense variants in the 12 multiplex families. After further validation by Sanger sequencing, segregation pattern, and evolutionary conservation of amino acid residues, we identified 4 variants in 4 different genes, which might lead to SNHI in 4 families compatible with autosomal dominant inheritance. These included GJB2 p.R75Q, MYO7A p.T381M, KCNQ4 p.S680F, and MYH9 p.E1256K. Among them, KCNQ4 p.S680F and MYH9 p.E1256K were novel. In conclusion, MPS allows genetic diagnosis in multiplex families with idiopathic SNHI by detecting mutations in relatively uncommon deafness genes. PMID:23451214

  13. 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

  14. 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

  15. Imaging Evaluation of Pediatric Sensorineural Hearing Loss in Potential Candidates for Cochlear Implantation.

    PubMed

    Jallu, Aleena Shafi; Jehangir, Majid; Ul Hamid, Waqar; Pampori, Rafiq Ahmad

    2015-12-01

    Computerized tomography (CT) and magnetic resonance (MR) are complementary in the imaging of the labyrinth, the internal auditory canal and the brain in children with sensorineural hearing loss who are being evaluated for cochlear implantation. An accurate anatomical description of the inner ear is essential in the preoperative work up. Computerized tomography visualizes the bony structures, whereas MR can discern soft-tissue components including intra labyrinthine fluid, cerebrospinal fluid (CSF), nerves, and vessels within the IAC. This prospective study was conducted in the Department of Otorhinolaryngology, Head & Neck Surgery, Government Medical College, Srinagar. 40 children in the age group of 1-16 years with unidentified causes of bilateral SNHL were analysed radiologically over the period of 2 years from Dec 2011 to Jan 2014. Each patient underwent MRI and high resolution CT scanning of temporal bone in axial and coronal planes. Out of the 40 patients 22 were males (55 %) and 18 were females (45 %). 30 patients (72.5 %)in our study had normal radiological scans. Five patients (12.5 %) had B/L large vestibular aqueduct and two patients (5 %) had internal auditory canal stenosis with cochlear nerve hypoplasia on CT and MR imaging. Cochlear dysplasia was present in two patients (5 %) and semicircular canal dysplasia was present in one patient (2.5 %) as an isolated finding on HRCT. In addition isolated cochlear nerve hypoplasia was present in one patient (2.5 %). Hyperintense basal ganglia lesion suggestive of kernicterus was present in one patient (2.5 %) and hyperintense posterior parietal and occipital white matter lesions suggestive of congenital CMV infection was present in one patient (2.5 %) on MR imaging. Arachnoid cysts of middle cranial fossa was an incidental finding present in one patient. Radiological abnormalities of the inner ear are not uncommon. Computerized tomography and MRI are important modalities to analyze the inner ear in

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

    PubMed

    Agrawal, Swati; Sharma, Nishi

    2016-01-01

    Idiopathic sudden sensorineural hearing loss (ISSHL), a common otologic emergency, presents mostly as an abrupt onset unilateral hearing loss, aural fullness, often with vertigo and tinnitus, usually upon awakening in the morning. Its etiopathogenesis is multifactorial, so a number of different therapeutic regimens are in practice, hyperbaric oxygen (HBO2) therapy being an effective yet underutilized one. Not all cases recover completely even after treatment. Here we describe two cases of ISSHL, diagnosed on the basis of clinical examination and pure-tone audiometry, who had a complete recovery following administration of HBO2 therapy in addition to medical treatment. These cases are reported to highlight the effectiveness of this modality in a physician's armamentarium. PMID:27265993

  17. Factors Affecting the Variation of Maximum Speech Intelligibility in Patients With Sensorineural Hearing Loss Other Than Apparent Retrocochlear Lesions

    PubMed Central

    Yahata, Izumi; Miyazaki, Hiromitsu; Takata, Yusuke; Yamauchi, Daisuke; Nomura, Kazuhiro; Katori, Yukio

    2015-01-01

    Objectives To examine the relationship between speech intelligibilities among the similar level of hearing loss and threshold elevation of the auditory brainstem response (ABR). Methods The relationship between maximum speech intelligibilities among similar levels of hearing loss and relative threshold elevation of the click-evoked ABR (ABR threshold - pure tone average at 2,000 and 4,000 Hz) was retrospectively reviewed in patients with sensorineural hearing loss (SNHL) other than apparent retrocochlear lesions as auditory neuropathy, vestibular schwannoma and the other brain lesions. Results Comparison of the speech intelligibilities in subjects with similar levels of hearing loss found that the variation in maximum speech intelligibility was significantly correlated with the threshold elevation of the ABR. Conclusion The present results appear to support the idea that variation in maximum speech intelligibility in patients with similar levels of SNHL may be related to the different degree of dysfunctions of the inner hair cells and/or cochlear nerves in addition to those of outer hair cells. PMID:26330909

  18. 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

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

    PubMed Central

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

    2010-01-01

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

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

    SciTech Connect

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

    2009-03-01

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

  1. 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)

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

    PubMed Central

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

    2008-01-01

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

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

    PubMed

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

    2016-05-01

    Conclusion Sudden sensorineural hearing loss (SSNHL) in pregnancy is rare. It usually occurs in the third trimester. SSNHL in pregnancy does not increase risks during delivery or subsequent stroke. Objectives This study aimed to investigate the incidence and to determine the factors associated with SSNHL in pregnancy. Method Data were retrieved from Taiwan's National Health Insurance Database (NHIRD), covering the years 2000-2009. Patients admitted for SSNHL during pregnancy were enrolled. An age-matched controlled cohort was randomly selected from pregnant women without SSNHL in the NHIRD. The clinical characteristics of both cohorts were collected for further analyses. Results Thirty-three patients with SSNHL in pregnancy were enrolled. The estimated incidence of SSNHL in pregnancy in Taiwan was 2.71 per 100,000 pregnancies. The incidence of SSNHL in pregnancy was lower than that of the general female population. The incidence of SSNHL in the third trimester was higher compared to the other two. The incidence of SSNHL occurring in the 30-39 years old age group was higher than other groups. Women with better socioeconomic status had a higher incidence of SSNHL. There were no identified systemic diseases before SSNHL. Two patients had pre-eclampsia and one patient had premature delivery. Nevertheless, SSNHL in pregnancy did not increase the risk for stroke. PMID:27052963

  4. Bilateral Sensorineural Hearing Loss and Polyneuropathy in a Patient with Sweet's Syndrome

    PubMed Central

    Cala, Cather M.; Kole, Lauren; Sami, Naveed

    2015-01-01

    Sweet's syndrome is an inflammatory systemic disease which has been associated with various underlying causes. The disease can involve multiple areas of the body including the skin and neurological system. There have been only two cases which have described otological involvement. This report presents a patient who developed loss of hearing secondary to Sweet's syndrome after developing cutaneous involvement along with peripheral neuropathy. Despite the patient's skin and neuropathy noticing improvement with intravenous immunoglobulin and azathioprine, he required bilateral cochlear implants for partial recovery of his hearing loss. This case highlights the need to recognize Sweet's syndrome as a complicated disease process where the role of otolaryngologists is important in the multidisciplinary coordination of care in both diagnosis and treatment. PMID:26770858

  5. 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. PMID:26235980

  6. 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. PMID:25987500

  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. 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

  9. Evaluation of the Endothelial Cell Antibodies in Serum and Perilymphatic Fluid of Cochlear Implanted Children with Sensorineural Hearing Loss

    PubMed Central

    Farhadi, Mohammad; Noorbakhsh, Samileh; Tabatabaei, Azardokht; Daneshi, Ahamad; Darestani, Sahar Ghavidel; Jomeh, Emam

    2013-01-01

    Introduction Serum Anti endothelial Cell Antibodies (AECAs) play a prominent role in idiopathic Sensorineural Hearing Loss (SNHL) in that they induce vascular damage (immune mediated). The of the current study is To compare AECAs in serum and perilymphatic fluid of idiopathic SNHL children (<15y) undergoing cochlear implant surgery. Methods This was a cross sectional study performed in the cochlear implant ward in Rasoul Akram hospital, Tehran, Iran (2008 -2010) on 99 SNHL children undergoing cochlear implant surgery. The data collected from47 idiopathic and 52 non-idiopathic SNHL cases. AECAs were measured by indirect immuno fluorescence assay and compared in sera and perilymphatic fluids between the two groups. P-value < 0.05 was considered significant. Results Idiopathic SNHL was diagnosed in 47.5% of cases. Positive AECA results in serum and perilymphatic fluid were 10% and 12%, respectively. Although AECA results in perilymphatic fluids were different between idiopathic and non-Idiopathic SNHL patients (PV < 0.05), AECAs in serum showed no significant difference between the two (PV = 0.1). No significant difference was detected between the mean age of idiopathic and non-idiopathic SNHL patients with positive AECAs in serum and perilymphatic fluids (PV = 0.2; PV = 0.2). Discussion Idiopathic SNHL was diagnosed in 47.5% of studied cases. Idiopathic SNHL has a poor out come in children. In cases with idiopathic SNHL, finding AECAs in perilymphatic fluids are more valuable than in the serum. We suggest that serum and perilymphatic fluids testing for AECAs would be helpful in management of idiopathic SNHL cases. Specific immunosuppressive treatments for selected cases suffering from Idiopathic SNHL (only in those older than 5) might be successful in disease management. However, this theory should first be validated by randomized clinical trials. PMID:25337353

  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. 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. PMID:25138102

  12. 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

  13. Risk Factors for Sensorineural Hearing Loss Among High-Risk Infants in Golestan Province, Iran in 2010 - 2011

    PubMed Central

    Alaee, Ehsan; Sirati, Mohsen; Taziki, Mohammad Hossein; Fouladinejad, Mahnaz

    2015-01-01

    Background: Hearing impairment, as one of the most common birth defects, is a hidden disability with negative impacts on speech and cognitive development. Objectives: The aim of this study was to assess the prevalence of sensorineural hearing loss (SNHL) and determine the associated risk factors among infants admitted to neonatal intensive care units (NICUs) and neonatal wards of teaching hospitals, affiliated to Golestan University of Medical Sciences, Gorgan, Iran. Patients and Methods: In this cross-sectional study, 791 infants were recruited via non-random sampling. Demographic and clinical characteristics of the subjects were gathered, and the Automated Auditory Brainstem Response (AABR) test was performed upon admission. Afterwards, the subjects were followed-up and re-assessed, using the AABR test. For infants with abnormal AABR results, the Auditory Brainstem Response (ABR) test was performed on the day of discharge. Results: The mean age of the infants was 3.75 ± 4.86 days upon admission, and 56.4% of the subjects were female. The mean length of hospital stay was 9.63 ± 1.1 days; the subjects were hospitalized for 3.50 ± 10.21 days in the NICUs and 6.1 ± 5.27 days in the neonatal wards. In total, 3.4% of the infants presented with SNHL. No significant difference was found between SNHL and neonates’ age (P = 0.52), sex (P = 0.5), or sepsis (P = 0.94). However, SNHL was significantly associated with gestational age (P = 0.045), birth weight (P < 0.001), length of hospital stay (P < 0.001), pathological jaundice (P=0.033), antibiotic treatments (P = 0.007), and total serum bilirubin level (P = 0.01). Additionally, binary logistic regression analysis demonstrated the association between SNHL and these factors. Conclusions: In this study, the prevalence of SNHL among hospitalized neonates was similar to previous reports in Iran and other countries. Based on the findings, administration of ototoxic drugs during the neonatal period can lead to SNHL

  14. 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

  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. 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

  18. 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. PMID:23254396

  19. 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. PMID:25987501

  20. A novel mutation of EYA4 in a large Chinese family with autosomal dominant middle-frequency sensorineural hearing loss by targeted exome sequencing.

    PubMed

    Sun, Yi; Zhang, Zhao; Cheng, Jing; Lu, Yu; Yang, Chang-Liang; Luo, Yan-Yun; Yang, Guang; Yang, Hui; Zhu, Li; Zhou, Jia; Yao, Hang-Qi

    2015-06-01

    The middle-frequency sensorineural hearing loss (MFSNHL) is rare among hereditary non-syndromic hearing loss. To date, only three genes are reported to be associated with MFSNHL, including TECTA, EYA4 and COL11A2. In this report, we analyzed and explored the clinical audiological characteristics and the causative gene of a Chinese family named HG-Z087 with non-syndromic autosomal dominant inherited MFSNHL. Clinical audiological characteristics and inheritance pattern of a family were evaluated, and pedigree was drawn based on medical history investigation. Our results showed that the Chinese family was characterized by late onset, progressive, non-sydromic autosomal dominant MFSNHL. Targeted exome sequencing, conducted using DNA samples of an affected member in this family, revealed a novel heterozygous missense mutation c.1643C>G in exon 18 of EYA4, causing amino-acid (aa) substitution Arg for Thr at a conserved position aa-548. The p.T548R mutation related to hearing loss in the selected Chinese family was validated by Sanger sequencing. However, the mutation was absent in control group containing 100 DNA samples from normal Chinese families. In conclusion, we identified the pathogenic gene and found that the novel missense mutation c.1643C>G (p.T548R) in EYA4 might have caused autosomal dominant non-syndromic hearing impairment in the selected Chinese family. PMID:25809937

  1. Malignant meningitis secondary to oesophageal adenocarcinoma presenting with sensorineural hearing loss: a series of three cases and discussion of the literature.

    PubMed

    Adams, Mark; Doherty, Carolynne; O'Kane, Anna; Hall, Samuel; Forbes, Raeburn B; Herron, Brian; McNaboe, Edward J

    2016-09-01

    Malignant meningitis may rarely present to the otolaryngologist. We report our experience with 3 patients presenting with sudden, progressive bilateral sensorineural hearing loss secondary to malignant meningitis. The primary tumour in all 3 cases was oesophageal adenocarcinoma. All 3 cases were notable for the rapidity of the patients' clinical deterioration; the interval from presentation to death ranged from 21 to 28 days. We suggest that otolaryngologists should remain vigilant to the possibility of this devastating diagnosis and have a low threshold for further investigation in patients with suspicious features. It is important to note that initial MRI and lumbar puncture may be negative and repeat testing should be undertaken if there is clinical suspicion. PMID:26645106

  2. 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)

  3. Exogenous IL-4-Expressing Bone Marrow Mesenchymal Stem Cells for the Treatment of Autoimmune Sensorineural Hearing Loss in a Guinea Pig Model

    PubMed Central

    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. PMID:24864261

  4. 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

  5. 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…

  6. 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

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

    PubMed

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

    2015-11-01

    We describe the molecular basis of a distinctive syndrome characterized by infantile stress-induced episodic weakness, ataxia, and sensorineural hearing loss, with permanent areflexia and optic nerve pallor. Whole exome sequencing identified a deleterious heterozygous c.2452 G>A, p.(E818K) variant in the ATP1A3 gene and structural analysis predicted its protein-destabilizing effect. This variant has not been reported in context with rapid-onset dystonia parkinsonism and alternating hemiplegia of childhood, the 2 main diseases associated with ATP1A3. The clinical presentation in the family described here differs categorically from these diseases in age of onset, clinical course, cerebellar over extrapyramidal movement disorder predominance, and peripheral nervous system involvement. While this paper was in review, a highly resembling phenotype was reported in additional patients carrying the same c.2452 G>A variant. Our findings substantiate this variant as the cause of a unique inherited autosomal dominant neurologic syndrome that constitutes a third allelic disease of the ATP1A3 gene. PMID:25895915

  8. 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. PMID:26620341

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

    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. PMID:26559533

  10. 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

  11. 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

  12. [Musical pseudo-hallucination in acquired hearing loss].

    PubMed

    Klostermann, W; Vieregge, P; Kömpf, D

    1992-07-01

    Auditory hallucinations take various forms including the perception of tinnitus, voices, and, rarely, music. While formed hallucinations are usually ascribed to psychiatric illness, we describe a syndrome of musical hallucinations in mentally sane patients, who are hard of hearing or deaf. 26 cases from the literature are supplemented by 6 own observations, including the first description of two cases in a single family. The different modes of emergence, the spectrum of clinical features and their course are outlined. Differential diagnostic, pathogenetic and therapeutic aspects are discussed. PMID:1500041

  13. A boy with mild mental retardation, mild sensorineural hearing loss and mild facial dysmorphism caused by a 19p13.2 deletion: a case report and review of the literature.

    PubMed

    Schwemmle, Cornelia; Rost, Imma; Spranger, Stephanie; Jungheim, Michael; Ptok, Martin

    2014-07-01

    The investigation of patients with congenital anomalies and/or intellectual disability with modern genetic methods allows the recognition of an increasing number of cases with these chromosomal rearrangements. Here, we present a mildly mentally retarded boy with mild facial dysmorphism, language development delay, mild sensorineural hearing loss due to a deletion of 1,14 Mb on chromosome 19p 13.2. The deletion was de novo and familial history negative for this disorder. To our knowledge this is the first description of a patient with symptoms mentioned above associated with a 19p13.2-p13.2 deletion. PMID:24814572

  14. 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

  15. Living with Hearing Loss

    MedlinePlus

    ... Issues Special Section: Focus on Communication Living with Hearing Loss Past Issues / Fall 2008 Table of Contents For ... Fast Facts There are two main types of hearing loss. Permanent hearing loss (called sensorineural) usually involves damage ...

  16. De novo 9 Mb deletion of 6q23.2q24.1 disrupting the gene EYA4 in a patient with sensorineural hearing loss, cardiac malformation, and mental retardation.

    PubMed

    Dutrannoy, Véronique; Klopocki, Eva; Wei, Ran; Bommer, Christiane; Mundlos, Stefan; Graul-Neumann, Luitgard M; Trimborn, Marc

    2009-01-01

    We report on a patient carrying a de novo interstitial deletion of chromosomal region 6q23.2-24.1. Interstitial deletions of 6q are rarely reported in the literature. Indeed, only four patients with interstitial deletions overlapping partially with the deleted region in our patient are described in the literature. The aberration was detected by GTG-banding. The size of the deletion was further refined by array-CGH and subsequently fine mapped by quantitative real-time PCR. The exact size of the deletion and the sequence composition of the breakpoints were determined by breakpoint spanning PCR and subsequent sequencing. The patient presented with microcephaly, short stature, patent ductus arteriosus, sensorineural hearing loss, mental retardation, reduced speech development, and abnormal behaviour. The deletion disrupts the gene EYA4. Mutations within this gene are associated with postlingual sensorineural hearing loss. The sequencing of the breakpoint indicated non homologous end joining as the most likely mechanism leading to the rearrangement. PMID:19576303

  17. 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

  18. Ear-canal acoustic admittance and reflectance measurements in human neonates. II. Predictions of middle-ear dysfunction and sensorineural hearing loss

    NASA Astrophysics Data System (ADS)

    Keefe, Douglas H.; Gorga, Michael P.; Neely, Stephen T.; Zhao, Fei; Vohr, Betty R.

    2003-01-01

    This report describes relationships between middle-ear measurements of acoustic admittance and energy reflectance (YR) and measurements of hearing status using visual reinforcement audiometry in a neonatal hearing-screening population. Analyses were performed on 2638 ears in which combined measurements were obtained [Norton et al., Ear Hear. 21, 348-356 (2000)]. The measurements included distortion-product otoacoustic emissions (DPOAE), transient evoked otoacoustic emissions (TEOAE), and auditory brainstem responses (ABR). Models to predict hearing status using DPOAEs, TEOAEs, or ABRs were each improved by the addition of the YR factors as interactions, in which factors were calculated using factor loadings from Keefe et al. [J. Acoust. Soc. Am. 113, 389-406 (2003)]. This result suggests that information on middle-ear status improves the ability to predict hearing status. The YR factors were used to construct a middle-ear dysfunction test on 1027 normal-hearing ears in which DPOAE and TEOAE responses were either both present or both absent, the latter condition being viewed as indicative of middle-ear dysfunction. The middle-ear dysfunction test classified these ears with a nonparametric area (A) under the relative operating characteristic curve of A=0.86, and classified normal-hearing ears that failed two-stage hearing-screening tests with areas A=0.84 for DPOAE/ABR, and A=0.81 for TEOAE/ABR tests. The middle-ear dysfunction test adequately generalized to a new sample population (A=0.82).

  19. 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

  20. Hair Cell Loss, Spiral Ganglion Degeneration, and Progressive Sensorineural Hearing Loss in Mice with Targeted Deletion of Slc44a2/Ctl2.

    PubMed

    Kommareddi, Pavan; Nair, Thankam; Kakaraparthi, Bala Naveen; Galano, Maria M; Miller, Danielle; Laczkovich, Irina; Thomas, Trey; Lu, Lillian; Rule, Kelli; Kabara, Lisa; Kanicki, Ariane; Hughes, Elizabeth D; Jones, Julie M; Hoenerhoff, Mark; Fisher, Susan G; Altschuler, Richard A; Dolan, David; Kohrman, David C; Saunders, Thomas L; Carey, Thomas E

    2015-12-01

    SLC44A2 (solute carrier 44a2), also known as CTL2 (choline transporter-like protein 2), is expressed in many supporting cell types in the cochlea and is implicated in hair cell survival and antibody-induced hearing loss. In mice with the mixed C57BL/6-129 background, homozygous deletion of Slc44a2 exons 3–10 (Slc44a2(Δ/Δ)resulted in high-frequency hearing loss and hair cell death. To reduce effects associated with age-related hearing loss (ARHL) in these strains, mice carrying the Slc44a2Δ allele were backcrossed to the ARHL-resistant FVB/NJ strain and evaluated after backcross seven(N7) (99 % FVB). Slc44a2(Δ/Δ) mice produced abnormally spliced Slc44a2 transcripts that contain a frame shift and premature stop codons. Neither full-length SLC44A2 nor a putative truncated protein could be detected in Slc44a2(Δ/Δ) mice, suggesting a likely null allele. Auditory brain stem responses (ABRs) of mice carrying the Slc44a2Δ allele on an FVB/NJ genetic background were tested longitudinally between the ages of 2 and 10 months. By 6 months of age,Slc44a2(Δ/Δ) mice exhibited hearing loss at 32 kHz,but at 12 and 24 kHz had sound thresholds similar to those of wild-type Slc44a2(+/+) and heterozygous +/Slc44a2Δ mice. After 6 months of age, Slc44a2(Δ/Δ) mutants exhibited progressive hearing loss at all frequencies and +/Slc44a2(Δ) mice exhibited moderate threshold elevations at high frequency. Histologic evaluation of Slc44a2(Δ/Δ) mice revealed extensive hair cell and spiral ganglion cell loss, especially in the basal turn of the cochlea. We conclude that Slc44a2 function is required for long-term hair cell survival and maintenance of hearing. PMID:26463873

  1. [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

  2. 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)

  3. Managing Hearing Loss | NIH MedlinePlus the Magazine

    MedlinePlus

    ... certain medications, or long-term exposure to loud noises. Sensorineural hearing loss occurs when there is damage ... Many people may have a combination of both noise-induced hearing loss and hearing loss from aging. ...

  4. Inflectional morphology in German hearing-impaired children.

    PubMed

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

    2016-01-01

    Despite modern hearing aids, children with hearing impairment often have only restricted access to spoken language input during the 'critical' years for language acquisition. Specifically, a sensorineural hearing impairment affects the perception of voiceless coronal consonants which realize verbal affixes in German. The aim of this study is to explore if German hearing-impaired children have problems in producing and/or acquiring inflectional suffixes expressed by such phonemes. The findings of two experiments (an elicitation task and a picture-naming task) conducted with a group of hearing-impaired monolingual German children (age 3-4 years) demonstrate that difficulties in perceiving specific phonemes relate to the avoidance of these same sounds in speech production independent of the grammatical function these phonemes have. PMID:25175166

  5. 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…

  6. 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…

  7. Human temporal bone findings in acquired hypothyroidism.

    PubMed

    Hald, J; Milroy, C M; Jensen, K D; Parving, A

    1991-11-01

    Histological studies of the auditory organ in patients with acquired hypothyroidism are scarce. Thus the aim of the present study was to examine the temporal bones and the brain in subjects with hypothyroidism. Four temporal bones and two brains from clinically and biochemically hypothyroid subjects were removed and evaluated by light microscopy determine to the morphological changes and deposition of neutral and acid glycosaminoglycans. An audiogram from one of the patients showed a sensorineural hearing loss, which could be ascribed to occupational noise exposure. The study revealed histological changes compatible with age and infectious disease. No accumulation of neutral or acid glycosaminoglycans could be demonstrated in the temporal bones, or in the brains. PMID:1761939

  8. 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,…

  9. 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…

  10. [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

  11. The WHO-DAS II: psychometric properties in the measurement of functional health status in adults with acquired hearing loss.

    PubMed

    Chisolm, Theresa H; Abrams, Harvey B; McArdle, Rachel; Wilson, Richard H; Doyle, Patrick J

    2005-01-01

    The World Health Organization's (WHO) Disability Assessment Scale II (WHO-DAS II) is a generic health-status instrument firmly grounded in the WHO's International Classification of Functioning, Disability and Health (WHO-ICF). As such, it assesses functioning for six domains: communication, mobility, self-care, interpersonal, life activities, and participation. Domain scores aggregate to a total score. Because the WHO-DAS II contains questions relevant to hearing and communication, it has good face validity for use as an outcome measure for audiologic intervention. The purpose of the present study was to determine the psychometric properties of the WHO-DAS II on a sample of individuals with adult-onset hearing loss, including convergent validity, internal consistency, and test-retest stability. Convergent validity was established by examining correlations between the WHO-DAS II (domain and total scores) and the Abbreviated Profile of Hearing Aid Benefit (APHAB) and the Hearing Aid Handicap for the Elderly (HHIE), two disease-specific measures, as well as with the Short Form-36 for veterans (SF-36V), a second generic measure. Data on all four measures were collected from 380 older individuals with adult-onset hearing loss who were not hearing aid users. The results of the convergent validity analysis revealed that the WHODAS II communication domain score was moderately and significantly correlated with scores on the APHAB and the HHIE. WHO-DAS II interpersonal and participation domain scores and the total scores were also moderately and significantly correlated with HHIE scores. These findings support the validity of using the WHO-DAS II for assessing activity limitations and participation restrictions of adult-onset hearing loss. Several WHO-DAS II domain scores and the total score were also significantly and moderately-markedly correlated with scores from the SF-36V. These findings support the validity of the WHO-DAS II as a generic health-status instrument

  12. 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

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

    PubMed

    Ahmed, Mohi; Ura, Kiyoe; Streit, Andrea

    2015-09-01

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

  14. 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

  15. Binaural Loudness Summation in the Hearing Impaired.

    ERIC Educational Resources Information Center

    Hawkins, David B.; And Others

    1987-01-01

    Binaural loudness summation was measured using three different paradigms with 10 normally hearing and 20 bilaterally symmetrical high-frequency sensorineural hearing loss subjects. Binaural summation increased with presentation level using the loudness matching procedure, with values in the 6-10 dB range. Summation decreased with level using the…

  16. 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…

  17. 38 CFR 17.149 - Sensori-neural aids.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2011-07-01 2011-07-01 false Sensori-neural aids. 17... Prosthetic, Sensory, and Rehabilitative Aids § 17.149 Sensori-neural aids. (a) Notwithstanding any other provision of this part, VA will furnish needed sensori-neural aids (i.e., eyeglasses, contact...

  18. 38 CFR 17.149 - Sensori-neural aids.

    Code of Federal Regulations, 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...

  19. 38 CFR 17.149 - Sensori-neural aids.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2010-07-01 2010-07-01 false Sensori-neural aids. 17... Prosthetic, Sensory, and Rehabilitative Aids § 17.149 Sensori-neural aids. (a) Notwithstanding any other provision of this part, VA will furnish needed sensori-neural aids (i.e., eyeglasses, contact...

  20. 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...

  1. 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...

