Performance Assessment of Hearing Protection and Communication Enhancement Devices: Peltor Comtac III and IV

DTIC Science & Technology

2015-07-01

providing a hear-thru, or active capability while mitigating hearing loss and tinnitus caused by exposure to loud, steady-state and impulse noise. Active...loss and tinnitus caused by exposure to loud, steady-state and impulse noise. The general approach was to use ANSI standard measurement procedures

Sensorineural hearing loss in hemorrhagic dengue?

PubMed

Ribeiro, Bruna Natália Freire; Guimarães, Alexandre Caixeta; Yazawa, Felipe; Takara, Tammy Fumiko Messias; de Carvalho, Guilherme Machado; Zappelini, Carlos Eduardo Monteiro

2015-01-01

Dengue is an acute febrile infectious disease, with high fever followed by symptoms flu-like. Dengue hemorrhagic fever (DHF) is a vascular leak syndrome and could present spontaneous bleeding and worsening of symptoms after some days. Dengue could have some ENT manifestations, however hearing loss is not one of them. Sudden hearing loss is considered as sensorineural or perceptual hearing loss with a sudden onset in a person without other prior otological history. The relation between infectious diseases and sudden hearing are been investigated, some viruses were already linked, but the relation between dengue virus and sudden hearing still remains unknown. This article has the goal of presenting a case of DHF that evolved with SSHL in his hospitalization process. We report a 60 years-male patient of with DHF who developed bilateral secretory otitis media and sensorineural hearing loss after the fifth day of onset of symptoms. His hearing loss remained even after 7 months and the patient was referred for hearing aid fitting. This is the first case report that brings together DHF and sudden hearing loss. In the development of this case no other cause to sudden hearing loss was found and the correlation between dengue and hearing loss was questioned. In the literature review was found that some viruses, as mumps virus, varicella-zoster virus and HSV-1 and HSV-2 are related to sudden hearing loss, all of them fit in the viral theory. Besides the viral theory of sudden hearing loss, there is the vascular theory that is the occlusion of the end artery that supplies the cochlea. DHF has a vascular commitment, and the hypothesis of a vascular cause could be elicited in this case. Many studies in this area are needed and this article has the objective of elicit the discussion about the subject. Could dengue be associated with sensorineural hearing loss? Copyright © 2015. Published by Elsevier Ltd.

[Tinnitus and deafness].

PubMed

Dauman, R

2000-01-15

The relationships between tinnitus and hearing loss are studied from a clinical prospect. Five critical points are discussed. 1. Some degree of hearing loss is found in the vast majority of tinnitus patients; but an individual may well have a sensorineural hearing loss and no tinnitus at all. 2. A minor adjunction to the neurophysiological model of Jastreboff is proposed to take account of the association between tinnitus and hearing loss. 3. Tinnitus appears to cause more distress when hearing loss is marked. 4. Self-reported hearing loss should be considered when implementing habituation sound therapy. 5. According to McKinney, the rate of success on tinnitus that can be expected with habituation sound therapy is not significantly affected by hearing level.

Facts about Jaundice and Kernicterus

MedlinePlus

... in a baby’s blood. It can cause athetoid cerebral palsy and hearing loss. Kernicterus also causes problems with ... or a lactation coach for help. Related Links Cerebral Palsy Hearing Loss in Children Intellectual Disabilities [PDF - 289K] ...

Genetics of non syndromic hearing loss.

PubMed

Venkatesh, M D; Moorchung, Nikhil; Puri, Bipin

2015-10-01

Non Syndromic Hearing Loss is an important cause for hearing loss. One in 1000 newborns have some hearing impairment. Over 400 genetic syndromes have been described. Non Syndromic Hearing Loss (NSHL) can be inherited in an Autosomal Dominant, Autosomal Recessive or a Sex Linked fashion. There are several reasons why genetic testing should be done in cases of NSHL, the main reasons being for genetic screening and for planning treatment. This review describes the genes involved in NSHL and the genetic mechanisms involved in the pathogenesis of the disease.

Inner ear disorders.

PubMed

Smouha, Eric

2013-01-01

To present a framework for the diagnosis and treatment of inner ear disorders, with an emphasis on problems common to neuro-rehabilitation. Disorders of the inner ear can cause hearing loss, tinnitus, vertigo and imbalance. Hearing loss can be conductive, sensorineural, or mixed; conductive hearing loss arises from the ear canal or middle ear, while sensorineural hearing loss arises from the inner ear or auditory nerve. Vertigo is a hallucination of motion, and is the cardinal symptom of vestibular system disease. It should be differentiated from other causes of dizziness: gait imbalance, disequilibrium, lightheadedness (pre-syncope). Vertigo can be caused by problems in the inner ear or central nervous system. The diagnosis of inner ear disorders begins with a targeted physical examination. The initial work-up of hearing loss is made by audiometry, and vertigo by electronystagmography (ENG). Supplemental tests and MRI are obtained when clinically indicated. The clinical pattern and duration of vertigo are the most important clinical features in the diagnosis. Common inner ear causes of vertigo include: vestibular neuritis (sudden, unilateral vestibular loss), Meniere's disease (episodic vertigo), benign paroxysmal positional vertigo (BPPV), and bilateral vestibular loss. Common central nervous system causes of vertigo include: post concussion syndrome, cervical vertigo, vestibular migraine, cerebrovascular disease, and acoustic neuroma. A basic knowledge of vestibular physiology, coupled with a understanding of common vestibular syndromes, will lead to correct diagnosis and treatment in most cases.

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…

Hearing impairment caused by mutations in two different genes responsible for nonsyndromic and syndromic hearing loss within a single family.

PubMed

Niepokój, Katarzyna; Rygiel, Agnieszka M; Jurczak, Piotr; Kujko, Aleksandra A; Śniegórska, Dominika; Sawicka, Justyna; Grabarczyk, Alicja; Bal, Jerzy; Wertheim-Tysarowska, Katarzyna

2018-02-01

Usher syndrome is rare genetic disorder impairing two human senses, hearing and vision, with the characteristic late onset of vision loss. This syndrome is divided into three types. In all cases, the vision loss is postlingual, while loss of hearing is usually prelingual. The vestibular functions may also be disturbed in Usher type 1 and sometimes in type 3. Vestibular areflexia is helpful in making a proper diagnosis of the syndrome, but, often, the syndrome is misdiagnosed as a nonsyndromic hearing loss. Here, we present a Polish family with hearing loss, which was clinically classified as nonsyndromic. After excluding mutations in the DFNB1 locus, we implemented the next-generation sequencing method and revealed that hearing loss was syndromic and mutations in the USH2A gene indicate Usher syndrome. This research highlights the importance of molecular analysis in establishing a clinical diagnosis of congenital hearing loss.

10 Ways to Identify Hearing Loss | NIH MedlinePlus the Magazine

MedlinePlus

... Does a hearing problem cause you difficulty when listening to TV or radio? Do you feel that any difficulty with your hearing limits or hampers your personal or social life? Does a hearing problem cause you difficulty ...

Impact of Aging on the Auditory System and Related Cognitive Functions: A Narrative Review

PubMed Central

Jayakody, Dona M. P.; Friedland, Peter L.; Martins, Ralph N.; Sohrabi, Hamid R.

2018-01-01

Age-related hearing loss (ARHL), presbycusis, is a chronic health condition that affects approximately one-third of the world's population. The peripheral and central hearing alterations associated with age-related hearing loss have a profound impact on perception of verbal and non-verbal auditory stimuli. The high prevalence of hearing loss in the older adults corresponds to the increased frequency of dementia in this population. Therefore, researchers have focused their attention on age-related central effects that occur independent of the peripheral hearing loss as well as central effects of peripheral hearing loss and its association with cognitive decline and dementia. Here we review the current evidence for the age-related changes of the peripheral and central auditory system and the relationship between hearing loss and pathological cognitive decline and dementia. Furthermore, there is a paucity of evidence on the relationship between ARHL and established biomarkers of Alzheimer's disease, as the most common cause of dementia. Such studies are critical to be able to consider any causal relationship between dementia and ARHL. While this narrative review will examine the pathophysiological alterations in both the peripheral and central auditory system and its clinical implications, the question remains unanswered whether hearing loss causes cognitive impairment or vice versa. PMID:29556173

[Urban noise pollution].

PubMed

Chouard, C H

2001-07-01

Noise is responsible for cochlear and general damages. Hearing loss and tinnitus greatly depend on sound intensity and duration. Short-duration sound of sufficient intensity (gunshot or explosion) will not be described because they are not currently encountered in our normal urban environment. Sound levels of less than 75 d (A) are unlikely to cause permanent hearing loss, while sound levels of about 85 d (A) with exposures of 8 h per day will produce permanent hearing loss after many years. Popular and largely amplified music is today one of the most dangerous causes of noise induced hearing loss. The intensity of noises (airport, highway) responsible for stress and general consequences (cardiovascular) is generally lower. Individual noise sensibility depends on several factors. Strategies to prevent damage from sound exposure should include the use of individual hearing protection devices, education programs beginning with school-age children, consumer guidance, increased product noise labelling, and hearing conservation programs for occupational settings.

Noise and Hearing Loss: A Review

ERIC Educational Resources Information Center

Daniel, Eileen

2007-01-01

Background: Noise-induced hearing loss is a major cause of deafness and hearing impairment in the United States. Though genetics and advanced age are major risk factors, temporary and permanent hearing impairments are becoming more common among young adults and children especially with the increased exposure to portable music players. Though…

Pathophysiology of the inner ear after blast injury caused by laser-induced shock wave

PubMed Central

Niwa, Katsuki; Mizutari, Kunio; Matsui, Toshiyasu; Kurioka, Takaomi; Matsunobu, Takeshi; Kawauchi, Satoko; Satoh, Yasushi; Sato, Shunichi; Shiotani, Akihiro; Kobayashi, Yasushi

2016-01-01

The ear is the organ that is most sensitive to blast overpressure, and ear damage is most frequently seen after blast exposure. Blast overpressure to the ear results in sensorineural hearing loss, which is untreatable and is often associated with a decline in the quality of life. In this study, we used a rat model to demonstrate the pathophysiological and structural changes in the inner ear that replicate pure sensorineural hearing loss associated with blast injury using laser-induced shock wave (LISW) without any conductive hearing loss. Our results indicate that threshold elevation of the auditory brainstem response (ABR) after blast exposure was primarily caused by outer hair cell dysfunction induced by stereociliary bundle disruption. The bundle disruption pattern was unique; disturbed stereocilia were mostly observed in the outermost row, whereas those in the inner and middle rows stereocilia remained intact. In addition, the ABR examination showed a reduction in wave I amplitude without elevation of the threshold in the lower energy exposure group. This phenomenon was caused by loss of the synaptic ribbon. This type of hearing dysfunction has recently been described as hidden hearing loss caused by cochlear neuropathy, which is associated with tinnitus or hyperacusis. PMID:27531021

Pathophysiology of the inner ear after blast injury caused by laser-induced shock wave.

PubMed

Niwa, Katsuki; Mizutari, Kunio; Matsui, Toshiyasu; Kurioka, Takaomi; Matsunobu, Takeshi; Kawauchi, Satoko; Satoh, Yasushi; Sato, Shunichi; Shiotani, Akihiro; Kobayashi, Yasushi

2016-08-17

The ear is the organ that is most sensitive to blast overpressure, and ear damage is most frequently seen after blast exposure. Blast overpressure to the ear results in sensorineural hearing loss, which is untreatable and is often associated with a decline in the quality of life. In this study, we used a rat model to demonstrate the pathophysiological and structural changes in the inner ear that replicate pure sensorineural hearing loss associated with blast injury using laser-induced shock wave (LISW) without any conductive hearing loss. Our results indicate that threshold elevation of the auditory brainstem response (ABR) after blast exposure was primarily caused by outer hair cell dysfunction induced by stereociliary bundle disruption. The bundle disruption pattern was unique; disturbed stereocilia were mostly observed in the outermost row, whereas those in the inner and middle rows stereocilia remained intact. In addition, the ABR examination showed a reduction in wave I amplitude without elevation of the threshold in the lower energy exposure group. This phenomenon was caused by loss of the synaptic ribbon. This type of hearing dysfunction has recently been described as hidden hearing loss caused by cochlear neuropathy, which is associated with tinnitus or hyperacusis.

Cochlear implantation for severe sensorineural hearing loss caused by lightning.

PubMed

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

2012-01-01

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

PubMed Central

PALUDETTI, G.; CONTI, G.; DI NARDO, W.; DE CORSO, E.; ROLESI, R.; PICCIOTTI, P.M.; FETONI, A.R.

2012-01-01

SUMMARY Hearing loss is one of the most common disabilities and has lifelong consequences for affected children and their families. Both conductive and sensorineural hearing loss (SNHL) may be caused by a wide variety of congenital and acquired factors. Its early detection, together with appropriate intervention, is critical to speech, language and cognitive development in hearing-impaired children. In the last two decades, the application of universal neonatal hearing screening has improved identification of hearing loss early in life and facilitates early intervention. Developments in molecular medicine, genetics and neuroscience have improved the aetiological classification of hearing loss. Once deafness is established, a systematic approach to determining the cause is best undertaken within a dedicated multidisciplinary setting. This review addresses the innovative evidences on aetiology and management of deafness in children, including universal neonatal screening, advances in genetic diagnosis and the contribution of neuroimaging. Finally, therapy remains a major challenge in management of paediatric SNHL. Current approaches are represented by hearing aids and cochlear implants. However, recent advances in basic medicine which are identifying the mechanisms of cochlear damage and defective genes causing deafness, may represent the basis for novel therapeutic targets including implantable devices, auditory brainstem implants and cell therapy. PMID:23349554

Social representation of "hearing loss": cross-cultural exploratory study in India, Iran, Portugal, and the UK.

PubMed

Manchaiah, Vinaya; Danermark, Berth; Ahmadi, Tayebeh; Tomé, David; Zhao, Fei; Li, Qiang; Krishna, Rajalakshmi; Germundsson, Per

2015-01-01

Hearing loss is one of the most common chronic conditions in older adults. In audiology literature, several studies have examined the attitudes and behavior of people with hearing loss; however, not much is known about the manner in which society in general views and perceives hearing loss. This exploratory study was aimed at understanding the social representation of hearing loss (among the general public) in the countries of India, Iran, Portugal, and the UK. We also compared these social representations. The study involved a cross-sectional design, and participants were recruited using the snowball sampling method. A total of 404 people from four countries participated in the study. Data were collected using a free-association task where participants were asked to produce up to five words or phrases that came to mind while thinking about hearing loss. In addition, they were also asked to indicate if each word they presented had positive, neutral, or negative associations in their view. Data were analyzed using various qualitative and quantitative methods. The most frequently occurring categories were: assessment and management; causes of hearing loss; communication difficulties; disability; hearing ability or disability; hearing instruments; negative mental state; the attitudes of others; and sound and acoustics of the environment. Some categories were reported with similar frequency in most countries (eg, causes of hearing loss, communication difficulties, and negative mental state), whereas others differed among countries. Participants in India reported significantly more positive and fewer negative associations when compared to participants from Iran, Portugal, and the UK. However, there was no statistical difference among neutral responses reported among these countries. Also, more differences were noted among these countries than similarities. These findings provide useful insights into the public perception of hearing loss that may prove useful in public education and counseling.

Variations in otological presentation of lightning strike victims: Clinical report of 3 patients.

PubMed

Kılıç, Erbil; Genç, Hakan; Aydın, Ümit; Aşık, Burak; Satar, Bülent

2017-03-01

Lightning strike can cause fatal or nonfatal injuries. Some nonfatal injuries are associated with otological symptoms and findings. Conductive hearing loss due to rupture of the tympanic membrane is the most common audiovestibular lesion of lightning strike. Various forms of sensorineural hearing loss and dizziness have also been reported. Presently described are 3 cases of lightning strike injury. First patient had mid-frequency hearing loss in right ear and high frequency sensorineural hearing loss in left ear. Second patient had high frequency sensorineural hearing loss in left ear, and the third had peripheral facial palsy with perilymphatic fistula on same side. This is the first documented case of mid-frequency hearing loss occurring after lightning strike.

Cellular and Deafness Mechanisms Underlying Connexin Mutation-Induced Hearing Loss – A Common Hereditary Deafness

PubMed Central

Wingard, Jeffrey C.; Zhao, Hong-Bo

2015-01-01

Hearing loss due to mutations in the connexin gene family, which encodes gap junctional proteins, is a common form of hereditary deafness. In particular, connexin 26 (Cx26, GJB2) mutations are responsible for ~50% of non-syndromic hearing loss, which is the highest incidence of genetic disease. In the clinic, Cx26 mutations cause various auditory phenotypes ranging from profound congenital deafness at birth to mild, progressive hearing loss in late childhood. Recent experiments demonstrate that congenital deafness mainly results from cochlear developmental disorders rather than hair cell degeneration and endocochlear potential reduction, while late-onset hearing loss results from reduction of active cochlear amplification, even though cochlear hair cells have no connexin expression. However, there is no apparent, demonstrable relationship between specific changes in connexin (channel) functions and the phenotypes of mutation-induced hearing loss. Moreover, new experiments further demonstrate that the hypothesized K+-recycling disruption is not a principal deafness mechanism for connexin deficiency induced hearing loss. Cx30 (GJB6), Cx29 (GJC3), Cx31 (GJB3), and Cx43 (GJA1) mutations can also cause hearing loss with distinct pathological changes in the cochlea. These new studies provide invaluable information about deafness mechanisms underlying connexin mutation-induced hearing loss and also provide important information for developing new protective and therapeutic strategies for this common deafness. However, the detailed cellular mechanisms underlying these pathological changes remain unclear. Also, little is known about specific mutation-induced pathological changes in vivo and little information is available for humans. Such further studies are urgently required. PMID:26074771

[Acute hearing loss and tinnitus caused by amplified recreational music].

PubMed

Metternich, F U; Brusis, T

1999-11-01

Hearing loss resulting from exposure to permanent or repeated amplified music in professional musicians and music consumers is described in literature. The risk of hearing loss does not exist only after prolonged exposure to music. Short-term exposure to very high sound levels, for example in concerts, can also cause hearing loss and tinnitus. The retrospective study includes 24 patients who required rheologic therapy between 1994 and 1997 due to a music related acoustic trauma. The type, intensity, and length of music exposure as well as the distance and the position to the source of noise were examined. The type of hearing damage and its development during rheological treatment was studied by pure-tone audiometry. In the majority of examined patients (67%) the hearing loss developed on the basis of one-time exposure at a rock concert or pop concert, followed by hearing loss from attending discotheques (17%) or parties (12%), and music exposure from personal cassette players (4%). The majority of patients showed a maximum hearing loss of 40-60 dB (A) in a frequency between 3 kHz and 4 kHz. Pure-tone audiometry in 58% of the patients exhibited a unilateral threshold in a frequency between 3 kHz and 4 kHz combined with ipsilateral tinnitus of the same frequency. Twenty-one percent of the patients showed a symmetric bilateral threshold and tinnitus between 3 kHz and 4 kHz. In 8% there was a unilateral tinnitus, and in 13% a bilateral tinnitus without any hearing loss. All patients improved their hearing loss during rheologic treatment. Improvement in the tinnitus was only achieved in 33% of the examined cases. The risk of permanent hearing loss resulting from short-term exposure to amplified music is low compared to the risk of continuous tinnitus. Given the lack of acceptance of personal ear protectors, the risk of acute hearing damage due to amplified music could be reduced by avoiding the immediate proximity to the speakers.

Curing Hearing Loss: Patient Expectations, Health Care Practitioners, and Basic Science

ERIC Educational Resources Information Center

Oshima, Kazuo; Suchert, Steffen; Blevins, Nikolas H.; Heller, Stefan

2010-01-01

Millions of patients are debilitated by hearing loss, mainly caused by degeneration of sensory hair cells in the cochlea. The underlying reasons for hair cell loss are highly diverse, ranging from genetic disposition, drug side effects, traumatic noise exposure, to the effects of aging. Whereas modern hearing aids offer some relief of the symptoms…

Auditory brainstem responses of CBA/J mice with neonatal conductive hearing losses and treatment with GM1 ganglioside.

PubMed

Money, M K; Pippin, G W; Weaver, K E; Kirsch, J P; Webster, D B

1995-07-01

Exogenous administration of GM1 ganglioside to CBA/J mice with a neonatal conductive hearing loss ameliorates the atrophy of spiral ganglion neurons, ventral cochlear nucleus neurons, and ventral cochlear nucleus volume. The present investigation demonstrates the extent of a conductive loss caused by atresia and tests the hypothesis that GM1 ganglioside treatment will ameliorate the conductive hearing loss. Auditory brainstem responses were recorded from four groups of seven mice each: two groups received daily subcutaneous injections of saline (one group had normal hearing; the other had a conductive hearing loss); the other two groups received daily subcutaneous injections of GM1 ganglioside (one group had normal hearing; the other had a conductive hearing loss). In mice with a conductive loss, decreases in hearing sensitivity were greatest at high frequencies. The decreases were determined by comparing mean ABR thresholds of the conductive loss mice with those of normal hearing mice. The conductive hearing loss induced in the mice in this study was similar to that seen in humans with congenital aural atresias. GM1 ganglioside treatment had no significant effect on ABR wave I thresholds or latencies in either group.

Isolated Sensorineural Hearing Loss as a Sequela after Lightning Strike

PubMed Central

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

2015-01-01

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

Performance Assessment of Communication Enhancement Devices TEA HI Threat Headset

DTIC Science & Technology

2015-08-01

while mitigating hearing loss and tinnitus caused by exposure to loud, steady-state and impulsive noise. Active devices should theoretically have...mitigating hearing loss and tinnitus caused by exposure to loud, steady-state and impulsive noise. The general approach for this assessment was to use

Cochlear Implantation in Siblings With Refsum's Disease.

PubMed

Stähr, Kerstin; Kuechler, Alma; Gencik, Martin; Arnolds, Judith; Dendy, Meaghan; Lang, Stephan; Arweiler-Harbeck, Diana

2017-08-01

Whether the origin of severe hearing loss in Refsum's syndrome is caused by cochlear impairment or retrocochlear degeneration remains unclear. This case report aims to investigate hearing performance before and after cochlear implantation to shed light on this question. Also, identification of new mutations causing Refsum's syndrome would be helpful in generating additional means of diagnosis. A family of 4 individuals was subjected to genetic testing. Two siblings (56 and 61 years old) suffered from severe hearing and vision loss and received bilateral cochlear implants. Genetic analysis, audiological outcome, and clinical examinations were performed. One new mutation in the PHYH gene (c.768del63bp) causing Refsum's disease was found. Preoperative distortion product otoacoustic emissions (DPAOEs) were absent. Postoperative speech perception in Freiburger speech test was 100% for bisyllabic words and 85% (patient No. 1) and 65% (patient No. 2), respectively, for monosyllabic words. Five years after implantation, speech perception remained stable for bisyllabic words but showed decreasing capabilities for monosyllabic words. A new mutation causing Refsum's disease is presented. Cochlear implantation in case of severe hearing loss leads to an improvement in speech perception and should be recommended for patients with Refsum's disease, especially when the hearing loss is combined with a severe loss of vision. Decrease of speech perception in the long-term follow-up could indicate an additional retrocochlear degeneration.

Treating hearing disorders with cell and gene therapy

NASA Astrophysics Data System (ADS)

Gillespie, Lisa N.; Richardson, Rachael T.; Nayagam, Bryony A.; Wise, Andrew K.

2014-12-01

Hearing loss is an increasing problem for a substantial number of people and, with an aging population, the incidence and severity of hearing loss will become more significant over time. There are very few therapies currently available to treat hearing loss, and so the development of new therapeutic strategies for hearing impaired individuals is of paramount importance to address this unmet clinical need. Most forms of hearing loss are progressive in nature and therefore an opportunity exists to develop novel therapeutic approaches to slow or halt hearing loss progression, or even repair or replace lost hearing function. Numerous emerging technologies have potential as therapeutic options. This paper details the potential of cell- and gene-based therapies to provide therapeutic agents to protect sensory and neural cells from various insults known to cause hearing loss; explores the potential of replacing lost sensory and nerve cells using gene and stem cell therapy; and describes the considerations for clinical translation and the challenges that need to be overcome.

Industrial hearing conservation.

PubMed

Glorig, A

1979-08-01

Hearing conservation programs, when appropriate in industry, are now a necessity. Even though one may not wish to adopt an altruistic attitude toward the conservation of hearing, one must, like it or not, initiate a hearing conservation program because of both federal and state regulations. Since industrial noise exposure produced more hearing loss in more people than all other causes of hearing loss combined, it is incumbent on all industries with noise makers to do something about protecting human hearing. The tragedy is that nearly all industrial hearing loss can be prevented with proper hearing conservation measures. The cost of hearing conservation is far less than the cost of hearing loss in terms of human suffering and dollars in the compensation courts. Proper education of both managment and labor can result in successful hearing conservation programs. The method of choice is reduction of the noise at the source, but in many cases this is infeasible both technically and economically and therefore protection at the ear must be used. Experience has shown that with proper supervision ear protection programs can prevent the majority of instances of hearing loss in the majority of individuals exposed.

Combination therapy using antioxidants and low level laser therapy (LLLT) on noise induced hearing loss (NIHL)

NASA Astrophysics Data System (ADS)

Chang, So-Young; Lim, Sung Kyu; Lee, Min young; Chung, Phil-Sang; Jung, Jae-Yun; Rhee, Chung-Ku

2016-02-01

One of the most common factors that cause hearing disorders is noise trauma. Noise is an increasing hazard and it is pervasive, which makes it difficult to take precautions and prevent noise-induced hearing loss (NIHL). The prevalence of hearing loss among factory workers to be 42 %[1]. Ocupational noise induced hearing loss (ONIHL) continues to be a significant occupational hazard. ONIHL is permanent and may cause significant disability, for which there currently exists no cure, but is largely preventable. More than 30 million Americans are potentially exposed to hazardous noise levels in occupations such as transportation, construction, and coal mining, as well as recreationally. In the mainstream setting, exposure avoidance strategies aimed to reduce the incidence of ONIHL remain the focus of public health and occupational medicine approaches[2]. In military conditions this is most often caused by such things as explosions, blasts, or loud noises from vehicles ranging from 100 to 140 dB[3] and military weapons generating approximately 140-185 dB peak sound pressure levels[4].

Taking the History and Performing the Physical Examination in a Child with Hearing Loss.

PubMed

Cushing, Sharon L; Papsin, Blake C

2015-12-01

Hearing loss is one of the most common childhood disorders and has far reaching effects on communication and socialization in children. Language acquisition, the most commonly sought and measured outcome, is tightly linked to age at diagnosis of the hearing loss and the speed with which rehabilitation is instituted. Treatment is often not affected by the underlying cause of the hearing loss and should be initiated at the time of initial identification. History-taking and physical examination in the setting of pediatric hearing loss are straightforward and should include an assessment of motor milestones, balance, and vestibular function. Copyright © 2015 Elsevier Inc. All rights reserved.

Hearing loss and the high speed dental handpiece.

PubMed Central

Zubick, H H; Tolentino, A T; Boffa, J

1980-01-01

A pure tone air conduction audiometric evaluation was administered to 137 dentists and 80 physicians. The physicians were found to have better hearing threshold levels, notably in the 4000HZ center frequency range. The left ear of right handed dentists showed a greater loss of hearing ostensibly related to proximity to the noise source. Dental specialists showed a loss pattern similar to those of the general dentists. The findings suggest that there may be a cause and effect relationship between hearing loss and use of the highspeed dental handpiece. PMID:6990802

Working memory, age, and hearing loss: susceptibility to hearing aid distortion.

PubMed

Arehart, Kathryn H; Souza, Pamela; Baca, Rosalinda; Kates, James M

2013-01-01

Hearing aids use complex processing intended to improve speech recognition. Although many listeners benefit from such processing, it can also introduce distortion that offsets or cancels intended benefits for some individuals. The purpose of the present study was to determine the effects of cognitive ability (working memory) on individual listeners' responses to distortion caused by frequency compression applied to noisy speech. The present study analyzed a large data set of intelligibility scores for frequency-compressed speech presented in quiet and at a range of signal-to-babble ratios. The intelligibility data set was based on scores from 26 adults with hearing loss with ages ranging from 62 to 92 years. The listeners were grouped based on working memory ability. The amount of signal modification (distortion) caused by frequency compression and noise was measured using a sound quality metric. Analysis of variance and hierarchical linear modeling were used to identify meaningful differences between subject groups as a function of signal distortion caused by frequency compression and noise. Working memory was a significant factor in listeners' intelligibility of sentences presented in babble noise and processed with frequency compression based on sinusoidal modeling. At maximum signal modification (caused by both frequency compression and babble noise), the factor of working memory (when controlling for age and hearing loss) accounted for 29.3% of the variance in intelligibility scores. Combining working memory, age, and hearing loss accounted for a total of 47.5% of the variability in intelligibility scores. Furthermore, as the total amount of signal distortion increased, listeners with higher working memory performed better on the intelligibility task than listeners with lower working memory did. Working memory is a significant factor in listeners' responses to total signal distortion caused by cumulative effects of babble noise and frequency compression implemented with sinusoidal modeling. These results, together with other studies focused on wide-dynamic range compression, suggest that older listeners with hearing loss and poor working memory are more susceptible to distortions caused by at least some types of hearing aid signal-processing algorithms and by noise, and that this increased susceptibility should be considered in the hearing aid fitting process.

Hearing Disorders and Deafness

MedlinePlus

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

Deafness and Hearing Loss. NICHCY Disability Fact Sheet #3

ERIC Educational Resources Information Center

National Dissemination Center for Children with Disabilities, 2010

2010-01-01

Hearing is one of the five senses. Hearing gives access to sounds in the world--people's voices, their words, a car horn blown in warning or as hello! When a child has a hearing loss, it is cause for immediate attention. That is because language and communication skills develop most rapidly in childhood, especially before the age of 3. When…

Overview of a public health approach to pediatric hearing impairment in the Pacific Islands.

PubMed

Kaspar, Annette; Kei, Joseph; Driscoll, Carlie; Swanepoel, De Wet; Goulios, Helen

2016-07-01

Childhood hearing impairment is a significant cause of disability in developing countries. Otitis media and meningitis are leading infectious causes of preventable hearing loss in children. It is estimated that the Pacific Islands have among the greatest global burden of childhood hearing impairment due to infectious causes, and yet there is currently very little in the research literature on pediatric hearing disorders in this region. (1) To review existing research literature on pediatric hearing impairment in the Pacific Islands, and (2) to present a public health approach to the development and improvement of childhood hearing services in the Pacific Islands. The primary tool was a comprehensive literature review. MEDLINE and ScienceDirect databases were searched for relevant journal articles. There was no limit on the date of publication. Any article reporting on hearing impairment in the Pacific Region was included. A total of 23 journal articles were found that satisfied the above inclusion criteria. The limited information available in the literature suggests that otitis media and vaccine-preventable infections are a significant cause of avoidable childhood hearing impairment in the Pacific Islands. Pediatric audiology services are limited in this region. Further research is required to develop effective public health programs that should reduce the burden of preventable childhood hearing loss in the Pacific Islands. There is limited information in the research literature on pediatric hearing impairment and audiology services in the Pacific Islands. Epidemiological data based on the WHO Ear and Hearing Disorders Survey Protocol are urgently needed, and the development of audiology services within the existing public and primary health care framework should reduce the burden of preventable hearing loss in the Pacific Islands. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Rehabilitation for disabling hearing loss: evaluating the need relative to provision of hearing aids in the public health care system.

PubMed

Hlayisi, Vera-Genevey; Ramma, Lebogang

2018-05-20

To determine the demand relative to supply of hearing rehabilitation through hearing aids for those with disabling hearing loss in a public health care setting in South Africa. Retrospective cross-sectional survey of medical records of all patients were seen at a public hospital (Polokwane Provincial Academic) during 2012-2014, was conducted. Audiological data from 3894 medical folders were accessed and reviewed; thereafter, results were analyzed using descriptive statistics. Of the 3894 medical folders reviewed, two-third (62%, n = 2402) were diagnosed with hearing loss, mostly bilaterally (81%). More than 30% of all patients diagnosed with hearing loss were ≤10 years old. Sensorineural (permanent) hearing loss was diagnosed most often (38%, n = 913) and 74% (n = 1778) of hearing losses diagnosed were of moderate or worse severity (i.e., disabling loss). Hearing aids were fitted to only 15% (n = 272) of those diagnosed with disabling hearing loss and most hearing aid fittings were to low-income adult patients (≥25 years old) with more severe-profound hearing losses. This study showed that the need for hearing aids to provide hearing rehabilitation far exceeds the supply. Therefore, a multi-pronged approach that includes increased budget allocation and exploring low-cost interventions for developing countries to meet the demand for hearing aids. Furthermore, study highlighted a high prevalence of hearing loss in those younger than 10 years of age, and thus highlights the need for early intervention as well as intensifying efforts to reduce preventable causes of hearing loss. Implications for Rehabilitation Audiologists need to advocate for an increase in budget allocation for hearing rehabilitation devices. Study indicates need to explore low-cost hearing devices/rehabilitation interventions for developing countries. Health professionals should consider preventative measures to reduce prevalence of preventable hearing loss.

A Qualitative Study on Knowledge and Attitude towards Risk Factors, Early Identification and Intervention of Infant Hearing Loss among Puerperal Mothers- A Short Survey.

PubMed

Dudda, Ravi; Muniyappa, Hanumanth Prasad; Puttaraju, Sahana; Lakshmi, M S

2017-07-01

Maternal active participation and their support are critical for the success of early hearing loss detection program. Erroneous maternal decisions may have large life long consequences on the infant's life. The mothers' knowledge and their attitudes towards infant hearing loss is the basis for their decisions. The present study was done to determine the mothers' knowledge and their attitude towards risk factors of infant hearing loss, its early identification and intervention and also awareness of effect of consanguinity on hearing loss. In this cross-sectional questionnaire survey study, a total of 100 mothers were interviewed using the questionnaire which consisted of three sections namely risk factors, early identification and early intervention of hearing loss. Chi-square test was used to establish relationship between consanguineous and non-consanguineous mother's responses to its effect on hearing loss. A p-value < 0.05 was considered as significant. Mothers' awareness was significantly high for visible causes (ear pain/discharge, head injury and slap to ear) of hearing loss. Positive attitude was seen for importance of screening programs and follow up testing. Moderate level of awareness was found on hazards of consanguinity and benefits of early identification. However, mothers were least aware of neonatal jaundice, NICU admission (>5 days), signs of late-onset and neural hearing loss, management of hearing loss, hearing aid fitting and therapy necessity, which might interfere in early detection and intervention of hearing loss. It is crucial to educate mothers on few risk factors and management of hearing loss to reduce its consequences.

Diphtheria and hearing loss.

PubMed

Schubert, C R; Cruickshanks, K J; Wiley, T L; Klein, R; Klein, B E; Tweed, T S

2001-01-01

To determine if infectious diseases usually experienced in childhood have an effect on hearing ability later in life. The Epidemiology of Hearing Loss Study (N = 3,753) is a population-based study of age-related hearing loss in adults aged 48 to 92 years in Beaver Dam, Wisconsin. As part of this study, infectious disease history was obtained and hearing was tested using pure-tone audiometry. Hearing loss was defined as a pure-tone average of thresholds at 500 Hz, 1,000 Hz, 2,000 Hz, and 4,000 Hz greater than 25 decibels hearing level in either ear. After adjusting for confounders, only a history of diphtheria (n = 37) was associated with hearing loss (odds ratio [OR] 2.79; 95% confidence interval [CI] 1.05, 7.36). There was no relationship between hearing loss and history of chickenpox, measles, mumps, pertussis, polio, rheumatic fever, rubella, or scarlet fever. Only two participants with a history of diphtheria and hearing loss reported having a hearing loss before age 20. Diphtheria in childhood may have consequences for hearing that do not become apparent until later in life. A possible biological mechanism for a diphtheria effect on hearing ability exists: The toxin produced by the Corynebacterium diphtheriae bacteria can cause damage to cranial nerves and therefore may affect the auditory neural pathway. These data may have important implications for areas facing a resurgence of diphtheria cases.

Social representation of “hearing loss”: cross-cultural exploratory study in India, Iran, Portugal, and the UK

PubMed Central

Manchaiah, Vinaya; Danermark, Berth; Ahmadi, Tayebeh; Tomé, David; Zhao, Fei; Li, Qiang; Krishna, Rajalakshmi; Germundsson, Per

2015-01-01

Background Hearing loss is one of the most common chronic conditions in older adults. In audiology literature, several studies have examined the attitudes and behavior of people with hearing loss; however, not much is known about the manner in which society in general views and perceives hearing loss. This exploratory study was aimed at understanding the social representation of hearing loss (among the general public) in the countries of India, Iran, Portugal, and the UK. We also compared these social representations. Materials and methods The study involved a cross-sectional design, and participants were recruited using the snowball sampling method. A total of 404 people from four countries participated in the study. Data were collected using a free-association task where participants were asked to produce up to five words or phrases that came to mind while thinking about hearing loss. In addition, they were also asked to indicate if each word they presented had positive, neutral, or negative associations in their view. Data were analyzed using various qualitative and quantitative methods. Results The most frequently occurring categories were: assessment and management; causes of hearing loss; communication difficulties; disability; hearing ability or disability; hearing instruments; negative mental state; the attitudes of others; and sound and acoustics of the environment. Some categories were reported with similar frequency in most countries (eg, causes of hearing loss, communication difficulties, and negative mental state), whereas others differed among countries. Participants in India reported significantly more positive and fewer negative associations when compared to participants from Iran, Portugal, and the UK. However, there was no statistical difference among neutral responses reported among these countries. Also, more differences were noted among these countries than similarities. Conclusion These findings provide useful insights into the public perception of hearing loss that may prove useful in public education and counseling. PMID:26604726

Imaging of post-traumatic hearing loss.

PubMed

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

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

Birth Outcomes Among U.S. Women With Hearing Loss.

PubMed

Mitra, Monika; Akobirshoev, Ilhom; McKee, Michael M; Iezzoni, Lisa I

2016-12-01

The purpose of this study is to estimate the national occurrence of deliveries in women with hearing loss and to compare their birth outcomes to women without hearing loss. This study examined the 2008-2011 Nationwide Inpatient Sample of the Healthcare Cost and Utilization Project in 2015 to compare birth outcomes in women with hearing loss and without. Birth outcomes included preterm birth and low birth weight. Multivariate regression analyses compared birth outcomes between women with and without hearing loss, controlling for maternal age, racial and ethnic identity, type of health insurance, comorbidity, region of hospital, location and teaching status of the hospital, ownership of the hospital, and median household income for mother's ZIP code. Of an estimated 17.9 million deliveries, 10,462 occurred in women with hearing loss. In adjusted regression analyses controlling for demographic characteristics, women with hearing loss were significantly more likely than those without hearing loss to have preterm birth (OR=1.28, 95% CI=1.08, 1.52, p<0.001) and low birth weight (OR=1.43, 95% CI=1.09, 1.90, p<0.05). This study provides a first examination of the pregnancy outcomes among women with hearing loss in the U.S. This analysis demonstrates significant disparities in birth outcomes between women with and without hearing loss. Understanding and addressing the causes of these disparities is critical to improving pregnancy outcomes among women with hearing loss. Copyright © 2016 American Journal of Preventive Medicine. Published by Elsevier Inc. All rights reserved.

TMTC2 variant associated with sensorineural hearing loss and auditory neuropathy spectrum disorder in a family dyad.

PubMed

Guillen-Ahlers, Hector; Erbe, Christy B; Chevalier, Frédéric D; Montoya, Maria J; Zimmerman, Kip D; Langefeld, Carl D; Olivier, Michael; Runge, Christina L

2018-04-19

Sensorineural hearing loss (SNHL) is a common form of hearing loss that can be inherited or triggered by environmental insults; auditory neuropathy spectrum disorder (ANSD) is a SNHL subtype with unique diagnostic criteria. The genetic factors associated with these impairments are vast and diverse, but causal genetic factors are rarely characterized. A family dyad, both cochlear implant recipients, presented with a hearing history of bilateral, progressive SNHL, and ANSD. Whole-exome sequencing was performed to identify coding sequence variants shared by both family members, and screened against genes relevant to hearing loss and variants known to be associated with SNHL and ANSD. Both family members are successful cochlear implant users, demonstrating effective auditory nerve stimulation with their devices. Genetic analyses revealed a mutation (rs35725509) in the TMTC2 gene, which has been reported previously as a likely genetic cause of SNHL in another family of Northern European descent. This study represents the first confirmation of the rs35725509 variant in an independent family as a likely cause for the complex hearing loss phenotype (SNHL and ANSD) observed in this family dyad. © 2018 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc.

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

PubMed

Tikka, T; Mistry, N; Janjua, A

2016-03-01

Unilateral sudden sensorineural hearing loss due to an infarct in the vertebrobasilar system has been widely reported. Most patients have a background of traditional coronary risk factors related to these cerebrovascular episodes. A 32-year-old male, a regular user of anabolic steroids, presented to the emergency department with unilateral sensorineural hearing loss and symptoms suggestive of an infarct of the anterior inferior cerebellar artery but in the absence of risk factors for ischaemic stroke. Magnetic resonance imaging confirmed the presence of infarction in the region supplied by the anterior inferior cerebellar artery. Polycythaemia was found on haematological analysis, which we believe was secondary to the use of anabolic steroids. The patient was commenced on aspirin as per the stroke management protocol. There was resolution of neurological symptomatology six weeks after the episode, but no improvement in hearing. To our knowledge, this is the first case report of unilateral sensorineural hearing loss secondary to the use of anabolic steroids causing polycythaemia. This cause should be considered in the differential diagnosis of patients presenting with sensorineural hearing loss, especially in young males, when no other risk factors can be identified.

Enhancing Intrinsic Cochlear Stress Defenses to Reduce Noise-Induced Hearing Loss

DTIC Science & Technology

2002-09-01

trauma were wearing hearing protection when the hearing loss causing the accident took place. Poor fit of the protector will further degrade ...acetylcysteine and oral me- thionine in paracetamol poisoning. S Afr Med J 1986;60: 279. 133. Di Rocco A, Tagliati M, Danisi F, et al. A pilot study

British university students' attitudes towards noise-induced hearing loss caused by nightclub attendance.

PubMed

Johnson, O; Andrew, B; Walker, D; Morgan, S; Aldren, A

2014-01-01

Over the past 30 years, the prevalence of noise-induced hearing loss among adolescents and young adults has increased. This study aimed to address the current dearth of literature implicating excessive nightclub sound levels (more than 85 dB) as a direct cause of auditory symptoms related to noise-induced hearing loss. A questionnaire was completed by 325 students to gauge the frequency of auditory symptoms after nightclub attendance, and to explore knowledge and opinions about noise levels in nightclubs. The findings showed that 88.3 per cent of students experienced tinnitus after leaving a nightclub and 66.2 per cent suffered impaired hearing the following morning. In terms of behaviour, 73.2 per cent of students said that the risk of hearing damage would not affect their nightclub attendance, but most students (70.2 per cent) felt that noise levels in nightclubs should be limited to safe volumes. A high proportion of students reported experiencing symptoms related to noise-induced hearing loss after attending a nightclub. These findings are relevant to policy makers.

Transient Hearing Loss in Adults Associated With Zika Virus Infection.

PubMed

Vinhaes, Eriko S; Santos, Luciane A; Dias, Lislane; Andrade, Nilvano A; Bezerra, Victor H; de Carvalho, Anderson T; de Moraes, Laise; Henriques, Daniele F; Azar, Sasha R; Vasilakis, Nikos; Ko, Albert I; Andrade, Bruno B; Siqueira, Isadora C; Khouri, Ricardo; Boaventura, Viviane S

2017-03-01

In 2015, during the outbreak of Zika virus (ZIKV) in Brazil, we identified 3 cases of acute hearing loss after exanthematous illness. Serology yielded finding compatible with ZIKV as the cause of a confirmed (n = 1) and a probable (n = 2) flavivirus infection, indicating an association between ZIKV infection and transient hearing loss. © The Author 2016. Published by Oxford University Press for the Infectious Diseases Society of America.

Hearing and hearing loss: Causes, effects, and treatments

NASA Astrophysics Data System (ADS)

Schmiedt, Richard A.

2003-04-01

Hearing loss can have multiple causes. The outer and middle ears are conductive pathways for acoustic energy to the inner ear (cochlea) and help shape our spectral sensitivity. Conductive hearing loss is mechanical in nature such that the energy transfer to the cochlea is impeded, often from eardrum perforations or middle ear fluid buildup. Beyond the middle ear, the cochlea comprises three interdependent systems necessary for normal hearing. The first is that of basilar-membrane micromechanics including the outer hair cells. This system forms the basis of the cochlear amplifier and is the most vulnerable to noise and drug exposure. The second system comprises the ion pumps in the lateral wall tissues of the cochlea. These highly metabolic cells provide energy to the cochlear amplifier in the form of electrochemical potentials. This second system is particularly vulnerable to the effects of aging. The third system comprises the inner hair cells and their associated sensory nerve fibers. This system is the transduction stage, changing mechanical vibrations to nerve impulses. New treatments for hearing loss are on the horizon; however, at present the best strategy is avoidance of cochlear trauma and the proper use of hearing aids. [Work supported by NIA and MUSC.

A small molecule mitigates hearing loss in a mouse model of Usher syndrome III.

PubMed

Alagramam, Kumar N; Gopal, Suhasini R; Geng, Ruishuang; Chen, Daniel H-C; Nemet, Ina; Lee, Richard; Tian, Guilian; Miyagi, Masaru; Malagu, Karine F; Lock, Christopher J; Esmieu, William R K; Owens, Andrew P; Lindsay, Nicola A; Ouwehand, Krista; Albertus, Faywell; Fischer, David F; Bürli, Roland W; MacLeod, Angus M; Harte, William E; Palczewski, Krzysztof; Imanishi, Yoshikazu

2016-06-01

Usher syndrome type III (USH3), characterized by progressive deafness, variable balance disorder and blindness, is caused by destabilizing mutations in the gene encoding the clarin-1 (CLRN1) protein. Here we report a new strategy to mitigate hearing loss associated with a common USH3 mutation CLRN1(N48K) that involves cell-based high-throughput screening of small molecules capable of stabilizing CLRN1(N48K), followed by a secondary screening to eliminate general proteasome inhibitors, and finally an iterative process to optimize structure-activity relationships. This resulted in the identification of BioFocus 844 (BF844). To test the efficacy of BF844, we developed a mouse model that mimicked the progressive hearing loss associated with USH3. BF844 effectively attenuated progressive hearing loss and prevented deafness in this model. Because the CLRN1(N48K) mutation causes both hearing and vision loss, BF844 could in principle prevent both sensory deficiencies in patients with USH3. Moreover, the strategy described here could help identify drugs for other protein-destabilizing monogenic disorders.

A Qualitative Study on Knowledge and Attitude towards Risk Factors, Early Identification and Intervention of Infant Hearing Loss among Puerperal Mothers- A Short Survey

PubMed Central

Dudda, Ravi; Muniyappa, Hanumanth Prasad; Lakshmi, M.S

2017-01-01

Introduction Maternal active participation and their support are critical for the success of early hearing loss detection program. Erroneous maternal decisions may have large life long consequences on the infant’s life. The mothers’ knowledge and their attitudes towards infant hearing loss is the basis for their decisions. Aim The present study was done to determine the mothers’ knowledge and their attitude towards risk factors of infant hearing loss, its early identification and intervention and also awareness of effect of consanguinity on hearing loss. Materials and Methods In this cross-sectional questionnaire survey study, a total of 100 mothers were interviewed using the questionnaire which consisted of three sections namely risk factors, early identification and early intervention of hearing loss. Chi-square test was used to establish relationship between consanguineous and non-consanguineous mother’s responses to its effect on hearing loss. A p-value < 0.05 was considered as significant. Results Mothers’ awareness was significantly high for visible causes (ear pain/discharge, head injury and slap to ear) of hearing loss. Positive attitude was seen for importance of screening programs and follow up testing. Moderate level of awareness was found on hazards of consanguinity and benefits of early identification. However, mothers were least aware of neonatal jaundice, NICU admission (>5 days), signs of late-onset and neural hearing loss, management of hearing loss, hearing aid fitting and therapy necessity, which might interfere in early detection and intervention of hearing loss. Conclusion It is crucial to educate mothers on few risk factors and management of hearing loss to reduce its consequences. PMID:28892940

Otologic manifestations of Larsen syndrome.

PubMed

Marques, Letícia Helena de Sousa; Martins, Daniela Vieira; Juares, Gabriel Liria; Lorenzetti, Fábio Tadeu Moura; Monsanto, Rafael da Costa

2017-10-01

To describe and discuss otologic manifestations of Larsen syndrome, based on a case report and a systematic review of the literature. We performed a PubMED database search, and we selected studies reporting otolaryngologic manifestations secondary to Larsen syndrome. The selected articles were read in full, and three researchers independently extracted data from the studies. In parallel, we report the case of a 14-year-old patient who had hearing loss secondary to Larsen syndrome. Fifteen studies met our selection criteria. Seven studies reported hearing loss in patients with Larsen syndrome (4 had conductive hearing loss and 3 had mixed hearing loss). The conductive hearing loss may be secondary to ossicular malformations and/or middle ear effusions. Other causes for conductive hearing loss are mesenchymal remnants in the middle ear, Eustachian tube dysfunction, and cleft palate. Surgical management of the hearing loss is possible in selected cases, although the surgical and anesthetic risks should be considered. Hearing aids seem to be safe and effective treatment options for the hearing loss secondary to Larsen syndrome. Although rare, patients with Larsen syndrome may have hearing loss. The most frequent type of deficit is conductive, either secondary to malformation of the ossicles or middle ear effusion. Possible surgical correction of these abnormalities should be weighed against the anesthetic risks of these patients. Copyright © 2017 Elsevier B.V. All rights reserved.

Three-Category Classification of Magnetic Resonance Hearing Loss Images Based on Deep Autoencoder.

PubMed

Jia, Wenjuan; Yang, Ming; Wang, Shui-Hua

2017-09-11

Hearing loss, a partial or total inability to hear, is known as hearing impairment. Untreated hearing loss can have a bad effect on normal social communication, and it can cause psychological problems in patients. Therefore, we design a three-category classification system to detect the specific category of hearing loss, which is beneficial to be treated in time for patients. Before the training and test stages, we use the technology of data augmentation to produce a balanced dataset. Then we use deep autoencoder neural network to classify the magnetic resonance brain images. In the stage of deep autoencoder, we use stacked sparse autoencoder to generate visual features, and softmax layer to classify the different brain images into three categories of hearing loss. Our method can obtain good experimental results. The overall accuracy of our method is 99.5%, and the time consuming is 0.078 s per brain image. Our proposed method based on stacked sparse autoencoder works well in classification of hearing loss images. The overall accuracy of our method is 4% higher than the best of state-of-the-art approaches.

Unique autosomal recessive variant of palmoplantar keratoderma associated with hearing loss not caused by known mutations*

PubMed Central

Hegazi, Moustafa Abdelaal; Manou, Sommen; Sakr, Hazem; Camp, Guy Van

2017-01-01

Inherited Palmoplantar Keratodermas are rare disorders of genodermatosis that are conventionally regarded as autosomal dominant in inheritance with extensive clinical and genetic heterogeneity. This is the first report of a unique autosomal recessive Inherited Palmoplantar keratoderma - sensorineural hearing loss syndrome which has not been reported before in 3 siblings of a large consanguineous family. The patients presented unique clinical features that were different from other known Inherited Palmoplantar Keratodermas - hearing loss syndromes. Mutations in GJB2 or GJB6 and the mitochondrial A7445G mutation, known to be the major causes of diverse Inherited Palmoplantar Keratodermas -hearing loss syndromes were not detected by Sanger sequencing. Moreover, the pathogenic mutation could not be identified using whole exome sequencing. Other known Inherited Palmoplantar keratoderma syndromes were excluded based on both clinical criteria and genetic analysis. PMID:29267478

Cochlear synaptopathy in acquired sensorineural hearing loss: Manifestations and mechanisms.

PubMed

Liberman, M Charles; Kujawa, Sharon G

2017-06-01

Common causes of hearing loss in humans - exposure to loud noise or ototoxic drugs and aging - often damage sensory hair cells, reflected as elevated thresholds on the clinical audiogram. Recent studies in animal models suggest, however, that well before this overt hearing loss can be seen, a more insidious, but likely more common, process is taking place that permanently interrupts synaptic communication between sensory inner hair cells and subsets of cochlear nerve fibers. The silencing of affected neurons alters auditory information processing, whether accompanied by threshold elevations or not, and is a likely contributor to a variety of perceptual abnormalities, including speech-in-noise difficulties, tinnitus and hyperacusis. Work described here will review structural and functional manifestations of this cochlear synaptopathy and will consider possible mechanisms underlying its appearance and progression in ears with and without traditional 'hearing loss' arising from several common causes in humans. Copyright © 2017 Elsevier B.V. All rights reserved.

How Loud Is Too Loud?

MedlinePlus

... turn Javascript on. Protect Your Hearing Know which noises can cause damage. Wear earplugs when you are ... to 12 about the causes and prevention of noise-induced hearing loss. 110 Decibels Regular exposure of ...

The effect of noise-induced hearing loss on the intelligibility of speech in noise

NASA Astrophysics Data System (ADS)

Smoorenburg, G. F.; Delaat, J. A. P. M.; Plomp, R.

1981-06-01

Speech reception thresholds, both in quiet and in noise, and tone audiograms were measured for 14 normal ears (7 subjects) and 44 ears (22 subjects) with noise-induced hearing loss. Maximum hearing loss in the 4-6 kHz region equalled 40 to 90 dB (losses exceeded by 90% and 10%, respectively). Hearing loss for speech in quiet measured with respect to the median speech reception threshold for normal ears ranged from 1.8 dB to 13.4 dB. For speech in noise the numbers are 1.2 dB to 7.0 dB which means that the subjects with noise-induced hearing loss need a 1.2 to 7.0 dB higher signal-to-noise ratio than normal to understand sentences equally well. A hearing loss for speech of 1 dB corresponds to a decrease in sentence intelligibility of 15 to 20%. The relation between hearing handicap conceived as a reduced ability to understand speech and tone audiogram is discussed. The higher signal-to-noise ratio needed by people with noise-induced hearing loss to understand speech in noisy environments is shown to be due partly to the decreased bandwidth of their hearing caused by the noise dip.

Prosthetic management of posttraumatic external auditory canal atresia: A rare cause of conductive hearing loss

PubMed Central

Adhershitha, A. R.; Anilkumar, S.; Rajesh, C.; Mohan, Deepak C.

2016-01-01

Acquired external auditory canal (EAC) atresia is an infrequent entity which can originate from a number of different causes including trauma, infection, neoplasia, inflammation, and radiotherapy. Posttraumatic atresias are exceptionally rare, only 10% of atresias are attributed to trauma in most of the series. The management of stenosis of the EAC is challenging as it is associated with residual hearing loss and late recurrence. Traditional stents often occlude the EAC, resulting in a temporary conductive hearing loss. This case report describes the technique of fabrication of a wide-bored acrylic stent which attained additional retention from the folds of the auricle. The customized earmold stent effectively prevented restenosis, while the large bore provided ventilation and improved hearing subjectively during the stenting period. PMID:27746605

Smartphone-Based Hearing Screening in Noisy Environments

PubMed Central

Na, Youngmin; Joo, Hyo Sung; Yang, Hyejin; Kang, Soojin; Hong, Sung Hwa; Woo, Jihwan

2014-01-01

It is important and recommended to detect hearing loss as soon as possible. If it is found early, proper treatment may help improve hearing and reduce the negative consequences of hearing loss. In this study, we developed smartphone-based hearing screening methods that can ubiquitously test hearing. However, environmental noise generally results in the loss of ear sensitivity, which causes a hearing threshold shift (HTS). To overcome this limitation in the hearing screening location, we developed a correction algorithm to reduce the HTS effect. A built-in microphone and headphone were calibrated to provide the standard units of measure. The HTSs in the presence of either white or babble noise were systematically investigated to determine the mean HTS as a function of noise level. When the hearing screening application runs, the smartphone automatically measures the environmental noise and provides the HTS value to correct the hearing threshold. A comparison to pure tone audiometry shows that this hearing screening method in the presence of noise could closely estimate the hearing threshold. We expect that the proposed ubiquitous hearing test method could be used as a simple hearing screening tool and could alert the user if they suffer from hearing loss. PMID:24926692

[Effects on hearing due to the occupational noise exposure of marble industry workers in the Federal District, Brazil].

PubMed

Harger, Marília Rabelo Holanda Camarano; Barbosa-Branco, Anadergh

2004-01-01

To evaluate the prevalence of hearing loss, its degree and type, among workers in the marble industry in the Brazilian Federal District (FD). Workers from eight marble industries in the FD were evaluated by means of a cross sectional epidemiological study. An audiometry screening test (air conduction) was performed. Workers with hearing loss were submitted to liminal tonal audiometry air & bone conduction and speech audiometry tests using an audiometer AD-28 (Interacoustics). All subjects studied were submitted to a visual inspection of the external acoustic meatus. One hundred and fifty two workers were examined; mean age was 32 years (SD = 8.6); average occupational noise exposure was of 8.3 years (SD = 6.8). Audiometries demonstrated that 48.0% (n = 73) had some type of hearing loss. Among the workers with hearing loss, 50.0% had results compatible with noise-induced hearing loss (NIHL); 41.0% with incipient noise induced hearing loss, 5.0% with sensorineural hearing loss (all except NIHL) and 4.0% with conductive and mixed hearing losses. Among workers with NIHL, 57.1% had bilateral involvement, 17.1% in the right ear and 25.7% in the left ear. Among those with incipient NIHL, 13.9% were bilateral, 19.4% were only in the right ear and 66.7% were only in the left ear. Abnormal audiograms were found in 48.0% of the sample. Among those with hearing loss, the predominant cause was NIHL, followed by those classified as having incipient noise induced hearing loss. Hearing loss usually started at 6 kHz, frequently in the left ear.

The Relationship between the Behavioral Hearing Thresholds and Maximum Bilirubin Levels at Birth in Children with a History of Neonatal Hyperbilirubinemia

PubMed Central

Panahi, Rasool; Jafari, Zahra; Sheibanizade, Abdoreza; Salehi, Masoud; Esteghamati, Abdoreza; Hasani, Sara

2013-01-01

Introduction: Neonatal hyperbilirubinemia is one of the most important factors affecting the auditory system and can cause sensorineural hearing loss. This study investigated the relationship between behavioral hearing thresholds in children with a history of jaundice and the maximum level of bilirubin concentration in the blood. Materials and Methods: This study was performed on 18 children with a mean age of 5.6 years and with a history of neonatal hyperbilirubinemia. Behavioral hearing thresholds, transient evoked emissions and brainstem evoked responses were evaluated in all children. Results: Six children (33.3%) had normal hearing thresholds and the remaining (66.7%) had some degree of hearing loss. There was no significant relationship (r=-0.28, P=0.09) between the mean total bilirubin levels and behavioral hearing thresholds in all samples. A transient evoked emission was seen only in children with normal hearing thresholds however in eight cases brainstem evoked responses had not detected. Conclusion: Increased blood levels of bilirubin at the neonatal period were potentially one of the causes of hearing loss. There was a lack of a direct relationship between neonatal bilirubin levels and the average hearing thresholds which emphasizes on the necessity of monitoring the various amounts of bilirubin levels. PMID:24303432

[Sensorineural hearing loss due to neonatal hyperbilirubinemia].