  2. Pigmentary anomalies and hearing loss.

    PubMed

    Toriello, Helga V

    2011-01-01

    A number of syndromes that include hearing loss in the phenotype also have pigmentary anomalies as a component manifestation. One of the most common of these is Waardenburg syndrome, which includes hypopigmentation and sensorineural hearing loss in the phenotype. There are four types of Waardenburg syndrome, distinguishable from each other by clinical findings. However, there are several other syndromes which include not only hypopigmentation, but also hyperpigmentation in the phenotype. This paper serves as a review of many of these syndromes. PMID:21358185

  3. Effects of hearing loss on the subcortical representation of speech cues.

    PubMed

    Anderson, Samira; Parbery-Clark, Alexandra; White-Schwoch, Travis; Drehobl, Sarah; Kraus, Nina

    2013-05-01

    Individuals with sensorineural hearing loss often report frustration with speech being loud but not clear, especially in background noise. Despite advanced digital technology, hearing aid users may resort to removing their hearing aids in noisy environments due to the perception of excessive loudness. In an animal model, sensorineural hearing loss results in greater auditory nerve coding of the stimulus envelope, leading to a relative deficit of stimulus fine structure. Based on the hypothesis that brainstem encoding of the temporal envelope is greater in humans with sensorineural hearing loss, speech-evoked brainstem responses were recorded in normal hearing and hearing impaired age-matched groups of older adults. In the hearing impaired group, there was a disruption in the balance of envelope-to-fine structure representation compared to that of the normal hearing group. This imbalance may underlie the difficulty experienced by individuals with sensorineural hearing loss when trying to understand speech in background noise. This finding advances the understanding of the effects of sensorineural hearing loss on central auditory processing of speech in humans. Moreover, this finding has clinical potential for developing new amplification or implantation technologies, and in developing new training regimens to address this relative deficit of fine structure representation. PMID:23654406

  4. 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,…

  5. 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. PMID:24040484

  6. [Equilibrium function in patients with acute sensorineural hearing loss].

    PubMed

    Pal'chun, V T; Ganichkina, I Ia; Luchikhin, L A; Derevianko, S N

    2002-01-01

    Equilibrium function was investigated with computer-assisted stabilography (CS) in patients with acute neurosensory hypoacusis. This new diagnostic tool was employed in combination with extended vestibulometric and audiologic examinations. Correlations were found between stabilographic and vestibulometric findings. CS is recommended as a method of screening diagnosis in examination of patients with imbalanced equilibrium. PMID:12227024

  7. 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

  8. 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…

  9. Occupational hearing loss in farmers.

    PubMed Central

    Plakke, B L; Dare, E

    1992-01-01

    Studies have shown that there is a great deal of high-frequency sensorineural hearing loss among farmers. The studies have failed, however, to differentiate farmers who have occupational noise exposure only from other potential hearing loss etiologies. This study, through extensive case history information, has isolated a farm noise-exposure group and matched its members by age with persons with no significant noise exposure. Results indicate that farmers exposed only to noise from farming have significantly poorer hearing sensitivity than persons not exposed to noise. PMID:1561302

  10. 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)

  11. 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

  12. 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.…

  13. 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.…

  14. 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…

  15. 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

  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. 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

  18. 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.

  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: Diagnosis and Evaluation.

    PubMed

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

    2015-07-01

    Hearing loss is a common disability in the United States, most frequent among men, elderly individuals, and veterans but is increasingly affecting other younger adults. Types of hearing loss include sensorineural, conductive, and mixed. Hearing loss in children often is related to infections, time spent in a neonatal intensive care unit, and genetic etiologies. Presbycusis (ie, age-related hearing loss) is the most common etiology in adults. Adverse effects of untreated hearing loss include isolation, depression, lower income, and higher unemployment. Hearing aid use reduces levels of disability, cognitive impairment, and psychosocial distress while improving quality of life. At least 75% of individuals with hearing loss are not receiving treatment for it. All infants should be screened for hearing loss, as should children and adults with risk factors. The Joint Commission on Infant Hearing Screening has a 1-3-6 goal for screening: identification by age 1 month, confirmation by age 3 months, and intervention by age 6 months. The presence of an ongoing physician-patient relationship increases the likelihood that a patient will admit to having a hearing loss. Adults can be screened using single-question or standardized instrument screens. All patients with suspected hearing loss should undergo audiometry by an audiology subspecialist. PMID:26161523

  1. Syntactic Performance of Hearing Impaired and Normal Hearing Individuals.

    ERIC Educational Resources Information Center

    Quigley, Stephen P.; King, Cynthia M.

    1980-01-01

    Describes two research programs on the syntactic abilities of hearing impaired and normal hearing individuals. The first program involved deaf students acquiring English structure; the second involved the construction of the Test of Syntactic Abilities and its application to deaf, hard of hearing, and normal hearing students in the United States,…

  2. Peaks in the Frequency Response of Hearing Aids: Evaluation of the Effects on Speech Intelligibility and Sound Quality.

    ERIC Educational Resources Information Center

    Buuren, Ronald A. van; And Others

    1996-01-01

    This study evaluated speech intelligibility under noise conditions of varying peaks (10, 20, and 30 decibels) in frequency response, with 26 listeners with sensorineural impaired hearing who used hearing aids and 10 listeners with normal hearing. Results indicated that the peaks affected speech intelligibility more for listeners with impaired than…

  3. [Progressive hearing loss].

    PubMed

    Reiss, M; Reiss, G

    2000-01-01

    Progressive sensorineural hearing loss (SNHL) is defined as hearing loss of unknown etiology with fairly high-speed progression. Its diagnostic criteria consist of the following: that it is 1) progressive, 2) with bilateral involvement, and 3) of unknown etiology. Due to recent advances in diagnostics, imaging and management, SNHL has gained much interest from otologists in the last few years. They provide new insight into the physiology and pathophysiology of hearing. SNHL which is sudden in onset, fluctuating, and/or progressive complicates medical management, hearing aid selection, and individualized educational planning for a hearing-impaired patient. Existing hypotheses on the etiology of SNHL are judged on experimental, clinical, laboratory and radiological evidence. Cardiovascular and rheologic diseases, hereditary disorders, immunological phenomena, infections, environmental causes like noise, ototoxic drugs and industrial substances and systemic maladies must be included in the diagnostic reflections. Potential concepts of treatment include rheologic medications and corticosteroids. Hearing aids and timely cochlear implant operation are further possible forms of treatment. PMID:10893764

  4. 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…

  5. 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. PMID:25256656

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

    ERIC Educational Resources Information Center

    Ruggirello, Caterina; Mayer, Connie

    2010-01-01

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

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

    ERIC Educational Resources Information Center

    Jutras, Benoit; Gagne, Jean-Pierre

    1999-01-01

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

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

    ERIC Educational Resources Information Center

    Reuterskiold, Christina; Ibertsson, Tina; Sahlen, Birgitta

    2010-01-01

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

  9. 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.…

  10. 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…

  11. HDR Syndrome (Hypoparathyroidism, Sensorineural Deafness and Renal Disease) Accompanied by Hirschsprung Disease

    PubMed Central

    Sepahi, Mohsen Akhavan; Baraty, Behrouz; Shooshtary, Fatemeh Khalifeh

    2010-01-01

    Background HDR syndrome (hypoparathyroidism, sensorineural deafness and renal disease) is an autosomal dominant condition, defined by the triad hypoparathyroidism, renal dysplasia and hearing loss. Hirschsprung (HSCR) disease is a variable congenital absence of ganglion cells of the enteric nervous system resulting in degrees of functional bowel obstruction. Rarer chromosomal anomalies are reported in combination with Hirschsprung disease like DiGeorge syndrome, mosaic trisomy 8, XXY chromosomal constitution, partial duplication of chromosome 2q, tetrasomy 9p, and 20p deletion. Case Presentation Here, we describe an 8 year-old girl with HDR syndrome accompanied by Hirschsprung disease. Although the association of Hirschsprung disease with chromosomal anomalies has been reported, according to our knowledge, this is the first report of associated HSCR with HDR syndrome. PMID:23056694

  12. 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)

  13. 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…

  14. 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. PMID:26890714

  15. Pontine hypoplasia in a child with sensorineural deafness.

    PubMed

    Maeoka, Y; Yamamoto, T; Ohtani, K; Takeshita, K

    1997-09-01

    A 2-year-old girl with bilateral sensorineural deafness showed pontine hypoplasia as well as a bulging contour of the pontine tegmentum on magnetic resonance imaging (MRI). There were no bilateral responses of brainstem auditory-evoked potentials (BAEPs). The absent late components of blink reflex (BR) indicated brainstem dysfunction. Chromosomal abnormalities and neurodegenerative or neurometabolic disorders, which might have been the cause of the pontine hypoplasia, were ruled out. The authors describe a rare case with pontine hypoplasia combined with sensorineural deafness and absent blink reflex and suggest that the brainstem in this child may become involved in the early gestation period. PMID:9339875

  16. 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 ...

  17. Observations From a Musician With Hearing Loss

    PubMed Central

    2012-01-01

    Extensive personal experience with professional recording and audio signal processing technology has enabled the author to continue his music career after experiencing sudden sensorineural hearing loss. The iPhone™ is one such device that has been found useful for many music and general listening situations that would otherwise be intractable. Additional techniques and technologies are described that the author has found useful for specific situations, including music composition, rehearsal, and enjoyment. PMID:23203415

  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. 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…

  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. Is Hearing Impairment Associated with Rheumatoid Arthritis? A Review

    PubMed Central

    Emamifar, Amir; Bjoerndal, Kristine; Hansen, Inger M.J.

    2016-01-01

    Background: Rheumatoid arthritis (RA) is a systemic, inflammatory disease that affects 1% of the population. The auditory system may be involved during the course of disease; however the association of RA and hearing impairment has not been clearly defined. Objective: The objective of this review is to evaluate published clinical reports related to hearing impairment in patients with RA. Furthermore, we discuss possible pathologies and associated factors as well as new treatment modalities. Method: A thorough literature search was performed using available databases including Pubmed, Embase, Cochrane and ComDisDome to cover all relative reports. The following keywords were used: hearing loss, hearing difficulties, hearing disorders, hearing impairment, sensorineural hearing loss, conductive hearing loss, mixed hearing loss, autoimmune hearing loss, drug ototoxicity, drug-induced hearing loss, hearing test, audiometry, auditory dysfunction and rheumatoid arthritis. Conclusion: Based on our review it can be postulated that patients with RA are at higher risk of hearing impairment compared to healthy subjects in their course of the disease. The hearing impairment in RA seems to be a multifactorial condition; however the mechanisms of injury, as well as the relative risk factors, are not completely clear. This review can aid to clarify this condition and is a guide for further evaluation. To the best of our knowledge, this is the first review of hearing impairment in RA. PMID:27053970

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

    PubMed

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

    2011-06-01

    The objectives of this study were to estimate prevalence, heritability and genetic correlations of congenital sensorineural deafness (CSD) and pigmentation phenotypes in the Border Collie. Entire litters of Border Collies that presented to the Animal Health Trust (1994-2008) for assessment of hearing status by brain stem auditory evoked response (BAER) at 4-10 weeks of age were included. Heritability and genetic correlations were estimated using residual maximum likelihood (REML). Of 4143 puppies that met the inclusion criteria, 97.6% had normal hearing status, 2.0% were unilaterally deaf and 0.4% were bilaterally deaf. Heritability of deafness as a trichotomous trait (normal/unilaterally deaf/bilaterally deaf) was estimated at 0.42 using multivariate analysis. Genetic correlations of deafness with iris colour and merle coat colour were 0.58 and 0.26, respectively. These results indicate that there is a significant genetic effect on CSD in Border Collies and that some of the genes determining deafness also influence pigmentation phenotypes. PMID:20570536

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

    PubMed Central

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

    2015-01-01

    Perceptual performance in persons with hearing loss, especially those using devices to restore hearing, is not fully predicted by traditional audiometric measurements designed to evaluate the status of peripheral function. The integrity of auditory brainstem synapses may vary with different forms of hearing loss, and differential effects on the auditory nerve-brain interface may have particularly profound consequences for the transfer of sound from ear to brain. Loss of auditory nerve synapses in ventral cochlear nucleus (VCN) has been reported after acoustic trauma, ablation of the organ of Corti, and administration of ototoxic compounds. The effects of gradually acquired forms deafness on these synapses are less well understood. We investigated VCN gross morphology and auditory nerve synapse integrity in DBA/2J mice with early-onset progressive sensorineural hearing loss. Hearing status was confirmed using auditory brainstem response audiometry and acoustic startle responses. We found no change in VCN volume, number of macroneurons, or number of VGLUT1-positive auditory nerve terminals between young adult and older, deaf DBA/2J. Cell-type specific analysis revealed no difference in the number of VGLUT1 puncta contacting bushy and multipolar cell body profiles, but the terminals were smaller in deaf DBA/2J mice. Transmission electron microscopy confirmed the presence of numerous healthy, vesicle-filled auditory nerve synapses in older, deaf DBA/2J mice. The present results suggest that synapses can be preserved over a relatively long time-course in gradually acquired deafness. Elucidating the mechanisms supporting survival of central auditory nerve synapses in models of acquired deafness may reveal new opportunities for therapeutic intervention. PMID:25686750

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

    PubMed

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

    2015-04-24

    Perceptual performance in persons with hearing loss, especially those using devices to restore hearing, is not fully predicted by traditional audiometric measurements designed to evaluate the status of peripheral function. The integrity of auditory brainstem synapses may vary with different forms of hearing loss, and differential effects on the auditory nerve-brain interface may have particularly profound consequences for the transfer of sound from ear to brain. Loss of auditory nerve synapses in ventral cochlear nucleus (VCN) has been reported after acoustic trauma, ablation of the organ of Corti, and administration of ototoxic compounds. The effects of gradually acquired forms deafness on these synapses are less well understood. We investigated VCN gross morphology and auditory nerve synapse integrity in DBA/2J mice with early-onset progressive sensorineural hearing loss. Hearing status was confirmed using auditory brainstem response audiometry and acoustic startle responses. We found no change in VCN volume, number of macroneurons, or number of VGLUT1-positive auditory nerve terminals between young adult and older, deaf DBA/2J. Cell-type specific analysis revealed no difference in the number of VGLUT1 puncta contacting bushy and multipolar cell body profiles, but the terminals were smaller in deaf DBA/2J mice. Transmission electron microscopy confirmed the presence of numerous healthy, vesicle-filled auditory nerve synapses in older, deaf DBA/2J mice. The present results suggest that synapses can be preserved over a relatively long time-course in gradually acquired deafness. Elucidating the mechanisms supporting survival of central auditory nerve synapses in models of acquired deafness may reveal new opportunities for therapeutic intervention. PMID:25686750

  5. Hearing parameters in noise exposed industrial workers.

    PubMed

    Celik, O; Yalçin, S; Oztürk, A

    1998-12-01

    This paper presents the results of a study carried out in a group of noise-exposed workers in a hydro-electric power plant. Thus, the main focus of the study is on 130 industrial workers who were exposed to high level of noise. The control group was consisted of 33 subjects with normal hearing. Hearing and acoustic reflex thresholds were obtained from all subjects and the results from age-matched subgroups were compared. The sensorineural hearing loss which were detected in 71 workers were bilateral, symmetrical and affected mainly frequencies of 4-6 kHz. In essence, the hearing losses were developed within the first 10 years of noise exposure and associated with slight progress in the following years. When acoustic reflex thresholds derived from the study and control groups were compared, statistically significant difference was determined only for the thresholds obtained at 4 kHz (p < 0.0005). PMID:9853659

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

    PubMed

    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. 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

  8. Reactive Oxygen Species, Apoptosis, and Mitochondrial Dysfunction in Hearing Loss

    PubMed Central

    Fujimoto, Chisato

    2015-01-01

    Reactive oxygen species (ROS) production is involved in several apoptotic and necrotic cell death pathways in auditory tissues. These pathways are the major causes of most types of sensorineural hearing loss, including age-related hearing loss, hereditary hearing loss, ototoxic drug-induced hearing loss, and noise-induced hearing loss. ROS production can be triggered by dysfunctional mitochondrial oxidative phosphorylation and increases or decreases in ROS-related enzymes. Although apoptotic cell death pathways are mostly activated by ROS production, there are other pathways involved in hearing loss that do not depend on ROS production. Further studies of other pathways, such as endoplasmic reticulum stress and necrotic cell death, are required. PMID:25874222

  9. 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

  10. 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. PMID:26433044

  11. 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. PMID:20610915

  12. 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…

  13. Effect of Two Approaches to Auditory Training on Speech Recognition by Hearing-Impaired Adults.

    ERIC Educational Resources Information Center

    Rubinstein, Adrienne; Boothroyd, Arthur

    1987-01-01

    Two approaches to auditory training for improved speech recognition using 20 adults with mild to moderate sensorineural hearing impairments were compared. No significant differences were found between group one which spent training time on activities involving sentence perception and perceptual strategy and group two which focused on consonant…

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

    ERIC Educational Resources Information Center

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

    2005-01-01

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

  15. Two sisters with mental retardation, cataract, ataxia, progressive hearing loss, and polyneuropathy.

    PubMed Central

    Begeer, J H; Scholte, F A; van Essen, A J

    1991-01-01

    Two sisters are described with a disorder characterised by mental retardation, congenital cataract, progressive spinocerebellar ataxia, sensorineural deafness, and signs of peripheral neuropathy. Progressive hearing loss, ataxia, and polyneuropathy became evident in the third decade. The differential diagnosis of this syndrome is discussed including the syndromes described by Berman et al and Koletzko et al. PMID:1661780

  16. 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

  17. [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

  18. 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. PMID:26152551

  19. 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 ...

  20. 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 ...

  1. Hearing Loss

    MedlinePlus

    ... Devices Can Help? Hearing aids. Hearing aids are electronic, battery-run devices that make sounds louder. There ... to turn up the volume. Cochlear implants. These electronic devices are for people with severe hearing loss. ...

  2. Cochlear implant assessment and candidacy for children with partial hearing.

    PubMed

    Wilson, Katherine; Ambler, Marette; Hanvey, Kate; Jenkins, Marsha; Jiang, Dan; Maggs, Justine; Tzifa, Konstance

    2016-04-01

    Children who have partial hearing (PH) in the low frequencies and profound sensorineural hearing loss in the high frequencies can present a challenge to cochlear implant (CI) teams in terms of referral, assessment, and candidacy. Neither clinical criteria nor optimal timing for implantation has been explored in the literature. Data from both the Hearing Implant Centres of Birmingham Children's Hospital and St Thomas' Hospital indicate that it is clinically appropriate to implant children with PH; they perform better with CIs than with hearing aids, even if their hearing is not fully preserved. We have also found that children need early access to high frequency sound in order to reach their full potential. PMID:26913562

  3. Function of a hearing aid under stressful conditions.

    PubMed

    Welsh, L W; Welsh, J J; Rosen, L F

    2000-10-01

    The auditory function of individuals with normal hearing was compared with that of hearing-aided subjects of similar age to determine whether amplification remediates hearing impairment under stressful auditory situations. The specific tests of listening in a competitive noise environment and identifying moderately compressed speech were introduced to adequately aided individuals. The data indicate that noise had an impact on auditory function to a much greater degree in aided individuals than in matched counterparts with normal hearing. The data derived from acceleration of simple sentences delivered to the aided group suggested that contrary to basic tonal sensitivity, the capacity to understand the stimulus was greatly compromised. The authors discuss cochlear damage and central auditory impairment as they relate to the limitations of amplification for sensorineural hearing loss. PMID:11051433

  4. 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

  5. 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

  6. 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.

  7. 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

  8. 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.

  9. 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

  10. 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

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

    PubMed Central

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

    2010-01-01

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

  12. [Recovery of hearing: results of delayed medical treatment in patients with idiopathic sudden hearing loss].

    PubMed

    Maassen, M M; Pfister, M; Plontke, S; Koitschev, A; Vögler, A; Löwenheim, H

    2002-12-01

    For the treatment of idiopathic sudden sensorineural hearing loss (ISSNHL), a variety of studies about intravenous drug administration with the beginning of treatment in the early period of less then one week after the onset of hearing loss have been performed. In contrast, very little information is available about the efficacy of intravenous drug therapy for ISSNHL with the beginning of treatment later than four weeks after the onset of hearing loss. In a retrospective chart review we studied the treatment results of 57 patients with ISSNHL with beginning of treatment later than four weeks after the onset of hearing loss with no spontaneous recovery of hearing. Patients received a treatment with intravenous administration of Dextran (concentration 40 g/l with NaCl 0.9%) and Procain-HCl (a derivative of the local anaesthetic lidocaine,400-800 mg in a 500 ml rheologic infusion of Dextran 40). 25% of the patients showed a significant improvement of 10 dB or more in hearing threshold at 1000 Hz measured in bone-conducted pure tone audiometry. In a subjective evaluation 53% of the patients noticed a subjective improvement of their individual hearing thresholds. PMID:12474128

  13. 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

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

    PubMed

    Doherty, Karen A; Desjardins, Jamie L

    2015-01-01

    Untreated hearing loss can interfere with an individual's cognitive abilities and intellectual function. Specifically, hearing loss has been shown to negatively impact working memory function, which is important for speech understanding, especially in difficult or noisy listening conditions. The purpose of the present study was to assess the effect of hearing aid use on auditory working memory function in middle-aged and young-older adults with mild to moderate sensorineural hearing loss. Participants completed two objective measures of auditory working memory in aided and unaided listening conditions. An aged matched control group followed the same experimental protocol except they were not fit with hearing aids. All participants' aided scores on the auditory working memory tests were significantly improved while wearing hearing aids. Thus, hearing aids worn during the early stages of an age-related hearing loss can improve a person's performance on auditory working memory tests. PMID:26097461

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

    PubMed Central

    Doherty, Karen A.; Desjardins, Jamie L.

    2015-01-01

    Untreated hearing loss can interfere with an individual’s cognitive abilities and intellectual function. Specifically, hearing loss has been shown to negatively impact working memory function, which is important for speech understanding, especially in difficult or noisy listening conditions. The purpose of the present study was to assess the effect of hearing aid use on auditory working memory function in middle-aged and young-older adults with mild to moderate sensorineural hearing loss. Participants completed two objective measures of auditory working memory in aided and unaided listening conditions. An aged matched control group followed the same experimental protocol except they were not fit with hearing aids. All participants’ aided scores on the auditory working memory tests were significantly improved while wearing hearing aids. Thus, hearing aids worn during the early stages of an age-related hearing loss can improve a person’s performance on auditory working memory tests. PMID:26097461

  16. 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

  17. Frequency Discrimination and Literacy Skills in Children with Mild to Moderate Sensorineural Hearing Loss

    ERIC Educational Resources Information Center

    Halliday, L. F.; Bishop, D. V. M.

    2005-01-01

    It has been suggested that specific reading disability (SRD) may be attributable to an impaired ability to perceive spectral differences between sounds that leads to a deficit in frequency discrimination and subsequent problems with language and literacy. The objective of the present study was threefold. We aimed to (a) determine whether children…

  18. 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…

  19. 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

  20. 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 ...

  1. 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

  2. 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

  3. 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 ...

  4. 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

  5. Genetics of Nonsyndromic Congenital Hearing Loss

    PubMed Central

    Egilmez, Oguz Kadir; Kalcioglu, M. Tayyar

    2016-01-01

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

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

    PubMed Central

    Charles, Lauren; Street, Nicole Drakopoulos

    2015-01-01

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

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

    PubMed

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

    2015-01-01

    Previous work suggested that individuals with low working memory capacity may be at a disadvantage in adverse listening environments, including situations with background noise or substantial modification of the acoustic signal. This study explored the relationship between patient factors (including working memory capacity) and intelligibility and quality of modified speech for older individuals with sensorineural hearing loss. The modification was created using a combination of hearing aid processing [wide-dynamic range compression (WDRC) and frequency compression (FC)] applied to sentences in multitalker babble. The extent of signal modification was quantified via an envelope fidelity index. We also explored the contribution of components of working memory by including measures of processing speed and executive function. We hypothesized that listeners with low working memory capacity would perform more poorly than those with high working memory capacity across all situations, and would also be differentially affected by high amounts of signal modification. Results showed a significant effect of working memory capacity for speech intelligibility, and an interaction between working memory, amount of hearing loss and signal modification. Signal modification was the major predictor of quality ratings. These data add to the literature on hearing-aid processing and working memory by suggesting that the working memory-intelligibility effects may be related to aggregate signal fidelity, rather than to the specific signal manipulation. They also suggest that for individuals with low working memory capacity, sensorineural loss may be most appropriately addressed with WDRC and/or FC parameters that maintain the fidelity of the signal envelope. PMID:25999874

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

    PubMed Central

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

    2015-01-01

    Previous work suggested that individuals with low working memory capacity may be at a disadvantage in adverse listening environments, including situations with background noise or substantial modification of the acoustic signal. This study explored the relationship between patient factors (including working memory capacity) and intelligibility and quality of modified speech for older individuals with sensorineural hearing loss. The modification was created using a combination of hearing aid processing [wide-dynamic range compression (WDRC) and frequency compression (FC)] applied to sentences in multitalker babble. The extent of signal modification was quantified via an envelope fidelity index. We also explored the contribution of components of working memory by including measures of processing speed and executive function. We hypothesized that listeners with low working memory capacity would perform more poorly than those with high working memory capacity across all situations, and would also be differentially affected by high amounts of signal modification. Results showed a significant effect of working memory capacity for speech intelligibility, and an interaction between working memory, amount of hearing loss and signal modification. Signal modification was the major predictor of quality ratings. These data add to the literature on hearing-aid processing and working memory by suggesting that the working memory-intelligibility effects may be related to aggregate signal fidelity, rather than to the specific signal manipulation. They also suggest that for individuals with low working memory capacity, sensorineural loss may be most appropriately addressed with WDRC and/or FC parameters that maintain the fidelity of the signal envelope. PMID:25999874

  9. 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 ...

  10. 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.

  11. Mitochondrial 12S ribosomal RNA A1555G mutation associated with cardiomyopathy and hearing loss following high-dose chemotherapy and repeated aminoglycoside exposure.

    PubMed

    Skou, Anne-Sofie; Tranebjærg, Lisbeth; Jensen, Tim; Hasle, Henrik

    2014-02-01

    A 19-month-old girl with the A1555G mitochondrial mutation in the 12S ribosomal RNA gene and acute myelogenous leukemia developed dilated cardiomyopathy and bilateral sensorineural hearing loss before undergoing allogeneic stem cell transplantation. She had received gentamicin during episodes of febrile neutropenia. Testing for the A1555G mutation is recommended in patients frequently treated with aminoglycosides. PMID:24252789

  12. 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

  13. 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. PMID:6417600

  14. [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

  15. Alcohol use among students with and without hearing loss.

    PubMed

    Pinquart, Martin; Pfeiffer, Jens P

    2015-01-01

    We compared alcohol use among adolescents with and without hearing loss. Adolescents with hearing loss reported consuming less alcohol, less binge drinking, fewer episodes of drunkenness, and a higher age at first drunkenness than their hearing peers. Alcohol use did not vary between students who were deaf or hard of hearing or between students with congenital versus acquired hearing loss. Although higher age, male gender, and larger friend networks predicted higher alcohol consumption among adolescents with and without hearing loss, worse grades at school were associated only with alcohol use among hearing students. Lower alcohol use among students with hearing loss when compared with hearing peers was, in part, explained by their lower level of peer-group integration. Although alcohol use is a less serious problem among students with hearing loss, a minority with risky consumption would benefit from interventions aimed at reducing alcohol use. PMID:25318927

  16. 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 ...