PubMed

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

2003-01-01

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

Overview of Usher's Syndrome: Congenital Deafness and Progressive Loss of Vision

ERIC Educational Resources Information Center

Vernon, McCay

1974-01-01

Usher's syndrome, a genetic condition causing congenital profound hearing loss and a progressive blindness due to retinitis pigmentosa, affects an estimated three to six percent of children in educational and rehabilitative programs for the hearing impaired. (Author)

Hearing loss in the royal Norwegian Navy: a cross-sectional study.

PubMed

Irgens-Hansen, Kaja; Sunde, Erlend; Bråtveit, Magne; Baste, Valborg; Oftedal, Gunnhild; Koefoed, Vilhelm; Lind, Ola; Moen, Bente Elisabeth

2015-07-01

Prior studies have indicated a high prevalence of noise-induced hearing loss (NIHL) among Navy personnel; however, it is not clear whether this is caused by work on board. The present study aimed to assess the prevalence of hearing loss among Navy personnel in the Royal Norwegian Navy (RNoN), and to investigate whether there is an association between work on board RNoN vessels and occurrence of hearing loss. Navy personnel currently working on board RNoN vessels were recruited to complete a questionnaire on noise exposure and health followed by pure tone audiometry. Hearing loss was defined as hearing threshold levels ≥25 dB in either ear at the frequencies 3,000, 4,000 or 6,000 Hz. Hearing thresholds were adjusted for age and gender using ISO 7029. The prevalence of hearing loss among Navy personnel was 31.4 %. The work exposure variables: years of work in the Navy, years on vessel(s) in the Navy and years of sailing in the Navy were associated with reduced hearing after adjusting for age, gender and otitis as an adult. Among the work exposure variables, years of sailing in the Navy was the strongest predictor of reduced hearing, and significantly reduced hearing was found at the frequencies 1,000, 3,000 and 4,000 Hz. Our results indicate that time spent on board vessels in the RNoN is a predictor of reduced hearing.

Hearing in young adults. Part I: The effects of attitudes and beliefs toward noise, hearing loss, and hearing protector devices.

PubMed

Keppler, Hannah; Dhooge, Ingeborg; Vinck, Bart

2015-01-01

There is great concern regarding the development of noise-induced hearing loss (NIHL) in youth caused by high sound levels during various leisure activities. Health-orientated behavior of young adults might be linked to the beliefs and attitudes toward noise, hearing loss, and hearing protector devices (HPDs). The objective of the current study was to evaluate the effects of attitudes and beliefs toward noise, hearing loss, and HPDs on young adults' hearing status. A questionnaire and an audiological test battery were completed by 163 subjects (aged 18-30 years). The questionnaire contained the Youth Attitude to Noise Scale (YANS) and Beliefs about Hearing Protection and Hearing Loss (BAHPHL). A more positive attitude or belief represented an attitude where noise or hearing loss is seen as unproblematic and attitudes and beliefs regarding HPDs is worse. Hearing was evaluated using (high frequency) pure tone audiometry (PTA), transient evoked and distortion product otoacoustic emissions. First, mean differences in hearing between the groups with different attitudes and beliefs were evaluated using one-way analysis of variance (ANOVA). Second, a χ² test was used to examine the usage of HPDs by the different groups with different attitudes and beliefs. Young adults with a positive attitude had significantly more deteriorated hearing and used HPDs less than the other subjects. Hearing conservation programs (HCPs) for young adults should provide information and knowledge regarding noise, hearing loss, and HPDs. Barriers wearing HPDs should especially be discussed. Further, those campaigns should focus on self-experienced hearing related symptoms that might serve as triggers for attitudinal and behavioral changes.

Hearing in young adults. Part I: The effects of attitudes and beliefs toward noise, hearing loss, and hearing protector devices

PubMed Central

Keppler, Hannah; Dhooge, Ingeborg; Vinck, Bart

2015-01-01

There is great concern regarding the development of noise-induced hearing loss (NIHL) in youth caused by high sound levels during various leisure activities. Health-orientated behavior of young adults might be linked to the beliefs and attitudes toward noise, hearing loss, and hearing protector devices (HPDs). The objective of the current study was to evaluate the effects of attitudes and beliefs toward noise, hearing loss, and HPDs on young adults’ hearing status. A questionnaire and an audiological test battery were completed by 163 subjects (aged 18-30 years). The questionnaire contained the Youth Attitude to Noise Scale (YANS) and Beliefs about Hearing Protection and Hearing Loss (BAHPHL). A more positive attitude or belief represented an attitude where noise or hearing loss is seen as unproblematic and attitudes and beliefs regarding HPDs is worse. Hearing was evaluated using (high frequency) pure tone audiometry (PTA), transient evoked and distortion product otoacoustic emissions. First, mean differences in hearing between the groups with different attitudes and beliefs were evaluated using one-way analysis of variance (ANOVA). Second, a χ2 test was used to examine the usage of HPDs by the different groups with different attitudes and beliefs. Young adults with a positive attitude had significantly more deteriorated hearing and used HPDs less than the other subjects. Hearing conservation programs (HCPs) for young adults should provide information and knowledge regarding noise, hearing loss, and HPDs. Barriers wearing HPDs should especially be discussed. Further, those campaigns should focus on self-experienced hearing related symptoms that might serve as triggers for attitudinal and behavioral changes. PMID:26356365

Endocrine Glands and Hearing: Auditory Manifestations of Various Endocrine and Metabolic Conditions

PubMed Central

Cherian, Kripa Elizabeth; Kapoor, Nitin; Mathews, Suma Susan; Paul, Thomas Vizhalil

2017-01-01

The aetiology of hearing loss in humans is multifactorial. Besides genetic, environmental and infectious causes, several endocrine and metabolic abnormalities are associated with varying degrees of hearing impairment. The pattern of hearing loss may be conductive, sensori-neural or mixed. The neurophysiology of hearing as well as the anatomical structure of the auditory system may be influenced by changes in the hormonal and metabolic milieu. Optimal management of these conditions requires the integrated efforts of the otolaryngologist and the endocrinologist. The presence of hearing loss especially in the young age group should prompt the clinician to explore the possibility of an associated endocrine or metabolic disorder for timely referral and early initiation of treatment. PMID:28553606

[Acoustic trauma generated by exposure to gun powder].

PubMed

Arch-Tirado, Emilio; Garnica-Escamilla, Marco Antonio; Delgado-Hernández, Alhelí; Campos-Muñoz, Teodora; Rodríguez-Rodríguez, Lourdes; Verduzco-Mendoza, Antonio

2014-01-01

Noise-induced hearing loss has increased due to factors such as industrialization. It is estimated that one third of the world's population suffers from some degree of hearing loss caused by exposure to high-intensity noise. Exposure to noise can cause disease of various ear structures, especially destruction of outer hair cells, causing varying degrees of hearing lossObjective: To describe the audiological findings in a group of subjects who were exposed to a source of fireworks explosion in the state of Tlaxcala Mexico. We carried out an audiometric study in eight patients admitted to CENIAQ-INR who were exposed to a firecracker explosion. In each subject, airway with conventional pure tone audiometry from 125 to 8000 Hz was assessed using a clinical audiometer (model 622, Minimate, Madsen) Clinical case: Hearing loss at all frequencies was found in all eight patients, from mild to severe in both ears. Audiogram showed decreases from 4000 Hz, diagnosing third-degree acoustic trauma in all patients. The main symptom was reported by patients with tinnitus. Other scenarios in which patients report the presence of tinnitus are acoustic trauma due to use of audio players in symphony orchestra musicians and detonating military firearms. Audiometric assessment is recommended after an explosive accident as an accurate method to determine if there is any hearing impairment.

Common founder effects of hereditary hemochromatosis, Wilson´s disease, the long QT syndrome and autosomal recessive deafness caused by two novel mutations in the WHRN and TMC1 genes.

PubMed

Olsson, K Sigvard; Wålinder, Olof; Jansson, Ulf; Wilbe, Maria; Bondeson, Marie-Louise; Stattin, Eva-Lena; Raha-Chowdhury, Ruma; Williams, Roger

2017-01-01

Genealogy and molecular genetic studies of a Swedish river valley population resulted in a large pedigree, showing that the hereditary hemochromatosis (HH) HFE/p. C282Y mutation is inherited with other recessive disorders such as Wilson´s disease (WND), a rare recessive disorder of copper overload. The population also contain individuals with the Swedish long QT syndrome (LQTS1) founder mutation ( KCNQ1 /p.Y111C) which in homozygotes causes the Jervell & Lange Nielsen syndrome (JLNS) and hearing loss (HL).Aims of the study were to test whether the Swedish long QT founder mutation originated in an ancestral HFE family and if carriers had an increased risk for hemochromatosis (HH), a treatable disorder. We also aimed to identify the pathogenic mutation causing the hearing loss disorder segregating in the pedigree. LQTS patients were asked about their ancestry and possible origin in a HH family. They were also offered a predictive testing for the HFE genotype. Church books were screened for families with hearing loss. One HH family had two members with hearing loss, who underwent molecular genetic analysis of the LQTS founder mutation, connexin 26 and thereafter exome sequencing. Another family with hearing loss in repeat generations was also analyzed for connexin 26 and underwent exome sequencing. Of nine LQTS patients studied, four carried a HFE mutation (two p.C282Y, two p.H63D), none was homozygous. Three LQTS patients confirmed origin in a female founder ( b 1694, identical to AJ b 1694, a HFE pedigree member from the Fax river. Her descent of 44 HH families, included also 29 families with hearing loss (HL) suggesting JLNS. Eleven LQTS probands confirmed origin in a second founder couple (b 1614/1605) in which the woman b 1605 was identical to a HFE pedigree member from the Fjällsjö river. In her descent there were not only 64 HH, six WND families, one JLNS, but also 48 hearing loss families. Most hearing loss was non syndromic and caused by founder effects of the late 16 th century. One was of Swedish origin carrying the WHRN, c.1977delC, (p.S660Afs*30) mutation, the other was a TMC1 (NM_138691),c.1814T>C,(p.L605P) mutation, possibly of Finnish origin. Deep human HFE genealogies show HFE to be associated with other genetic disorders like Wilson´s disease, LQTS, JLNS, and autosomal recessive hearing loss. Two new homozygous HL mutations in WHRN /p.S660Afs*30 and TMC1/ p.L605P were identified,none of them previously reported from Scandinavia. The rarity of JLNS was possibly caused by miscarriage or intrauterine death. Most hearing loss (81.7%) was seen after 1844 when first cousin marriages were permitted. However, only 10 (10.3%) came from 1 st cousin unions and only 2 (2.0 %) was born out of wedlock.

Noise-induced hearing loss caused by gunshot in South Korean military service.

PubMed

Moon, In Seok

2007-04-01

Noise-induced hearing loss is a preventive disease and yet the effective treatment modality has not been established. Acute acoustic traumas caused by an exposure to gunshot noise are common in young South Korean males in military service. Considering the significant lack of awareness on this serious issue as well as the absence of proper protective gear, an in-depth analysis is desperately needed. All 3650 soldiers performed regular periodic gunfire exercise without any hearing protective measures. Seven patients with hearing impairment after periodic gunfire visited the aeromedical squadron; all were right-handed males. Six were tested with the K-2 rifle and one was tested with a K-5 revolver. History taking, physical examination, pure-tone audiometry, and impedance audiometry were conducted. In the next periodic gunfire exercise, all 3650 soldiers performed gunfire with unilateral hearing protection. The average outcome of postexposure air conduction thresholds was 6.5 dB in the right ear and 33.1 dB in the left ear. After medical treatment, hearing impairment was much improved; however, tinnitus was not diminished. In the next periodic gunfire exercise, the result of a supplement of unilateral earplug protection proved its effectiveness on acoustic trauma caused by gunfire noise. Asymmetry in hearing loss is related to the position of the head during gunfire. A unilateral hearing protection device was enough to protect hearing from gunfire noise. At the same time, it can effectively prevent a potential firearm accident that can be caused by trainees mishearing the instruction of a firearm instructor if both earplugs are worn. Thus, providing a unilateral earplug for protection against acoustic trauma must be taken into serious consideration.

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

PubMed

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

2016-09-02

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

Parental knowledge and attitudes to childhood hearing loss and hearing services in the Solomon Islands.

PubMed

Kaspar, Annette; Newton, Obiga; Kei, Joseph; Driscoll, Carlie; Swanepoel, De Wet; Goulios, Helen

2017-12-01

An understanding of parental knowledge and attitudes towards childhood hearing loss is essential to the successful implementation of audiology services. The present study aimed to investigate parental knowledge and attitudes among parents in the Solomon Islands. A total of 100 mothers and 50 fathers were administered a questionnaire via semi-structured interviews. Highest parental awareness of aetiology of childhood hearing loss was noted for otitis media (94%), noise exposure (87.3%), and family history (72.7%). The highest parental awareness concerning public health initiatives to reduce/prevent otitis media was noted for routine childhood immunizations (84%) and breast-feeding (76%). Higher rates of knowledge in fathers than in mothers included otitis media (p = 0.038), noise exposure (p = 0.007), and breast-feeding (p = 0.031). Approximately half of parents (56%) agreed that curses may cause hearing loss. Overall parental responses showed positive support for infant hearing screening programs (96%) and school-based ear and hearing health examinations (99.3%). High levels of parental readiness and support for childhood hearing services in the Solomon Islands was evident. Knowledge of aetiology of childhood hearing loss was highest for otitis media, noise exposure, and family history. Knowledge and attitudes of fathers to childhood hearing loss and hearing services was either the same or better than that of mothers. Copyright © 2017 Elsevier B.V. All rights reserved.

‘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

Aging and Hearing Health: The Life-course Approach.

PubMed

Davis, Adrian; McMahon, Catherine M; Pichora-Fuller, Kathleen M; Russ, Shirley; Lin, Frank; Olusanya, Bolajoko O; Chadha, Shelly; Tremblay, Kelly L

2016-04-01

Sensory abilities decline with age. More than 5% of the world's population, approximately 360 million people, have disabling hearing loss. In adults, disabling hearing loss is defined by thresholds greater than 40 dBHL in the better hearing ear.Hearing disability is an important issue in geriatric medicine because it is associated with numerous health issues, including accelerated cognitive decline, depression, increased risk of dementia, poorer balance, falls, hospitalizations, and early mortality. There are also social implications, such as reduced communication function, social isolation, loss of autonomy, impaired driving ability, and financial decline. Furthermore, the onset of hearing loss is gradual and subtle, first affecting the detection of high-pitched sounds and with difficulty understanding speech in noisy but not in quiet environments. Consequently, delays in recognizing and seeking help for hearing difficulties are common. Age-related hearing loss has no known cure, and technologies (hearing aids, cochlear implants, and assistive devices) improve thresholds but do not restore hearing to normal. Therefore, health care for persons with hearing loss and people within their communication circles requires education and counseling (e.g., increasing knowledge, changing attitudes, and reducing stigma), behavior change (e.g., adapting communication strategies), and environmental modifications (e.g., reducing noise). In this article, we consider the causes, consequences, and magnitude of hearing loss from a life-course perspective. We examine the concept of "hearing health," how to achieve it, and implications for policy and practice. © The Author 2016. Published by Oxford University Press on behalf of The Gerontological Society of America. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Organic solvent exposure and hearing loss in a cohort of aluminium workers.

PubMed

Rabinowitz, P M; Galusha, D; Slade, M D; Dixon-Ernst, C; O'Neill, A; Fiellin, M; Cullen, M R

2008-04-01

Organic solvent exposure has been shown to cause hearing loss in animals and humans. Less is known about the risk of hearing loss due to solvent exposures typically found in US industry. The authors performed a retrospective cohort study to examine the relationship between solvent exposure and hearing loss in US aluminium industry workers. A cohort of 1319 workers aged 35 years or less at inception was followed for 5 years. Linkage of employment, industrial hygiene and audiometric surveillance records allowed for estimation of noise and solvent exposures and hearing loss rates over the study period. Study subjects were classified as "solvent exposed" or not, on the basis of industrial hygiene records linked with individual job histories. High frequency hearing loss was modelled as both a continuous and a dichotomous outcome. Typical solvent exposures involved mixtures of xylene, toluene and/or methyl ethyl ketone (MEK). Recorded solvent exposure levels varied widely both within and between jobs. In a multivariate logistic model, risk factors for high frequency hearing loss included age (OR = 1.06, p = 0.004), hunting or shooting (OR = 1.35, p = 0.049), noisy hobbies (OR = 1.74, p = 0.01), baseline hearing level (OR = 1.04, p<0.001) and solvent exposure (OR = 1.87, p = 0.004). A multivariate linear regression analysis similarly found significant associations between high frequency hearing loss and age (p<0.001), hunting or shooting (p<0.001), noisy hobbies (p = 0.03), solvent exposure (p<0.001) and baseline hearing (p = 0.03). These results suggest that occupational exposure to organic solvent mixtures is a risk factor for high frequency hearing loss, although the data do not allow conclusions about dose-response relationships. Industries with solvent-exposed workers should include such workers in hearing conservation programs.

Hearing loss and the central auditory system: Implications for hearing aids

NASA Astrophysics Data System (ADS)

Frisina, Robert D.

2003-04-01

Hearing loss can result from disorders or damage to the ear (peripheral auditory system) or the brain (central auditory system). Here, the basic structure and function of the central auditory system will be highlighted as relevant to cases of permanent hearing loss where assistive devices (hearing aids) are called for. The parts of the brain used for hearing are altered in two basic ways in instances of hearing loss: (1) Damage to the ear can reduce the number and nature of input channels that the brainstem receives from the ear, causing plasticity of the central auditory system. This plasticity may partially compensate for the peripheral loss, or add new abnormalities such as distorted speech processing or tinnitus. (2) In some situations, damage to the brain can occur independently of the ear, as may occur in cases of head trauma, tumors or aging. Implications of deficits to the central auditory system for speech perception in noise, hearing aid use and future innovative circuit designs will be provided to set the stage for subsequent presentations in this special educational session. [Work supported by NIA-NIH Grant P01 AG09524 and the International Center for Hearing & Speech Research, Rochester, NY.

Attention to Language in Day Care Attending Children: A Mediating Factor in the Developmental Effects of Otitis Media.

ERIC Educational Resources Information Center

Feagans, Lynne V.; And Others

Three ways in which otitis media may affect development of hearing are explored. First, developmental effects may be due to illness in general; second, otitis media may cause fluctuating hearing loss which may lead to deficits in language in many areas; and, third, fluctuating hearing loss may have only a temporary effect on the acquisition of…

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

PubMed

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

2017-01-01

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

Autophagy is essential for hearing in mice.

PubMed

Fujimoto, Chisato; Iwasaki, Shinichi; Urata, Shinji; Morishita, Hideaki; Sakamaki, Yuriko; Fujioka, Masato; Kondo, Kenji; Mizushima, Noboru; Yamasoba, Tatsuya

2017-05-11

Hearing loss is the most frequent sensory disorder in humans. Auditory hair cells (HCs) are postmitotic at late-embryonic differentiation and postnatal stages, and their damage is the major cause of hearing loss. There is no measurable HC regeneration in the mammalian cochlea, and the maintenance of cell function is crucial for preservation of hearing. Here we generated mice deficient in autophagy-related 5 (Atg5), a gene essential for autophagy, in the HCs to investigate the effect of basal autophagy on hearing acuity. Deletion of Atg5 resulted in HC degeneration and profound congenital hearing loss. In autophagy-deficient HCs, polyubiquitinated proteins and p62/SQSTM1, an autophagy substrate, accumulated as inclusion bodies during the first postnatal week, and these aggregates increased in number. These findings revealed that basal autophagy has an important role in maintenance of HC morphology and hearing acuity.

Does the accuracy of blood pressure measurement correlate with hearing loss of the observer?

PubMed

Song, Soohwa; Lee, Jongshill; Chee, Youngjoon; Jang, Dong Pyo; Kim, In Young

2014-02-01

The auscultatory method is influenced by the hearing level of the observers. If the observer has hearing loss, it is possible to measure blood pressure inaccurately by misreading the Korotkoff sounds at systolic blood pressure (SBP) and diastolic blood pressure (DBP). Because of the potential clinical problems this discrepancy may cause, we used a hearing loss simulator to determine how hearing level affects the accuracy of blood pressure measurements. Two data sets (data set A, 32 Korotkoff sound video clips recorded by the British Hypertension Society; data set B, 28 Korotkoff sound data acquired from the Korotkoff sound recording system developed by Hanyang University) were used and all the data were attenuated to simulate a hearing loss of 5, 10, 15, 20, and 25 dB using the hearing loss simulator. Five observers with normal hearing assessed the blood pressures from these data sets and the differences between the values measured from the original recordings (no attenuation) and the attenuated versions were analyzed. Greater attenuation of the Korotkoff sounds, or greater hearing loss, resulted in larger blood pressure measurement differences when compared with the original data. When measuring blood pressure with hearing loss, the SBP tended to be underestimated and the DBP was overestimated. The mean differences between the original data and the 25 dB hearing loss data for the two data sets combined were 1.55±2.71 and -4.32±4.21 mmHg for SBP and DBP, respectively. This experiment showed that the accuracy of blood pressure measurements using the auscultatory method is affected by observer hearing level. Therefore, to reduce possible error using the auscultatory method, observers' hearing should be tested.

Consanguinity Among Parents of Iranian Deaf Children.

PubMed

Ajallouyan, Mohammad; Radfar, Shokofeh; Nouhi, Sima; Tavallaie, Seid Abbas; Amirsalari, Susan; Yousefi, Jaleh; Hasanali Fard, Mahdieh

2016-11-01

It seems that there is a relationship between consanguinity and profound hearing loss but there is little data about the association of consanguinity and hearing loss in Iran. The aim of this study is to demonstrate the causes of profound bilateral sensorineural hearing loss among Iranian samples who are candidates for cochlear implantation. This study was retrospective, analytical, and designed to collect information about profound hearing impaired cases referred to the Baqiyatallah Cochlear implantation center using enumeration. A total of 310 children with profound hearing impairments participated in this study. They were aged from 6 months to 4 years old. The study was done between January 2007 and April 2009. Chi-square tests were used to show whether there was any statistical difference between the incidence of marital consanguinity of their parents and the normal population. Sixty-five percent of those 310 children had parents who had married with their relatives. Of the 203 (65%) parents that had consanguineous marriages, 132 were first cousins, which includes the children of two brothers (37 [11.8%] patrilateral parallel cousins), the children of two sisters (38 [12.2%] multi-lateral parallel cousins), or the children of a brother and a sister (57 [18.3%] cross cousins). Fifty-four (17.4%) of the parents were second cousins and 17 (5.2%) were beyond second cousins. Also, hearing loss etiology was obvious in 237 (76.3%) of the patients with profound hearing loss but was unknown in 73 (23.7%). Hereditary was identified as the most common cause in 33% of the cases. Our data demonstrated a 65% occurrence of consanguineous marriage among the parents of deaf children, which is statistically different from the percentage of consanguineous marriage among Iranian population (38%). This indicates an obvious relationship between severe hearing loss and consanguineous marriage.

Phenotypic Heterogeneity in a DFNA20/26 family segregating a novel ACTG1 mutation.

PubMed

Yuan, Yongyi; Gao, Xue; Huang, Bangqing; Lu, Jingqiao; Wang, Guojian; Lin, Xi; Qu, Yan; Dai, Pu

2016-02-01

Genetic factors play an important role in hearing loss, contributing to approximately 60% of cases of congenital hearing loss. Autosomal dominant deafness accounts for approximately 20% of cases of hereditary hearing loss. Diseases with autosomal dominant inheritance often show pleiotropy, different degrees of penetrance, and variable expressivity. A three-generation Chinese family with autosomal dominant nonsyndromic hearing impairment (ADNSHI) was enrolled in this study. Audiometric data and blood samples were collected from the family. In total, 129 known human deafness genes were sequenced using next-generation sequencing (NGS) to identify the responsible gene mutation in the family. Whole Exome Sequencing (WES) was performed to exclude any other variant that cosegregated with the phenotype. The age of onset of the affected family members was the second decade of life. The condition began with high-frequency hearing impairment in all family members excluding III:2. The novel ACTG1 c.638A > G (p.K213R) mutation was found in all affected family members and was not found in the unaffected family members. A heterozygous c.638A > G mutation in ACTG1 and homozygous c.109G > A (p.V37I) mutation in GJB2 were found in III:2, who was born with hearing loss. The WES result concurred with that of targeted sequencing of known deafness genes. The novel mutation p.K213R in ACTG1 was found to be co-segregated with hearing loss and the genetic cause of ADNSHI in this family. A homozygous mutation associated with recessive inheritance only rarely co-acts with a dominant mutation to result in hearing loss in a dominant family. In such cases, the mutations in the two genes, as in ACTG1 and GJB2 in the present study, may result in a more severe phenotype. Targeted sequencing of known deafness genes is one of the best choices to identify the genetic cause in hereditary hearing loss families.

Childhood Otitis Media: A Cohort Study With 30-Year Follow-Up of Hearing (The HUNT Study).

PubMed

Aarhus, Lisa; Tambs, Kristian; Kvestad, Ellen; Engdahl, Bo

2015-01-01

To study the extent to which otitis media (OM) in childhood is associated with adult hearing thresholds. Furthermore, to study whether the effects of OM on adult hearing thresholds are moderated by age or noise exposure. Population-based cohort study of 32,786 participants who had their hearing tested by pure-tone audiometry in primary school and again at ages ranging from 20 to 56 years. Three thousand sixty-six children were diagnosed with hearing loss; the remaining sample had normal childhood hearing. Compared with participants with normal childhood hearing, those diagnosed with childhood hearing loss caused by otitis media with effusion (n = 1255), chronic suppurative otitis media (CSOM; n = 108), or hearing loss after recurrent acute otitis media (rAOM; n = 613) had significantly increased adult hearing thresholds in the whole frequency range (2 dB/17-20 dB/7-10 dB, respectively). The effects were adjusted for age, sex, and noise exposure. Children diagnosed with hearing loss after rAOM had somewhat improved hearing thresholds as adults. The effects of CSOM and hearing loss after rAOM on adult hearing thresholds were larger in participants tested in middle adulthood (ages 40 to 56 years) than in those tested in young adulthood (ages 20 to 40 years). Eardrum pathology added a marginally increased risk of adult hearing loss (1-3 dB) in children with otitis media with effusion or hearing loss after rAOM. The study could not reveal significant differences in the effect of self-reported noise exposure on adult hearing thresholds between the groups with OM and the group with normal childhood hearing. This cohort study indicates that CSOM and rAOM in childhood are associated with adult hearing loss, underlining the importance of optimal treatment in these conditions. It appears that ears with a subsequent hearing loss after OM in childhood age at a faster rate than those without; however this should be confirmed by studies with several follow-up tests through adulthood.

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. Copyright © 2016 Elsevier Inc. All rights reserved.

Quinine-induced hearing loss in the guinea pig is not affected by the Ca2+ channel antagonist verapamil.

PubMed

Jäger, W; Idrizbegovic, E; Karlsson, K K; Alván, G

1997-01-01

It is well documented that quinine induces reversible hearing loss and tinnitus. The purpose in this study was to induce a quinine hearing loss and to investigate if verapamil, a Ca2+ channel antagonist of L-type might affect the response. Pigmented guinea pigs (n = 24) were anaesthetized by atropine. Hypnorm and midazolam but permitting spontaneous respiration. An electrode of platinum was placed on the round window and short (10 msec) tone pulses at 8 kHz were presented to the external ear. A typical deflection of the N1-wave was determined as the hearing threshold. Quinine hydrochloride 40 mg/kg and verapamil 1 mg/kg were given intravenously. Quinine induced a significant and reversible hearing loss (mean 16 dB). This hearing loss was not at all affected by verapamil given before or after quinine. Verapamil often caused acute cardiac arrest and particularly the combination verapamil followed by quinine-induced death to the animal. We conclude that verapamil and quinine had no in vivo interaction with regard to the hearing ability.

[Analysis on occupational noise-induced hearing loss of different type workers in underground mining].

PubMed

Liu, Q C; Duo, C H; Wang, Z; Yan, K; Zhang, J; Xiong, W; Zhu, M

2017-11-20

Objective: To investigate hearing loss status of blasters, drillers mechanics and so on in underground mining, and put forward suggestion diagnosis of occupational explosive deafness and occupational deafness. Methods: Underground excavation workers in a metal mine were recruited in this study, those with a history of ear disease and non-occupational deafness were all excluded. Finally, the features of pure tone audiometry of 459 noise-exposed workers were analyzed. Results: High-frequency hearing loss occurred on 351workers and the positive detection rate was 74.29%, workers who had both high-frequency and linguistic frequency hearing loss were 51 and the positive detection rate was 11.11%. The positive detection of high-frequency hearing loss in right ear (χ(2)=9.427 and P = 0.024) and in left ear (χ(2)=14.375, P =0.002) was significantly different between different exposure age groups. The positive detection of high-frequency hearing loss of driving group was the highest, followed by blasting group, mining group and machine repair group. The characteristics of the hearing loss caused by drilling noise of the blasting workers with no accident occurred were in line with that of noise-induced hearing loss. Conclusion: The diagnosis grading should be carried out according to the diagnostic criteria of occupational noise-induced deafness for the employees who engaged in the blasting operation with no record of blast accident.

Recurrent variants in OTOF are significant contributors to prelingual nonsydromic hearing loss in Saudi patients

PubMed Central

Almontashiri, Naif A M; Alswaid, Abdulrahman; Oza, Andrea; Al-Mazrou, Khalid A; Elrehim, Omnia; Tayoun, Ahmad Abou; Rehm, Heidi L; Amr, Sami S

2018-01-01

Purpose Hearing loss is more prevalent in the Saudi Arabian population than in other populations; however, the full range of genetic etiologies in this population is unknown. We report the genetic findings from 33 Saudi hearing-loss probands of tribal ancestry, with predominantly prelingual severe to profound hearing loss. Methods Testing was performed over the course of 2012–2016, and involved initial GJB2 sequence and GJB6-D13S1830 deletion screening, with negative cases being reflexed to a next-generation sequencing panel with 70, 71, or 87 hearing-loss genes. Results A “positive” result was reached in 63% of probands, with two recurrent OTOF variants (p.Glu57* and p.Arg1792His) accountable for a third of all “positive” cases. The next most common cause was pathogenic variants in MYO7A and SLC26A4, each responsible for three “positive” cases. Interestingly, only one “positive” diagnosis had a DFNB1-related cause, due to a homozygous GJB6-D13S1830 deletion, and no sequence variants in GJB2 were detected. Conclusion Our findings implicate OTOF as a potential major contributor to hearing loss in the Saudi population, while highlighting the low contribution of GJB2, thus offering important considerations for clinical testing strategies for Saudi patients. Further screening of Saudi patients is needed to characterize the genetic spectrum in this population. PMID:29048421

Characterization of a 4-inch Portable Shock Tube

DTIC Science & Technology

2014-12-01

This page is intentionally left blank. 1 Introduction Tinnitus and hearing loss have been reported as the two most prevalent service-connected...propelled grenades (RPGs), and/or land mines are known to cause both tinnitus and hearing loss (Sayer, 2008). Intensity levels exceeding 120 decibels (dB

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

Lead and the deafness of Ludwig van Beethoven.

PubMed

Stevens, Michael H; Jacobsen, Teemarie; Crofts, Alicia Kay

2013-11-01

To reexamine the cause of Beethoven's hearing loss because of significant recent articles. Medical and musical literature online, in print, and personal communication. Relevant literature review. Evidence of otosclerosis is lacking because close gross examination of Beethoven's middle ears at autopsy did not find any otosclerotic foci. His slowly progressive hearing loss over a period of years differs from reported cases of autoimmune hearing loss, which are rapidly progressive over a period of months. He also lacked bloody diarrhea that is invariably present with autoimmune inflammatory bowel disease. The absence of mercury in Beethoven's hair and bone samples leads us to conclude that his deafness was not due to syphilis because in that era syphilis was treated with mercury. High levels of lead deep in the bone suggest repeated exposure over a long period of time rather than limited exposure prior to the time of death. The finding of shrunken cochlear nerves at his autopsy is consistent with axonal degeneration due to heavy metals such as lead. Chronic low-level lead exposure causes a slowly progressive hearing loss with sensory and autonomic findings, rather than the classic wrist drop due to motor neuropathy from sub-acute poisoning. Beethoven's physicians thought that he had alcohol dependence. He particularly liked wine that happened to be tainted with lead. Beethoven's chronic consumption of wine tainted with lead is a better explanation of his hearing loss than other causes. Copyright © 2013 The American Laryngological, Rhinological and Otological Society, Inc.

Adverse perinatal conditions and the inner ear.

PubMed

Newton, V

2001-12-01

Epidemiological studies in a number of Western countries have attributed 6-14% of sensorineural hearing impairment from birth or early childhood to problems relating to the birth process [1-4]. In spite of this association it is not always clear in individual instances whether adverse perinatal conditions identified subsequently have been causative of a hearing loss or not. It is possible that the hearing impairment was the consequence of an earlier intrauterine insult or is a coincidental finding. Infants carrying genetic mutations for hearing loss may coincidentally experience perinatal stress. It is therefore important to investigate the cause of a hearing loss discovered in infancy irrespective of the history of the infant having experienced adverse conditions surrounding the time of birth. Copyright 2002 Published by Elsevier Science Ltd.

The Prevention of Noise Induced Hearing Loss in Children

PubMed Central

Harrison, Robert V.

2012-01-01

Increasingly, our acoustic environment is filled with amplified sound sources (e.g., MP3 players, video game stations, and sports/entertainment venues). There is serious concern and also some controversy about the risks of acoustic trauma in children. This overview provides some basic information on the physiological mechanisms that lead to noise induced hearing loss, a survey of various studies that, on balance, indicates that there is cause for concern, and finally a discussion on measures that can help to prevent noise induced hearing loss in children. This paper is designed for public health and other healthcare professions (ENT, audiologists, family doctors, and pediatricians) who should understand the risks of noise induced hearing loss and its prevention. PMID:23304173

Understanding the psychosocial experiences of adults with mild-moderate hearing loss: An application of Leventhal’s self-regulatory model

PubMed Central

Heffernan, Eithne; Coulson, Neil S.; Henshaw, Helen; Barry, Johanna G.; Ferguson, Melanie A

2017-01-01

Objective This study explored the psychosocial experiences of adults with hearing loss using the self-regulatory model as a theoretical framework. The primary components of the model, namely cognitive representations, emotional representations, and coping responses, were examined. Design Individual semi-structured interviews were conducted. The data were analysed using an established thematic analysis procedure. Study sample Twenty-five adults with mild-moderate hearing loss from the UK and nine hearing healthcare professionals from the UK, USA, and Canada were recruited via maximum variation sampling. Results Cognitive representations: Most participants described their hearing loss as having negative connotations and consequences, although they were not particularly concerned about the progression or controllability/curability of the condition. Opinions differed regarding the benefits of understanding the causes of one’s hearing loss in detail. Emotional representations: negative emotions dominated, although some experienced positive emotions or muted emotions. Coping responses: engaged coping (e.g. hearing aids, communication tactics) and disengaged coping (e.g. withdrawal from situations, withdrawal within situations): both had perceived advantages and disadvantages. Conclusions This novel application of the self-regulatory model demonstrates that it can be used to capture the key psychosocial experiences (i.e. perceptions, emotions, and coping responses) of adults with mild-moderate hearing loss within a single, unifying framework. PMID:26754550

Hearing loss in a glue sniffer.

PubMed

Williams, D M

1988-10-01

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

Hearing aids: indications, technology, adaptation, and quality control

PubMed Central

Hoppe, Ulrich; Hesse, Gerhard

2017-01-01

Hearing loss can be caused by a number of different pathological conditions. Some of them can be successfully treated, mainly by surgery, depending on the individual’s disease process. However, the treatment of chronic sensorineural hearing loss with damaged cochlear structures usually needs hearing rehabilitation by means of technical amplification. During the last two decades tremendous improvements in hearing aid technology led to a higher quality of the hearing rehabilitation process. For example, due to sophisticated signal processing acoustic feedback could be reduced and hence open fitting options are available even for more subjects with higher degrees of hearing loss. In particular for high-frequency hearing loss, the use of open fitting is an option. Both the users’ acceptance and the perceived sound quality were significantly increased by open fittings. However, we are still faced with a low level of readiness in many hearing impaired subjects to accept acoustic amplification. Since ENT specialists play a key-role in hearing aid provision, they should promote early hearing aid rehabilitation and include this in the counselling even in subjects with mild and moderate hearing loss. Recent investigations demonstrated the benefit of early hearing aid use in this group of patients since this may help to reduce subsequent damages as auditory deprivation, social isolation, development of dementia, and cognitive decline. For subjects with tinnitus, hearing aids may also support masking by environmental sounds and enhance cortical inhibition. The present paper describes the latest developments of hearing aid technology and the current state of the art for amplification modalities. Implications for both hearing aid indication and provision are discussed. PMID:29279726

[Peripheral vertigo classification. Consensus document. Otoneurology committee of the Spanish otorhinolaryngology society (2003-2006)].

PubMed

Morera, Constantino; Pérez, Herminio; Pérez, Nicolás; Soto, Andrés

2008-02-01

There are many different vertigo classifications and different denominations are frequently used for the same clinical processes. The Otoneurology Committee of the Spanish Society for Otorhinolaryngology and Head and Neck Pathology proposes an eminently practical classification of peripheral vertigo to facilitate a common terminology that can be easily used by the general ENT practitioners. The methodology used has been by consensus within our Society and especially among the most outstanding work groups in the area of otoneurology in Spain. Initially vertigo is divided into single-episode vertigo and recurring attacks of vertigo, and these are then sub-divided into 2 groups, depending on whether or not hearing loss is present. Acute vertigo without hearing loss corresponds to vestibular neuritis and if it is associated with hearing loss, it is due to labyrinthitis of different aetiologies and cochleo-vestibular neuritis. Recurrent vertigos without hearing loss are classified as induced, either by posture (BPPV) or pressure (perilymphatic fistula), or as spontaneous, including migraine-associated vertigo, metabolic vertigo, childhood paroxysmal vertigo and vertigo of vascular causes (AITs, vertebral-basilar failure). Finally, recurrent vertigo with hearing loss includes Ménière's disease and others such as vertigo-migraine (with hearing loss), autoimmune pathology of the inner ear, syphilitic infection, and perilymphatic fistula (with hearing loss).

Progressive Hearing Loss in Mice Carrying a Mutation in Usp53

PubMed Central

Kazmierczak, Marcin; Harris, Suzan L.; Kazmierczak, Piotr; Shah, Prahar; Starovoytov, Valentin; Ohlemiller, Kevin K.

2015-01-01

Disordered protein ubiquitination has been linked to neurodegenerative disease, yet its role in inner ear homeostasis and hearing loss is essentially unknown. Here we show that progressive hearing loss in the ethylnitrosourea-generated mambo mouse line is caused by a mutation in Usp53, a member of the deubiquitinating enzyme family. USP53 contains a catalytically inactive ubiquitin-specific protease domain and is expressed in cochlear hair cells and a subset of supporting cells. Although hair cell differentiation is unaffected in mambo mice, outer hair cells degenerate rapidly after the first postnatal week. USP53 colocalizes and interacts with the tight junction scaffolding proteins TJP1 and TJP2 in polarized epithelial cells, suggesting that USP53 is part of the tight junction complex. The barrier properties of tight junctions of the stria vascularis appeared intact in a biotin tracer assay, but the endocochlear potential is reduced in adult mambo mice. Hair cell degeneration in mambo mice precedes endocochlear potential decline and is rescued in cochlear organotypic cultures in low potassium milieu, indicating that hair cell loss is triggered by extracellular factors. Remarkably, heterozygous mambo mice show increased susceptibility to noise injury at high frequencies. We conclude that USP53 is a novel tight junction-associated protein that is essential for the survival of auditory hair cells and normal hearing in mice, possibly by modulating the barrier properties and mechanical stability of tight junctions. SIGNIFICANCE STATEMENT Hereditary hearing loss is extremely prevalent in the human population, but many genes linked to hearing loss remain to be discovered. Forward genetics screens in mice have facilitated the identification of genes involved in sensory perception and provided valuable animal models for hearing loss in humans. This involves introducing random mutations in mice, screening the mice for hearing defects, and mapping the causative mutation. Here, we have identified a mutation in the Usp53 gene that causes progressive hearing loss in the mambo mouse line. We demonstrate that USP53 is a catalytically inactive deubiquitinating enzyme and a novel component of tight junctions that is necessary for sensory hair cell survival and inner ear homeostasis. PMID:26609154

Severe hearing impairment among military veterans--United States, 2010.

PubMed

2011-07-22

A substantial proportion of hearing loss in the United States is attributable to employment-related exposure to noise. Among military veterans, the most common service-connected disabilities are hearing impairments, suggesting that occupational noise exposure during military service might cause more veterans to have hearing loss than nonveterans. However, a recent analysis of data from the 1993-1995 Epidemiology of Hearing Loss Study did not find significant differences between the two groups. To further investigate hearing loss among veterans, specifically the prevalence of severe hearing impairment (SHI), data from the 2010 Annual Social and Economic Supplement (ASEC) to the Current Population Survey (CPS) were analyzed. This report describes the results of those analyses, which indicated that the prevalence of SHI among veterans was significantly greater than among nonveterans. Veterans were 30% more likely to have SHI than nonveterans after adjusting for age and current occupation, and veterans who served in the United States or overseas during September 2001-March 2010, the era of overseas contingency operations (including Operations Enduring Freedom and Iraqi Freedom), were four times more likely than nonveterans to have SHI. These findings suggest a need for increased emphasis on improving military hearing conservation programs (HCPs) and on hearing loss surveillance in military and veterans' health systems.

Evaluation of Hearing Loss in Pilots

PubMed Central

Atalay, Hayriye; Babakurban, Seda Türkoğlu; Aydın, Erdinç

2015-01-01

Objective High-intensity noise sources with an increase in air traffic and sudden changes in atmospheric pressure can cause hearing loss in pilots. The main goal of this research is to examine hearing loss due to age, the total flight hours and aircraft types and to evaluate the effects of personal conditions that can influence the hearing level. Methods We examined the data of 234 Turkish pilots aged between 25 and 54 years who were examined due to the aviation Law for annual control from January 2005 to January 2014 at Başkent University Medical Faculty, Ankara Hospital. The audiometric results of the pilots were used. While 1, 2, 3, 4, 6, and 8 KHz were used for the airway threshold, 1, 2, and 4 KHz were used for the bone conduction threshold. Results According to the data of the 234 pilots, there was a significant correlation between high-frequency hearing loss and the total flight hours and pilots’ ages. The average hearing loss was higher, particularly in the left ear, in pilots using helicopters than in those using other aircraft types. There was no statistically significant correlation between hearing loss and diabetes, hypercholesterolemia, high blood pressure, anemia, obesity, and smoking. Conclusion A significant correlation was observed between high frequency hearing loss and the total flight hours, pilots’ age, and aircraft types in our study. PMID:29392000

The audiological characteristics of a hereditary Y-linked hearing loss in a Chinese ethnic Tujia pedigree.

PubMed

Fu, Siqing; Yan, Ju; Wang, Xiyin; Dong, Jiashu; Chen, Peiwei; Wang, Chunfang; Chen, Guanming

2011-02-01

To investigate audiometric characteristics of hearing loss in a large Chinese ethnic Tujia family and determine its hereditary type. Total 76 live individuals were investigated in the notable 84 members of this family. The detailed audiometric evaluations were undertaken for the proband and his 47 family members. The degrees of sensorineural hearing impairment were defined as an air/bone gap <15dB hearing loss averaged over 0.5, 1 and 2kHz. The severity of hearing loss was established based on the hearing ability of the better ear, averaged over 0.5, 1, 2 and 4kHz, and classified into four categories: mild, moderate, severe and profound. Nineteen patrilineal relatives of the 76 live members had hearing impairment. The age of onset ranged from 7 to 21 years old with the average of 13.2 years. The audiometric defect was described by auditory curves of a high frequency in 47% of the patients. Affected members in this family demonstrated a non-syndromic, late onset, bilateral, symmetrical, postlingual and sensorineural hearing loss. The audiometric configuration in males of the pedigree is consistent with the hereditary Y-linked hearing loss. Thus we speculate that a putative gene on the Y chromosome could contribute to the cause of the disease. Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.

Occupational noise-induced hearing loss in auto part factory workers in welding units in Thailand.

PubMed

Sriopas, Apiradee; Chapman, Robert S; Sutammasa, Saravudh; Siriwong, Wattasit

2017-01-24

Most workers in auto part factories in Thailand are usually exposed to excessive noise in their workplace. This study aimed to assess the level of occupational noise-induced hearing loss and investigate risk factors causing hearing loss in auto part factory workers in the welding units in Thailand. This was a cross-sectional study. One hundred eighty subjects were recruited from 356 workers in the welding unit of three factories. Sixty eligible subjects in each factory were selected by systemic random sampling. The subjects were interviewed using a face-to-face questionnaire. Noise exposure levels and audiograms were measured by a noise dosimeter and an audiometer, respectively. The findings confirmed that noise exposure levels of 86-90 dB (A) and exceeding 90 dB (A) significantly increased the risk of hearing loss in either ear. A noise exposure level exceeding 90 dB (A) significantly increased the prevalence of hearing loss in both ears. Regarding, a 10-pack-year smoking history increased the prevalence of hearing loss in either ear or both ears. In addition, subjects with employment duration exceeding 10 years significantly developed hearing loss in either ear. The engineering control or personal control by wearing hearing protection device should be used to decrease noise exposure levels lower than 85 dB (A) for 8 h. Moreover, if the exposure level reaches 85 dB (A) for 8 h, the employer needs to implement a hearing conservation program in the workplace.

[Progressive noise induced hearing loss caused by hearing AIDS, a dilemma for the worker and the expert alike].

PubMed

Feldmann, H

2001-12-01

Investigating cases of noise induced hearing loss the expert is often confronted with the situation that the hearing loss is progressive although the noise exposure has been reduced to almost non-damaging levels. Other causes such as age, hereditary deafness, head injuries, blasts, internal diseases can be excluded. Hearing aids as sources of damaging noise? By consulting the protocol of the hearing-aid acoustician and by own examinations the expert should obtain the following data: loudness level that yields best discrimination score of speech; level of discomfort for tones and speech, discrimination score that is achieved under free field condition with a speech level of 65 dB, using the hearing aids. Furthermore he should explore the circumstances under which the hearing aids are used: how many hours per day, at what occasions etc.? It is likely that in using the hearing aids they are adjusted to emit an intensity level identical to the one yielding the optimal discrimination score. If this e. g. is 100 dB and the hearing aids are used for 2 hours per day this would be equivalent to an exposure to industrial noise of 94 dB (A) for 8 hours daily without ear protection. Among all individuals working under industrial noise exposure today only about 1 - 2 % having unusually vulnerable inner ears will suffer a noise induced hearing loss. On the other hand workers in industrial noise are accustomed to loud noise levels, usually have a raised threshold of discomfort and therefore are likely to adjust their hearing aids to such high intensities. The expert will have to decide whether in an individual case the industrial noise exposure or the use of the hearing aids is the dominant risk for further damage. The consequences in respect to the regulations of the workers' health insurance are discussed.

Self-Evaluation Scores of Hearing Difficulties and Quality of Life Components among Retired Workers with Noise-Related Hearing Loss

NASA Astrophysics Data System (ADS)

MIYAKITA, T.; UEDA, A.; ZUSHO, H.; KUDOH, Y.

2002-02-01

This study is intended to clarify the relationships between hearing loss caused both by noise exposure and aging and self-rated scores of hearing disabilities and to elucidate the structure of the quality of life (QOL) determinants and their moderating conditions among retired workers with noise-related hearing loss. The questionnaire consisted of three parts: background questions, the hearing disabilities and handicap scale (HDHS), and questions regarding QOL covering five areas, self-rated health, personal health practice, social support network, life satisfaction, and life events. Two hundred ten retired workers aged 56-65 years old (60·6±1·6) with noise-related hearing loss responded to our questionnaire. All were previously engaged in noise exposed work such as shipbuilding, steel and woodwork. According to the hearing disability score (DIS score), subjects were divided into three groups and comparisons were made of the hearing handicap score (HD score) among those groups. Although groups with a higher DIS score showed a higher HD score, a large individual difference in HD score was observed in each of the three groups with the same DIS score level. The results of multiple regression analysis including two variables (life satisfaction and HD score) as the dependent variables and seven variables as the independent variables showed that the strongest explanatory variable for life satisfaction was social support network, followed by handicaps caused by hearing disabilities, self-rated health and personal health practice. It was demonstrated that hearing disabilities and handicap measured by the Japanese version of the HDHS were directly associated with the deterioration in QOL. Measures of the social support network, life satisfaction, and hearing disabilities and handicaps may assist in the detection of workers who can be targeted for a variety of interventions, such as audiological rehabilitation or the creation of a barrier-free community that is supportive of the hearing impaired.

76 FR 44489 - Medical Devices; Neurological Devices; Classification of Repetitive Transcranial Magnetic...

Federal Register 2010, 2011, 2012, 2013, 2014

2011-07-26

...; Hazards caused by electromagnetic interference and electrostatic discharge hazards; and Hearing loss. FDA... electromagnetic Electromagnetic compatibility. interference and electrostatic discharge hazards. Labeling. Hearing...

Waardenburg syndrome type 2: an orthodontic perspective.

PubMed

Şuhani, Raluca Diana; Şuhani, Mihai Flaviu; Muntean, Alexandrina; Mesaroş, Michaela Florica; Badea, Mîndra Eugenia

2015-01-01

Waardenburg syndrome is a rare form of neurocristopathy. It is a disorder in the development of neural crest cells, caused by an altered cellular migration during the embryonic phase. That alteration causes an association of different abnormalities such as pigmentary disturbances of the hair, iris, skin, stria vascularis of the cochlea, dystopia canthorum and sensorineural hearing loss. We report a case of a 14-year-old Romanian male, with a family history of Waardenburg syndrome (mother) and Usher syndrome (father - congenitally sensorineural hearing loss and retinal degeneration). The case particularities are: the correlation between malocclusion and Waardenburg syndrome due to hypoplastic alae nasi and also factors that produced hearing loss, which could be Waardenburg syndrome, Usher syndrome or the presence of the connexin 26 (W24X) gene mutation.

The relationship between chronic otitis media-induced hearing loss and the acquisition of social skills.

PubMed

Bidadi, Sanam; Nejadkazem, Mohammad; Naderpour, Masoud

2008-11-01

To investigate the effects of hearing loss caused by chronic otitis media (COM) on acquiring social skills. A case-control study of 90 patients, including patients with COM, age range 15-30 years, was conducted in the otorhinolaryngology ward of Tabriz University Hospital. Social skills were assessed with a social skills questionnaire. Social skill scores were found to be lower in hearing-impaired COM patients compared with the control group (P < 0.001). Social skill disabilities in patients with bilateral COM were more severe than in patients with unilateral COM and the controls (P < 0.001). Correlation between social skills score and degree of hearing loss was significantly negative (P = 0.014, rho = -0.314). These data indicate an inverse relationship between hearing loss and social skills. Social skills and educational level of COM patients are affected because of hearing impairment. This study suggests that COM has effects on social development and education.

Ups and Downs of Viagra: Revisiting Ototoxicity in the Mouse Model

PubMed Central

Au, Adrian; Stuyt, John Gerka; Chen, Daniel; Alagramam, Kumar

2013-01-01

Sildenafil citrate (Viagra), a phosphodiesterase 5 inhibitor (PDE5i), is a commonly prescribed drug for erectile dysfunction. Since the introduction of Viagra in 1997, several case reports have linked Viagra to sudden sensorineural hearing loss. However, these studies are not well controlled for confounding factors, such as age and noise-induced hearing loss and none of these reports are based on prospective double-blind studies. Further, animal studies report contradictory data. For example, one study (2008) reported hearing loss in rats after long-term and high-dose exposure to sildenafil citrate. The other study (2012) showed vardenafil, another formulation of PDE5i, to be protective against noise-induced hearing loss in mice and rats. Whether or not clinically relevant doses of sildenafil citrate cause hearing loss in normal subjects (animals or humans) is controversial. One possibility is that PDE5i exacerbates age-related susceptibility to hearing loss in adults. Therefore, we tested sildenafil citrate in C57BL/6J, a strain of mice that displays increased susceptibility to age-related hearing loss, and compared the results to those obtained from the FVB/N, a strain of mice with no predisposition to hearing loss. Six-week-old mice were injected with the maximum tolerated dose of sildenafil citrate (10 mg/kg/day) or saline for 30 days. Auditory brainstem responses (ABRs) were recorded pre- and post injection time points to assess hearing loss. Entry of sildenafil citrate in the mouse cochlea was confirmed by qRT-PCR analysis of a downstream target of the cGMP-PKG cascade. ABR data indicated no statistically significant difference in hearing between treated and untreated mice in both backgrounds. Results show that the maximum tolerated dose of sildenafil citrate administered daily for 4 weeks does not affect hearing in the mouse. Our study gives no indication that Viagra will negatively impact hearing and it emphasizes the need to revisit the issue of Viagra related ototoxicity in humans. PMID:24244454

Ups and downs of Viagra: revisiting ototoxicity in the mouse model.

PubMed

Au, Adrian; Stuyt, John Gerka; Chen, Daniel; Alagramam, Kumar

2013-01-01

Sildenafil citrate (Viagra), a phosphodiesterase 5 inhibitor (PDE5i), is a commonly prescribed drug for erectile dysfunction. Since the introduction of Viagra in 1997, several case reports have linked Viagra to sudden sensorineural hearing loss. However, these studies are not well controlled for confounding factors, such as age and noise-induced hearing loss and none of these reports are based on prospective double-blind studies. Further, animal studies report contradictory data. For example, one study (2008) reported hearing loss in rats after long-term and high-dose exposure to sildenafil citrate. The other study (2012) showed vardenafil, another formulation of PDE5i, to be protective against noise-induced hearing loss in mice and rats. Whether or not clinically relevant doses of sildenafil citrate cause hearing loss in normal subjects (animals or humans) is controversial. One possibility is that PDE5i exacerbates age-related susceptibility to hearing loss in adults. Therefore, we tested sildenafil citrate in C57BL/6J, a strain of mice that displays increased susceptibility to age-related hearing loss, and compared the results to those obtained from the FVB/N, a strain of mice with no predisposition to hearing loss. Six-week-old mice were injected with the maximum tolerated dose of sildenafil citrate (10 mg/kg/day) or saline for 30 days. Auditory brainstem responses (ABRs) were recorded pre- and post injection time points to assess hearing loss. Entry of sildenafil citrate in the mouse cochlea was confirmed by qRT-PCR analysis of a downstream target of the cGMP-PKG cascade. ABR data indicated no statistically significant difference in hearing between treated and untreated mice in both backgrounds. Results show that the maximum tolerated dose of sildenafil citrate administered daily for 4 weeks does not affect hearing in the mouse. Our study gives no indication that Viagra will negatively impact hearing and it emphasizes the need to revisit the issue of Viagra related ototoxicity in humans.

The effect of progressive hearing loss on the morphology of endbulbs of Held and bushy cells.

PubMed

Connelly, Catherine J; Ryugo, David K; Muniak, Michael A

2017-01-01

Studies of congenital and early-onset deafness have demonstrated that an absence of peripheral sound-evoked activity in the auditory nerve causes pathological changes in central auditory structures. The aim of this study was to establish whether progressive acquired hearing loss could lead to similar brain changes that would degrade the precision of signal transmission. We used complementary physiologic hearing tests and microscopic techniques to study the combined effect of both magnitude and duration of hearing loss on one of the first auditory synapses in the brain, the endbulb of Held (EB), along with its bushy cell (BC) target in the anteroventral cochlear nucleus. We compared two hearing mouse strains (CBA/Ca and heterozygous shaker-2 +/- ) against a model of early-onset progressive hearing loss (DBA/2) and a model of congenital deafness (homozygous shaker-2 -/- ), examining each strain at 1, 3, and 6 months of age. Furthermore, we employed a frequency model of the mouse cochlear nucleus to constrain our analyses to regions most likely to exhibit graded changes in hearing function with time. No significant differences in the gross morphology of EB or BC structure were observed in 1-month-old animals, indicating uninterrupted development. However, in animals with hearing loss, both EBs and BCs exhibited a graded reduction in size that paralleled the hearing loss, with the most severe pathology seen in deaf 6-month-old shaker-2 -/- mice. Ultrastructural pathologies associated with hearing loss were less dramatic: minor changes were observed in terminal size but mitochondrial fraction and postsynaptic densities remained relatively stable. These results indicate that acquired progressive hearing loss can have consequences on auditory brain structure, with prolonged loss leading to greater pathologies. Our findings suggest a role for early intervention with assistive devices in order to mitigate long-term pathology and loss of function. Copyright © 2016 Elsevier B.V. All rights reserved.

Protocol of a randomized controlled trial of hearing protection interventions for farm operators.

PubMed

McCullagh, Marjorie C; Ronis, David L

2015-04-18

Hearing loss and tinnitus are prevalent in America, and noise-induced hearing loss is a leading cause of hearing loss. Noise-induced hearing loss has negative impact on quality of life, physical and emotional functioning, social life, and employment. In addition, noise-induced hearing loss results in heavy social and economic burdens on families and communities from all ethnic and socioeconomic groups. Farmers are a group that is particularly high risk for noise-induced hearing loss, and is underserved by programs designed to limit that risk. They are among the most noise-exposed group of workers, and experience the second highest prevalence of noise-induced hearing loss among all occupational categories. In agriculture, 1.5 million workers (43.3%) report exposure to hazardous noise. Although use of hearing protection devices (HPDs) would protect them from noise-induced hearing loss, use among farmers is low. The purpose of this project is to compare the effectiveness of several approaches to influencing hearing protector use. Approaches include: a) an interactive, predictors-based intervention delivered via the Internet; b) a static informational web site; and c) a mailed sampler of hearing protectors. The goals are to further develop an intervention to promote farmers' use of HPDs, and compare the effectiveness of the interventions delivered in various combinations. Participants will include 701 farmers. Sites will be affiliates of a major farmer organization. Data will be collected at baseline, 6, and 12 months. A random intercept mixed model will be used to explore the fixed effects of the three NIHL prevention interventions over time while adjusting for age and gender. This project will involve a partnership between the University of Michigan and a major farmer organization to accomplish project aims. Results of this study will be used to inform future research-to-practice studies to increase hearing protector use. Increased use of hearing protectors is expected to reduce rates of noise-induced hearing loss and other negative effects of high noise exposure, and improve quality of life in this high-risk and underserved group. Clinicaltrials.gov NCT01454895 Registered 14 October, 2011.

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…

CDK2 inhibitors as candidate therapeutics for cisplatin- and noise-induced hearing loss.

PubMed

Teitz, Tal; Fang, Jie; Goktug, Asli N; Bonga, Justine D; Diao, Shiyong; Hazlitt, Robert A; Iconaru, Luigi; Morfouace, Marie; Currier, Duane; Zhou, Yinmei; Umans, Robyn A; Taylor, Michael R; Cheng, Cheng; Min, Jaeki; Freeman, Burgess; Peng, Junmin; Roussel, Martine F; Kriwacki, Richard; Guy, R Kiplin; Chen, Taosheng; Zuo, Jian

2018-04-02

Hearing loss caused by aging, noise, cisplatin toxicity, or other insults affects 360 million people worldwide, but there are no Food and Drug Administration-approved drugs to prevent or treat it. We screened 4,385 small molecules in a cochlear cell line and identified 10 compounds that protected against cisplatin toxicity in mouse cochlear explants. Among them, kenpaullone, an inhibitor of multiple kinases, including cyclin-dependent kinase 2 (CDK2), protected zebrafish lateral-line neuromasts from cisplatin toxicity and, when delivered locally, protected adult mice and rats against cisplatin- and noise-induced hearing loss. CDK2-deficient mice displayed enhanced resistance to cisplatin toxicity in cochlear explants and to cisplatin- and noise-induced hearing loss in vivo. Mechanistically, we showed that kenpaullone directly inhibits CDK2 kinase activity and reduces cisplatin-induced mitochondrial production of reactive oxygen species, thereby enhancing cell survival. Our experiments have revealed the proapoptotic function of CDK2 in postmitotic cochlear cells and have identified promising therapeutics for preventing hearing loss. © 2018 Teitz et al.

CDK2 inhibitors as candidate therapeutics for cisplatin- and noise-induced hearing loss

PubMed Central

Teitz, Tal; Fang, Jie; Goktug, Asli N.; Bonga, Justine D.; Diao, Shiyong; Iconaru, Luigi; Morfouace, Marie; Currier, Duane; Zhou, Yinmei; Umans, Robyn A.; Taylor, Michael R.; Cheng, Cheng; Peng, Junmin; Roussel, Martine F.; Kriwacki, Richard; Guy, R. Kiplin; Chen, Taosheng

2018-01-01

Hearing loss caused by aging, noise, cisplatin toxicity, or other insults affects 360 million people worldwide, but there are no Food and Drug Administration–approved drugs to prevent or treat it. We screened 4,385 small molecules in a cochlear cell line and identified 10 compounds that protected against cisplatin toxicity in mouse cochlear explants. Among them, kenpaullone, an inhibitor of multiple kinases, including cyclin-dependent kinase 2 (CDK2), protected zebrafish lateral-line neuromasts from cisplatin toxicity and, when delivered locally, protected adult mice and rats against cisplatin- and noise-induced hearing loss. CDK2-deficient mice displayed enhanced resistance to cisplatin toxicity in cochlear explants and to cisplatin- and noise-induced hearing loss in vivo. Mechanistically, we showed that kenpaullone directly inhibits CDK2 kinase activity and reduces cisplatin-induced mitochondrial production of reactive oxygen species, thereby enhancing cell survival. Our experiments have revealed the proapoptotic function of CDK2 in postmitotic cochlear cells and have identified promising therapeutics for preventing hearing loss. PMID:29514916

Perforated Eardrum

MedlinePlus

... the loss of hearing can be great and tinnitus (ringing in the ear) may occur. Chronic infection ... could cause ear infection), improved hearing, and lessened tinnitus. It also may prevent the development of cholesteatoma ( ...

Mutations of human NARS2, encoding the mitochondrial asparaginyl-tRNA synthetase, cause nonsyndromic deafness and Leigh syndrome.

PubMed

Simon, Mariella; Richard, Elodie M; Wang, Xinjian; Shahzad, Mohsin; Huang, Vincent H; Qaiser, Tanveer A; Potluri, Prasanth; Mahl, Sarah E; Davila, Antonio; Nazli, Sabiha; Hancock, Saege; Yu, Margret; Gargus, Jay; Chang, Richard; Al-Sheqaih, Nada; Newman, William G; Abdenur, Jose; Starr, Arnold; Hegde, Rashmi; Dorn, Thomas; Busch, Anke; Park, Eddie; Wu, Jie; Schwenzer, Hagen; Flierl, Adrian; Florentz, Catherine; Sissler, Marie; Khan, Shaheen N; Li, Ronghua; Guan, Min-Xin; Friedman, Thomas B; Wu, Doris K; Procaccio, Vincent; Riazuddin, Sheikh; Wallace, Douglas C; Ahmed, Zubair M; Huang, Taosheng; Riazuddin, Saima

2015-03-01

Here we demonstrate association of variants in the mitochondrial asparaginyl-tRNA synthetase NARS2 with human hearing loss and Leigh syndrome. A homozygous missense mutation ([c.637G>T; p.Val213Phe]) is the underlying cause of nonsyndromic hearing loss (DFNB94) and compound heterozygous mutations ([c.969T>A; p.Tyr323*] + [c.1142A>G; p.Asn381Ser]) result in mitochondrial respiratory chain deficiency and Leigh syndrome, which is a neurodegenerative disease characterized by symmetric, bilateral lesions in the basal ganglia, thalamus, and brain stem. The severity of the genetic lesions and their effects on NARS2 protein structure cosegregate with the phenotype. A hypothetical truncated NARS2 protein, secondary to the Leigh syndrome mutation p.Tyr323* is not detectable and p.Asn381Ser further decreases NARS2 protein levels in patient fibroblasts. p.Asn381Ser also disrupts dimerization of NARS2, while the hearing loss p.Val213Phe variant has no effect on NARS2 oligomerization. Additionally we demonstrate decreased steady-state levels of mt-tRNAAsn in fibroblasts from the Leigh syndrome patients. In these cells we show that a decrease in oxygen consumption rates (OCR) and electron transport chain (ETC) activity can be rescued by overexpression of wild type NARS2. However, overexpression of the hearing loss associated p.Val213Phe mutant protein in these fibroblasts cannot complement the OCR and ETC defects. Our findings establish lesions in NARS2 as a new cause for nonsyndromic hearing loss and Leigh syndrome.

Hearing loss and the risk of disability pension in Norway: the Hunt Study.

PubMed

Helvik, Anne-Sofie; Krokstad, Steinar; Tambs, Kristian

2013-12-01

The purpose was to explore the possible associations between measured hearing thresholds and work related disability pension granted for other medical reasons in a Norwegian population. This study included 25,537 persons from the Nord-Trøndelag Health Study (HUNT) aged 20-54 years at baseline in HUNT1 (1984-1986) who also participated in the follow-up study 11 years later, HUNT2 (1995-1997) that included a hearing examination. Logistic regression analyses of disability pension granted up to 1996 or earlier in life were conducted for men and women separately and in two age strata. Effects of low, middle and high-frequency hearing thresholds were explored, adjusting the effects of each hearing frequency for each other. Further adjustment was made for educational level, category of work (seven categories) and general health in HUNT1. In all, 0.4% (16 of 4306) of the disability pensions granted up to 1996 was due to hearing related diagnoses. The risk of being granted disability pension up to 1996 with registered diagnoses not related to hearing loss increased with degree of loss of low-frequency hearing in young and middle-aged men and middle-aged women (OR 1.72, 95% CI 1.25-2.37; OR 1.16, 95% CI 1.04-1.30; OR 1.11, 95% CI 1.00-1.23). Hearing loss diagnoses are rarely reported as main causes in disability statistics, however, degree of hearing loss increased the risk of being granted with disability pensioning with diagnoses not related to hearing loss.

Utility of genetic testing for the detection of late-onset hearing loss in neonates.

PubMed

Lim, B Gail; Clark, Reese H; Kelleher, Amy S; Lin, Zhili; Spitzer, Alan R

2013-12-01

The purpose of this study was to demonstrate the utility of molecular testing in the detection of potentially important causes of delayed hearing loss missed by current audiometric screening at birth. We enrolled infants who had received a newborn audiometric hearing screen and a filter paper blood collection for state newborn screening. A central laboratory ran the SoundGene® panel. Of 3,681 infants studied, 35 (0.95%) had a positive SoundGene panel, 16 had mitochondrial mutations, 9 had Pendred mutations, 5 were cytomegalovirus (CMV) DNA positive, 2 had connexin mutations, and 3 had a combination of different mutations. Infants with an abnormal SoundGene panel were at increased risk for hearing loss compared to neonates without mutations. Three (8.6%) of the 35 subjects had persistent hearing loss compared to 5 (0.21%) of 2,398 subjects with no report of mutation (p < .01). Of 3,681 infants studied, 8 (0.22%) had persistent hearing loss: 5 (62.5%) had abnormal newborn audiometric screens, 2 (25%) had an abnormal SoundGene panel (1 was CMV positive, 1 had a mitochondrial mutation), and 1 (12.5%) had no identifiable risk factors. A positive SoundGene panel identifies infants who are not identified by audiometric testing and may be at risk for hearing loss.

The carrier rate and mutation spectrum of genes associated with hearing loss in South China hearing female population of childbearing age

PubMed Central

2013-01-01

Background Given that hearing loss occurs in 1 to 3 of 1,000 live births and approximately 90 to 95 percent of them are born into hearing families, it is of importance and necessity to get better understanding about the carrier rate and mutation spectrum of genes associated with hearing impairment in the general population. Methods 7,263 unrelated women of childbearing age with normal hearing and without family history of hearing loss were tested with allele-specific PCR-based universal array. Further genetic testing were provided to the spouses of the screened carriers. For those couples at risk, multiple choices were provided, including prenatal diagnosis. Results Among the 7,263 normal hearing participants, 303 subjects carried pathogenic mutations included in the screening chip, which made the carrier rate 4.17%. Of the 303 screened carriers, 282 harbored heterozygous mutated genes associated with autosomal recessive hearing loss, and 95 spouses took further genetic tests. 8 out of the 9 couples harbored deafness-causing mutations in the same gene received prenatal diagnosis. Conclusions Given that nearly 90 to 95 percent of deaf and hard-of-hearing babies are born into hearing families, better understanding about the carrier rate and mutation spectrum of genes associated with hearing impairment in the female population of childbearing age may be of importance in carrier screening and genetic counseling. PMID:23718755

The carrier rate and mutation spectrum of genes associated with hearing loss in South China hearing female population of childbearing age.

PubMed

Yin, Aihua; Liu, Chang; Zhang, Yan; Wu, Jing; Mai, Mingqin; Ding, Hongke; Yang, Jiexia; Zhang, Xiaozhuang

2013-05-29

Given that hearing loss occurs in 1 to 3 of 1,000 live births and approximately 90 to 95 percent of them are born into hearing families, it is of importance and necessity to get better understanding about the carrier rate and mutation spectrum of genes associated with hearing impairment in the general population. 7,263 unrelated women of childbearing age with normal hearing and without family history of hearing loss were tested with allele-specific PCR-based universal array. Further genetic testing were provided to the spouses of the screened carriers. For those couples at risk, multiple choices were provided, including prenatal diagnosis. Among the 7,263 normal hearing participants, 303 subjects carried pathogenic mutations included in the screening chip, which made the carrier rate 4.17%. Of the 303 screened carriers, 282 harbored heterozygous mutated genes associated with autosomal recessive hearing loss, and 95 spouses took further genetic tests. 8 out of the 9 couples harbored deafness-causing mutations in the same gene received prenatal diagnosis. Given that nearly 90 to 95 percent of deaf and hard-of-hearing babies are born into hearing families, better understanding about the carrier rate and mutation spectrum of genes associated with hearing impairment in the female population of childbearing age may be of importance in carrier screening and genetic counseling.

Accelerometer-Determined Physical Activity and Mortality in a National Prospective Cohort Study: Considerations by Hearing Sensitivity.

PubMed

Loprinzi, Paul D

2015-12-01

Previous work demonstrates that hearing impairment and physical inactivity are associated with premature all-cause mortality. The purpose of this study was to discern whether increased physical activity among those with hearing impairment can produce survival benefits. Data from the 2003-2006 National Health and Nutrition Examination Survey were used, with follow-up through 2011. Physical activity was objectively measured over 7 days via accelerometry. Hearing sensitivity was objectively measured using a modified Hughson Westlake procedure. Among the 1,482 participants, 152 died during the follow-up period (10.26%, unweighted); the unweighted median follow-up period was 89 months (interquartile range = 74-98 months). For those with normal hearing and after adjustments, for every 60-min increase in physical activity, adults had a 19% (HR [Hazard Ratio] = 0.81; 95% confidence interval [CI] [0.48-1.35]; p = .40) reduced risk of all-cause mortality; however, this association was not statistically significant. In a similar manner, physical activity was not associated with all-cause mortality among those with mild hearing loss (HR = 0.76; 95% CI [0.51-1.13]; p = .17). However, after adjustments, and for every 60-min increase in physical activity for those with moderate or greater hearing loss, there was a 20% (HR = 0.20; 95% CI [0.67-0.95]; p = .01) reduced risk of all-cause mortality. Physical activity may help to prolong survival among those with greater hearing impairment.

The Impact of Single-Sided Deafness upon Music Appreciation.

PubMed

Meehan, Sarah; Hough, Elizabeth A; Crundwell, Gemma; Knappett, Rachel; Smith, Mark; Baguley, David M

2017-05-01

Many of the world's population have hearing loss in one ear; current statistics indicate that up to 10% of the population may be affected. Although the detrimental impact of bilateral hearing loss, hearing aids, and cochlear implants upon music appreciation is well recognized, studies on the influence of single-sided deafness (SSD) are sparse. We sought to investigate whether a single-sided hearing loss can cause problems with music appreciation, despite normal hearing in the other ear. A tailored questionnaire was used to investigate music appreciation for those with SSD. We performed a retrospective survey of a population of 51 adults from a University Hospital Audiology Department SSD clinic. SSD was predominantly adult-onset sensorineural hearing loss, caused by a variety of etiologies. Analyses were performed to assess for statistical differences between groups, for example, comparing music appreciation before and after the onset of SSD, or before and after receiving hearing aid(s). Results demonstrated that a proportion of the population experienced significant changes to the way music sounded; music was found to sound more unnatural (75%), unpleasant (71%), and indistinct (81%) than before hearing loss. Music was reported to lack the perceptual qualities of stereo sound, and to be confounded by distortion effects and tinnitus. Such changes manifested in an altered music appreciation, with 44% of participants listening to music less often, 71% of participants enjoying music less, and 46% of participants reporting that music played a lesser role in their lives than pre-SSD. Negative effects surrounding social occasions with music were revealed, along with a strong preference for limiting background music. Hearing aids were not found to significantly ameliorate these effects. Results could be explained in part through considerations of psychoacoustic changes intrinsic to an asymmetric hearing loss and impaired auditory scene analysis. Given the prevalence of music and its capacity to influence an individual's well-being, results here present strong indications that the potential effects of SSD on music appreciation should be considered in a clinical context; an investigation into relevant rehabilitation techniques may prove valuable. American Academy of Audiology

Hearing-Impaired Children in Venezuela: 1985.

ERIC Educational Resources Information Center

Schildroth, Arthur; And Others

1987-01-01

The 1984-85 Venezuelan Survey of Deaf Children collected information on 804 deaf students between ages 3 and 14. Among findings were that 29% of the cases were caused by maternal rubella, that 36% had additional handicaps, and that meningitis was the most frequently reported after-birth cause of hearing losses. (Author/DB)

Ectopic Mineralization and Conductive Hearing Loss in Enpp1asj Mutant Mice, a New Model for Otitis Media and Tympanosclerosis.

PubMed

Tian, Cong; Harris, Belinda S; Johnson, Kenneth R

2016-01-01

Otitis media (OM), inflammation of the middle ear, is a common cause of hearing loss in children and in patients with many different syndromic diseases. Studies of the human population and mouse models have revealed that OM is a multifactorial disease with many environmental and genetic contributing factors. Here, we report on otitis media-related hearing loss in asj (ages with stiffened joints) mutant mice, which bear a point mutation in the Enpp1 gene. Auditory-evoked brainstem response (ABR) measurements revealed that around 90% of the mutant mice (Enpp1asj/asj) tested had moderate to severe hearing impairment in at least one ear. The ABR thresholds were variable and generally elevated with age. We found otitis media with effusion (OME) in all of the hearing-impaired Enpp1asj/asj mice by anatomic and histological examinations. The volume and inflammatory cell content of the effusion varied among the asj mutant mice, but all mutants exhibited a thickened middle ear epithelium with fibrous polyps and more mucin-secreting goblet cells than controls. Other abnormalities observed in the Enpp1 mutant mice include over-ossification at the round window ridge, thickened and over-calcified stapedial artery, fusion of malleus and incus, and white patches on the inside of tympanic membrane, some of which are typical symptoms of tympanosclerosis. An excessive yellow discharge was detected in the outer ear canal of older asj mutant mice, with 100% penetrance by 5 months of age, and contributes to the progressive nature of the hearing loss. This is the first report of hearing loss and ear pathology associated with an Enpp1 mutation in mice. The Enpp1asj mutant mouse provides a new animal model for studying tympanosclerotic otitis and otitis media with effusion, and also provides a specific model for the hearing loss recently reported to be associated with human ENPP1 mutations causing generalized arterial calcification of infancy and hypophosphatemic rickets.

Consanguinity Among Parents of Iranian Deaf Children

PubMed Central

Ajallouyan, Mohammad; Radfar, Shokofeh; Nouhi, Sima; Tavallaie, Seid Abbas; Amirsalari, Susan; Yousefi, Jaleh; Hasanali Fard, Mahdieh

2016-01-01

Background It seems that there is a relationship between consanguinity and profound hearing loss but there is little data about the association of consanguinity and hearing loss in Iran. Objectives The aim of this study is to demonstrate the causes of profound bilateral sensorineural hearing loss among Iranian samples who are candidates for cochlear implantation. Methods This study was retrospective, analytical, and designed to collect information about profound hearing impaired cases referred to the Baqiyatallah Cochlear implantation center using enumeration. A total of 310 children with profound hearing impairments participated in this study. They were aged from 6 months to 4 years old. The study was done between January 2007 and April 2009. Chi-square tests were used to show whether there was any statistical difference between the incidence of marital consanguinity of their parents and the normal population. Results Sixty-five percent of those 310 children had parents who had married with their relatives. Of the 203 (65%) parents that had consanguineous marriages, 132 were first cousins, which includes the children of two brothers (37 [11.8%] patrilateral parallel cousins), the children of two sisters (38 [12.2%] multi-lateral parallel cousins), or the children of a brother and a sister (57 [18.3%] cross cousins). Fifty-four (17.4%) of the parents were second cousins and 17 (5.2%) were beyond second cousins. Also, hearing loss etiology was obvious in 237 (76.3%) of the patients with profound hearing loss but was unknown in 73 (23.7%). Hereditary was identified as the most common cause in 33% of the cases. Conclusions Our data demonstrated a 65% occurrence of consanguineous marriage among the parents of deaf children, which is statistically different from the percentage of consanguineous marriage among Iranian population (38%). This indicates an obvious relationship between severe hearing loss and consanguineous marriage. PMID:28191326

Ectopic Mineralization and Conductive Hearing Loss in Enpp1asj Mutant Mice, a New Model for Otitis Media and Tympanosclerosis

PubMed Central

Tian, Cong; Harris, Belinda S.; Johnson, Kenneth R.

2016-01-01

Otitis media (OM), inflammation of the middle ear, is a common cause of hearing loss in children and in patients with many different syndromic diseases. Studies of the human population and mouse models have revealed that OM is a multifactorial disease with many environmental and genetic contributing factors. Here, we report on otitis media-related hearing loss in asj (ages with stiffened joints) mutant mice, which bear a point mutation in the Enpp1 gene. Auditory-evoked brainstem response (ABR) measurements revealed that around 90% of the mutant mice (Enpp1asj/asj) tested had moderate to severe hearing impairment in at least one ear. The ABR thresholds were variable and generally elevated with age. We found otitis media with effusion (OME) in all of the hearing-impaired Enpp1asj/asj mice by anatomic and histological examinations. The volume and inflammatory cell content of the effusion varied among the asj mutant mice, but all mutants exhibited a thickened middle ear epithelium with fibrous polyps and more mucin-secreting goblet cells than controls. Other abnormalities observed in the Enpp1 mutant mice include over-ossification at the round window ridge, thickened and over-calcified stapedial artery, fusion of malleus and incus, and white patches on the inside of tympanic membrane, some of which are typical symptoms of tympanosclerosis. An excessive yellow discharge was detected in the outer ear canal of older asj mutant mice, with 100% penetrance by 5 months of age, and contributes to the progressive nature of the hearing loss. This is the first report of hearing loss and ear pathology associated with an Enpp1 mutation in mice. The Enpp1asj mutant mouse provides a new animal model for studying tympanosclerotic otitis and otitis media with effusion, and also provides a specific model for the hearing loss recently reported to be associated with human ENPP1 mutations causing generalized arterial calcification of infancy and hypophosphatemic rickets. PMID:27959908

A review of the perceptual effects of hearing loss for frequencies above 3 kHz.

PubMed

Moore, Brian C J

2016-12-01

Hearing loss caused by exposure to intense sounds usually has its greatest effects on audiometric thresholds at 4 and 6 kHz. However, in several countries compensation for occupational noise-induced hearing loss is calculated using the average of audiometric thresholds for selected frequencies up to 3 kHz, based on the implicit assumption that hearing loss for frequencies above 3 kHz has no material adverse consequences. This paper assesses whether this assumption is correct. Studies are reviewed that evaluate the role of hearing for frequencies above 3 kHz. Several studies show that frequencies above 3 kHz are important for the perception of speech, especially when background sounds are present. Hearing at high frequencies is also important for sound localization, especially for resolving front-back confusions. Hearing for frequencies above 3 kHz is important for the ability to understand speech in background sounds and for the ability to localize sounds. The audiometric threshold at 4 kHz and perhaps 6 kHz should be taken into account when assessing hearing in a medico-legal context.

Parental training and externalizing behaviors of children with severe hearing loss.

PubMed

Movallali, Guita; Amiri, Mohsen; Nesayan, Abas; Assady Gandomani, Roghaye

2017-01-01

Background: Externalizing behavior in deaf children causes many psychological problems for their parents. Aggression and rule breaking behaviors in children with severe hearing loss may cause psychological problems in parents and in managing children's behavior. Consequently, the present study aimed at investigating the effect of parental behavioral training of mothers on reducing the externalized behaviors (aggression and rule breaking behavior) of their 9- to 10- year old children with severe hearing loss. Methods: This was an experimental study with pretest, posttest, and a control group. The research population included all students with severe hearing loss and their mothers. Thus, 30 mothers whose children had the most severe hearing loss were selected as our main sample among 80 mothers who had completed the Child Behavior Checklist. Then, they were placed into the experimental (n=15) and control groups (n=15) after being matched based on age, educational level, and socioeconomic status. The research tool was Child Behavior Checklist-Persian Version. The parental behavior training was provided for the experimental group in nine 90- minute sessions. Results: The results of the present study indicated the effectiveness of experimental treatment on decreasing the aggressive behaviors of children of mothers in the experimental group compared to mothers of the control group (p=0.001). Moreover, the results revealed a decrease in rule breaking behaviors (p= 0.007) in children of mothers of the experimental group compared to mothers of the control group. Conclusion: Parents' behavioral training is important in decreasing the aggressive and offensive behaviors in children with severe hearing loss who are at risk of behavioral problems. Furthermore, some research has highlighted the necessity of such trainings for parents of these children. Thus, the present study, emphasizing the importance of childhood problems, found that mothers' behavioral training could prevent behavioral problems of school-aged children with hearing loss and reduce the recurrence of such problems.

Hearing Loss in Stranded Odontocete Dolphins and Whales

PubMed Central

Mann, David; Hill-Cook, Mandy; Manire, Charles; Greenhow, Danielle; Montie, Eric; Powell, Jessica; Wells, Randall; Bauer, Gordon; Cunningham-Smith, Petra; Lingenfelser, Robert; DiGiovanni, Robert; Stone, Abigale; Brodsky, Micah; Stevens, Robert; Kieffer, George; Hoetjes, Paul

2010-01-01

The causes of dolphin and whale stranding can often be difficult to determine. Because toothed whales rely on echolocation for orientation and feeding, hearing deficits could lead to stranding. We report on the results of auditory evoked potential measurements from eight species of odontocete cetaceans that were found stranded or severely entangled in fishing gear during the period 2004 through 2009. Approximately 57% of the bottlenose dolphins and 36% of the rough-toothed dolphins had significant hearing deficits with a reduction in sensitivity equivalent to severe (70–90 dB) or profound (>90 dB) hearing loss in humans. The only stranded short-finned pilot whale examined had profound hearing loss. No impairments were detected in seven Risso's dolphins from three different stranding events, two pygmy killer whales, one Atlantic spotted dolphin, one spinner dolphin, or a juvenile Gervais' beaked whale. Hearing impairment could play a significant role in some cetacean stranding events, and the hearing of all cetaceans in rehabilitation should be tested. PMID:21072206

Reprogramming Glia Into Neurons in the Peripheral Auditory System as a Solution for Sensorineural Hearing Loss: Lessons From the Central Nervous System

PubMed Central

Meas, Steven J.; Zhang, Chun-Li; Dabdoub, Alain

2018-01-01

Disabling hearing loss affects over 5% of the world’s population and impacts the lives of individuals from all age groups. Within the next three decades, the worldwide incidence of hearing impairment is expected to double. Since a leading cause of hearing loss is the degeneration of primary auditory neurons (PANs), the sensory neurons of the auditory system that receive input from mechanosensory hair cells in the cochlea, it may be possible to restore hearing by regenerating PANs. A direct reprogramming approach can be used to convert the resident spiral ganglion glial cells into induced neurons to restore hearing. This review summarizes recent advances in reprogramming glia in the CNS to suggest future steps for regenerating the peripheral auditory system. In the coming years, direct reprogramming of spiral ganglion glial cells has the potential to become one of the leading biological strategies to treat hearing impairment. PMID:29593497

Hearing loss in stranded odontocete dolphins and whales.

PubMed

Mann, David; Hill-Cook, Mandy; Manire, Charles; Greenhow, Danielle; Montie, Eric; Powell, Jessica; Wells, Randall; Bauer, Gordon; Cunningham-Smith, Petra; Lingenfelser, Robert; DiGiovanni, Robert; Stone, Abigale; Brodsky, Micah; Stevens, Robert; Kieffer, George; Hoetjes, Paul

2010-11-03

The causes of dolphin and whale stranding can often be difficult to determine. Because toothed whales rely on echolocation for orientation and feeding, hearing deficits could lead to stranding. We report on the results of auditory evoked potential measurements from eight species of odontocete cetaceans that were found stranded or severely entangled in fishing gear during the period 2004 through 2009. Approximately 57% of the bottlenose dolphins and 36% of the rough-toothed dolphins had significant hearing deficits with a reduction in sensitivity equivalent to severe (70-90 dB) or profound (>90 dB) hearing loss in humans. The only stranded short-finned pilot whale examined had profound hearing loss. No impairments were detected in seven Risso's dolphins from three different stranding events, two pygmy killer whales, one Atlantic spotted dolphin, one spinner dolphin, or a juvenile Gervais' beaked whale. Hearing impairment could play a significant role in some cetacean stranding events, and the hearing of all cetaceans in rehabilitation should be tested.

Ménière's Disease

MedlinePlus

... causes severe dizziness (vertigo), ringing in the ears (tinnitus), hearing loss, and a feeling of fullness or ... on suddenly or after a short period of tinnitus or muffled hearing. Some people will have single ...

Rurality and determinants of hearing healthcare in adult hearing aid recipients.

PubMed

Chan, Stephen; Hixon, Brian; Adkins, Margaret; Shinn, Jennifer B; Bush, Matthew L

2017-10-01

The objective of this study was to compare the timing of hearing aid (HA) acquisition between adults in rural and urban communities. We hypothesized that time of acquisition of HA after onset of hearing loss is greater in rural adults compared with urban adults. Secondary objectives included assessment of socioeconomic/educational status and impact of hearing loss and hearing rehabilitation of urban and rural HA recipients. Cross-sectional questionnaire survey. We assessed demographics, timing of HA fitting from onset of hearing loss, and impact of hearing impairment in 336 adult HA recipients (273 urban, 63 rural) from a tertiary referral center. Amplification benefit was assessed using the International Outcome Inventory for Hearing Aids (IOI). The time to HA acquisition was greater for rural participants compared to urban participants (19.1 vs. 25.7 years, P = 0.024) for those with untreated hearing loss for at least 8 years. Age at hearing loss onset was correlated with time to HA acquisition (P = -0.54, P < 0.001). Rural HA participants experienced longer commutes to hearing specialists (68 vs. 32 minutes, P < 0.001), were less likely to achieve a degree beyond high school (P < 0.001), and were more likely to possess Medicaid coverage (P = 0.012) compared to urban participants. Hearing impairment caused job performance difficulty in 60% of all participants. Rural adults are at risk for delayed HA acquisition, which may be related to distance to hearing specialists. Further research is indicated to investigate barriers to care and expand access for vulnerable populations. 4. Laryngoscope, 127:2362-2367, 2017. © 2017 The American Laryngological, Rhinological and Otological Society, Inc.

Irreversible atorvastatin-associated hearing loss.

PubMed

Liu, Michael; Alafris, Antonia; Longo, Anthony J; Cohen, Henry

2012-02-01

Drug-associated ototoxicity is a potentially irreversible adverse event. Among the several 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitors (statins) available in the United States, only atorvastatin is associated with tinnitus, but none are associated with any forms of hearing loss. A search of the published literature (1950-August 2011) revealed no published case reports of ototoxicity associated with statins. To our knowledge, we describe the first case of progressive, irreversible hearing loss in a 32-year-old man 18 months after starting atorvastatin therapy. He began taking atorvastatin 20 mg every evening for treatment of hypercholesterolemia. Six months later, he complained of occasional episodes of tinnitus, which resolved spontaneously. An audiogram was obtained and was normal. By 18 months, the tinnitus became continuous. Another audiogram revealed bilateral "cookie-bite" middle-frequency hearing loss. Atorvastatin was immediately discontinued, and the patient was fitted with hearing aids. Four years after drug discontinuation, his hearing loss had neither progressed nor regressed. Use of the Naranjo adverse drug reaction probability scale indicated a possible (score of 2) temporal and causal relationship between the patient's hearing loss and atorvastatin. Causes of "cookie-bite" hearing loss include chronic exposure to loud noises, presbycusis, genetic predisposition, and drugs. The manufacturer of atorvastatin has received three unpublished cases of deafness, but claims that causal relationships were not established. Despite these claims by the manufacturer, based on this case report, we recommend that clinicians and patients be aware of the risk of atorvastatin-associated tinnitus and permanent hearing loss. Further research is needed to better understand the mechanism and frequency of this adverse event. © 2012 Pharmacotherapy Publications, Inc.

Hearing-Impaired Children under Age 6: 1977 and 1984.

ERIC Educational Resources Information Center

Schildroth, Arthur

1986-01-01

A review of annual survey data revealed that hearing impaired children under age 6 reported in 1984, when compared to those reported in 1977, tended to be younger; had higher percentages of heredity, meningitis, and prematurity as causes of hearing loss; and were more likely to have additional handicaps. (CL)

Long-Term Asymmetric Hearing Affects Cochlear Implantation Outcomes Differently in Adults with Pre- and Postlingual Hearing Loss

PubMed Central

Boisvert, Isabelle; McMahon, Catherine M.; Dowell, Richard C.; Lyxell, Björn

2015-01-01

In many countries, a single cochlear implant is offered as a treatment for a bilateral hearing loss. In cases where there is asymmetry in the amount of sound deprivation between the ears, there is a dilemma in choosing which ear should be implanted. In many clinics, the choice of ear has been guided by an assumption that the reorganisation of the auditory pathways caused by longer duration of deafness in one ear is associated with poorer implantation outcomes for that ear. This assumption, however, is mainly derived from studies of early childhood deafness. This study compared outcomes following implantation of the better or poorer ear in cases of long-term hearing asymmetries. Audiological records of 146 adults with bilateral hearing loss using a single hearing aid were reviewed. The unaided ear had 15 to 72 years of unaided severe to profound hearing loss before unilateral cochlear implantation. 98 received the implant in their long-term sound-deprived ear. A multiple regression analysis was conducted to assess the relative contribution of potential predictors to speech recognition performance after implantation. Duration of bilateral significant hearing loss and the presence of a prelingual hearing loss explained the majority of variance in speech recognition performance following cochlear implantation. For participants with postlingual hearing loss, similar outcomes were obtained by implanting either ear. With prelingual hearing loss, poorer outcomes were obtained when implanting the long-term sound-deprived ear, but the duration of the sound deprivation in the implanted ear did not reliably predict outcomes. Contrary to an apparent clinical consensus, duration of sound deprivation in one ear has limited value in predicting speech recognition outcomes of cochlear implantation in that ear. Outcomes of cochlear implantation are more closely related to the period of time for which the brain is deprived of auditory stimulation from both ears. PMID:26043227

[Hearing capacity and speech production in 417 children with facial cleft abnormalities].

PubMed

Schönweiler, R; Schönweiler, B; Schmelzeisen, R

1994-11-01

Children with cleft palates often suffer from chronic conductive hearing losses, delayed language acquisition and speech disorders. This study presents results of speech and language outcomes in relation to hearing function and types of palatal malformations found. 417 children with cleft palates were examined during followup evaluations that extended over several years. Disorders were studied as they affected the ears, nose and throat, audiometry and speech and language pathology. Children with isolated cleft lips were excluded. Among the total group, 8% had normal speech and language development while 92% had speech or language disorders. 80% of these latter children had hearing problems that predominantly consisted of fluctuating conductive hearing losses caused by otitis media with effusion. 5% had sensorineural hearing losses. Fifty-eight children (14%) with rhinolalia aperta were not improved by speech therapy and required velopharyngoplasties, using a cranial-based pharyngeal flap. Language skills did not depend on the type of cleft palate presents but on the frequency and amount of hearing loss found. Otomicroscopy and audiometric follow-ups with insertions of ventilation tubes were considered to be most important for language development in those children with repeated middle ear infections. Speech or language therapy was necessary in 49% of the children.

Obsessive-compulsive disorder presenting with musical obsessions in otosclerosis: a case report.

PubMed

Islam, Lucrezia; Scarone, Silvio; Gambini, Orsola

2014-11-24

Musical obsessions consist of intrusive recollections of music fragments that are experienced as unwanted. Otosclerosis is caused by an abnormal bone homeostasis of the otic capsule and represents a frequent cause of hearing impairment. Many conditions causing hearing loss have been associated with musical hallucinations, but the association between musical obsessions and hearing loss is frequently overlooked. We present the case of a 51-year-old Caucasian woman with a history of obsessive-compulsive disorder who developed musical obsessions soon after being diagnosed with otosclerosis. She was referred to our obsessive-compulsive disorder outpatient unit by her general psychiatrist. At the time of our first evaluation, she had severe musical obsessions that interfered with her social functioning and made her unable to follow conversations. She was started on 40mg of paroxetine and 2.5mg of aripiprazole, which led to significant improvement of her symptoms and of her social and work functioning. To the best of our knowledge, this is the first report of musical obsessions in a patient with hearing loss due to otosclerosis and a history of obsessive-compulsive disorder. This case suggests that a differential diagnosis of obsessive-compulsive disorder should be carefully considered in patients with hearing impairment who complain of involuntary musical imagery, especially in those patients who have a previous history of obsessive-compulsive disorder.

Noise-induced hearing loss in small-scale metal industry in Nepal.

PubMed

Whittaker, J D; Robinson, T; Acharya, A; Singh, D; Smith, M

2014-10-01

There has been no previous research to demonstrate the risk of noise-induced hearing loss in industry in Nepal. Limited research on occupational noise-induced hearing loss has been conducted within small-scale industry worldwide, despite it being a substantial and growing cause of deafness in the developing world. The study involved a cross-sectional audiometric assessment, with questionnaire-based examinations of noise and occupational history, and workplace noise level assessment. A total of 115 metal workers and 123 hotel workers (control subjects) were recruited. Noise-induced hearing loss prevalence was 30.4 per cent in metal workers and 4.1 per cent in hotel workers, with a significant odds ratio of 10.3. Except for age and time in occupation, none of the demographic factors were significant in predicting outcomes in regression analyses. When adjusted for this finding, and previous noise-exposed occupations, the odds ratio was 13.8. Workplace noise was significantly different between the groups, ranging from 65.3 to 84.7 dBA in metal worker sites, and from 51.4 to 68.6 dBA in the control sites. Metal workers appear to have a greater risk of noise-induced hearing loss than controls. Additional research on occupational noise-induced hearing loss in Nepal and small-scale industry globally is needed.

Factors associated with Hearing Loss in a Normal-Hearing Guinea Pig Model of Hybrid Cochlear Implants

PubMed Central

Tanaka, Chiemi; Nguyen-Huynh, Anh; Loera, Katherine; Stark, Gemaine; Reiss, Lina

2014-01-01

The Hybrid cochlear implant (CI), also known as Electro- Acoustic Stimulation (EAS), is a new type of CI that preserves residual acoustic hearing and enables combined cochlear implant and hearing aid use in the same ear. However, 30-55% of patients experience acoustic hearing loss within days to months after activation, suggesting that both surgical trauma and electrical stimulation may cause hearing loss. The goals of this study were to: 1) determine the contributions of both implantation surgery and EAS to hearing loss in a normal-hearing guinea pig model; 2) determine which cochlear structural changes are associated with hearing loss after surgery and EAS. Two groups of animals were implanted (n=6 per group), with one group receiving chronic acoustic and electric stimulation for 10 weeks, and the other group receiving no direct acoustic or electric stimulation during this time frame. A third group (n=6) was not implanted, but received chronic acoustic stimulation. Auditory brainstem response thresholds were followed over time at 1, 2, 6, and 16 kHz. At the end of the study, the following cochlear measures were quantified: hair cells, spiral ganglion neuron density, fibrous tissue density, and stria vascularis blood vessel density; the presence or absence of ossification around the electrode entry was also noted. After surgery, implanted animals experienced a range of 0-55 dB of threshold shifts in the vicinity of the electrode at 6 and 16 kHz. The degree of hearing loss was significantly correlated with reduced stria vascularis vessel density and with the presence of ossification, but not with hair cell counts, spiral ganglion neuron density, or fibrosis area. After 10 weeks of stimulation, 67% of implanted, stimulated animals had more than 10 dB of additional threshold shift at 1 kHz, compared to 17% of implanted, non-stimulated animals and 0% of non-implanted animals. This 1-kHz hearing loss was not associated with changes in any of the cochlear measures quantified in this study. The variation in hearing loss after surgery and electrical stimulation in this animal model is consistent with the variation in human patients. Further, these findings illustrate an advantage of a normal-hearing animal model for quantification of hearing loss and damage to cochlear structures without the confounding effects of chemical- or noise-induced hearing loss. Finally, this study is the first to suggest a role of the stria vascularis and damage to the lateral wall in implantation-induced hearing loss. Further work is needed to determine the mechanisms of implantation- and electrical-stimulation-induced hearing loss. PMID:25128626

Otitis Media: Implications of Fluctuating, Conductive Hearing Loss on Learning and Behaviour in High School Age Students.

ERIC Educational Resources Information Center

Stenton, Jan

This paper provides an overview of the effects of otitis media on the learning and behavior of children and youth. It begins by describing the conductive hearing loss that is caused by otitis media and the classroom behavior that can result, including poor concentration and attention, disobedience, irritability, and poor social skills. Discussed…

Mechanisms of Cisplatin-Induced Ototoxicity and Otoprotection

PubMed Central

Sheth, Sandeep; Mukherjea, Debashree; Rybak, Leonard P.; Ramkumar, Vickram

2017-01-01

Evidence of significant hearing loss during the early days of use of cisplatin as a chemotherapeutic agent in cancer patients has stimulated research into the causes and treatment of this side effect. It has generally been accepted that hearing loss is produced by excessive generation of reactive oxygen species (ROS) in cell of the cochlea, which led to the development of various antioxidants as otoprotective agents. Later studies show that ROS could stimulate cochlear inflammation, suggesting the use of anti-inflammatory agents for treatment of hearing loss. In this respect, G-protein coupled receptors, such as adenosine A1 receptor and cannabinoid 2 receptors, have shown efficacy in the treatment of hearing loss in experimental animals by increasing ROS scavenging, suppressing ROS generation, or by decreasing inflammation. Inflammation could be triggered by activation of transient receptor potential vanilloid 1 (TRPV1) channels in the cochlea and possibly other TRP channels. Targeting TRPV1 for knockdown has also been shown to be a useful strategy for ensuring otoprotection. Cisplatin entry into cochlear hair cells is mediated by various transporters, inhibitors of which have been shown to be effective for treating hearing loss. Finally, cisplatin-induced DNA damage and activation of the apoptotic process could be targeted for cisplatin-induced hearing loss. This review focuses on recent development in our understanding of the mechanisms underlying cisplatin-induced hearing loss and provides examples of how drug therapies have been formulated based on these mechanisms. PMID:29163050

Opening the Market for Lower Cost Hearing Aids: Regulatory Change Can Improve the Health of Older Americans.

PubMed

Blustein, Jan; Weinstein, Barbara E

2016-06-01

Hearing loss is a leading cause of disability among older people. Yet only one in seven US adults who could benefit from a hearing aid uses one. This fraction has not increased over the past 30 years, nor have hearing aid prices dropped, despite trends of steady improvements and price reductions in the consumer electronics industry. The President's Council on Science and Technology has proposed changes in the regulation of hearing aids, including the creation of a "basic" low-cost over-the-counter category of devices. We discuss the potential to reduce disability as well as to improve public health, stakeholder responses to the president's council's proposal, and public health efforts to further mitigate the burden of disability stemming from age-related hearing loss.

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

PubMed

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

2017-01-01

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

Influenza Virus Induces Bacterial and Nonbacterial Otitis Media

PubMed Central

Diavatopoulos, Dimitri A.; Thornton, Ruth; Pedersen, John; Strugnell, Richard A.; Wise, Andrew K.; Reading, Patrick C.; Wijburg, Odilia L.

2011-01-01

Otitis media (OM) is one of the most common childhood diseases. OM can arise when a viral infection enables bacteria to disseminate from the nasopharynx to the middle ear. Here, we provide the first infant murine model for disease. Mice coinfected with Streptococcus pneumoniae and influenza virus had high bacterial load in the middle ear, middle ear inflammation, and hearing loss. In contrast, mice colonized with S. pneumoniae alone had significantly less bacteria in the ear, minimal hearing loss, and no inflammation. Of interest, infection with influenza virus alone also caused some middle ear inflammation and hearing loss. Overall, this study provides a clinically relevant and easily accessible animal model to study the pathogenesis and prevention of OM. Moreover, we provide, to our knowledge, the first evidence that influenza virus alone causes middle ear inflammation in infant mice. This inflammation may then play an important role in the development of bacterial OM. PMID:21930608

[Chronicle myeloid leukemia and hiperviscosity syndrome: case report].

PubMed

Amâncio, Juliana; Scuro, Gisele; Gazoni, Fernanda Martins; Guimarães, Hélio Penna; Vendrame, Letícia Sandre; Lopes, Renato Delascio; Lopes, Antônio Carlos

2008-03-01

Hyperleukocytosis (> 100 x 10(9)/L) is an uncommon presentation of chronic leukemias and it can present clinical symptoms of hyperviscosity syndrome. Hearing loss and blindness rarely occurs in patients with leukemia; however, it can be strong association with hyper-viscosity syndrome. The purpose of this paper is to report a case of acute hearing loss as the initial manifestation of acute leukemia and hyper-viscosity syndrome and also mainly aspects of the intensive care treatment. A 41 year-old, male patient, who has been complaining about dizziness for six months with no response to symptomatic medications, was admitted to the emergency department with acute hearing loss. The physical examination was normal except for a bilateral hearing loss without an apparent cause. Laboratory exams showed total leukocyte: 645.000 with 66.4% blasts, hemoglobin: 7.0, hematocrit: 20.5, urea: 94, creatinine: 1.59, K: 5.6, Na: 138, INR: 1.38, TTPa: 0.89, troponin lower than 0.2, CK: 218, CKMB: 50, uric acid: 11.1. After a first hypothesis of leukemia with a high risk of hyper-viscosity complications, the patient was admitted to the Intensive Care Unit for monitoring and treatment. A bone marrow biopsy was performed and than started hidratation, hydroxyurea, allopurinol, dexamethasone. According to hematologists the patient had a chronic myeloid leukemia. Leukopheresis was performed one week after admission when total blood leukocytes were around 488.000. Ten days after the procedure the patient had no improvement of the hearing loss but total leukocytes were 10.100. He was discharge to the ward and 2 weeks later went home to continue ambulatory treatment. The frequency of sensitive manifestations in patients with leukemia include not only visual and hearing loss but also many others manifestations such as conductive vertigo, facial palsy and infections. Hyperviscosity syndrome due to hyperleukocytosis is also a possible cause of sensorial loss, but the syndrome is often dependent on leukocyte counts greater than (>100 x 10(9)/L).This case is a representative of rare cases in which acute sensorineural hearing loss occurred as the initial manifestation of hyper-viscosity syndrome due to leukemia.

Lassa fever-induced sensorineural hearing loss: A neglected public health and social burden.

PubMed

Mateer, Elizabeth J; Huang, Cheng; Shehu, Nathan Y; Paessler, Slobodan

2018-02-01

Although an association between Lassa fever (LF) and sudden-onset sensorineural hearing loss (SNHL) was confirmed clinically in 1990, the prevalence of LF-induced SNHL in endemic countries is still underestimated. LF, a viral hemorrhagic fever disease caused by Lassa virus (LASV), is endemic in West Africa, causing an estimated 500,000 cases and 5,000 deaths per year. Sudden-onset SNHL, one complication of LF, occurs in approximately one-third of survivors and constitutes a neglected public health and social burden. In the endemic countries, where access to hearing aids is limited, SNHL results in a decline of the quality of life for those affected. In addition, hearing loss costs Nigeria approximately 43 million dollars per year. The epidemiology of LF-induced SNHL has not been characterized well. The complication of LF induced by SNHL is also an important consideration for vaccine development and treatments. However, research into the mechanism has been hindered by the lack of autopsy samples and relevant small animal models. Recently, the first animal model that mimics the symptoms of SNHL associated with LF was developed. Preliminary data from the new animal model as well as the clinical case studies support the mechanism of immune-mediated injury that causes SNHL in LF patients. This article summarizes clinical findings of hearing loss in LF patients highlighting the association between LASV infection and SNHL as well as the potential mechanism(s) for LF-induced SNHL. Further research is necessary to identify the mechanism and the epidemiology of LF-induced SNHL.

Massively Parallel Sequencing of a Chinese Family with DFNA9 Identified a Novel Missense Mutation in the LCCL Domain of COCH

PubMed Central

Gu, Xiaodong; Su, Wenling; Tang, Mingliang; Guo, Luo; Zhao, Liping

2016-01-01

DFNA9 is a late-onset, progressive, autosomal dominantly inherited sensorineural hearing loss with vestibular dysfunction, which is caused by mutations in the COCH (coagulation factor C homology) gene. In this study, we investigated a Chinese family segregating autosomal dominant nonsyndromic sensorineural hearing loss. We identified a missense mutation c.T275A p.V92D in the LCCL domain of COCH cosegregating with the disease and absent in 100 normal hearing controls. This mutation leads to substitution of the hydrophobic valine to an acidic amino acid aspartic acid. Our data enriched the mutation spectrum of DFNA9 and implied the importance for mutation screening of COCH in age related hearing loss with vestibular dysfunctions. PMID:28116169

Can Removal of Back Teeth Contribute to Chronic Earwax Obstruction?

ERIC Educational Resources Information Center

Fransman, Denny

2006-01-01

Impacted earwax is extremely common in people with learning disabilities. Although it is not a serious medical condition it causes a number of very unpleasant problems. These symptoms include discomfort in and around the ear, hearing loss, dizziness and noises in the ears. It also causes secondary problems with ear infections and hearing aid use.…

Heterogeneity of Hereditary Hearing Loss in Iran: a Comprehensive Review

PubMed Central

Beheshtian, Maryam; Babanejad, Mojgan; Azaiez, Hela; Bazazzadegan, Niloofar; Kolbe, Diana; Sloan-Heggen, Christina; Arzhangi, Sanaz; Booth, Kevin; Mohseni, Marzieh; Frees, Kathy; Hossein Azizi, Mohammad; Daneshi, Ahmad; Farhadi, Mohammad; Kahrizi, Kimia; Smith, Richard JH; Najmabadi, Hossein

2017-01-01

A significant contribution to the causes of hereditary hearing impairment comes from genetic factors. More than 120 genes and 160 loci have been identified to be involved in hearing impairment. Given that consanguine populations are more vulnerable to most inherited diseases, such as hereditary hearing loss (HHL), the genetic picture of HHL among the Iranian population, which consists of at least eight ethnic subgroups with a high rate of intermarriage, is expected to be highly heterogeneous. Using an electronic literature review through various databases such as PubMed, MEDLINE, and Scopus, we review the current picture of HHL in Iran. In this review, we present more than 39 deafness genes reported to cause non-syndromic HHL in Iran, of which the most prevalent causative genes include GJB2, SLC26A4, MYO15A, and MYO7A. In addition, we highlight some of the more common genetic causes of syndromic HHL in Iran. These results are of importance for further investigation and elucidation of the molecular basis of HHL in Iran and also for developing a national diagnostic tool tailored to the Iranian context enabling early and efficient diagnosis of hereditary hearing impairment. PMID:27743438

Current concepts in the pathogenesis and treatment of chronic suppurative otitis media

PubMed Central

Mittal, Rahul; Lisi, Christopher V.; Gerring, Robert; Mittal, Jeenu; Mathee, Kalai; Narasimhan, Giri; Azad, Rajeev K.; Yao, Qi; Grati, M'hamed; Yan, Denise; Eshraghi, Adrien A.; Angeli, Simon I.; Telischi, Fred F.

2015-01-01

Otitis media (OM) is an inflammation of the middle ear associated with infection. Despite appropriate therapy, acute OM (AOM) can progress to chronic suppurative OM (CSOM) associated with ear drum perforation and purulent discharge. The effusion prevents the middle ear ossicles from properly relaying sound vibrations from the ear drum to the oval window of the inner ear, causing conductive hearing loss. In addition, the inflammatory mediators generated during CSOM can penetrate into the inner ear through the round window. This can cause the loss of hair cells in the cochlea, leading to sensorineural hearing loss. Pseudomonas aeruginosa and Staphylococcus aureus are the most predominant pathogens that cause CSOM. Although the pathogenesis of AOM is well studied, very limited research is available in relation to CSOM. With the emergence of antibiotic resistance as well as the ototoxicity of antibiotics and the potential risks of surgery, there is an urgent need to develop effective therapeutic strategies against CSOM. This warrants understanding the role of host immunity in CSOM and how the bacteria evade these potent immune responses. Understanding the molecular mechanisms leading to CSOM will help in designing novel treatment modalities against the disease and hence preventing the hearing loss. PMID:26248613

Otitis media.

PubMed

Schilder, Anne G M; Chonmaitree, Tasnee; Cripps, Allan W; Rosenfeld, Richard M; Casselbrant, Margaretha L; Haggard, Mark P; Venekamp, Roderick P

2016-09-08

Otitis media (OM) or middle ear inflammation is a spectrum of diseases, including acute otitis media (AOM), otitis media with effusion (OME; 'glue ear') and chronic suppurative otitis media (CSOM). OM is among the most common diseases in young children worldwide. Although OM may resolve spontaneously without complications, it can be associated with hearing loss and life-long sequelae. In developing countries, CSOM is a leading cause of hearing loss. OM can be of bacterial or viral origin; during 'colds', viruses can ascend through the Eustachian tube to the middle ear and pave the way for bacterial otopathogens that reside in the nasopharynx. Diagnosis depends on typical signs and symptoms, such as acute ear pain and bulging of the tympanic membrane (eardrum) for AOM and hearing loss for OME; diagnostic modalities include (pneumatic) otoscopy, tympanometry and audiometry. Symptomatic management of ear pain and fever is the mainstay of AOM treatment, reserving antibiotics for children with severe, persistent or recurrent infections. Management of OME largely consists of watchful waiting, with ventilation (tympanostomy) tubes primarily for children with chronic effusions and hearing loss, developmental delays or learning difficulties. The role of hearing aids to alleviate symptoms of hearing loss in the management of OME needs further study. Insertion of ventilation tubes and adenoidectomy are common operations for recurrent AOM to prevent recurrences, but their effectiveness is still debated. Despite reports of a decline in the incidence of OM over the past decade, attributed to the implementation of clinical guidelines that promote accurate diagnosis and judicious use of antibiotics and to pneumococcal conjugate vaccination, OM continues to be a leading cause for medical consultation, antibiotic prescription and surgery in high-income countries.

Musical hallucination associated with hearing loss.

PubMed

Sanchez, Tanit Ganz; Rocha, Savya Cybelle Milhomem; Knobel, Keila Alessandra Baraldi; Kii, Márcia Akemi; Santos, Rosa Maria Rodrigues dos; Pereira, Cristiana Borges

2011-01-01

In spite of the fact that musical hallucination have a significant impact on patients' lives, they have received very little attention of experts. Some researchers agree on a combination of peripheral and central dysfunctions as the mechanism that causes hallucination. The most accepted physiopathology of musical hallucination associated to hearing loss (caused by cochlear lesion, cochlear nerve lesion or by interruption of mesencephalon or pontine auditory information) is the disinhibition of auditory memory circuits due to sensory deprivation. Concerning the cortical area involved in musical hallucination, there is evidence that the excitatory mechanism of the superior temporal gyrus, as in epilepsies, is responsible for musical hallucination. In musical release hallucination there is also activation of the auditory association cortex. Finally, considering the laterality, functional studies with musical perception and imagery in normal individuals showed that songs with words cause bilateral temporal activation and melodies activate only the right lobe. The effect of hearing aids on the improvement of musical hallucination as a result of the hearing loss improvement is well documented. It happens because auditory hallucination may be influenced by the external acoustical environment. Neuroleptics, antidepressants and anticonvulsants have been used in the treatment of musical hallucination. Cases of improvement with the administration of carbamazepine, meclobemide and donepezil were reported, but the results obtained were not consistent.

Noise, impulse noise, and other physical factors: combined effects on hearing.

PubMed

Pekkarinen, J

1995-01-01

In most of the epidemiologic studies conducted during the last 20 years, impulse noise caused increased risk of hearing loss in comparison to continuous noise with the same acoustical energy. The interaction between noise exposure (broadband at 100 dB(A)) and hand-arm vibration (125 Hz at 2 ms-2 acceleration level) has been proven for people having vibration-induced white finger symptoms. This interaction is evidenced as a permanent hearing loss. However, why the interaction is seen only in people with VWF is not known. The mechanisms may be related to individual susceptibility, and hypotheses are given on the role of the autonomous nervous system regulating the peripheral vascular reaction. Whole-body vibration (2-10 Hz, at 10 ms-2 level) seems to increase the TTS when noise (broadband at 90 dB(A)) is present. This effect is more pronounced at higher temperatures. The hypothermia protects hearing against the effects of noise in animal studies. The interaction between noise and temperature decrease seems obvious in animal studies. Exercise has both increased and decreased the TTS during noise exposure. The effects have been successfully explained as the depression of the stapedius reflex. Thus, less protection against noise is provided for the inner ear in exercise conditions. The increase of the blood temperature also has been suggested to increase noise-induced TTS during exercise. Electromagnetic fields have been found to cause acoustical interactions in the inner ear. Animal studies and human studies have given contradictory results on the effects of magnetic coil devices on hearing. The MR imaging devices produce noise levels of 82-93 dB, which is not sufficient to produce the risk of permanent hearing loss when short exposure durations are taken into consideration. More systematic research is needed with accurately defined electromagnetic characteristics to reveal the potential interactions. The interactions seem to exist, but relatively high levels and durations of exposure are needed to produce an observable effect on hearing. More investigations are still needed on the permanent hearing loss in humans caused by simultaneous long-term exposures to interacting environmental factors.

Variable hearing impairment in a DFNB2 family with a novel MYO7A missense mutation.

PubMed

Hildebrand, M S; Thorne, N P; Bromhead, C J; Kahrizi, K; Webster, J A; Fattahi, Z; Bataejad, M; Kimberling, W J; Stephan, D; Najmabadi, H; Bahlo, M; Smith, R J H

2010-06-01

Myosin VIIA mutations have been associated with non-syndromic hearing loss (DFNB2; DFNA11) and Usher syndrome type 1B (USH1B). We report clinical and genetic analyses of a consanguineous Iranian family segregating autosomal recessive non-syndromic hearing loss (ARNSHL). The hearing impairment was mapped to the DFNB2 locus using Affymetrix 50K GeneChips; direct sequencing of the MYO7A gene was completed. The Iranian family (L-1419) was shown to segregate a novel homozygous missense mutation (c.1184G>A) that results in a p.R395H amino acid substitution in the motor domain of the myosin VIIA protein. As one affected family member had significantly less severe hearing loss, we used a candidate approach to search for a genetic modifier. This novel MYO7A mutation is the first reported to cause DFNB2 in the Iranian population and this DFNB2 family is the first to be associated with a potential modifier. The absence of vestibular and retinal defects, and less severe low frequency hearing loss, is consistent with the phenotype of a recently reported Pakistani DFNB2 family. Thus, we conclude this family has non-syndromic hearing loss (DFNB2) rather than USH1B, providing further evidence that these two diseases represent discrete disorders.

Noise Exposure and Hearing Capabilities of Quarry Workers in Ghana: A Cross-Sectional Study

PubMed Central

Gyamfi, Charles Kwame R.; Amankwaa, Isaac; Owusu Sekyere, Frank; Boateng, Daniel

2016-01-01

Introduction. Although quarry operations have high economic significance, the effects they cause to the workers in terms of excessive noise production cannot be overlooked. This cross-sectional study assessed the extent of noise exposure and its influence on hearing capabilities among quarry workers in Ashanti region. Methods. The study involved 400 workers randomly selected from five quarries in Ashanti region from April to June 2012. Data was collected using structured questionnaires, physical examination, and audiological assessments. A logistic regression model was fitted to assess independent predictors of hearing loss. Results. All the machines used at the various quarries produced noise that exceeded the minimum threshold with levels ranging from 85.5 dBA to 102.7 dBA. 176 (44%) of study respondents had hearing threshold higher than 25 dBA. 18% and 2% of these were moderately (41–55 dBA) and severely (71–90 dBA) impaired, respectively. Age, duration of work, and use of earplugs independently predicted the development of hearing loss. Use of earplugs showed a protective effect on the development of hearing loss (OR = 0.45; 95% CI = 0.25, 0.84). Conclusion. This study provides empirical evidence on the extent of damage caused to quarry workers as a result of excessive noise exposure. This will support the institution of appropriate protective measures to minimize this threat. PMID:26904137

Reference-Free Assessment of Speech Intelligibility Using Bispectrum of an Auditory Neurogram.