  17. 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

  18. 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

  19. Degree of satisfaction among hearing aid users

    PubMed Central

    Mondelli, Maria Fernanda Capoani Garcia; Rocha, Andressa Vital; Honório, Heitor Marques

    2013-01-01

    Summary Introduction: Hearing loss (HL) is defined as the complete or partial loss of hearing ability. Aims: To characterize (1) the degree of satisfaction among adult and elderly hearing aid (HA) users who were treated by a public hearing health service and (2) the relationship between satisfaction and the variables of gender, age, degree of HL, and type of HA. Method: The clinical and experimental study included the administration of the Satisfaction with Amplification in Daily Life (SADL) questionnaire to 110 patients who had used HAs for more than 3 months and were 18 years of age or older. Results: Test patients were sex-balanced (48% were women) and had a mean age of 67 years. A relatively high incidence of sensorineural moderate HL was detected in the study patients (66%) and device B was the most commonly used HA type (48%). No significant differences were evident between HA satisfaction and sex. The importance placed on services/costs and personal image varied between age groups. Correlation was evident at all levels between user satisfaction and amplification. Decreased satisfaction was observed in individuals with severe and/or profound HL. The type of HA used yielded statistically significant differences in the positive effects referring. Conclusion: No correlations were evident between the different factors proposed. HA users exhibited high levels of satisfaction in all SADL areas. PMID:25991994

  20. [Indication for and verification of hearing aid benefit using modern methods of speech audiometry in German].

    PubMed

    Kollmeier, B; Lenarz, T; Winkler, A; Zokoll, M A; Sukowski, H; Brand, T; Wagener, K C

    2011-10-01

    The current guidelines for hearing aid supply in Germany employ the often criticised Freiburg monosyllabic speech test (FBE) in quiet. This test can be replaced with the monosyllabic rhyme test by von Wallenberg and Kollmeier (WaKo) in quiet and by the measurement of a speech reception threshold in an interfering noise at a moderate level (45 dB SPL) using either the Göttingen or the Oldenburg sentence test (criterion: 2 dB improvement in SNR, "signal-to-noise ratio"). This procedure was investigated in a group of 38 participants with a sensorineural hearing impairment (mild, moderate or severe hearing loss) and 11 volunteers with normal hearing. On average, comparable indications were achieved. Participants with a mild hearing loss and a selective problem with listening in interfering noise were assessed more fairly. PMID:21769581

  1. 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

  2. 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

  3. 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

  4. Treatment for Progressive Hearing Loss Due to Paget's Disease of Bone - A Case Report and Literature Review.

    PubMed

    Aoki, Mitsuhiro; Tanahashi, Shigeaki; Mizuta, Keisuke; Kato, Hiroki

    2015-12-01

    Paget's disease is a common bone remodeling disorder that typically begins with excessive bone resorption in the elderly. Bilateral progressive hearing loss is the most frequently encountered complication of Paget's disease. The types of hearing loss identified by audiometry are conductive, sensorineural, or both. However, the precise mechanism of hearing loss remains unclear, and the treatment has been controversial. We present a 73-year-old man who suffered from bilateral progressive hearing loss due to Paget's disease. Potent bisphosphonates, oral risedronate in daily adjusted dosages for 6 months, did not decrease or suppress the worsening of the hearing loss. The Nucleus CI24 Contour electrode array was successfully inserted on the left side without surgical and postoperative complications. The Japanese open set monosyllable word recognition test in a sound field at 65 dB had a result of 74%. This cochlear implantation can be an indication for cases of profound hearing loss due to Paget's disease. PMID:26915163

  5. Virtual instrument for testing the hearing impaired

    NASA Astrophysics Data System (ADS)

    Norian, K. H.

    2001-02-01

    LABVIEW programing was used to build a virtual instrument to assess the needs of individual hearing impaired subjects to enable them to hear speech in background noise. The instrument acquires the noisy speech, adaptively removes noise from speech, and then feeds the speech to the subject. Design changes can quickly and easily be made to the instrument to assess the specific requirements of the electronic circuit of the hearing aid for the individual subject. The instrument provides a novel way of providing custom electronics for hearing aids.

  6. 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

  7. 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

  8. Rapid word-learning in normal-hearing and hearing-impaired children

    PubMed Central

    Pittman, A. L.; Lewis, D. E.; Hoover, B. M.; Stelmachowicz, P. G.

    2008-01-01

    Objective This study examined rapid word learning in 5- to 14-year-old children with normal and impaired hearing. The effects of age and receptive vocabulary were examined as well as those of high-frequency amplification. Novel words were low-pass filtered at 4 kHz (typical of current amplification devices) and at 9 kHz. It was hypothesized that: 1) the children with normal hearing would learn more words than the children with hearing loss, 2) word learning would increase with age and receptive vocabulary for both groups, and 3) both groups would benefit from a broader frequency bandwidth. Design Sixty children with normal hearing and 37 children with moderate sensorineural hearing losses participated in this study. Each child viewed a 4-minute animated slideshow containing 8 nonsense words created using the 24 English consonant phonemes (3 consonants per word). Each word was repeated 3 times. Half of the 8 words were low-pass filtered at 4 kHz and half were filtered at 9 kHz. After viewing the story twice, each child was asked to identify the words from among pictures in the slide show. Prior to testing, a measure of current receptive vocabulary was obtained using the Peabody Picture Vocabulary Test (PPVT-III). Results The PPVT-III scores of the hearing-impaired children were consistently poorer than those of the normal-hearing children across the age range tested. A similar pattern of results was observed for word-learning in that the performance of the hearing-impaired children was significantly poorer than that of the normal-hearing children. Further analysis of the PPVT and word learning scores suggested that although word learning was delayed in the hearing-impaired children, their performance was consistent with their receptive vocabularies. Additionally, no correlation was found between overall performance and the age of identification, age of amplification, or years of amplification in the children with hearing loss. Results also revealed a small increase

  9. Hearing Impairments

    NASA Astrophysics Data System (ADS)

    Cavender, Anna; Ladner, Richard E.

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

  10. Attitudes to hearing difficulty and hearing aids and the outcome of audiological rehabilitation.

    PubMed

    Brooks, D N; Hallam, R S

    1998-08-01

    First time hearing aid candidates (N = 135) in a NHS setting were administered the Hearing Attitudes in Rehabilitation Questionnaire (HARQ) designed to assess attitudes to acquired hearing loss and hearing aids and 92% of them were followed up 3-9 months after fitting. Attitude scores, age, sex and sensory thresholds were related to six self-report outcome measures by use of logistic regression. The major findings were that patients who were least distressed by their hearing difficulties and reported not wanting or needing a hearing aid used their aids least frequently and evaluated them less highly in listening situations. An attitude that wearing a hearing aid was stigmatizing was not predictive of outcome except a report of more difficulty in handling the aid. There were some low but significant correlations between attitudes and sensory thresholds and thresholds also contributed to the prediction of outcome in a few instances. PMID:9923983

  11. 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.

  12. 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

  13. 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)

  14. 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 ...

  15. 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 ...

  16. Early Prediction of Postmeningitic Hearing Loss in Children Using Magnetic Resonance Imaging

    PubMed Central

    Kopelovich, Jonathan C.; Germiller, John A.; Laury, Adrienne M.; Shah, Samir S.; Pollock, Avrum N.

    2013-01-01

    Objective To determine whether early gadolinium-enhanced magnetic resonance imaging (GdMRI) can reliably detect meningitic labyrinthitis and thereby predict which children are at high risk for hearing loss. Permanent sensorineural hearing loss (SNHL) remains a common sequela of bacterial meningitis, and early diagnosis of the associated suppurative labyrinthitis can be difficult, especially in critically ill, sedated patients and young children. Design Retrospective cohort study. Setting Tertiary pediatric hospital. Participants Twenty-three survivors of bacterial meningitis (median age, 15 months [range, 3 months–14 years]) who had undergone brain GdMRI during the acute disease and had subsequent ear-specific audiometric data. Main Outcome Measure Blinded to disease and outcome, a neuroradiologist rated the relative enhancement of each cochlea on T1-weighted images using a 4-point scale. Scores were then correlated with the degree of hearing loss on subsequent testing. Results Sensorineural hearing loss occurred in 15 of 46 ears (8 of 23 patients). Enhancement on GdMRI was detected in 13 of the 15 ears that later developed SNHL but was absent in all 31 unaffected ears. Thus, GdMRI was 87% sensitive and 100% specific for predicting which ears would develop permanent SNHL. In the subgroup with pneumococcal meningitis (n=15), GdMRI was 100% sensitive and 100% specific. Labyrinthine enhancement was detectable as early as 1 day after diagnosis. Conclusion Gadolinium-enhanced MRI detected meningitic labyrinthitis at early stages and accurately predicted which patients would later develop hearing loss. PMID:21339394

  17. Evaluation of the use of a questionnaire to detect hearing loss in Kenyan pre-school children.

    PubMed

    Newton, V E; Macharia, I; Mugwe, P; Ototo, B; Kan, S W

    2001-03-01

    In developing countries, there is a lack of trained personnel and testing equipment to facilitate the early detection of hearing impairment in children. A questionnaire offers a low cost option and the value of this for detecting hearing impairment in pre-school children was determined in several districts in Kenya. The questionnaire was completed by either teachers, parents/carers or community nurses. The children were subsequently tested using pure tone audiometry and visual examination of the ear by ENT Clinical Officers, who were not given prior access to the results of the questionnaire. A total of 757 (88%) questionnaires were completed. Of the 735 children, who could be tested using pure tone audiometry, four were found to have a unilateral hearing impairment and one was detected by the questionnaire. A total of 13 children had a bilateral hearing impairment >40 dB HL. All were detected using the questionnaire. There were eight males and five females with ages ranging from 4.2 to 6.9 years, mean age 5.7 years and median age 5.8 years. Eight had a sensorineural hearing impairment and two a mixed hearing impairment. Three of the children with a sensorineural hearing loss had a family history of hearing impairment. No question detected all children with a hearing impairment and some questions were more discerning than others. There was 100% sensitivity for the questionnaire when a hearing loss of >40 dB was considered, but specificity was lower at 75%. Negative predictive value was 100%, but the positive predictive value was low, 6.75%. It was concluded that a questionnaire of this nature could be usefully applied at Primary Health Care level for detecting hearing impairment at the pre-school stage. There would be need for services available for diagnosis, treatment and habilitation before a screening programme was introduced. PMID:11223455

  18. [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

  19. Sudden hearing loss due to oxaliplatin use in a patient with colon cancer.

    PubMed

    Güvenç, M Güven; Dizdar, Denizhan; Dizdar, Senem Kurt; Okutur, Sadi Kerem; Demir, Gökhan

    2016-08-01

    Oxaliplatin is used to treat advanced colorectal cancer. Platinum-containing chemotherapeutic agents are known to be ototoxic. However, ototoxicity is rare with newer generation platinum-derived agents, such as oxaliplatin. This case report presents a rare case of sudden unilateral sensorineural hearing loss following intravenous (IV) infusion of oxaliplatin in a 64-year-old woman with advanced colon cancer. The hearing loss was severe and did not respond to treatment. To the best of our knowledge, this is the fifth reported case of oxaliplatin ototoxicity. Although oxaliplatin ototoxicity is rare, physicians must be aware of this important adverse effect, and an audiometric evaluation must be performed when necessary. Patients treated with oxaliplatin should be followed closely for early signs and symptoms of hearing loss, and if hearing loss is detected, treatment should be stopped immediately. PMID:25872564

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

    NASA Astrophysics Data System (ADS)

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

    2002-05-01

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

  1. Seizures, sensorineural deafness, ataxia, mental retardation, and electrolyte imbalance (SeSAME syndrome) caused by mutations in KCNJ10

    PubMed Central

    Scholl, Ute I.; Choi, Murim; Liu, Tiewen; Ramaekers, Vincent T.; Häusler, Martin G.; Grimmer, Joanne; Tobe, Sheldon W.; Farhi, Anita; Nelson-Williams, Carol; Lifton, Richard P.

    2009-01-01

    We describe members of 4 kindreds with a previously unrecognized syndrome characterized by seizures, sensorineural deafness, ataxia, mental retardation, and electrolyte imbalance (hypokalemia, metabolic alkalosis, and hypomagnesemia). By analysis of linkage we localize the putative causative gene to a 2.5-Mb segment of chromosome 1q23.2–23.3. Direct DNA sequencing of KCNJ10, which encodes an inwardly rectifying K+ channel, identifies previously unidentified missense or nonsense mutations on both alleles in all affected subjects. These mutations alter highly conserved amino acids and are absent among control chromosomes. Many of these mutations have been shown to cause loss of function in related K+ channels. These findings demonstrate that loss-of-function mutations in KCNJ10 cause this syndrome, which we name SeSAME. KCNJ10 is expressed in glia in the brain and spinal cord, where it is believed to take up K+ released by neuronal repolarization, in cochlea, where it is involved in the generation of endolymph, and on the basolateral membrane in the distal nephron. We propose that KCNJ10 is required in the kidney for normal salt reabsorption in the distal convoluted tubule because of the need for K+ recycling across the basolateral membrane to enable normal activity of the Na+-K+-ATPase; loss of this function accounts for the observed electrolyte defects. Mice deficient for KCNJ10 show a related phenotype with seizures, ataxia, and hearing loss, further supporting KCNJ10's role in this syndrome. These findings define a unique human syndrome, and establish the essential role of basolateral K+ channels in renal electrolyte homeostasis. PMID:19289823

  2. 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

  3. Frequency selectivity in canaries with a hereditary hearing loss

    NASA Astrophysics Data System (ADS)

    Lauer, Amanda M.; Dooling, Robert J.

    2002-05-01

    Sensorineural hearing loss is associated with reduced frequency selectivity due to the broadening of the auditory filters in mammals. In European starlings, hearing loss caused by the ototoxic drug kanamycin results in the widening of auditory filters at 5 kHz [Marean et al. (1998)]. In the present study, we examine frequency selectivity in a bird with a permanent hereditary hearing impairment, the Belgian Waterslager (BWS) canary. This strain of canary has long been bred for its loud, low-frequency song, and has been shown to have a hearing loss primarily at higher frequencies (2 kHz and above). Using operant conditioning and the method of constant stimuli, thresholds for detecting pure tones in flat-spectrum broadband noise were measured in BWS and non-BWS canaries. Critical ratios were calculated for comparison with other species of birds. At higher frequencies, critical ratios for BWS canaries were much larger than those of non-BWS canaries and other birds, suggesting reduced frequency selectivity in the region of the birds' hearing loss. [Work supported by NIDCD R01DC001372 to RJD and Brenda M. Ryals.

  4. Informational Masking in Normal-Hearing and Hearing-Impaired Listeners Measured in a Nonspeech Pattern Identification Task.

    PubMed

    Roverud, Elin; Best, Virginia; Mason, Christine R; Swaminathan, Jayaganesh; Kidd, Gerald

    2016-01-01

    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

  5. 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

  6. Masking of spondees by interrupted noise in hearing-impaired listeners.

    PubMed

    Punch, J L

    1978-01-01

    The effects of changing the duty cycle of an interrupted-broad band masker on the spondee thresholds of hearing-impaired subjects were explored. Two diagnostic groups of sensorineural hearing loss, presbycusics and cochlear otosclerotics, were investigated. The interruption rate of the masker was 10/sec, and its duty cycle was varied at 25% intervals. Thresholds also were obtained in continuous noise and in quiet. Results when compared with earlier data from normal listeners revealed that subjects with sensorineural impairment exhibited poorer performance in all instances, including the continuous noise condition, when mean masker levels were adjusted to comparable sound pressure levels. The overall pattern of masking was more similar for the normal and hearing-impaired groups when performance was equated in terms of the mean threshold shift each group experienced in continuous noise. Even under these circumstances, however, hearing-impaired subjects demostrated notably greater residual masking under the 25 and 50% noise-on conditions. A major determinant of speech reception in fluctuating noise backgrounds is the dependence of the pattern of masking upon the difference between an individual's masked threshold in continuous noise and his threshold in quiet, rather than upon the sound pressure level of the masker. PMID:681196

  7. Frequency of Sensory Neural Hearing Loss in Major Beta-Thalassemias in Southern Iran

    PubMed Central

    Faramarzi, Abolhassan; Karimi, Mehran; Heydari, Seyed-Taghi; Shishegar, Mahmoud; Kaviani, Masoud

    2010-01-01

    Objective The thalassemias are among the most common genetic disorders worldwide, occurring more frequently in the Mediterranean region. The aim of this study was to determined frequency of sensory-neural hearing loss in major ß- thalassemias transfusion dependent patients in south of Iran. Methods A cross sectional study on 308 cases of major beta-thalassemia patients referring to Thalassemia Center of Shiraz University of Medical Sciences between 2006–2007 years. The diagnosis of ß- thalassemia major was based on clinical history, complete blood count and hemoglobine electrophoresis. Clinical data such as serum ferritin level, deferoxamine (DFO) dose, mean daily doses of DFO (mg/kg) and audiometric variables was recorded. Findings Out of 308 cases, 283 (96.5%) had normal hearing and 10 (3.5%) sensorineural hearing loss. There was no statically significant difference between two groups regarding mean age, weight, age at the first blood transfusion, age at the first DFO infusion. Conclusion We found the lowest incidence of sensorineural hearing loss in a large population of patients suffered from major thalassemia who received DFO. We show that DFO is not ototoxic at a low dose. When considering all related literature, as a whole there has been much critical misrepresentation about DFO ototoxicity. PMID:23056722

  8. Evaluation of hearing functions in patients with euthyroid Hashimoto's thyroiditis.

    PubMed

    Arduc, Ayse; Isık, Serhat; Allusoglu, Serpil; Iriz, Ayse; Dogan, Bercem Aycicek; Gocer, Celil; Tuna, Mazhar Muslim; Berker, Dilek; Guler, Serdar

    2015-12-01

    Sensorineural hearing loss has been reported in various autoimmune diseases. The relationship between Hashimoto's thyroiditis (HT) and the auditory system has not been previously evaluated. In this study, we investigated the effect of euthyroid HT on the hearing ability of adult patients. The study included 30 patients with newly diagnosed euthyroid HT and 30 age- and gender-matched healthy controls. All subjects had a normal otoscopic examination and tympanometry, and they were negative for rheumatoid factor, antinuclear, anti-smooth muscle, antimitochondrial, antineutrophilcytoplasmic, and antigliadin antibodies. Pure tone audiometry exams at 250, 500, 1000, 2000, 4000, 6000, and 8000 Hertz (Hz) were performed in both groups. Thyroid peroxidase antibody and thyroglobulin antibody (anti-Tg) levels were higher in HT group while TSH, free T4, free T3, plasma electrolytes, glucose, lipid profile, vitamin B12, and blood pressure measurements were similar between the two groups. Higher audiometric thresholds and a higher prevalence of hearing loss at 250, 500, and 6000 Hz were detected in the HT patients than in the healthy controls (P < 0.05). Hearing levels at 250 and 500 Hz correlated positively with anti-Tg levels (ρ = 0.650, P = 0.002; ρ = 0.719, P < 0.001, respectively), and this association remained significant in linear regression analysis. Anti-Tg-positive HT patients had higher hearing thresholds at 250 and 500 Hz than anti-Tg-negative HT patients. Hearing thresholds were similar between anti-Tg-negative HT patients and the control subjects. This study demonstrated that hearing functions are impaired in HT patients. Thyroid autoimmunity seems to have an important impact on a decreased hearing ability, particularly at lower frequencies, in this population of patients. PMID:25963023

  9. Relative benefits of linear analogue and advanced digital hearing aids.

    PubMed

    Wood, Sally A; Lutman, Mark E

    2004-03-01

    Speech recognition performance and self-reported benefit from linear analogue and advanced (digital) hearing aids were compared in 100 first-time hearing aid users with mild-to-moderate sensorineural hearing loss fitted monaurally with a behind-the-ear (BTE) hearing aid in a single-blind randomized crossover trial. Subjects used each aid for 5 weeks in turn, with aid order balanced across subjects. Three alternative models of digital hearing aid were assigned to subjects according to a balanced design. Aid type was disguised to keep subjects blind within practical limitations. Aided speech recognition performance in noise was measured at speech levels of 65 and 75dB at a speech-to-noise ratio (SNR) of +2dB for closed sets of single words. Self-rated benefit was measured using the Abbreviated Profile of Hearing Aid Benefit (APHAB) and the Glasgow Hearing Aid Benefit Profile (GHABP). Quality of life, hearing aid use and user preferences were also assessed. Speech recognition scores with the digital aids were significantly better at 75dB than with the analogue aids Self-reported benefit (APHAB, GHABP) and improvement in quality of life were generally not significantly different between analogue and digital aids, although aversiveness measured with the APHAB was significantly lower with digital aids, and satisfaction measured with the GHABP was greater. The digital aids were preferred significantly more often than the analogue aids, with 61 subjects choosing their digital aid, 26 choosing the analogue aid, and nine being equivocal. Overall, this study shows advantages for advanced digital over simple linear analogue aids in terms of both objective and subjective outcomes, although average differences are not large. PMID:15198378

  10. 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

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

    SciTech Connect

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

    1988-03-01

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

  12. 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

  13. The A1555G Mutation in the 12S rRNA Gene of Human mtDNA: Recurrent Origins and Founder Events in Families Affected by Sensorineural Deafness

    PubMed Central

    Torroni, Antonio; Cruciani, Fulvio; Rengo, Chiara; Sellitto, Daniele; López-Bigas, Núria; Rabionet, Raquel; Govea, Nancy; López de Munain, Adolfo; Sarduy, Maritza; Romero, Lourdes; Villamar, Manuela; del Castillo, Ignacio; Moreno, Felipe; Estivill, Xavier; Scozzari, Rosaria

    1999-01-01

    Summary The mtDNA variation of 50 Spanish and 4 Cuban families affected by nonsyndromic sensorineural deafness due to the A1555G mutation in the 12S rRNA gene was studied by high-resolution RFLP analysis and sequencing of the control region. Phylogenetic analyses of haplotypes and detailed survey of population controls revealed that the A1555G mutation can be attributed to ⩾30 independent mutational events among the 50 Spanish families and that it occurs on mtDNA haplogroups that are common in all European populations. This indicates that the relatively high detection rate of this mutation in Spain is not due to sampling biases or to a single major founder event. Moreover, the distribution of these mutational events on different haplogroups is compatible with a random occurrence of the A1555G mutation and tends to support the conclusion that mtDNA backgrounds do not play a significant role in the expression of the mutation. Overall, these findings appear to indicate that the rare detection of this mutation in other populations is most likely due to inadequacy in patient ascertainment and molecular screening. This probable lack of identification of the A1555G mutation in subjects affected by sensorineural hearing loss implies that their maternally related relatives are not benefiting from presymptomatic detection and information concerning their increased risk of ototoxicity due to aminoglycoside treatments. PMID:10521300

  14. Hospital-acquired pneumonia

    MedlinePlus

    ... this page: //medlineplus.gov/ency/article/000146.htm Hospital-acquired pneumonia To use the sharing features on this page, please enable JavaScript. Hospital-acquired pneumonia is an infection of the lungs ...

  15. 12 CFR 622.7 - Conduct of hearings.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... the record. In the event the hearing is public, transcripts will be furnished to interested persons... communication has knowledge that the proceeding will be noticed, the prohibitions apply from the time such knowledge is acquired....

  16. Hearing Disorders and Deafness

    MedlinePlus

    ... you from hearing sound at all. What causes hearing loss? Some possibilities are Heredity Diseases such as ear ... noise Aging There are two main types of hearing loss. One happens when your inner ear or auditory ...

  17. 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. ...

  18. 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 ...

  19. 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

  20. 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

  1. 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 ...

  2. 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

  3. 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

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

    PubMed Central

    van Capelle, Carine I.; Goedegebure, Andre; Homans, Nienke C.; Hoeve, Hans L. J.; Reuser, Arnold J.

    2010-01-01

    Little information is available regarding the auditory function in Pompe patients. Hearing loss has been reported in classic infantile patients, but it is still unknown whether central nervous system involvement interferes with auditory function and whether enzyme replacement therapy can improve hearing. Auditory function has not been studied in children with milder forms of the disease. We analyzed repetitive auditory brainstem response measurements and pure tone audiometry in 24 children with Pompe disease. Only 1 of 13 patients with milder phenotypes showed recurrent conductive hearing loss, while 10 out of 11 classic infantile patients had sensorineural hearing defects. These patients also had a high prevalence of conductive hearing loss. Five patients showed evidence of mild retrocochlear pathology, suggestive of glycogen accumulation in the central nervous system. Hearing loss persisted during therapy in all patients. The results emphasize the need for careful monitoring of auditory function in classic infantile Pompe patients, and for early implementation of hearing aids to protect speech and language development. PMID:20596893

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

    PubMed Central

    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

    2015-01-01

    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. PMID:26464487

  7. [Hearing disorders in obliteration of the carotid artery. 2. Contribution to hearing loss in the aged].

    PubMed

    Böhme, G

    1989-07-01

    Otologic-audiologic examination was carried out in 75 patients (between 42 and 86 years of age; average age: 65 years) with confirmed internal angiologic obliteration of the carotid artery, either unilateral or bilateral. There were 51 unilateral and 24 bilateral stenoses/occlusions. Diseases of the ear were excluded clinically and audiologically. - The mean hearing loss shows a sensorineural high tone loss in the tone audiogram. The range of scatter of high tone loss increases proportionally to the increase of frequency. - Compared with the physiological examination of geriatric patients, the total word comprehension and especially the minimal discrimination loss point towards a pathologic impairment of hearing. The total word comprehension amounts to 250.79% in the 51-60 age group, 237.79% in the 61-70 age group, 175.83% in persons aged 71-80 years, and 118.33% for those over 80 years of age. The minimal discrimination loss comprises 5.83% in the 51-60 age group, 9.79% in the 61-70 age group, 22.50% in patients between 71 and 80 years, and 48.00% in those over 80 years. - No connection between the extent of loss of hearing and the stage of obliteration of the carotid artery can be shown. However, the decompensation of total word comprehension and especially the minimal discrimination loss is a very important sign. - These findings contribute towards a differentiation of physiologic and pathologic hearing diseases in old age with particulas reference to the underlying arteriosclerotic disease. PMID:2669772

  8. 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

  9. 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

    2016-01-01

    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

  10. Acquired reactive perforating collagenosis.

    PubMed

    Basak, P Y; Turkmen, C

    2001-01-01

    Acquired perforating disorder has been recognized as an uncommon distinct dermatosis in which altered collagen is eliminated through the epidermis. Several disorders accompanied by itching and scratching were reported to be associated with reactive perforating collagenosis. A 67-year-old white woman diagnosed as acquired reactive perforating collagenosis with poorly controlled diabetes mellitus and congestive cardiac failure is presented. PMID:11525959

  11. [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

  12. The master hearing aid.

    PubMed

    Curran, James R; Galster, Jason A

    2013-06-01

    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

  13. 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

  14. Audiologic Phenotype and Progression in GJB2 (Connexin 26) Hearing Loss

    PubMed Central

    Kenna, Margaret A.; Feldman, Henry A.; Neault, Marilyn W.; Frangulov, Anna; Wu, Bai-Lin; Fligor, Brian; Rehm, Heidi L.