PubMed

Hossain, Mohammad E; Jassim, Wissam A; Zilany, Muhammad S A

2016-01-01

Sensorineural hearing loss occurs due to damage to the inner and outer hair cells of the peripheral auditory system. Hearing loss can cause decreases in audibility, dynamic range, frequency and temporal resolution of the auditory system, and all of these effects are known to affect speech intelligibility. In this study, a new reference-free speech intelligibility metric is proposed using 2-D neurograms constructed from the output of a computational model of the auditory periphery. The responses of the auditory-nerve fibers with a wide range of characteristic frequencies were simulated to construct neurograms. The features of the neurograms were extracted using third-order statistics referred to as bispectrum. The phase coupling of neurogram bispectrum provides a unique insight for the presence (or deficit) of supra-threshold nonlinearities beyond audibility for listeners with normal hearing (or hearing loss). The speech intelligibility scores predicted by the proposed method were compared to the behavioral scores for listeners with normal hearing and hearing loss both in quiet and under noisy background conditions. The results were also compared to the performance of some existing methods. The predicted results showed a good fit with a small error suggesting that the subjective scores can be estimated reliably using the proposed neural-response-based metric. The proposed metric also had a wide dynamic range, and the predicted scores were well-separated as a function of hearing loss. The proposed metric successfully captures the effects of hearing loss and supra-threshold nonlinearities on speech intelligibility. This metric could be applied to evaluate the performance of various speech-processing algorithms designed for hearing aids and cochlear implants.

Reference-Free Assessment of Speech Intelligibility Using Bispectrum of an Auditory Neurogram

PubMed Central

Hossain, Mohammad E.; Jassim, Wissam A.; Zilany, Muhammad S. A.

2016-01-01

Sensorineural hearing loss occurs due to damage to the inner and outer hair cells of the peripheral auditory system. Hearing loss can cause decreases in audibility, dynamic range, frequency and temporal resolution of the auditory system, and all of these effects are known to affect speech intelligibility. In this study, a new reference-free speech intelligibility metric is proposed using 2-D neurograms constructed from the output of a computational model of the auditory periphery. The responses of the auditory-nerve fibers with a wide range of characteristic frequencies were simulated to construct neurograms. The features of the neurograms were extracted using third-order statistics referred to as bispectrum. The phase coupling of neurogram bispectrum provides a unique insight for the presence (or deficit) of supra-threshold nonlinearities beyond audibility for listeners with normal hearing (or hearing loss). The speech intelligibility scores predicted by the proposed method were compared to the behavioral scores for listeners with normal hearing and hearing loss both in quiet and under noisy background conditions. The results were also compared to the performance of some existing methods. The predicted results showed a good fit with a small error suggesting that the subjective scores can be estimated reliably using the proposed neural-response-based metric. The proposed metric also had a wide dynamic range, and the predicted scores were well-separated as a function of hearing loss. The proposed metric successfully captures the effects of hearing loss and supra-threshold nonlinearities on speech intelligibility. This metric could be applied to evaluate the performance of various speech-processing algorithms designed for hearing aids and cochlear implants. PMID:26967160

Opening the Market for Lower Cost Hearing Aids: Regulatory Change Can Improve the Health of Older Americans

PubMed Central

Weinstein, Barbara E.

2016-01-01

Hearing loss is a leading cause of disability among older people. Yet only one in seven US adults who could benefit from a hearing aid uses one. This fraction has not increased over the past 30 years, nor have hearing aid prices dropped, despite trends of steady improvements and price reductions in the consumer electronics industry. The President’s Council on Science and Technology has proposed changes in the regulation of hearing aids, including the creation of a “basic” low-cost over-the-counter category of devices. We discuss the potential to reduce disability as well as to improve public health, stakeholder responses to the president’s council’s proposal, and public health efforts to further mitigate the burden of disability stemming from age-related hearing loss. PMID:27077339

Adenosine receptors regulate susceptibility to noise-induced neural injury in the mouse cochlea and hearing loss.

PubMed

Vlajkovic, Srdjan M; Ambepitiya, Kaushi; Barclay, Meagan; Boison, Detlev; Housley, Gary D; Thorne, Peter R

2017-03-01

Our previous studies have shown that the stimulation of A 1 adenosine receptors in the inner ear can mitigate the loss of sensory hair cells and hearing loss caused by exposure to traumatic noise. Here, we focus on the role of adenosine receptors (AR) in the development of noise-induced neural injury in the cochlea using A 1 AR and A 2A AR null mice (A 1 AR -/- and A 2A AR -/- ). Wildtype (WT) and AR deficient mice were exposed to octave band noise (8-16 kHz, 100 dB SPL) for 2 h to induce cochlear injury and hearing loss. Auditory thresholds and input/output functions were assessed using auditory brainstem responses (ABR) before and two weeks post-exposure. The loss of outer hair cells (OHC), afferent synapses and spiral ganglion neurons (SGN) were assessed by quantitative histology. A 1 AR -/- mice (6-8 weeks old) displayed a high frequency hearing loss (ABR threshold shift and reduced ABR wave I and II amplitudes). This hearing loss was further aggravated by acute noise exposure and exceeded the hearing loss in the WT and A 2A AR -/- mice. All mice experienced the loss of OHC, synaptic ribbons and SGN after noise exposure, but the loss of SGN was significantly higher in A 1 AR -/- mice than in the A 2A AR -/- and WT genotypes. The A 2A AR -/- demonstrated better preservation of OHC and afferent synapses and the minimal loss of SGN after noise exposure. The findings suggest that the loss of A 1 AR expression results in an increased susceptibility to cochlear neural injury and hearing loss, whilst absence of A 2A AR increases cochlear resistance to acoustic trauma. Copyright © 2016 Elsevier B.V. All rights reserved.

Effect of low-level laser treatment on cochlea hair-cell recovery after ototoxic hearing loss

NASA Astrophysics Data System (ADS)

Rhee, Chung-Ku; He, Peijie; Jung, Jae Yun; Ahn, Jin-Chul; Chung, Phil-Sang; Lee, Min Young; Suh, Myung-Whan

2013-12-01

The primary cause of hearing loss includes damage to cochlear hair cells. Low-level laser therapy (LLLT) has become a popular treatment for damaged nervous systems. Based on the idea that cochlea hair cells and neural cells are from same developmental origin, the effect of LLLT on hearing loss in animal models is evaluated. Hearing loss animal models were established, and the animals were irradiated by 830-nm diode laser once a day for 10 days. Power density of the laser treatment was 900 mW/cm2, and the fluence was 162 to 194 J. The tympanic membrane was evaluated after LLLT. Thresholds of auditory brainstem responses were evaluated before treatment, after gentamicin, and after 10 days of LLLT. Quantitative scanning electron microscopic (SEM) observations were done by counting remaining hair cells. Tympanic membranes were intact at the end of the experiment. No adverse tissue reaction was found. On SEM images, LLLT significantly increased the number of hair cells in middle and basal turns. Hearing was significantly improved by laser irradiation. After LLLT treatment, both the hearing threshold and hair-cell count significantly improved.

A novel KCNQ4 one-base deletion in a large pedigree with hearing loss: implication for the genotype-phenotype correlation.

PubMed

Kamada, Fumiaki; Kure, Shigeo; Kudo, Takayuki; Suzuki, Yoichi; Oshima, Takeshi; Ichinohe, Akiko; Kojima, Kanako; Niihori, Tetsuya; Kanno, Junko; Narumi, Yoko; Narisawa, Ayumi; Kato, Kumi; Aoki, Yoko; Ikeda, Katsuhisa; Kobayashi, Toshimitsu; Matsubara, Yoichi

2006-01-01

Autosomal-dominant, nonsyndromic hearing impairment is clinically and genetically heterogeneous. We encountered a large Japanese pedigree in which nonsyndromic hearing loss was inherited in an autosomal-dominant fashion. A genome-wide linkage study indicated linkage to the DFNA2 locus on chromosome 1p34. Mutational analysis of KCNQ4 encoding a potassium channel revealed a novel one-base deletion in exon 1, c.211delC, which generated a profoundly truncated protein without transmembrane domains (p.Q71fsX138). Previously, six missense mutations and one 13-base deletion, c.211_223del, had been reported in KCNQ4. Patients with the KCNQ4 missense mutations had younger-onset and more profound hearing loss than patients with the 211_223del mutation. In our current study, 12 individuals with the c.211delC mutation manifested late-onset and pure high-frequency hearing loss. Our results support the genotype-phenotype correlation that the KCNQ4 deletions are associated with later-onset and milder hearing impairment than the missense mutations. The phenotypic difference may be caused by the difference in pathogenic mechanisms: haploinsufficiency in deletions and dominant-negative effect in missense mutations.

Defects in middle ear cavitation cause conductive hearing loss in the Tcof1 mutant mouse.

PubMed

Richter, Carol A; Amin, Susan; Linden, Jennifer; Dixon, Jill; Dixon, Michael J; Tucker, Abigail S

2010-04-15

Conductive hearing loss (CHL) is one of the most common forms of human deafness. Despite this observation, a surprising gap in our understanding of the mechanisms underlying CHL remains, particularly with respect to the molecular mechanisms underlying middle ear development and disease. Treacher Collins syndrome (TCS) is an autosomal dominant disorder of facial development that results from mutations in the gene TCOF1. CHL is a common feature of TCS but the causes of the hearing defect have not been studied. In this study, we have utilized Tcof1 mutant mice to dissect the developmental mechanisms underlying CHL. Our results demonstrate that effective cavitation of the middle ear is intimately linked to growth of the auditory bulla, the neural crest cell-derived structure that encapsulates all middle ear components, and that defects in these processes have a profoundly detrimental effect on hearing. This research provides important insights into a poorly characterized cause of human deafness, and provides the first mouse model for the study of middle ear cavity defects, while also being of direct relevance to a human genetic disorder.

Effects of Smoking on Eustachian Tube and Hearing.

PubMed

Pezzoli, Matteo; Lofaro, Denise; Oliva, Alessandro; Orione, Monica; Cupi, Daniela; Albera, Andrea; Bongioannini, Guido; Albera, Roberto

2017-12-01

The purpose of this study was to evaluate the effect of tobacco use on the Eustachian tube and inner ear function. Case-control study. Thirty-one nonsmoking volunteers and 34 smoking subjects recruited in an University Hospital, submitted to an audiological evaluation including pure tone audiometry, basal tympanogram, stapedial reflexes analysis, and nine-step eustachian tube (ET) function test. Pure Tone Average (PTA) threshold at all frequencies tested was 12.5 dB in smokers and 3.7 in nonsmoking subjects. Nine smokers (27%) presented some degree of hearing loss versus none in the nonsmoker group. Linear regression analysis showed a higher degree of sensorineural hearing loss with age in smokers. Among the smokers, 20 subjects (59%) presented an impaired tubal function for the nine-step inflation/deflation tympanometric test, while only 6 (19%) subjects in the group of nonsmokers showed a tubal dysfunction. Tobacco use may reduce the ability to hear, mainly causing a sensorineural hearing loss for higher frequencies. We also found the presence of a high number of smokers suffering from tubal dysfunction. This has an important clinical relevance, not only because smoking increases the incidence of middle ear diseases, but also because tubal dysfunction may cause nonspecific symptoms characterised by ear fullness and difficulties in middle ear equalisation.

Mutation Analysis of the Common Deafness Genes in Patients with Nonsyndromic Hearing Loss in Linyi by SNPscan Assay.

PubMed

Zhang, Fengguo; Xiao, Yun; Xu, Lei; Zhang, Xue; Zhang, Guodong; Li, Jianfeng; Lv, Huaiqing; Bai, Xiaohui; Wang, Haibo

2016-01-01

Hearing loss is a common sensory disorder, and at least 50% of cases are due to a genetic etiology. Although hundreds of genes have been reported to be associated with nonsyndromic hearing loss, GJB2, SLC26A4, and mtDNA12SrRNA are the major contributors. However, the mutation spectrum of these common deafness genes varies among different ethnic groups. The present work summarized mutations in these three genes and their prevalence in 339 patients with nonsyndromic hearing loss at three different special education schools and one children's hospital in Linyi, China. A new multiplex genetic screening system "SNPscan assay" was employed to detect a total of 115 mutations of the above three genes. Finally, 48.67% of the patients were identified with hereditary hearing loss caused by mutations in GJB2, SLC26A4, and mtDNA12SrRNA. The carrying rate of mutations in the three genes was 37.76%, 19.75%, and 4.72%, respectively. This mutation profile in our study is distinct from other parts of China, with high mutation rate of GJB2 suggesting a unique mutation spectrum in this area.

Audiologic characteristics in a sample of recently-separated military Veterans: The Noise Outcomes in Servicemembers Epidemiology Study (NOISE Study).

PubMed

Gordon, J S; Griest, S E; Thielman, E J; Carlson, K F; Helt, W J; Lewis, M S; Blankenship, C; Austin, D; Theodoroff, S M; Henry, J A

2017-06-01

Military Service Members are often exposed to high levels of occupational noise, solvents, and other exposures that can be damaging to the auditory system. Little is known about hearing loss and how it progresses in Veterans following military service. This epidemiology study is designed to evaluate and monitor a cohort of Veterans for 20 years or more to determine how hearing loss changes over time and how those changes are related to noise exposure and other ototoxic exposures encountered during military service. Data reported here are from baseline assessments of the first 100 study participants (84 males; 16 females; mean age 33.5 years; SD 8.8; range 21-58). Each participant was asked to complete a comprehensive audiologic examination and self-report questionnaires regarding sociodemographic characteristics, noise and solvent exposures, health conditions common among post-deployment Veterans, and the social and emotional consequences of hearing loss. For this relatively young cohort, 29% exhibited hearing loss, defined as average hearing threshold >20 dB HL in the conventional audiometric range. Forty-two percent exhibited hearing loss in the extended-high-frequency audiometric range using the same criterion (average hearing threshold >20 dB HL). Certain factors were found to be associated with poorer hearing in both conventional and extended-high-frequency ranges, including age, type of military branch, years of military service, number of military deployments, noise exposure, tinnitus, and a positive screen for post-traumatic stress disorder. Although the majority of participants had hearing within normal limits, 27% reported a self-perceived mild/moderate hearing handicap and 14% reported a significant handicap. Further research is needed to identify a cause for this discrepancy in audiologic results versus self-report. The information obtained from this longitudinal study could be used in future resource planning with the goal of preventing, as much as possible, the development of hearing loss during military service, and the exacerbation of prevalent hearing loss after military service and over Veterans' lifetimes. Published by Elsevier B.V.

Congenital cytomegalovirus infection as a cause of sensorineural hearing loss in a highly immune population.

PubMed

Yamamoto, Aparecida Y; Mussi-Pinhata, Marisa Marcia; Isaac, Myriam de Lima; Amaral, Fabiana R; Carvalheiro, Cristina G; Aragon, Davi C; Manfredi, Alessandra K da Silva; Boppana, Suresh B; Britt, William J

2011-12-01

The burden of congenital cytomegalovirus (CMV)-associated sensorineural hearing loss (SNHL) in populations with CMV seroprevalence approaching 100% is unknown. The purpose of this study was to assess the rate, associated factors, and predictors of SNHL in CMV-infected infants identified by newborn screening in a highly seropositive maternal population. Newborns with positive saliva CMV-DNA that was confirmed by virus isolation in the first 2 weeks of life were enrolled in a prospective follow-up study to monitor hearing outcome. Of 12,195 infants screened, 121 (1%) were infected with CMV and 12 (10%) had symptomatic infection at birth. Hearing function could be assessed in 102/121 children who underwent at least one auditory brainstem evoked response testing at a median age of 12 months. SNHL was observed in 10/102 (9.8%; 95% confidence interval: 5.1-16.7) children. Median age at the latest hearing evaluation was 47 months (12-84 months). Profound loss (>90 dB) was found in 4/5 children with bilateral SNHL while all 5 children with unilateral loss had moderate to severe deficit. The presence of symptomatic infection at birth (odds ratio, 38.1; 95% confidence interval: 1.6-916.7) was independently associated with SNHL after adjusting for intrauterine growth restriction, gestational age, gravidity, and maternal age. Among 10 infants with SNHL, 6 (60%) were born to mothers with nonprimary CMV infection. Even in populations with near universal immunity to CMV, congenital CMV infection is a significant cause of SNHL demonstrating the importance of CMV as a major cause of SNHL in children worldwide. As in other populations, SNHL is more frequently observed in symptomatic CMV infection.

Pejvakin, a Candidate Stereociliary Rootlet Protein, Regulates Hair Cell Function in a Cell-Autonomous Manner

PubMed Central

Kazmierczak, Piotr; Harris, Suzan L.; Shah, Prahar; Puel, Jean-Luc; Lenoir, Marc

2017-01-01

Mutations in the Pejvakin (PJVK) gene are thought to cause auditory neuropathy and hearing loss of cochlear origin by affecting noise-induced peroxisome proliferation in auditory hair cells and neurons. Here we demonstrate that loss of pejvakin in hair cells, but not in neurons, causes profound hearing loss and outer hair cell degeneration in mice. Pejvakin binds to and colocalizes with the rootlet component TRIOBP at the base of stereocilia in injectoporated hair cells, a pattern that is disrupted by deafness-associated PJVK mutations. Hair cells of pejvakin-deficient mice develop normal rootlets, but hair bundle morphology and mechanotransduction are affected before the onset of hearing. Some mechanotransducing shorter row stereocilia are missing, whereas the remaining ones exhibit overextended tips and a greater variability in height and width. Unlike previous studies of Pjvk alleles with neuronal dysfunction, our findings reveal a cell-autonomous role of pejvakin in maintaining stereocilia architecture that is critical for hair cell function. SIGNIFICANCE STATEMENT Two missense mutations in the Pejvakin (PJVK or DFNB59) gene were first identified in patients with audiological hallmarks of auditory neuropathy spectrum disorder, whereas all other PJVK alleles cause hearing loss of cochlear origin. These findings suggest that complex pathogenetic mechanisms underlie human deafness DFNB59. In contrast to recent studies, we demonstrate that pejvakin in auditory neurons is not essential for normal hearing in mice. Moreover, pejvakin localizes to stereociliary rootlets in hair cells and is required for stereocilia maintenance and mechanosensory function of the hair bundle. Delineating the site of the lesion and the mechanisms underlying DFNB59 will allow clinicians to predict the efficacy of different therapeutic approaches, such as determining compatibility for cochlear implants. PMID:28209736

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

PubMed

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

2016-04-01

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

Cochlear implantation in patients with bilateral cochlear trauma.

PubMed

Serin, Gediz Murat; Derinsu, Ufuk; Sari, Murat; Gergin, Ozgül; Ciprut, Ayça; Akdaş, Ferda; Batman, Cağlar

2010-01-01

Temporal bone fracture, which involves the otic capsule, can lead to complete loss of auditory and vestibular functions, whereas the patients without fractures may experience profound sensorineural hearing loss due to cochlear concussion. Cochlear implant is indicated in profound sensorineural hearing loss due to cochlear trauma but who still have an intact auditory nerve. This is a retrospective review study. We report 5 cases of postlingually deafened patients caused by cochlear trauma, who underwent cochlear implantation. Preoperative and postoperative hearing performance will be presented. These patients are cochlear implanted after the cochlear trauma in our department between 2001 and 2006. All patients performed very well with their implants, obtained open-set speech understanding. They all became good telephone users after implantation. Their performance in speech understanding was comparable to standard postlingual adult patients implanted. Cochlear implantation is an effective aural rehabilitation in profound sensorineural hearing loss caused by temporal bone trauma. Preoperative temporal bone computed tomography, magnetic resonance imaging, and promontorium stimulation testing are necessary to make decision for the surgery and to determine the side to be implanted. Surgery could be challenging and complicated because of anatomical irregularity. Moreover, fibrosis and partial or total ossification within the cochlea must be expected. Copyright 2010. Published by Elsevier Inc.

Autoinflammatory Diseases

MedlinePlus

... damage. Vision loss. Hearing loss. Mental retardation. Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS) is associated ... red spots on the skin caused by burst blood vessels (purpura). Joint pain. Permanent shortening of a muscle ...

Inner ear symptoms and disease: Pathophysiological understanding and therapeutic options

PubMed Central

Ciuman, Raphael R.

2013-01-01

In recent years, huge advances have taken place in understanding of inner ear pathophysiology causing sensorineural hearing loss, tinnitus, and vertigo. Advances in understanding comprise biochemical and physiological research of stimulus perception and conduction, inner ear homeostasis, and hereditary diseases with underlying genetics. This review describes and tabulates the various causes of inner ear disease and defines inner ear and non-inner ear causes of hearing loss, tinnitus, and vertigo. The aim of this review was to comprehensively breakdown this field of otorhinolaryngology for specialists and non-specialists and to discuss current therapeutic options in distinct diseases and promising research for future therapies, especially pharmaceutic, genetic, or stem cell therapy. PMID:24362017

Comparison of Cochlear Cell Death Caused by Cisplatin, Alone and in Combination with Furosemide

PubMed Central

Xia, Li; Chen, Zhengnong; Su, Kaiming; Yin, Shankai; Wang, Jian

2014-01-01

Establishment of appropriate animal models is an important step in exploring the mechanisms of drug-induced ototoxicity. In the present study, using guinea pigs we compared cochlear lesions induced by cisplatin administered in two regimens: consecutive application alone and in combination with furosemide. The effects of furosemide alone were also evaluated; it was found to cause temporary hearing loss and reversible damage to the stria vascularis. Consecutive application of cisplatin alone appeared to be disadvantageous because it resulted in progressive body weight loss and higher mortality compared to the combined regimen, which used a smaller cisplatin dose. The combined regimen resulted in comparable hearing loss and hair cell loss but a markedly lower mortality. However, their coadministration failed to cause similar damage to spiral ganglion neurons (SGN), as seen in animals that received cisplatin alone. This difference suggests that the combined regimen did not mimic the damage to cochlear neuronal innervation caused by the clinical application of cisplatin. The difference also suggests that the SGN lesion is not caused by cisplatin entering the cochlea via the stria vascularis. PMID:23548607

Ear tube insertion

MedlinePlus

... eardrum may cause some hearing loss. But most children do not have long-term damage to their hearing or speech, even when the ... not go away with treatment, or if a child has many ear infections ... or that damages nearby nerves Injury to the ear after sudden ...

Recent Advances in Cerebellar Ischemic Stroke Syndromes Causing Vertigo and Hearing Loss.

PubMed

Kim, Hyun-Ah; Yi, Hyon-Ah; Lee, Hyung

2016-12-01

Cerebellar ischemic stroke is one of the common causes of vascular vertigo. It usually accompanies other neurological symptoms or signs, but a small infarct in the cerebellum can present with vertigo without other localizing symptoms. Approximately 11 % of the patients with isolated cerebellar infarction simulated acute peripheral vestibulopathy, and most patients had an infarct in the territory of the medial branch of the posterior inferior cerebellar artery (PICA). A head impulse test can differentiate acute isolated vertigo associated with PICA territory cerebellar infarction from more benign disorders involving the inner ear. Acute hearing loss (AHL) of a vascular cause is mostly associated with cerebellar infarction in the territory of the anterior inferior cerebellar artery (AICA), but PICA territory cerebellar infarction rarely causes AHL. To date, at least eight subgroups of AICA territory infarction have been identified according to the pattern of neurotological presentations, among which the most common pattern of audiovestibular dysfunction is the combined loss of auditory and vestibular functions. Sometimes acute isolated audiovestibular loss can be the initial symptom of impending posterior circulation ischemic stroke (particularly within the territory of the AICA). Audiovestibular loss from cerebellar infarction has a good long-term outcome than previously thought. Approximately half of patients with superior cerebellar artery territory (SCA) cerebellar infarction experienced true vertigo, suggesting that the vertigo and nystagmus in the SCA territory cerebellar infarctions are more common than previously thought. In this article, recent findings on clinical features of vertigo and hearing loss from cerebellar ischemic stroke syndrome are summarized.

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

Toluene-induced hearing loss in the guinea pig.

PubMed

Waniusiow, Delphine; Campo, Pierre; Venet, Thomas; Cossec, Benoît; Cosnier, Frédéric; Beydon, Dominique; Rieger, Benoît; Burgart, Manuella; Ferrari, Luc; Parietti-Winkler, Cécile

2009-10-01

Toluene is a high-production industrial solvent, which can disrupt the auditory system in rats. However, toluene-induced hearing loss is species dependent. For instance, despite long-lasting exposures to high concentrations of aromatic solvent, no study has yet succeeded in causing convincing hearing loss in the guinea pig. This latter species can be characterized by two metabolic particularities: a high amount of hepatic cytochrome P-450s (P-450s) and a high concentration of glutathione in the cochlea. It is therefore likely that the efficiency of both the hepatic and cochlear metabolisms plays a key role in the innocuousness of the hearing of guinea pigs to exposure to solvent. The present study was carried out to test the auditory resistance to toluene in glutathione-depleted guinea pigs whose the P-450 activity was partly inhibited. To this end, animals on a low-protein diet received a general P-450 inhibitor, namely SKF525-A. Meanwhile, they were exposed to 1750 ppm toluene for 4 weeks, 5 days/week, 6 h/day. Auditory function was tested by electrocochleography and completed by histological analyses. For the first time, a significant toluene-induced hearing loss was provoked in the P-450-inhibited guinea pigs. However, the ototoxic process caused by the solvent exposure was different from that observed in the rat. Only the stria vascularis and the spiral fibers were disrupted in the apical coil of the cochlea. The protective mechanisms developed by guinea pigs are discussed in the present publication.

Using software to predict occupational hearing loss in the mining industry.

PubMed

Azman, A S; Li, M; Thompson, J K

2016-01-01

Powerful mining systems typically generate high-level noise that can damage the hearing ability of miners. Engineering noise controls are the most desirable and effective control for overexposure to noise. However, the effects of these noise controls on the actual hearing status of workers are not easily measured. A tool that can provide guidance in assigning workers to jobs based on the noise levels to which they will be exposed is highly desirable. Therefore, the Pittsburgh Mining Research Division (PMRD) of the U.S. National Institute for Occupational Safety and Health (NIOSH) developed a tool to estimate in a systematic way the hearing loss due to occupational noise exposure and to evaluate the effectiveness of developed engineering controls. This computer program is based on the ISO 1999 standard and can be used to estimate the loss of hearing ability caused by occupational noise exposures. In this paper, the functionalities of this software are discussed and several case studies related to mining machinery are presented to demonstrate the functionalities of this software.

Phenotypical features of two patients diagnosed with PHARC syndrome and carriers of a new homozygous mutation in the ABHD12 gene.

PubMed

Frasquet, Marina; Lupo, Vincenzo; Chumillas, María José; Vázquez-Costa, Juan Francisco; Espinós, Carmen; Sevilla, Teresa

2018-04-15

PHARC (Polyneuropathy, Hearing loss, Ataxia, Retinitis pigmentosa and Cataracts) (MIM# 612674) is an autosomal recessive neurodegenerative disease caused by mutations in the ABHD12 gene. We evaluated two Spanish siblings affected with pes cavus, sensorimotor neuropathy, hearing loss, retinitis pigmentosa and juvenile cataracts in whom the genetic test of ABHD12 revealed a novel homozygous frameshift mutation, c.211_223del (p.Arg71Tyrfs*26). The earliest clinical manifestation in these patients was a demyelinating neuropathy manifested with a Charcot-Marie-Tooth phenotype over three decades. Progressive hearing loss, cataracts and retinitis pigmentosa appeared after the age of 30. We herein describe the complete clinical picture of these two patients, and focus particularly on neuropathy characteristics. This study supports the fact that although PHARC is rare, its phenotype is very characteristic and we should include its study in patients affected with demyelinating polyneuropathy, hearing loss and retinopathy. Copyright © 2018 Elsevier B.V. All rights reserved.

[Subclinical sensorineural hearing loss in female patients with rheumatoid arthritis].

PubMed

Treviño-González, José Luis; Villegas-González, Mario Jesús; Muñoz-Maldonado, Gerardo Enrique; Montero-Cantu, Carlos Alberto; Nava-Zavala, Arnulfo Hernán; Garza-Elizondo, Mario Alberto

2015-01-01

The rheumatoid arthritis is a clinical entity capable to cause hearing impairment that can be diagnosed promptly with high frequencies audiometry. To detect subclinical sensorineural hearing loss in patients with rheumatoid arthritis. Cross-sectional study on patients with rheumatoid arthritis performing high frequency audiometry 125Hz to 16,000Hz and tympanometry. The results were correlated with markers of disease activity and response to therapy. High frequency audiometry was performed in 117 female patients aged from 19 to 65 years. Sensorineural hearing loss was observed at a sensitivity of pure tones from 125 to 8,000 Hz in 43.59%, a tone threshold of 10,000 to 16,000Hz in 94.02% patients in the right ear and in 95.73% in the left ear. Hearing was normal in 8 (6.84%) patients. Hearing loss was observed in 109 (93.16%), and was asymmetric in 36 (30.77%), symmetric in 73 (62.37%), bilateral in 107 (91.45%), unilateral in 2 (1.71%), and no conduction and/or mixed hearing loss was encountered. Eight (6.83%) patients presented vertigo, 24 (20.51%) tinnitus. Tympanogram type A presented in 88.90% in the right ear and 91.46% in the left ear, with 5.98 to 10.25% type As. Stapedius reflex was present in 75.3 to 85.2%. Speech discrimination in the left ear was significantly different (p = 0.02)in the group older than 50 years. No association was found regarding markers of disease activity, but there was an association with the onset of rheumatoid arthritis disease. Patients with rheumatoid arthritis had a high prevalence of sensorineural hearing loss for high and very high frequencies. Copyright © 2015 Academia Mexicana de Cirugía A.C. Published by Masson Doyma México S.A. All rights reserved.

Exome Sequencing Identifies a Founder Frameshift Mutation in an Alternative Exon of USH1C as the Cause of Autosomal Recessive Retinitis Pigmentosa with Late-Onset Hearing Loss

PubMed Central

Khateb, Samer; Zelinger, Lina; Ben-Yosef, Tamar; Crystal-Shalit, Ornit; Gross, Menachem; Banin, Eyal; Sharon, Dror

2012-01-01

We used a combined approach of homozygosity mapping and whole exome sequencing (WES) to search for the genetic cause of autosomal recessive retinitis pigmentosa (arRP) in families of Yemenite Jewish origin. Homozygosity mapping of two arRP Yemenite Jewish families revealed a few homozygous regions. A subsequent WES analysis of the two index cases revealed a shared homozygous novel nucleotide deletion (c.1220delG) leading to a frameshift (p.Gly407Glufs*56) in an alternative exon (#15) of USH1C. Screening of additional Yemenite Jewish patients revealed a total of 16 homozygous RP patients (with a carrier frequency of 0.008 in controls). Funduscopic and electroretinography findings were within the spectrum of typical RP. While other USH1C mutations usually cause Usher type I (including RP, vestibular dysfunction and congenital deafness), audiometric screening of 10 patients who are homozygous for c.1220delG revealed that patients under 40 years of age had normal hearing while older patients showed mild to severe high tone sensorineural hearing loss. This is the first report of a mutation in a known USH1 gene that causes late onset rather than congenital sensorineural hearing loss. The c.1220delG mutation of USH1C accounts for 23% of RP among Yemenite Jewish patients in our cohort. PMID:23251578

Missense mutation in the USH2A gene: association with recessive retinitis pigmentosa without hearing loss.

PubMed

Rivolta, C; Sweklo, E A; Berson, E L; Dryja, T P

2000-06-01

Microdeletions Glu767(1-bp del), Thr967(1-bp del), and Leu1446(2-bp del) in the human USH2A gene have been reported to cause Usher syndrome type II, a disorder characterized by retinitis pigmentosa (RP) and mild-to-severe hearing loss. Each of these three frameshift mutations is predicted to lead to an unstable mRNA transcript that, if translated, would result in a truncated protein lacking the carboxy terminus. Here, we report Cys759Phe, a novel missense mutation in this gene that changes an amino-acid residue within the fifth laminin-epidermal growth factor-like domain of the USH2A gene and that is associated with recessive RP without hearing loss. This single mutation was found in 4.5% of 224 patients with recessive RP, suggesting that USH2A could cause more cases of nonsyndromic recessive RP than does any other gene identified to date.

Sonar-induced temporary hearing loss in dolphins

PubMed Central

Mooney, T. Aran; Nachtigall, Paul E.; Vlachos, Stephanie

2009-01-01

There is increasing concern that human-produced ocean noise is adversely affecting marine mammals, as several recent cetacean mass strandings may have been caused by animals' interactions with naval ‘mid-frequency’ sonar. However, it has yet to be empirically demonstrated how sonar could induce these strandings or cause physiological effects. In controlled experimental studies, we show that mid-frequency sonar can induce temporary hearing loss in a bottlenose dolphin (Tursiops truncatus). Mild-behavioural alterations were also associated with the exposures. The auditory effects were induced only by repeated exposures to intense sonar pings with total sound exposure levels of 214 dB re: 1 μPa2 s. Data support an increasing energy model to predict temporary noise-induced hearing loss and indicate that odontocete noise exposure effects bear trends similar to terrestrial mammals. Thus, sonar can induce physiological and behavioural effects in at least one species of odontocete; however, exposures must be of prolonged, high sound exposures levels to generate these effects. PMID:19364712

Activation of SIRT3 by the NAD⁺ precursor nicotinamide riboside protects from noise-induced hearing loss.

PubMed

Brown, Kevin D; Maqsood, Sadia; Huang, Jing-Yi; Pan, Yong; Harkcom, William; Li, Wei; Sauve, Anthony; Verdin, Eric; Jaffrey, Samie R

2014-12-02

Intense noise exposure causes hearing loss by inducing degeneration of spiral ganglia neurites that innervate cochlear hair cells. Nicotinamide adenine dinucleotide (NAD(+)) exhibits axon-protective effects in cultured neurons; however, its ability to block degeneration in vivo has been difficult to establish due to its poor cell permeability and serum instability. Here, we describe a strategy to increase cochlear NAD(+) levels in mice by administering nicotinamide riboside (NR), a recently described NAD(+) precursor. We find that administration of NR, even after noise exposure, prevents noise-induced hearing loss (NIHL) and spiral ganglia neurite degeneration. These effects are mediated by the NAD(+)-dependent mitochondrial sirtuin, SIRT3, since SIRT3-overexpressing mice are resistant to NIHL and SIRT3 deletion abrogates the protective effects of NR and expression of NAD(+) biosynthetic enzymes. These findings reveal that administration of NR activates a NAD(+)-SIRT3 pathway that reduces neurite degeneration caused by noise exposure. Copyright © 2014 Elsevier Inc. All rights reserved.

Identifying Early Onset of Hearing Loss in Young Adults With Diabetes Mellitus Type 2 Using High Frequency Audiometry.

PubMed

Vignesh, S S; Jaya, V; Moses, Anand; Muraleedharan, A

2015-09-01

Diabetes mellitus (DM) is a metabolic disorder caused by hyperglycemia which leads to dysfunction of various organs. Hearing acuity is equally hindered by this disorder. Among individuals with DM audiological characteristics of DM type 1 are of great concern in the literature. This study aims at establishing high frequency audiometry (HFA) as a useful tool in identifying early onset of hearing loss in individuals with DM type 2. 20 non-diabetic participants and 20 individuals with DM type 2 in the age range of 20-40 years were considered for the study. Subjects in both groups underwent otoscopic examination, PTA at 0.25, 0.5, 1, 2, 4 and 8 kHz and HFA at 9, 10, 11.2, 12.5, 14 and 16 kHz. Results revealed statistically significant difference in thresholds of both PTA and HFA at all frequencies across the group, but the mean threshold difference between the diabetic and non-diabetic group was marked in HFA than in PTA. In the diabetic subjects the thresholds of PTA was within 25 dBHL at all frequencies when compared to the thresholds of HFA. Individuals with DM type 2 showed bilateral symmetrical mild hearing loss in HFA and the hearing loss increased with ascending test frequencies from 9,000 to 16,000 Hz. Mild hearing loss in HFA is an indicator for early onset of hearing loss in DM type 2. Hence this present study emphasis the clinical utility of HFA in young adults with DM type 2.

Hearing Status in Pediatric Renal Transplant Recipients.

PubMed

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

2015-08-01

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

SLC44A4 mutation causes autosomal dominant hereditary postlingual non-syndromic mid-frequency hearing loss.

PubMed

Ma, Zhaoxin; Xia, Wenjun; Liu, Fei; Ma, Jing; Sun, Shaoyang; Zhang, Jin; Jiang, Nan; Wang, Xu; Hu, Jiongjiong; Ma, Duan

2017-01-15

Clinical, genetic, and functional investigations were performed to identify the causative mutation in a distinctive Chinese family with postlingual non-syndromic mid-frequency sensorineural hearing loss. Whole-exome sequencing revealed SLC44A4, which encodes the choline transport protein, as the pathogenic gene in this family. In the zebrafish model, downregulation of slc44a4 using morpholinos led to significant abnormalities in the zebrafish inner ear and lateral line neuromasts and contributed, to some extent, to disabilities in hearing and balance. SH-SY5Y cells transfected with SLC44A4 showed higher choline uptake and acetylcholine release than that of cells transfected with mutant SLC44A4. We concluded that mutation of SLC44A4 may cause defects in the Choline- acetylcholine system, which is crucial to the efferent innervation of hair cells in the olivocochlear bundle for the maintenance of physiological function of outer hair cells and the protection of hair cells from acoustic injury, leading to hearing loss. © The Author 2016. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

JS-X syndrome: A multiple congenital malformation with vocal cord paralysis, ear deformity, hearing loss, shoulder musculature underdevelopment, and X-linked recessive inheritance.

PubMed

Hoeve, Hans L J; Brooks, Alice S; Smit, Liesbeth S

2015-07-01

We report on a family with a not earlier described multiple congenital malformation. Several male family members suffer from laryngeal obstruction caused by bilateral vocal cord paralysis, outer and middle ear deformity with conductive and sensorineural hearing loss, facial dysmorphisms, and underdeveloped shoulder musculature. The affected female members only have middle ear deformity and hearing loss. The pedigree is suggestive of an X-linked recessive inheritance pattern. SNP-array revealed a deletion and duplication on Xq28 in the affected family members. A possible aetiology is a neurocristopathy with most symptoms expressed in structures derived from branchial arches. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Sector Retinitis Pigmentosa Associated With Novel Compound Heterozygous Mutations of CDH23.

PubMed

Branson, Sara V; McClintic, Jedediah I; Stamper, Tara H; Haldeman-Englert, Chad R; John, Vishak J

2016-02-01

Usher syndrome is an autosomal recessive condition characterized by retinitis pigmentosa (RP) and congenital hearing loss, with or without vestibular dysfunction. Allelic variants of CDH23 cause both Usher syndrome type 1D (USH1D) and a form of nonsyndromic hearing loss (DFNB12). The authors describe here a 34-year-old patient with congenital hearing loss and a new diagnosis of sector RP who was found to have two novel compound heterozygous mutations in CDH23, including one missense (c.8530C > A; p.Pro2844Thr) and one splice-site (c.5820 + 5G > A) mutation. This is the first report of sector RP associated with these types of mutations in CDH23. Copyright 2016, SLACK Incorporated.

A Challenge for Cochlear Implantation: Duplicated Internal Auditory Canal.

PubMed

Binnetoğlu, Adem; Bağlam, Tekin; Sarı, Murat; Gündoğdu, Yavuz; Batman, Çağlar

2016-08-01

Duplication of the internal auditory canal is an uncommon, congenital malformation that can be associated with sensorineural hearing loss owing to aplasia/hypoplasia of the vestibulocochlear nerve. Only 14 such cases have been reported to date. We report the case of a 13-month-old girl with bilateral, congenital, sensorineural hearing loss caused by narrow, duplicated internal auditory canals and discuss the challenges encountered in the diagnosis and treatment of this condition.

[Tuberculous chronic otitis media. A clinical case].

PubMed

Pérez Fernández, C A; Armengot Canceller, M; Campos Catalá, A; Abril, V; Calabuig Crespo, M C; Basterra Alegría, J

2000-01-01

Tuberculous otitis media is a rare cause of chronic suppurative infection of the middle ear and mastoid. Patients typically have chronic tympanic membrane perforation and ear discharge associated with progressive, profound hearing loss, and resistance to antibiotic treatment. Diagnosis is often delayed by a low clinical suspicion, thus leading to complications such as irreversible hearing loss and facial nerve paralysis. Histological examination of a biopsy specimen reveals tuberculous changes. The disease is treated with antituberculosis agents.

Performance Assessment of the 3M Combat Arms Generation 4.0 Tactical Military Shooter’s Ear Plug

DTIC Science & Technology

2016-06-24

blast , impulsive noise attenuation, REAT, IPIL 16. SECURITY CLASSIFICATION OF: 17. LIMITATION OF ABSTRACT 18. NUMBER OF PAGES 19a. NAME OF...fire could eventually result in permanent hearing loss. Noise exposures from larger weapons and blasts could instantly cause permanent hearing loss... noise . The general approach for this assessment was to use American National Standards Institute (ANSI) measurement procedures for continuous

Knowledge and Cultural Beliefs of Mothers Regarding the Risk Factors of Infant Hearing Loss and Awareness of Audiology Services

PubMed Central

Govender, Samantha M.; Khan, Nasim Banu

2017-01-01

The aim of the paper is to describe the knowledge of mothers in Durban, South Africa, regarding risk factors of hearing loss in infants and their awareness of audiology services, and to describe their cultural beliefs about the risk factors for hearing loss in infants. A descriptive survey design with quantitative methods of analysis were used. Conveniently sampled mothers (n=102) receiving postnatal care for their infants from eight provincial clinics within Durban consented to participate, yielding a response rate of 48%. A questionnaire was used to collect the data and the Cronbach α was calculated yielding a score of 0.835, indicating good internal consistency and reliability of the questionnaire. Sixty percent of the mothers were aware of risk factors, such as middle ear infections, ototoxic medication and consumption of alcohol during pregnancy. Seventy percent were unaware that NICU/mechanical ventilation for more than 5 days, prematurity, rubella and jaundice are considered risk factors for hearing loss, implying a need to create awareness amongst mothers regarding such risk factors. Sixty percent (n=62) believed that bewitchment and ancestral curses can cause hearing loss. Cultural beliefs were associated with hearing loss, therefore, health professionals need to demonstrate cultural competence when providing audiology services, especially in a culturally and linguistically diverse countries such as South Africa. Although the mothers had an average knowledge about risk factors, two thirds did not know which professional to seek help from. There is a need to create awareness amongst mothers regarding the risk factors of infant hearing loss as well as audiology services in order to facilitate early detection and intervention. There is a need for health professionals to demonstrate cultural competence when working with their patients. PMID:28890772

Knowledge and Cultural Beliefs of Mothers Regarding the Risk Factors of Infant Hearing Loss and Awareness of Audiology Services.

PubMed

Govender, Samantha M; Khan, Nasim Banu

2017-06-23

The aim of the paper is to describe the knowledge of mothers in Durban, South Africa, regarding risk factors of hearing loss in infants and their awareness of audiology services, and to describe their cultural beliefs about the risk factors for hearing loss in infants. A descriptive survey design with quantitative methods of analysis were used. Conveniently sampled mothers (n=102) receiving postnatal care for their infants from eight provincial clinics within Durban consented to participate, yielding a response rate of 48%. A questionnaire was used to collect the data and the Cronbach α was calculated yielding a score of 0.835, indicating good internal consistency and reliability of the questionnaire. Sixty percent of the mothers were aware of risk factors, such as middle ear infections, ototoxic medication and consumption of alcohol during pregnancy. Seventy percent were unaware that NICU/mechanical ventilation for more than 5 days, prematurity, rubella and jaundice are considered risk factors for hearing loss, implying a need to create awareness amongst mothers regarding such risk factors. Sixty percent (n=62) believed that bewitchment and ancestral curses can cause hearing loss. Cultural beliefs were associated with hearing loss, therefore, health professionals need to demonstrate cultural competence when providing audiology services, especially in a culturally and linguistically diverse countries such as South Africa. Although the mothers had an average knowledge about risk factors, two thirds did not know which professional to seek help from. There is a need to create awareness amongst mothers regarding the risk factors of infant hearing loss as well as audiology services in order to facilitate early detection and intervention. There is a need for health professionals to demonstrate cultural competence when working with their patients.

[Hearing loss and idoneity--the segnalation of noise-induced hearing loss hearing Loss].

PubMed

Albera, Roberto; Dagna, Federico; Cassandro, Claudia; Canale, Andrea

2011-01-01

Work idoneity in hearing loss must be related to working ability and evolution risks. Working ability is referred to the difficulties found in speech comprehension and in signals perception. As regards hearing loss evolution it is necessary to define if the subject is affected by conductive or neurosensorial hearing loss. In conductive hearing loss it is necessary to evaluate entity and frequential distribution of the deficit. In neurosensorial hearing loss it is necessary to distinguish between noise-induced hearing loss and extraprofessional hearing loss. In noise-induced hearing loss the evolution risk is high if the noise exposure is less than 10-15 years or the actual noise exposure is louder than the former. In case of extraprofessional hearing loss the evolution risk is higher in presbycusis, endolymphatic hydrops and toxic hearing loss. The necessity to report the presence on professionale noise-induced hearing loss arises if audiometric threshold is more than 25 dB at 0.5-1-2-3-4 kHz and if it is verified the professional origine of hearing loss.

Vertigo-associated disorders

MedlinePlus

... in the part of the inner ear that controls balance. These areas are called the vestibular labyrinth or ... Dizziness Hearing loss in one ear Loss of balance (may cause falls) Ringing in the ears If you have vertigo ...

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

PubMed

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

2012-01-01

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

Hearing Loss and Quality of Life (QOL) among Human Immunodeficiency Virus (HIV)-Infected and Uninfected Adults

PubMed Central

Duong, N; Torre, P; Springer, G; Cox, C; Plankey, MW

2017-01-01

Objective Research has established that human immunodeficiency virus (HIV) causes hearing loss. Studies have yet to evaluate the impact on quality of life (QOL). This project evaluates the effect of hearing loss on QOL by HIV status. Methods The study participants were from the Multicenter AIDS Cohort Study (MACS) and the Women's Interagency HIV study (WIHS). A total of 248 men and 127 women participated. Pure-tone air conduction thresholds were collected for each ear at frequencies from 250 through 8000 Hz. Pure-tone averages (PTAs) for each ear were calculated as the mean of air conduction thresholds in low frequencies (i.e., 250, 500, 1000 and 2000 Hz) and high frequencies (i.e., 3000, 4000, 6000 and 8000 Hz). QOL data were gathered with the Short Form 36 Health Survey and Medical Outcome Study (MOS)-HIV instrument in the MACS and WIHS, respectively. A median regression analysis was performed to test the association of PTAs with QOL by HIV status. Results There was no significant association between hearing loss and QOL scores at low and high pure tone averages in HIV positive and negative individuals. HIV status, HIV biomarkers and treatment did not change the lack of association of low and high pure tone averages with poorer QOL. Conclusion Although we did not find a statistically significant association of hearing loss with QOL by HIV status, testing for hearing loss with aging and recommending treatment may offset any presumed later life decline in QOL. PMID:28217403

Can You Hear Me Now: The Leading Army Injury & Disability

DTIC Science & Technology

2011-03-07

99 An acute onset of noise induced hearing loss should be treated as an emergency. Prompt management of improving the blood flow to the inner ear...antioxidants to alleviate cochlear damage caused by noise and ototoxicity; supplements could provide an additional layer of protection.117...various compounds used for hearing protection ranging from antioxidants that scavenge free radicals to agents that increase blood flow . Effective

Coping together with hearing loss: a qualitative meta-synthesis of the psychosocial experiences of people with hearing loss and their communication partners.

PubMed

Barker, Alex B; Leighton, Paul; Ferguson, Melanie A

2017-05-01

To examine the psychosocial experiences of hearing loss from the perspectives of both the person with hearing loss and their communication partner. A meta-synthesis of the qualitative literature. From 880 records, 12 qualitative papers met the inclusion criteria, (i) adults with hearing loss, communication partners, or both, and (ii) explored psychosocial issues. Four themes related to the psychosocial experience of hearing loss were found, (i) the effect of the hearing loss, (ii) the response to hearing aids, (iii) stigma and identity, and (iv) coping strategies. Hearing loss affected both people with hearing loss and communication partners. Hearing aids resulted in positive effects, however, these were often outnumbered by negative effects. Non-use of hearing aids was often influenced by stigma. Coping strategies used were related to how the person with hearing loss perceived their self and how the communication partner perceived the relationship. Aligned coping strategies appeared to have a positive effect. Hearing loss affects both people with hearing loss and their communication partners. Aligned coping strategies can facilitate adjustment to hearing loss.

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

PubMed

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

2002-12-07

Usher syndrome is an autosomal recessively inherited disease, characterised by sensorineural hearing loss, tapetoretinal degeneration and in some cases vestibular problems. Based on the clinical heterogeneity, the disease can be classified into three clinical types (I, II and III), which have their own genetic subtypes (Usher 1A-Usher IG, Usher 2A-Usher 2C and Usher 3). The majority of the Usher type I cases are caused by mutations in the MYO7A gene (Usher 1B) while mutations in the USH2A gene (Usher 2A) are the cause of most cases of type II. Usher syndrome type III, caused by mutations in the USH3 gene, is frequently seen only in Finland.

Audiovestibular consequences of congenital cytomegalovirus infection.

PubMed

Teissier, N; Bernard, S; Quesnel, S; Van Den Abbeele, T

2016-12-01

Congenital cytomegalovirus (CMV) infection is the second most frequent cause of mental retardation and sensorineural hearing loss, after genetic factors. Recently, pediatric forensic and fetopathological studies have led to progress in understanding the pathophysiological mechanisms underlying the various neurosensory sequelae. Thanks to the identification of certain prognostic factors of hearing loss, therapeutic protocols based on antiviral molecules are now proposed for target populations. This treatment has shown efficacy in limiting hearing threshold deterioration and even, in some cases, seems to provide partial recovery of hearing in symptomatic congenitally infected CMV neonates. However, optimal treatment duration and administration modalities are not clearly defined. This article reviews recent data concerning audiovestibular sequelae and their management in children congenitally infected by CMV. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Influence of implantable hearing aids and neuroprosthesison music perception.

PubMed

Rahne, Torsten; Böhme, Lars; Götze, Gerrit

2012-01-01

The identification and discrimination of timbre are essential features of music perception. One dominating parameter within the multidimensional timbre space is the spectral shape of complex sounds. As hearing loss interferes with the perception and enjoyment of music, we approach the individual timbre discrimination skills in individuals with severe to profound hearing loss using a cochlear implant (CI) and normal hearing individuals using a bone-anchored hearing aid (Baha). With a recent developed behavioral test relying on synthetically sounds forming a spectral continuum, the timbre difference was changed adaptively to measure the individual just noticeable difference (JND) in a forced-choice paradigm. To explore the differences in timbre perception abilities caused by the hearing mode, the sound stimuli were varied in their fundamental frequency, thus generating different spectra which are not completely covered by a CI or Baha system. The resulting JNDs demonstrate differences in timbre perception between normal hearing individuals, Baha users, and CI users. Beside the physiological reasons, also technical limitations appear as the main contributing factors.

Public awareness of audiology, hearing and hearing health in the Limpopo Province, South Africa

PubMed Central

Sebothoma, Ben; Kgare, Khomotjo S.

2017-01-01

Background The burden of hearing loss is on the increase, especially in low-income countries such as South Africa. The need for urgent action to prevent ear and hearing problems is a priority, especially as in many cases permanent hearing loss is preventable. In South Africa, as in other developing countries, there is a limited number of hearing health professionals and audiological resources. The lack of hearing health services may impact the general public’s awareness of hearing and hearing health. Limited information is available on the South African public’s knowledge of audiologists and the services they provide, especially in underserved rural communities. Aim The aim of this study was to describe individuals’ awareness of the audiology profession, hearing and hearing loss, and hearing health in a rural area of the Limpopo Province. Method A cross-sectional survey design was employed for the purpose of this study. Using a random sampling strategy, 297 households in four rural villages were selected and a self-developed questionnaire was administered to one individual (18 years and older) per household. The questionnaire consisted of 23 questions targeting awareness of the audiology profession, as well as knowledge of hearing, hearing loss and hearing health. Results Only 14% of participants were aware of the audiology profession, indicating that individuals living in rural communities are not aware of the role of audiologists and the services they provide. Doctors and nurses were identified by participants as the individuals who assist them with hearing-related problems. Although most participants (87%) acknowledged that it is very important to undergo a hearing test, only 5% have previously visited an audiologist. Most participants were aware that ear infections and excessive noise exposure can cause hearing loss. The majority also believed that ears must be kept clean at all times and used cotton buds to maintain ear hygiene. Conclusion There is a general lack of public awareness of audiologists and the services they offer. This study highlighted the need for the National Department of Health in collaboration with professional associations and hearing health professionals to develop and implement effective strategies to increase the South African public’s awareness of the profession and the services they provide. South African universities can also play a significant role in teaching students to develop context-relevant strategies to increase awareness of the profession. PMID:29041791

Novel myosin mutations for hereditary hearing loss revealed by targeted genomic capture and massively parallel sequencing

PubMed Central

Brownstein, Zippora; Abu-Rayyan, Amal; Karfunkel-Doron, Daphne; Sirigu, Serena; Davidov, Bella; Shohat, Mordechai; Frydman, Moshe; Houdusse, Anne; Kanaan, Moien; Avraham, Karen B

2014-01-01

Hereditary hearing loss is genetically heterogeneous, with a large number of genes and mutations contributing to this sensory, often monogenic, disease. This number, as well as large size, precludes comprehensive genetic diagnosis of all known deafness genes. A combination of targeted genomic capture and massively parallel sequencing (MPS), also referred to as next-generation sequencing, was applied to determine the deafness-causing genes in hearing-impaired individuals from Israeli Jewish and Palestinian Arab families. Among the mutations detected, we identified nine novel mutations in the genes encoding myosin VI, myosin VIIA and myosin XVA, doubling the number of myosin mutations in the Middle East. Myosin VI mutations were identified in this population for the first time. Modeling of the mutations provided predicted mechanisms for the damage they inflict in the molecular motors, leading to impaired function and thus deafness. The myosin mutations span all regions of these molecular motors, leading to a wide range of hearing phenotypes, reinforcing the key role of this family of proteins in auditory function. This study demonstrates that multiple mutations responsible for hearing loss can be identified in a relatively straightforward manner by targeted-gene MPS technology and concludes that this is the optimal genetic diagnostic approach for identification of mutations responsible for hearing loss. PMID:24105371

The R245X mutation of PCDH15 in Ashkenazi Jewish children diagnosed with nonsyndromic hearing loss foreshadows retinitis pigmentosa.

PubMed

Brownstein, Zippora; Ben-Yosef, Tamar; Dagan, Orit; Frydman, Moshe; Abeliovich, Dvorah; Sagi, Michal; Abraham, Fabian A; Taitelbaum-Swead, Riki; Shohat, Mordechai; Hildesheimer, Minka; Friedman, Thomas B; Avraham, Karen B

2004-06-01

Usher syndrome is a frequent cause of the combination of deafness and blindness due to retinitis pigmentosa (RP). Five genes are known to underlie different forms of Usher syndrome type I (USH1). In the Ashkenazi Jewish population, the R245X mutation of the PCDH15 gene may be the most common cause of USH1 (Ben-Yosef T, Ness SL, Madeo AC, Bar-Lev A, Wolfman JH, Ahmed ZM, Desnick RK, Willner JP, Avraham KB, Ostrer H, Oddoux C, Griffith AJ, Friedman TB N Engl J Med 348: 1664-1670, 2003). To estimate what percentage of Ashkenazi Jewish children born with profound hearing loss will develop RP due to R245X, we examined the prevalence of the R245X PCDH15 mutation and its carrier rate among Ashkenazi Jews in Israel. Among probands diagnosed with nonsyndromic hearing loss not due to mutations of connexin 26 (GJB2) and/or connexin 30 (GJB6), and below the age of 10, 2 of 20 (10%) were homozygous for the R245X mutation. Among older nonsyndromic deaf individuals, no homozygotes were detected, although one individual was heterozygous for R245X. The carrier rate of the R245X mutation among the normal hearing Ashkenazi population in Israel was estimated at 1%. Ashkenazi Jewish children with profound prelingual hearing loss should be evaluated for the R245X PCDH15 mutation and undergo ophthalmologic evaluation to determine whether they will develop RP. Rehabilitation can then begin before loss of vision. Early use of cochlear implants in such cases may rescue these individuals from a dual neurosensory deficit.

The Novel Mouse Mutation Oblivion Inactivates the PMCA2 Pump and Causes Progressive Hearing Loss

PubMed Central

de Angelis, Martin Hrabé; Fuchs, Helmut; Lim, Dmitry; Ortolano, Saida; Ingham, Neil J.; Brini, Marisa; Carafoli, Ernesto; Mammano, Fabio; Steel, Karen P.

2008-01-01

Progressive hearing loss is common in the human population, but we have few clues to the molecular basis. Mouse mutants with progressive hearing loss offer valuable insights, and ENU (N-ethyl-N-nitrosourea) mutagenesis is a useful way of generating models. We have characterised a new ENU-induced mouse mutant, Oblivion (allele symbol Obl), showing semi-dominant inheritance of hearing impairment. Obl/+ mutants showed increasing hearing impairment from post-natal day (P)20 to P90, and loss of auditory function was followed by a corresponding base to apex progression of hair cell degeneration. Obl/Obl mutants were small, showed severe vestibular dysfunction by 2 weeks of age, and were completely deaf from birth; sensory hair cells were completely degenerate in the basal turn of the cochlea, although hair cells appeared normal in the apex. We mapped the mutation to Chromosome 6. Mutation analysis of Atp2b2 showed a missense mutation (2630C→T) in exon 15, causing a serine to phenylalanine substitution (S877F) in transmembrane domain 6 of the PMCA2 pump, the resident Ca2+ pump of hair cell stereocilia. Transmembrane domain mutations in these pumps generally are believed to be incompatible with normal targeting of the protein to the plasma membrane. However, analyses of hair cells in cultured utricular maculae of Obl/Obl mice and of the mutant Obl pump in model cells showed that the protein was correctly targeted to the plasma membrane. Biochemical and biophysical characterisation showed that the pump had lost a significant portion of its non-stimulated Ca2+ exporting ability. These findings can explain the progressive loss of auditory function, and indicate the limits in our ability to predict mechanism from sequence alone. PMID:18974863

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)

Superficial Siderosis of the Central Nervous System Caused by Hemorrhagic Intraventricular Craniopharyngioma: Case Report and Literature Review

PubMed Central

TOSAKA, Masahiko; SATO, Koji; AMANUMA, Makoto; HIGUCHI, Tetsuya; ARAI, Motohiro; AISHIMA, Kaoru; SHIMIZU, Tatsuya; HORIGUCHI, Keishi; SUGAWARA, Kenichi; YOSHIMOTO, Yuhei

2015-01-01

Superficial siderosis is a rare condition caused by hemosiderin deposits in the central nervous system (CNS) due to prolonged or recurrent low-grade bleeding into the cerebrospinal fluid (CSF). CNS tumor could be one of the sources of bleeding, both pre- and postoperatively. We report an extremely rare case of superficial siderosis associated with purely third ventricle craniopharyngioma, and review previously reported cases of superficial siderosis associated with CNS tumor. A 69-year-old man presented with headache, unsteady gait, blurred vision, and progressive hearing loss. Brain magnetic resonance (MR) imaging with gadolinium revealed a well enhanced, intraventricular mass in the anterior part of the third ventricle. T2*-weighted gradient echo (GE) MR imaging revealed a hypointense rim around the brain particularly marked within the depth of the sulci. Superficial siderosis was diagnosed based on these findings. The tumor was diffusely hypointense on T2*-weighted GE imaging, indicating intratumoral hemorrhage. The lateral ventricles were dilated, suggesting hydrocephalus. [18F]fluorodeoxyglucose positron emission tomography revealed increased uptake in the tumor. The whole brain surface appeared dark ocher at surgery. Histological examination showed the hemorrhagic tumor was papillary craniopharyngioma. His hearing loss progressed after removal of the tumor. T2*-weighted GE MR imaging demonstrated not only superficial siderosis but also diffuse intratumoral hemorrhage in the tumor. Superficial siderosis and its related symptoms, including hearing loss, should be considered in patients with hemorrhagic tumor related to the CSF space. Purely third ventricle craniopharyngioma rarely has hemorrhagic character, which could cause superficial siderosis and progressive hearing loss. PMID:24670310

A mutation causing Alport syndrome with tardive hearing loss is common in the western United States

DOE Office of Scientific and Technical Information (OSTI.GOV)

Barker, D.F.; Denison, J.C.; Atkin, C.L.

1996-06-01

Mutations in the COL4A5 gene, located at Xq22, cause Alport syndrome (AS), a nephritis characterized by progressive deterioration of the glomerular basement membrane and usually associated with progressive hearing loss. We have identified a novel mutation, L1649R, present in 9 of 121 independently ascertained families. Affected males shared the same haplotype of eight polymorphic markers tightly linked to COL4A5, indicating common ancestry. Genealogical studies place the birth of this ancestor >200 years ago. The L1649R mutation is a relatively common cause of Alport syndrome in the western United States, in part because of the rapid growth and migratory expansion ofmore » mid-nineteenth-century pioneer populations carrying the gene. L1649R affects a highly conserved residue in the NC1 domain, which is involved in key inter- and intramolecular interactions, but results in a relatively mild disease phenotype. Renal failure in an L1649R male typically occurs in the 4th or 5th decade and precedes the onset of significant hearing loss by {approximately}10 years. 45 refs., 5 figs.« less

Relationship Among Signal Fidelity, Hearing Loss, and Working Memory for Digital Noise Suppression.

PubMed

Arehart, Kathryn; Souza, Pamela; Kates, James; Lunner, Thomas; Pedersen, Michael Syskind

2015-01-01

This study considered speech modified by additive babble combined with noise-suppression processing. The purpose was to determine the relative importance of the signal modifications, individual peripheral hearing loss, and individual cognitive capacity on speech intelligibility and speech quality. The participant group consisted of 31 individuals with moderate high-frequency hearing loss ranging in age from 51 to 89 years (mean = 69.6 years). Speech intelligibility and speech quality were measured using low-context sentences presented in babble at several signal-to-noise ratios. Speech stimuli were processed with a binary mask noise-suppression strategy with systematic manipulations of two parameters (error rate and attenuation values). The cumulative effects of signal modification produced by babble and signal processing were quantified using an envelope-distortion metric. Working memory capacity was assessed with a reading span test. Analysis of variance was used to determine the effects of signal processing parameters on perceptual scores. Hierarchical linear modeling was used to determine the role of degree of hearing loss and working memory capacity in individual listener response to the processed noisy speech. The model also considered improvements in envelope fidelity caused by the binary mask and the degradations to envelope caused by error and noise. The participants showed significant benefits in terms of intelligibility scores and quality ratings for noisy speech processed by the ideal binary mask noise-suppression strategy. This benefit was observed across a range of signal-to-noise ratios and persisted when up to a 30% error rate was introduced into the processing. Average intelligibility scores and average quality ratings were well predicted by an objective metric of envelope fidelity. Degree of hearing loss and working memory capacity were significant factors in explaining individual listener's intelligibility scores for binary mask processing applied to speech in babble. Degree of hearing loss and working memory capacity did not predict listeners' quality ratings. The results indicate that envelope fidelity is a primary factor in determining the combined effects of noise and binary mask processing for intelligibility and quality of speech presented in babble noise. Degree of hearing loss and working memory capacity are significant factors in explaining variability in listeners' speech intelligibility scores but not in quality ratings.

Development of a Comprehensive Blast-Related Auditory Injury Database (BRAID)

DTIC Science & Technology

2016-05-01

these, may result in temporary or persistent noise-induced hear- ing loss (NIHL) and tinnitus . NIHL and tinnitus present a problematic public...uals deployed to battle zones are increasingly at risk for NIHL, tinnitus , and other otologic injuries [4]. The effects of blast exposure on the...primary interest because it may be permanent and cause persistent symptoms of tinnitus [18–20] and hear- ing loss [6,17,21–23]. Blast-related middle

Usher Syndrome

MedlinePlus

Usher syndrome is an inherited disease that causes serious hearing loss and retinitis pigmentosa, an eye disorder that causes ... and vision. There are three types of Usher syndrome: People with type I are deaf from birth ...

Next-generation sequencing identifies a novel compound heterozygous mutation in MYO7A in a Chinese patient with Usher Syndrome 1B.

PubMed

Wei, Xiaoming; Sun, Yan; Xie, Jiansheng; Shi, Quan; Qu, Ning; Yang, Guanghui; Cai, Jun; Yang, Yi; Liang, Yu; Wang, Wei; Yi, Xin

2012-11-20

Targeted enrichment and next-generation sequencing (NGS) have been employed for detection of genetic diseases. The purpose of this study was to validate the accuracy and sensitivity of our method for comprehensive mutation detection of hereditary hearing loss, and identify inherited mutations involved in human deafness accurately and economically. To make genetic diagnosis of hereditary hearing loss simple and timesaving, we designed a 0.60 MB array-based chip containing 69 nuclear genes and mitochondrial genome responsible for human deafness and conducted NGS toward ten patients with five known mutations and a Chinese family with hearing loss (never genetically investigated). Ten patients with five known mutations were sequenced using next-generation sequencing to validate the sensitivity of the method. We identified four known mutations in two nuclear deafness causing genes (GJB2 and SLC26A4), one in mitochondrial DNA. We then performed this method to analyze the variants in a Chinese family with hearing loss and identified compound heterozygosity for two novel mutations in gene MYO7A. The compound heterozygosity identified in gene MYO7A causes Usher Syndrome 1B with severe phenotypes. The results support that the combination of enrichment of targeted genes and next-generation sequencing is a valuable molecular diagnostic tool for hereditary deafness and suitable for clinical application. Copyright © 2012 Elsevier B.V. All rights reserved.

Hearing loss in children with e-waste lead and cadmium exposure.

PubMed

Liu, Yu; Huo, Xia; Xu, Long; Wei, Xiaoqin; Wu, Wengli; Wu, Xianguang; Xu, Xijin

2018-05-15

Environmental chemical exposure can cause neurotoxicity and has been recently linked to hearing loss in general population, but data are limited in early life exposure to lead (Pb) and cadmium (Cd) especially for children. We aimed to evaluate the association of their exposure with pediatric hearing ability. Blood Pb and urinary Cd were collected form 234 preschool children in 3-7years of age from an electronic waste (e-waste) recycling area and a reference area matched in Shantou of southern China. Pure-tone air conduction (PTA) was used to test child hearing thresholds at frequencies of 0.25, 0.5, 1, 2, 4 and 8kHz. A PTA≥25dB was defined as hearing loss. A higher median blood Pb level was found in the exposed group (4.94±0.20 vs 3.85±1.81μg/dL, p<0.001), while no significance was found for creatinine-adjusted Cd. Compared with the reference group, the exposed group had a higher prevalence of hearing loss (28.8% vs 13.6%, p<0.001). The PTA in the left, right and both ears, and hearing thresholds at average low and high frequency, and single frequency of 0.5, 1 and 2kHz were all increased in the exposed group. Positive correlations of child age and nail biting habit with Pb, and negative correlations of parent education level and child washing hands before dinner with Pb and Cd exposure were observed. Logistic regression analyses showed the adjusted OR of hearing loss for Pb exposure was 1.24 (95% CI: 1.029, 1.486). Our data suggest that early childhood exposure to Pb may be an important risk factor for hearing loss, and the developmental auditory system might be affected in e-waste polluted areas. Copyright © 2017 Elsevier B.V. All rights reserved.

Effects of noise-induced hearing loss on parvalbumin and perineuronal net expression in the mouse primary auditory cortex.

PubMed

Nguyen, Anna; Khaleel, Haroun M; Razak, Khaleel A

2017-07-01

Noise induced hearing loss is associated with increased excitability in the central auditory system but the cellular correlates of such changes remain to be characterized. Here we tested the hypothesis that noise-induced hearing loss causes deterioration of perineuronal nets (PNNs) in the auditory cortex of mice. PNNs are specialized extracellular matrix components that commonly enwrap cortical parvalbumin (PV) containing GABAergic interneurons. Compared to somatosensory and visual cortex, relatively less is known about PV/PNN expression patterns in the primary auditory cortex (A1). Whether changes to cortical PNNs follow acoustic trauma remains unclear. The first aim of this study was to characterize PV/PNN expression in A1 of adult mice. PNNs increase excitability of PV+ inhibitory neurons and confer protection to these neurons against oxidative stress. Decreased PV/PNN expression may therefore lead to a reduction in cortical inhibition. The second aim of this study was to examine PV/PNN expression in superficial (I-IV) and deep cortical layers (V-VI) following noise trauma. Exposing mice to loud noise caused an increase in hearing threshold that lasted at least 30 days. PV and PNN expression in A1 was analyzed at 1, 10 and 30 days following the exposure. No significant changes were observed in the density of PV+, PNN+, or PV/PNN co-localized cells following hearing loss. However, a significant layer- and cell type-specific decrease in PNN intensity was seen following hearing loss. Some changes were present even at 1 day following noise exposure. Attenuation of PNN may contribute to changes in excitability in cortex following noise trauma. The regulation of PNN may open up a temporal window for altered excitability in the adult brain that is then stabilized at a new and potentially pathological level such as in tinnitus. Copyright © 2017 Elsevier B.V. All rights reserved.

Habilitation of auditory and vestibular dysfunction.

PubMed

Snapp, Hillary A; Schubert, Michael C

2012-04-01

Although unilateral hearing loss is often the initial sign of vestibular schwannoma (VS), the pathogenesis of the associated structures within the cerebellopontine angle can result in vestibular, facial, or vascular symptoms. Removal of a VS causes deficits in hearing, balance, and gaze stability. The resulting hearing loss eliminates the benefits of binaural listening that provide localization, loudness summation, and listening-in-noise ability. Reduced balance and gaze stability increase fall risk. This review discusses modern treatment options for auditory and vestibular rehabilitation including contralateral routing of signals (CROS), bilateral CROS, bone-anchored implants, tinnitus management, gaze and gait stability exercises. Copyright © 2012 Elsevier Inc. All rights reserved.

Sound localization in noise in hearing-impaired listeners.

PubMed

Lorenzi, C; Gatehouse, S; Lever, C

1999-06-01

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

(De)stigmatizing the silent epidemic: representations of hearing loss in entertainment television.

PubMed

Foss, Katherine A

2014-01-01

The number of adolescents, young adults, and senior citizens experiencing hearing loss has significantly increased over the last 30 years. Despite this prevalence, hearing loss receives little attention in popular and political discourse, except in its connection to aging. Thus, hearing loss and the use of hearing aids have been stigmatized, discouraging adults from seeking hearing evaluation and screening, and justifying the lack of insurance coverage for hearing devices. This research explored how and why hearing loss continues to be stigmatized through a study of media messages about hearing loss. A textual analysis was conducted on 276 television episodes that involved d/Deaf characters and/or storylines about hearing loss and deafness from 1987 through 2013 (see Table 1). Only 11 fictional programs addressed the experience of hearing loss through 47 episodes, including Criminal Minds, Switched at Birth, House, M.D., and New Girl. Contrary to the assumption that hearing loss exclusively impacts older people, characters were typically young, attractive, working professionals who held prominent roles in the programs. For most characters, hearing loss developed suddenly and was restored by the end of the episode, with only four characters using hearing aids. Hearing loss was depicted as comical, embarrassing, lonely, and threatening to one's work. The scarcity of hearing loss portrayals, combined with the negative representations of hearing loss, could help explain why hearing loss continues to be stigmatized and overlooked, even though almost half of all Americans will eventually experience difficulty hearing.

Audiometric Characteristics of Hyperacusis Patients

PubMed Central

Sheldrake, Jacqueline; Diehl, Peter U.; Schaette, Roland

2015-01-01

Hyperacusis is a frequent auditory disorder where sounds of normal volume are perceived as too loud or even painfully loud. There is a high degree of co-morbidity between hyperacusis and tinnitus, most hyperacusis patients also have tinnitus, but only about 30–40% of tinnitus patients also show symptoms of hyperacusis. In order to elucidate the mechanisms of hyperacusis, detailed measurements of loudness discomfort levels (LDLs) across the hearing range would be desirable. However, previous studies have only reported LDLs for a restricted frequency range, e.g., from 0.5 to 4 kHz or from 1 to 8 kHz. We have measured audiograms and LDLs in 381 patients with a primary complaint of hyperacusis for the full standard audiometric frequency range from 0.125 to 8 kHz. On average, patients had mild high-frequency hearing loss, but more than a third of the tested ears had normal hearing thresholds (HTs), i.e., ≤20 dB HL. LDLs were found to be significantly decreased compared to a normal-hearing reference group, with average values around 85 dB HL across the frequency range. However, receiver operating characteristic analysis showed that LDL measurements are neither sensitive nor specific enough to serve as a single test for hyperacusis. There was a moderate positive correlation between HTs and LDLs (r = 0.36), i.e., LDLs tended to be higher at frequencies where hearing loss was present, suggesting that hyperacusis is unlikely to be caused by HT increase, in contrast to tinnitus for which hearing loss is a main trigger. Moreover, our finding that LDLs are decreased across the full range of audiometric frequencies, regardless of the pattern or degree of hearing loss, indicates that hyperacusis might be due to a generalized increase in auditory gain. Tinnitus on the other hand is thought to be caused by neuroplastic changes in a restricted frequency range, suggesting that tinnitus and hyperacusis might not share a common mechanism. PMID:26029161

Sound generator associated with the counseling in the treatment of tinnitus: evaluation of the effectiveness.

PubMed

Rocha, Andressa Vital; Mondelli, Maria Fernanda Capoani Garcia

The relations between the tinnitus and the hearing loss are due to the sensory deprivation caused by hearing loss, since this is followed by the functional and structural alteration of the auditory system as a whole. The cochlear lesions are accompanied by a reduction in the activity of the cochlear nerve, and the neural activity keeps increased in mainly all the central auditory nervous system to compensate this deficit. This study aimed to verify the effectiveness of the sound generator (SG) associated with the counseling in the treatment of the tinnitus in individuals with and without hearing loss regarding the improvement of the nuisance through Tinnitus Handicap Inventory (THI) and Visual Analogue Scale (VAS). The sample consisted of 30 individuals of both genders divided into two groups: Group 1 (G1) was comprised of 15 individuals with tinnitus and normal hearing, adapted to SG; Group 2 (G2) was comprised of 15 individuals with complaints of hearing acuity and tinnitus, adapted with SG and an individual hearing aid device (HA). Both groups underwent the following procedures: anamnesis and history of complaint, high frequency audiometry (HFA), imitanciometry, acuphenometry with the survey of psychoacoustic pitch and loudness thresholds and application of the tools THI and VAS. All of them were adapted with HA and Siemens SG and participated in a session of counseling. The individuals were assessed in three situations: initial assessment (before the adaptation of the HA and SG), monitoring and final assessment (6 months after adaptation). The comparison of the tinnitus nuisance and handicap in the three stages of assessment showed a significant improvement for both groups. The use of the SG was similarly effective in the treatment of the tinnitus in individuals with and without hearing loss, causing an improvement of the nuisance and handicap. Copyright © 2016 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

Influence of leisure-time noise on outer hair cell activity in medical students.

PubMed

Rosanowski, Frank; Eysholdt, Ulrich; Hoppe, Ulrich

2006-10-01

Noise exceeding a certain level can damage outer hair cells and thus cause hearing loss. In the past, noise-induced hearing loss was mainly caused by occupational noise. Leisure-time noise may be a promoting factor, particularly in young adults. The purpose of this study was to investigate whether transient evoked otoacoustic emissions (TEOAE) can be used to evaluate outer hair cell damage in young adults with no history of hearing complaints. The data obtained from the measurement of TEOAE were correlated with the participants' listening habits and exposure to leisure-time noise. Eighty-eight young adults (47 women, 41 men; age 22.9+/-2.9 years) were examined. TEOAE were measured using standard ILO 88 equipment. All participants had normal hearing (hearing thresholds better than 20 dB HL; frequency range 0.125-10 kHz). None of the participants suffered from permanent tinnitus. All participants answered a questionnaire concerning their listening habits. On average, the participants frequented a discotheque 1.4 times a month; 25% had never visited a discotheque, 35% visited once a month and 32% twice or three times a month. Sixteen per cent reported transient tinnitus after every visit to a discotheque and 58% after nearly every visit. Eight per cent suffered from transient hearing loss after every visit to a disco and 37% after nearly every visit. Three per cent (4%) reported tinnitus (nearly) every morning after visiting a discotheque. The TEOAE level was above 6 dB in all participants [9.2+/-3.6 dB (mean +/- SD)] and reproducibility was above 60% (90+/-9%). All values matched pass criteria for normal TEOAE under clinical conditions. However, TEOAE levels and reproducibility decreased significantly with an increased number of visits to discotheques. Outer hair cell damage could be measured using TEOAE in individuals exposed to leisure-time noise, although these individuals exhibited no measurable puretone hearing loss.

[Vestibulo-cochlear disturbances in the course of vaso-neural conflict of vestibule-cochlear nerve--diagnostic and therapeutic problems].

PubMed

Orendorz-Fraczkowska, Krystyna; Jaworska, Marzena; Gawron, Wojciech; Badowski, Roman; Nadolska, Beata

2007-01-01

Symptoms encompassing sensorineural hearing loss, tinnitus and vertigo occur in many diseases of various origin. The diagnostics in such cases is especially difficult and often requires interdisciplinary cooperation. Despite of that many cases remain unexplained. The two cases with above mentioned symptoms (52 year-old woman and 46 year-old man) with differentiated clinical course were presented. The woman for one year experienced left sided, extreme tinnitus with paroxysmal vertigo and dizziness. The man with sudden monolateral hearing loss and tinnitus that disappeared after corticosteroid therapy, complained about recurrence of fluctuating hearing loss and tinnitus accompanied by chronic instability. The diagnostics of hearing and balance organs was performed (pure tone audiometry, impedance audiometry, DPOAE, ABR, ENG) complemented with computed tomography and Nuclear Resonance. Female patient presented bilateral mild sensorineural hearing loss, more intensive on the left side, male patient right sided sensorineural hearing loss in the frequencies from 250 to 1500 Hz. The elongation of some peak values in ABR test (with bilaterally proper otoacoustic emissions) as well as partial canal paresis on the hearing loss side suggested primary diagnosis of neoplasmatic process. It was an indication to perform radiological diagnostics. The radiologic findings revealed the contact of vestibulo-cochlear nerve with the loop of cerebellar inferior anterior artery within the internal acoustic canal. The course of the disease and diagnostic tests do not provide characteristic data that let diagnose neuro-vascular conflict. One has to be aware of such possibility in patients with intensive vertigo and dizziness that do not improve after treatment when other causes do not justify the symptoms.

Delayed low frequency hearing loss caused by cochlear implantation interventions via the round window but not cochleostomy.

PubMed

Rowe, David; Chambers, Scott; Hampson, Amy; Eastwood, Hayden; Campbell, Luke; O'Leary, Stephen

2016-03-01

Cochlear implant recipients show improved speech perception and music appreciation when residual acoustic hearing is combined with the cochlear implant. However, up to one third of patients lose their pre-operative residual hearing weeks to months after implantation, for reasons that are not well understood. This study tested whether this "delayed" hearing loss was influenced by the route of electrode array insertion and/or position of the electrode array within scala tympani in a guinea pig model of cochlear implantation. Five treatment groups were monitored over 12 weeks: (1) round window implant; (2) round window incised with no implant; (3) cochleostomy with medially-oriented implant; (4) cochleostomy with laterally-oriented implant; and (5) cochleostomy with no implant. Hearing was measured at selected time points by the auditory brainstem response. Cochlear condition was assessed histologically, with cochleae three-dimensionally reconstructed to plot electrode paths and estimate tissue response. Electrode array trajectories matched their intended paths. Arrays inserted via the round window were situated nearer to the basilar membrane and organ of Corti over the majority of their intrascalar path compared with arrays inserted via cochleostomy. Round window interventions exhibited delayed, low frequency hearing loss that was not seen after cochleostomy. This hearing loss appeared unrelated to the extent of tissue reaction or injury within scala tympani, although round window insertion was histologically the most traumatic mode of implantation. We speculate that delayed hearing loss was related not to the electrode position as postulated, but rather to the muscle graft used to seal the round window post-intervention, by altering cochlear mechanics via round window fibrosis. Copyright © 2015 Elsevier B.V. All rights reserved.

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

PubMed

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

2009-07-01

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

Mutations in Cockayne Syndrome-Associated Genes (Csa and Csb) Predispose to Cisplatin-Induced Hearing Loss in Mice

PubMed Central

Rainey, Robert N.; Ng, Sum-yan; Llamas, Juan; van der Horst, Gijsbertus T. J.

2016-01-01

Cisplatin is a common and effective chemotherapeutic agent, yet it often causes permanent hearing loss as a result of sensory hair cell death. The causes of sensitivity to DNA-damaging agents in nondividing cell populations, such as cochlear hair and supporting cells, are poorly understood, as are the specific DNA repair pathways that protect these cells. Nucleotide excision repair (NER) is a conserved and versatile DNA repair pathway for many DNA-distorting lesions, including cisplatin-DNA adducts. Progressive sensorineural hearing loss is observed in a subset of NER-associated DNA repair disorders including Cockayne syndrome and some forms of xeroderma pigmentosum. We investigated whether either of the two overlapping branches that encompass NER, transcription-coupled repair or global genome repair, which are implicated in Cockayne syndrome and xeroderma pigmentosum group C, respectively, modulates cisplatin-induced hearing loss and cell death in the organ of Corti, the auditory sensory epithelium of mammals. We report that cochlear hair cells and supporting cells in transcription-coupled repair-deficient Cockayne syndrome group A (Csa−/−) and group B (Csb−/−) mice are hypersensitive to cisplatin, in contrast to global genome repair-deficient Xpc−/− mice, both in vitro and in vivo. We show that sensory hair cells in Csa−/− and Csb−/− mice fail to remove cisplatin-DNA adducts efficiently in vitro; and unlike Xpc−/− mice, Csa−/− and Csb−/− mice lose hearing and manifest outer hair cell degeneration after systemic cisplatin treatment. Our results demonstrate that Csa and Csb deficiencies predispose to cisplatin-induced hearing loss and hair/supporting cell damage in the mammalian organ of Corti, and emphasize the importance of transcription-coupled DNA repair in the protection against cisplatin ototoxicity. SIGNIFICANCE STATEMENT The utility of cisplatin in chemotherapy remains limited due to serious side effects, including sensorineural hearing loss. We show that mouse models of Cockayne syndrome, a progeroid disorder resulting from a defect in the transcription-coupled DNA repair (TCR) branch of nucleotide excision repair, are hypersensitive to cisplatin-induced hearing loss and sensory hair cell death in the organ of Corti, the mammalian auditory sensory epithelium. Our work indicates that Csa and Csb, two genes involved in TCR, are preferentially required to protect against cisplatin ototoxicity, relative to global genome repair-specific elements of nucleotide excision repair, and suggests that TCR is a major force maintaining DNA integrity in the cochlea. The Cockayne syndrome mice thus represent a model for testing the contribution of DNA repair mechanisms to cisplatin ototoxicity. PMID:27122034

Fish in a Dish: Drug Discovery for Hearing Habilitation.

PubMed

Esterberg, Robert; Coffin, Allison B; Ou, Henry; Simon, Julian A; Raible, David W; Rubel, Edwin W

2013-01-01

The majority of hearing loss is caused by the permanent loss of inner ear hair cells. The identification of drugs that modulate the susceptibility to hair cell loss or spur their regeneration is often hampered by the difficulties of assaying for such complex phenomena in mammalian models. The zebrafish has emerged as a powerful animal model for chemical screening in many contexts. Several characteristics of the zebrafish, such as its small size and external location of sensory hair cells, uniquely position it as an ideal model organism for the study of hair cell toxicity, protection, and regeneration. We have used this model to screen for drugs that affect each of these aspects of hair cell biology and have identified compounds that affect each of these processes. The identification of such drugs and drug-like compounds holds promise in the future ability to stem hearing loss in the human population.

Cytomegalovirus (CMV) Infection Causes Degeneration of Cochlear Vasculature and Hearing Loss in a Mouse Model.

PubMed

Carraro, Mattia; Almishaal, Ali; Hillas, Elaine; Firpo, Matthew; Park, Albert; Harrison, Robert V

2017-04-01

Cytomegalovirus (CMV) infection is one of the most common causes of congenital hearing loss in children. We have used a murine model of CMV infection to reveal functional and structural cochlear pathogenesis. The cerebral cortex of Balb/c mice (Mus musculus) was inoculated with 2000 pfu (plaque forming units) of murine CMV on postnatal day 3. At 6 weeks of age, cochlear function was monitored using auditory brainstem response (ABR) and distortion product otoacoustic emission (DPOAE) measures. Histological assessment of cochlear vasculature using a corrosion cast technique was made at 8 weeks. Vascular casts of mCMV-damaged cochleas, and those of untreated control animals, were examined using scanning electron microscopy. We find very large variations in the degree of vascular damage in animals given identical viral injections (2000 pfu). The primary lesion caused by CMV infection is to the stria vascularis and to the adjacent spiral limbus capillary network. Capillary beds of the spiral ligament are generally less affected. The initial vascular damage is found in the mid-apical turn and appears to progress to more basal cochlear regions. After viral migration to the inner ear, the stria vascularis is the primary affected structure. We suggest that initial auditory threshold losses may relate to the poor development or maintenance of the endocochlear potential caused by strial dysfunction. Our increased understanding of the pathogenesis of CMV-related hearing loss is important for defining methods for early detection and treatment.

Acoustic Issues in Human Spaceflight

NASA Technical Reports Server (NTRS)

Clark, Jonathan B.

2001-01-01

NASA is concerned about acute effect of sound on crew performance on International Space Station (ISS), and is developing strategies to assess and reduce acute, chronic, and delayed effects of sound. High noise levels can cause headaches, irritation, fatigue, impaired sleep, headache, and tinnitus and have resulted in an inability to hear alarms. Speech intelligibility may be more impaired for crew understanding non-native language in a noisy environment. No hearing loss occurred, but significant effects on crew performance and communication occurred. Permanent Threshold Shifts (PTS) have not been observed in the US shuttle program. Russian specification for noise in spacecraft is 60 dBA (awake) and 50 dBA (asleep) while the U.S. noise specification on ISS is NC 50 (awake) and NC 40 (asleep) with a 85 dBA hazard limit. Background noise levels of ISS modules have measured 56-69 dBA. Treadmill exercise operations measure 77 dBA. Alarms are required to be 20 dBA above ambient. Hearing protection is recommended when noise exceeds 60 dB 24 hour Leq. Countermeasures include hearing protection and design/ engineering controls. Advanced composite materials with excellent low frequency attenuation properties could be applied as a barrier protection around noisy equipment, or used on personal protective equipment worn by the crew. Hearing protection countermeasures include foam ear inserts, passive muff headsets, and active noise reduction headsets. Oto-acoustic emissions (OAE) could be used to monitor effectiveness of hearing protection countermeasures and tailor hearing protection countermeasures to individual crewmembers. Micro-gravity, vibration, toxic fumes, air quality/composition, stress, temperature, physical exertion or some combination of the above factors may have interacted with moderate long-term noise exposure to cause significant hearing loss. Longitudinal studies will need to address what co-morbidity factors, such as radiation, toxicology, microgravity effects (fluid shift), aging, are involved with hearing loss.

Impact of Aging and Cognition on Hearing Assistive Technology Use

PubMed Central

Jorgensen, Lindsey E.; Messersmith, Jessica J.

2015-01-01

Many factors go into appropriate recommendation and use of hearing assistive technology (HAT). The aging auditory system presents with its own complications and intricacies; there are many types of age-related hearing loss, and it is possible that the underlying cause of hearing loss can significantly impact the recommendations and performance with HATs. The audiologist should take into consideration peripheral and central auditory function when selecting HATs for the aging adult population as well as when selecting appropriate types of technology including personal sound amplification products, hearing aids, cochlear implants, and other assistive technology. The cognitive ability of the patient plays a central role in the recommendations of HAT. It is possible that the use of HATs could mitigate some of the effects of cognitive decline and thus should be considered as early as possible. Assessment of ability and appropriate recommendations are crucial to consistent use of HAT devices. PMID:27516716

Using software to predict occupational hearing loss in the mining industry

PubMed Central

Azman, A.S.; Li, M.; Thompson, J.K.

2017-01-01

Powerful mining systems typically generate high-level noise that can damage the hearing ability of miners. Engineering noise controls are the most desirable and effective control for overexposure to noise. However, the effects of these noise controls on the actual hearing status of workers are not easily measured. A tool that can provide guidance in assigning workers to jobs based on the noise levels to which they will be exposed is highly desirable. Therefore, the Pittsburgh Mining Research Division (PMRD) of the U.S. National Institute for Occupational Safety and Health (NIOSH) developed a tool to estimate in a systematic way the hearing loss due to occupational noise exposure and to evaluate the effectiveness of developed engineering controls. This computer program is based on the ISO 1999 standard and can be used to estimate the loss of hearing ability caused by occupational noise exposures. In this paper, the functionalities of this software are discussed and several case studies related to mining machinery are presented to demonstrate the functionalities of this software. PMID:28596700

Noise Levels Associated With New York City's Mass Transit Systems

PubMed Central

Gershon, Robyn R. M.; Zeltser, Marina; Canton, Allison; Akram, Muhammad

2009-01-01

Objectives. We measured noise levels associated with various forms of mass transit and compared them to exposure guidelines designed to protect against noise-induced hearing loss. Methods. We used noise dosimetry to measure time-integrated noise levels in a representative sample of New York City mass transit systems (subways, buses, ferries, tramway, and commuter railways) aboard transit vehicles and at vehicle boarding platforms or terminals during June and July 2007. Results. Of the transit types evaluated, subway cars and platforms had the highest associated equivalent continuous average (Leq) and maximum noise levels. All transit types had Leq levels appreciably above 70 A-weighted decibels, the threshold at which noise-induced hearing loss is considered possible. Conclusions. Mass transit noise exposure has the potential to exceed limits recommended by the World Health Organization and the US Environmental Protection Agency and thus cause noise-induced hearing loss among riders of all forms of mass transit given sufficient exposure durations. Environmental noise–control efforts in mass transit and, in cases in which controls are infeasible, the use of personal hearing protection would benefit the ridership's hearing health. PMID:19542046

A Comprehensive Study on the Etiology of Patients Receiving Cochlear Implantation With Special Emphasis on Genetic Epidemiology

PubMed Central

Miyagawa, Maiko; Nishio, Shin-Ya; Usami, Shin-Ichi

2016-01-01

Objective: Cochlear implantation is the most important treatment currently available for profound sensorineural hearing loss. The aim of this study was to investigate the etiology of hearing loss in patients with cochlear implantation, and to compare outcomes. Methods: Japanese hearing loss patients who received cochlear implants (CIs) or electric acoustic stimulation (EAS) in Shinshu University hospital (n = 173, prelingual onset: 92, postlingual onset: 81) participated in this study. Invader assay followed by the targeted exon-sequencing of 63 deafness genes using Massively parallel DNA sequencing (MPS) was applied. For prelingual patients, additional imaging examination, cCMV screening, and pediatric examination were performed for precise diagnosis. Results: Genetic screening successfully identified the causative mutation in 60% of patients with prelingual onset hearing loss and in 36% of those with postlingual hearing loss. Differences in the kinds of genes identified were observed between the two groups. Although there were marked variations in the outcome of cochlear implantation, patients with specific deafness gene mutations showed relatively good results. Conclusion: The present study showed genetic etiology is a major cause of hearing loss in CI/EAS patients. Patients possessing mutations in a number of deafness genes known to be expressed within inner ear have achieved satisfactory auditory performance, suggesting that the identification of the genetic background facilitates the prediction of post-CI performance. MPS is a powerful tool for the identification of causative deafness genes in patients receiving cochlear implantation. Therefore, determination of the involved region inside/outside of the cochlea by identification of the responsible gene is essential. PMID:26756145

[Strategy for minimally invasive cochlear implantation and residual hearing preservation].

PubMed

Huang, Y Y; Chen, J Y; Shen, M; Yang, J

2018-01-07

In the past few decades, considerable development was achieved in the cochlear implantation following the emergence of innovative electrode array and advances in minimally invasive surgery. Minimally invasive technique led to a better preservation of residual low-frequency hearing. The loss of residual hearing was caused by complicated factors. According to previous studies, a slower and stable speed of electrode insertion and the use of perioperative steroids were demonstrated to have a positive impact on hearing preservation. The selection of electrode array or its insertion approaches didn't show any distinctive benefits in hearing preservation.

A novel PAX3 mutation in a Japanese boy with Waardenburg syndrome type 1.

PubMed

Yoshida, Yu; Doi, Rieko; Adachi, Kaori; Nanba, Eiji; Kodani, Isamu; Ryoke, Kazuo

2016-01-01

Waardenburg syndrome type 1 (WS1) is a rare autosomal dominant disorder characterized by hair hypopigmentation, abnormal iris pigmentation, and congenital hearing loss. WS1 is caused by mutations in paired box gene 3 (PAX3). We identified a novel PAX3 mutation (c.1107 C>G, p.Ser369Arg) in a Japanese WS1 patient showing abnormal right iris pigmentation, right-sided congenital hearing loss, synophrys, incomplete left cleft lip, and cryptorchidism.

A novel PAX3 mutation in a Japanese boy with Waardenburg syndrome type 1

PubMed Central

Yoshida, Yu; Doi, Rieko; Adachi, Kaori; Nanba, Eiji; Kodani, Isamu; Ryoke, Kazuo

2016-01-01

Waardenburg syndrome type 1 (WS1) is a rare autosomal dominant disorder characterized by hair hypopigmentation, abnormal iris pigmentation, and congenital hearing loss. WS1 is caused by mutations in paired box gene 3 (PAX3). We identified a novel PAX3 mutation (c.1107 C>G, p.Ser369Arg) in a Japanese WS1 patient showing abnormal right iris pigmentation, right-sided congenital hearing loss, synophrys, incomplete left cleft lip, and cryptorchidism. PMID:27081571

Prevalence and causes of hearing impairment in Africa.

PubMed

Mulwafu, W; Kuper, H; Ensink, R J H

2016-02-01

To systematically assess the data on the prevalence and causes of hearing impairment in Africa. Systematic review on the prevalence and causes of hearing loss in Africa. We undertook a literature search of seven electronic databases (EMBASE, PubMed, Medline, Global Health, Web of Knowledge, Academic Search Complete and Africa Wide Information) and manually searched bibliographies of included articles. The search was restricted to population-based studies on hearing impairment in Africa. Data were extracted using a standard protocol. We identified 232 articles and included 28 articles in the final analysis. The most common cut-offs used for hearing impairment were 25 and 30 dB HL, but this ranged between 15 and 40 dB HL. For a cut-off of 25 dB, the median was 7.7% for the children- or school-based studies and 17% for population-based studies. For a cut-off of 30 dB HL, the median was 6.6% for the children or school-based studies and 31% for population-based studies. In schools for the deaf, the most common cause of hearing impairment was cryptogenic deafness (50%) followed by infectious causes (43%). In mainstream schools and general population, the most common cause of hearing impairment was middle ear disease (36%), followed by undetermined causes (35%) and cerumen impaction (24%). There are very few population-based studies available to estimate the prevalence of hearing impairment in Africa. Those studies that are available use different cut-offs, making comparison difficult. However, the evidence suggests that the prevalence of hearing impairment is high and that much of it is avoidable or treatable. © 2015 John Wiley & Sons Ltd.

Genetics Home Reference: hereditary sensory and autonomic neuropathy type IE

MedlinePlus

... loss of sensation in the feet and legs (peripheral neuropathy). People with HSAN IE develop hearing loss that ... control, become apparent before problems with thinking skills. Peripheral neuropathy is caused by impaired function of nerve cells ...

Molecular and clinical studies of X-linked deafness among Pakistani families.

PubMed

Waryah, Ali M; Ahmed, Zubair M; Bhinder, Munir A; Binder, Munir A; Choo, Daniel I; Sisk, Robert A; Shahzad, Mohsin; Khan, Shaheen N; Friedman, Thomas B; Riazuddin, Sheikh; Riazuddin, Saima

2011-07-01

There are 68 sex-linked syndromes that include hearing loss as one feature and five sex-linked nonsyndromic deafness loci listed in the OMIM database. The possibility of additional such sex-linked loci was explored by ascertaining three unrelated Pakistani families (PKDF536, PKDF1132 and PKDF740) segregating X-linked recessive deafness. Sequence analysis of POU3F4 (DFN3) in affected members of families PKDF536 and PKDF1132 revealed two novel nonsense mutations, p.Q136X and p.W114X, respectively. Family PKDF740 is segregating congenital blindness, mild-to-profound progressive hearing loss that is characteristic of Norrie disease (MIM#310600). Sequence analysis of NDP among affected members of this family revealed a novel single nucleotide deletion c.49delG causing a frameshift and premature truncation (p.V17fsX1) of the encoded protein. These mutations were not found in 150 normal DNA samples. Identification of pathogenic alleles causing X-linked recessive deafness will improve molecular diagnosis, genetic counseling and molecular epidemiology of hearing loss among Pakistanis.

Molecular and Clinical Studies of X-linked Deafness Among Pakistani Families

PubMed Central

Waryah, Ali M.; Ahmed, Zubair M.; Choo, Daniel I.; Sisk, Robert A.; Binder, Munir A.; Shahzad, Mohsin; Khan, Shaheen N.; Friedman, Thomas B.; Riazuddin, Sheikh; Riazuddin, Saima

2011-01-01

There are 68 sex-linked syndromes that include hearing loss as one feature and five sex-linked nonsyndromic deafness loci listed in the OMIM database. The possibility of additional such sex-linked loci was explored by ascertaining three unrelated Pakistani families (PKDF536, PKDF1132, PKDF740) segregating X-linked recessive deafness. Sequence analysis of POU3F4 (DFN3) in affected members of families PKDF536 and PKDF1132 revealed two novel nonsense mutations, p.Q136X and p.W114X, respectively. Family PKDF740 is segregating congenital blindness, mild to profound progressive hearing loss that is characteristic of Norrie disease (MIM#310600). Sequence analysis of NDP among affected members of this family revealed a novel single nucleotide deletion c.49delG causing a frameshift and premature truncation (p.V17fsX1) of the encoded protein. These mutations were not found in 150 normal DNA samples. Identification of pathogenic alleles causing X-linked recessive deafness will improve molecular diagnosis, genetic counseling, and molecular epidemiology of hearing loss among Pakistanis. PMID:21633365

The Physiological Bases of Hidden Noise-Induced Hearing Loss: Protocol for a Functional Neuroimaging Study

PubMed Central

Hall, Deborah A; Guest, Hannah; Prendergast, Garreth; Plack, Christopher J; Francis, Susan T

2018-01-01

Background Rodent studies indicate that noise exposure can cause permanent damage to synapses between inner hair cells and high-threshold auditory nerve fibers, without permanently altering threshold sensitivity. These demonstrations of what is commonly known as hidden hearing loss have been confirmed in several rodent species, but the implications for human hearing are unclear. Objective Our Medical Research Council–funded program aims to address this unanswered question, by investigating functional consequences of the damage to the human peripheral and central auditory nervous system that results from cumulative lifetime noise exposure. Behavioral and neuroimaging techniques are being used in a series of parallel studies aimed at detecting hidden hearing loss in humans. The planned neuroimaging study aims to (1) identify central auditory biomarkers associated with hidden hearing loss; (2) investigate whether there are any additive contributions from tinnitus or diminished sound tolerance, which are often comorbid with hearing problems; and (3) explore the relation between subcortical functional magnetic resonance imaging (fMRI) measures and the auditory brainstem response (ABR). Methods Individuals aged 25 to 40 years with pure tone hearing thresholds ≤20 dB hearing level over the range 500 Hz to 8 kHz and no contraindications for MRI or signs of ear disease will be recruited into the study. Lifetime noise exposure will be estimated using an in-depth structured interview. Auditory responses throughout the central auditory system will be recorded using ABR and fMRI. Analyses will focus predominantly on correlations between lifetime noise exposure and auditory response characteristics. Results This paper reports the study protocol. The funding was awarded in July 2013. Enrollment for the study described in this protocol commenced in February 2017 and was completed in December 2017. Results are expected in 2018. Conclusions This challenging and comprehensive study will have the potential to impact diagnostic procedures for hidden hearing loss, enabling early identification of noise-induced auditory damage via the detection of changes in central auditory processing. Consequently, this will generate the opportunity to give personalized advice regarding provision of ear defense and monitoring of further damage, thus reducing the incidence of noise-induced hearing loss. PMID:29523503

Overview on Deaf-Blindness

ERIC Educational Resources Information Center

Miles, Barbara

2008-01-01

It may seem that deaf-blindness refers to a total inability to see or hear. However, in reality deaf-blindness is a condition in which the combination of hearing and visual losses in children cause "such severe communication and other develop mental and educational needs that they cannot be accommodated in special education programs solely for…

Verbal Therapy with a Girl Who Could Not Hear

ERIC Educational Resources Information Center

Finney, Deborah A.

2006-01-01

This report considers the effective and varied components of treatment of an uncommon manifestation of conversion disorder in an adolescent girl. The patient was referred for treatment of hearing loss with no apparent organic cause. The treatment approach was multifaceted and based on general principles for treatment of conversion disorder in…

Occupationally-acquired noise-induced hearing loss: a senseless workplace hazard.

PubMed

Kurmis, Andrew P; Apps, Stacey A

2007-01-01

Occupational noise-induced hearing loss (ONIHL) describes an acquired hearing deficiency directly attributable to excessive workplace noise exposure. Data suggest that excessive noise attributes to approximately 37% of all adult causes of hearing loss and remains a significant contributor to employment-related morbidity internationally. Typically insidiously-acquired, often without frank progressive symptomatology, regional medical agencies continue to struggle with this potentially debilitating condition. The aim of the study was to provide a synopsis of the current understanding of ONIHL, its impact on individual workers and the wider international community, and to identify barriers to more uniform adoption of personal hearing protection. A review of the contemporary literature was performed using defined keyword searches and OVID, PubMed, and Google Scholar as primary electronic search engines. A number of published works were identified, describing aspects of the relationship between workplace-related noise exposure and subsequent development of employee hearing impairment, which demonstrate an overwhelming gender imbalance, with up to 97% of affected individuals being male. Industry-specific associations (e.g., mining, manufacturing and heavy construction) were well documented, as were links to toxin-specific exposures, in the recognized development of hearing loss. However, evidence of integration of appraisal of the topically-current area of genetic susceptibility was often lacking. Much discordance still exists among international agencies in the prescriptive regulation and enforcement of "safe" exposure limits. Despite a high level of public awareness regarding the importance of hearing preservation and increasingly stringent international occupational health, safety and welfare requirements mandating provision of safer work environments, ONIHL continues to be a significant occupational hazard. ONIHL is permanent and may cause significant disability, for which there currently exists no cure, but is largely overtly-preventable. The impact of ONIHL on the global transition toward dominant communication-rich white-collar employment roles is difficult to quantitate, but is likely to be substantive upon the afflicted individual. In the mainstream setting, exposure-avoidance strategies aimed to reduce the incidence of ONIHL remain the focus of public health and occupational medicine approaches.

Genetics of Hearing Loss

MedlinePlus

... Hearing Loss Homepage Basics Noise-Induced Hearing Loss Genetics of Hearing Loss Screening & Diagnosis Types of Hearing Loss About Sound Treatment & Intervention Services Learning Language Bacterial Meningitis Studies Data & Statistics EHDI Annual Data 2016 2015 2014 2013 ...

Hearing Loss in Children

MedlinePlus

... Hearing Loss Homepage Basics Noise-Induced Hearing Loss Genetics of Hearing Loss Screening & Diagnosis Types of Hearing Loss About Sound Treatment & Intervention Services Learning Language Bacterial Meningitis Studies Data & Statistics EHDI Annual Data 2016 2015 2014 2013 ...

PAX3 gene deletion detected by microarray analysis in a girl with hearing loss.

PubMed

Drozniewska, Malgorzata; Haus, Olga

2014-01-01

Deletions of the PAX3 gene have been rarely reported in the literature. Mutations of this gene are a common cause of Waardenburg syndrome type 1 and 3. We report a 16 year old female presenting hearing loss and normal intellectual development, without major features of Waardenburg syndrome type 1, and without family history of the syndrome. Her phenotype, however, overlaps with features of craniofacial-deafness-hand syndrome. Microarray analysis showed ~862 kb de novo deletion at 2q36.1 including PAX3. The above findings suggest that the rearrangement found in our patient appeared de novo and with high probability is a cause of her phenotype.

The clinical features and prognosis of mumps-associated hearing loss: a retrospective, multi-institutional investigation in Japan.

PubMed

Morita, Shinya; Fujiwara, Keishi; Fukuda, Atsushi; Fukuda, Satoshi; Nishio, Shin-Ya; Kitoh, Ryosuke; Hato, Naohito; Ikezono, Tetsuo; Ishikawa, Kotaro; Kaga, Kimitaka; Matsubara, Atsushi; Matsunaga, Tatsuo; Murata, Takaaki; Naito, Yasushi; Nishizaki, Kazunori; Ogawa, Kaoru; Sano, Hajime; Sato, Hiroaki; Sone, Michihiko; Suzuki, Mikio; Takahashi, Haruo; Tono, Tetsuya; Yamashita, Hiroshi; Yamasoba, Tatsuya; Usami, Shin-Ichi

2017-01-01

The majority of hearing loss due to mumps presents as unilateral profound sensorineural hearing loss, which is refractory to treatment. In rare cases of bilateral total deafness, cochlear implants were beneficial for speech perception. Vaccination against mumps is recommended to prevent mumps-associated hearing loss. The objective of this study is to investigate the clinical characteristics of hearing loss due to mumps and to evaluate hearing outcomes. The clinical parameters were analyzed under a retrospective multi-institutional study design in patients diagnosed with hearing loss due to mumps at the Otolaryngology departments of 19 hospitals between 1987 and 2016. Sixty-seven patients with hearing loss due to mumps were enrolled. The study population consisted of 35 males and 32 females, ranging in age from 1 to 54, with a median age of 9.5 years. Sixty-three patients presented with unilateral, and 4 with bilateral hearing loss. Profound hearing loss was observed in 65 ears. Only one ear with severe hearing loss showed complete recovery. Four patients with bilateral hearing loss received cochlear implant surgery. Most of the patients with hearing loss due to mumps had no history of vaccination.

No dramatic age-related loss of hair cells and spiral ganglion neurons in Bcl-2 over-expression mice or Bax null mice

PubMed Central

2010-01-01

Age-related decline of neuronal function is associated with age-related structural changes. In the central nervous system, age-related decline of cognitive performance is thought to be caused by synaptic loss instead of neuronal loss. However, in the cochlea, age-related loss of hair cells and spiral ganglion neurons (SGNs) is consistently observed in a variety of species, including humans. Since age-related loss of these cells is a major contributing factor to presbycusis, it is important to study possible molecular mechanisms underlying this age-related cell death. Previous studies suggested that apoptotic pathways were involved in age-related loss of hair cells and SGNs. In the present study, we examined the role of Bcl-2 gene in age-related hearing loss. In one transgenic mouse line over-expressing human Bcl-2, there were no significant differences between transgenic mice and wild type littermate controls in their hearing thresholds during aging. Histological analysis of the hair cells and SGNs showed no significant conservation of these cells in transgenic animals compared to the wild type controls during aging. These data suggest that Bcl-2 overexpression has no significant effect on age-related loss of hair cells and SGNs. We also found no delay of age-related hearing loss in mice lacking Bax gene. These findings suggest that age-related hearing loss is not through an apoptotic pathway involving key members of Bcl-2 family. PMID:20637089

Association of hearing loss with decreased employment and income among adults in the United States.

PubMed

Jung, David; Bhattacharyya, Neil

2012-12-01

We evaluated the association of hearing loss with employment and income in adults. Patients with a coded diagnosis of hearing loss were identified from the 2006 and 2008 Medical Expenditure Panel Survey linked household and medical conditions files and compared to patients without hearing loss. Differences in employment, wage income, and Supplemental Security Income were evaluated with multivariate regression models after adjustment for several demographic and Charlson comorbidity variables. An estimated 933,921 +/- 88,474 adults were identified with hearing loss (54.7% of whom were male; mean age for all, 51.0 years). Patients with hearing loss were more likely to be unemployed or partly unemployed than those without hearing loss (adjusted odds ratio, 2.2; p < 0.001). Similarly, adults with hearing loss were less likely to have any wage income than those without hearing loss (adjusted odds ratio, 2.5; p < 0.001). The population with hearing loss earned a mean wage of $23,481 +/- $3,366, versus $31,272 +/- $517 for the population without hearing loss (difference in wages, $7,791; p < 0.001). The association between hearing loss and receiving Supplemental Security Income was not significant (p = 0.109). Adults with hearing loss are more likely to be unemployed and on average earn significantly less wage income than adults without hearing loss. Further work is needed to determine the potential impact of treatment on these differences.

Nurses with Undiagnosed Hearing Loss: Implications for Practice.

PubMed

Spencer, Cara S; Pennington, Karen

2015-01-05

Hearing loss affects 36 million people in the United States of America, including 17% of the adult population. This suggests some nurses will have hearing losses that affect their communication skills and their ability to perform auscultation assessments, potentially compromising patient care and safety. In this article, the authors begin by reviewing the hearing process, describing various types of hearing loss, and discussing noise-induced hearing loss and noise levels in hospitals. Next, they consider the role of hearing in nursing practice, review resources for hearing-impaired nurses, identify the many costs associated with untreated hearing loss, and note nurses' responsibility for maintaining their hearing health. The authors conclude that nurses need to be aware of their risk for hearing loss and have their hearing screened every five years.

Peri-implant bony overgrowth as a cause of revision surgery in auditory osseointegrated implantation.

PubMed

Tompkins, Jared J; Petersen, Dana K; Sharbel, Daniel D; McKinnon, Brian J; MacDonald, C Bruce

2016-07-01

Implantation of auditory osseointegrated implants, also known as bone-anchored hearing systems (BAHS), represents a surgical option for select pediatric patients aged 5 years or older with hearing loss. Functional indications in this patient population include conductive or mixed hearing loss. Common complications of implantation include skin infections, chronic skin irritation, hypertrophic skin overgrowth, and loose abutments. In a case series of 15 pediatric patients, we discovered an unexpectedly high skin-related complication rate requiring surgical revision of 53%. During revision surgery, we discovered 5 patients who exhibited significant bony overgrowth at the abutment site, a complication infrequently noted in past literature. Published by Elsevier Ireland Ltd.

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.

Cisplatin and Aminoglycoside Antibiotics: Hearing Loss and Its Prevention

PubMed Central

Schacht, Jochen; Talaska, Andra E.; Rybak, Leonard P.

2013-01-01

This review introduces the pathology of aminoglycoside antibiotic and the cisplatin chemotherapy classes of drugs, discusses oxidative stress in the inner ear as a primary trigger for cell damage, and delineates the ensuing cell death pathways. Among potentially ototoxic (damaging the inner ear) therapeutics, the platinum-based anti-cancer drugs and the aminoglycoside antibiotics are of critical clinical importance. Both drugs cause sensorineural hearing loss in patients, a side effect that can be reproduced in experimental animals. Hearing loss is reflected primarily in damage to outer hair cells, beginning in the basal turn of the cochlea. In addition, aminoglycosides might affect the vestibular system while cisplatin seems to have a much lower likelihood to do so. Finally, based on an understanding the mechanisms of ototoxicity pharmaceutical ways of protection of the cochlea are presented PMID:23045231

Identification of a novel mutation in the myosin VIIA motor domain in a family with autosomal dominant hearing loss (DFNA11).

PubMed

Di Leva, Francesca; D'Adamo, Pio; Cubellis, Maria Vittoria; D'Eustacchio, Angela; Errichiello, Monica; Saulino, Claudio; Auletta, Gennaro; Giannini, Pasquale; Donaudy, Francesca; Ciccodicola, Alfredo; Gasparini, Paolo; Franzè, Annamaria; Marciano, Elio

2006-01-01

We ascertained a large Italian family with an autosomal dominant form of non-syndromic sensorineural hearing loss with vestibular involvement. A genome-wide scan found linkage to locus DFNA11. Sequencing of the MYO7A gene in the linked region identified a new missense mutation resulting in an Ala230Val change in the motor domain of the myosin VIIA. Myosin VIIA has already been implicated in several forms of deafness, but this is the third mutation causing a dominant form of deafness, located in the myosin VIIA motor domain in a region never involved in hearing loss until now. A modelled protein structure of myosin VII motor domain provides evidence for a significant functional effect of this missense mutation. Copyright (c) 2006 S. Karger AG, Basel.

[Risk of noise-induced hearing loss caused by radio communication? Audiologic findings in helicopter crews and pilots of propeller airplanes].

PubMed

Matschke, R G

1987-12-01

The affects of noise on the human inner ear have been well known for a long time, and measures to prevent occupational noise-induced hearing loss show a clear reduction in the statistics of morbidity. Nevertheless, there are working environments in which the use of ear protection seems to be inapplicable, because communication by speech is indispensable, for example in the cockpit of aircraft. Noise exposure measurements were performed on pilots of helicopters and propeller-machines of the German Federal Navy during realistic flight situations. The ambient noise levels during regular flight service were maintained at levels between 89 dB and 120 dB. Sound protection by flight-helmets and headphones is not only neutralised while using radio and intercom, but the noise during radio-communication is even louder than the noise of the engines. The use of ear protection to avoid excessive noise exposure is only of limited effectiveness. While pilots with normal hearing show only little impairment of speech intelligibility, those with noise-induced hearing loss show substantial impairment that varies in proportion to their hearing loss. Communication abilities may be drastically reduced which may compromise the reliability of radio-communication. The problem may be possibly solved in future by an electronic compensation system for noise.

Complying with a corporate global noise health surveillance procedure--do the benefits outweigh the costs?

PubMed

Bertsche, Patricia K; Mensah, Edward; Stevens, Thomas

2006-08-01

The purpose of this study was to determine whether the benefits of early identification of work-related noise-induced hearing loss outweigh the costs of complying with a Global Noise Medical Surveillance Procedure of a large corporation. Hearing is fundamental to language, communication, and socialization. Its loss is a common cause of disability, affecting an estimated 20 to 40 million individuals in the United States (Daniell et al., 1998). NIOSH reported that approximately 30 million U.S. workers are exposed to noise on the job and that noise-induced hearing loss is one of the most common occupational diseases. It is irreversible (NIOSH, 2004). The average cost of a noise-induced hearing loss is reported to range from dollars 4,726 to dollars 25,500. Corporate history indicates a range of dollars 44 to dollars 20,157 per case. During this 4-year study in one plant, the average annual cost of complying with the Global Noise Medical Surveillance Procedure was dollars 19,509 to screen an average of 390 employees, or dollars 50 per worker. The study identified 11 non-work-related standard threshold shifts. All cases were referred for appropriate early intervention. Given the results, this hearing health program is considered beneficial to the corporation for both work- and non-work-related reasons.

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. Copyright © 2014 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

Seeing the Talker's Face Improves Free Recall of Speech for Young Adults With Normal Hearing but Not Older Adults With Hearing Loss.

PubMed

Rudner, Mary; Mishra, Sushmit; Stenfelt, Stefan; Lunner, Thomas; Rönnberg, Jerker

2016-06-01

Seeing the talker's face improves speech understanding in noise, possibly releasing resources for cognitive processing. We investigated whether it improves free recall of spoken two-digit numbers. Twenty younger adults with normal hearing and 24 older adults with hearing loss listened to and subsequently recalled lists of 13 two-digit numbers, with alternating male and female talkers. Lists were presented in quiet as well as in stationary and speech-like noise at a signal-to-noise ratio giving approximately 90% intelligibility. Amplification compensated for loss of audibility. Seeing the talker's face improved free recall performance for the younger but not the older group. Poorer performance in background noise was contingent on individual differences in working memory capacity. The effect of seeing the talker's face did not differ in quiet and noise. We have argued that the absence of an effect of seeing the talker's face for older adults with hearing loss may be due to modulation of audiovisual integration mechanisms caused by an interaction between task demands and participant characteristics. In particular, we suggest that executive task demands and interindividual executive skills may play a key role in determining the benefit of seeing the talker's face during a speech-based cognitive task.

Murine CMV-Induced Hearing Loss Is Associated with Inner Ear Inflammation and Loss of Spiral Ganglia Neurons

PubMed Central

Golemac, Mijo; Pugel, Ester Pernjak; Jonjic, Stipan; Britt, William J.

2015-01-01

Congenital human cytomegalovirus (HCMV) occurs in 0.5–1% of live births and approximately 10% of infected infants develop hearing loss. The mechanism(s) of hearing loss remain unknown. We developed a murine model of CMV induced hearing loss in which murine cytomegalovirus (MCMV) infection of newborn mice leads to hematogenous spread of virus to the inner ear, induction of inflammatory responses, and hearing loss. Characteristics of the hearing loss described in infants with congenital HCMV infection were observed including, delayed onset, progressive hearing loss, and unilateral hearing loss in this model and, these characteristics were viral inoculum dependent. Viral antigens were present in the inner ear as were CD3+ mononuclear cells in the spiral ganglion and stria vascularis. Spiral ganglion neuron density was decreased after infection, thus providing a mechanism for hearing loss. The lack of significant inner ear histopathology and persistence of inflammation in cochlea of mice with hearing loss raised the possibility that inflammation was a major component of the mechanism(s) of hearing loss in MCMV infected mice. PMID:25875183

The contribution of family history to hearing loss in an older population.