    2015-01-01

    Objectives To document the audiologic phenotype of children with biallelic GJB2 (connexin 26) mutations, and to correlate it with the genotype. Design Prospective, observational study. Setting Tertiary care children’s hospital. Patients Infants and children with sensorineural hearing loss (SNHL). Intervention Sequencing of the GJB2 (connexin 26) gene. Main Outcome Measures Degree and progression of SNHL. Results From December 1, 1998, through November 30, 2006, 126 children with biallelic GJB2 mutations were identified. Of the 30 different mutations identified, 13 (43%) were truncating and 17 (57%) were nontruncating; 62 patients had 2 truncating, 30 had 1 truncating and 1 nontruncating, and 17 had 2 nontruncating mutations. Eighty-four patients (67%) initially had measurable hearing in the mild to severe range in at least 1 of 4 frequencies (500, 1000, 2000, or 4000 Hz). Of these 84 patients with residual hearing, 47 (56%) had some degree of progressive hearing loss. Patients with 2 truncating mutations had significantly worse hearing compared with all other groups. Patients who had 1 or 2 copies of either an M34T or a V37I allele had the mildest hearing loss. Conclusions Hearing loss owing to GJB2 mutations ranges from mild to profound and is usually congenital. More than 50% of patients will experience some hearing loss progression, generally gradually but occasionally precipitously. Hearing loss severity may be influenced by genetic factors, such as the degree of preserved protein function in nontruncating mutations, whereas hearing loss progression may be dependent on factors other than the connexin 26 protein. Genetic counseling for patients with GJB2 mutations should include the variable audiologic phenotype and the possibility of progression. PMID:20083784

  15. Carboxy alkyl esters of Uncaria tomentosa augment recovery of sensorineural functions following noise injury.

    PubMed

    Guthrie, O'neil W; Gearhart, Caroline A; Fulton, Sherry; Fechter, Laurence D

    2011-08-17

    This study tested the hypothesis that hydrophilic chemotypes of the medicinal vine Uncaria tomentosa (UT) would facilitate recovery of sensorineural functions following exposure to a damaging level of noise. The particular chemotypes investigated were carboxy alkyl esters (CAE) which are known to exhibit multifunctional cytoprotective properties that include: enhanced cellular DNA repair, antioxidation and anti-inflammation. Long-Evans rats were divided into four treatment groups: vehicle-control, noise-only, CAE-only and CAE+noise. The noise exposure was an 8kHz octave band of noise at 105dB SPL for 4h. Outer hair cell (OHC) function was measured with the cubic 2f(1)-f(2) distortion product otoacoustic emissions (DPOAE) at the start of the study (baseline) and at time-points that corresponded to 1day, 1week and 4weeks post-noise exposure to determine within-group effects. Compound action potentials to puretone stimuli were recorded from the VIIIth craniofacial nerve at 4weeks post-noise exposure to determine between-group effects. Additionally, cytocochleograms were constructed for each row of OHCs from each group. Noise exposure produced significant sensorineural impairments. However, CAE treatment facilitated almost complete recovery of OHC function and limited the magnitude of cell loss. The loss of neural sensitivity to puretone stimuli was inhibited with CAE treatment. Therefore, it appears that the multifunctional cytoprotective capacity of CAE from UT may generalize to otoprotection from acoustic over-exposure. PMID:21762882

  16. 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

  17. 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

  18. MARVELD2 (DFNB49) Mutations in the Hearing Impaired Central European Roma Population - Prevalence, Clinical Impact and the Common Origin

    PubMed Central

    Mátyás, Petra; Ficek, Andrej; Hučková, Miloslava; Sůrová, Martina; Šafka-Brožková, Dana; Anwar, Saima; Bene, Judit; Straka, Slavomír; Janicsek, Ingrid; Ahmed, Zubair M.; Seeman, Pavel; Melegh, Béla; Profant, Milan; Klimeš, Iwar; Riazuddin, Saima; Kádasi, Ľudevít; Gašperíková, Daniela

    2015-01-01

    Background In the present study we aimed: 1) To establish the prevalence and clinical impact of DFNB49 mutations in deaf Roma from 2 Central European countries (Slovakia and Hungary), and 2) to analyze a possible common origin of the c.1331+2T>C mutation among Roma and Pakistani mutation carriers identified in the present and previous studies. Methods We sequenced 6 exons of the MARVELD2 gene in a group of 143 unrelated hearing impaired Slovak Roma patients. Simultaneously, we used RFLP to detect the c.1331+2T>C mutation in 85 Hungarian deaf Roma patients, control groups of 702 normal hearing Romanies from both countries and 375 hearing impaired Slovak Caucasians. We analyzed the haplotype using 21 SNPs spanning a 5.34Mb around the mutation c.1331+2T>C. Results One pathogenic mutation (c.1331+2T>C) was identified in 12 homozygous hearing impaired Roma patients. Allele frequency of this mutation was higher in Hungarian (10%) than in Slovak (3.85%) Roma patients. The identified common haplotype in Roma patients was defined by 18 SNP markers (3.89 Mb). Fourteen common SNPs were also shared among Pakistani and Roma homozygotes. Biallelic mutation carriers suffered from prelingual bilateral moderate to profound sensorineural hearing loss. Conclusions We demonstrate different frequencies of the c.1331+2T>C mutation in hearing impaired Romanies from 3 Central European countries. In addition, our results provide support for the hypothesis of a possible common ancestor of the Slovak, Hungarian and Czech Roma as well as Pakistani deaf patients. Testing for the c.1331+2T>C mutation may be recommended in GJB2 negative Roma cases with early-onset sensorineural hearing loss. PMID:25885414

  19. Comparison of Consanguinity between Parents of Hearing Impaired and Public School Children with Estimation of Risk.

    PubMed

    Sattar, M A; Sultana, M T

    2015-10-01

    Deafness is the hidden disability and the most common human sensory defects which lead to poor educational and employment prospects of childhood. Is there any association of consanguinity and hearing loss or are there any difference of association of consanguinity and hearing loss in specialized and public school children and how much risk is associated?--were the research questions of this study. Total 428 participants have been selected randomly. Hearing impaired were 186 participants and 242 participants were normal hearing school boy. This was a case control, analytical, hypotheses testing study. In normal public school children group, consanguinity was present in 2.5% parents. The rest were married with non relatives. In parents of hearing impaired children group, consanguinity was very high (17.2%). Pearson chi-square test and Odds ratio analysis was done. The value was less than 0.05 and ratio was 8.173. The 'p' value of Pearson chi-square test was less than 0.05. So, the test was highly significant at 95% confidence interval. Odds ratio showed that the risk of profound sensorineural hearing loss in the baby of parents of consanguineous marriages 8.173 times higher than that of non consanguineous marriages. PMID:26620013

  20. Massively Parallel Sequencing for Genetic Diagnosis of Hearing Loss: The New Standard of Care

    PubMed Central

    Shearer, A. Eliot; Smith, Richard J.H.

    2016-01-01

    Objective To evaluate the use of new genetic sequencing techniques for comprehensive genetic testing for hearing loss. Data Sources Articles were identified from PubMed and Google Scholar databases using pertinent search terms. Review Methods Literature search identified 30 studies as candidates that met search criteria. Three studies were excluded and eight studies were found to be case reports. 20 studies were included for review analysis including seven studies that evaluated controls and 16 studies that evaluated patients with unknown causes of hearing loss; three studies evaluated both controls and patients. Conclusions In the 20 studies included in review analysis, 426 control samples and 603 patients with unknown causes of hearing loss underwent comprehensive genetic diagnosis for hearing loss using massively parallel sequencing. Control analysis showed a sensitivity and specificity > 99%, sufficient for clinical use of these tests. The overall diagnostic rate was 41% (range 10% to 83%) and varied based on several factors including inheritance and pre-screening prior to comprehensive testing. There were significant differences in platforms available in regards to number and type of genes included and whether copy number variations were examined. Based on these results, comprehensive genetic testing should form the cornerstone of a tiered approach to clinical evaluation of patients with hearing loss along with history, physical exam, and audiometry and can determine further testing that may be required, if any. Implications for Practice Comprehensive genetic testing has become the new standard of care for genetic testing for patients with sensorineural hearing loss. PMID:26084827

  1. 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

  2. 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

  3. Targeting Cholesterol Homeostasis to Fight Hearing Loss: A New Perspective

    PubMed Central

    Malgrange, Brigitte; Varela-Nieto, Isabel; de Medina, Philippe; Paillasse, Michael R.

    2015-01-01

    Sensorineural hearing loss (SNHL) is a major pathology of the inner ear that affects nearly 600 million people worldwide. Despite intensive researches, this major health problem remains without satisfactory solutions. The pathophysiological mechanisms involved in SNHL include oxidative stress, excitotoxicity, inflammation, and ischemia, resulting in synaptic loss, axonal degeneration, and apoptosis of spiral ganglion neurons. The mechanisms associated with SNHL are shared with other neurodegenerative disorders. Cholesterol homeostasis is central to numerous pathologies including neurodegenerative diseases and cholesterol regulates major processes involved in neurons survival and function. The role of cholesterol homeostasis in the physiopathology of inner ear is largely unexplored. In this review, we discuss the findings concerning cholesterol homeostasis in neurodegenerative diseases and whether it should be translated into potential therapeutic strategies for the treatment of SNHL. PMID:25688206

  4. Sensorineural deafness

    MedlinePlus

    ... or dizzy (more common with Meniere's disease and acoustic neuromas ) Ringing or buzzing sound in the ears ( ... a long time Meniere's disease Tumor, such as acoustic neuroma Use of certain medicines Working around loud ...

  5. Acquired Cystic Kidney Disease

    MedlinePlus

    ... a kidney transplant or blood-filtering treatments called dialysis. The cysts are more likely to develop in people who are on kidney dialysis. The chance of developing acquired cystic kidney disease ...

  6. 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.

  7. The effects of hearing loss on the contribution of high- and low-frequency speech information to speech understanding. II. Sloping hearing lossa)

    PubMed Central

    Hornsby, Benjamin W. Y.; Ricketts, Todd A.

    2009-01-01

    The speech understanding of persons with sloping high-frequency (HF) hearing impairment (HI) was compared to normal hearing (NH) controls and previous research on persons with “flat” losses to examine how hearing loss configuration affects the contribution of speech information in various frequency regions. Speech understanding was assessed at multiple low- and high-pass filter cutoff frequencies. Crossover frequencies, defined as the cutoff frequencies at which low- and high-pass filtering yielded equivalent performance, were significantly lower for the sloping HI, compared to NH, group suggesting that HF HI limits the utility of HF speech information. Speech intelligibility index calculations suggest this limited utility was not due simply to reduced audibility but also to the negative effects of high presentation levels and a poorer-than-normal use of speech information in the frequency region with the greatest hearing loss (the HF regions). This deficit was comparable, however, to that seen in low-frequency regions of persons with similar HF thresholds and “flat” hearing losses suggesting that sensorineural HI results in a “uniform,” rather than frequency-specific, deficit in speech understanding, at least for persons with HF thresholds up to 60-80 dB HL. PMID:16583917

  8. Spectral and binaural loudness summation for hearing-impaired listeners.

    PubMed

    Oetting, Dirk; Hohmann, Volker; Appell, Jens-E; Kollmeier, Birger; Ewert, Stephan D

    2016-05-01

    Sensorineural hearing loss typically results in a steepened loudness function and a reduced dynamic range from elevated thresholds to uncomfortably loud levels for narrowband and broadband signals. Restoring narrowband loudness perception for hearing-impaired (HI) listeners can lead to overly loud perception of broadband signals and it is unclear how binaural presentation affects loudness perception in this case. Here, loudness perception quantified by categorical loudness scaling for nine normal-hearing (NH) and ten HI listeners was compared for signals with different bandwidth and different spectral shape in monaural and in binaural conditions. For the HI listeners, frequency- and level-dependent amplification was used to match the narrowband monaural loudness functions of the NH listeners. The average loudness functions for NH and HI listeners showed good agreement for monaural broadband signals. However, HI listeners showed substantially greater loudness for binaural broadband signals than NH listeners: on average a 14.1 dB lower level was required to reach "very loud" (range 30.8 to -3.7 dB). Overall, with narrowband loudness compensation, a given binaural loudness for broadband signals above "medium loud" was reached at systematically lower levels for HI than for NH listeners. Such increased binaural loudness summation was not found for loudness categories below "medium loud" or for narrowband signals. Large individual variations in the increased loudness summation were observed and could not be explained by the audiogram or the narrowband loudness functions. PMID:27006003

  9. 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

  10. 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

  11. 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, ...

  12. 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

  13. Effects of release time and directionality on unilateral and bilateral hearing aid fittings in complex sound fields.

    PubMed

    Novick, M L; Bentler, R A; Dittberner, A; Flamme, G A

    2001-01-01

    In studies to date, the effectiveness of the directional microphone has been investigated independently of the signal processing scheme used in the hearing aid. In addition, the number and placement of the background noise speakers can create an advantage for a particular polar pattern (i.e., cardioid, supercardioid, and hypercardioid) in any laboratory design. With these considerations in mind, the purpose of this investigation was twofold: (1) to determine the effect of different amplitude-compression release times on speech perception ability in noise, measured with directional microphone hearing aids, and (2) to determine the impact of environment (classroom vs anechoic chamber) on those measures. Ten subjects with mild to moderate sensorineural hearing loss participated. Using an eight-speaker complex sound field, speech perception was assessed in an anechoic chamber and a typical classroom environment. None of the release times resulted in superior performance in either the anechoic or classroom environment. PMID:11791940

  14. The Effect of Language Development on the Acquisition of Reading Skills in the Elementary Mainstreamed Hearing Impaired Student.

    ERIC Educational Resources Information Center

    Johnston, Judy F.; Reed, Shirley A.

    The language of hearing impaired students differs from that of their hearing peers, and can affect their ability to comprehend printed material. Language can be defined as the knowledge of the integration of semantics, syntax, and pragmatics. Hearing impaired children will have difficulty in acquiring language at a normal developmental rate…

  15. ‘Ecstasy’ Enhances Noise-Induced Hearing Loss

    PubMed Central

    Church, Michael W.; Zhang, Jinsheng S.; Langford, Megan M.; Perrine, Shane A.

    2013-01-01

    ‘Ecstasy’ or 3,4-methylenedioxy-N-methamphetamine (MDMA) is an amphetamine abused for its euphoric, empathogenic, hallucinatory, and stimulant effects. It is also used to treat certain psychiatric disorders. Common settings for Ecstasy use are nightclubs and “rave” parties where participants consume MDMA and dance to loud music. One concern with the club setting is that exposure to loud sounds can cause permanent sensorineural hearing loss. Another concern is that consumption of MDMA may enhance such hearing loss. Whereas this latter possibility has not been investigated, this study tested the hypothesis that MDMA enhances noise-induced hearing loss (NIHL) by exposing rats to either MDMA, noise trauma, both MDMA and noise, or neither treatment. MDMA was given in a binge pattern of 5 mg/kg per intraperitoneal injections every 2 h for a total of four injections to animals in the two MDMA-treated groups (MDMA-only and Noise+MDMA). Saline injections were given to the animals in the two non-MDMA groups (Control and Noise-only). Following the final injection, noise trauma was induced by a 10 kHz tone at 120 dB SPL for 1 h to animals in the two noise trauma-treated groups (Noise-only and Noise+MDMA). Hearing loss was assessed by the auditory brainstem response (ABR) and cochlear histology. Results showed that MDMA enhanced NIHL compared to Noise-only and that MDMA alone caused no hearing loss. This implies that “clubbers” and “rave-goers” are exacerbating the amount of NIHL when they consume MDMA and listen to loud sounds. In contrast to earlier reports, the present study found that MDMA by itself caused no changes in the click-evoked ABR’s wave latencies or amplitudes. PMID:23711768

  16. 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

  17. 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

  18. Pontobulbar palsy and sensorineural deafness (Brown-Vialetto-van Laere syndrome): the first case from Libya.

    PubMed

    Dakhil, Fathi O; Bensreiti, Salah M; Zew, Mohammad H

    2010-08-01

    We report the first case of Brown-Vialetto-van Laere syndrome from Libya. A 27-year-old female patient with progressive pontobulbar palsy, sensorineural deafness and generalized muscle weakness is described. Although she was born to consanguineous first cousin parents, she was the only one affected among five brothers and six sisters. The first symptoms appeared at the age of 18 years with tinnitus, weakness and unsteadiness that progressed over the course of a few weeks to deafness, blurring of vision, dysphagia, and dysarthria. Over the last nine years her symptoms progressed slowly, although interrupted with periods of rapid deterioration. Fifty-eight cases have been reported since the first case was described in 1894. PMID:20001484

  19. Infantile variant of Bartter syndrome and sensorineural deafness: A new autosomal recessive disorder

    SciTech Connect

    Landau, D.; Shalev, H.; Carmi, Rivka; Ohaly, M.

    1995-12-04

    The infantile variant of Bartter syndrome (IBS) is usually associated with maternal polyhydramnios, premature birth, postnatal polyuria and hypokalemic hypochloremic metabolic alkalosis and a typical appearance. IBS is thought to be an autosomal recessive trait. Several congenital tubular defects are associated with sensorineural deafness (SND). However, an association between the IBS and SND has not been reported so far. Here we describe 5 children of an extended consanguineous Bedouin family with IBS and SND. In 3 of the cases, the typical electrolyte imbalance and facial appearance were detected neonatally. SND was detected as early as age 1 month, suggesting either coincidental homozygotization of 2 recessive genes or a pleiotropic effect of one autosomal recessive gene. This association suggests that evaluation of SND is warranted in every case of IBS. 35 refs., 2 figs., 2 tabs.

  20. X-linked ocular albinism and sensorineural deafness: Linkage to Xp22. 3

    SciTech Connect

    Winship, I.M.; Babaya, M.; Ramesar, R.S. )

    1993-11-01

    X-linked ocular albinism with late-onset sensorineural deafness (OASD) is an autonomous disorder that poses significant clinical problems, causing affected individuals to be blind and deaf by early middle age. Classical X-linked ocular albinism (without deafness; OA1) has recently been linked to markers in the Xp22.2-Xp22.3 region of the human genome. In the present report, a large South African family with OASD was investigated at the molecular level and tight linkage was found to the DXS452 locus at Xp22.3 using 25 informative meioses, with a maximum lod score of 7.1 at a recombination fraction of 0.00. These findings suggest that OA1 and OASD are allelic variants or that they may be due to contiguous gene defects. 12 refs., 1 fig.

  1. Genetic Analysis of Genes Related to Tight Junction Function in the Korean Population with Non-Syndromic Hearing Loss

    PubMed Central

    Sagong, Borum; Cho, Hyun-Ju; Bae, Jae Woong; Kim, Jeongho; Lee, Jinwook; Park, Hong-Joon; Choi, Jae Young; Lee, Kyu-Yup; Kim, Un-Kyung

    2014-01-01

    Tight junctions (TJs) are essential components of eukaryotic cells, and serve as paracellular barriers and zippers between adjacent tissues. TJs are critical for normal functioning of the organ of Corti, a part of the inner ear that causes loss of sensorineural hearing when damaged. To investigate the relation between genes involved in TJ function and hereditary loss of sensorineural hearing in the Korean population, we selected the TJP2 and CLDN14 genes as candidates for gene screening of 135 Korean individuals. The TJP2 gene, mutation of which causes autosomal dominant non-syndromic hearing loss (ADNSHL), lies at the DFNA51 locus on chromosome 9. The CLDN14 gene, mutation of which causes autosomal recessive non-syndromic hearing loss (ARNSHL), lies at the DFNB29 locus on chromosome 21. In the present study, we conducted genetic analyses of the TJP2 and CLDN14 genes in 87 unrelated patients with ADNSHL and 48 unrelated patients with either ARNSHL or potentially sporadic hearing loss. We identified two pathogenic variations, c.334G>A (p.A112T) and c.3562A>G (p.T1188A), and ten single nucleotide polymorphisms (SNPs) in the TJP2 gene. We found eight non-pathogenic variations in the CLDN14 gene. These findings indicate that, whereas mutation of the TJP2 gene might cause ADNSHL, CLDN14 is not a major causative gene for ARNSHL in the Korean population studied. Our findings may improve the understanding of the genetic cause of non-syndromic hearing loss in the Korean population. PMID:24752540

  2. 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.

  3. Living with hearing loss

    MedlinePlus

    ... medlineplus.gov/ency/patientinstructions/000360.htm Living with hearing loss To use the sharing features on this page, please enable JavaScript. If you are living with hearing loss, you know that it takes extra effort to ...

  4. Hearing Loss: Screening Newborns

    MedlinePlus

    ... of this page please turn JavaScript on. Feature: Hearing Loss Screening Newborns Past Issues / Spring 2015 Table of ... of newborns in the U.S. are screened for hearing loss before they leave the hospital. Research improves the ...

  5. 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 ...

  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. 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 ...

  8. Hearing Disorders and Deafness

    MedlinePlus

    ... If you have trouble hearing, you can get help. Possible treatments include hearing aids, cochlear implants, special training, certain medicines, and surgery. NIH: National Institute on Deafness and Other Communication Disorders

  9. 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

  10. Simultaneous Screening of Multiple Mutations by Invader Assay Improves Molecular Diagnosis of Hereditary Hearing Loss: A Multicenter Study

    PubMed Central

    Usami, Shin-ichi; Nishio, Shin-ya; Nagano, Makoto; Abe, Satoko; Yamaguchi, Toshikazu

    2012-01-01

    Although etiological studies have shown genetic disorders to be a common cause of congenital/early-onset sensorineural hearing loss, there have been no detailed multicenter studies based on genetic testing. In the present report, 264 Japanese patients with bilateral sensorineural hearing loss from 33 ENT departments nationwide participated. For these patients, we first applied the Invader assay for screening 47 known mutations of 13 known deafness genes, followed by direct sequencing as necessary. A total of 78 (29.5%) subjects had at least one deafness gene mutation. Mutations were more frequently found in the patients with congenital or early-onset hearing loss, i.e., in those with an awareness age of 0–6 years, mutations were significantly higher (41.8%) than in patients with an older age of awareness (16.0%). Among the 13 genes, mutations in GJB2 and SLC26A4 were mainly found in congenital or early-onset patients, in contrast with mitochondrial mutations (12S rRNA m.1555A>G, tRNA(Leu(UUR)) m.3243A>G), which were predominantly found in older-onset patients. The present method of simultaneous screening of multiple deafness mutations by Invader assay followed by direct sequencing will enable us to detect deafness mutations in an efficient and practical manner for clinical use. PMID:22384008

  11. Deafness and Hearing Loss.

    ERIC Educational Resources Information Center

    National Information Center for Children and Youth with Disabilities, Washington, DC.

    This brief overview provides information on the definition, incidence, and characteristics of children with hearing impairments and deafness. The federal definitions of hearing impairment and deafness are provided. The different types of hearing loss are noted, including: (1) conductive (caused by diseases or obstructions in the outer or middle…

  12. 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. 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…

  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. Hearing Problems in Children

    MedlinePlus

    ... age 6 months. That's because children start learning speech and language long before they talk. Hearing problems can be temporary or permanent. Sometimes, ear infections, injuries or diseases affect hearing. If your child does not hear well, get help. NIH: National ...

  16. ABE. The Hearing Impaired.

    ERIC Educational Resources Information Center

    Carver, L. Sue

    This handbook was written to help teachers of adult basic education (ABE) adapt their teaching methods for hearing impaired persons. Written in a narrative format, the guide covers the following topics: ABE for the hearing impaired, hints for working with the hearing impaired without an interpreter, peer pairing, interpreters in the classroom…

  17. 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)

  18. 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.

  19. 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…

  20. Acquired von Willebrand disease.

    PubMed

    Petrini, P

    1999-05-01

    Acquired von Willebrand disease (AvWD) is a syndrome that has clinical and laboratory features similar to hereditary vWD. In contrast to the latter it occurs in patients without a family history of previous bleeding tendency. PMID:23401904

  1. Hearing impairment in patients with rheumatoid arthritis: association with anti-citrullinated protein antibodies.

    PubMed

    Lobo, Fabrício Silva; Dossi, Mario Orlando; Batista, Lígia; Shinzato, Márcia Midori

    2016-09-01

    It has been suggested that hearing impairment (HI) is one of the extra-articular features of rheumatoid arthritis (RA). Nevertheless, the prevalence and nature of HI in RA is still uncertain. The objectives were to study hearing function in patients with RA using audiometric tests and to examine whether HI correlates with autoantibodies. Hearing functions were investigated in 43 consecutive RA patients and 23 control subjects (less than 60 years old). Their sera were evaluated for the presence of rheumatoid factor (RF), anti-cyclic citrullinated peptide (anti-CCP), and anti-mutated citrullinated vimentin (anti-MCV) antibodies. HI was observed in 46.5 % of RA patients and in 30.4 % of control subjects, p = 0.32. HI was characterized as sensorineural in 80 and 85.7 % of RA patients and control subjects with HI, respectively, p = 1.00. RA patients had a worse hearing threshold for air conduction at 6 kHz in the right ear (p = 0.019) and had a decreased amplitude of otoacoustic emissions (OAEs) at 2 kHz bilaterally (p = 0.04) compared with control subjects. In the RA group, patients with and without HI were 80 and 34.78 % anti-CCP positive, respectively, p = 0.008. RA patients with and without HI were 85 and 43.48 % anti-MCV positive, respectively, p = 0.013. HI in RA patients was mainly sensorineural and was associated with anti-CCP and anti-MCV antibodies. PMID:27112144

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

    PubMed

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

    2016-04-21

    Ototoxicity is one of the most important adverse effects of cisplatin chemotherapy. As a common treatment of acute sensorineural hearing loss, systemic administration of steroids was demonstrated ineffective against cisplatin-induced hearing loss (CIHL) in published studies. The current study aimed to evaluate the potential protective effect of dexamethasone (DEX) encapsulated in polyethyleneglycol-coated polylactic acid (PEG-PLA) nanoparticles (DEX-NPs) against cisplatin-induced hearing loss following systemic administration. DEX was fabricated into PEG-PLA nanoparticles using emulsion and evaporation technique as previously reported. DEX or DEX-NPs was administered intraperitoneally to guinea pigs 1h before cisplatin administration. Auditory brainstem response (ABR) threshold shifts were measured at four frequencies (4, 8, 16, and 24kHz) 1 day before and three days after cisplatin injection. Cochlear morphology was examined to evaluate inner ear injury induced by cisplatin exposure. A single dose of DEX-NPs 1h before cisplatin treatment resulted in a significant preservation of the functional and structural properties of the cochlea, which was equivalent to the effect of multidose (3 days) DEX injection. In contrast, no significant protective effect was observed by single dose injection of DEX. The results of histological examination of the cochleae were consistent with the functional measurements. In conclusion, a single dose DEX-NPs significantly attenuated cisplatin ototoxicity in guinea pigs after systemic administration at both histological and functional levels indicating the potential therapeutic benefits of these nanoparticles for enhancing the delivery of DEX in acute sensorineural hearing loss. PMID:26971701

  3. Self-reported hearing loss among workers potentially exposed to industrial noise-United States

    SciTech Connect

    Not Available

    1988-04-15

    Noise-induced loss of hearing has been recognized as an occupational health problem since the 18th century. Occupational deafness is an irreversible, sensorineural condition that results from damage to the nerve cells of the inner ear. Recent estimates from surveys indicate that between 7.4 and 10.2 million people work at sites where the level of noise presents an increased risk of hearing loss (85 decibels (dBA) or higher). During the period of 1978-1987, an estimated $835 million was paid in workers' compensation claims for occupationally induced hearing impairment. To assess the prevalence of hearing-loss symptoms among adult workers in the United States, investigators from the National Institute of Occupational Safety and Health (NIOSH) recently analyzed data collected during the 1971 and 1977 National Health Interview Surveys (NHIS) conducted by the National Center for Health Statistics (NCHS). For this study, the prevalence of self-reported hearing loss was obtained for all persons over 17 years of age who were in the labor force at the time of interview. Data from the 1972-1974 National Occupational Hazard Survey (NOHS) were used to classify worksites by noise level. NOHS was conducted by NIOSH from 1972 to 1974 on a probability sample of approximately 5000 workplaces across the United States. The survey provides information on potential exposures of workers to chemical and physical agents. These data identified industries and occupations in which employees are exposed to continuous noise.