PubMed

McMahon, Catherine M; Kifley, Annette; Rochtchina, Elena; Newall, Philip; Mitchell, Paul

2008-08-01

Although it has been well established that the prevalence of and severity of hearing loss increase with age, the contribution of familial factors to age-related hearing loss cannot be quantified. This is largely because hearing loss in older people has both genetic and environmental contributions. As environmental factors play an increasing role with age, it is difficult to delineate the separate contribution of genetic factors to age-related hearing loss. In a population-based survey of hearing loss in a representative older Australian community, we attempted to overcome this using logistic regression analysis, accounting for known factors associated with hearing loss including age, sex, noise exposure at work, diabetes, and current smoking. We tested hearing thresholds using pure tone audiometry and used a forced choice questionnaire to determine the nature of family history in a population of individuals aged 50 yrs or older in a defined region, west of Sydney, Australia (N = 2669). We compared the characteristics of participants with and without family history of hearing loss. Of those reporting a positive family history, we compared subgroups for age, gender and severity of hearing loss, and trends by the severity of hearing loss. Logistic regression was used to obtain odds ratios (ORs) with 95% confidence intervals (CIs) that compared the chances of having hearing loss in participants with and without family history, after adjusting for other factors known associated with hearing loss. Our findings indicate that family history was most strongly associated with moderate to severe age-related hearing loss. We found a strong association between maternal family history of hearing loss and moderate to severe hearing loss in women (adjusted OR 3.0; 95% CI 1.6-5.6 in women with without a maternal history). Paternal family history of hearing loss was also significantly, though less strongly, associated with moderate-severe hearing loss in men (adjusted OR 2.0; CI 1.01-3.9 in men with than without a paternal history). Findings from this study are important in the identification of individuals whose auditory system may be genetically susceptible to aging and environmental insult. Genetic counseling may assist in ameliorating the effects of hearing loss.

Evaluation of Hearing Handicap in Adults with Auditory Neuropathy Spectrum Disorder.

PubMed

Prabhu, Prashanth

2017-08-01

The present study attempted to evaluate hearing handicap in adults with auditory neuropathy spectrum disorder (ANSD). The study also attempted to determine if gender, pure tone average, speech identification scores (SIS), and reported duration of hearing loss could predict the hearing handicap in adults with ANSD. Hearing Handicap Inventory for Adults and Hearing Handicap Questionnaire were administered to 50 adults with ANSD. Using both the scales, there was a significant hearing handicap in both the social and emotional domains in adults with ANSD. SIS was a good predictor of hearing handicap compared to other variables. The poor SIS can affect communication skills leading to higher degree of social handicap. The ignorance regarding the exact cause for their hearing problems and lack of appropriate management strategies could lead to emotional problems in individuals with ANSD. However, further studies are essential for determining hearing handicap with the use of hearing aids and cochlear implants.

Vaccine May Reduce Incidence of Meningitis-Related Hearing Impairment.

ERIC Educational Resources Information Center

Smith, Dorothy

1988-01-01

Hearing loss as a result of meningitis, now the leading nongenetic cause of deafness in infants and young children, may be reduced by the introduction of the HiB (Hemophilus influenzae type B) vaccine. It is highly effective, relatively safe, and recommended for most children over 24 months and high risk children 18-24 months old. (VW)

Raising and Educating a Deaf Child.

ERIC Educational Resources Information Center

Marschark, Marc

This book discusses the development of children who are deaf or hard of hearing and the educational and practical issues that face them and their families. Chapters discuss: (1) the effect of a child with deafness on a family, and family adjustment to early childhood hearing loss; (2) the population of people who are deaf, the causes of early…

Targeting the cMET pathway augments radiation response without adverse effect on hearing in NF2 schwannoma models.

PubMed

Zhao, Yingchao; Liu, Pinan; Zhang, Na; Chen, Jie; Landegger, Lukas D; Wu, Limeng; Zhao, Fu; Zhao, Yanxia; Zhang, Yanling; Zhang, Jing; Fujita, Takeshi; Stemmer-Rachamimov, Anat; Ferraro, Gino B; Liu, Hao; Muzikansky, Alona; Plotkin, Scott R; Stankovic, Konstantina M; Jain, Rakesh K; Xu, Lei

2018-02-27

Neurofibromatosis type II (NF2) is a disease that needs new solutions. Vestibular schwannoma (VS) growth causes progressive hearing loss, and the standard treatment, including surgery and radiotherapy, can further damage the nerve. There is an urgent need to identify an adjunct therapy that, by enhancing the efficacy of radiation, can help lower the radiation dose and preserve hearing. The mechanisms underlying deafness in NF2 are still unclear. One of the major limitations in studying tumor-induced hearing loss is the lack of mouse models that allow hearing testing. Here, we developed a cerebellopontine angle (CPA) schwannoma model that faithfully recapitulates the tumor-induced hearing loss. Using this model, we discovered that cMET blockade by crizotinib (CRZ) enhanced schwannoma radiosensitivity by enhancing DNA damage, and CRZ treatment combined with low-dose radiation was as effective as high-dose radiation. CRZ treatment had no adverse effect on hearing; however, it did not affect tumor-induced hearing loss, presumably because cMET blockade did not change tumor hepatocyte growth factor (HGF) levels. This cMET gene knockdown study independently confirmed the role of the cMET pathway in mediating the effect of CRZ. Furthermore, we evaluated the translational potential of cMET blockade in human schwannomas. We found that human NF2-associated and sporadic VSs showed significantly elevated HGF expression and cMET activation compared with normal nerves, which correlated with tumor growth and cyst formation. Using organoid brain slice culture, cMET blockade inhibited the growth of patient-derived schwannomas. Our findings provide the rationale and necessary data for the clinical translation of combined cMET blockade with radiation therapy in patients with NF2.

Residual Inhibition Functions Overlap Tinnitus Spectra and the Region of Auditory Threshold Shift

PubMed Central

Moffat, Graeme; Baumann, Michael; Ward, Lawrence M.

2008-01-01

Animals exposed to noise trauma show augmented synchronous neural activity in tonotopically reorganized primary auditory cortex consequent on hearing loss. Diminished intracortical inhibition in the reorganized region appears to enable synchronous network activity that develops when deafferented neurons begin to respond to input via their lateral connections. In humans with tinnitus accompanied by hearing loss, this process may generate a phantom sound that is perceived in accordance with the location of the affected neurons in the cortical place map. The neural synchrony hypothesis predicts that tinnitus spectra, and heretofore unmeasured “residual inhibition functions” that relate residual tinnitus suppression to the center frequency of masking sounds, should cover the region of hearing loss in the audiogram. We confirmed these predictions in two independent cohorts totaling 90 tinnitus subjects, using computer-based tools designed to assess the psychoacoustic properties of tinnitus. Tinnitus spectra and residual inhibition functions for depth and duration increased with the amount of threshold shift over the region of hearing impairment. Residual inhibition depth was shallower when the masking sounds that were used to induce residual inhibition showed decreased correspondence with the frequency spectrum and bandwidth of the tinnitus. These findings suggest that tinnitus and its suppression in residual inhibition depend on processes that span the region of hearing impairment and not on mechanisms that enhance cortical representations for sound frequencies at the audiometric edge. Hearing thresholds measured in age-matched control subjects without tinnitus implicated hearing loss as a factor in tinnitus, although elevated thresholds alone were not sufficient to cause tinnitus. PMID:18712566

High-frequency Audiometry Hearing on Monitoring of Individuals Exposed to Occupational Noise: A Systematic Review.

PubMed

Antonioli, Cleonice Aparecida Silva; Momensohn-Santos, Teresa Maria; Benaglia, Tatiana Aparecida Silva

2016-07-01

The literature reports on high-frequency audiometry as one of the exams used on hearing monitoring of individuals exposed to high sound pressure in their work environment, due to the method́s greater sensitivity in early identification of hearing loss caused by noise. The frequencies that compose the exam are generally between 9 KHz and 20KHz, depending on the equipment. This study aims to perform a retrospective and secondary systematic revision of publications on high-frequency audiometry on hearing monitoring of individuals exposed to occupational noise. This systematic revision followed the methodology proposed in the Cochrane Handbook, focusing on the question: "Is High-frequency Audiometry more sensitive than Conventional Audiometry in the screening of early hearing loss individuals exposed to occupational noise?" The search was based on PubMed data, Base, Web of Science (Capes), Biblioteca Virtual em Saúde (BVS), and in the references cited in identified and selected articles. The search resulted in 6059 articles in total. Of these, only six studies were compatible with the criteria proposed in this study. The performed meta-analysis does not definitively answer the study's proposed question. It indicates that the 16 KHz high frequency audiometry (HFA) frequency is sensitive in early identification of hearing loss in the control group (medium difference (MD = 8.33)), as well as the 4 KHz frequency (CA), this one being a little less expressive (MD = 5.72). Thus, others studies are necessary to confirm the HFA importance for the early screening of hearing loss on individuals exposed to noise at the workplace.

Hearing and neurodevelopmental outcomes for children with asymptomatic congenital cytomegalovirus infection: A systematic review.

PubMed

Bartlett, Adam W; McMullan, Brendan; Rawlinson, William D; Palasanthiran, Pamela

2017-09-06

Congenital CMV is one of the commonest congenital infections and a recognised cause of sensorineural hearing loss and neurodevelopmental impairment. Ninety percent are clinically inapparent at birth but are reported to be at risk of developing such abnormalities throughout childhood, the extent of which requires further elucidation. A systematic literature review was conducted using Medline and Embase databases, manual citation review, and personal libraries for articles reporting primary data on hearing and neurodevelopmental outcomes for children with asymptomatic congenital CMV. PROSPERO registration number CRD42015025407. Thirty-seven of 480 articles identified between 1969 and 2016 met the eligibility criteria. Twenty-nine of these contributed primary data on hearing outcomes and 20 on neurodevelopmental outcomes (12 of the 37 studies contributed data on both). Cumulative incidence of sensorineural hearing loss with follow-up to at least 5 years was 7% to 11%, which is more than healthy controls but less than children with symptomatic congenital CMV (34%-41%). The onset, course, and severity of hearing loss was variable with no reliable virological prognostic marker. In comparison to controls, children with asymptomatic congenital CMV did not perform worse than controls in neurodevelopmental assessments and performed better than children with symptomatic congenital CMV. Studies show children with asymptomatic congenital CMV are at increased risk of developing hearing loss but perform equally well on neurodevelopmental assessments when compared with healthy controls. There is no reliable virological marker to determine which infants will develop sequelae. Regular follow-up until school entry is supported by the literature. Copyright © 2017 John Wiley & Sons, Ltd.

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

PubMed

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

1997-10-01

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

Socioeconomic differences in hearing among middle-aged and older adults: cross-sectional analyses using the Health Survey for England

PubMed Central

Scholes, Shaun; Biddulph, Jane; Davis, Adrian; Mindell, Jennifer S.

2018-01-01

Background Hearing loss impacts on cognitive, social and physical functioning. Both hearing loss and hearing aid use vary across population subgroups. We examined whether hearing loss, and reported current hearing aid use among persons with hearing loss, were associated with different markers of socioeconomic status (SES) in a nationally representative sample of community-dwelling middle-aged and older adults. Methods Hearing was measured using an audiometric screening device in the Health Survey for England 2014 (3292 participants aged 45 years and over). Hearing loss was defined as >35 dB HL at 3.0 kHz in the better-hearing ear. Using sex-specific logistic regression modelling, we evaluated the associations between SES and hearing after adjustment for potential confounders. Results 26% of men and 20% of women aged 45 years and over had hearing loss. Hearing loss was higher among men in the lowest SES groups. For example, the multivariable-adjusted odds of hearing loss were almost two times as high for those in the lowest versus the highest income tertile (OR 1.77, 95% CI 1.15 to 2.74). Among those with hearing loss, 30% of men and 27% of women were currently using a hearing aid. Compared with men in the highest income tertile, the multivariable-adjusted odds of using a hearing aid nowadays were lower for men in the middle (OR 0.50, 95% CI 0.25 to 0.99) and the lowest (OR 0.47, 95% CI 0.23 to 0.97) income tertiles. Associations between SES and hearing were weaker or null among women. Conclusions While the burden of hearing loss fell highest among men in the lowest SES groups, current hearing aid use was demonstrably lower. Initiatives to detect hearing loss early and increase the uptake and the use of hearing aids may provide substantial public health benefits and reduce socioeconomic inequalities in health. PMID:29391384

[Hearing voices does not always constitute a psychosis].

PubMed

Sommer, I E C; van der Spek, D W

2016-01-01

Hearing voices (i.e. auditory verbal hallucinations) is mainly known as part of schizophrenia and other psychotic disorders. However, hearing voices is a symptom that can occur in many psychiatric, neurological and general medical conditions. We present three cases of non-psychotic patients with auditory verbal hallucinations caused by different disorders. The first patient is a 74-year-old male with voices due to hearing loss, the second is a 20-year-old woman with voices due to traumatisation. The third patient is a 27-year-old woman with voices caused by temporal lobe epilepsy. Hearing voices is a phenomenon that occurs in a variety of disorders. Therefore, identification of the underlying disorder is essential to indicate treatment. Improvement of coping with the voices can reduce their impact on a patient. Antipsychotic drugs are especially effective when hearing voices is accompanied by delusions or disorganization. When this is not the case, the efficacy of antipsychotic drugs will probably not outweigh the side-effects.

Hearing loss and social support in urban and rural communities.

PubMed

Hay-McCutcheon, Marcia J; Hyams, Adriana; Yang, Xin; Parton, Jason

2018-04-19

Perceived social support and hearing handicap were assessed in adults with and without hearing loss who lived in different geographical regions of Alabama. The Hearing Handicap Inventory for Adults (HHIA) assessed emotional and social consequences of hearing loss. The Medical Outcomes Study (MOS) Social Support Survey and the Social Functioning, Role Emotional and Mental Health scales of the SF-36 were administered. Data were collected from 71 study participants with hearing loss and from 45 adults without hearing loss. Degree of hearing loss and outcomes from the HHIA did not differ between adults who lived in rural or urban settings. Tangible support was poorer for adults with hearing loss who lived in rural settings compared to those who lived in urban settings. For adults without hearing loss, residency was not associated with tangible support. For these adults, income was associated with other types of social support (i.e. informational support, affection, positive social interaction). Adults with hearing loss living in rural areas had poor perceived tangible support. The provision of support to address a hearing loss could be worse for these adults compared to adults who lived in urban settings.

Emerging therapeutic interventions against noise-induced hearing loss

PubMed Central

Sha, Su-Hua; Schacht, Jochen

2017-01-01

Introduction Noise-induced hearing loss (NIHL) due to industrial, military, and recreational noise exposure is a major, but also potentially preventable cause of acquired hearing loss. For the United States it is estimated that 26 million people (15% of the population) between the ages of 20 and 69 have a high-frequency NIHL at a detriment to the quality of life of the affected individuals and great economic cost to society. Areas covered This review outlines the pathology and pathophysiology of hearing loss as seen in humans and animal models. Results from molecular studies are presented that have provided the basis for therapeutic strategies successfully applied to animals. Several compounds emerging from these studies (mostly antioxidants) are now being tested in field trials. Expert opinion Although no clinically applicable intervention has been approved yet, recent trials are encouraging. In order to maximize protective therapies, future work needs to apply stringent criteria for noise exposure and outcome parameters. Attention needs to be paid not only to permanent NIHL due to death of sensory cells but also to temporary effects that may show delayed consequences. Existing results combined with the search for efficacious new therapies should establish a viable treatment within a decade. PMID:27918210

Vezatin, an integral membrane protein of adherens junctions, is required for the sound resilience of cochlear hair cells

PubMed Central

Bahloul, Amel; Simmler, Marie-Christine; Michel, Vincent; Leibovici, Michel; Perfettini, Isabelle; Roux, Isabelle; Weil, Dominique; Nouaille, Sylvie; Zuo, Jian; Zadro, Cristina; Licastro, Danilo; Gasparini, Paolo; Avan, Paul; Hardelin, Jean-Pierre; Petit, Christine

2009-01-01

Loud sound exposure is a significant cause of hearing loss worldwide. We asked whether a lack of vezatin, an ubiquitous adherens junction protein, could result in noise-induced hearing loss. Conditional mutant mice bearing non-functional vezatin alleles only in the sensory cells of the inner ear (hair cells) indeed exhibited irreversible hearing loss after only one minute exposure to a 105 dB broadband sound. In addition, mutant mice spontaneously underwent late onset progressive hearing loss and vestibular dysfunction related to substantial hair cell death. We establish that vezatin is an integral membrane protein with two adjacent transmembrane domains, and cytoplasmic N- and C-terminal regions. Late recruitment of vezatin at junctions between MDCKII cells indicates that the protein does not play a role in the formation of junctions, but rather participates in their stability. Moreover, we show that vezatin directly interacts with radixin in its actin-binding conformation. Accordingly, we provide evidence that vezatin associates with actin filaments at cell–cell junctions. Our results emphasize the overlooked role of the junctions between hair cells and their supporting cells in the auditory epithelium resilience to sound trauma. PMID:20049712

Variety of audiologic manifestations in patients with superior semicircular canal dehiscence.

PubMed

Chi, Fang-Lu; Ren, Dong-Dong; Dai, Chun-Fu

2010-01-01

To present diverse symptoms, signs, and findings on diagnostic tests of 11 patients with superior semicircular canal dehiscence (SSCD) syndrome and surgical treatments for dehiscence of 3 patients. Retrospective case review. Tertiary neurotologic and audiologic center. Patients with SSCD documented by history, physical examination, vestibular function testing, and high-resolution computed tomographic scans. Two patients underwent surgical procedures through middle fossa approach, and 1 patient underwent transmastoid repair. The rest underwent conservative treatments. Eleven patients were identified as SSCD. The variety of clinical manifestations, including 1) hearing presentations: mix hearing loss (4 of 11), conductive hearing loss (3 of 11), profound sensorineural hearing loss (2 of 11), and normal hearing (2 of 11); 2) vestibular manifestations: chronic disequilibrium (8 of 11), Tullio phenomenon (7 of 11), Hennebert sign (8 of 11), tinnitus (3 of 11), and autophony (3 of 11); and 3) accompanying disorders: encephalomeningocele (1 of 11). Abnormal nystagmus was identified in 3 patients. Dehiscence of bone overlying superior semicircular canal was confirmed by high-resolution computed tomographic scan in all cases. Three patients underwent operative management. (2 through the middle fossa approach and 1 through a transmastoid repair). Superior semicircular canal dehiscence demonstrates diverse and complex clinical features. Tegmental or petrosal bone rarefaction or mild dehiscences can be a harbinger or an aggressive cause of developing SSCD. No correlation was observed between the size of dehiscence and the severity of vestibular symptoms or the degree of hearing loss. Surgical repair of dehiscence can relieve symptoms with low morbidity.

Gene Therapy Restores Balance and Auditory Functions in a Mouse Model of Usher Syndrome.

PubMed

Isgrig, Kevin; Shteamer, Jack W; Belyantseva, Inna A; Drummond, Meghan C; Fitzgerald, Tracy S; Vijayakumar, Sarath; Jones, Sherri M; Griffith, Andrew J; Friedman, Thomas B; Cunningham, Lisa L; Chien, Wade W

2017-03-01

Dizziness and hearing loss are among the most common disabilities. Many forms of hereditary balance and hearing disorders are caused by abnormal development of stereocilia, mechanosensory organelles on the apical surface of hair cells in the inner ear. The deaf whirler mouse, a model of human Usher syndrome (manifested by hearing loss, dizziness, and blindness), has a recessive mutation in the whirlin gene, which renders hair cell stereocilia short and dysfunctional. In this study, wild-type whirlin cDNA was delivered to the inner ears of neonatal whirler mice using adeno-associated virus serotype 2/8 (AAV8-whirlin) by injection into the posterior semicircular canal. Unilateral whirlin gene therapy injection was able to restore balance function as well as improve hearing in whirler mice for at least 4 months. Our data indicate that gene therapy is likely to become a treatment option for hereditary disorders of balance and hearing. Copyright © 2017. Published by Elsevier Inc.

A novel mutation of the EYA4 gene associated with post-lingual hearing loss in a proband is co-segregating with a novel PAX3 mutation in two congenitally deaf family members.

PubMed

Cesca, Federica; Bettella, Elisa; Polli, Roberta; Cama, Elona; Scimemi, Pietro; Santarelli, Rosamaria; Murgia, Alessandra

2018-01-01

This work was aimed at establishing the molecular etiology of hearing loss in a 9-year old girl with post-lingual non-syndromic mild sensorineural hearing loss with a complex family history of clinically heterogeneous deafness. The proband's DNA was subjected to NGS analysis of a 59-targeted gene panel, with the use of the Ion Torrent PGM platform. Conventional Sanger sequencing was used for segregation analysis in all the affected relatives. The proband and all the other hearing impaired members of the family underwent a thorough clinical and audiological evaluation. A new likely pathogenic mutation in the EYA4 gene (c.1154C > T; p.Ser385Leu) was identified in the proband and in her 42-year-old father with post-lingual non-syndromic profound sensorineural hearing loss. The EYA4 mutation was also found in the proband's grandfather and uncle, both showing clinical features of Waardenburg syndrome type 1. A novel pathogenic splice-site mutation (c.321+1G > A) of the PAX3 gene was found to co-segregate with the EYA4 mutation in these two subjects. The identified novel EYA4 mutation can be considered responsible of the hearing loss observed in the proband and her father, while a dual molecular diagnosis was reached in the relatives co-segregating the EYA4 and the PAX3 mutations. In these two subjects the DFNA10 phenotype was masked by Waardenburg syndrome. The use of NGS targeted gene-panel, in combination with an extensive clinical and audiological examination led us to identify the genetic cause of the hearing loss in members of a family in which different forms of autosomal dominant deafness segregate. These results provide precise and especially important prognostic and follow-up information for the future audiologic management in the youngest affected member. Copyright © 2017 Elsevier B.V. All rights reserved.

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 hearing loss was conductive in nature, likely due to frequent ear infections, and many children with hearing loss qualified for hearing aids. Screening strategies need to be developed and tested since caregivers were not reliable at identifying hearing loss, and often mis-identified children with normal hearing as having hearing loss. Children with frequent ear infections, ear drainage, TB, severe HIV disease, or low BMI should receive more frequent ear assessments and hearing evaluations. PMID:27551970

The Bjornstad syndrome (sensorineural hearing loss and pili torti) disease gene maps to chromosome 2q34-36.

PubMed Central

Lubianca Neto, J F; Lu, L; Eavey, R D; Flores, M A; Caldera, R M; Sangwatanaroj, S; Schott, J J; McDonough, B; Santos, J I; Seidman, C E; Seidman, J G

1998-01-01

We report that the Bjornstad syndrome gene maps to chromosome 2q34-36. The clinical association of sensorineural hearing loss with pili torti (broken, twisted hairs) was described >30 years ago by Bjornstad; subsequently, several small families have been studied. We evaluated a large kindred with Bjornstad syndrome in which eight members inherited pili torti and prelingual sensorineural hearing loss as autosomal recessive traits. A genomewide search using polymorphic loci demonstrated linkage between the disease gene segregating in this kindred and D2S434 (maximum two-point LOD score = 4.98 at theta = 0). Haplotype analysis of recombination events located the disease gene in a 3-cM region between loci D2S1371 and D2S163. We speculate that intermediate filament and intermediate filament-associated proteins are good candidate genes for causing Bjornstad syndrome. PMID:9545407

Disorders of cochlear blood flow.

PubMed

Nakashima, Tsutomu; Naganawa, Shinji; Sone, Michihiko; Tominaga, Mitsuo; Hayashi, Hideo; Yamamoto, Hiroshi; Liu, Xiuli; Nuttall, Alfred L

2003-09-01

The cochlea is principally supplied from the inner ear artery (labyrinthine artery), which is usually a branch of the anterior inferior cerebellar artery. Cochlear blood flow is a function of cochlear perfusion pressure, which is calculated as the difference between mean arterial blood pressure and inner ear fluid pressure. Many otologic disorders such as noise-induced hearing loss, endolymphatic hydrops and presbycusis are suspected of being related to alterations in cochlear blood flow. However, the human cochlea is not easily accessible for investigation because this delicate sensory organ is hidden deep in the temporal bone. In patients with sensorineural hearing loss, magnetic resonance imaging, laser-Doppler flowmetry and ultrasonography have been used to investigate the status of cochlear blood flow. There have been many reports of hearing loss that were considered to be caused by blood flow disturbance in the cochlea. However, direct evidence of blood flow disturbance in the cochlea is still lacking in most of the cases.

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

PubMed

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

2012-10-01

Although newborn hearing screening programs have been introduced in most states in Australia, the prevalence of conductive hearing loss and middle ear pathology in the infants referred through these programs is not known. This study was designed to (1) evaluate the prevalence of conductive hearing loss and middle ear pathology in infants referred by a newborn hearing screening program in north Queensland, (2) compare prevalence rates of conductive hearing loss and middle ear pathology in indigenous and nonindigenous infants, and (3) review the outcomes of those infants diagnosed with conductive hearing loss and middle ear pathology. Retrospective chart review of infants referred to the Audiology Department of The Townsville Hospital was conducted. Chart review of 234 infants referred for one or both ears from a newborn hearing screening program in north Queensland was conducted. A total of 211 infants attended the diagnostic appointment. Review appointments to monitor hearing status were completed for 46 infants with middle ear pathology or conductive hearing loss. Diagnosis of hearing impairment was made using an age-appropriate battery of audiological tests. Results were analyzed for both initial and review appointments. Mean age at initial diagnostic assessment was 47.5 days (SD = 31.3). Of the 69 infants with middle ear pathology during initial diagnostic assessment, 18 had middle ear pathology with normal hearing, 47 had conductive hearing loss, and 4 had mixed hearing loss. Prevalence of conductive hearing loss in the newborns was 2.97 per 1,000 while prevalence of middle ear pathology (with or without conductive hearing loss) was 4.36 per 1,000. Indigenous Australians or Aboriginal and Torres Strait Islander (ATSI) infants had a significantly higher prevalence of conductive hearing loss and middle ear pathology than non-ATSI infants (35.19 and 44.45% vs 17.83 and 28.66%, respectively). ATSI infants also showed poor resolution of conductive hearing loss over time with 66.67% of ATSI infants reviewed showing persistent conductive hearing loss compared to 17.86% of non-ATSI infants. Medical management of 17 infants with persistent conductive hearing loss included monitoring, antibiotic treatment, examination under anesthesia, and grommet insertion. Conductive hearing loss was found to be a common diagnosis among infants referred through screening. ATSI infants had significantly higher rates of middle ear pathology and conductive hearing loss at birth and showed poor resolution of middle ear pathology over time compared to non-ATSI infants. Future research using a direct measure of middle ear function as an adjunct to the automated auditory brainstem response screening tool to distinguish conductive from sensorineural hearing loss may facilitate prioritization of infants for assessment, thus reducing parental anxiety and streamlining the management strategies for the respective types of hearing loss. American Academy of Audiology.

Prevalence and causes of hearing impairment in Fundong Health District, North-West Cameroon.

PubMed

Ferrite, Silvia; Mactaggart, Islay; Kuper, Hannah; Oye, Joseph; Polack, Sarah

2017-04-01

To estimate the prevalence and causes of hearing impairment in Fundong Health District, North-West Cameroon. We selected 51 clusters of 80 people (all ages) through probability proportionate to size sampling. Initial hearing screening was undertaken through an otoacoustic emission (OAE) test. Participants aged 4+ years who failed this test in both ears or for whom an OAE reading could not be taken underwent a manual pure-tone audiometry (PTA) screening. Cases of hearing impairment were defined as those with pure-tone average ≥41 dBHL in adults and ≥35 dBHL in children in the better ear, or children under age 4 who failed the OAE test in both ears. Each case with hearing loss was examined by an ear, nose and throat nurse who indicated the main likely cause. We examined 3567 (86.9%) of 4104 eligible people. The overall prevalence of hearing impairment was 3.6% (95% confidence interval [CI]: 2.8-4.6). The prevalence was low in people aged 0-17 (1.1%, 0.7-1.8%) and 18-49 (1.1%, 0.5-2.6%) and then rose sharply in people aged 50+ (14.8%, 11.7-19.1%). Among cases, the majority were classified as moderate (76%), followed by severe (15%) and profound (9%). More than one-third of cases of hearing impairment were classified as unknown (37%) or conductive (37%) causes, while sensorineural causes were less common (26%). Prevalence of hearing impairment in North-West Cameroon is in line with the WHO estimate for sub-Saharan Africa. The majority of cases with known causes are treatable, with impacted wax playing a major role. © 2017 John Wiley & Sons Ltd.

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

PubMed Central

Henry, Kenneth S.; Heinz, Michael G.

2013-01-01

People with sensorineural hearing loss have substantial difficulty understanding speech under degraded listening conditions. Behavioral studies suggest that this difficulty may be caused by changes in auditory processing of the rapidly-varying temporal fine structure (TFS) of acoustic signals. In this paper, we review the presently known effects of sensorineural hearing loss on processing of TFS and slower envelope modulations in the peripheral auditory system of mammals. Cochlear damage has relatively subtle effects on phase locking by auditory-nerve fibers to the temporal structure of narrowband signals under quiet conditions. In background noise, however, sensorineural loss does substantially reduce phase locking to the TFS of pure-tone stimuli. For auditory processing of broadband stimuli, sensorineural hearing loss has been shown to severely alter the neural representation of temporal information along the tonotopic axis of the cochlea. Notably, auditory-nerve fibers innervating the high-frequency part of the cochlea grow increasingly responsive to low-frequency TFS information and less responsive to temporal information near their characteristic frequency (CF). Cochlear damage also increases the correlation of the response to TFS across fibers of varying CF, decreases the traveling-wave delay between TFS responses of fibers with different CFs, and can increase the range of temporal modulation frequencies encoded in the periphery for broadband sounds. Weaker neural coding of temporal structure in background noise and degraded coding of broadband signals along the tonotopic axis of the cochlea are expected to contribute considerably to speech perception problems in people with sensorineural hearing loss. PMID:23376018

Hearing loss diagnosis followed by meningitis in Danish children, 1995-2004.

PubMed

Parner, Erik T; Reefhuis, Jennita; Schendel, Diana; Thomsen, Janus L; Ovesen, Therese; Thorsen, Poul

2007-03-01

A higher risk of meningitis associated with cochlear implants may be explained in part by a generally higher risk of meningitis in children with severe to profound hearing loss. We investigated whether children with hearing loss have an increased risk of meningitis. A historical cohort study of all children born in Denmark between January 1, 1995, and December 31, 2004, was conducted. The cohort was selected through the Danish Medical Birth Registry, and information on hearing loss and meningitis was obtained from the National Hospital Registry. We identified 39 children with both hearing loss and meningitis. Of these children, five were diagnosed first with hearing loss and later with meningitis. The relative risk of meningitis in the group of children with a hearing loss diagnosis, as compared with the non-hearing loss group, was 5.0 (95% CI, 2.0 to 12.0). The study provides evidence for an association between hearing loss and the development of meningitis. Parents and health care providers of children with hearing loss should be more alert for possible signs and symptoms of meningitis, and vaccination should be considered.

Validation of questionnaire-reported hearing with medical records: A report from the Swiss Childhood Cancer Survivor Study

PubMed Central

Scheinemann, Katrin; Grotzer, Michael; Kompis, Martin; Kuehni, Claudia E.

2017-01-01

Background Hearing loss is a potential late effect after childhood cancer. Questionnaires are often used to assess hearing in large cohorts of childhood cancer survivors and it is important to know if they can provide valid measures of hearing loss. We therefore assessed agreement and validity of questionnaire-reported hearing in childhood cancer survivors using medical records as reference. Procedure In this validation study, we studied 361 survivors of childhood cancer from the Swiss Childhood Cancer Survivor Study (SCCSS) who had been diagnosed after 1989 and had been exposed to ototoxic cancer treatment. Questionnaire-reported hearing was compared to the information in medical records. Hearing loss was defined as ≥ grade 1 according to the SIOP Boston Ototoxicity Scale. We assessed agreement and validity of questionnaire-reported hearing overall and stratified by questionnaire respondents (survivor or parent), sociodemographic characteristics, time between follow-up and questionnaire and severity of hearing loss. Results Questionnaire reports agreed with medical records in 85% of respondents (kappa 0.62), normal hearing was correctly assessed in 92% of those with normal hearing (n = 249), and hearing loss was correctly assessed in 69% of those with hearing loss (n = 112). Sensitivity of the questionnaires was 92%, 74%, and 39% for assessment of severe, moderate and mild bilateral hearing loss; and 50%, 33% and 10% for severe, moderate and mild unilateral hearing loss, respectively. Results did not differ by sociodemographic characteristics of the respondents, and survivor- and parent-reports were equally valid. Conclusions Questionnaires are a useful tool to assess hearing in large cohorts of childhood cancer survivors, but underestimate mild and unilateral hearing loss. Further research should investigate whether the addition of questions with higher sensitivity for mild degrees of hearing loss could improve the results. PMID:28333999

Voice Handicap Index in Persian Speakers with Various Severities of Hearing Loss.

PubMed

Aghadoost, Ozra; Moradi, Negin; Dabirmoghaddam, Payman; Aghadoost, Alireza; Naderifar, Ehsan; Dehbokri, Siavash Mohammadi

2016-01-01

The purpose of this study was to assess and compare the total score and subscale scores of the Voice Handicap Index (VHI) in speakers with and without hearing loss. A further aim was to determine if a correlation exists between severities of hearing loss with total scores and VHI subscale scores. In this cross-sectional, descriptive analytical study, 100 participants, divided in 2 groups of participants with and without hearing loss, were studied. Background information was gathered by interview, and VHI questionnaires were filled in by all participants. For all variables, including mean total score and VHI subscale scores, there was a considerable difference in speakers with and without hearing loss (p < 0.05). The correlation between severity of hearing loss with total score and VHI subscale scores was significant. Speakers with hearing loss were found to have higher mean VHI scores than speakers with normal hearing. This indicates a high voice handicap related to voice in speakers with hearing loss. In addition, increased severity of hearing loss leads to more severe voice handicap. This finding emphasizes the need for a multilateral assessment and treatment of voice disorders in speakers with hearing loss. © 2017 S. Karger AG, Basel.

Noise-induced hearing loss: a military perspective.

PubMed

Pfannenstiel, Travis J

2014-10-01

To summarize relevant literature occurring over the past 12-18 months forwarding understanding of noise-induced hearing loss in relation to military service. Hearing loss prior to entry into military service is highly predictive of subsequent hearing loss and hearing loss disability. Tightly controlled organic solvent exposure may not be a significant risk factor for noise-induced hearing loss. Increasingly detailed analysis of high intensity noise, impulse and blast noise exposures, and the methods used to mitigate these exposures are leading to breakthroughs in understanding and predicting hearing loss in military service. Prevention, mitigation, treatment, and prediction of the effects of hazardous noise exposure in military service continue to require a multidisciplinary team of individuals from around the world fully aware of the detrimental effect to service members and their societies of hearing loss disability.

Effect of HIV Infection and Highly Active Antiretroviral Therapy on Hearing Function: A Prospective Case-Control Study From Cameroon.

PubMed

Fokouo, Jean Valentin F; Vokwely, Jean Espoir E; Noubiap, Jean Jacques N; Nouthe, Brice Enid; Zafack, Joseline; Minka Ngom, Esthelle Stéphanie; Dalil, Asmaou Bouba; Ngo Nyeki, Adèle-Rose; Bengono, Géneviève; Njock, Richard

2015-05-01

Human immunodeficiency virus (HIV) infection remains a major cause of morbidity and mortality worldwide. Many studies have found a higher prevalence of hearing impairment among HIV-positive individuals. To investigate the effect of HIV and highly active antiretroviral treatment (HAART) on the hearing function in a Cameroonian population. We conducted a prospective case-control study from March 1, 2012, through January 31, 2013. The study took place at the National Social Insurance Fund Hospital in Yaoundé, Cameroon, a public health facility. We included 90 HIV-positive case patients and 90 HIV-negative control patients aged 15 to 49 years without any history of hearing loss or treatment with a known ototoxic drug. The case group was further divided into 3 subgroups: 30 HAART-naive patients, 30 patients receiving first-line HAART, and 30 patients receiving second-line HAART. Hearing function was assessed by pure-tone audiometry and classified according to the criteria of the Bureau International d'Audio-Phonologie. Hearing loss due to HIV and HAART. The HIV-positive patients had more otologic symptoms (hearing loss, dizziness, tinnitus, and otalgia) than HIV-negative patients (41 vs 13, P = .04). There were 49 cases (27.2%) of hearing loss in the HIV-positive group vs 10 (5.6%) in the HIV-negative group (P = .04). Compared with HIV-negative individuals, the odds of hearing loss were higher among HIV-infected HAART-naive patients (right ear: odds ratio [OR], 6.7; 95% CI, 4.3-9.7; P = .004; left ear: OR, 6.2; 95% CI, 3.5-8.3; P = .006), patients receiving first-line HAART (right ear: OR, 5.6; 95% CI, 1.9-10.5; P = .01; left ear: OR, 12.5; 95% CI, 8.5-15.4; P < .001), and patients receiving second-line HAART (right ear: OR, 6.7; 95% CI, 3.3-9.6; P = .004; left ear: OR, 3.7; 95% CI, 3.0-5.0; P = .08). Hearing loss is more frequent in HIV-infected patients compared with uninfected patients. Therefore, HIV-infected patients need special audiologic care. Further studies are needed because controversy remains regarding the factors that lead to ear damage.

The Physiological Bases of Hidden Noise-Induced Hearing Loss: Protocol for a Functional Neuroimaging Study.

PubMed

Dewey, Rebecca Susan; Hall, Deborah A; Guest, Hannah; Prendergast, Garreth; Plack, Christopher J; Francis, Susan T

2018-03-09

Rodent studies indicate that noise exposure can cause permanent damage to synapses between inner hair cells and high-threshold auditory nerve fibers, without permanently altering threshold sensitivity. These demonstrations of what is commonly known as hidden hearing loss have been confirmed in several rodent species, but the implications for human hearing are unclear. Our Medical Research Council-funded program aims to address this unanswered question, by investigating functional consequences of the damage to the human peripheral and central auditory nervous system that results from cumulative lifetime noise exposure. Behavioral and neuroimaging techniques are being used in a series of parallel studies aimed at detecting hidden hearing loss in humans. The planned neuroimaging study aims to (1) identify central auditory biomarkers associated with hidden hearing loss; (2) investigate whether there are any additive contributions from tinnitus or diminished sound tolerance, which are often comorbid with hearing problems; and (3) explore the relation between subcortical functional magnetic resonance imaging (fMRI) measures and the auditory brainstem response (ABR). Individuals aged 25 to 40 years with pure tone hearing thresholds ≤20 dB hearing level over the range 500 Hz to 8 kHz and no contraindications for MRI or signs of ear disease will be recruited into the study. Lifetime noise exposure will be estimated using an in-depth structured interview. Auditory responses throughout the central auditory system will be recorded using ABR and fMRI. Analyses will focus predominantly on correlations between lifetime noise exposure and auditory response characteristics. This paper reports the study protocol. The funding was awarded in July 2013. Enrollment for the study described in this protocol commenced in February 2017 and was completed in December 2017. Results are expected in 2018. This challenging and comprehensive study will have the potential to impact diagnostic procedures for hidden hearing loss, enabling early identification of noise-induced auditory damage via the detection of changes in central auditory processing. Consequently, this will generate the opportunity to give personalized advice regarding provision of ear defense and monitoring of further damage, thus reducing the incidence of noise-induced hearing loss. ©Rebecca Susan Dewey, Deborah A Hall, Hannah Guest, Garreth Prendergast, Christopher J Plack, Susan T Francis. Originally published in JMIR Research Protocols (http://www.researchprotocols.org), 09.03.2018.

Speech perception in noise in unilateral hearing loss.

PubMed

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

2016-01-01

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

Validation of the use of self-reported hearing loss and the Hearing Handicap Inventory for elderly among rural Indian elderly population.

PubMed

Deepthi, R; Kasthuri, Arvind

2012-01-01

Hearing loss is a potentially disabling problem among elderly leading to physical and social dysfunction. Though audiometric assessment of hearing loss is considered as gold standard, it is not feasible in community settings. Several questionnaires measuring hearing handicap have been developed. Knowledge regarding applicability of these questionnaires among rural elderly is limited, hence a study was planned to validate single question and Shortened Hearing Handicap Inventory for Elderly (HHIE-S) in detecting hearing loss against pure tone-audiometry among rural Indian elderly. A single question 'do you feel you have a hearing loss?' and the HHIE-S was administered to 175 elderly in two rural areas. Hearing ability was assessed using pure tone audiometry. Sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of both screening tools were compared with pure tone averages (PTAs) greater than 25, 40 and 55 dB hearing level (mild, moderate and severe hearing loss, respectively). The single question yielded low sensitivity (30.9%) and high specificity (93.9%) for mild hearing loss. Similarly HHIE-S yielded a sensitivity of 26.2% and specificity of 95.9%. Sensitivity with single question increased to 76.2% and specificity decreased to 83.1% with severe hearing loss. Sensitivity with HHIE-S also increased to 76.2% and specificity decreased to 87.7% with severe hearing loss. These hearing screening questionnaires will be useful in identifying more disabling hearing losses among rural elderly which helps in rehabilitation services planning. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

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

PubMed

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

2015-09-01

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

Hearing testing in the U.S. Department of Defense: Potential impact on Veterans Affairs hearing loss disability awards.

PubMed

Nelson, J T; Swan, A A; Swiger, B; Packer, M; Pugh, M J

2017-06-01

Hearing loss is the second most common disability awarded by the U.S. Department of Veterans Affairs (VA) to former members of the U.S. uniformed services. Hearing readiness and conservation practices differ among the four largest uniformed military services (Air Force, Army, Marine Corps, and Navy). Utilizing a data set consisting of all hearing loss claims submitted to the VA from fiscal years 2003-2013, we examined characteristics of veterans submitting claims within one year of separation from military service. Our results indicate that having a hearing loss disability claim granted was significantly more likely for men, individuals over the age of 26 years at the time of the claim, individuals most recently serving in the U.S. Army, and those with at least one hearing loss diagnosis. Importantly, individuals with at least one test record in the Defense Occupational and Environmental Health Readiness System-Hearing Conservation (DOEHRS-HC) system were significantly less likely to have a hearing loss disability claim granted by the VA. Within the DOEHRS-HC cohort, those with at least one threshold shift or clinical hearing loss diagnosis while on active duty were more than two and three times more likely to have a hearing loss disability claim granted, respectively. These findings indicate that an established history of reduced hearing ability while on active duty was associated with a significantly increased likelihood of an approved hearing loss disability claim relative to VA claims without such a history. Further, our results show a persistent decreased rate of hearing loss disability awards overall. These findings support increased inclusion of personnel in DoD hearing readiness and conservation programs to reduce VA hearing loss disability awards. Copyright © 2016 Elsevier B.V. All rights reserved.

Perception of Musical Emotion in the Students with Cognitive and Acquired Hearing Loss.

PubMed

Mazaheryazdi, Malihe; Aghasoleimani, Mina; Karimi, Maryam; Arjmand, Pirooz

2018-01-01

Hearing loss can affect the perception of emotional reaction to the music. The present study investigated whether the students with congenital hearing loss exposed to the deaf culture, percept the same emotion from the music as students with acquired hearing loss. Participants were divided into two groups; 30 students with bilaterally congenital moderate to severe hearing loss that were selected from deaf schools located in Tehran, Iran and 30 students with an acquired hearing loss with the same degree of hearing loss selected from Amiralam Hospital, Tehran, Iran and compared with the group of 30 age and gender-matched normal hearing subjects served our control in 2012. The musical stimuli consisted of three different sequences of music, (sadness, happiness, and fear) each with the duration of 60 sec. The students were asked to point to the lists of words that best matched with their emotions. Emotional perception of sadness, happiness, and fear in congenital hearing loss children was significantly poorly than acquired hearing loss and normal hearing group ( P <0.001). There was no significant difference in the emotional perception of sadness, happiness, and fear among the group of acquired hearing loss and normal hearing group ( P =0.75), ( P =1) and ( P =0.16) respectively. Neural plasticity induced by hearing assistant devises may be affected by the time when a hearing aid was first fitted and how the auditory system responds to the reintroduction of certain sounds via amplification. Therefore, children who experienced auditory input of different sound patterns in their early childhood will show more perceptual flexibility in different situations than the children with congenital hearing loss and Deaf culture.

Hydrogen-Saturated Saline Protects Intensive Narrow Band Noise-Induced Hearing Loss in Guinea Pigs through an Antioxidant Effect

PubMed Central

Chen, Liwei; Yu, Ning; Lu, Yan; Wu, Longjun; Chen, Daishi; Guo, Weiwei; Zhao, Lidong; Liu, Mingbo; Yang, Shiming; Sun, Xuejun; Zhai, Suoqiang

2014-01-01

The purpose of the current study was to evaluate hydrogen-saturated saline protecting intensive narrow band noise-induced hearing loss. Guinea pigs were divided into three groups: hydrogen-saturated saline; normal saline; and control. For saline administration, the guinea pigs were given daily abdominal injections (1 ml/100 g) 3 days before and 1 h before narrow band noise exposure (2.5–3.5 kHz 130 dB SPL, 1 h). The guinea pigs in the control group received no treatment. The hearing function was assessed by the auditory brainstem response (ABR) and distortion product otoacoustic emission (DPOAE) recording. The changes of free radicals in the cochlea before noise exposure, and immediately and 7 days after noise exposure were also examined. By Scanning electron microscopy and succinate dehydrogenase staining, we found that pre-treatment with hydrogen-saturated saline significantly reduced noise-induced hair cell damage and hearing loss. We also found that the malondialdehyde, lipid peroxidation, and hydroxyl levels were significantly lower in the hydrogen-saturated saline group after noise trauma, indicating that hydrogen-saturated saline can decrease the amount of harmful free radicals caused by noise trauma. Our findings suggest that hydrogen-saturated saline is effective in preventing intensive narrow band noise-induced hearing loss through the antioxidant effect. PMID:24945316

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

Function and expression pattern of nonsyndromic deafness genes

PubMed Central

Hilgert, Nele; Smith, Richard J.H.; Van Camp, Guy

2010-01-01

Hearing loss is the most common sensory disorder, present in 1 of every 500 newborns. To date, 46 genes have been identified that cause nonsyndromic hearing loss, making it an extremely heterogeneous trait. This review provides a comprehensive overview of the inner ear function and expression pattern of these genes. In general, they are involved in hair bundle morphogenesis, form constituents of the extracellular matrix, play a role in cochlear ion homeostasis or serve as transcription factors. During the past few years, our knowledge of genes involved in hair bundle morphogenesis has increased substantially. We give an up-to-date overview of both the nonsyndromic and Usher syndrome genes involved in this process, highlighting proteins that interact to form macromolecular complexes. For every gene, we also summarize its expression pattern and impact on hearing at the functional level. Gene-specific cochlear expression is summarized in a unique table by structure/cell type and is illustrated on a cochlear cross-section, which is available online via the Hereditary Hearing Loss Homepage. This review should provide auditory scientists the most relevant information for all identified nonsyndromic deafness genes. PMID:19601806

Exome Sequencing Identifies a Novel Nonsense Mutation of MYO6 as the Cause of Deafness in a Brazilian Family.

PubMed

Sampaio-Silva, Juliana; Batissoco, Ana Carla; Jesus-Santos, Rafaela; Abath-Neto, Osório; Scarpelli, Luciano Cesar; Nishimura, Patricia Yoshie; Galindo, Layla Testa; Bento, Ricardo Ferreira; Oiticica, Jeanne; Lezirovitz, Karina

2018-01-01

We investigated 313 unrelated subjects who presented with hearing loss to identify the novel genetic causes of this condition in Brazil. Causative GJB2/GJB6 mutations were found in 12.7% of the patients. Among the familial cases (100/313), four were selected for exome sequencing. In one case, two novel heterozygous variants were found and were predicted to be pathogenic based on bioinformatics tools, that is, p.Ser906* (MYO6) and p.Arg42Cys (GJB3). We confirmed that this nonsense MYO6 mutation segregated with deafness in this family. Only the proband and her unaffected mother exhibited the GJB3 mutation, which is in the same amino acid of a known Erythrokeratodermia variabilis mutation. None of the patients exhibited this skin disease, but the proband exhibited a more severe hearing loss. Hence, the GJB3 mutation was considered to be a variant of uncertain significance. In conclusion, we described a novel nonsense MYO6 mutation that was responsible for the hearing loss in a Brazilian family. This mutation resides in the neck domain of myosin-VI after the motor domain. Thus, our data give further support for genotype-phenotype correlations, which state that when the motor domain of the protein is functioning, the hearing loss is milder and has a later onset. The three remaining families without mutations in the known genes suggest that there are still deafness genes to be revealed. © 2017 John Wiley & Sons Ltd/University College London.

A Homozygous Mutation in a Novel Zinc-Finger Protein, ERIS, Is Responsible for Wolfram Syndrome 2

PubMed Central

Amr, Sami ; Heisey, Cindy ; Zhang, Min ; Xia, Xia-Juan ; Shows, Kathryn H. ; Ajlouni, Kamel ; Pandya, Arti ; Satin, Leslie S. ; El-Shanti, Hatem ; Shiang, Rita 

2007-01-01

A single missense mutation was identified in a novel, highly conserved zinc-finger gene, ZCD2, in three consanguineous families of Jordanian descent with Wolfram syndrome (WFS). It had been shown that these families did not have mutations in the WFS1 gene (WFS1) but were mapped to the WFS2 locus at 4q22-25. A G→C transversion at nucleotide 109 predicts an amino acid change from glutamic acid to glutamine (E37Q). Although the amino acid is conserved and the mutation is nonsynonymous, the pathogenesis for the disorder is because the mutation also causes aberrant splicing. The mutation was found to disrupt messenger RNA splicing by eliminating exon 2, and it results in the introduction of a premature stop codon. Mutations in WFS1 have also been found to cause low-frequency nonsyndromic hearing loss, progressive hearing loss, and isolated optic atrophy associated with hearing loss. Screening of 377 probands with hearing loss did not identify mutations in the WFS2 gene. The WFS1-encoded protein, Wolframin, is known to localize to the endoplasmic reticulum and plays a role in calcium homeostasis. The ZCD2-encoded protein, ERIS (endoplasmic reticulum intermembrane small protein), is also shown to localize to the endoplasmic reticulum but does not interact directly with Wolframin. Lymphoblastoid cells from affected individuals show a significantly greater rise in intracellular calcium when stimulated with thapsigargin, compared with controls, although no difference was observed in resting concentrations of intracellular calcium. PMID:17846994

Recoverable hearing loss with amphetamines and other drugs.

PubMed

Iqbal, Nayyer

2004-06-01

Prolonged and sustained consumption of alcohol, heroin and volatiles had been reported to impair hearing. Amphetamine related hearing loss is clinically different from the hearing loss seen with other agents. It seems that illicit drug use could result in two clinically different types of hearing losses. In May and June of 2001, 183 men aged 18 and above who met DSM-IV criteria for substance dependence were studied in a hospital in Saudia Arabia. The purpose of the study was to ascertain the prevalence of amphetamine-related recoverable hearing loss, establish whether similar hearing loss also occurred with other drugs of abuse and determine if drug-related psychosis was more prevalent in those amphetamine users who developed this type of hearing loss. Recoverable type of hearing loss was not just seen in amphetamine users but also occurred with cannabis, heroin, alcohol, dextromethorphan and glue use. Drug-induced psychosis was three and a half times more common in those amphetamine users who developed a hearing loss. Major depression and suicidality was also more common in these individuals. This association of major depression and subsequent development of hearing loss was also found in those using other type of drugs. It was concluded that a history of major depression was a good predictor of later development of both drug-induced psychosis and hearing loss in amphetamine users, and hypoperfusion was proposed as the possible explanation.

A mouse model with postnatal endolymphatic hydrops and hearing loss

PubMed Central

Megerian, Cliff A.; Semaan, Maroun T.; Aftab, Saba; Kisley, Lauren B.; Zheng, Qing Yin; Pawlowski, Karen S.; Wright, Charles G.; Alagramam, Kumar N.

2010-01-01

Endolymphatic hydrops (ELH), hearing loss and neuronal degeneration occur together in a variety of clinically significant disorders, including Meniere’s disease (MD). However, the sequence of these pathological changes and their relationship to each other are not well understood. In this regard, an animal model that spontaneously develops these features postnatally would be useful for research purposes. A search for such a model led us to the PhexHyp-Duk mouse, a mutant allele of the Phex gene causing X-linked hypophosphatemic rickets. The hemizygous male (PhexHyp-Duk/Y) was previously reported to exhibit various abnormalities during adulthood, including thickening of bone, ELH and hearing loss. The reported inner-ear phenotype was suggestive of progressive pathology and spontaneous development of ELH postnatally, but not conclusive. The main focuses of this report are to further characterize the inner ear phenotype in PhexHyp-Duk/Y mice and to test the hypotheses that (a) the PhexHyp-Duk/Y mouse develops ELH and hearing loss postnatally, and (b) the development of ELH in the PhexHyp-Duk/Y mouse is associated with obstruction of the endolymphatic duct (ED) due to thickening of the surrounding bone. Auditory brainstem response (ABR) recordings at various times points and histological analysis of representative temporal bones reveal that PhexHyp-Duk/Y mice typically develop adult onset, asymmetric, progressive hearing loss closely followed by the onset of ELH. ABR and histological data show that functional degeneration precedes structural degeneration. The major degenerative correlate of hearing loss and ELH in the mutants is the primary loss of spiral ganglion cells. Further, PhexHyp-Duk/Y mice develop ELH without evidence of ED obstruction, supporting the idea that ELH can be induced by a mechanism other than the blockade of longitudinal flow of endolymphatic fluid, and occlusion of ED is not a prerequisite for the development of ELH in patients. PMID:18289812

Mathematics Motivation, Anxiety, and Performance in Female Deaf/Hard-of-Hearing and Hearing Students

ERIC Educational Resources Information Center

Ariapooran, Saeed

2017-01-01

Hearing loss can be a major detriment to academic achievement among students. The present comparative study examines the differences in mathematics motivation, anxiety, and performance in female students with hearing loss and their hearing peers. A total of 63 female students with hearing loss (deaf and hard-of-hearing) and 63 hearing female…

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

PubMed

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

2013-01-01

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

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

PubMed

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

2007-01-01

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

What is in a cause? Exploring the relationship between genetic cause and felt stigma

PubMed Central

Sankar, Pamela; Cho, Mildred K.; Wolpe, Paul Root; Schairer, Cynthia

2008-01-01

Purpose Concern over stigma as a consequence of genetic testing has grown in response to the recent increase in genetic research and testing resulting from the Human Genome Project. However, whether a genetic or hereditary basis necessarily confers a stigma to a condition remains unexamined. Methods We performed a qualitative interview study with 86 individuals with one of four conditions: deafness or hearing loss, breast cancer, sickle cell disease, and cystic fibrosis. The first two groups were divided approximately between people who ascribed their conditions to a genetic or hereditary cause and those who did not. Results Respondents interpreted genetic or hereditary causes and nongenetic causes in a variety of ways. Subjects with breast cancer reported the most consistently negative interpretation of genetic cause. This response concerned future ill health, not an enduring sense of stigma. Deaf and hard of hearing subjects provided the most consistently positive comments about a genetic or hereditary basis to their condition, casting familial hearing loss as a vital component of group and individual identity. Respondents with sickle cell disease and cystic fibrosis offered similar and positive interpretations of the genetic cause of their condition insofar as it meant their conditions were not contagious. Conclusions Although some subjects report feeling stigmatized as a result of their condition, this stigmatization is not uniformly associated with the condition’s cause, genetic or otherwise. Instead, stigma emerges from a variety of sources in the context of the lived experience of a particular condition. PMID:16418597

Update of the GJB2/DFNB1 mutation spectrum in Russia: a founder Ingush mutation del(GJB2-D13S175) is the most frequent among other large deletions

PubMed Central

Bliznetz, Elena A; Lalayants, Maria R; Markova, Tatiana G; Balanovsky, Oleg P; Balanovska, Elena V; Skhalyakho, Roza A; Pocheshkhova, Elvira A; Nikitina, Natalya V; Voronin, Sergey V; Kudryashova, Elena K; Glotov, Oleg S; Polyakov, Alexander V

2017-01-01

Although mutations in the GJB2 gene sequence make up the majority of variants causing autosomal-recessive non-syndromic hearing loss, few large deletions have been shown to contribute to DFNB1 deafness. Currently, genetic testing for DFNB1 hearing loss includes GJB2 sequencing and DFNB1 deletion analysis for two common large deletions, del(GJB6-D13S1830) and del(GJB6-D13S1854). Here, we report frequency in Russia, clinical significance and evolutionary origins of a 101 kb deletion, del(GJB2-D13S175), recently identified by us. In multiethnic cohort of 1104 unrelated hearing loss patients with biallelic mutations at the DFNB1 locus, the del(GJB2-D13S175) allele frequency of up to 0.5% (11/2208) was determined and this allele was shown to be predominantly associated with profound sensorineural hearing loss. Additionally, eight previously unpublished GJB2 mutations were described in this study. All patients carrying del(GJB2-D13S175) were of the Ingush ancestry. Among normal hearing individuals, del(GJB2-D13S175) was observed in Russian Republic of Ingushetia with a carrier rate of ~1% (2/241). Analysis of haplotypes associated with the deletion revealed a common founder in the Ingushes, with age of the deletion being ~3000 years old. Since del(GJB2-D13S175) was missed by standard methods of GJB2 analysis, del(GJB2-D13S175) detection has been added to our routine testing strategy for DFNB1 hearing loss. PMID:28405014

Development and modification of a Gaussian and non-Gaussian noise exposure system

NASA Astrophysics Data System (ADS)

Schlag, Adam W.

Millions of people across the world currently have noise induced hearing loss, and many are working in conditions with both continuous Gaussian and non-Gaussian noises that could affect their hearing. It was hypothesized that the energy of the noise was the cause of the hearing loss and did not depend on temporal pattern of a noise. This was referred to as the equal energy hypothesis. This hypothesis has been shown to have limitations though. This means that there is a difference in the types of noise a person receives to induce hearing loss and it is necessary to build a system that can easily mimic various conditions to conduct research. This study builds a system that can produce both non-Gaussian impulse/impact noises and continuous Gaussian noise. It was found that the peak sound pressure level of the system could reach well above the needed 120 dB level to represent acoustic trauma and could replicate well above the 85 dB A-weighted sound pressure level to produce conditions of gradual developing hearing loss. The system reached a maximum of 150 dB sound peak pressure level and a maximum of 133 dB A-weighted sound pressure level. Various parameters could easily be adjusted to control the sound, such as the high and low cutoff frequency to center the sound at 4 kHz. The system build can easily be adjusted to create numerous sound conditions and will hopefully be modified and improved in hopes of eventually being used for animal studies to lead to the creation of a method to treat or prevent noise induced hearing loss.

Experiences of hearing loss and views towards interventions to promote uptake of rehabilitation support among UK adults.

PubMed

Rolfe, Crystal; Gardner, Benjamin

2016-11-01

Effective hearing loss rehabilitation support options are available. Yet, people often experience delays in receiving rehabilitation support. This study aimed to document support-seeking experiences among a sample of UK adults with hearing loss, and views towards potential strategies to increase rehabilitation support uptake. People with hearing loss were interviewed about their experiences of seeking support, and responses to hypothetical intervention strategies, including public awareness campaigns, a training programme for health professionals, and a national hearing screening programme. Semi-structured qualitative interview design with thematic analysis. Twenty-two people with hearing loss, aged 66-88. Three themes, representing barriers to receiving rehabilitation support and potential areas for intervention, were identified: making the journey from realization to readiness, combatting social stigma, and accessing appropriate services. Barriers to receiving support mostly focused on appraisal of hearing loss symptoms. Interventions enabling symptom appraisal, such as routine screening, or demonstrating how to raise the topic effectively with a loved one, were welcomed. Interventions to facilitate realization of hearing loss should be prioritized. Raising awareness of the symptoms and prevalence of hearing loss may help people to identify hearing problems and reduce stigma, in turn increasing hearing loss acceptance.