  4. Auditory steady state response in hearing assessment in infants with cytomegalovirus

    PubMed Central

    Silva, Daniela Polo C.; Lopez, Priscila Suman; Montovani, Jair Cortez

    2013-01-01

    OBJECTIVE: To report an infant with congenital cytomegalovirus and progressive sensorineural hearing loss, who was assessed by three methods of hearing evaluation. CASE DESCRIPTION: In the first audiometry, at four months of age, the infant showed abnormal response in Otoacoustic Emissions and normal Auditory Brainstem Response (ABR), with electrophysiological threshold in 30dBnHL, in both ears. With six months of age, he showed bilateral absence of the ABR at 100dBnHL. The behavioral observational audiometry was impaired due to the delay in neuropsychomotor development. At eight months of age, he was submitted to Auditory Steady State Response (ASSR) and the thresholds were 50, 70, absent in 110 and in 100dB, respectively for 500, 1,000, 2,000 and 4,000Hz in the right ear, and 70, 90, 90 and absent in 100dB, respectively for 500, 1,000, 2,000 and 4,000Hz in the left ear. COMMENTS: In the first evaluation, the infant had abnormal Otoacoustic Emission and normal ABR, which became altered at six months of age. The hearing loss severity could be identified only by the ASSR, which allowed the best procedure for hearing aids adaptation. The case description highlights the importance of the hearing status follow-up for children with congenital cytomegalovirus. PMID:24473963

  5. Developmental hearing loss impairs signal detection in noise: putative central mechanisms

    PubMed Central

    Gay, Jennifer D.; Voytenko, Sergiy V.; Galazyuk, Alexander V.; Rosen, Merri J.

    2014-01-01

    Listeners with hearing loss have difficulty processing sounds in noisy environments. This is most noticeable for speech perception, but is reflected in a basic auditory processing task: detecting a tonal signal in a noise background, i.e., simultaneous masking. It is unresolved whether the mechanisms underlying simultaneous masking arise from the auditory periphery or from the central auditory system. Poor detection in listeners with sensorineural hearing loss (SNHL) is attributed to cochlear hair cell damage. However, hearing loss alters neural processing in the central auditory system. Additionally, both psychophysical and neurophysiological data from normally hearing and impaired listeners suggest that there are additional contributions to simultaneous masking that arise centrally. With SNHL, it is difficult to separate peripheral from central contributions to signal detection deficits. We have thus excluded peripheral contributions by using an animal model of early conductive hearing loss (CHL) that provides auditory deprivation but does not induce cochlear damage. When tested as adults, animals raised with CHL had increased thresholds for detecting tones in simultaneous noise. Furthermore, intracellular in vivo recordings in control animals revealed a cortical correlate of simultaneous masking: local cortical processing reduced tone-evoked responses in the presence of noise. This raises the possibility that altered cortical responses which occur with early CHL can influence even simple signal detection in noise. PMID:25249949

  6. Normal hearing is required for the emergence of long-lasting inhibitory potentiation in cortex

    PubMed Central

    Xu, Han; Kotak, Vibhakar C.; Sanes, Dan H.

    2010-01-01

    Long-term synaptic plasticity is a putative mechanism for learning in adults. However, there is little understanding of how synaptic plasticity mechanisms develop or whether their maturation depends on experience. Since inhibitory synapses are particularly malleable to sensory stimulation, long-lasting potentiation of inhibitory synapses was characterized in auditory thalamocortical slices. Intracortical high frequency electrical stimulation led to a 67% increase in inhibitory synaptic currents. In the absence of stimulation, inhibitory potentiation was induced by a brief exposure to exogenous BDNF. BDNF exposure occluded any further potentiation by high frequency afferent stimulation, suggesting that BDNF signaling is sufficient to account for inhibitory potentiation. Moreover, inhibitory potentiation was reduced significantly by extracellular application of a BDNF scavenger or by intracellular blockade of BDNF receptor (TrkB) signaling. In contrast, glutamatergic or GABAergic antagonists did not prevent the induction of inhibitory potentiation. Since BDNF and TrkB expression are influenced strongly by activity, we predicted that inhibitory potentiation would be diminished by manipulations that decrease central auditory activity, such as hearing loss. Two forms of hearing loss were examined: conductive hearing loss in which the cochleae are not damaged or sensorineural hearing loss in which both cochleae are removed. Both forms of hearing loss were found to reduce significantly the magnitude of inhibitory potentiation. These data indicate that early experience is necessary for the normal development of BDNF-mediated long-lasting inhibitory potentiation, which may be associated with perceptual deficits at later ages. PMID:20053914

  7. Hearing impairment and language delay in infants: Diagnostics and genetics.

    PubMed

    Lang-Roth, Ruth

    2014-01-01

    This overview study provides information on important phoniatric and audiological aspects of early childhood hearing and language development with the aim of presenting diagnostic and therapeutic approaches. The article first addresses the universal newborn hearing screening that has been implemented in Germany for all infants since January 2009. The process of newborn hearing screening from the maternity ward to confirmation diagnostics is presented in accordance with a decision by the Federal Joint Committee (G-BA). The second topic is pediatric audiology diagnostics. Following confirmation of a permanent early childhood hearing disorder, the search for the cause plays an important role. Hereditary hearing disorders and intrauterine cytomegalovirus (CMV) infection, probably the most common cause of an acquired hearing disorder, are discussed and compared with the most common temporary hearing disorder, otitis media with effusion, which in some cases is severe enough to be relevant for hearing and language development and therefore requires treatment. The third topic covered in this article is speech and language development in the first 3 years of life, which is known today to be crucial for later language development and learning to read and write. There is a short overview and introduction to modern terminology, followed by the abnormalities and diagnostics of early speech and language development. Only some aspects of early hearing and language development are addressed here. Important areas such as the indication for a cochlear implant in the first year of life or because of unilateral deafness are not included due to their complexity. PMID:25587365

  8. Hearing impairment and language delay in infants: Diagnostics and genetics

    PubMed Central

    Lang-Roth, Ruth

    2014-01-01

    This overview study provides information on important phoniatric and audiological aspects of early childhood hearing and language development with the aim of presenting diagnostic and therapeutic approaches. The article first addresses the universal newborn hearing screening that has been implemented in Germany for all infants since January 2009. The process of newborn hearing screening from the maternity ward to confirmation diagnostics is presented in accordance with a decision by the Federal Joint Committee (G-BA). The second topic is pediatric audiology diagnostics. Following confirmation of a permanent early childhood hearing disorder, the search for the cause plays an important role. Hereditary hearing disorders and intrauterine cytomegalovirus (CMV) infection, probably the most common cause of an acquired hearing disorder, are discussed and compared with the most common temporary hearing disorder, otitis media with effusion, which in some cases is severe enough to be relevant for hearing and language development and therefore requires treatment. The third topic covered in this article is speech and language development in the first 3 years of life, which is known today to be crucial for later language development and learning to read and write. There is a short overview and introduction to modern terminology, followed by the abnormalities and diagnostics of early speech and language development. Only some aspects of early hearing and language development are addressed here. Important areas such as the indication for a cochlear implant in the first year of life or because of unilateral deafness are not included due to their complexity. PMID:25587365

  9. Consumer preferences for hearing aid attributes: a comparison of rating and conjoint analysis methods.

    PubMed

    Bridges, John F P; Lataille, Angela T; Buttorff, Christine; White, Sharon; Niparko, John K

    2012-03-01

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

  10. Hearing loss in space

    NASA Technical Reports Server (NTRS)

    Buckey, J. C. Jr; Musiek, F. E.; Kline-Schoder, R.; Clark, J. C.; Hart, S.; Havelka, J.

    2001-01-01

    BACKGROUND: Temporary and, in some cases, permanent hearing loss has been documented after long-duration spaceflights. METHODS: We examined all existing published data on hearing loss after space missions to characterize the losses. RESULTS: Data from Russian missions suggest that the hearing loss, when it occurs, affects mainly mid to high frequencies and that using hearing protection often might prevent the loss. Several significant questions remain about hearing loss in space. While the hearing loss has been presumed to be noise-induced, no clear link has been established between noise exposure and hearing loss during spaceflight. In one documented case of temporary hearing loss from the Shuttle-Mir program, the pattern of loss was atypical for a noise-induced loss. Continuous noise levels that have been measured on the Mir and previous space stations, while above engineering standards, are not at levels usually associated with hearing loss in ground-based studies (which have usually been limited to 8-10 h exposure periods). Attempts to measure hearing in space using threshold-based audiograms have been unsuccessful in both the American and Russian programs due to noise interference with the measurements. CONCLUSIONS: The existing data highlight the need for reliable monitoring of both hearing and noise in long-duration spaceflight.

  11. Typical Acquisition by Atypical Children: Initial Consonant Cluster Acquisition by Israeli Hebrew-Acquiring Children with Cochlear Implants

    ERIC Educational Resources Information Center

    Adi-Bensaid, Limor; Ben-David, Avivit

    2010-01-01

    This paper studies the developmental stages of word initial consonant clusters (CCs) in the speech of six monolingual Israeli Hebrew (IH) acquiring hearing impaired children using cochlear implant (CI). Focusing on the patterns of cluster reduction, this study compares the CI children with typically-developing hearing children. All the CI…

  12. Acquired von Willebrand disease.

    PubMed

    Kumar, Shaji; Pruthi, Rajiv K; Nichols, William L

    2002-02-01

    Acquired von Willebrand disease (AvWD) is a relatively rare acquired bleeding disorder that usually occurs in elderly patients, in whom its recognition may be delayed. Patients usually present predominantly with mucocutaneous bleeding, with no previous history of bleeding abnormalities and no clinically meaningful family history. Various underlying diseases have been associated with AvWD, most commonly hematoproliferative disorders, including monoclonal gammopathies, lymphoproliferative disorders, and myeloproliferative disorders. The pathogenesis of AvWD remains incompletely understood but includes autoantibodies directed against the von Willebrand factor (vWF), leading to a more rapid clearance from the circulation or interference with its function, adsorption of vWF by tumor cells, and nonimmunologic mechanisms of destruction. Laboratory evaluation usually reveals a pattern of prolonged bleeding time and decreased levels of vWF antigen, ristocetin cofactor activity, and factor VIII coagulant activity consistent with a diagnosis of vWD. Acquired vWD is distinguished from the congenital form by age at presentation, absence of a personal and family history of bleeding disorders, and, often, presence of a hematoproliferative or autoimmune disorder. The severity of the bleeding varies considerably among patients. Therapeutic options include desmopressin and certain factor VIII concentrates that also contain vWF. Successful treatment of the associated illness can reverse the clinical and laboratory manifestations. Intravenous immunoglobulins have also shown some efficacy in the management of AvWD, especially cases associated with monoclonal gammopathies. Awareness of AvWD is essential for diagnosis and appropriate management. PMID:11838652

  13. Hearing Loss in Children: Types of Hearing Loss

    MedlinePlus

    ... the ear to the brain so that our brain pathways are part of our hearing. There are four types of hearing loss: Conductive Hearing Loss Hearing loss caused by something that stops sounds from getting through the outer or middle ear. This type of hearing loss can often ...

  14. 12 CFR 1209.12 - Public hearings; closed hearings.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... 12 Banks and Banking 10 2014-01-01 2014-01-01 false Public hearings; closed hearings. 1209.12... PRACTICE AND PROCEDURE Rules of Practice and Procedure § 1209.12 Public hearings; closed hearings. (a... hearings shall be open to the public, except that the Director, in his discretion, may determine...

  15. 12 CFR 1209.12 - Public hearings; closed hearings.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... 12 Banks and Banking 9 2012-01-01 2012-01-01 false Public hearings; closed hearings. 1209.12... PRACTICE AND PROCEDURE Rules of Practice and Procedure § 1209.12 Public hearings; closed hearings. (a... hearings shall be open to the public, except that the Director, in his discretion, may determine...

  16. Genetic variants for long QT syndrome among infants and children from a statewide newborn hearing screening program cohort

    PubMed Central

    Chang, Ruey-Kang R.; Lan, Yueh-Tze; Silka, Michael J.; Morrow, Hallie; Kwong, Alan; Smith-Lang, Janna; Wallerstein, Robert; Lin, Henry J.

    2014-01-01

    Objectives Autosomal recessive long QT syndrome (LQTS), or Jervell and Lange-Nielsen syndrome (JLNS), can be associated with sensorineural hearing loss (SNHL). We aimed to explore newborn hearing screening combined with ECGs for early JLNS detection. Study design We conducted California statewide, prospective ECG screening of children ≤6 years of age with unilateral or bilateral, severe or profound, sensorineural or mixed hearing loss. Families were identified through newborn hearing screening and interviewed about medical and family histories. Twelve-lead ECGs were obtained. Those with positive histories or QTc intervals ≥450 ms had repeat ECGs. DNA sequencing of 12 LQTS genes was performed for repeat QTc intervals ≥450 ms. Results We screened 707 subjects by ECGs (number screened/number of responses = 91%; number of responses/number of families who were mailed invitations = 54%). Of these, 73 had repeat ECGs, and 19 underwent gene testing. No subject had homozygous or compound heterozygous LQTS mutations, as in JLNS. However, 3 individuals (with QTc intervals of 472, 457, and 456 ms, respectively) were heterozygous for variants that cause truncation or missplicing: 2 in KCNQ1 (c.1343dupC or p.Glu449Argfs*14; c.1590+1G>A or p.Glu530sp) and 1 in SCN5A (c.5872C>T or p.Arg1958*). Conclusions In contrast to reports of JLNS in up to 4% of children with SNHL, we found no examples of JLNS. Because the 3 variants identified were unrelated to hearing, they likely represent the prevalence of potential LQTS mutations in the general population. Further studies are needed to define consequences of such mutations and assess the overall prevalence. PMID:24388587

  17. Music and hearing aids.

    PubMed

    Madsen, Sara M K; Moore, Brian C J

    2014-01-01

    The signal processing and fitting methods used for hearing aids have mainly been designed to optimize the intelligibility of speech. Little attention has been paid to the effectiveness of hearing aids for listening to music. Perhaps as a consequence, many hearing-aid users complain that they are not satisfied with their hearing aids when listening to music. This issue inspired the Internet-based survey presented here. The survey was designed to identify the nature and prevalence of problems associated with listening to live and reproduced music with hearing aids. Responses from 523 hearing-aid users to 21 multiple-choice questions are presented and analyzed, and the relationships between responses to questions regarding music and questions concerned with information about the respondents, their hearing aids, and their hearing loss are described. Large proportions of the respondents reported that they found their hearing aids to be helpful for listening to both live and reproduced music, although less so for the former. The survey also identified problems such as distortion, acoustic feedback, insufficient or excessive gain, unbalanced frequency response, and reduced tone quality. The results indicate that the enjoyment of listening to music with hearing aids could be improved by an increase of the input and output dynamic range, extension of the low-frequency response, and improvement of feedback cancellation and automatic gain control systems. PMID:25361601

  18. Music and Hearing Aids

    PubMed Central

    Moore, Brian C. J.

    2014-01-01

    The signal processing and fitting methods used for hearing aids have mainly been designed to optimize the intelligibility of speech. Little attention has been paid to the effectiveness of hearing aids for listening to music. Perhaps as a consequence, many hearing-aid users complain that they are not satisfied with their hearing aids when listening to music. This issue inspired the Internet-based survey presented here. The survey was designed to identify the nature and prevalence of problems associated with listening to live and reproduced music with hearing aids. Responses from 523 hearing-aid users to 21 multiple-choice questions are presented and analyzed, and the relationships between responses to questions regarding music and questions concerned with information about the respondents, their hearing aids, and their hearing loss are described. Large proportions of the respondents reported that they found their hearing aids to be helpful for listening to both live and reproduced music, although less so for the former. The survey also identified problems such as distortion, acoustic feedback, insufficient or excessive gain, unbalanced frequency response, and reduced tone quality. The results indicate that the enjoyment of listening to music with hearing aids could be improved by an increase of the input and output dynamic range, extension of the low-frequency response, and improvement of feedback cancellation and automatic gain control systems. PMID:25361601

  19. Hearing loss and contributing factors among airport workers in Malaysia.

    PubMed

    Nasir, H M; Rampal, K G

    2012-02-01

    Sensorineural hearing loss is a common and important source of disability among the workers and often caused by occupational noise exposure. Aims of the study were to determine the prevalence and contributing factors of hearing loss among airport workers. A cross-sectional study was carried out at an airport in Malaysia. This study used stratified sampling method that involved 358 workers who were working in 3 different units between November 2008 and March 2009. Data for this study were collected by using questionnaires eliciting sociodemographic, occupational exposure history (previous and present), life-style including smoking habits and health-related data. Otoscopic and pure-tone audiometric tests were conducted for hearing assessment. Noise exposure status was categorize by using a noise logging dosimeter to obtain 8-hour Time-Weighted Average (TWA). Data was analyzed by using SPSS version 12.0.1 and EpiInfo 6.04. The prevalence of hearing loss was 33.5%. Age >40 years old (aOR 4.3, 95%CI 2.2-8.3) is the main risk factors for hearing loss followed by duration of noise exposure >5 years (aOR 2.5, 95%CI 1.4-4.7), smoking (aOR 2.1, 95%CI 1.2-3.4), duration of service >5 years (aOR 2.1, 95%CI 1.1-3.9), exposure to explosion (aOR 6.1, 95%CI 1.3-29.8), exposure to vibration (aOR 2.2, 95%CI 1.1-4.3) and working in engineering unit (aOR 5.9, 95%CI 1.1-30.9). The prevalence rate ratio of hearing loss for nonsmokers aged 40 years old and younger, smokers aged 40 years old and younger, non-smokers older than 40 years old and smokers older than 40 years old was 1.0, 1.7, 2.8 and 4.6 respectively. This result contributes towards better understanding of risk factors for hearing loss, which is relatively common among Malaysian workers. PMID:22582554

  20. Identification of a novel pathogenic OTOF variant causative of nonsyndromic hearing loss with high frequency in the Ashkenazi Jewish population.

    PubMed

    Fedick, Anastasia M; Jalas, Chaim; Swaroop, Ananya; Smouha, Eric E; Webb, Bryn D

    2016-01-01

    Mutations in the OTOF gene have previously been shown to cause nonsyndromic prelingual deafness (DFNB9, OMIM 601071) as well as auditory neuropathy/dys-synchrony. In this study, the OTOF NM_194248.2 c.5332G>T, p.Val1778Phe variant was identified in a large Ashkenazi Jewish family as the causative variant in four siblings with hearing loss. Our analysis reveals a carrier frequency of the OTOF c.5332G>T, p.Val1778Phe variant of 1.27% in the Ashkenazi Jewish population, suggesting that this variant may be a significant contributor to nonsyndromic sensorineural hearing loss and should be considered for inclusion in targeted hearing loss panels for this population. Of note, the degree of hearing loss associated with this phenotype ranged from mild to moderately severe, with two of the four siblings not known to have hearing loss until they were genotyped and underwent pure tone audiometry and auditory brainstem response testing. The phenotypic variability along with the auditory neuropathy/dys-synchrony, which allows for the production of otoacoustic emissions, supports that nonsyndromic hearing loss caused by OTOF mutations may be much more common in the Ashkenazi Jewish population than currently appreciated due to a lack of diagnosis. PMID:27621663

  1. Identification of a novel pathogenic OTOF variant causative of nonsyndromic hearing loss with high frequency in the Ashkenazi Jewish population

    PubMed Central

    Fedick, Anastasia M; Jalas, Chaim; Swaroop, Ananya; Smouha, Eric E; Webb, Bryn D

    2016-01-01

    Mutations in the OTOF gene have previously been shown to cause nonsyndromic prelingual deafness (DFNB9, OMIM 601071) as well as auditory neuropathy/dys-synchrony. In this study, the OTOF NM_194248.2 c.5332G>T, p.Val1778Phe variant was identified in a large Ashkenazi Jewish family as the causative variant in four siblings with hearing loss. Our analysis reveals a carrier frequency of the OTOF c.5332G>T, p.Val1778Phe variant of 1.27% in the Ashkenazi Jewish population, suggesting that this variant may be a significant contributor to nonsyndromic sensorineural hearing loss and should be considered for inclusion in targeted hearing loss panels for this population. Of note, the degree of hearing loss associated with this phenotype ranged from mild to moderately severe, with two of the four siblings not known to have hearing loss until they were genotyped and underwent pure tone audiometry and auditory brainstem response testing. The phenotypic variability along with the auditory neuropathy/dys-synchrony, which allows for the production of otoacoustic emissions, supports that nonsyndromic hearing loss caused by OTOF mutations may be much more common in the Ashkenazi Jewish population than currently appreciated due to a lack of diagnosis. PMID:27621663

  2. Effects of noise reduction on speech intelligibility, perceived listening effort, and personal preference in hearing-impaired listeners.

    PubMed

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

    2014-01-01

    This study evaluates the perceptual effects of single-microphone noise reduction in hearing aids. Twenty subjects with moderate sensorineural hearing loss listened to speech in babble noise processed via noise reduction from three different linearly fitted hearing aids. Subjects performed (a) speech-intelligibility tests, (b) listening-effort ratings, and (c) paired-comparison ratings on noise annoyance, speech naturalness, and overall preference. The perceptual effects of noise reduction differ between hearing aids. The results agree well with those of normal-hearing listeners in a previous study. None of the noise-reduction algorithms improved speech intelligibility, but all reduced the annoyance of noise. The noise reduction that scored best with respect to noise annoyance and preference had the worst intelligibility scores. The trade-off between intelligibility and listening comfort shows that preference measurements might be useful in addition to intelligibility measurements in the selection of noise reduction. Additionally, this trade-off should be taken into consideration to create realistic expectations in hearing-aid users. PMID:25315377

  3. Do You Hear What Horton Hears?

    ERIC Educational Resources Information Center

    Snyder, Robert; Johnson, Jordan

    2010-01-01

    "I've never heard of a small speck of dust that is able to yell" says Horton of a sound he hears well (Geisel 1954). It is always valuable to connect science to student's interests and their everyday world--so what better way to teach concepts relating to sound than to read "Horton Hears a Who" by Dr. Seuss? Here the authors present several…

  4. [Acquired von Willebrand syndrome].

    PubMed

    Franchini, Massimo

    2006-01-01

    Acquired von Willebrand syndrome (aVWS) is a rare, but probably underestimated, bleeding disorder that mimics the congenital form of von Willebrand disease (VWD) in terms of laboratory findings and clinical presentation. However, unlike congenital VWD, it arises in individuals with no personal or family history of bleeding. AVWS occurs in association with a variety of underlying disorders, including lymphoproliferative disorders, myeloproliferative disorders and cardiovascular diseases. The main pathogenic, clinical, laboratory and therapeutic aspects of this syndrome are concisely reported in this review. PMID:16913181

  5. Use of Hearing Aids by Adults with Hearing Loss

    MedlinePlus

    ... Epidemiology Use of Hearing Aids by Adults with Hearing Loss [text version] Note: Higher numbers are better. *This ... 2010 and 2020. The number of persons with hearing loss is calculated based on National Health and Nutrition ...

  6. The effect of stimulus bandwidth on auditory skills in normal-hearing and hearing-impaired children

    PubMed Central

    Stelmachowicz, Patricia G.; Lewis, Dawna E.; Choi, Sangsook; Hoover, Brenda

    2008-01-01

    Objective Previous studies from our laboratory have shown that a restricted stimulus bandwidth can have a negative effect upon the perception of the phonemes / s/ and / z/, which serve multiple linguistic functions in the English language. These findings may have important implications for the development of speech and language in young children with hearing loss because the bandwidth of current hearing aids generally is restricted to 6–7 kHz. The primary goal of the current study was to expand our previous work to examine the effects of stimulus bandwidth on a wide range of speech materials, to include a variety of auditory-related tasks, and to include the effects of background noise. Design Thirty-two children with normal hearing and 24 children with sensorineural hearing loss (7–14 years) participated in this study. To assess the effects of stimulus bandwidth, four different auditory tasks were used: 1) nonsense syllable perception, 2) word recognition, 3) novel-word learning, and 4) listening effort. Auditory stimuli recorded by a female talker were low-pass filtered at 5 and 10 kHz and presented in noise. Results For the children with normal-hearing, significant bandwidth effects were observed for the perception of nonsense syllables and for words, but not for novel-word learning or listening effort. In the 10-kHz bandwidth condition, children with hearing loss showed significant improvements for monosyllabic words, but not for nonsense syllables, novel-word learning, or listening effort. Further examination, however, revealed marked improvements for the perception of specific phonemes. For example, bandwidth effects for the perception of / s/ and / z/ were not only significant, but much greater than that seen in the group with normal hearing. Conclusions The current results are consistent with previous studies which have shown that a restricted stimulus bandwidth can negatively affect the perception of / s/ and / z/ spoken by female talkers. Given the

  7. Analysis of the Prevalence of and Factors Associated with Hearing Loss in Korean Adolescents

    PubMed Central

    Hong, Seok Min; Park, Il-Seok; Kim, Yong Bok; Hong, Seok Jin; Lee, Byungho

    2016-01-01

    Background Hearing loss can lead to a number of disabilities, subsequently reducing the quality of life. In general, hearing thresholds of adolescents are better than adults and the elderly. However, occasionally, adolescents acquire hearing loss for a number of reasons. In this study, our goal was to estimate the prevalence of hearing loss in the Korean population and to investigate the factors related to hearing thresholds in adolescents. Methods A cross-sectional study was conducted using data from the Korean National Health and Nutrition Examination Survey (KNHANES) between 2010 and 2012. We enrolled a total of 1,658 participants, ages 13 to 18 years. We investigated the prevalence of hearing loss and the factors associated with hearing thresholds at various frequencies (0.5, 1, 2, 3, 4, and 6 KHz). Results Weighted prevalence of unilateral and bilateral hearing loss in Korean adolescents was 2.2% and 0.4%, respectively. Weighted prevalence of hearing thresholds ≥ 20 dB at speech and high frequencies were 3.1% and 5.0%, respectively, for unilateral hearing loss and 0.7% and 1.9%, respectively, for bilateral. Age group, tympanometric data, and household income were significantly related to unilateral or bilateral hearing thresholds ≥ 20 dB at speech frequencies. Earphone use in noisy places was associated with bilateral hearing thresholds ≥ 20 dB at high frequencies. Conclusions The prevalence of hearing loss in Korean adolescents was 2.6% using the general standard threshold associated with hearing loss. However, the prevalence of hearing thresholds ≥ 20 dB for speech and high frequencies was much higher. The results from this study provide an estimate of hearing loss in adolescents and suggest the need for early detection and hearing preservation programs among adolescents. PMID:27513659

  8. Binaural speech discrimination under noise in hearing-impaired listeners

    NASA Technical Reports Server (NTRS)

    Kumar, K. V.; Rao, A. B.

    1988-01-01

    This paper presents the results of an assessment of speech discrimination by hearing-impaired listeners (sensori-neural, conductive, and mixed groups) under binaural free-field listening in the presence of background noise. Subjects with pure-tone thresholds greater than 20 dB in 0.5, 1.0 and 2.0 kHz were presented with a version of the W-22 list of phonetically balanced words under three conditions: (1) 'quiet', with the chamber noise below 28 dB and speech at 60 dB; (2) at a constant S/N ratio of +10 dB, and with a background white noise at 70 dB; and (3) same as condition (2), but with the background noise at 80 dB. The mean speech discrimination scores decreased significantly with noise in all groups. However, the decrease in binaural speech discrimination scores with an increase in hearing impairment was less for material presented under the noise conditions than for the material presented in quiet.