A large deletion in GPR98 causes type IIC Usher syndrome in male and female members of an Iranian family.

PubMed

Hilgert, N; Kahrizi, K; Dieltjens, N; Bazazzadegan, N; Najmabadi, H; Smith, R J H; Van Camp, G

2009-04-01

Usher syndrome (USH) is a clinically and genetically heterogeneous disease. The three recognised clinical phenotypes (types I, II and III; USH1, USH2 and USH3) are caused by mutations in nine different genes. USH2C is characterised by moderate to severe hearing loss, retinitis pigmentosa and normal vestibular function. One earlier report describes mutations in GPR98 (VLGR1) in four families segregating this phenotype. To detect the disease-causing mutation in an Iranian family segregating USH2C. In this family, five members had a phenotype compatible with Usher syndrome, and two others had nonsyndromic hearing loss. Mutation analysis of all 90 coding exons of GPR98. Consistent with these clinical findings, the five subjects with USH carried a haplotype linked to the USH2C locus, whereas the two subjects with nonsyndromic hearing loss did not. We identified a new mutation in GPR98 segregating with USH2C in this family. The mutation is a large deletion g.371657_507673del of exons 84 and 85, presumably leading to a frameshift. A large GPR98 deletion of 136 017 bp segregates with USH2C in an Iranian family. To our knowledge, this is only the second report of a GPR98 mutation, and the first report on male subjects with USH2C and a GPR98 mutation.

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

PubMed

Aarhus, Lisa; Tambs, Kristian; Engdahl, Bo

2015-12-01

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

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

PubMed Central

Tambs, Kristian; Engdahl, Bo

2015-01-01

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

Risk factors and prevalence of newborn hearing loss in a private health care system of Porto Velho, Northern Brazil

PubMed Central

de Oliveira, Juliana Santos; Rodrigues, Liliane Barbosa; Aurélio, Fernanda Soares; da Silva, Virgínia Braz

2013-01-01

OBJECTIVE: To determine the prevalence of hearing loss and to analyze the results of newborn hearing screening and audiological diagnosis in private health care systems. METHODS Cross-sectional and retrospective study in a database of newborn hearing screening performed by a private clinic in neonates born in private hospitals of Porto Velho, Rondônia, Northern Brazil. The screening results, the risk for hearing loss, the risk indicators for hearing loss and the diagnosis were descriptively analyzed. Newborns cared in rooming in with their mothers were compared to those admitted to the Intensive Care Unit regarding risk factors for hearing loss. RESULTS: Among 1,146 (100%) enrolled newborns, 1,064 (92.8%) passed and 82 (7.2%) failed the hearing screening. Among all screened neonates, 1,063 (92.8%) were cared in rooming and 83 (7.2%) needed intensive care; 986 (86.0%) were considered at low risk and 160 (14.0%) at high risk for hearing problems. Of the 160 patients identified as having high risk for hearing loss, 83 (37.7%) were admitted to an hospitalized in the Intensive Care Unit, 76 (34.5%) used ototoxic drugs and 38 (17.2%) had a family history of hearing loss in childhood. Hearing loss was diagnosed in two patients (0.2% of the screened sample). CONCLUSIONS: The prevalence of hearing loss in newborns from private hospitals was two cases per 1,000 evaluated patients. The use of ototoxic drugs, admission to Intensive Care Unit and family history of hearing loss were the most common risk factors for hearing loss in the studied population. PMID:24142311

Deletion of SLC19A2, the high affinity thiamine transporter, causes selective inner hair cell loss and an auditory neuropathy phenotype.

PubMed

Liberman, M C; Tartaglini, E; Fleming, J C; Neufeld, E J

2006-09-01

Mutations in the gene coding for the high-affinity thiamine transporter Slc19a2 underlie the clinical syndrome known as thiamine-responsive megaloblastic anemia (TRMA) characterized by anemia, diabetes, and sensorineural hearing loss. To create a mouse model of this disease, a mutant line was created with targeted disruption of the gene. Cochlear function is normal in these mutants when maintained on a high-thiamine diet. When challenged with a low-thiamine diet, Slc19a2-null mice showed 40-60 dB threshold elevations by auditory brainstem response (ABR), but only 10-20 dB elevation by otoacoustic emission (OAE) measures. Wild-type mice retain normal hearing on either diet. Cochlear histological analysis showed a pattern uncommon for sensorineural hearing loss: selective loss of inner hair cells after 1-2 weeks on low thiamine and significantly greater inner than outer hair cell loss after longer low-thiamine challenges. Such a pattern is consistent with the observed discrepancy between ABR and OAE threshold shifts. The possible role of thiamine transport in other reported cases of selective inner hair cell loss is considered.

The Dose Response Relationship between In Ear Occupational Noise Exposure and Hearing Loss

PubMed Central

Rabinowitz, Peter M.; Galusha, Deron; Dixon-Ernst, Christine; Clougherty, Jane E.; Neitzel, Richard L.

2014-01-01

Objectives Current understanding of the dose-response relationship between occupational noise and hearing loss is based on cross-sectional studies prior to the widespread use hearing protection and with limited data regarding noise exposures below 85dBA. We report on the hearing loss experience of a unique cohort of industrial workers with daily monitoring of noise inside of hearing protection devices. Methods At an industrial facility, workers exhibiting accelerated hearing loss were enrolled in a mandatory program to monitor daily noise exposures inside of hearing protection. We compared these noise measurements (as time-weighted LAVG) to interval rates of high frequency hearing loss over a six year period using a mixed effects model, adjusting for potential confounders. Results Workers’ high frequency hearing levels at study inception averaged more than 40 dB hearing threshold level (HTL). Most noise exposures were less than 85dBA (mean LAVG 76 dBA, interquartile range 74 to 80 dBA). We found no statistical relationship between LAvg and high frequency hearing loss (p = 0.53). Using a metric for monthly maximum noise exposure did not improve model fit. Conclusion At-ear noise exposures below 85dBA did not show an association with risk of high frequency hearing loss among workers with substantial past noise exposure and hearing loss at baseline. Therefore, effective noise control to below 85dBA may lead to significant reduction in occupational hearing loss risk in such individuals. Further research is needed on the dose response relationship of noise and hearing loss in individuals with normal hearing and little prior noise exposure. PMID:23825197

The influence of mood on the perception of hearing-loss related quality of life in people with hearing loss and their significant others.

PubMed

Preminger, Jill E; Meeks, Suzanne

2010-04-01

The purpose of this research was to investigate the congruent/incongruent perceptions of hearing-loss related quality of life between members of couples and to determine how incongruence was affected by individual psychosocial characteristics, specifically measures of mood (negative affect and positive affect), stress, and communication in the marriage. An exploratory correlational analysis was performed on data for 52 couples in which only one member had a hearing loss. In the regression analyses the independent variables were hearing-loss related quality of life scores measured in people with hearing loss, measured in significant others, and differences in hearing-loss related quality of life among members of a couple. The results demonstrate that both in people with hearing loss and their significant others, perceptions of hearing-loss related quality of life is highly correlated with negative mood scores. Incongruence in hearing-loss related quality of life scores reported by members of a couple were highly correlated with negative affect measured within each individual. Future research evaluating the effectiveness of audiologic rehabilitation can use measures of mood as an outcome variable.

Correlation Between Cerebellar Retraction and Hearing Loss After Microvascular Decompression for Hemifacial Spasm: A Prospective Study.

PubMed

Li, Ning; Zhao, Wei-Guo; Pu, Chun-Hua; Yang, Wen-Lei

2017-06-01

This study prospectively investigated the relationship between cerebellar retraction factors measured on preoperative magnetic resonance and the development of postoperative hearing loss and evaluated their potential role in predicting the possibility of hearing loss after microvascular decompression (MVD) for hemifacial spasm (HFS). The study included 110 patients clinically diagnosed with primary HFS who underwent MVD in our department. The cerebellar retraction factors were quantitatively measured on preoperative magnetic resonance. Associations of cerebellar retraction and other risk factors with postoperative hearing loss were analyzed. Eleven patients (10%) developed nonserviceable hearing loss after MVD. Compared with the group without hearing loss, the cerebellar retraction distance and depth of the group with hearing loss were significantly greater (P < 0.05). Multivariate logistic regression analysis showed that greater cerebellar retraction depth was significantly associated with the higher incidence of postoperative hearing loss (P < 0.05). The results in this study strongly suggested the correlation between the cerebellar retraction depth and the possibility of hearing loss after MVD for HFS. In addition, cerebellar retraction depth could be considered as a useful tool to predict the risk of post-MVD hearing loss. Copyright © 2017 Elsevier Inc. All rights reserved.

High-frequency Audiometry Hearing on Monitoring of Individuals Exposed to Occupational Noise: A Systematic Review

PubMed Central

Antonioli, Cleonice Aparecida Silva; Momensohn-Santos, Teresa Maria; Benaglia, Tatiana Aparecida Silva

2015-01-01

Introduction  The literature reports on high-frequency audiometry as one of the exams used on hearing monitoring of individuals exposed to high sound pressure in their work environment, due to the method́s greater sensitivity in early identification of hearing loss caused by noise. The frequencies that compose the exam are generally between 9 KHz and 20KHz, depending on the equipment. Objective  This study aims to perform a retrospective and secondary systematic revision of publications on high-frequency audiometry on hearing monitoring of individuals exposed to occupational noise. Data Synthesis  This systematic revision followed the methodology proposed in the Cochrane Handbook, focusing on the question: “Is High-frequency Audiometry more sensitive than Conventional Audiometry in the screening of early hearing loss individuals exposed to occupational noise?” The search was based on PubMed data, Base, Web of Science (Capes), Biblioteca Virtual em Saúde (BVS), and in the references cited in identified and selected articles. The search resulted in 6059 articles in total. Of these, only six studies were compatible with the criteria proposed in this study. Conclusion  The performed meta-analysis does not definitively answer the study's proposed question. It indicates that the 16 KHz high frequency audiometry (HFA) frequency is sensitive in early identification of hearing loss in the control group (medium difference (MD = 8.33)), as well as the 4 KHz frequency (CA), this one being a little less expressive (MD = 5.72). Thus, others studies are necessary to confirm the HFA importance for the early screening of hearing loss on individuals exposed to noise at the workplace. PMID:27413413

A Data-Driven Synthesis of Research Evidence for Domains of Hearing Loss, as Reported by Adults With Hearing Loss and Their Communication Partners

PubMed Central

Akeroyd, Michael A.

2017-01-01

A number of assessment tools exist to evaluate the impact of hearing loss, with little consensus among researchers as to either preference or psychometric adequacy. The item content of hearing loss assessment tools should seek to capture the impact of hearing loss on everyday life, but to date no one has synthesized the range of hearing loss complaints from the perspectives of the person with hearing loss and their communication partner. The current review aims to synthesize the evidence on person with hearing loss- and communication partner-reported complaints of hearing loss. Searches were conducted in Cos Conference Papers Index, the Cumulative Index to Nursing and Allied Health Literature, Excerpta Medica Database, PubMed, Web of Science, and Google Scholar to identify publications from May 1982 to August 2015. A manual search of four relevant journals updated the search to May 2017. Of the 9,516 titles identified, 78 records (comprising 20,306 participants) met inclusion criteria and were taken through to data collection. Data were analyzed using meta-ethnography to form domains representing the person with hearing loss- and communication partner-reported complaints of hearing loss as reported in research. Domains and subdomains mutual to both perspectives are related to “Auditory” (listening, communicating, and speaking), “Social” (relationships, isolation, social life, occupational, and interventions), and “Self” (effort and fatigue, emotions, identity, and stigma). Our framework contributes fundamental new knowledge and a unique resource that enables researchers and clinicians to consider the broader impacts of hearing loss. Our findings can also be used to guide questions during diagnostic assessment and to evaluate existing measures of hearing loss. PMID:28982021

Studies of Physician-Patient Communication with Older Patients: How Often is Hearing Loss Considered? A Systematic Literature Review.

PubMed

Cohen, Jamie M; Blustein, Jan; Weinstein, Barbara E; Dischinger, Hannah; Sherman, Scott; Grudzen, Corita; Chodosh, Joshua

2017-08-01

Hearing loss is remarkably prevalent in the geriatric population: one-quarter of adults aged 60-69 and 80% of adults aged 80 years and older have bilateral disabling loss. Only about one in five adults with hearing loss wears a hearing aid, leaving many vulnerable to poor communication with healthcare providers. We quantified the extent to which hearing loss is mentioned in studies of physician-patient communication with older patients, and the degree to which hearing loss is incorporated into analyses and findings. We conducted a structured literature search within PubMed for original studies of physician-patient communication with older patients that were published since 2000, using the natural language phrase "older patient physician communication." We identified 409 papers in the initial search, and included 67 in this systematic review. Of the 67 papers, only 16 studies (23.9%) included any mention of hearing loss. In six of the 16 studies, hearing loss was mentioned only; in four studies, hearing loss was used as an exclusion criterion; and in two studies, the extent of hearing loss was measured and reported for the sample, with no further analysis. Three studies examined or reported on an association between hearing loss and the quality of physician-patient communication. One study included an intervention to temporarily mitigate hearing loss to improve communication. Less than one-quarter of studies of physician-elderly patient communication even mention that hearing loss may affect communication. Methodologically, this means that many studies may have omitted an important potential confounder. Perhaps more importantly, research in this field has largely overlooked a highly prevalent, important, and remediable influence on the quality of communication. © 2017, Copyright the Authors Journal compilation © 2017, The American Geriatrics Society.

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. ...

Do ambient noise exposure levels predict hearing loss in a modern industrial cohort?

PubMed Central

Rabinowitz, P M; Galusha, D; Dixon‐Ernst, C; Slade, M D; Cullen, M R

2007-01-01

Background Much of what is known about the exposure–response relationship between occupational noise exposures and hearing loss comes from cross‐sectional studies conducted before the widespread implementation of workplace hearing conservation programmes. Little is known about the current relationship of ambient noise exposure measurements to hearing loss risk. Aim To examine the relationship between rates of high frequency hearing loss and measured levels of noise exposure in a modern industrial workforce. Methods Ten‐year hearing loss rates were determined for 6217 employees of an aluminium manufacturing company. Industrial hygiene and human resources records allowed for reconstruction of individual noise exposures. Hearing loss rates were compared to ANSI 3.44 predictions based on age and noise exposure. Associations between hearing loss, noise exposure, and covariate risk factors were assessed using multivariate regression. Results Workers in higher ambient noise jobs tended to experience less high frequency hearing loss than co‐workers exposed at lower noise levels. This trend was also seen in stratified analyses of white males and non‐hunters. At higher noise exposure levels, the magnitude of hearing loss was less than predicted by ANSI 3.44 formulae. There was no indication that a healthy worker effect could explain these findings. The majority of 10 dB standard threshold shifts (STS) occurred in workers whose calculated ambient noise exposures were less than or equal to 85 dBA. Conclusions In this modern industrial cohort, hearing conservation efforts appear to be reducing hearing loss rates, especially at higher ambient noise levels. This could be related to differential use of hearing protection. The greatest burden of preventable occupational hearing loss was found in workers whose noise exposure averaged 85 dBA or less. To further reduce rates of occupational hearing loss, hearing conservation programmes may require innovative approaches targeting workers with noise exposures close to 85 dBA. PMID:16973736

Candidate's thesis: Platelet-activating factor-induced hearing loss: mediated by nitric oxide?

PubMed

Rhee, Chung-Ku

2003-12-01

Platelet-activating factor (PAF)in middle ear effusion is thought to induce hearing loss. The purpose of this study is to investigate the role of nitric oxide (NO) in the mechanism of PAF-induced hearing loss by studying the effects of PAF application on the round window membrane (RWM) with and without PAF-antagonist NO-blocker. Longitudinal study on randomized guinea pigs using PAF to induce hearing loss. METHODS Guinea pigs were divided into four groups: PBS, PAF, PAF-antagonist, and L-NAME. The PBS group received phosphate buffered saline (PBS) and the PAF groups received 10, 20, and 40 microg of PAF soaked into gelfoam and placed on the RWM. PAF-antagonist (WEB 2170) and NOS inhibitor NG-nitro-l-arginine-methylester (L-NAME) were injected intraperitoneally prior to PAF 20 microg application on the RWM. The following three tests were performed on each animal group: Hearing was tested with an auditory brainstem response (ABR) test over 24 hours. At the end of 24 hours, cochlear hair cells were examined by scanning electron microscopy (SEM) and immunohistochemistry was carried out on the cochlea to test the expression of inducible nitric oxide synthase (iNOS). The PAF group developed significant elevation of ABR threshold and cochlear hair cell damage in the SEM group as compared with the PBS control group. The PAF-antagonist (WEB 2170) and the L-NAME groups did not show significant elevation of ABR threshold and cochlear hair cell damage compared with the group administered PAF 20 microg, but in the PAF-antagonist group, the elevation of ABR threshold was significant compared with that of the PBS control group, whereas it was not significant compared with the PBS group in the L-NAME group. Strong expression of iNOS on cochlea was observed in the PAF group and lighter expression was seen in PBS, WEB 2170, and L-NAME groups. This study demonstrated that PAF placed on the RWM induced hearing loss and cochlear hair cell damage. The PAF-antagonists and L-NAME prevented the PAF-induced hearing loss and inhibited iNOS expression in the cochlea. These findings suggest that the PAF-induced hearing loss caused by cochlear hair cell damage may have been mediated by NO. PAF-antagonists and L-NAME may have future therapeutic implications in preventing sensorineural hearing loss associated with chronic otitis media. The results of this study have significant potential clinical application.

Phonological Awareness at 5 years of age in Children who use Hearing Aids or Cochlear Implants

PubMed Central

Ching, Teresa Y.C.; Cupples, Linda

2015-01-01

Children with hearing loss typically underachieve in reading, possibly as a result of their underdeveloped phonological skills. This study addressed the questions of whether the development of phonological awareness (PA) is influenced by 1) the degree of hearing loss; and 2) whether performance of children with severe-profound hearing loss differed according to the hearing devices used. Drawing on data collected as part of the Longitudinal Outcomes of Children with Hearing Impairment (LOCHI, www.outcomes.nal.gov.au) study, the authors found that sound-matching scores of children with hearing loss ranging from mild to profound degrees were, on average, within the normal range. The degree of hearing loss did not have a significant impact on scores, but there was a non-significant tendency for the proportion of children who achieved zero scores to increase with increase in hearing loss. For children with severe hearing loss, there was no significant group difference in scores among children who used bilateral hearing aids, bimodal fitting (a cochlear implant and a hearing aid in contralateral ears), and bilateral cochlear implants. Although there is a need for further prospective research, professionals have an important role in targeting PA skills for rehabilitation of young children with hearing loss. PMID:26929789

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

PubMed Central

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

2018-01-01

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

PubMed Central

Forli, F.; Guglielmi, V.; De Corso, E.; Paludetti, G.; Berrettini, S.; Fetoni, A.R.

2016-01-01

SUMMARY Age-related hearing loss (ARHL) has a multifactorial pathogenesis and it is an inevitable hearing impairment associated with reduction of communicative skills related to ageing. Increasing evidence has linked ARHL to more rapid progression of cognitive decline and incidental dementia. Many aspects of daily living of elderly people have been associated to hearing abilities, showing that hearing loss (HL) affects the quality of life, social relationships, motor skills, psychological aspects and function and morphology in specific brain areas. Epidemiological and clinical studies confirm the assumption of a relationship between these conditions. However, the mechanisms are still unclear and are reviewed herein. Long-term hearing deprivation of auditory inputs can impact cognitive performance by decreasing the quality of communication leading to social isolation and depression and facilitate dementia. On the contrary, the limited cognitive skills may reduce the cognitive resources available for auditory perception, increasing the effects of HL. In addition, hearing loss and cognitive decline may reflect a 'common cause' on the auditory pathway and brain. In fact, some pathogenetic factors are recongised in common microvascular disease factors such as diabetes, atherosclerosis and hypertension. Interdisciplinary efforts to investigate and address HL in the context of brain and cognitive ageing are needed. Surprisingly, few studies have been adressed on the effectiveness of hearing aids in changing the natural history of cognitive decline. Effective interventions with hearing aids or cochlear implant may improve social and emotional function, communication, cognitive function and positively impact quality of life. The aim of this review is to overview new insights on this challenging topic and provide new ideas for future research. PMID:27214827

The challenges of starting a cochlear implant programme in a developing country.

PubMed

Krishnamoorthy, Kumaresh; Samy, Ravi N; Shoman, Nael

2014-10-01

Deafness is indeed a silent disability in many parts of the world, and the majority of people who have hearing impairment live in developing countries. With rising economy and developing nations becoming hub of industrialization, hearing loss may increase in these countries. In this review, the authors have elected to focus the discussion on India to frame the challenges of cochlear implants in a developing country. This article reviews the common causes of hearing loss, the challenges faced by those with hearing impairment and why the penetration of these devices is low and also reviews some reasons for the inability of the government to support the implant programme in India. Early identification of hearing is crucial towards ensuring appropriate hearing rehabilitation; it is, however, challenged by various factors, including public awareness, absence of a national new born screening programme, accessibility to diagnostic centres, availability of trained personnel and equipment and patient affordability. Cochlear implants are a proven auditory rehabilitative option for individuals with severe to profound sensorineural hearing loss, who otherwise do not benefiting from hearing aids. Nevertheless, only a small percentage of these individuals receive cochlear implants, and cost remains a leading prohibitive factor, particularly in developing countries. For example, in India, the personal average annual income is well below US $2000, whereas these devices cost between $12,000 and $25,000, exclusive of hospital and staff fees. Hence, the technology is virtually unavailable to the masses. To overcome the cost limitation of those who would benefit from cochlear implants countries such as India and China have started to develop their own indigenous implants.

76 FR 69743 - The Development and Evaluation of Human Cytomegalovirus Vaccines; Public Workshop

Federal Register 2010, 2011, 2012, 2013, 2014

2011-11-09

... immune systems. Congenital HCMV infection causes mental retardation, learning disabilities, hearing loss, vision loss, and other disabilities. Patients undergoing stem cell or solid-organ transplants are at... available basis beginning at 8 a.m. If you need special accommodations due to a disability, please contact...

Dementia and Hearing Loss: Interrelationships and Treatment Considerations.

PubMed

Hubbard, H Isabel; Mamo, Sara K; Hopper, Tammy

2018-07-01

Hearing loss is common among typically aging older adults and those with dementia. In recent years, there has been a renewed interest in the relationship between hearing and cognition among older adults, and in hearing loss as a modifiable risk factor for dementia. However, relatively less attention has been focused on the management of hearing loss among individuals with dementia and the key roles of speech-language pathologists and audiologists in providing such care. In this article, the authors review the literature on hearing loss and dementia, and analyze the research evidence for treatment of hearing loss in the context of major neurocognitive disorders, such as Alzheimer's disease. This article provides an up-to-date review of research evidence for hearing interventions, as well as recommendations for speech-language pathologists and audiologists to work together to ensure access to hearing health care and increased opportunities for meaningful life engagement for people with dementia and hearing loss. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

A new mutation in the COL4A3 gene responsible for autosomal dominant Alport syndrome, which only generates hearing loss in some carriers.

PubMed

Rosado, Consolación; Bueno, Elena; Fraile, Pilar; García-Cosmes, Pedro; González-Sarmiento, Rogelio

2015-01-01

Bilateral sensorineural hearing loss is a characteristic feature of Alport syndrome, which is always linked to renal manifestations so they have a parallel evolution and prognosis, and deafness helps to identify the renal disease. We report a family that suffers an autosomal dominant Alport syndrome caused by a previously undescribed mutation in the COL4A3 gene, in which several members have hearing impairment as the only clinical manifestation, suggesting that in this family deafness can occur independent of renal disease. This mutation is also present in a patient with anterior lenticonus, an observation only found in families with recessive and sex-linked Alport disease. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

Hearing loss in children with growth hormone deficiency.

PubMed

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

2017-09-01

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

Binaural integration: a challenge to overcome for children with hearing loss.

PubMed

Gordon, Karen A; Cushing, Sharon L; Easwar, Vijayalakshmi; Polonenko, Melissa J; Papsin, Blake C

2017-12-01

Access to bilateral hearing can be provided to children with hearing loss by fitting appropriate hearing devices to each affected ear. It is not clear, however, that bilateral input is properly integrated through hearing devices to promote binaural hearing. In the present review, we examine evidence indicating that abnormal binaural hearing continues to be a challenge for children with hearing loss despite early access to bilateral input. Behavioral responses and electrophysiological data in children, combined with data from developing animal models, reveal that deafness in early life disrupts binaural hearing and that present hearing devices are unable to reverse these changes and/or promote expected development. Possible limitations of hearing devices include mismatches in binaural place, level, and timing of stimulation. Such mismatches could be common in children with hearing loss. One potential solution is to modify present device fitting beyond providing audibility to each ear by implementing binaural fitting targets. Efforts to better integrate bilateral input could improve spatial hearing in children with hearing loss.

Risk of Bacterial Meningitis in Children with Cochlear Implants

MedlinePlus

... Hearing Loss Homepage Basics Noise-Induced Hearing Loss Genetics of Hearing Loss Screening & Diagnosis Types of Hearing Loss About Sound Treatment & Intervention Services Learning Language Bacterial Meningitis Studies Data & Statistics EHDI Annual Data 2016 2015 2014 2013 ...

Automatic Speech Recognition Predicts Speech Intelligibility and Comprehension for Listeners With Simulated Age-Related Hearing Loss.

PubMed

Fontan, Lionel; Ferrané, Isabelle; Farinas, Jérôme; Pinquier, Julien; Tardieu, Julien; Magnen, Cynthia; Gaillard, Pascal; Aumont, Xavier; Füllgrabe, Christian

2017-09-18

The purpose of this article is to assess speech processing for listeners with simulated age-related hearing loss (ARHL) and to investigate whether the observed performance can be replicated using an automatic speech recognition (ASR) system. The long-term goal of this research is to develop a system that will assist audiologists/hearing-aid dispensers in the fine-tuning of hearing aids. Sixty young participants with normal hearing listened to speech materials mimicking the perceptual consequences of ARHL at different levels of severity. Two intelligibility tests (repetition of words and sentences) and 1 comprehension test (responding to oral commands by moving virtual objects) were administered. Several language models were developed and used by the ASR system in order to fit human performances. Strong significant positive correlations were observed between human and ASR scores, with coefficients up to .99. However, the spectral smearing used to simulate losses in frequency selectivity caused larger declines in ASR performance than in human performance. Both intelligibility and comprehension scores for listeners with simulated ARHL are highly correlated with the performances of an ASR-based system. In the future, it needs to be determined if the ASR system is similarly successful in predicting speech processing in noise and by older people with ARHL.

Impact of hearing loss in the workplace: raising questions about partnerships with professionals.

PubMed

Jennings, Mary Beth; Shaw, Lynn

2008-01-01

The number of adults with hearing loss who continue to work later in life is growing. Persons with hearing loss are generally unaware of the role that audiologists, occupational therapists, and vocational rehabilitation counsellors might play in the assessment of the workplace environment and appropriate accommodations. Three narratives of adults with hearing loss are used to demonstrate the gaps in accessing information, technology and services needed to maintain optimal work performance and productivity. The lack of recognition of the multidimensional needs of older workers with hearing loss and the lack of timely coordination of services led to all three persons acting alone in trying to access services and supports. In two of the three cases the impact of the hearing loss resulted in further unexpected losses such as the loss of employment and the loss of a worker-identity. There is an urgent need for partnering with persons who are hard of hearing to develop new strategies for knowledge exchange, more thorough assessment of hearing demands and modifications in the workplace, and interdisciplinary approaches to service specific to the needs of hard of hearing persons.

A cool approach to reducing electrode-induced trauma: Localized therapeutic hypothermia conserves residual hearing in cochlear implantation.

PubMed

Tamames, Ilmar; King, Curtis; Bas, Esperanza; Dietrich, W Dalton; Telischi, Fred; Rajguru, Suhrud M

2016-09-01

The trauma caused during cochlear implant insertion can lead to cell death and a loss of residual hair cells in the cochlea. Various therapeutic approaches have been studied to prevent cochlear implant-induced residual hearing loss with limited success. In the present study, we show the efficacy of mild to moderate therapeutic hypothermia of 4 to 6 °C applied to the cochlea in reducing residual hearing loss associated with the electrode insertion trauma. Rats were randomly distributed in three groups: control contralateral cochleae, normothermic implanted cochleae and hypothermic implanted cochleae. Localized hypothermia was delivered to the middle turn of the cochlea for 20 min before and after implantation using a custom-designed probe perfused with cooled fluorocarbon. Auditory brainstem responses (ABRs) were recorded to assess the hearing function prior to and post-cochlear implantation at various time points up to 30 days. At the conclusion of the trials, inner ears were harvested for histology and cell count. The approach was extended to cadaver temporal bones to study the potential surgical approach and efficacy of our device. In this case, the hypothermia probe was placed next to the round window niche via the facial recess or a myringotomy. A significant loss of residual hearing was observed in the normothermic implant group. Comparatively, the residual hearing in the cochleae receiving therapeutic hypothermia was significantly conserved. Histology confirmed a significant loss of outer hair cells in normothermic cochleae receiving the surgical trauma when compared to the hypothermia treated group. In human temporal bones, a controlled and effective cooling of the cochlea was achieved using our approach. Collectively, these results suggest that therapeutic hypothermia during cochlear implantation may reduce traumatic effects of electrode insertion and improve conservation of residual hearing. Copyright © 2016 The Authors. Published by Elsevier B.V. All rights reserved.

Experiences of hearing loss and views towards interventions to promote uptake of rehabilitation support among UK adults

PubMed Central

Rolfe, Crystal; Gardner, Benjamin

2016-01-01

Abstract Objective: Effective hearing loss rehabilitation support options are available. Yet, people often experience delays in receiving rehabilitation support. This study aimed to document support-seeking experiences among a sample of UK adults with hearing loss, and views towards potential strategies to increase rehabilitation support uptake. People with hearing loss were interviewed about their experiences of seeking support, and responses to hypothetical intervention strategies, including public awareness campaigns, a training programme for health professionals, and a national hearing screening programme. Design: Semi-structured qualitative interview design with thematic analysis. Study sample: Twenty-two people with hearing loss, aged 66–88. Results: Three themes, representing barriers to receiving rehabilitation support and potential areas for intervention, were identified: making the journey from realization to readiness, combatting social stigma, and accessing appropriate services. Barriers to receiving support mostly focused on appraisal of hearing loss symptoms. Interventions enabling symptom appraisal, such as routine screening, or demonstrating how to raise the topic effectively with a loved one, were welcomed. Conclusions: Interventions to facilitate realization of hearing loss should be prioritized. Raising awareness of the symptoms and prevalence of hearing loss may help people to identify hearing problems and reduce stigma, in turn increasing hearing loss acceptance. PMID:27379464

Masking Release in Children and Adults With Hearing Loss When Using Amplification

PubMed Central

McCreery, Ryan; Kopun, Judy; Lewis, Dawna; Alexander, Joshua; Stelmachowicz, Patricia

2016-01-01

Purpose This study compared masking release for adults and children with normal hearing and hearing loss. For the participants with hearing loss, masking release using simulated hearing aid amplification with 2 different compression speeds (slow, fast) was compared. Method Sentence recognition in unmodulated noise was compared with recognition in modulated noise (masking release). Recognition was measured for participants with hearing loss using individualized amplification via the hearing-aid simulator. Results Adults with hearing loss showed greater masking release than the children with hearing loss. Average masking release was small (1 dB) and did not depend on hearing status. Masking release was comparable for slow and fast compression. Conclusions The use of amplification in this study contrasts with previous studies that did not use amplification. The results suggest that when differences in audibility are reduced, participants with hearing loss may be able to take advantage of dips in the noise levels, similar to participants with normal hearing. Although children required a more favorable signal-to-noise ratio than adults for both unmodulated and modulated noise, masking release was not statistically different. However, the ability to detect a difference may have been limited by the small amount of masking release observed. PMID:26540194

Universal newborn screening for congenital CMV infection: what is the evidence of potential benefit?†

PubMed Central

Cannon, Michael J.; Griffiths, Paul D.; Aston, Van; Rawlinson, William D.

2015-01-01

SUMMARY Congenital CMV infection is a leading cause of childhood disability. Many children born with congenital CMV infection are asymptomatic or have nonspecific symptoms and therefore are typically not diagnosed. A strategy of newborn CMV screening could allow for early detection and intervention to improve clinical outcomes. Interventions might include antiviral drugs or nonpharmaceutical therapies such as speech-language therapy or cochlear implants. Using published data from developed countries, we analyzed existing evidence of potential benefit that could result from newborn CMV screening. We first estimated the numbers of children with the most important CMV-related disabilities (i.e. hearing loss, cognitive deficit, and vision impairment), including the age at which the disabilities occur. Then, for each of the disabilities, we examined the existing evidence for the effectiveness of various interventions. We concluded that there is good evidence of potential benefit from nonpharmaceutical interventions for children with delayed hearing loss that occurs by 9 months of age. Similarly, we concluded that there is fair evidence of potential benefit from antiviral therapy for children with hearing loss at birth and from nonpharmaceutical interventions for children with delayed hearing loss occurring between 9 and 24 months of age and for children with CMV-related cognitive deficits. We found poor evidence of potential benefit for children with delayed hearing loss occurring after 24 months of age and for children with vision impairment. Overall, we estimated that in the United States, several thousand children with congenital CMV could benefit each year from newborn CMV screening, early detection, and interventions. Copyright © 2014 John Wiley & Sons, Ltd. PMID:24760655

Early age noise exposure increases loudness perception - A novel animal model of hyperacusis.

PubMed

Alkharabsheh, Ana'am; Xiong, Fen; Xiong, Binbin; Manohar, Senthilvelan; Chen, Guangdi; Salvi, Richard; Sun, Wei

2017-04-01

The neural mechanisms that give rise to hyperacusis, a reduction in loudness tolerance, are largely unknown. Some reports suggest that hyperacusis is linked to childhood hearing loss. However, the evidence for this is largely circumstantial. In order to rigorously test this hypothesis, we studied loudness changes in rats caused by intense noise exposure (12 kHz narrow band noise, 115 dB SPL, 4 h) at postnatal 16 days. Rats without noise exposure were used as controls. The exposed noise group (n = 7) showed a mean 40-50 dB hearing loss compared to the control group (n = 8) at high frequencies (>= 8 kHz) and less hearing loss at lower frequencies. Loudness was evaluated using sound reaction time and loudness response functions in an operant conditioning-based behavioral task using narrow-band noise (40-110 dB SPL, centered at 2, 4 and 12 kHz). Interestingly, the sound reaction time of the noise group was significantly shorter than the control group at supra-threshold levels. The average reaction time was less than 100 ms in the noise group at 100 dB SPL, which was three times shorter than the control group. Our results indicate that early noise-induced hearing loss leads to a significant increase of loudness, a behavior indicative of hyperacusis. Our results are consistent with clinical reports suggesting that hearing loss at an early age is a significant risk factor for hyperacusis. Published by Elsevier B.V.

Prevalence of GJB2 Mutations in Affected Individuals from United Arab Emirates with Autosomal Recessive Nonsyndromic Hearing Loss.

PubMed

Tlili, Abdelaziz; Al Mutery, Abdullah; Kamal Eddine Ahmad Mohamed, Walaa; Mahfood, Mona; Hadj Kacem, Hassen

2017-11-01

Mutations in the gap junction protein beta 2 (GJB2) gene are responsible for more cases of nonsyndromic recessive hearing loss than any other gene. The purpose of our study was to evaluate the prevalence of GJB2 mutations among affected individuals from United Arab Emirates (UAE). There were 50 individuals diagnosed with hereditary hearing loss and 120 healthy individuals enrolled in the study. The Sanger sequencing method was used to screen the GJB2 coding region in all affected individuals. The c.-1G>A variant was determined by the polymerase chain reaction-restriction fragment length polymorphism method in normal individuals. Nine cases with bi-allelic mutations and three cases with mono-allelic mutations were detected in 12 out of 50 patients (24%). The homozygous mutation c.35delG was identified as the cause of hearing loss in six participants (12%). The mutation c.506G>A was identified in three affected individuals (6%). The allelic frequency (14%) and low percentage of individuals that were homozygous (2%) for the c.35delG mutation suggest that there are other genes responsible for nonsyndromic deafness in the UAE population. The results reported here are a preliminary step in collecting epidemiological data regarding autosomal recessive nonsyndromic hearing loss related to GJB2 gene mutations among the UAE population. The c.35delG mutation of the GJB2 gene is the most frequently seen causative mutation in the UAE and is followed by the p.Cys169Tyr mutation.

Spiral ganglion degeneration and hearing loss as a consequence of satellite cell death in saposin B-deficient mice.

PubMed

Akil, Omar; Sun, Ying; Vijayakumar, Sarath; Zhang, Wujuan; Ku, Tiffany; Lee, Chi-Kyou; Jones, Sherri; Grabowski, Gregory A; Lustig, Lawrence R

2015-02-18

Saposin B (Sap B) is an essential activator protein for arylsulfatase A in the hydrolysis of sulfatide, a lipid component of myelin. To study Sap B's role in hearing and balance, a Sap B-deficient (B(-/-)) mouse was evaluated. At both light and electron microscopy (EM) levels, inclusion body accumulation was seen in satellite cells surrounding spiral ganglion (SG) neurons from postnatal month 1 onward, progressing into large vacuoles preceding satellite cell degeneration, and followed by SG degeneration. EM also revealed reduced or absent myelin sheaths in SG neurons from postnatal month 8 onwards. Hearing loss was initially seen at postnatal month 6 and progressed thereafter for frequency-specific stimuli, whereas click responses became abnormal from postnatal month 13 onward. The progressive hearing loss correlated with the accumulation of inclusion bodies in the satellite cells and their subsequent degeneration. Outer hair cell numbers and efferent function measures (distortion product otoacoustic emissions and contralateral suppression) were normal in the B(-/-) mice throughout this period. Alcian blue staining of SGs demonstrated that these inclusion bodies corresponded to sulfatide accumulation. In contrast, changes in the vestibular system were much milder, but caused severe physiologic deficits. These results demonstrate that loss of Sap B function leads to progressive sulfatide accumulation in satellite cells surrounding the SG neurons, leading to satellite cell degeneration and subsequent SG degeneration with a resultant loss of hearing. Relative sparing of the efferent auditory and vestibular neurons suggests that alternate glycosphingolipid metabolic pathways predominate in these other systems. Copyright © 2015 the authors 0270-6474/15/353263-13$15.00/0.

The effects of maternal stress and child language ability on behavioral outcomes of children with congenital hearing loss at 18-24months.

PubMed

Topol, Deborah; Girard, Nicole; St Pierre, Lucille; Tucker, Richard; Vohr, Betty

2011-12-01

Prior studies have shown that children with congenital hearing loss have increased rates of behavior disorders. Child hearing loss has also been reported to be associated with increased maternal stress. Little is known about the behavior or the predictors of behavioral outcomes of children with hearing loss identified early and receiving Early Intervention services. The objective of this study was to identify the behavioral outcomes in early identified children with hearing loss and control hearing children at 18-24 months of age and to examine the impact of stress on early behavior development. It was hypothesized that children with hearing loss will have more behavior problems, and maternal stress will be associated with child behavior problems. Prospective observational. Children with and without congenital hearing loss and their mothers. The Parenting Stress Index and the Child Behavior Checklist. Children with hearing loss had increased scores for withdrawn and internalizing behavior. In multivariate analyses after adjusting for hearing loss, Neonatal Intensive Care Unit stay, and socioeconomic status, maternal stress independently contributed to higher scores for internalizing behavior, externalizing behavior, and total behavior problems. Maternal stress is an important correlate of behavior problems for children with hearing loss and should be considered by Early Intervention providers. Copyright © 2011 Elsevier Ltd. All rights reserved.

Non-syndromic hearing loss caused by the dominant cis mutation R75Q with the recessive mutation V37I of the GJB2 (Connexin 26) gene.

PubMed

Kim, Juwon; Jung, Jinsei; Lee, Min Goo; Choi, Jae Young; Lee, Kyung-A

2015-06-19

GJB2 alleles containing two cis mutations have been rarely found in non-syndromic hearing loss. Herein, we present a Korean patient with non-syndromic hearing loss caused by the R75Q cis mutation with V37I, which arose de novo in the father and was inherited by the patient. Biochemical coupling and hemichannel permeability assays were performed after molecular cloning and transfection of HEK293T cells. Student's t-tests or analysis of variance followed by Tukey's multiple comparison test was used as statistical analysis. Biochemical coupling was significantly reduced in connexin 26 (Cx26)-R75Q- and Cx26-V37I-transfected cells, with greater extent in Cx26-R75Q and Cx26-R75Q+V37I cells. Interestingly, our patient and his father with the mutations had more residual hearing compared with patients with the dominant mutation alone. Although the difference in hemichannel activity between R75Q alone and R75Q in combination with V37I failed to reach significance, it is of note that there is a possibility that V37I located upstream of R75Q might have the ability to ameliorate R75Q expression. Our study emphasizes the importance of cis mutations with R75Q, as the gene effect of R75Q can be modulated depending on the type of additional mutation.

Cochlear Homocysteine Metabolism at the Crossroad of Nutrition and Sensorineural Hearing Loss.

PubMed

Partearroyo, Teresa; Vallecillo, Néstor; Pajares, María A; Varela-Moreiras, Gregorio; Varela-Nieto, Isabel

2017-01-01

Hearing loss (HL) is one of the most common causes of disability, affecting 360 million people according to the World Health Organization (WHO). HL is most frequently of sensorineural origin, being caused by the irreversible loss of hair cells and/or spiral ganglion neurons. The etiology of sensorineural HL (SNHL) is multifactorial, with genetic and environmental factors such as noise, ototoxic substances and aging playing a role. The nutritional status is central in aging disability, but the interplay between nutrition and SNHL has only recently gained attention. Dietary supplementation could therefore constitute the first step for the prevention and potential repair of hearing damage before it reaches irreversibility. In this context, different epidemiological studies have shown correlations among the nutritional condition, increased total plasma homocysteine (tHcy) and SNHL. Several human genetic rare diseases are also associated with homocysteine (Hcy) metabolism and SNHL confirming this potential link. Accordingly, rodent experimental models have provided the molecular basis to understand the observed effects. Thus, increased tHcy levels and vitamin deficiencies, such as folic acid (FA), have been linked with SNHL, whereas long-term dietary supplementation with omega-3 fatty acids improved Hcy metabolism, cell survival and hearing acuity. Furthermore, pharmacological supplementations with the anti-oxidant fumaric acid that targets Hcy metabolism also improved SNHL. Overall these results strongly suggest that cochlear Hcy metabolism is a key player in the onset and progression of SNHL, opening the way for the design of prospective nutritional therapies.

Cochlear Homocysteine Metabolism at the Crossroad of Nutrition and Sensorineural Hearing Loss

PubMed Central

Partearroyo, Teresa; Vallecillo, Néstor; Pajares, María A.; Varela-Moreiras, Gregorio; Varela-Nieto, Isabel

2017-01-01

Hearing loss (HL) is one of the most common causes of disability, affecting 360 million people according to the World Health Organization (WHO). HL is most frequently of sensorineural origin, being caused by the irreversible loss of hair cells and/or spiral ganglion neurons. The etiology of sensorineural HL (SNHL) is multifactorial, with genetic and environmental factors such as noise, ototoxic substances and aging playing a role. The nutritional status is central in aging disability, but the interplay between nutrition and SNHL has only recently gained attention. Dietary supplementation could therefore constitute the first step for the prevention and potential repair of hearing damage before it reaches irreversibility. In this context, different epidemiological studies have shown correlations among the nutritional condition, increased total plasma homocysteine (tHcy) and SNHL. Several human genetic rare diseases are also associated with homocysteine (Hcy) metabolism and SNHL confirming this potential link. Accordingly, rodent experimental models have provided the molecular basis to understand the observed effects. Thus, increased tHcy levels and vitamin deficiencies, such as folic acid (FA), have been linked with SNHL, whereas long-term dietary supplementation with omega-3 fatty acids improved Hcy metabolism, cell survival and hearing acuity. Furthermore, pharmacological supplementations with the anti-oxidant fumaric acid that targets Hcy metabolism also improved SNHL. Overall these results strongly suggest that cochlear Hcy metabolism is a key player in the onset and progression of SNHL, opening the way for the design of prospective nutritional therapies. PMID:28487633

Pre-enlistment hearing loss and hearing loss disability among US soldiers and marines.

PubMed

Gubata, Marlene E; Packnett, Elizabeth R; Feng, Xiaoshu; Cowan, David N; Niebuhr, David W

2013-01-01

Hearing loss is a common condition among US adults, with some evidence of increasing prevalence in young adults. Noise-induced hearing loss attributable to employment is a significant source of preventable morbidity world-wide. The US military population is largely comprised of young adult males serving in a wide variety of occupations, many in high noise-level conditions, at least episodically. To identify accession and service-related risk factors for hearing-related disability, matched case-control study of US military personnel was conducted. Individuals evaluated for hearing loss disability in the US Army and Marine Corps were frequency matched to controls without history of disability evaluation on service and enlistment year. Conditional logistic regression was used to examine the association between accession and service-related factors and hearing-related disability evaluations between October 2002 and September 2010. Individuals with medically disqualifying audiograms or hearing loss diagnoses at application for military service were 8 and 4 times more likely, respectively, to have a disability evaluation related to hearing loss, after controlling for relevant accession, demographic, and service-related factors. Conservative hearing loss thresholds on pre-enlistment audiograms, stricter hearing loss medical waiver policies or qualified baseline audiograms pre-enlistment are needed in the U.S military. Industrial corporations or labor unions may also benefit from identifying individuals with moderate hearing loss at the time of employment to ensure use of personal protective equipment and engineer controls of noise.

A Taxonomy of Fatigue Concepts and Their Relation to Hearing Loss

PubMed Central

Hornsby, Benjamin W.Y.; Naylor, Graham; Bess, Fred H.

2016-01-01

Fatigue is common in individuals with a variety of chronic health conditions and can have significant negative effects on quality of life. Although limited in scope, recent work suggests persons with hearing loss may be at increased risk for fatigue, in part due to effortful listening that is exacerbated by their hearing impairment. However, the mechanisms responsible for hearing loss-related fatigue, and the efficacy of audiologic interventions for reducing fatigue, remain unclear. To improve our understanding of hearing loss-related fatigue, as a field it is important to develop a common conceptual understanding of this construct. In this paper the broader fatigue literature is reviewed to identify and describe core constructs, consequences and methods for assessing fatigue and related constructs. Finally, our current knowledge linking hearing loss and fatigue is described and may be summarised as follows: Hearing impairment increases the risk of subjective fatigue and vigor deficits.Adults with hearing loss require more time to recover from fatigue after work, and have more work absences.Sustained, effortful, listening can be fatiguing.Optimal methods for eliciting and measuring fatigue in persons with hearing loss remain unclear and may vary with listening condition.Amplification may minimize decrements in cognitive processing speed during sustained effortful listening. Future research is needed to develop reliable measurement methods to quantify hearing loss-related fatigue; explore factors responsible for modulating fatigue in people with hearing loss; and identify and evaluate potential interventions for reducing hearing loss-related fatigue. PMID:27355763

Attainment of Developmental Tasks by Adolescents with Hearing Loss Attending Special Schools

ERIC Educational Resources Information Center

Pinquart, Martin; Pfeiffer, Jens P.

2014-01-01

The investigators compared the perceived attainment of developmental tasks by 181 German adolescents with hearing loss and 254 peers without hearing loss. The adolescents with hearing loss were attending special schools for students who are deaf or hard of hearing. On average, the two groups perceived similar levels of success across the assessed…

Influences of Working Memory and Audibility on Word Learning in Children with Hearing Loss

ERIC Educational Resources Information Center

Stiles, Derek Jason

2010-01-01

As a group, children with hearing loss demonstrate delays in language development relative to their peers with normal hearing. Early intervention has a profound impact on language outcomes in children with hearing loss. Data examining the relationship between degree of hearing loss and language outcomes are variable. Two approaches are used in the…

The projected burden of hearing loss in New Zealand (2011-2061) and the implications for the hearing health workforce.

PubMed

Exeter, Daniel J; Wu, Billy; Lee, Arier C; Searchfield, Grant D

2015-08-07

There is considerable evidence that New Zealand's population is ageing. For example, the median age increased from 29 years in 1951 to 37 years in 2011-12, and will likely increase to 44 years by 2061. While the implications of an ageing population have been studied, to date there is no study investigating the impacts that population ageing will have on hearing health in New Zealand. To explore the changing population structure and estimate the burden of hearing loss in New Zealand between 2011 and 2061. Using three alternative population projections from Statistics New Zealand, we quantify the likely distribution of the population between 2011 and 2061 by age and sex. Published estimates of hearing loss stratified by age and severity of hearing loss were then applied to the population projections to highlight the potential impact that population ageing will have on hearing loss in New Zealand in the next 50 years. We estimated that there were 330,269 people aged ≥14 years with hearing loss and this would increase to 449,453 in 2061. Overall, males have a higher prevalence of hearing loss than females, and while the prevalence of hearing loss among those aged 14-49 years is expected to decrease, the prevalence among the population aged ≥70 years is expected to double between 2011 and 2061. Age, sex and geographical variations in hearing loss are expected in the next 50 years. Further research into ethnic and variations in hearing loss will be instrumental in targeting the future hearing health workforce required to accommodate these increases.

Swept-sine noise-induced damage as a hearing loss model for preclinical assays

PubMed Central

Sanz, Lorena; Murillo-Cuesta, Silvia; Cobo, Pedro; Cediel-Algovia, Rafael; Contreras, Julio; Rivera, Teresa; Varela-Nieto, Isabel; Avendaño, Carlos

2015-01-01

Mouse models are key tools for studying cochlear alterations in noise-induced hearing loss (NIHL) and for evaluating new therapies. Stimuli used to induce deafness in mice are usually white and octave band noises that include very low frequencies, considering the large mouse auditory range. We designed different sound stimuli, enriched in frequencies up to 20 kHz (“violet” noises) to examine their impact on hearing thresholds and cochlear cytoarchitecture after short exposure. In addition, we developed a cytocochleogram to quantitatively assess the ensuing structural degeneration and its functional correlation. Finally, we used this mouse model and cochleogram procedure to evaluate the potential therapeutic effect of transforming growth factor β1 (TGF-β1) inhibitors P17 and P144 on NIHL. CBA mice were exposed to violet swept-sine noise (VS) with different frequency ranges (2–20 or 9–13 kHz) and levels (105 or 120 dB SPL) for 30 min. Mice were evaluated by auditory brainstem response (ABR) and otoacoustic emission tests prior to and 2, 14 and 28 days after noise exposure. Cochlear pathology was assessed with gross histology; hair cell number was estimated by a stereological counting method. Our results indicate that functional and morphological changes induced by VS depend on the sound level and frequency composition. Partial hearing recovery followed the exposure to 105 dB SPL, whereas permanent cochlear damage resulted from the exposure to 120 dB SPL. Exposure to 9–13 kHz noise caused an auditory threshold shift (TS) in those frequencies that correlated with hair cell loss in the corresponding areas of the cochlea that were spotted on the cytocochleogram. In summary, we present mouse models of NIHL, which depending on the sound properties of the noise, cause different degrees of cochlear damage, and could therefore be used to study molecules which are potential players in hearing loss protection and repair. PMID:25762930

Noise-induced hearing loss: a recreational noise perspective.

PubMed

Ivory, Robert; Kane, Rebecca; Diaz, Rodney C

2014-10-01

This review will discuss the real-world risk factors involved in noise-induced hearing loss as a result of common and popular recreational activities prone to mid and high levels of noise exposure. Although there are currently no interventional measures available to reverse or mitigate preexisting hearing loss from noise, we discuss the vital importance of hearing loss prevention from noise exposure avoidance and reduction. Despite a seeming understanding of the effects of noise exposure from various recreational activities and devices, a large percentage of the general public who is at risk of such noise-induced hearing loss still chooses to refrain from using hearing protection instruments. While occupational exposures pose the greatest traditional risk to hearing conservation in selected workers, recreational risk factors for noise-induced hearing loss may be more insidious in overall effect given the indifferent attitude of much of the general public and particularly our youths toward hearing protection during recreational activities. Active counseling regarding the consequences of excessive noise exposure and the potential benefits to hearing from usage of hearing protection instruments is critical to providing best possible care in the hearing health professions.

Identifying hearing loss by means of iridology.

PubMed

Stearn, Natalie; Swanepoel, De Wet

2006-11-13

Isolated reports of hearing loss presenting as markings on the iris exist, but to date the effectiveness of iridology to identify hearing loss has not been investigated. This study therefore aimed to determine the efficacy of iridological analysis in the identification of moderate to profound sensorineural hearing loss in adolescents. A controlled trial was conducted with an iridologist, blind to the actual hearing status of participants, analyzing the irises of participants with and without hearing loss. Fifty hearing impaired and fifty normal hearing subjects, between the ages of 15 and 19 years, controlled for gender, participated in the study. An experienced iridologist analyzed the randomised set of participants' irises. A 70% correct identification of hearing status was obtained by iridological analyses with a false negative rate of 41% compared to a 19% false positive rate. The respective sensitivity and specificity rates therefore came to 59% and 81%. Iridological analysis of hearing status indicated a statistically significant relationship to actual hearing status (P < 0.05). Although statistically significant sensitivity and specificity rates for identifying hearing loss by iridology were not comparable to those of traditional audiological screening procedures.

The Need for Improved Detection and Management of Adult-Onset Hearing Loss in Australia

PubMed Central

McMahon, Catherine M.; Gopinath, Bamini; Schneider, Julie; Reath, Jennifer; Hickson, Louise; Leeder, Stephen R.; Mitchell, Paul; Cowan, Robert

2013-01-01

Adult-onset hearing loss is insidious and typically diagnosed and managed several years after onset. Often, this is after the loss having led to multiple negative consequences including effects on employment, depressive symptoms, and increased risk of mortality. In contrast, the use of hearing aids is associated with reduced depression, longer life expectancy, and retention in the workplace. Despite this, several studies indicate high levels of unmet need for hearing health services in older adults and poor use of prescribed hearing aids, often leading to their abandonment. In Australia, the largest component of financial cost of hearing loss (excluding the loss of well-being) is due to lost workplace productivity. Nonetheless, the Australian public health system does not have an effective and sustainable hearing screening strategy to tackle the problem of poor detection of adult-onset hearing loss. Given the increasing prevalence and disease burden of hearing impairment in adults, two key areas are not adequately met in the Australian healthcare system: (1) early identification of persons with chronic hearing impairment; (2) appropriate and targeted referral of these patients to hearing health service providers. This paper reviews the current literature, including population-based data from the Blue Mountains Hearing Study, and suggests different models for early detection of adult-onset hearing loss. PMID:23710184

Hearing loss in Usher syndrome type II is nonprogressive.

PubMed

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

2002-12-01

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

Variable phenotypic expression and onset in MYH14 distal hereditary motor neuropathy phenotype in a large, multigenerational North American family.

PubMed

Iyadurai, Stanley; Arnold, W David; Kissel, John T; Ruhno, Corey; Mcgovern, Vicki L; Snyder, Pamela J; Prior, Thomas W; Roggenbuck, Jennifer; Burghes, Arthur H; Kolb, Stephen J

2017-08-01

Distal hereditary motor neuropathy (dHMN) causes distal-predominant weakness without prominent sensory loss. Myosin heavy chain disorders most commonly result in distal myopathy and cardiomyopathy with or without hearing loss, but a complex phenotype with dHMN, myopathy, hoarseness, and hearing loss was reported in a Korean family with a c.2822G>T mutation in MYH14. In this study we report phenotypic features in a North American family with the c.2822G>T in MYH14. Clinical and molecular characterization was performed in a large, 6-generation, Caucasian family with MYH14 dHMN. A total of 11 affected and 7 unaffected individuals were evaluated and showed varying age of onset and severity of weakness. Genotypic concordance was confirmed with molecular analysis. Electrophysiological studies demonstrated distal motor axonal degeneration without myopathy in all affected subjects tested. Mutation of MYH14 can result in a range of neuromuscular phenotypes that includes a dHMN and hearing loss phenotype with variable age of onset. Muscle Nerve 56: 341-345, 2017. © 2016 Wiley Periodicals, Inc.

Meningococcal ACWY Vaccines (MenACWY and MPSV4)

MedlinePlus

... disabilities such as hearing loss, brain damage, kidney damage, amputations, nervous system problems, or severe scars from skin grafts.Meningococcal ACWY vaccines can help prevent meningococcal disease caused by serogroups ...

Hearing aid fitting in older persons with hearing impairment: the influence of cognitive function, age, and hearing loss on hearing aid benefit.

PubMed

Meister, Hartmut; Rählmann, Sebastian; Walger, Martin; Margolf-Hackl, Sabine; Kießling, Jürgen

2015-01-01

To examine the association of cognitive function, age, and hearing loss with clinically assessed hearing aid benefit in older hearing-impaired persons. Hearing aid benefit was assessed using objective measures regarding speech recognition in quiet and noisy environments as well as a subjective measure reflecting everyday situations captured using a standardized questionnaire. A broad range of general cognitive functions such as attention, memory, and intelligence were determined using different neuropsychological tests. Linear regression analyses were conducted with the outcome of the neuropsychological tests as well as age and hearing loss as independent variables and the benefit measures as dependent variables. Thirty experienced older hearing aid users with typical age-related hearing impairment participated. Most of the benefit measures revealed that the participants obtained significant improvement with their hearing aids. Regression models showed a significant relationship between a fluid intelligence measure and objective hearing aid benefit. When individual hearing thresholds were considered as an additional independent variable, hearing loss was the only significant contributor to the benefit models. Lower cognitive capacity - as determined by the fluid intelligence measure - was significantly associated with greater hearing loss. Subjective benefit could not be predicted by any of the variables considered. The present study does not give evidence that hearing aid benefit is critically associated with cognitive function in experienced hearing aid users. However, it was found that lower fluid intelligence scores were related to higher hearing thresholds. Since greater hearing loss was associated with a greater objective benefit, these results strongly support the advice of using hearing aids regardless of age and cognitive function to counter hearing loss and the adverse effects of age-related hearing impairment. Still, individual cognitive capacity might be relevant for hearing aid benefit during an initial phase of hearing aid provision if acclimatization has not yet taken place.