  9. Hearing disorders in Turner's syndrome: a survey from Iran.

    PubMed

    Bakhshaee, Mehdi; Vakili, Rahim; Nourizadeh, Navid; Rajati, Mohsen; Ahrari, Asma; Movahed, Rahman

    2015-12-01

    Turner syndrome (TS) is one of the most frequently encountered sex-linked chromosomal abnormalities, occurring in one per 2,000 female births. These patients present with short stature and failure to begin puberty. In this syndrome, there are multiple organ abnormalities, including auditory disorders. TS patients were referred to the ENT clinic by a pediatric endocrinologist. A questionnaire was filled out and the patients went through a complete otologic examination. They were then referred to the audiology clinic to undergo audiologic test battery plus high-frequency pure tone audiometry. From a total of 48 ears examined, 11 (22.9 %) had a normal audiometry. Mid-frequency sensorineural hearing loss (SNHL), high-frequency SNHL, combined and mixed hearing loss were diagnosed in 6 (12/5 %), 20 (41/7 %), 6 (12/5 %) and 1 (2/1 %) ear, respectively. Tympanogram results showed normal compliance (A, As, Ad) in the majority of cases. B and C patterns were found in a few cases. Speech discrimination score was normal in all patients whereas speech reception threshold was normal in 92 % of the ears. Audiometry abnormality especially SNHL is common in TS patients, with the high-frequency pattern being the most frequent. PMID:25534285

  10. Attenuation of noise-induced hearing loss using methylene blue

    PubMed Central

    Park, J-S; Jou, I; Park, S M

    2014-01-01

    The overproduction of reactive oxygen species (ROS) and reactive nitrogen species (RNS) has been known to contribute to the pathogenesis of noise-induced hearing loss. In this study, we discovered that in BALB/c mice pretreatment with methylene blue (MB) for 4 consecutive days significantly protected against cochlear injury by intense broad-band noise for 3 h. It decreased both compound threshold shift and permanent threshold shift and, further, reduced outer hair cell death in the cochlea. MB also reduced ROS and RNS formation after noise exposure. Furthermore, it protected against rotenone- and antimycin A-induced cell death and also reversed ATP generation in the in vitro UB-OC1 cell system. Likewise, MB effectively attenuated the noise-induced impairment of complex IV activity in the cochlea. In addition, it increased the neurotrophin-3 (NT-3) level, which could affect the synaptic connections between hair cells and spiral ganglion neurons in the noise-exposed cochlea, and also promoted the conservation of both efferent and afferent nerve terminals on the outer and inner hair cells. These findings suggest that the amelioration of impaired mitochondrial electron transport and the potentiation of NT-3 expression by treatment with MB have a significant therapeutic value in preventing ROS-mediated sensorineural hearing loss. PMID:24763057

  11. [Inner Ear Hearing Loss].

    PubMed

    Hesse, G

    2016-06-01

    Hearing loss is one of the most dominant handicaps in modern societies, which additionally very often is not realized or not admitted. About one quarter of the general population suffers from inner ear hearing loss and is therefore restricted in communicational skills. Demographic factors like increasing age play an important role as well as environmental influences and an increasing sound and noise exposure especially in leisure activities. Thus borders between a "classical" presbyacusis - if it ever existed - and envirionmentally induced hearing loss disappear. Today restrictions in hearing ability develop earlier in age but at the same time they are detected and diagnosed earlier. This paper can eventually enlighten the wide field of inner ear hearing loss only fragmentarily; therefore mainly new research, findings and developments are reviewed. The first part discusses new aspects of diagnostics of inner ear hearing loss and different etiologies. PMID:27259171

  12. Using Repeated Reading and Explicit Instruction to Teach Vocabulary to Preschoolers with Hearing Loss

    ERIC Educational Resources Information Center

    Bobzien, Jonna L.; Richels, Corrin; Schwartz, Kathryn; Raver, Sharon A.; Hester, Peggy; Morin, Lisa

    2015-01-01

    Children with hearing loss often experience communication and language delays that result in difficulties acquiring novel vocabulary and literacy skills. This research examined the effectiveness of using repeated storybook reading paired with explicit teacher instruction to teach novel vocabulary to young children with hearing loss who were…

  13. Differences in Arithmetic Subtraction of Nonsymbolic Numerosities by Deaf and Hearing Adults

    ERIC Educational Resources Information Center

    Masataka, Nobuo

    2006-01-01

    Previous research has demonstrated that both hearing adults and hearing children with no training in arithmetic successfully performed approximate arithmetic on large sets of elements. Here, the possibility is explored that the same phenomenon can be confirmed in deaf adults who have acquired a signed language as their first language. Results…

  14. Comparing Deaf and Hearing Dutch Infants: Changes in the Vowel Space in the First 2 Years

    ERIC Educational Resources Information Center

    van der Stelt, Jeannette M.; Wempe, Ton G.; Pols, Louis C. W.

    2008-01-01

    The influence of the mother tongue on vowel productions in infancy is different for deaf and hearing babies. Audio material of five hearing and five deaf infants acquiring Dutch was collected monthly from month 5-18, and at 24 months. Fifty unlabelled utterances were digitized for each recording. This study focused on developmental paths in vowel…

  15. Postural control in children with typical development and children with profound hearing loss

    PubMed Central

    de Sousa, Aneliza Maria Monteiro; de França Barros, Jônatas; de Sousa Neto, Brígido Martins

    2012-01-01

    Purpose To describe the behavior of the postural control in children with profound sensorineural hearing loss and compare the results of experimental tests with hearing children aged 7 to 10 years. Patients and methods This is a cross-sectional study where 100 children were divided into experimental and control groups. We used a force platform, AccuSway Plus, where the tests were conducted under the experimental conditions: open base, eyes open (OBEO); open base, eyes closed (OBEC); closed base, eyes open (CBEO); closed base, eyes closed (CBEC). The body sway velocity (V) of the center of pressure, the displacement in the anteroposterior direction (COPap) and mediolateral (COPml) of the center of pressure were the parameters to evaluate the postural control. For statistical analysis we used the nonparametric Mann–Whitney U test, with a significance level of 5%. Results In comparisons of variables between the groups, the experimental group outperformed by at least 75% of the control group values. In terms of global trends, the experimental group shows higher values of body oscillations in all experimental conditions and variables evaluated. Children with hearing loss had poorer balance performance compared to the group of hearing. The inferential analysis revealed a statistically significant difference in the balance between deaf and hearing children in the OBEC experimental condition in relation to the COPml parameter (P = 0.04). There were no statistically significant differences in comparisons between the sexes when the groups were analyzed separately. The prevalence of unknown etiology showed 58% of cases and congenital rubella in 16%. The discovery of deafness occurred in 70% of children before the age of 3 years. Conclusion In this study, children with hearing loss had poorer balance performance compared to the group of hearing children. This finding confirms the need to investigate postural control through longitudinal studies to identify the area of

  16. Development and Preliminary Verification of a Mandarin-Based Hearing-Aid Fitting Strategy

    PubMed Central

    Lai, Ying-Hui; Liu, Tien-Chen; Li, Pei-Chun; Shih, Wan-Ting; Young, Shuenn-Tsong

    2013-01-01

    Objective The purpose of this study was to design and to verify a new hearing-aid fitting strategy (Aescu HRL-1) based on the acoustic features of Mandarin. The subjective and objective outcomes were compared to those fitted with NAL-NL1 (National Acoustic Laboratory Non-Linear, version1) in Mandarin-speaking hearing-aid users. Design Fifteen subjects with sensorineural hearing loss participated in this preliminary study. Each subject wore a pair of four-channel hearing aids fitted with the Aescu HRL-1 and NAL-NL1 prescriptions alternatively for 1 month. Objective and subjective tests including the Mandarin Monosyllable Recognition Test (MMRT), Mandarin Hearing in Noise Test (MHINT), International Outcome Inventory for Hearing Aids (IOI-HA), and a sound-quality questionnaire were used to evaluate the performance of the two prescriptions. Results The mean MMRT scores were 79.9% and 81.1% for NAL-NL1 and Aescu HRL-1 respectively. They are not statistically different. The corresponding MHINT signal-to-noise ratios were 0.87 and 0.85 dB, also, no significant difference was found between these two strategies. However, in subjective questionnaires, overall, the sound-quality and IOI-HA scores were higher for Aescu HRL-1. Conclusions The speech recognition performance based on Aescu HRL-1 is as good as that of NAL-NL1 for Mandarin-speaking hearing-aid users. Moreover, the subjects generally responded that Aescu HRL-1 provides a more natural, richer, and better sound quality than does NAL-NL1. PMID:24278326

  17. Generation and validation of a zebrafish model of EAST (epilepsy, ataxia, sensorineural deafness and tubulopathy) syndrome

    PubMed Central

    Mahmood, Fahad; Mozere, Monika; Zdebik, Anselm A.; Stanescu, Horia C.; Tobin, Jonathan; Beales, Philip L.; Kleta, Robert; Bockenhauer, Detlef; Russell, Claire

    2013-01-01

    SUMMARY Recessive mutations in KCNJ10, which encodes an inwardly rectifying potassium channel, were recently identified as the cause of EAST syndrome, a severe and disabling multi-organ disorder consisting of epilepsy, ataxia, sensorineural deafness and tubulopathy that becomes clinically apparent with seizures in infancy. A Kcnj10 knockout mouse shows postnatal mortality and is therefore not suitable for drug discovery. Because zebrafish are ideal for in vivo screening for potential therapeutics, we tested whether kcnj10 knockdown in zebrafish would fill this need. We cloned zebrafish kcnj10 and demonstrated that its function is equivalent to that of human KCNJ10. We next injected splice- and translation-blocking kcnj10 antisense morpholino oligonucleotides and reproduced the cardinal symptoms of EAST syndrome – ataxia, epilepsy and renal tubular defects. Several of these phenotypes could be assayed in an automated manner. We could rescue the morphant phenotype with complementary RNA (cRNA) encoding human wild-type KCNJ10, but not with cRNA encoding a KCNJ10 mutation identified in individuals with EAST syndrome. Our results suggest that zebrafish will be a valuable tool to screen for compounds that are potentially therapeutic for EAST syndrome or its individual symptoms. Knockdown of kcnj10 represents the first zebrafish model of a salt-losing tubulopathy, which has relevance for blood pressure control. PMID:23471908

  18. Hearing or speech impairment - resources

    MedlinePlus

    ... resources for information on hearing impairment or speech impairment: Alexander Graham Bell Association for the Deaf and Hard of Hearing -- www.agbell.org American Speech-Language-Hearing Association -- www.asha.org/public Center for ...

  19. Screening Newborns' Hearing Now Standard

    MedlinePlus

    ... this page please turn Javascript on. Feature: Taste, Smell, Hearing, Language, Voice, Balance Screening Newborns' Hearing Now ... emailing NIDCDinfo@nidcd.nih.gov . Read More "Taste, Smell, Hearing, Language, Voice, Balance" Articles At Last: A ...

  20. Age-Related Hearing Loss

    MedlinePlus

    ... hearing loss. Here are the most common ones: Styles of hearing aids Source: NIH/NIDCD Hearing aids ... list of organizations, contact: NIDCD Information Clearinghouse 1 Communication Avenue Bethesda, MD 20892-3456 Toll-free Voice: ( ...

  1. Hearing or speech impairment - resources

    MedlinePlus

    Resources - hearing or speech impairment ... The following organizations are good resources for information on hearing impairment or speech impairment: Alexander Graham Bell Association for the Deaf and Hard of Hearing -- www.agbell. ...

  2. Hearing protection for miners

    SciTech Connect

    Schulz, T.

    2008-10-15

    A NIOSH analysis showed that at age 50 approximately 90% of coal miners have a hearing impairment, yet noise included hearing loss is 100% preventable. The article discusses requirements of the MSHA regulations, 30 CFR Part 62 - occupational noise exposure (2000) and a 2008-MSHA document describing technologically achievable and promising controls for several types of mining machinery. Hearing protection is still required for exposure to greater than 90 dBA. These are now commercially available ways to determine how much attenuation an individual gets from a given hearing protector, known as 'fit testing'. 3 refs., 1 fig., 1 tab., 1 photo.

  3. [Implantable hearing aids].

    PubMed

    Luers, J C; Beutner, D; Hüttenbrink, K-B

    2011-10-01

    Strictly speaking, implantable hearing aids are technical systems that process audiological signals and convey these by direct mechanical stimulation of the ossicular chain or cochlea. They have certain benefits over conventional hearing aids in terms of wearing comfort and general acceptance. As current studies lack convincing audiological results, the indications for implantable hearing aids are primarily of medical or cosmetic nature. To date, three systems are available in Germany: Vibrant Soundbridge®, Carina®, and Esteem®. Because the performance of the different implantable and nonimplantable hearing systems together with various surgical procedures are currently undergoing major changes, audiological indications may also develop in the future. PMID:21956678

  4. Hearing Function in Patients Living with HIV/AIDS

    PubMed Central

    Luque, Amneris E.; Orlando, Mark S.; Leong, U-Cheng; Allen, Paul D.; Guido, Joseph J.; Yang, Hongmei; Wu, Hulin

    2014-01-01

    Background During the earlier years of the HIV/AIDS epidemic, initial reports described sensorineural hearing loss in up to 49% of individuals with HIV/AIDS. During those years, patients commonly progressed to advanced stages of HIV disease, and frequently had neurological complications. However, the abnormalities on pure-tone audiometry and brainstem evoked responses outlined in small studies were not always consistently correlated with advanced stages of HIV/AIDS. Moreover, these studies could not exclude the confounding effect of concurrent opportunistic infections and syphilis. Additional reports also have indicated that some antiretroviral (ARV) medications may be ototoxic, thus it has been difficult to make conclusions regarding the cause of changes in hearing function in HIV-infected patients. More recently, accelerated aging has been suggested as a potential explanation for the disproportionate increase in complications of aging described in many HIV-infected patients, hence accelerated aging associated hearing loss may also be playing a role in these patients. Methods We conducted a large cross-sectional analysis of hearing function in over 300 patients with HIV-1 infection and in 137 HIV-uninfected controls. HIV-infected participants and HIV-uninfected controls underwent a two-hour battery of hearing tests including the Hearing Handicap Inventory, standard audiometric pure-tone air and bone conduction testing, tympanometric testing and speech reception and discrimination testing. Results Three-way ANOVA and logistic regression analysis of 278 eligible HIV-infected subjects stratified by disease stage in early HIV disease (n= 127) and late HIV disease (n=148) and 120 eligible HIV-uninfected controls revealed no statistical significant differences among the three study groups in either overall 4-PTA or hearing loss prevalence in either ear. Three-way ANOVA showed significant differences in word recognition scores (WRS) in the right ear among groups; a

  5. Progressive hearing loss associated with a unique cervical node due to a homozygous SLC29A3 mutation: a very mild phenotype.

    PubMed

    Jonard, Laurence; Couloigner, Vincent; Pierrot, Sébastien; Louha, Malek; Gherbi, Souad; Denoyelle, Françoise; Marlin, Sandrine

    2012-01-01

    In 2008, SLC29A3 has been implicated in a syndromic form of genodermatosis: H syndrome. The major features encountered in H syndrome are Hearing loss, Hyperglycaemia, Heart anomalies, Hypertrichosis, Hyperpigmentation, Hepatomegaly and Hypogonadism. More recently, SLC29A3 mutations have been described in families presenting syndromes associating generalized histiocytosis to systemic progressive features: severe camptodactyly, hearing loss, hypogonadism, hepatomegaly, heart defects and skin hyperpigmentation. We have identified a homozygous missense SLC29A3 mutation in a patient presenting with only a progressive sensorineural hearing impairment and a single cervical node (Rosai Dorfman). SLC29A3 mutations appear to be involved in a large phenotypic continuum which should prompt physicians to study this gene even in mild clinical presentations. PMID:21888995

  6. Absence of Neuroplastin-65 Affects Synaptogenesis in Mouse Inner Hair Cells and Causes Profound Hearing Loss

    PubMed Central

    Carrott, Leanne; Aguilar, Carlos; Johnson, Stuart L.; Chessum, Lauren; West, Melissa; Morse, Susan; Dorning, Joanne; Smart, Elizabeth; Hardisty-Hughes, Rachel; Ball, Greg; Parker, Andrew; Barnard, Alun R.; MacLaren, Robert E.; Wells, Sara; Marcotti, Walter

    2016-01-01

    The Neuroplastin gene encodes two synapse-enriched protein isoforms, Np55 and Np65, which are transmembrane glycoproteins that regulate several cellular processes, including the genesis, maintenance, and plasticity of synapses. We found that an absence of Np65 causes early-onset sensorineural hearing loss and prevented the normal synaptogenesis in inner hair cells (IHCs) in the newly identified mouse mutant pitch. In wild-type mice, Np65 is strongly upregulated in the cochlea from around postnatal day 12 (P12), which corresponds to the onset of hearing. Np65 was specifically localized at the presynaptic region of IHCs. We found that the colocalization of presynaptic IHC ribbons and postsynaptic afferent terminals is greatly reduced in pitch mutants. Moreover, IHC exocytosis is also reduced with mutant mice showing lower rates of vesicle release. Np65 appears to have a nonessential role in vision. We propose that Np65, by regulating IHC synaptogenesis, is critical for auditory function in mammals. SIGNIFICANCE STATEMENT In the mammalian cochlea, the sensory inner hair cells (IHCs) encode auditory information. They do this by converting sound wave-induced mechanical motion of their hair bundles into an electrical current. This current generates a receptor potential that controls release of glutamate neurotransmitter from their ribbon synapses onto the auditory afferent fiber. We show that the synapse-enriched protein Np65, encoded by the Neuroplastin gene, is localized at the IHC presynaptic region. In mutant mice, absence of Np65 causes early-onset sensorineural hearing loss and prevents normal neurotransmitter release in IHCs and colocalization of presynaptic ribbons with postsynaptic afferents. We identified Neuroplastin as a novel deafness gene required for ribbon synapse formation and function, which is critical for sound perception in mammals. PMID:26740663

  7. AIDS: acquired immunodeficiency syndrome *

    PubMed Central

    Gilmore, N.J.; Beaulieu, R.; Steben, M.; Laverdière, M.

    1992-01-01

    Acquired immunodeficiency syndrome, or AIDS, is a new illness that occurs in previously healthy individuals. It is characterized by immunodeficiency, opportunistic infections and unusual malignant diseases. Life-threatening single or multiple infections with viruses, mycobacteria, fungi or protozoa are common. A rare neoplasm, Kaposi's sarcoma, has developed in approximately one third of patients with AIDS. More than 800 cases of AIDS have been reported in North America, over 24 of them in Canada. The majority of patients are male homosexuals, although AIDS has also developed in abusers of intravenously administered drugs, Haitian immigrants, individuals with hemophilia, recipients of blood transfusions, prostitutes, and infants, spouses and partners of patients with AIDS. The cause of AIDS is unknown, but the features are consistent with an infectious process. Early diagnosis can be difficult owing to the nonspecific symptoms and signs of the infections and malignant diseases. Therefore, vigilance by physicians is of the utmost importance. PMID:1544049

  8. Community-acquired pneumonia.

    PubMed

    Polverino, E; Torres Marti, A

    2011-02-01

    Despite the remarkable advances in antibiotic therapies, diagnostic tools, prevention campaigns and intensive care, community-acquired pneumonia (CAP) is still among the primary causes of death worldwide, and there have been no significant changes in mortality in the last decades. The clinical and economic burden of CAP makes it a major public health problem, particularly for children and the elderly. This issue provides a clinical overview of CAP, focusing on epidemiology, economic burden, diagnosis, risk stratification, treatment, clinical management, and prevention. Particular attention is given to some aspects related to the clinical management of CAP, such as the microbial etiology and the available tools to achieve it, the usefulness of new and old biomarkers, and antimicrobial and other non-antibiotic adjunctive therapies. Possible scenarios in which pneumonia does not respond to treatment are also analyzed to improve clinical outcomes of CAP. PMID:21242952

  9. Acquired Porphyria Cutanea Tarda

    PubMed Central

    Koval, Andrew; Danby, C. W. E.; Petermann, H.

    1965-01-01

    Currently, the porphyrias are classified in four main groups: congenital porphyria, acute intermittent porphyria, porphyria cutanea tarda hereditaria, and porphyria cutanea tarda symptomatica. The acquired form of porphyria (porphyria cutanea tarda symptomatica) occurs in older males and is nearly always associated with chronic alcoholism and hepatic cirrhosis. The main clinical changes are dermatological, with excessive skin fragility and photosensitivity resulting in erosions and bullae. Biochemically, high levels of uroporphyrin are found in the urine and stools. Treatment to date has been symptomatic and usually unsuccessful. A case of porphyria cutanea tarda symptomatica is presented showing dramatic improvement of both the skin lesions and porphyrin levels in urine and blood following repeated phlebotomy. Possible mechanisms of action of phlebotomy on porphyria cutanea tarda symptomatica are discussed. ImagesFig. 1Fig. 2 PMID:14341652

  10. [ICU acquired neuromyopathy].

    PubMed

    Gueret, G; Guillouet, M; Vermeersch, V; Guillard, E; Talarmin, H; Nguyen, B-V; Rannou, F; Giroux-Metges, M-A; Pennec, J-P; Ozier, Y

    2013-09-01

    ICU acquired neuromyopathy (IANM) is the most frequent neurological pathology observed in ICU. Nerve and muscle defects are merged with neuromuscular junction abnormalities. Its physiopathology is complex. The aim is probably the redistribution of nutriments and metabolism towards defense against sepsis. The main risk factors are sepsis, its severity and its duration of evolution. IANM is usually diagnosed in view of difficulties in weaning from mechanical ventilation, but electrophysiology may allow an earlier diagnosis. There is no curative therapy, but early treatment of sepsis, glycemic control as well as early physiotherapy may decrease its incidence. The outcomes of IANM are an increase in morbi-mortality and possibly long-lasting neuromuscular abnormalities as far as tetraplegia. PMID:23958176

  11. Exploring the Limits of Frequency Lowering

    ERIC Educational Resources Information Center

    Souza, Pamela E.; Arehart, Kathryn H.; Kates, James M.; Croghan, Naomi B. H.; Gehani, Namita

    2013-01-01

    Purpose: This study examined how frequency lowering affected sentence intelligibility and quality for adults with postlingually acquired, mild-to-moderate hearing loss. Method: Listeners included adults aged 60-92 years with sloping sensorineural hearing loss and a control group of similarly aged adults with normal hearing. Sentences were…

  12. Tauroursodeoxycholic acid prevents hearing loss and hair cell death in Cdh23(erl/erl) mice.

    PubMed

    Hu, J; Xu, M; Yuan, J; Li, B; Entenman, S; Yu, H; Zheng, Q Y

    2016-03-01

    Sensorineural hearing loss has long been the subject of experimental and clinical research for many years. The recently identified novel mutation of the Cadherin23 (Cdh23) gene, Cdh23(erl/erl), was proven to be a mouse model of human autosomal recessive nonsyndromic deafness (DFNB12). Tauroursodeoxycholic acid (TUDCA), a taurine-conjugated bile acid, has been used in experimental research and clinical applications related to liver disease, diabetes, neurodegenerative diseases, and other diseases associated with apoptosis. Because hair cell apoptosis was implied to be the cellular mechanism leading to hearing loss in Cdh23(erl/erl) mice (erl mice), this study investigated TUDCA's otoprotective effects in erl mice: preventing hearing impairment and protecting against hair cell death. Our results showed that systemic treatment with TUDCA significantly alleviated hearing loss and suppressed hair cell death in erl mice. Additionally, TUDCA inhibited apoptotic genes and caspase-3 activation in erl mouse cochleae. The data suggest that TUDCA could be a potential therapeutic agent for human DFNB12. PMID:26748055

  13. Speech perception and quality of life of open-fit hearing aid users

    PubMed Central

    GARCIA, Tatiana Manfrini; JACOB, Regina Tangerino de Souza; MONDELLI, Maria Fernanda Capoani Garcia

    2016-01-01

    ABSTRACT Objective To relate the performance of individuals with hearing loss at high frequencies in speech perception with the quality of life before and after the fitting of an open-fit hearing aid (HA). Methods The WHOQOL-BREF had been used before the fitting and 90 days after the use of HA. The Hearing in Noise Test (HINT) had been conducted in two phases: (1) at the time of fitting without an HA (situation A) and with an HA (situation B); (2) with an HA 90 days after fitting (situation C). Study Sample Thirty subjects with sensorineural hearing loss at high frequencies. Results By using an analysis of variance and the Tukey’s test comparing the three HINT situations in quiet and noisy environments, an improvement has been observed after the HA fitting. The results of the WHOQOL-BREF have showed an improvement in the quality of life after the HA fitting (paired t-test). The relationship between speech perception and quality of life before the HA fitting indicated a significant relationship between speech recognition in noisy environments and in the domain of social relations after the HA fitting (Pearson’s correlation coefficient). Conclusions The auditory stimulation has improved speech perception and the quality of life of individuals. PMID:27383708

  14. Investigation of the long-term effects of unilateral hearing loss in adults.

    PubMed

    Colletti, V; Fiorino, F G; Carner, M; Rizzi, R

    1988-05-01

    The recent audiological literature has put forward the hypothesis that children with unilateral hearing loss (UHL) show delays in educational achievement and academic progress and some behavioural difficulties. This motivated us to investigate the long-term effects of monaural auditory deprivation in a group of adults who had suffered from UHL since childhood. A group of subjects, ranging in age from 30 to 55 years, suffering from sensorineural UHL since early childhood, has been examined for psychosocial and psychoacoustical effects and statistically compared with a control group matched for age and sex. We prepared a questionnaire directed to provide some objective and subjective indices of psychosocial disability and handicap. Some questions were directed towards specific aspects of auditory function; others assessed the degree of education and the type of working performed. The results of the investigation confirmed the superiority of binaural v. monaural hearing. This was clearly demonstrated in psycho-acoustical performance in sound localisation, speech recognition in noise, together with the appreciation of music. On the other hand, the parameters concerned with educational, social and employment achievement did not support the existence of any significant difference between binaurally and monaurally hearing subjects. The data obtained in the present study thus do not support the existence of non-auditory, long-term effects of monaural hearing loss. PMID:3390628

  15. Antioxidant-enriched diet does not delay the progression of age-related hearing loss.

    PubMed

    Sha, Su-Hua; Kanicki, Ariane; Halsey, Karin; Wearne, Kimberly Anne; Schacht, Jochen

    2012-05-01

    Oxidative stress has been linked to noise- and drug-induced as well as age-related hearing loss. Antioxidants can attenuate the decline of cochlear structure and function after exposure to noise or drugs, but it is debated as to whether they can protect from age-related hearing loss. In a long-term longitudinal study, 10-month-old female CBA/J mice were placed on either a control or antioxidant-enriched diet and monitored through 24 months of age. Supplementation with vitamins A, C, and E, L-carnitine, and α-lipoic acid significantly increased the antioxidant capacity of inner ear tissues. However, by 24 months of age, the magnitude of hearing loss was equal between the two groups. Likewise, there were no significant differences in hair cell loss or degeneration of spiral ganglion cells. We conclude that dietary manipulations can alter cochlear antioxidant capacity but do not ameliorate age-related sensorineural hearing loss in the CBA/J mouse. PMID:22154190

  16. Understanding speech in modulated interference: Cochlear implant users and normal-hearing listeners

    NASA Astrophysics Data System (ADS)

    Nelson, Peggy B.; Jin, Su-Hyun; Carney, Arlene Earley; Nelson, David A.

    2003-02-01

    Many competing noises in real environments are modulated or fluctuating in level. Listeners with normal hearing are able to take advantage of temporal gaps in fluctuating maskers. Listeners with sensorineural hearing loss show less benefit from modulated maskers. Cochlear implant users may be more adversely affected by modulated maskers because of their limited spectral resolution and by their reliance on envelope-based signal-processing strategies of implant processors. The current study evaluated cochlear implant users' ability to understand sentences in the presence of modulated speech-shaped noise. Normal-hearing listeners served as a comparison group. Listeners repeated IEEE sentences in quiet, steady noise, and modulated noise maskers. Maskers were presented at varying signal-to-noise ratios (SNRs) at six modulation rates varying from 1 to 32 Hz. Results suggested that normal-hearing listeners obtain significant release from masking from modulated maskers, especially at 8-Hz masker modulation frequency. In contrast, cochlear implant users experience very little release from masking from modulated maskers. The data suggest, in fact, that they may show negative effects of modulated maskers at syllabic modulation rates (2-4 Hz). Similar patterns of results were obtained from implant listeners using three different devices with different speech-processor strategies. The lack of release from masking occurs in implant listeners independent of their device characteristics, and may be attributable to the nature of implant processing strategies and/or the lack of spectral detail in processed stimuli.

  17. Spectral tilt change in stop consonant perception by listeners with hearing impairment

    PubMed Central

    Alexander, Joshua M.; Kluender, Keith R.

    2009-01-01

    Purpose To evaluate how spectral tilt changes in perceptual importance when formant information is degraded by sensorineural hearing loss. Method Eighteen listeners with mild to moderate hearing loss (HI listeners) and 20–23 listeners with normal hearing (NH listeners) identified synthesized stimuli that varied in second formant (F2) frequency and spectral tilt. Experiments 1 and 2 examined utterance-initial stops (/ba/ and /da/) and Experiments 3 and 4 examined medial stops (/aba/ and /ada/). Spectral tilt was manipulated at either consonant onset (Experiments 1 and 3), vowels (Experiments 2 and 4), or both (Experiment 5). Results Regression analysis revealed that HI listeners weighted F2 substantially less than NH listeners. There was no difference in absolute tilt weights between groups. However, HI listeners emphasized tilt as much as F2 for medial stops. NH listeners weighted tilt primarily when F2 was ambiguous, whereas HI listeners weighted tilt significantly more than NH listeners on unambiguous F2 endpoints. Conclusions Attenuating changes in spectral tilt can be as deleterious as taking away F2 information for HI listeners. Recordings through a wide-dynamic range compression hearing aid show compromised spectral tilt change, compressed in range by up to 50%. PMID:18952854

  18. A novel biallelic splice site mutation of TECTA causes moderate to severe hearing impairment in an Algerian family.

    PubMed

    Behlouli, Asma; Bonnet, Crystel; Abdi, Samia; Hasbellaoui, Mokhtar; Boudjenah, Farid; Hardelin, Jean-Pierre; Louha, Malek; Makrelouf, Mohamed; Ammar-Khodja, Fatima; Zenati, Akila; Petit, Christine

    2016-08-01

    Congenital deafness is certainly one of the most common monogenic diseases in humans, but it is also one of the most genetically heterogeneous, which makes molecular diagnosis challenging in most cases. Whole-exome sequencing in two out of three Algerian siblings affected by recessively-inherited, moderate to severe sensorineural deafness allowed us to identify a novel splice donor site mutation (c.5272+1G > A) in the gene encoding α-tectorin, a major component of the cochlear tectorial membrane. The mutation was present at the homozygous state in the three affected siblings, and at the heterozygous state in their unaffected, consanguineous parents. To our knowledge, this is the first reported TECTA mutation leading to the DFNB21 form of hearing impairment among Maghrebian individuals suffering from congenital hearing impairment, which further illustrates the diversity of the genes involved in congenital deafness in the Maghreb. PMID:27368438

  19. Current state of knowledge: outcomes research in children with mild to severe hearing impairment--approaches and methodological considerations.

    PubMed

    Tomblin, Bruce; Hebbeler, Kathleen

    2007-12-01

    This paper will provide a backdrop to the others in this section on outcomes of children with mild to moderate sensorineural hearing loss. Our objective here is to provide an overview of the research approaches used to study the outcomes of these children to provide guidance for future studies of children with mild to severe sensorineural hearing impairment (HI). During the past 20 yr there has been a gradual coalescence of research practices concerned with examining outcomes. Those who have done this work have come from different disciplines and in many cases were motivated by different circumstances. As a result, terminology and perspectives on outcomes research often still bears the features of these different disciplines and objectives of this research. This paper is an effort to provide the reader with an overview of this research endeavor that will highlight the diversity of the work being conducted on outcomes but also emphasizes the common properties. This overview will emphasize the kinds of research questions that are asked in this area of research and the associated evidence obtained to address these research questions. After this initial section there will be a consideration of the methodological issues that need to be considered, particularly for outcome research in children with HI. PMID:17982360

  20. Hearing Loss and Cytomegalovirus.

    ERIC Educational Resources Information Center

    Strauss, Melvin

    1997-01-01

    Cytomegalovirus is the most common cause of congenital virally induced hearing loss. Maternal infection is most often asymptomatic as is the infection in the newborn. Hearing loss occurs in both clinically apparent infection and in the asymptomatic infection. Current methods of detection, treatment, and prevention and research efforts are…

  1. Hereditary Hearing Loss.

    ERIC Educational Resources Information Center

    Tran, LenhAnh P.; Grundfast, Kenneth M.

    1997-01-01

    This article discusses inheritance patterns in hearing loss, epidemiology, clues to genetic causes, locating genes that cause hereditary disorders, genes related to hearing loss disorders in individuals with Usher syndrome, Waardenburg syndrome, Treacher-Collins syndrome, Branchio-oto-renal and Pendred syndromes, and the significance of finding…

  2. The Hearing Environment

    ERIC Educational Resources Information Center

    Capewell, Carmel

    2014-01-01

    Glue ear, a condition resulting in intermittent hearing loss in young children, affects about 80% of young children under seven years old. About 60% of children will spend a third of their time unable to hear within normal thresholds. Teachers are unlikely to consider the sound quality in classrooms. In my research young people provided…

  3. Molecular biology of hearing

    PubMed Central

    Stöver, Timo; Diensthuber, Marc

    2012-01-01

    The inner ear is our most sensitive sensory organ and can be subdivided into three functional units: organ of Corti, stria vascularis and spiral ganglion. The appropriate stimulus for the organ of hearing is sound, which travels through the external auditory canal to the middle ear where it is transmitted to the inner ear. The inner ear houses the hair cells, the sensory cells of hearing. The inner hair cells are capable of mechanotransduction, the transformation of mechanical force into an electrical signal, which is the basic principle of hearing. The stria vascularis generates the endocochlear potential and maintains the ionic homeostasis of the endolymph. The dendrites of the spiral ganglion form synaptic contacts with the hair cells. The spiral ganglion is composed of neurons that transmit the electrical signals from the cochlea to the central nervous system. In recent years there has been significant progress in research on the molecular basis of hearing. An increasing number of genes and proteins related to hearing are being identified and characterized. The growing knowledge of these genes contributes not only to greater appreciation of the mechanism of hearing but also to a deeper understanding of the molecular basis of hereditary hearing loss. This basic research is a prerequisite for the development of molecular diagnostics and novel therapies for hearing loss. PMID:22558056

  4. Hearing sensitivity in farmers.

    PubMed Central

    Karlovich, R S; Wiley, T L; Tweed, T; Jensen, D V

    1988-01-01

    Hearing sensitivity was measured for tones from 1,000 through 8,000 hertz (Hz) in 534 males and 278 females who resided in rural Wisconsin and ranged in age from 16 to 85 years. The hearing sensitivity for all subjects decreased with advancing age and at higher frequencies, but hearing loss over the range most susceptible to excessive noise exposure (3,000-6,000 Hz) was much greater for males than for females at all ages. The hearing loss was greater than could be accounted for by age and was similar whether the subject was a farmer or not. The results suggested that approximately 25 percent of the males had a communication handicap due to hearing loss by age 30, and the proportion rose to 50 percent by age 50. Less than 20 percent of farmers reported consistent use of personal hearing protection in their farm-related duties. Overall, the findings suggest that men who live in rural areas, including farmers, demonstrate a high prevalence of hearing loss and associated communication problems due to excessive noise exposure. This, in turn, clearly indicates a need for intensification of educational hearing conservation programs for the rural population. PMID:3124200

  5. Hearing Aid Tester

    NASA Technical Reports Server (NTRS)

    1978-01-01

    Hearing aids often develop malfunctions that are not detected by the wearer. This is particularly true when the wearers are school-age children. Studies of selected groups showed that from 30 to more than 50 percent of school children were not getting adequate benefit from their hearing aids because of unrecognized malfunctions, usually low or dead batteries. This can be serious because hearing impairment retards a child's educational progress. NASA technology incorporated in the Hearing Aid Malfunction Detection Unit (HAMDU), the device pictured, is expected to provide an effective countermeasure to the childrens' hearing aid problem. A patent license has been awarded to a minority-owned firm, Hopkins International Company, a subsidiary of H. H. Aerospace Design Co., Inc., Elmford, New York. The company plans early commercial availability of its version of the device.

  6. Individual Hearing Loss

    PubMed Central

    Dau, Torsten; Christensen-Dalsgaard, Jakob; Tranebjærg, Lisbeth; Andersen, Ture; Poulsen, Torben

    2016-01-01

    It is well-established that hearing loss does not only lead to a reduction of hearing sensitivity. Large individual differences are typically observed among listeners with hearing impairment in a wide range of suprathreshold auditory measures. In many cases, audiometric thresholds cannot fully account for such individual differences, which make it challenging to find adequate compensation strategies in hearing devices. How to characterize, model, and compensate for individual hearing loss were the main topics of the fifth International Symposium on Auditory and Audiological Research (ISAAR), held in Nyborg, Denmark, in August 2015. The following collection of papers results from some of the work that was presented and discussed at the symposium. PMID:27566802

  7. Hearing Aids and Hearing Impaired Students in Rural Schools.

    ERIC Educational Resources Information Center

    Woodford, Charles

    This paper describes functions of the components of hearing aids and provides a detailed procedure to detect hearing aid dysfunctions. The most common type of hearing aids for school children are the behind the ear type. Various hearing aid components change sound into an electrical signal, which is amplified and adjusted by a volume control. The…

  8. KCNJ10 gene mutations causing EAST syndrome (epilepsy, ataxia, sensorineural deafness, and tubulopathy) disrupt channel function

    PubMed Central

    Reichold, Markus; Zdebik, Anselm A.; Lieberer, Evelyn; Rapedius, Markus; Schmidt, Katharina; Bandulik, Sascha; Sterner, Christina; Tegtmeier, Ines; Penton, David; Baukrowitz, Thomas; Hulton, Sally-Anne; Witzgall, Ralph; Ben-Zeev, Bruria; Howie, Alexander J.; Kleta, Robert; Bockenhauer, Detlef; Warth, Richard

    2010-01-01

    Mutations of the KCNJ10 (Kir4.1) K+ channel underlie autosomal recessive epilepsy, ataxia, sensorineural deafness, and (a salt-wasting) renal tubulopathy (EAST) syndrome. We investigated the localization of KCNJ10 and the homologous KCNJ16 in kidney and the functional consequences of KCNJ10 mutations found in our patients with EAST syndrome. Kcnj10 and Kcnj16 were found in the basolateral membrane of mouse distal convoluted tubules, connecting tubules, and cortical collecting ducts. In the human kidney, KCNJ10 staining was additionally observed in the basolateral membrane of the cortical thick ascending limb of Henle's loop. EM of distal tubular cells of a patient with EAST syndrome showed reduced basal infoldings in this nephron segment, which likely reflects the morphological consequences of the impaired salt reabsorption capacity. When expressed in CHO and HEK293 cells, the KCNJ10 mutations R65P, G77R, and R175Q caused a marked impairment of channel function. R199X showed complete loss of function. Single-channel analysis revealed a strongly reduced mean open time. Qualitatively similar results were obtained with coexpression of KCNJ10/KCNJ16, suggesting a dominance of KCNJ10 function in native renal KCNJ10/KCNJ16 heteromers. The decrease in the current of R65P and R175Q was mainly caused by a remarkable shift of pH sensitivity to the alkaline range. In summary, EAST mutations of KCNJ10 lead to impaired channel function and structural changes in distal convoluted tubules. Intriguingly, the metabolic alkalosis present in patients carrying the R65P mutation possibly improves residual function of KCNJ10, which shows higher activity at alkaline pH. PMID:20651251

  9. Mutations in PTPRQ are a cause of autosomal-recessive nonsyndromic hearing impairment DFNB84 and associated with vestibular dysfunction.

    PubMed

    Schraders, Margit; Oostrik, Jaap; Huygen, Patrick L M; Strom, Tim M; van Wijk, Erwin; Kunst, Henricus P M; Hoefsloot, Lies H; Cremers, Cor W R J; Admiraal, Ronald J C; Kremer, Hannie

    2010-04-01

    We identified overlapping homozygous regions within the DFNB84 locus in a nonconsanguineous Dutch family and a consanguineous Moroccan family with sensorineural autosomal-recessive nonsyndromic hearing impairment (arNSHI). The critical region of 3.17 Mb harbored the PTPRQ gene and mouse models with homozygous mutations in the orthologous gene display severe hearing loss. We show that the human PTPRQ gene was not completely annotated and that additional, alternatively spliced exons are present at the 5' end of the gene. Different PTPRQ isoforms are encoded with a varying number of fibronectin type 3 (FN3) domains, a transmembrane domain, and a phosphatase domain. Sequence analysis of the PTPRQ gene in members of the families revealed a nonsense mutation in the Dutch family and a missense mutation in the Moroccan family. The missense mutation is located in one of the FN3 domains. The nonsense mutation results in a truncated protein with only a small number of FN3 domains and no transmembrane or phosphatase domain. Hearing loss in the patients with PTPRQ mutations is likely to be congenital and moderate to profound and most severe in the family with the nonsense mutation. Progression of the hearing loss was observed in both families. The hearing loss is accompanied by vestibular dysfunction in all affected individuals. Although we show that PTPRQ is expressed in many tissues, no symptoms other than deafness were observed in the patients. PMID:20346435

  10. Hearing Impairment and Retirement

    PubMed Central

    Fischer, Mary E; Cruickshanks, Karen J; Pinto, Alex; Klein, Barbara E K; Klein, Ronald; Dalton, Dayna S

    2013-01-01

    BACKGROUND Many factors influence the decision to retire including age, insurance and pension availability along with physical and mental health. Hearing impairment may be one such factor. PURPOSE The purpose of this study was to compare the 15 year retirement rate among subjects with and without hearing impairment. RESEARCH DESIGN Prospective, population-based study STUDY SAMPLE Subjects were participants in the Epidemiology of Hearing Loss Study (EHLS), a longitudinal investigation of age-related hearing loss. Participants who were working full- or part-time in 1993–1995 were included (n=1410, mean age=57.8 years). DATA COLLECTION AND ANALYSIS Data from four EHLS phases (1993–1995, 1998–2000, 2003–2005, and 2009–2010) were analyzed in 2010–2012. Hearing impairment was defined as a pure tone threshold average (at 0.5,1,2 and 4 kHz) greater than 25 dB HL in the worse ear. Employment status was determined at each of the four phases. Kaplan-Meier estimates of the cumulative incidence of retirement were calculated and Cox discrete-time modeling was used to determine the effect of hearing impairment on the rate of retirement. RESULTS The cumulative incidence of retirement was significantly (p < 0.02) higher in those with a hearing impairment (77%) compared to those without a hearing impairment (74%). After adjustment for age, gender, self-reported health, and history of chronic disease, there was no significant difference in the rate of retirement between those with and without a hearing impairment (Hazard Ratio (HR) = 0.9, 95% Confidence Interval (CI) = 0.7, 1.1). Similar results were observed when hearing aid users were excluded, when hearing impairment was based on the better ear thresholds, and when analyses were restricted to those less than 65 years of age and working full-time at baseline. Participants with a hearing impairment were less likely to state that the main reason for retirement was that the time seemed right. CONCLUSIONS Hearing impairment

  11. Globodontia in the Otodental Syndrome: A Rare Defect of Tooth Morphology Occurring with Hearing Loss in an Eight-Year-Old.

    PubMed

    Enright, Sinead; Humphrys, A Karen; Rea, Gillian; James, Jacqueline A

    2015-12-01

    Otodental syndrome is a hereditary disorder comprising globodontia and sensorineural hearing loss. Globodontia is characterized by distinctively bulbous, enlarged crowns of molar and primary canine teeth. Anomalies including taurodontism and hypodontia also occur. We report on the dental treatment and multidisciplinary management of an eight-year-old girl with this rare condition. Referral to Clinical Genetics and Oral Pathology was instrumental in establishing a diagnosis of otodental syndrome for this young patient and her mother, who had similar dental defects. CPD/Clinical Relevance: To increase awareness among practitioners of this rare dental disorder and highlight the need for multidisciplinary management of such cases. PMID:26855998

  12. The Grammatical Morpheme Deficit in Moderate Hearing Impairment

    ERIC Educational Resources Information Center

    McGuckian, Maria; Henry, Alison

    2007-01-01

    Background: Much remains unknown about grammatical morpheme (GM) acquisition by children with moderate hearing impairment (HI) acquiring spoken English. Aims: To investigate how moderate HI impacts on the use of GMs in speech and to provide an explanation for the pattern of findings. Methods & Procedures: Elicited and spontaneous speech data were…

  13. Working Memory Compensates for Hearing Related Phonological Processing Deficit

    ERIC Educational Resources Information Center

    Classon, Elisabet; Rudner, Mary; Ronnberg, Jerker

    2013-01-01

    Acquired hearing impairment is associated with gradually declining phonological representations. According to the Ease of Language Understanding (ELU) model, poorly defined representations lead to mismatch in phonologically challenging tasks. To resolve the mismatch, reliance on working memory capacity (WMC) increases. This study investigated…

  14. Performance analysis of ten brands of batteries for hearing aids

    PubMed Central

    Penteado, Silvio Pires; Bento, Ricardo Ferreira

    2013-01-01

    Summary Introduction: Comparison of the performance of hearing instrument batteries from various manufacturers can enable otologists, audiologists, or final consumers to select the best products, maximizing the use of these materials. Aim: To analyze the performance of ten brands of batteries for hearing aids available in the Brazilian marketplace. Methods: Hearing aid batteries in four sizes were acquired from ten manufacturers and subjected to the same test conditions in an acoustic laboratory. Results: The results obtained in the laboratory contrasted with the values reported by manufacturers highlighted significant discrepancies, besides the fact that certain brands in certain sizes perform better on some tests, but does not indicate which brand is the best in all sizes. Conclusions: It was possible to investigate the performance of ten brands of hearing aid batteries and describe the procedures to be followed for leakage, accidental intake, and disposal. PMID:25992026

  15. Acute Acquired Concomitant Esotropia

    PubMed Central

    Chen, Jingchang; Deng, Daming; Sun, Yuan; Shen, Tao; Cao, Guobin; Yan, Jianhua; Chen, Qiwen; Ye, Xuelian

    2015-01-01

    Abstract Acute acquired concomitant esotropia (AACE) is a rare, distinct subtype of esotropia. The purpose of this retrospective study was to describe the clinical characteristics and discuss the classification and etiology of AACE. Charts from 47 patients with AACE referred to our institute between October 2010 and November 2014 were reviewed. All participants underwent a complete medical history, ophthalmologic and orthoptic examinations, and brain and orbital imaging. Mean age at onset was 26.6 ± 12.2 years. Of the 18 cases with deviations ≤ 20 PD, 16 presented with diplopia at distance and fusion at near vision at the onset of deviation; differences between distance and near deviations were < 8 PD; all cases except one were treated with prism and diplopia resolved. Of the 29 cases with deviations > 20 PD, 5 were mild hypermetropic with age at onset between 5 and 19 years, 16 were myopic, and 8 were emmetropic with age at onset > 12 years; 24 were surgically treated and 5 cases remained under observation; all 24 cases achieved normal retinal correspondence or fusion or stereopsis on postoperative day 1 in synoptophore; in 23 cases diplopia or visual confusion resolved postoperatively. Of the 47 cases, brain and orbital imaging in 2 cases revealed a tumor in the cerebellopontine angle and 1 case involved spinocerebellar ataxia as revealed by genetic testing. AACE in this study was characterized by a sudden onset of concomitant nonaccommodative esotropia with diplopia or visual confusion at 5 years of age or older and the potential for normal binocular vision. We suggest that AACE can be divided into 2 subgroups consisting of patients with relatively small versus large angle deviations. Coexisting or underlying neurological diseases were infrequent in AACE. PMID:26705210

  16. A Comparison of Auditory Perception in Hearing-Impaired and Normal-Hearing Listeners: An Auditory Scene Analysis Study

    PubMed Central

    Bayat, Arash; Farhadi, Mohammad; Pourbakht, Akram; Sadjedi, Hamed; Emamdjomeh, Hesam; Kamali, Mohammad; Mirmomeni, Golshan

    2013-01-01

    Background Auditory scene analysis (ASA) is the process by which the auditory system separates individual sounds in natural-world situations. ASA is a key function of auditory system, and contributes to speech discrimination in noisy backgrounds. It is known that sensorineural hearing loss (SNHL) detrimentally affects auditory function in complex environments, but relatively few studies have focused on the influence of SNHL on higher level processes which are likely involved in auditory perception in different situations. Objectives The purpose of the current study was to compare the auditory system ability of normally hearing and SNHL subjects using the ASA examination. Materials and Methods A total of 40 right-handed adults (age range: 18 - 45 years) participated in this study. The listeners were divided equally into control and mild to moderate SNHL groups. ASA ability was measured using an ABA-ABA sequence. The frequency of the "A" was kept constant at 500, 1000, 2000 or 4000 Hz, while the frequency of the "B" was set at 3 to 80 percent above the" A" tone. For ASA threshold detection, the frequency of the B stimulus was decreased until listeners reported that they could no longer hear two separate sounds. Results The ASA performance was significantly better for controls than the SNHL group; these differences were more obvious at higher frequencies. We found no significant differences between ASA ability as a function of tone durations in both groups. Conclusions The present study indicated that SNHL may cause a reduction in perceptual separation of the incoming acoustic information to form accurate representations of our acoustic world. PMID:24719695

  17. The influence of audibility on speech recognition with nonlinear frequency compression for children and adults with hearing loss

    PubMed Central

    McCreery, Ryan W.; Alexander, Joshua; Brennan, Marc A.; Hoover, Brenda; Kopun, Judy; Stelmachowicz, Patricia G.

    2014-01-01

    Objective The primary goal of nonlinear frequency compression (NFC) and other frequency lowering strategies is to increase the audibility of high-frequency sounds that are not otherwise audible with conventional hearing-aid processing due to the degree of hearing loss, limited hearing aid bandwidth or a combination of both factors. The aim of the current study was to compare estimates of speech audibility processed by NFC to improvements in speech recognition for a group of children and adults with high-frequency hearing loss. Design Monosyllabic word recognition was measured in noise for twenty-four adults and twelve children with mild to severe sensorineural hearing loss. Stimuli were amplified based on each listener’s audiogram with conventional processing (CP) with amplitude compression or with NFC and presented under headphones using a software-based hearing aid simulator. A modification of the speech intelligibility index (SII) was used to estimate audibility of information in frequency-lowered bands. The mean improvement in SII was compared to the mean improvement in speech recognition. Results All but two listeners experienced improvements in speech recognition with NFC compared to CP, consistent with the small increase in audibility that was estimated using the modification of the SII. Children and adults had similar improvements in speech recognition with NFC. Conclusion Word recognition with NFC was higher than CP for children and adults with mild to severe hearing loss. The average improvement in speech recognition with NFC (7%) was consistent with the modified SII, which indicated that listeners experienced an increase in audibility with NFC compared to CP. Further studies are necessary to determine if changes in audibility with NFC are related to speech recognition with NFC for listeners with greater degrees of hearing loss, with a greater variety of compression settings, and using auditory training. PMID:24535558

  18. Effects of noise reduction on AM perception for hearing-impaired listeners.

    PubMed

    Ives, D Timothy; Kalluri, Sridhar; Strelcyk, Olaf; Sheft, Stanley; Miermont, Franck; Coez, Arnaud; Bizaguet, Eric; Lorenzi, Christian

    2014-10-01

    Noise reduction (NR) systems are commonplace in modern digital hearing aids. Though not improving speech intelligibility, NR helps the hearing-aid user in terms of lowering noise annoyance, reducing cognitive load and improving ease of listening. Previous psychophysical work has shown that NR does in fact improve the ability of normal-hearing (NH) listeners to discriminate the slow amplitude-modulation (AM) cues representative of those found in speech. The goal of this study was to assess whether this improvement of AM discrimination with NR can also be observed for hearing-impaired (HI) listeners. AM discrimination was measured at two audio frequencies of 500 Hz and 2 kHz in a background noise with a signal-to-noise ratio of 12 dB. Discrimination was measured for ten HI and ten NH listeners with and without NR processing. The HI listeners had a moderate sensorineural hearing loss of about 50 dB HL at 2 kHz and normal hearing (≤ 20 dB HL) at 500 Hz. The results showed that most of the HI listeners tended to benefit from NR at 500 Hz but not at 2 kHz. However, statistical analyses showed that HI listeners did not benefit significantly from NR at any frequency region. In comparison, the NH listeners showed a significant benefit from NR at both frequencies. For each condition, the fidelity of AM transmission was quantified by a computational model of early auditory processing. The parameters of the model were adjusted separately for the two groups (NH and HI) of listeners. The AM discrimination performance of the HI group (with and without NR) was best captured by a model simulating the loss of the fast-acting amplitude compression applied by the normal cochlea. This suggests that the lack of benefit from NR for HI listeners results from loudness recruitment. PMID:24899379

  19. Modern prescription theory and application: realistic expectations for speech recognition with hearing AIDS.

    PubMed

    Johnson, Earl E

    2013-01-01

    A major decision at the time of hearing aid fitting and dispensing is the amount of amplification to provide listeners (both adult and pediatric populations) for the appropriate compensation of sensorineural hearing impairment across a range of frequencies (e.g., 160-10000 Hz) and input levels (e.g., 50-75 dB sound pressure level). This article describes modern prescription theory for hearing aids within the context of a risk versus return trade-off and efficient frontier analyses. The expected return of amplification recommendations (i.e., generic prescriptions such as National Acoustic Laboratories-Non-Linear 2, NAL-NL2, and Desired Sensation Level Multiple Input/Output, DSL m[i/o]) for the Speech Intelligibility Index (SII) and high-frequency audibility were traded against a potential risk (i.e., loudness). The modeled performance of each prescription was compared one with another and with the efficient frontier of normal hearing sensitivity (i.e., a reference point for the most return with the least risk). For the pediatric population, NAL-NL2 was more efficient for SII, while DSL m[i/o] was more efficient for high-frequency audibility. For the adult population, NAL-NL2 was more efficient for SII, while the two prescriptions were similar with regard to high-frequency audibility. In terms of absolute return (i.e., not considering the risk of loudness), however, DSL m[i/o] prescribed more outright high-frequency audibility than NAL-NL2 for either aged population, particularly, as hearing loss increased. Given the principles and demonstrated accuracy of desensitization (reduced utility of audibility with increasing hearing loss) observed at the group level, additional high-frequency audibility beyond that of NAL-NL2 is not expected to make further contributions to speech intelligibility (recognition) for the average listener. PMID:24253361

  20. Modern Prescription Theory and Application: Realistic Expectations for Speech Recognition With Hearing Aids

    PubMed Central

    2013-01-01

    A major decision at the time of hearing aid fitting and dispensing is the amount of amplification to provide listeners (both adult and pediatric populations) for the appropriate compensation of sensorineural hearing impairment across a range of frequencies (e.g., 160–10000 Hz) and input levels (e.g., 50–75 dB sound pressure level). This article describes modern prescription theory for hearing aids within the context of a risk versus return trade-off and efficient frontier analyses. The expected return of amplification recommendations (i.e., generic prescriptions such as National Acoustic Laboratories—Non-Linear 2, NAL-NL2, and Desired Sensation Level Multiple Input/Output, DSL m[i/o]) for the Speech Intelligibility Index (SII) and high-frequency audibility were traded against a potential risk (i.e., loudness). The modeled performance of each prescription was compared one with another and with the efficient frontier of normal hearing sensitivity (i.e., a reference point for the most return with the least risk). For the pediatric population, NAL-NL2 was more efficient for SII, while DSL m[i/o] was more efficient for high-frequency audibility. For the adult population, NAL-NL2 was more efficient for SII, while the two prescriptions were similar with regard to high-frequency audibility. In terms of absolute return (i.e., not considering the risk of loudness), however, DSL m[i/o] prescribed more outright high-frequency audibility than NAL-NL2 for either aged population, particularly, as hearing loss increased. Given the principles and demonstrated accuracy of desensitization (reduced utility of audibility with increasing hearing loss) observed at the group level, additional high-frequency audibility beyond that of NAL-NL2 is not expected to make further contributions to speech intelligibility (recognition) for the average listener. PMID:24253361

  1. Noise and Hearing Loss Prevention

    MedlinePlus

    ... SafeInSound Noise and Hearing Loss on the NIOSH Science Blog Smartphone Sound Apps Music-induced Hearing Loss ... SafeInSound Noise and Hearing Loss on the NIOSH Science Blog Smartphone Sound Apps Music-induced Hearing Loss ...

  2. Can You Hear Me Now?

    ERIC Educational Resources Information Center

    Black, Susan

    2003-01-01

    Almost 15 percent of children from 6 to 19 years old have some degree of hearing loss in one or both ears. Offers guidelines for screening students' hearing and recommends that interventions begin with classroom acoustics, which affect all children including those with normal hearing. Includes strategies for accommodating hearing-impaired…

  3. 16 CFR 801.2 - Acquiring and acquired persons.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... confer control of X and therefore will file as an acquiring person. Because A held the plant prior to the... within two persons, “A” and “B.” Under this section, if V is to acquire corporation X, both “A” and “B... person. Examples: 1. Assume that person “Q” will acquire voting securities of corporation X held by...

  4. 16 CFR 801.2 - Acquiring and acquired persons.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... confer control of X and therefore will file as an acquiring person. Because A held the plant prior to the... within two persons, “A” and “B.” Under this section, if V is to acquire corporation X, both “A” and “B... person. Examples: 1. Assume that person “Q” will acquire voting securities of corporation X held by...

  5. Hearing in Cavefishes.

    PubMed

    Soares, Daphne; Niemiller, Matthew L; Higgs, Dennis M

    2016-01-01

    Caves and associated subterranean habitats represent some of the harshest environments on Earth, yet many organisms, including fishes, have colonized and thrive in these habitats despite the complete absence of light, and other abiotic and biotic constraints. Over 170 species of fishes are considered obligate subterranean inhabitants (stygobionts) that exhibit some degree of troglomorphy, including degeneration of eyes and reduction in pigmentation. To compensate for lack of vision, many species have evolved constructive changes to non-visual sensory modalities. In this chapter we review hearing in cavefishes, with particular emphasize on our own studies on amblyopsid cavefishes. Hearing in cavefishes has not been well studied to date, as hearing ability has only been examined in four species. Two species show no differences in hearing ability relative to their surface relatives, while the other two species (family Amblyopsidae) exhibit regression in the form of reduced hearing range and reduction in hair cell densities on sensory epithelia. In addition to reviewing our current knowledge on cavefish hearing, we offer suggestions for future avenues of research on cavefish hearing and discuss the influence of Popper and Fay on the field of cavefish bioacoustics. PMID:26515315

  6. HAPPEN CAN'T HEAR: An Analysis of Code-Blends in Hearing, Native Signers of American Sign Language

    ERIC Educational Resources Information Center

    Bishop, Michele

    2011-01-01

    Hearing native signers often learn sign language as their first language and acquire features that are characteristic of sign languages but are not present in equivalent ways in English (e.g., grammatical facial expressions and the structured use of space for setting up tokens and surrogates). Previous research has indicated that bimodal…

  7. [The mutation spectrum of the GJB2 gene in Belarussian patients with hearing loss. Results of pilot genetic screening of hearing impairment in newborns].

    PubMed

    Bliznets, E A; Marcul', D N; Khorov, O G; Markova, T G; Poliakov, A V

    2014-02-01

    A total of 111 unrelated probands and their 8 sibs from Grodno oblast (Belarus) with bilateral isolated sensorineural hearing impairment were studied for the presence of mutations in the connexin 26--GJB2gene. Mutations were detected in 51 probands (46% of the sample). A significantly higher frequency of the GJB2gene mutations was observed in familial cases of the disease with the autosomal recessive type of inheritance (in 78% of families). Detected peculiarities of the GJB2 gene mutation spectrum demonstrated that use of the algorithm, which was developed for Russian patients, is optimal for the molecular study of patients from Be- larus. In the sample of patients with hearing loss, the highest (among other similar samples studied in the world) allele frequency of c.313_326de114 mutation (7% out of all pathological GJB2 alleles) was registered; Polish origin of this deletion was suggested. It was demonstrated that detection of the GJB2 gene mutation on only one patient's chromosome is insufficient to confirm a molecular genetic diagnosis of hearing loss of the DFNB1 genetic type (autosomal recessive hearing loss caused by the GJB2 gene mutations). Pilot screening in the presence of GJB2 gene mutations in newborns from Grodno oblast was conducted. The material from 235 children was studied during the screening; nine heterozygous carriers of the mutation were found. The c.35delG mutation was detected in a homozygous state in a single newborn (hearing loss of moderate severity was subsequently audiologically confirmed in this child). PMID:25711030

  8. Micromechanics of hearing

    NASA Astrophysics Data System (ADS)

    Hudspeth, A. J.

    2015-12-01

    The following summarizes the key points addressed during a tutorial session on the Micromechanics of Hearing that took place at the 12th International Workshop on the Mechanics of Hearing held at Cape Sounio, Greece, in June 2014. The tutorial was intended to present an overview of basic ideas and to address topics of current interest relevant to the Workshop. The session was recorded, and the audio file and accompanying visual content of the presentation can be found in the Mechanics of Hearing Digital Library (www.mechanicsofhearing.org).

  9. Upcoming hearings in Congress

    NASA Astrophysics Data System (ADS)

    The following hearings have been tentatively scheduled for the coming weeks by the Senate and House of Representatives. Dates and times should be verified with the committee or subcommittee holding the hearing; all congressional offices may be reached by telephoning 202-224-3121. For guidelines on contacting a member of Congress, see AGU's Guide to Legislative Information and Contacts (Eos, August 28, 1984, p. 669).April 25: Oversight hearing on submerged lands by the Public Lands Subcommittee of the House Interior and Insular Affairs Committee. Room to be assigned, 9:45 A.M.

  10. Clinical and molecular analysis of a four-generation Chinese family with aminoglycoside-induced and nonsyndromic hearing loss associated with the mitochondrial 12S rRNA C1494T mutation

    SciTech Connect

    Wang Qiuju; Li Qingzhong; Han Dongyi . E-mail: hdy301@263.net; Zhao Yali; Zhao Lidong; Qian Yaping; Yuan Hu; Li Ronghua; Zhai Suoqiang; Young Wieyen . E-mail: ywy301@263.net; Guan Minxin . E-mail: min-xin.guan@chmcc.org

    2006-02-10

    We report here the clinical, genetic, and molecular characterization of a four-generation Chinese family with aminoglycoside-induced and nonsyndromic hearing loss. Five of nine matrilineal relatives had aminoglycoside-induced hearing loss. These matrilineal relatives exhibited variable severity and audiometric configuration of hearing impairment, despite sharing some common features: being bilateral and having sensorineural hearing impairment. Sequence analysis of mitochondrial DNA (mtDNA) in the pedigree identified 16 variants and the homoplasmic 12S rRNA C1494T mutation, which was associated with hearing loss in the other large Chinese family. In fact, the occurrence of the C1494T mutation in these genetically unrelated pedigrees affected by hearing impairment strongly indicated that this mutation is involved in the pathogenesis of aminoglycoside-induced and nonsyndromic hearing loss. However, incomplete penetrance of hearing loss indicated that the C1494T mutation itself is not sufficient to produce a clinical phenotype but requires the involvement of modifier factors for the phenotypic expression. Those mtDNA variants, showing no evolutional conservation, may not have a potential modifying role in the pathogenesis of the C1494T mutation. However, nuclear background seems to contribute to the phenotypic variability of matrilineal relatives in this family. Furthermore, aminoglycosides modulate the expressivity and penetrance of deafness associated with the C1494T mutation in this family.

  11. 16 CFR 801.2 - Acquiring and acquired persons.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... acquired person is the pre-acquisition ultimate parent entity of the entity. (ii) The value of an... directors of B. A is deemed to be acquiring all of the assets of B as a result. (g) Transfers of patent... transfer of patent rights covered by this paragraph constitutes an asset acquisition; and (3) Patent...

  12. Can Baby Hear?

    MedlinePlus

    ... Current Issue Past Issues Special Section: Focus on Communication Can Baby Hear? Past Issues / Fall 2008 Table ... to the National Institute on Deafness and Other Communication Disorders (NIDCD). Prior to this, the average age ...

  13. Devices for hearing loss

    MedlinePlus

    ... bring the sound from your TV, radio, or music player directly to your inner ear. Many listening devices now work through a wireless link and can connect directly to your hearing aid. There is also television closed-captioning, which ...

  14. Implantable digital hearing aid

    NASA Technical Reports Server (NTRS)

    Kissiah, A. M., Jr.

    1979-01-01

    Hearing aid converts analog output of microphone into digital pulses in about 10 channels of audiofrequencies. Each pulse band could be directly connected to portion of auditory nerve most sensitive to that range.

  15. Types of Hearing Aids

    MedlinePlus

    ... They also have greater flexibility in hearing aid programming so that the sound they transmit can be ... 10903 New Hampshire Avenue Silver Spring, MD 20993 1-888-INFO-FDA (1-888-463-6332) Contact ...

  16. Living with Hearing Loss

    MedlinePlus

    ... Current Issue Past Issues Special Section: Focus on Communication Living with Hearing Loss Past Issues / Fall 2008 ... the United States suffer some form of disordered communication. The National Institute on Deafness and Other Communication ...

  17. Occupational hearing loss

    MedlinePlus

    Over time, repeated exposure to loud noise and music can cause hearing loss. Sounds above 80 decibels ( ... Airline ground maintenance Construction Farming Jobs involving loud music or machinery In the U.S., laws regulate the ...

  18. Occupational hearing loss

    MedlinePlus

    ... risks connected with recreation such as shooting a gun, driving snowmobiles, or other, similar activities. Learn how ... hearing from recreational activities such as shooting a gun or driving snowmobiles. Do not listen to loud ...

  19. Hearing loss - infants

    MedlinePlus

    ... to sounds through play. These tests, known as visual response audiometry and play audiometry, can better determine ... the cause of hearing loss. Treatment may include: Speech therapy Learning sign language Cochlear implant (for those ...

  20. Hearing loss and music

    MedlinePlus

    ... iPod or MP3 Player The small ear bud style headphones (inserted into the ears) do not block ... Hearing Loss. National Institute on Deafness and Other Communication Disorders. NIH Pub. No. 14-4233. Updated: March ...

  1. A novel DFNA36 mutation in TMC1 orthologous to the Beethoven (Bth) mouse associated with autosomal dominant hearing loss in a Chinese family.

    PubMed

    Zhao, Yali; Wang, Dayong; Zong, Liang; Zhao, Feifan; Guan, Liping; Zhang, Peng; Shi, Wei; Lan, Lan; Wang, Hongyang; Li, Qian; Han, Bing; Yang, Ling; Jin, Xin; Wang, Jian; Wang, Jun; Wang, Qiuju

    2014-01-01

    Mutations in the transmembrane channel-like gene 1 (TMC1) can cause both DFNA36 and DFNB7/11 hearing loss. More than thirty DFNB7/11 mutations have been reported, but only three DFNA36 mutations were reported previously. In this study, we found a large Chinese family with 222 family members showing post-lingual, progressive sensorineural hearing loss which were consistent with DFNA36 hearing loss. Auditory brainstem response (ABR) test of the youngest patient showed a special result with nearly normal threshold but prolonged latency, decreased amplitude, and the abnormal waveform morphology. Exome sequencing of the proband found four candidate variants in known hearing loss genes. Sanger sequencing in all family members found a novel variant c.1253T>A (p.M418K) in TMC1 at DFNA36 that co-segregated with the phenotype. This mutation in TMC1 is orthologous to the mutation found in the hearing loss mouse model named Bth ten years ago. In another 51 Chinese autosomal dominant hearing loss families, we screened the segments containing the dominant mutations of TMC1 and no functional variants were found. TMC1 is expressed in the hair cells in inner ear. Given the already known roles of TMC1 in the mechanotransduction in the cochlea and its expression in inner ear, our results may provide an interesting perspective into its function in inner ear. PMID:24827932

  2. Diagnosis of Hearing Loss.

    ERIC Educational Resources Information Center

    World Federation of the Deaf, Rome (Italy).

    Seven conference papers from the U.S.S.R., India, Poland, Czechoslovakia, and Yugoslavia consider the diagnosis of hearing loss. They are "Examination of Hearing of Children, Aged from 2 to 5, by Means of Playing Audiometry" by A. P. Kossacheva, "A Study of the Etiology and Pattern of Deafness in a School for the Deaf in Madras, South India" by Y.…

  3. Hearing Aids and Music

    PubMed Central

    Chasin, Marshall; Russo, Frank A.

    2004-01-01

    Historically, the primary concern for hearing aid design and fitting is optimization for speech inputs. However, increasingly other types of inputs are being investigated and this is certainly the case for music. Whether the hearing aid wearer is a musician or merely someone who likes to listen to music, the electronic and electro-acoustic parameters described can be optimized for music as well as for speech. That is, a hearing aid optimally set for music can be optimally set for speech, even though the converse is not necessarily true. Similarities and differences between speech and music as inputs to a hearing aid are described. Many of these lead to the specification of a set of optimal electro-acoustic characteristics. Parameters such as the peak input-limiting level, compression issues—both compression ratio and knee-points—and number of channels all can deleteriously affect music perception through hearing aids. In other cases, it is not clear how to set other parameters such as noise reduction and feedback control mechanisms. Regardless of the existence of a “music program,” unless the various electro-acoustic parameters are available in a hearing aid, music fidelity will almost always be less than optimal. There are many unanswered questions and hypotheses in this area. Future research by engineers, researchers, clinicians, and musicians will aid in the clarification of these questions and their ultimate solutions. PMID:15497032

  4. Acquired Immune Deficiency Syndrome, AIDS: A Selected Bibliography of Federal Government Publications. Research Guide 90 104.

    ERIC Educational Resources Information Center

    Alexander, Margaret

    This research guide presents a selected bibliography of federal government publications about the Acquired Immune Deficiency Syndrome (AIDS). These documents are listed in five categories: (1) Bibliographies (7); (2) Congressional Publications (69 hearings and reports); (3) Executive Branch Publications (43 reports); (4) Federal Government…

  5. An efficient strategy for establishing a model of sensorineural deafness in rats.

    PubMed

    Ma, Long; Yi, Hai-Jin; Yuan, Fen-Qian; Guo, Wei-Wei; Yang, Shi-Ming

    2015-10-01

    Ototoxic drugs can be used to produce a loss of cochlear hair cells to create animal models of deafness. However, to the best of our knowledge, there is no report on the establishment of a rat deafness model through the combined application of aminoglycosides and loop diuretics. The aim of this study was to use single or combined administration of furosemide and kanamycin sulfate to establish rat models of deafness. The rats received intravenous injections of different doses of furosemide and/or intramuscular injections of kanamycin sulfate. The auditory brainstem response was measured to determine the hearing threshold after drug application. Immunocytochemistry and confocal microscopy were performed to evaluate inner ear morphology. In the group receiving combined administration of furosemide and kanamycin, the auditory brainstem response threshold showed significant elevation 3 days after administration, higher than that produced by furosemide or kanamycin alone. The hair cells showed varying degrees of injury, from the apical turn to the basal turn of the cochlea and from the outer hair cells to the inner hair cells. The spiral ganglion cells maintained a normal morphology during the first week after the hair cells completely disappeared, and then gradually degenerated. After 2 months, the majority of spiral ganglion cells disappeared, but a few remained. These findings demonstrate that the combined administration of furosemide and kanamycin has a synergistic ototoxic effect, and that these drugs can produce hair cell loss and hearing loss in rats. These findings suggest that even in patients with severe deafness, electronic cochlear implants may partially restore hearing. PMID:26692870

  6. An efficient strategy for establishing a model of sensorineural deafness in rats

    PubMed Central

    Ma, Long; Yi, Hai-jin; Yuan, Fen-qian; Guo, Wei-wei; Yang, Shi-ming

    2015-01-01

    Ototoxic drugs can be used to produce a loss of cochlear hair cells to create animal models of deafness. However, to the best of our knowledge, there is no report on the establishment of a rat deafness model through the combined application of aminoglycosides and loop diuretics. The aim of this study was to use single or combined administration of furosemide and kanamycin sulfate to establish rat models of deafness. The rats received intravenous injections of different doses of furosemide and/or intramuscular injections of kanamycin sulfate. The auditory brainstem response was measured to determine the hearing threshold after drug application. Immunocytochemistry and confocal microscopy were performed to evaluate inner ear morphology. In the group receiving combined administration of furosemide and kanamycin, the auditory brainstem response threshold showed significant elevation 3 days after administration, higher than that produced by furosemide or kanamycin alone. The hair cells showed varying degrees of injury, from the apical turn to the basal turn of the cochlea and from the outer hair cells to the inner hair cells. The spiral ganglion cells maintained a normal morphology during the first week after the hair cells completely disappeared, and then gradually degenerated. After 2 months, the majority of spiral ganglion cells disappeared, but a few remained. These findings demonstrate that the combined administration of furosemide and kanamycin has a synergistic ototoxic effect, and that these drugs can produce hair cell loss and hearing loss in rats. These findings suggest that even in patients with severe deafness, electronic cochlear implants may partially restore hearing. PMID:26692870

  7. Children Acquire Emotion Categories Gradually

    ERIC Educational Resources Information Center

    Widen, Sherri C.; Russell, James A.

    2008-01-01

    Some accounts imply that basic-level emotion categories are acquired early and quickly, whereas others imply that they are acquired later and more gradually. Our study examined this question for fear, happiness, sadness, and anger in the context of children's categorization of emotional facial expressions. Children (N=168, 2-5 years) first labeled…

  8. FGF23 Deficiency Leads to Mixed Hearing Loss and Middle Ear Malformation in Mice

    PubMed Central

    Lysaght, Andrew C.; Yuan, Quan; Fan, Yi; Kalwani, Neil; Caruso, Paul; Cunnane, MaryBeth; Lanske, Beate; Stanković, Konstantina M.

    2014-01-01

    Fibroblast growth factor 23 (FGF23) is a circulating hormone important in phosphate homeostasis. Abnormal serum levels of FGF23 result in systemic pathologies in humans and mice, including renal phosphate wasting diseases and hyperphosphatemia. We sought to uncover the role FGF23 plays in the auditory system due to shared molecular mechanisms and genetic pathways between ear and kidney development, the critical roles multiple FGFs play in auditory development and the known hearing phenotype in mice deficient in klotho (KL), a critical co-factor for FGF23 signaling. Using functional assessments of hearing, we demonstrate that Fgf mice are profoundly deaf. Fgf mice have moderate hearing loss above 20 kHz, consistent with mixed conductive and sensorineural pathology of both middle and inner ear origin. Histology and high-voltage X-ray computed tomography of Fgf mice demonstrate dysplastic bulla and ossicles; Fgf mice have near-normal morphology. The cochleae of mutant mice appear nearly normal on gross and microscopic inspection. In wild type mice, FGF23 is ubiquitously expressed throughout the cochlea. Measurements from Fgf mice do not match the auditory phenotype of Kl−/− mice, suggesting that loss of FGF23 activity impacts the auditory system via mechanisms at least partially independent of KL. Given the extensive middle ear malformations and the overlap of initiation of FGF23 activity and Eustachian tube development, this work suggests a possible role for FGF23 in otitis media. PMID:25243481

  9. 41 CFR 105-57.005 - Hearing.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ...-ADMINISTRATION WAGE GARNISHMENT § 105-57.005 Hearing. (a) GSA will provide a hearing, which at the hearing... summary of the facts presented; (2) The hearing official's findings, analysis and conclusions; and (3)...

  10. Suprathreshold auditory processing deficits in noise: Effects of hearing loss and age.

    PubMed

    Kortlang, Steffen; Mauermann, Manfred; Ewert, Stephan D

    2016-01-01

    People with sensorineural hearing loss generally suffer from a reduced ability to understand speech in complex acoustic listening situations, particularly when background noise is present. In addition to the loss of audibility, a mixture of suprathreshold processing deficits is possibly involved, like altered basilar membrane compression and related changes, as well as a reduced ability of temporal coding. A series of 6 monaural psychoacoustic experiments at 0.5, 2, and 6 kHz was conducted with 18 subjects, divided equally into groups of young normal-hearing, older normal-hearing and older hearing-impaired listeners, aiming at disentangling the effects of age and hearing loss on psychoacoustic performance in noise. Random frequency modulation detection thresholds (RFMDTs) with a low-rate modulator in wide-band noise, and discrimination of a phase-jittered Schroeder-phase from a random-phase harmonic tone complex are suggested to characterize the individual ability of temporal processing. The outcome was compared to thresholds of pure tones and narrow-band noise, loudness growth functions, auditory filter bandwidths, and tone-in-noise detection thresholds. At 500 Hz, results suggest a contribution of temporal fine structure (TFS) to pure-tone detection thresholds. Significant correlation with auditory thresholds and filter bandwidths indicated an impact of frequency selectivity on TFS usability in wide-band noise. When controlling for the effect of threshold sensitivity, the listener's age significantly correlated with tone-in-noise detection and RFMDTs in noise at 500 Hz, showing that older listeners were particularly affected by background noise at low carrier frequencies. PMID:26471199

  11. SLC26A4 gene is frequently involved in nonsyndromic hearing impairment with enlarged vestibular aqueduct in Caucasian populations.

    PubMed

    Albert, Sébastien; Blons, Hélène; Jonard, Laurence; Feldmann, Delphine; Chauvin, Pierre; Loundon, Nathalie; Sergent-Allaoui, Annie; Houang, Muriel; Joannard, Alain; Schmerber, Sébastien; Delobel, Bruno; Leman, Jacques; Journel, Hubert; Catros, Hélène; Dollfus, Hélène; Eliot, Marie-Madeleine; David, Albert; Calais, Catherine; Drouin-Garraud, Valérie; Obstoy, Marie-Françoise; Tran Ba Huy, Patrice; Lacombe, Didier; Duriez, Françoise; Francannet, Christine; Bitoun, Pierre; Petit, Christine; Garabédian, Eréa-Noël; Couderc, Rémy; Marlin, Sandrine; Denoyelle, Françoise

    2006-06-01

    Sensorineural hearing loss is the most frequent sensory deficit of childhood and is of genetic origin in up to 75% of cases. It has been shown that mutations of the SLC26A4 (PDS) gene were involved in syndromic deafness characterized by congenital sensorineural hearing impairment and goitre (Pendred's syndrome), as well as in congenital isolated deafness (DFNB4). While the prevalence of SLC26A4 mutations in Pendred's syndrome is clearly established, it remains to be studied in large cohorts of patients with nonsyndromic deafness and detailed clinical informations. In this report, 109 patients from 100 unrelated families, aged from 1 to 32 years (median age: 10 years), with nonsyndromic deafness and enlarged vestibular aqueduct, were genotyped for SLC26A4 using DHPLC molecular screening and sequencing. In all, 91 allelic variants were observed in 100 unrelated families, of which 19 have never been reported. The prevalence of SLC26A4 mutations was 40% (40/100), with biallelic mutation in 24% (24/100), while six families were homozygous. All patients included in this series had documented deafness, associated with EVA and without any evidence of syndromic disease. Among patients with SLC26A4 biallelic mutations, deafness was more severe, fluctuated more than in patients with no mutation. In conclusion, the incidence of SLC26A4 mutations is high in patients with isolated deafness and enlarged vestibular aqueduct and could represent up to 4% of nonsyndromic hearing impairment. SLC26A4 could be the second most frequent gene implicated in nonsyndromic deafness after GJB2, in this Caucasian population. PMID:16570074

  12. A novel frameshift mutation of POU4F3 gene associated with autosomal dominant non-syndromic hearing loss

    SciTech Connect

    Lee, Hee Keun; Park, Hong-Joon; Lee, Kyu-Yup; Park, Rekil; Kim, Un-Kyung

    2010-06-04

    Autosomal dominant mutations in the transcription factor POU4F3 gene are associated with non-syndromic hearing loss in humans; however, there have been few reports of mutations in this gene worldwide. We performed a mutation analysis of the POU4F3 gene in 42 unrelated Koreans with autosomal dominant non-syndromic hearing loss, identifying a novel 14-bp deletion mutation in exon 2 (c.662del14) in one patient. Audiometric examination revealed severe bilateral sensorineural hearing loss in this patient. The novel mutation led to a truncated protein that lacked both functional POU domains. We further investigated the functional distinction between wild-type and mutant POU4F3 proteins using in vitro assays. The wild-type protein was completely localized in the nucleus, while the truncation of protein seriously affected its nuclear localization. In addition, the mutant failed to activate reporter gene expression. This is the first report of a POU4F3 mutation in Asia, and moreover our data suggest that further investigation will need to delineate ethnicity-specific genetic background for autosomal dominant non-syndromic hearing loss within Asian populations.

  13. 18 CFR 401.84 - Hearing procedure.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... ADMINISTRATIVE MANUAL RULES OF PRACTICE AND PROCEDURE Administrative and Other Hearings § 401.84 Hearing procedure. (a) Participation in the hearing. In any hearing, the person requesting the hearing shall be... 18 Conservation of Power and Water Resources 2 2010-04-01 2010-04-01 false Hearing procedure....

  14. 18 CFR 1308.33 - Hearings.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 18 Conservation of Power and Water Resources 2 2011-04-01 2011-04-01 false Hearings. 1308.33... Prehearing and Hearing Procedures § 1308.33 Hearings. (a) TVA shall arrange for the verbatim reporting of evidentiary hearings before the Hearing Officer, and shall provide the Hearing Officer with the...

  15. 18 CFR 1308.33 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 18 Conservation of Power and Water Resources 2 2010-04-01 2010-04-01 false Hearings. 1308.33... Prehearing and Hearing Procedures § 1308.33 Hearings. (a) TVA shall arrange for the verbatim reporting of evidentiary hearings before the Hearing Officer, and shall provide the Hearing Officer with the...

  16. 20 CFR 658.417 - Hearings.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 20 Employees' Benefits 3 2010-04-01 2010-04-01 false Hearings. 658.417 Section 658.417 Employees... Hearings. (a) Hearings shall be held by State hearing officials. A State hearing official may be any State official authorized to hold hearings under State law. They may be, for example, the same referees who...

  17. 42 CFR 431.222 - Group hearings.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 42 Public Health 4 2010-10-01 2010-10-01 false Group hearings. 431.222 Section 431.222 Public... Recipients Right to Hearing § 431.222 Group hearings. The agency— (a) May respond to a series of individual requests for hearing by conducting a single group hearing; (b) May consolidate hearings only in cases...

  18. 42 CFR 431.222 - Group hearings.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 42 Public Health 4 2011-10-01 2011-10-01 false Group hearings. 431.222 Section 431.222 Public... Recipients Right to Hearing § 431.222 Group hearings. The agency— (a) May respond to a series of individual requests for hearing by conducting a single group hearing; (b) May consolidate hearings only in cases...

  19. 20 CFR 410.645 - Joint hearings.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 20 Employees' Benefits 2 2011-04-01 2011-04-01 false Joint hearings. 410.645 Section 410.645..., Finality of Decisions, and Representation of Parties § 410.645 Joint hearings. When two or more hearings... joint hearing, a joint hearing may not be held. Where joint hearings are held, a single record of...

  20. 20 CFR 410.645 - Joint hearings.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 20 Employees' Benefits 2 2010-04-01 2010-04-01 false Joint hearings. 410.645 Section 410.645..., Finality of Decisions, and Representation of Parties § 410.645 Joint hearings. When two or more hearings... joint hearing, a joint hearing may not be held. Where joint hearings are held, a single record of...