Effects of Aging and Adult-Onset Hearing Loss on Cortical Auditory Regions

PubMed Central

Cardin, Velia

2016-01-01

Hearing loss is a common feature in human aging. It has been argued that dysfunctions in central processing are important contributing factors to hearing loss during older age. Aging also has well documented consequences for neural structure and function, but it is not clear how these effects interact with those that arise as a consequence of hearing loss. This paper reviews the effects of aging and adult-onset hearing loss in the structure and function of cortical auditory regions. The evidence reviewed suggests that aging and hearing loss result in atrophy of cortical auditory regions and stronger engagement of networks involved in the detection of salient events, adaptive control and re-allocation of attention. These cortical mechanisms are engaged during listening in effortful conditions in normal hearing individuals. Therefore, as a consequence of aging and hearing loss, all listening becomes effortful and cognitive load is constantly high, reducing the amount of available cognitive resources. This constant effortful listening and reduced cognitive spare capacity could be what accelerates cognitive decline in older adults with hearing loss. PMID:27242405

Unilateral and Mild Bilateral Hearing Loss in Children: Past and Current Perspectives

PubMed Central

Tharpe, Anne Marie

2008-01-01

Since the early 1980s, audiologists have become increasingly aware of the potential effect of even mild degrees of hearing loss on the psychoeducational and psychosocial outcomes of children. This review describes some of the key research findings during the past several decades that have led us to our current thinking about unilateral and mild bilateral hearing loss in children. The first section addresses unilateral hearing loss. This is followed by a review of the literature on mild bilateral hearing loss. Specifically, the issues addressed include the significance of permanent mild degrees of hearing loss on children's psychoeducational and psychosocial development and the speech, language, and auditory characteristics of children with mild degrees of hearing loss. Finally, some recommendations regarding the direction of future research are offered. This review is followed by 2 articles summarizing the proceedings of a 2005 workshop convened by the Centers for Disease Control and Prevention (CDC), Early Hearing Detection and Intervention (EHDI) program, and the Marion Downs Hearing Center to address concerns about the underidentification of—and professionals' apparent lack of awareness of—permanent unilateral and minimal to mild hearing loss in children.56,57 PMID:18270174

[The comparison of clinical features and laboratory indexes between flat descending hearing loss and total hearing loss].

PubMed

Wang, R L; Zhang, D M

2017-12-20

Objective: To discuss similarities and differences in clinical features and laboratory indexes between patients with flat descending type sudden hearing loss and those with total hearing loss. Method: The clinical data of 123 patients with full frequencies hearing loss were retrospectively analyzed. The differences in clinical features and laboratory tests(platelet, coagulation series, D-dimer, blood lipids, hemorheology) between patients with flat descending hearing loss and those with total hearing loss were analyzed by gender, age and ear side, treatment time, concomitant symptom (tinnitus, dizziness), original underlying diseases (hypertension, diabetes), etc. Result: In the clinical features,among 51 flat descending cases, the ratio of male and female was 2.401:1; among 72 total hearing loss cases, the ratio of men and women ratio was 1.058:1 ( P <0.05). Among two groups of patients,the majority received treatment within 7 days, among whom 66.7% were flat descending population, and 83.3% were total hearing loss population ( P <0.05). Flat descending population with dizziness only accounted for 35.3% while this figure was up to 70.8% when it came to total hearing loss patients ( P <0.01). Two groups showed no differences in age, ear side, tinnitus, the original underlying diseases (hypertension, diabetes). In the laboratory tests, the total hearing loss population overtopped the plat descending population in PLT and PCT ( P <0.05), while falling below the plat descending population in APTT ( P <0.01). Two groups showed no differences in other indicators of platelet and coagulation series and laboratory data of D-dimer, blood lipids, hemorheology. Conclusion: Compared with flat descending sudden hearing loss, sudden total hearing loss more frequently happened to females who also were accompanied by dizziness. The treatment rate within 7 days was high and the patients with hypercoagulable state accounted for a higher proportion. Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.

Neural tracking of attended versus ignored speech is differentially affected by hearing loss.

PubMed

Petersen, Eline Borch; Wöstmann, Malte; Obleser, Jonas; Lunner, Thomas

2017-01-01

Hearing loss manifests as a reduced ability to understand speech, particularly in multitalker situations. In these situations, younger normal-hearing listeners' brains are known to track attended speech through phase-locking of neural activity to the slow-varying envelope of the speech. This study investigates how hearing loss, compensated by hearing aids, affects the neural tracking of the speech-onset envelope in elderly participants with varying degree of hearing loss (n = 27, 62-86 yr; hearing thresholds 11-73 dB hearing level). In an active listening task, a to-be-attended audiobook (signal) was presented either in quiet or against a competing to-be-ignored audiobook (noise) presented at three individualized signal-to-noise ratios (SNRs). The neural tracking of the to-be-attended and to-be-ignored speech was quantified through the cross-correlation of the electroencephalogram (EEG) and the temporal envelope of speech. We primarily investigated the effects of hearing loss and SNR on the neural envelope tracking. First, we found that elderly hearing-impaired listeners' neural responses reliably track the envelope of to-be-attended speech more than to-be-ignored speech. Second, hearing loss relates to the neural tracking of to-be-ignored speech, resulting in a weaker differential neural tracking of to-be-attended vs. to-be-ignored speech in listeners with worse hearing. Third, neural tracking of to-be-attended speech increased with decreasing background noise. Critically, the beneficial effect of reduced noise on neural speech tracking decreased with stronger hearing loss. In sum, our results show that a common sensorineural processing deficit, i.e., hearing loss, interacts with central attention mechanisms and reduces the differential tracking of attended and ignored speech. The present study investigates the effect of hearing loss in older listeners on the neural tracking of competing speech. Interestingly, we observed that whereas internal degradation (hearing loss) relates to the neural tracking of ignored speech, external sound degradation (ratio between attended and ignored speech; signal-to-noise ratio) relates to tracking of attended speech. This provides the first evidence for hearing loss affecting the ability to neurally track speech. Copyright © 2017 the American Physiological Society.

Categorical loudness scaling and equal-loudness contours in listeners with normal hearing and hearing loss

PubMed Central

Rasetshwane, Daniel M.; Trevino, Andrea C.; Gombert, Jessa N.; Liebig-Trehearn, Lauren; Kopun, Judy G.; Jesteadt, Walt; Neely, Stephen T.; Gorga, Michael P.

2015-01-01

This study describes procedures for constructing equal-loudness contours (ELCs) in units of phons from categorical loudness scaling (CLS) data and characterizes the impact of hearing loss on these estimates of loudness. Additionally, this study developed a metric, level-dependent loudness loss, which uses CLS data to specify the deviation from normal loudness perception at various loudness levels and as function of frequency for an individual listener with hearing loss. CLS measurements were made in 87 participants with hearing loss and 61 participants with normal hearing. An assessment of the reliability of CLS measurements was conducted on a subset of the data. CLS measurements were reliable. There was a systematic increase in the slope of the low-level segment of the CLS functions with increase in the degree of hearing loss. ELCs derived from CLS measurements were similar to standardized ELCs (International Organization for Standardization, ISO 226:2003). The presence of hearing loss decreased the vertical spacing of the ELCs, reflecting loudness recruitment and reduced cochlear compression. Representing CLS data in phons may lead to wider acceptance of CLS measurements. Like the audiogram that specifies hearing loss at threshold, level-dependent loudness loss describes deficit for suprathreshold sounds. Such information may have implications for the fitting of hearing aids. PMID:25920842

Prevalence and an analysis of noise--induced hearing loss in army helicopter pilots and aircraft mechanics.

PubMed

Jaruchinda, Pariyanan; Thongdeetae, Taninsak; Panichkul, Suthee; Hanchumpol, Pongtep

2005-11-01

Hearing impairment from noise exposure has been reported in fix-wing pilots, especially in civilized countries. However, there are few studies on rotary wing aviators and aircraft mechanics, especially in developing countries whose hearing conservative program is not well established. The present study, therefore, was done to evaluate the prevalence of noise induced hearing loss and the contributing factors that may effect both groups of noise-exposed population. Report questionnaires were reviewed and physical examination combined with audiometric records of 34 pilots and 42 mechanics in the Royal Thai Army Aviation Center, Lobburi, were examined. Hearing loss was studied using four categories of significant threshold shift (STS). Amplitude of noise radiated by aircraft was also measured at different distances. No significant difference was found in prevalence of hearing loss in aviators (32.4%) and aircraft mechanics (47.6%), but in the aircraft mechanics group there were more damage of frequency involvement including speech frequency and high frequency and more decibels loss than aviators. The type of hearing protection and smoking index were strongly correlated with hearing loss. Age, flight time and alcohol habit had no significant effect and ninety percent of the subjects had no self awareness of hearing loss. Aircraft mechanics had more severity on hearing loss than aviators. Types of noise protector and cigarette smoking had significant association with hearing loss.

Pitch and Loudness from Tinnitus in Individuals with Noise-induced Hearing Loss

PubMed Central

Flores, Leticia Sousa; Teixeira, Adriane Ribeiro; Rosito, Leticia Petersen Schmidt; Seimetz, Bruna Macagnin; Dall'Igna, Celso

2015-01-01

Introduction  Tinnitus is one of the symptoms that affects individuals suffering from noise induced hearing loss. This condition can be disabling, leading the affected individual to turn away from work. Objective  This literature review aims to analyze the possible association between gender and tinnitus pitch and loudness, the degree of hearing loss and the frequencies affected in subjects with noise-induced hearing loss. Methods  This contemporary cohort study was conducted through a cross-sectional analysis. The study sample consisted of adults with unilateral or bilateral tinnitus, who had been diagnosed with noise-induced hearing loss. The patients under analysis underwent an otorhinolaryngological evaluation, pure tone audiometry, and acuphenometry. Results  The study included 33 subjects with noise-induced hearing loss diagnoses, of which 22 (66.7%) were men. Authors observed no statistical difference between gender and loudness/pitch tinnitus and loudness/pitch in subjects with bilateral tinnitus. Authors found an inverse relation between tinnitus loudness with intensity greater hearing threshold and the average of the thresholds and the grade of hearing loss. The tinnitus pitch showed no association with higher frequency of hearing threshold. Conclusion  Data analysis shows that, among the individuals evaluated, the greater the hearing loss, the lower the loudness of tinnitus. We did not observe an association between hearing loss and tinnitus pitch. PMID:27413408

Mechanisms of Noise-Induced Hearing Loss Indicate Multiple Methods of Prevention

PubMed Central

Le Prell, Colleen G.; Yamashita, Daisuke; Minami, Shujiro B.; Yamasoba, Tatsuya; Miller, Josef M.

2007-01-01

Recent research has shown the essential role of reduced blood flow and free radical formation in the cochlea in noise-induced hearing loss (NIHL). The amount, distribution, and time course of free radical formation have been defined, including a clinically significant late formation 7–10 days following noise exposure, and one mechanism underlying noise-induced reduction in cochlear blood flow has finally been identified. These new insights have led to the formulation of new hypotheses regarding the molecular mechanisms of NIHL; and, from these, we have identified interventions that prevent NIHL, even with treatment onset delayed up to 3 days post-noise. It is essential to now assess the additive effects of agents intervening at different points in the cell death pathway to optimize treatment efficacy. Finding safe and effective interventions that attenuate NIHL will provide a compelling scientific rationale to justify human trials to eliminate this single major cause of acquired hearing loss. PMID:17141991

Self-Esteem in Children and Adolescents With Hearing Loss

PubMed Central

Loy, Betty A.; Evans, Christine; Wetsel, Ashton; Tobey, Emily A.

2015-01-01

Children with hearing loss are at risk for lower self-esteem due to differences from hearing peers relative to communication skills, physical appearance, and social maturity. This study examines the influence of generic factors unrelated to hearing loss (e.g., age, gender, temperament) and specific factors associated with hearing loss (e.g., age at identification, communication skills) on how children with hearing loss wearing cochlear implants or hearing aids appraise self-esteem. Fifty children with hearing loss wearing cochlear implants or hearing aids participated (Mean age: 12.88 years; mean duration of device use: 3.43 years). Participants independently completed online questionnaires to assess communication skills, social engagement, self-esteem, and temperament. Children with hearing loss rated global self-esteem significantly more positively than hearing peers, t = 2.38, p = .02. Self-esteem ratings attained significant positive correlations with affiliation (r = .42, p = .002) and attention (r = .45, p = .001) temperaments and a significant negative association with depressive mood (r = − .60, p < .0001). No significant correlations emerged between self-esteem and demographic factors, communication skills, or social engagement. Because successful communication abilities do not always co-occur with excellent quality of life, clinicians and professionals working with children with hearing loss need to understand components contributing to self-esteem to improve identification, counseling, and external referrals for children in this population. PMID:25755025

Self-esteem in children and adolescents with hearing loss.

PubMed

Warner-Czyz, Andrea D; Loy, Betty A; Evans, Christine; Wetsel, Ashton; Tobey, Emily A

2015-03-09

Children with hearing loss are at risk for lower self-esteem due to differences from hearing peers relative to communication skills, physical appearance, and social maturity. This study examines the influence of generic factors unrelated to hearing loss (e.g., age, gender, temperament) and specific factors associated with hearing loss (e.g., age at identification, communication skills) on how children with hearing loss wearing cochlear implants or hearing aids appraise self-esteem. Fifty children with hearing loss wearing cochlear implants or hearing aids participated (Mean age: 12.88 years; mean duration of device use: 3.43 years). Participants independently completed online questionnaires to assess communication skills, social engagement, self-esteem, and temperament. Children with hearing loss rated global self-esteem significantly more positively than hearing peers, t = 2.38, p = .02. Self-esteem ratings attained significant positive correlations with affiliation (r = .42, p = .002) and attention (r = .45, p = .001) temperaments and a significant negative association with depressive mood (r = - .60, p < .0001). No significant correlations emerged between self-esteem and demographic factors, communication skills, or social engagement. Because successful communication abilities do not always co-occur with excellent quality of life, clinicians and professionals working with children with hearing loss need to understand components contributing to self-esteem to improve identification, counseling, and external referrals for children in this population. © The Author(s) 2015.

Are persons with fibromyalgia or other musculoskeletal pain more likely to report hearing loss? A HUNT study.

PubMed

Stranden, Magne; Solvin, Håvard; Fors, Egil A; Getz, Linn; Helvik, Anne-S

2016-11-16

Leading theories about the pathogenesis of fibromyalgia focus on central nervous dysregulation or sensitization, which can cause altered perception. There is growing evidence that fibromyalgia involves altered perception not only of pain, but also other sensory stimuli. On this basis, we investigated whether individuals with fibromyalgia are more likely to report subjective loss of hearing, adjusted for audiometrically measured loss of hearing, compared to persons without any musculoskeletal pain disorders. In addition, we studied persons with other musculoskeletal pain than fibromyalgia and persons who did not have any musculoskeletal pain. The study includes 44 494 persons from the second health survey in Nord-Trøndelag (HUNT2) who had undergone audiometry and answered a comprehensive questionnaire that mapped fibromyalgia, musculoskeletal pain at various sites and subjective hearing loss. Respondents with other musculoskeletal pain problems than fibromyalgia were divided into two groups with respectively localized and widespread musculoskeletal pain. Data were analyzed with logistic regression models adjusting for age, education, anxiety, depression and hearing thresholds. In adjusted analysis, individuals with fibromyalgia had increased likelihood to report subjective hearing loss, compared to persons without fibromyalgia or other musculoskeletal pain (OR 4.578, 95% CI 3.622-5.787 and OR 4.523, 95% CI 3.077-6.647 in women and men). Furthermore, people with local and widespread musculoskeletal pain not diagnosed with fibromyalgia, also had increased likelihood to report subjective hearing loss, compared to people with no musculoskeletal pain. This relationship was greater for widespread pain than for localized pain (OR 1.915, 95% CI 1.627-2.255, and 1.796, 95% CI 1.590-2.029, in women and men with local musculoskeletal pain and OR 3.073, 95% CI 2.668-3.539, OR 3.618, 95% CI 3.225-4.058, in women and men with widespread pain, respectively). Our findings are consistent with the hypothesis that fibromyalgia is related to a general dysregulation of the central nervous system. The same might also be the case for other local and, in particular, other widespread, musculoskeletal pain.

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

PubMed

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

2014-01-01

Noise induced hearing loss (NIHL) is an irreversible sensorineural hearing loss associated with exposure to high levels of excessive noise. Prevention measures are not well established in developing countries. This comparative cross sectional study aims to determine the prevalence of hearing loss in both a group of high risk workers and a control group and to assess their knowledge of the effects of noise on hearing health. A total of 101 market mill workers and 103 controls employed within markets in the city of Accra, Ghana, were evaluated using a structured questionnaire and pure tone audiometry. The questionnaire assessed factors including self-reported hearing loss, tinnitus, knowledge on the effects of noise on hearing health and the use of hearing protective devices. Pure tone audiometric testing was conducted for both mill workers and controls. Noise levels at the work premises of the mill workers and controls were measured. Symptoms of hearing loss were reported by 24 (23.76%) and 8 (7.7%) mill workers and controls respectively. Fifty-five (54.5%) and fifty-four (52.37%) mill workers and controls exhibited knowledge of the effects of noise on hearing health. Five (5.0%) mill workers used hearing protective devices. There was significant sensorineural hearing loss and the presence of a 4 kHz audiometric notch among mill workers when compared with controls for the mean thresholds of 2 kHz, 3 kHz and 4 kHz (P = 0. 001). The prevalence of hearing loss in the better hearing ears of the mill workers and controls was 24.8% and 4.8% respectively (P < 0.5). The prevalence of hearing loss, which may be characteristic of NIHL in the better hearing ears of the mill workers and controls was 24.8% and 4.8% respectively. The majority of mill workers did not use hearing protection.

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

PubMed

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

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

Postnatal risk factors associated with hearing loss among high-risk preterm infants: tertiary center results from Turkey.

PubMed

Eras, Zeynep; Konukseven, Ozlem; Aksoy, Hatice Tatar; Canpolat, Fuat Emre; Genç, Aydan; Sakrucu, Evrim Durgut; Develioğlu, Omer; Dilmen, Ugur

2014-06-01

The aim of this study was to determine the postnatal risk factors associated with hearing loss as well as the prevalence of hearing loss among high-risk preterm infants in newborn hearing screening (NHS). We performed a retrospective study of high-risk preterm infants born with a gestational age ≤32 weeks and/or a birth weight ≤1,500 g. A NHS procedure was performed by automated auditory brainstem response (AABR) and automated evoked otoacoustic emission (TEOAE). Infants who failed TEOAE or AABR or both tests were referred to a tertiary audiology center for diagnosis confirmation and management. Postnatal risk factors associated with hearing loss were evaluated and compared for preterm infants with and without hearing loss. 1,360 high-risk preterm infants were assessed. Permanent hearing loss was found in 19 (1.4%) infants. Multivariate analysis revealed that proven sepsis (p = 0.019), mechanical ventilation ≥5 days (p = 0.024), loop diuretics (p = 0.001), patent ductus arteriosus ligation (p = 0.018) and operation for retinopathy of prematurity (ROP) (p = 0.034) were significant related factors for the hearing loss. This study showed a low prevalence of hearing loss and an association between operation for ROP and hearing loss in preterm infants, which has not been defined previously. Our results suggest that every neonatal intensive care unit should determine their own risk factors and take precautions to prevent hearing loss for these high-risk preterm infants.

Prevalence of Hearing Loss in Teachers of Singing and Voice Students.

PubMed

Isaac, Mitchell J; McBroom, Deanna H; Nguyen, Shaun A; Halstead, Lucinda A

2017-05-01

Singers and voice teachers are exposed to a range of noise levels during a normal working day. This study aimed to assess the hearing thresholds in a large sample of generally healthy professional voice teachers and voice students to determine the prevalence of hearing loss in this population. A cross-sectional study was carried out. Voice teachers and vocal students had the option to volunteer for a hearing screening of six standard frequencies in a quiet room with the Shoebox audiometer (Clearwater Clinical Limited) and to fill out a brief survey. Data were analyzed for the prevalence and severity of hearing loss in teachers and students based on several parameters assessed in the surveys. All data were analyzed using Microsoft Excel (Microsoft Corp.) and SPSS Statistics Software (IBM Corp.). A total of 158 participants were included: 58 self-identified as voice teachers, 106 as voice students, and 6 as both. The 6 participants who identified as both, were included in both categories for statistical purposes. Of the 158 participants, 36 had some level of hearing loss: 51.7% of voice teachers had hearing loss, and 7.5% of voice students had hearing loss. Several parameters of noise exposure were found to positively correlate with hearing loss and tinnitus (P < 0.05). Years as a voice teacher and age were both predictors of hearing loss (P < 0.05). Hearing loss in a cohort of voice teachers appears to be more prevalent and severe than previously thought. There is a significant association between years teaching and hearing loss. Raising awareness in this population may prompt teachers and students to adopt strategies to protect their hearing. Copyright © 2017 The Voice Foundation. Published by Elsevier Inc. All rights reserved.

Centre-level variation in outcomes and treatment for otitis media with effusion and hearing loss and the association of hearing loss with developmental outcomes at ages 5 and 7 years in children with non-syndromic unilateral cleft lip and palate: The Cleft Care UK study. Part 2.

PubMed

Hall, A; Wills, A K; Mahmoud, O; Sell, D; Waylen, A; Grewal, S; Sandy, J R; Ness, A R

2017-06-01

To explore centre-level variation in otitis media with effusion (OME), hearing loss and treatments in children in Cleft Care UK (CCUK) and to examine the association between OME, hearing loss and developmental outcomes at 5 and 7 years. Two hundred and sixty-eight 5-year-old British children with non-syndromic unilateral cleft lip and palate (UCLP) recruited to CCUK. Children had air and bone conduction audiometry at age 5. Information on grommet and hearing aid treatment was obtained from parental questionnaire and medical notes. Hearing loss at age 5 was defined as >20 dB in the better ear and history of OME and hearing loss was determined from past treatment. Children with sensorineural hearing loss were excluded. Associations were examined with speech, behaviour and self-confidence at age 5 and educational attainment at age 7. Centre variation was examined using hierarchical models and associations between hearing variables and developmental outcomes were examined using logistic regression. There was centre-level variation in early grommet placement (variance partition coefficient (VPC) 18%, P=.001) and fitting of hearing aids (VPC 8%, P=.03). A history of OME and hearing loss was associated with poor intelligibility of speech (adjusted odds ratio=2.87, 95% CI 1.42-5.77) and aspects of educational attainment. Hearing loss is an important determinant of poor speech and treatment variation across centres suggest management of OME and hearing loss could be improved. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

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

PubMed

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

2013-10-01

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

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

PubMed Central

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

2013-01-01

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

Causes and Consequences of Sensory Hair Cell Damage and Recovery in Fishes.

PubMed

Smith, Michael E; Monroe, J David

2016-01-01

Sensory hair cells are the mechanotransductive receptors that detect gravity, sound, and vibration in all vertebrates. Damage to these sensitive receptors often results in deficits in vestibular function and hearing. There are currently two main reasons for studying the process of hair cell loss in fishes. First, fishes, like other non-mammalian vertebrates, have the ability to regenerate hair cells that have been damaged or lost via exposure to ototoxic chemicals or acoustic overstimulation. Thus, they are used as a biomedical model to understand the process of hair cell death and regeneration and find therapeutics that treat or prevent human hearing loss. Secondly, scientists and governmental natural resource managers are concerned about the potential effects of intense anthropogenic sounds on aquatic organisms, including fishes. Dr. Arthur N. Popper and his students, postdocs and research associates have performed pioneering experiments in both of these lines of fish hearing research. This review will discuss the current knowledge regarding the causes and consequences of both lateral line and inner ear hair cell damage in teleost fishes.

[Investigation of occupational noise exposure and hearing loss in large automobile manufacturing enterprise during 2006-2010 in Guangzhou, China].

PubMed

Zhao, Yuan; Zhou, Hao; Li, Yanhua; Xiao, Lvwu; Wu, Lin; Du, Weijia; Liu, Yimin

2014-02-01

To analyze the relationship between occupational noise exposure and hearing loss among workers in large automobile manufacturing enterprise during 2006-2010 in Guangzhou, China. A retrospective cohort study was conducted. The subjects were divided into noise exposure group and control group. Their hearing examination results and noise exposure levels in different workplaces were collected during 2006-2010, and the relationship between noise exposure in workplaces and hearing loss was analyzed. The incidence of hearing loss for the noise exposure group was 9.34%, versus 2.75% for the control group; the noise exposure group had a significantly higher risk of hearing loss than the control group (R = 3.378, 95%CI = 1.467∼ 9.083). The noise intensity and over-limit rate were significantly higher in the stamping, welding, and general assembly workshops than in other workshops. The risk of hearing loss significantly increased with years of noise exposure in 80, 85, and 90 dB (A) groups (χ(2) = 6.377, P = 0.041; χ(2) = 8.570, P = 0.014; χ(2) = 7.037, P = 0.030). The risk of hearing loss also increased with noise intensity in all working age groups (χ(2) = 5.068, P = 0.024; χ(2) = 71.497, P < 0.01; χ(2) = 24.226, P < 0.01). Noise exposure increases the risk of noise-induced hearing loss in workers. The incidence of hearing loss increases with the noise intensity in workplaces and years of noise exposure. The noise exposure level and incidence of hearing loss are higher in the stamping workshop than in other workshops. Controlling the noise intensity in automobile manufacturing enterprise may reduce the risk of hearing loss in workers.

Evaluation of family history of permanent hearing loss in childhood as a risk indicator in universal screening.

PubMed

Valido Quintana, Mercedes; Oviedo Santos, Ángeles; Borkoski Barreiro, Silvia; Santana Rodríguez, Alfredo; Ramos Macías, Ángel

Sixty percent of prelingual hearing loss is of genetic origin. A family history of permanent childhood hearing loss is a risk factor. The objective of the study is to determine the relationship between this risk factor and hearing loss. We have evaluated clinical and epidemiological characteristics and related nonsyndromic genetic variation. This was a retrospective, descriptive and observational study of newborns between January 2007 and December 2010 with family history as risk factor for hearing loss using transient evoked otoacoustic emissions and auditory brainstem response. A total of 26,717 children were born. Eight hundred and fifty-seven (3.2%) had family history. Fifty-seven(0.21%) failed to pass the second test. A percentage of 29.1 (n=16) had another risk factor, and 17.8% (n=9) had no classical risk factor. No risk factor was related to the hearing loss except heart disease. Seventy-six point four percent had normal hearing and 23.6% hearing loss. The mean of family members with hearing loss was 1.25. On genetic testing, 82.86% of homozygotes was normal, 11.43% heterozygosity in Connexin 26 gene (35delG), 2.86% R143W heterozygosity in the same gene and 2.86% mutant homozygotes (35delG). We found no relationship between hearing loss and mutated allele. The percentage of children with a family history and hearing loss is higher than expected in the general population. The genetic profile requires updating to clarify the relationship between hearing loss and heart disease, family history and the low prevalence in the mutations analyzed. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

[Phenotypic and genetic analysis of a patient presented with Tietz/Waardenburg type II a syndrome].

PubMed

Wang, Huanhuan; Tang, Lifang; Zhang, Jingmin; Hu, Qin; Chen, Yingwei; Xiao, Bing

2015-08-01

To determine the genetic cause for a patient featuring decreased pigmentation of the skin and iris, hearing loss and multiple congenital anomalies. Routine chromosomal banding was performed to analyze the karyotype of the patient and his parents. Single nucleotide polymorphism array (SNP array) was employed to identify cryptic chromosome aberrations, and quantitative real-time PCR was used to confirm the results. Karyotype analysis has revealed no obvious anomaly for the patient and his parents. SNP array analysis of the patient has demonstrated a 3.9 Mb deletion encompassing 3p13p14.1, which caused loss of entire MITF gene. The deletion was confirmed by quantitative real-time PCR. Clinical features of the patient have included severe bilateral hearing loss, decreased pigmentation of the skin and iris and multiple congenital anomalies. The patient, carrying a 3p13p14.1 deletion, has features of Tietz syndrome/Waardenburg syndrome type IIa. This case may provide additional data for the study of genotype-phenotype correlation of this disease.

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

PubMed

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

2005-01-01

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

Hearing loss from cockpit noise in motor gliders.

PubMed

Stueben, U

2001-09-01

Over the past 15 yr, remarkable progress has been made in the development of touring motor gliders and self-launching sail-planes with retractable propulsion units (RPU gliders.) Annually, over 50% of the gliders produced have had such units, often two-stroke engines which produce high frequency sound emissions. Sound emission regulations require that all power gliders be insulated to avoid external emissions and noise pollution in the airspace near airfields. However, these regulations do not cover noise within the cockpit. To determine the noise level in the cockpits of powered gliders and how this noise affects the hearing of pilots, cockpit noise in six touring motor gliders and nine RPU gliders were measured. Both types of motor gliders have noise levels that may be hazardous to pilots' hearing if they are not equipped with personal noise protection. In extreme cases an exposure time of only 20 seconds to the cockpit noise of an RPU glider may cause permanent hearing loss. Pilots must be warned not to fly motor gliders without personal noise protection.

Prevalence of noise-induced hearing loss among woodworkers in Nepal: a pilot study

PubMed Central

Robinson, Tim; Whittaker, Joshua; Acharya, Aanand; Singh, Devesh; Smith, Michael

2015-01-01

Background: The woodworking industry represents an important cause of occupational noise-induced hearing loss (NIHL), a significant yet underappreciated problem in many developing countries. Objectives: To describe the prevalence of occupational NIHL among woodworkers in Nepal and measure noise levels at workplaces. Methods: We conducted a cross-sectional study with 124 woodworkers (88 carpenters, 36 sawyers), recruited through convenience sampling from 26 workplaces. Pure-tone audiometry between the frequencies 0·5 and 8 kHz ascertained participants’ hearing status, and noise readings were taken at selected workplaces. Results: In all, 31% of carpenters and 44% of sawyers met criteria for NIHL, with 7 and 17% meeting World Health Organization (WHO) criteria for hearing impairment (HI), respectively. Noise levels at various workplaces ranged from 71·2 to 93·9 dBA. Conclusions: Woodworkers in Nepal are at risk of occupational NIHL. As the industry develops, this problem will likely become more extensive, highlighting the need for workplace interventions and additional research. PMID:25335826

Age-related hearing loss: quality of care for quality of life.

PubMed

Li-Korotky, Ha-Sheng

2012-04-01

Age-related hearing loss (ARHL), known as presbycusis, is characterized by progressive deterioration of auditory sensitivity, loss of the auditory sensory cells, and central processing functions associated with the aging process. ARHL is the third most prevalent chronic condition in older Americans, after hypertension and arthritis, and is a leading cause of adult hearing handicaps in the United States. The prevalence of ARHL is expected to rise for the next several decades with the increasing aging Baby Boomer population. Nevertheless, ARHL remains an often undetected, underestimated and neglected condition in the geriatric population due to a slow development process of the disease. If left untreated, the impact of ARHL on patients, significant others, and the society as a whole would be significant. The purpose of this review is to raise the awareness of ARHL, to update our current understanding of ARHL with a focus on age-related deficits in auditory and cognitive processing of speech, and to explore strategies of prevention, identification, amplification, and aural rehabilitation. The ultimate goal is to improve the quality of hearing health care and the overall quality of life of the Baby Boomer generation.

Immune-Mediated Inner Ear Disease: Diagnostic and therapeutic approaches.

PubMed

Penêda, José Ferreira; Lima, Nuno Barros; Monteiro, Francisco; Silva, Joana Vilela; Gama, Rita; Condé, Artur

2018-03-07

Immune Mediated Inner Ear Disease (IMIED) is a rare form of sensorineural bilateral hearing loss, usually progressing in weeks to months and responsive to immunosuppressive treatment. Despite recent advances, there is no consensus on diagnosis and optimal treatment. A review of articles on IMIED from the last 10 years was conducted using PubMed ® database. IMIED is a rare disease, mostly affecting middle aged women. It may be a primary ear disease or secondary to autoimmune systemic disease. A dual immune response (both cellular and humoral) seems to be involved. Cochlin may be the inner ear protein targeted in this disease. Distinction from other (core common) forms of neurosensory hearing loss is a challenge. Physical examination is mandatory for exclusion of other causes of hearing loss; audiometry identifies characteristic hearing curves. Laboratory and imaging studies are controversial since no diagnostic marker is available. Despite recent research, IMIED diagnosis remains exclusive. Steroids are the mainstay treatment; other therapies need further investigation. For refractory cases, cochlear implantation is an option and with good relative outcome. Copyright © 2018 Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. Publicado por Elsevier España, S.L.U. All rights reserved.

Minocycline Protection of Neomycin Induced Hearing Loss in Gerbils

PubMed Central

Robinson, Alan M.; Vujanovic, Irena; Richter, Claus-Peter

2015-01-01

This animal study was designed to determine if minocycline ameliorates cochlear damage is caused by intratympanic injection of the ototoxic aminoglycoside antibiotic neomycin. Baseline auditory-evoked brainstem responses were measured in gerbils that received 40 mM intratympanic neomycin either with 0, 1.2, or 1.5 mg/kg intraperitoneal minocycline. Four weeks later auditory-evoked brainstem responses were measured and compared to the baseline measurements. Minocycline treatments of 1.2 mg/kg and 1.5 mg/kg resulted in significantly lower threshold increases compared to 0 mg/kg, indicating protection of hearing loss between 6 kHz and 19 kHz. Cochleae were processed for histology and sectioned to allow quantification of the spiral ganglion neurons and histological evaluation of organ of Corti. Significant reduction of spiral ganglion neuron density was demonstrated in animals that did not receive minocycline, indicating that those receiving minocycline demonstrated enhanced survival of spiral ganglion neurons, enhanced survival of sensory hairs cells and spiral ganglion neurons, and reduced hearing threshold elevation correlates with minocycline treatment demonstrating that neomycin induced hearing loss can be reduced by the simultaneous application of minocycline. PMID:25950003

[The characteristics of auditory brainstem response in preterm very low birth weight babies].

PubMed

Wang, Xiaoya; Luo, Renzhong; Wen, Ruijin; Chen, Qian; Zhou, Jialin; Zou, Yu

2009-08-01

To discuss the characteristics of auditory brainstem response in preterm very low birth weight (VLBW) babies and to investigate the correlations between the ABR and clinical characteristics. Fifty-nine VLBW babies (118 ears) were enrolled in the study and 30 term normal babies as the control group. Tympanometry, acoustic reflex, DPOAE, ABR were obtained in all the babies. The prevalence of hearing loss in VLBW babies was higher than normal term babies and babies with perinatal complications higher than those without perinatal complications. There was no correlations between ABR threshold and gestational age, birth weight, postconceptional age, negative correlations between wave I, III and V latencies I - III, III - V and I - V intervals and postconceptional age. Wave I and V latencies, I - III and III - V intervals differed significantly between the two groups. The perinatal complications were the most important causes of the hearing loss in preterm VLBW babies than the gestational age and birth weight. There was a high prevalence of peripheral hearing loss in the preterm VLBW babies. Combining OAE and automated ABR should be applied for hearing screening. Regular follow-up was very important in all the preterm VLBW neonatal.

Results of a Targeted Screening Program for Congenital Cytomegalovirus Infection in Infants Who Fail Newborn Hearing Screening.

PubMed

Vancor, Emily; Shapiro, Eugene D; Loyal, Jaspreet

2018-01-24

Congenital cytomegalovirus (CMV) infection is a major cause of sensorineural hearing loss. By law, newborns in Connecticut who fail newborn hearing screening are tested for infection with CMV. This targeted screening is controversial, because most children with congenital CMV infection are asymptomatic, and CMV-related hearing loss can have a delayed onset. Our hospital uses a saliva polymerase chain reaction (PCR) assay (confirmed by a urine PCR assay) to detect CMV. Here, we report the results of the first year of our screening program. We reviewed the medical records of newborns in the Yale New Haven Health System who failed the newborn hearing screening test between January 1 and December 31, 2016. Of 10964 newborns, 171 failed newborn hearing screening, and 3 of these newborns had positive saliva CMV PCR test results. Of these 3 newborns, 2 had positive results on the confirmatory test (for 1 of them the confirmatory test was not performed until the infant was 10 weeks old), and 1 had a negative result on the confirmatory test. Three additional newborns with congenital CMV infection were tested because of clinical indications (1 for ventriculomegaly on prenatal ultrasound and 2 for CMV infection of the mother). Results of audiology follow-up were available for 149 (87.1%) of the 171 newborns who failed newborn hearing screening; 127 (85.2%) had normal results. Our targeted screening program for congenital CMV infection had a low yield. Consideration should be given to other strategies for identifying children at risk of hearing loss as a result of congenital CMV infection. © The Author(s) 2018. Published by Oxford University Press on behalf of The Journal of the Pediatric Infectious Diseases Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Hearing loss may be a non-motor feature of Parkinson's disease in older people in Taiwan.

PubMed

Lai, S-W; Liao, K-F; Lin, C-L; Lin, C-C; Sung, F-C

2014-05-01

The aim of this study was to explore whether hearing loss is associated with the risk of Parkinson's disease in the elderly in Taiwan. Using claims data of the Taiwan National Health Insurance Program, 4976 patients (aged 65 years or older) with newly diagnosed hearing loss from 2000 to 2010 were identified and 19 904 subjects without hearing loss were randomly selected as comparisons, frequency matched by sex, age and index year of diagnosing hearing loss. The incidence of Parkinson's disease by the end of 2010 and the associated risk factors were investigated. The incidence of Parkinson's disease in the hearing loss group was 1.77-fold higher than that in the non-hearing-loss group (3.11 vs. 1.76 per 1000 person-years). After controlling for confounding factors, the adjusted hazard ratio (HR) of Parkinson's disease was 1.53 (95% CI 1.17, 1.99) for the hearing loss group compared with the non-hearing-loss group. Male sex (HR = 1.33, 95% CI 1.02, 1.74), age (for each year, HR = 1.06, 95% CI 1.04, 1.09), hypertension (HR = 1.70, 95% CI 1.26, 2.30) and cerebrovascular disease (HR = 1.78, 95% CI 1.37, 2.32) were also significantly associated with the risk of Parkinson's disease. Hearing loss correlates with an increased risk of Parkinson's disease in the elderly. Further studies are needed to confirm whether hearing loss could be a non-motor feature of Parkinson's disease. © 2014 The Author(s) European Journal of Neurology © 2014 EFNS.

Triple Difficulties in Japanese Women with Hearing Loss: Marriage, Smoking, and Mental Health Issues

PubMed Central

Kobayashi, Yoko; Tamiya, Nanako; Moriyama, Yoko; Nishi, Akihiro

2015-01-01

Objective To examine the consequences of early-onset hearing loss on several social and health measures and any related gender differences in Japanese populations. Methods Data from a 2007 nationally representative cross-sectional household survey of 136,849 men and women aged 20 to 39 years were obtained (prevalence of self-reported hearing loss: 0.74%). We focused particularly on four social and health measures: employment status (employed/unemployed), marital status (married/unmarried), smoking behavior (yes/no), and psychological distress (K6 instrument: ≥ 5 or not). We examined the association of hearing loss for each measure using generalized estimating equations to account for correlated individuals within households. Findings There was no significant association with employment status (p = 0.447). Men with hearing loss were more likely to be married, whereas women with hearing loss were less likely to be married (p < 0.001 for interaction). Although hearing loss was not associated with a current smoking status in men, women with hearing loss were more likely to be current smokers (p < 0.001 for interaction). Moreover, hearing loss was associated with psychological distress in men and women (both p < 0.001). Conclusion These findings suggest that hearing loss is related to social and health issues in daily life, including a lower likelihood of marriage, more frequent smoking, and poorer mental health, especially in women. These issues may reflect a gap between the actual needs of women with hearing loss and the formal support received as a result of existing public health policies in Japan. PMID:25651532

Triple difficulties in Japanese women with hearing loss: marriage, smoking, and mental health issues.

PubMed

Kobayashi, Yoko; Tamiya, Nanako; Moriyama, Yoko; Nishi, Akihiro

2015-01-01

To examine the consequences of early-onset hearing loss on several social and health measures and any related gender differences in Japanese populations. Data from a 2007 nationally representative cross-sectional household survey of 136,849 men and women aged 20 to 39 years were obtained (prevalence of self-reported hearing loss: 0.74%). We focused particularly on four social and health measures: employment status (employed/unemployed), marital status (married/unmarried), smoking behavior (yes/no), and psychological distress (K6 instrument: ≥ 5 or not). We examined the association of hearing loss for each measure using generalized estimating equations to account for correlated individuals within households. There was no significant association with employment status (p = 0.447). Men with hearing loss were more likely to be married, whereas women with hearing loss were less likely to be married (p < 0.001 for interaction). Although hearing loss was not associated with a current smoking status in men, women with hearing loss were more likely to be current smokers (p < 0.001 for interaction). Moreover, hearing loss was associated with psychological distress in men and women (both p < 0.001). These findings suggest that hearing loss is related to social and health issues in daily life, including a lower likelihood of marriage, more frequent smoking, and poorer mental health, especially in women. These issues may reflect a gap between the actual needs of women with hearing loss and the formal support received as a result of existing public health policies in Japan.

“Targeted” Screening for Cytomegalovirus (CMV)-Related Hearing Loss: It’s Time for Universal CMV Screening in the NICU!

PubMed Central

Medoro, Alexandra; Malhotra, Prashant; Shimamura, Masako; Hounam, Gina; Findlen, Ursula; Wozniak, Phillip; Foor, Nicholas; Adunka, Oliver; Sanchez, Pablo J

2017-01-01

Abstract Background Congenital CMV infection is the leading cause of non-genetic sensorineural hearing loss in infancy. Antiviral therapy has been shown to improve hearing outcomes, and thus “targeted” CMV screening for newborns who do not pass the hearing screen has been recommended. Diagnosis of congenital CMV infection requires that the infant be tested for CMV in the first 3 weeks of age. Our objective was to determine when infants in the neonatal intensive care unit (NICU) have their first hearing screen performed and thus inform the practice of targeted screening for determination of CMV-related hearing loss. Methods Retrospective review of the electronic health records of all infants admitted to the Level 4 outborn NICU at Nationwide Children’s Hospital, Columbus, OH from August 2016 to May 2017. Demographic and clinical data were obtained, and the age that the first hearing screen was performed was assessed. Results During the 10 month study period, 362 infants were admitted to the NICU and had a first hearing screen performed. The majority of neonates (204, 56%) had a first hearing screen performed in the first 3 weeks of age. However, 158 (44%; median birth weight [IQR], 1072 g [747–1766]; median gestational age [IQR], 28 weeks [25–32]) infants received the first hearing screen at >3 weeks of age when a positive CMV PCR or culture cannot distinguish congenital infection from intrapartum/postnatal acquisition of CMV. Of the 158 infants, 20 (13%) did not pass the first hearing screen (13, unilateral; 7, bilateral), and subsequently, 9 of them did pass a second hearing screen. However, 11 of the 20 infants did not pass a second hearing screen and had urine CMV PCR testing, and 1 (9%) was positive. This latter infant’s newborn dried blood spot CMV DNA PCR was negative so a diagnosis of congenital CMV infection was not possible. Conclusion Targeted screening in the NICU for CMV-related hearing loss is problematic as a substantial number of infants do not have a hearing screen performed until after 21 days of age, thus representing a missed opportunity for diagnosis of congenital CMV infection and institution of antiviral therapy if indicated. Our findings support universal CMV screening of neonates on admission to the NICU. Disclosures O. Adunka, MED-EL Corporation: Consultant, Consulting fee, Educational grant and Research support; Advanced Bionics: Consultant, Consulting fee and Licensing agreement or royalty; Advanced Cochlear Diagnostics: President, Ownership interest; AGTC Corporation: Consultant, Consulting fee

Print Knowledge of Preschool Children with Hearing Loss

ERIC Educational Resources Information Center

Werfel, Krystal L.; Lund, Emily; Schuele, C. Melanie

2015-01-01

Measures of print knowledge were compared across preschoolers with hearing loss and normal hearing. Alphabet knowledge did not differ between groups, but preschoolers with hearing loss performed lower on measures of print concepts and concepts of written words than preschoolers with normal hearing. Further study is needed in this area.

Acoustic Trauma - Hearing Loss in Teenagers

MedlinePlus

... caused by exposure to loud noises, such as music played through headphones. The authors of another study ... going on around them while listening to the music, according to the AAP. See Music: How Loud ...

Speech and Language Delay

MedlinePlus

... the child just doesn’t want to talk). Cerebral palsy (a movement disorder caused by brain damage). Why ... staff Categories: Family Health, Kids and TeensTags: autism, cerebral palsy, child, developmental delay, hearing loss, teenager June 1, ...

Assessment of hearing and hearing disorders in rock/jazz musicians.

PubMed

Kähärit, Kim; Zachau, Gunilla; Eklöf, Mats; Sandsjö, Leif; Möller, Claes

2003-07-01

The aim of this study was to assess hearing and hearing disorders among rock/jazz musicians. One hundred and thirty-nine (43 women and 96 men) musicians participated. The results are based on pure-tone audiometry and questionnaire responses. According to our definition of hearing loss, tinnitus, hyperacusis, distortion and/or diplacusis as hearing disorders, we found disorders in 74%, of the rock/jazz musicians studied. Hearing loss, tinnitus and hyperacusis were most common, and the latter two were found significantly more frequently than in different reference populations. The women showed bilateral, significantly better hearing thresholds at 3-6 kHz than the men. Hyperacusis, and the combination of both hyperacusis and tinnitus, were found to be significantly more frequent among women than among men. Hearing loss and tinnitus were significantly more common among men than among women. It is important to evaluate all kinds of hearing problems (other than hearing loss) in musicians, since they represent an occupational group especially dependent on optimal, functional hearing. On the basis of our results, we suggest that hearing problems such as tinnitus, hyperacusis, distortion and/or diplacusis should, in addition to hearing loss, be defined as hearing disorders.

Factors Influencing Hearing Aid Use in the Classroom: A Pilot Study.

PubMed

Gustafson, Samantha J; Davis, Hilary; Hornsby, Benjamin W Y; Bess, Fred H

2015-12-01

This pilot study examined factors influencing classroom hearing aid use in school-age children with hearing loss. The research team visited classrooms of 38 children with mild-to-moderate hearing loss (Grades 1-7) on 2 typical school days, twice per day, to document hearing aid use. In addition, parents reported the number of hours their children used hearing aids at school. Nearly 24% of children were observed not wearing their hearing aids in the classroom on either observation day. Both grade level and degree of hearing loss appeared to affect hearing aid use. Children in Grades 5-7 and those with milder hearing losses were less likely to wear hearing aids. Overall, parents accurately reported classroom hearing aid use; however, those with children in Grades 5-7 were less accurate than those with children in earlier grades. These preliminary results suggest that children with milder hearing loss and those in Grades 5-7 are at increased risk for reduced hearing aid use in the classroom. Also, parents of school-age children in these later grades are less accurate reporters of classroom hearing aid use compared to parents of children in earlier grades.

A homozygous founder missense variant in arylsulfatase G abolishes its enzymatic activity causing atypical Usher syndrome in humans.

PubMed

Khateb, Samer; Kowalewski, Björn; Bedoni, Nicola; Damme, Markus; Pollack, Netta; Saada, Ann; Obolensky, Alexey; Ben-Yosef, Tamar; Gross, Menachem; Dierks, Thomas; Banin, Eyal; Rivolta, Carlo; Sharon, Dror

2018-01-04

PurposeWe aimed to identify the cause of disease in patients suffering from a distinctive, atypical form of Usher syndrome.MethodsWhole-exome and genome sequencing were performed in five patients from three families of Yemenite Jewish origin, suffering from distinctive retinal degeneration phenotype and sensorineural hearing loss. Functional analysis of the wild-type and mutant proteins was performed in human fibrosarcoma cells.ResultsWe identified a homozygous founder missense variant, c.133G>T (p.D45Y) in arylsulfatase G (ARSG). All patients shared a distinctive retinal phenotype with ring-shaped atrophy along the arcades engirdling the fovea, resulting in ring scotoma. In addition, patients developed moderate to severe sensorineural hearing loss. Both vision and hearing loss appeared around the age of 40 years. The identified variant affected a fully conserved amino acid that is part of the catalytic site of the enzyme. Functional analysis of the wild-type and mutant proteins showed no basal activity of p.D45Y.ConclusionHomozygosity for ARSG-p.D45Y in humans leads to protein dysfunction, causing an atypical combination of late-onset Usher syndrome. Although there is no evidence for generalized clinical manifestations of lysosomal storage diseases in this set of patients, we cannot rule out the possibility that mild and late-onset symptoms may appear.GENETICS in MEDICINE advance online publication, 4 January 2018; doi:10.1038/gim.2017.227.

Clinical variability of Waardenburg-Shah syndrome in patients with proximal 13q deletion syndrome including the endothelin-B receptor locus.

PubMed

Tüysüz, Beyhan; Collin, Anna; Arapoğlu, Müjde; Suyugül, Nezir

2009-10-01

Waardenburg-Shah syndrome (Waardenburg syndrome type IV-WS4) is an auditory-pigmentary disorder that combines clinical features of pigmentary abnormalities of the skin, hair and irides, sensorineural hearing loss, and Hirschsprung disease (HSCR). Mutations in the endothelin-B receptor (EDNRB) gene on 13q22 have been found to cause this syndrome. Mutations in both alleles cause the full phenotype, while heterozygous mutations cause isolated HSCR or HSCR with minor pigmentary anomalies and/or sensorineural deafness. We investigated the status of the EDNRB gene, by FISH analysis, in three patients with de novo proximal 13q deletions detected at cytogenetic analysis and examined the clinical variability of WS4 among these patients. Chromosome 13q was screened with locus specific FISH probes and breakpoints were determined at 13q22.1q31.3 in Patients 1 and 3, and at 13q21.1q31.3 in Patient 2. An EDNRB specific FISH probe was deleted in all three patients. All patients had common facial features seen in proximal 13q deletion syndrome and mild mental retardation. However, findings related to WS4 were variable; Patient 1 had hypopigmentation of the irides and HSCR, Patient 2 had prominent bicolored irides and mild bilateral hearing loss, and Patient 3 had only mild unilateral hearing loss. These data contribute new insights into the pathogenesis of WS4.

Morphological Correlates of Hearing Loss after Cochlear Implantation and Electro-Acoustic Stimulation in a Hearing-Impaired Guinea Pig Model

PubMed Central

Reiss, Lina A.J.; Stark, Gemaine; Nguyen-Huynh, Anh T.; Spear, Kayce A.; Zhang, Hongzheng; Tanaka, Chiemi; Li, Hongzhe

2016-01-01

Hybrid or electro-acoustic stimulation (EAS) cochlear implants (CIs) are designed to provide high-frequency electric hearing together with residual low-frequency acoustic hearing. However, 30-50% of EAS CI recipients lose residual hearing after implantation. The objective of this study was to determine the mechanisms of EAS-induced hearing loss in an animal model with high-frequency hearing loss. Guinea pigs were exposed to 24 hours of noise (12-24 kHz at 116 dB) to induce a high-frequency hearing loss. After recovery, two groups of animals were implanted (n=6 per group), with one group receiving chronic acoustic and electric stimulation for 10 weeks, and the other group receiving no stimulation during this time frame. A third group (n=6) was not implanted, but received chronic acoustic stimulation. Auditory brainstem responses were recorded biweekly to monitor changes in hearing. The organ of Corti was immunolabeled with phalloidin, anti-CtBP2, and anti-GluR2 to quantify hair cells, ribbons and post-synaptic receptors. The lateral wall was immunolabeled with phalloidin and lectin to quantify stria vascularis capillary diameters. Bimodal or trimodal diameter distributions were observed; the number and location of peaks were objectively determined using the Aikake Information Criterion and Expectation Maximization algorithm. Noise exposure led to immediate hearing loss at 16-32 kHz for all groups. Cochlear implantation led to additional hearing loss at 4-8 kHz; this hearing loss was negatively and positively correlated with minimum and maximum peaks of the bimodal or trimodal distributions of stria vascularis capillary diameters, respectively. After chronic stimulation, no significant group changes in thresholds were seen; however, elevated thresholds at 1 kHz in implanted, stimulated animals were significantly correlated with decreased presynaptic ribbon and postsynaptic receptor counts. Inner and outer hair cell counts did not differ between groups and were not correlated with threshold shifts at any frequency. As in the previous study in a normal-hearing model, stria vascularis capillary changes were associated with immediate hearing loss after implantation, while little to no hair cell loss was observed even in cochlear regions with threshold shifts as large as 40-50 dB. These findings again support a role of lateral wall blood flow changes, rather than hair cell loss, in hearing loss after surgical trauma, and implicate the endocochlear potential as a factor in implantation-induced hearing loss. Further, the analysis of the hair cell ribbons and post-synaptic receptors suggest that delayed hearing loss may be linked to synapse or peripheral nerve loss due to stimulation excitotoxicity or inflammation. Further research is needed to separate these potential mechanisms of delayed hearing loss. PMID:26087114

Association Between Childhood Hearing Disorders and Tinnitus in Adulthood.

PubMed

Aarhus, Lisa; Engdahl, Bo; Tambs, Kristian; Kvestad, Ellen; Hoffman, Howard J

2015-11-01

The association between childhood hearing disorders and adult tinnitus has not been examined in longitudinal cohort studies. To determine the association between different types of childhood hearing loss and tinnitus in adulthood and evaluate whether tinnitus risk is mediated by adult hearing loss. Population-based cohort study of 32 430 adults (aged 20-56 years) who underwent pure-tone audiometry and completed a tinnitus questionnaire in the Nord-Trøndelag Hearing Loss Study, which was a part of the Nord-Trøndelag Health Study 2 (HUNT2). The study was conducted from January 1, 2014, to April 1, 2015. Data analysis was performed from April 1, 2014, to April 1, 2015. As children, the same individuals had undergone screening audiometry in a longitudinal primary school hearing investigation, including ear, nose, and throat examinations when indicated. Pure-tone audiometry, questionnaires, and ear, nose, and throat examinations. Self-reported tinnitus (yes or no) in adulthood measured by questionnaires. Adults who had hearing loss at the time of the school investigation (n = 3026) reported more tinnitus, measured as odds ratio (95% CI), than did adults with normal childhood hearing (n = 29 404) (1.4 [1.3-1.6]). Childhood hearing disorders associated with tinnitus in adulthood included sensorineural hearing loss, chronic suppurative otitis media, and hearing loss associated with a history of recurrent acute otitis media (2.4 [1.9-3.0], 2.4 [1.5-3.9], and 1.6 [1.3-2.0], respectively). These estimates were adjusted for age, sex, and noise exposure in adulthood. After further analyses that included adjustment for adult hearing threshold, none of these childhood hearing disorders remained positively associated with tinnitus. Childhood hearing disorders associated with tinnitus in adulthood include sensorineural hearing loss, chronic suppurative otitis media, and hearing loss associated with a history of recurrent acute otitis media. After adjustment for the adult hearing threshold, none of the childhood hearing disorders was positively associated with tinnitus. Hence, it appears that these significant associations are mediated or transmitted through adult hearing loss.

Hearing loss in the developing world: evaluating the iPhone mobile device as a screening tool.

PubMed

Peer, S; Fagan, J J

2015-01-01

Developing countries have the world's highest prevalence of hearing loss, and hearing screening programmes are scarce. Mobile devices such as smartphones have potential for audiometric testing. To evaluate the uHear app using an Apple iPhone as a possible hearing screening tool in the developing world, and to determine accuracy of certain hearing thresholds that could prove useful in early detection of hearing loss for high-risk populations in resource-poor communities. This was a quasi-experimental study design. Participants recruited from the Otolaryngology Clinic, Groote Schuur Hospital, Cape Town, South Africa, completed a uHear test in three settings--waiting room (WR), quiet roon (QR) and soundproof room (SR). Thresholds were compared with formal audiograms. Twenty-five patients were tested (50 ears). The uHear test detected moderate or worse hearing loss (pure-tone average (PTA) > 40 dB accurately with a sensitivity of 100% in all three environments. Specificity was 88% (SR), 73% (QR) and 68% (WR). Its was highly accurate in detecting high-frequency hearing loss (2 000, 4 000, 6 000 Hz) in the QR and SR with 'good' and 'very good' kappa values, showing statistical significance (p < 0.05). It was moderately accurate in low-frequency hearing loss (250, 500, 1 000 Hz) in the SR, and poor in the QR and WR. Using the iPhone, uHear is a feasible screening test to rule out significant hearing loss (PTA > 40 dB). It is highly sensitive for detecting threshold changes at high frequencies, making it reasonably well suited to detect presbycusis and ototoxic hearing loss from HIV, tuberculosis therapy and chemotherapy. Portability and ease of use make it appropriate to use in developing world communities that lack screening programmes.

The prevalence of noise-induced occupational hearing loss in dentistry personnel.

PubMed

Khaimook, Wandee; Suksamae, Puwanai; Choosong, Thitiworn; Chayarpham, Satit; Tantisarasart, Ratchada

2014-09-01

Occupational hearing loss is the second most common health problem in the industrialized world. Dental personnel exposed to occupational noise may experience hearing loss. This article compares the prevalence of hearing loss in the general population to that of dental personnel exposed to noise during work hours and identifies risk factors for hearing loss among workers at a dental school. This prospective study included 76 dental personnel on the faculty of dentistry at a major university in Asia who were exposed to noise and 76 individuals in a control group. Nearly 16% of the study group and 21% of the control group had lost hearing, a nonsignificant difference (p = .09). Hearing loss was significantly related to work tenure longer than 15 years and age older than 40 years (p < .001 ).

The importance of creating a social business to produce low-cost hearing aids.

PubMed

Caccamo, Samantha; Voloshchenko, Anastasia; Dankyi, Nana Yaa

2014-09-01

The World Health Organization (WHO) estimates that about 280 million people worldwide have a bilateral hearing loss, mostly living in poor countries. Hearing loss causes heavy social burdens on individuals, families, communities and countries. However, due to the lack of accessibility and affordability, the vast majority of people in the world who need hearing aids do not have access to them. Low-income countries are thus pulled into a disability/poverty spiral. From this standpoint, the production of available, accessible and affordable hearing aids for the poorest populations of our planet should be one of the main issues in global hearing healthcare. Designing and producing a brand new low-cost hearing aid is the most effective option. Involving a large producer of hearing aids in the creation of a social business to solve the problem of access to affordable hearing aids is an essential step to reduce hearing disability on a large scale globally. Today's technology allows for the creation of a "minimal design" product that does not exceed $100-$150, that can be further lowered when purchased in large quantities and dispensed with alternative models. It is conceivable that by making a sustainable social business, the low cost product could be sold with a cross-subsidy model in order to recover the overhead costs. Social business is an economic model that has the potential to produce and distribute affordable hearing aids in low- and middle-income countries. Rehabilitation of hearing impaired children will be carried out in partnership with Sahic (Society of Assistance to Hearing Impaired Children) in Dhaka, Bangladesh and the ENT Department of Ospedale Burlo di Trieste, Dr. Eva Orzan.

"I know you can hear me": neural correlates of feigned hearing loss.

PubMed

McPherson, Bradley; McMahon, Katie; Wilson, Wayne; Copland, David

2012-08-01

In the assessment of human hearing, it is often important to determine whether hearing loss is organic or nonorganic in nature. Nonorganic, or functional, hearing loss is often associated with deceptive intention on the part of the listener. Over the past decade, functional neuroimaging has been used to study the neural correlates of deception, and studies have consistently highlighted the contribution of the prefrontal cortex in such behaviors. Can patterns of brain activity be similarly used to detect when an individual is feigning a hearing loss? To answer this question, 15 adult participants were requested to respond to pure tones and simple words correctly, incorrectly, randomly, or with the intent to feign a hearing loss. As predicted, more activity was observed in the prefrontal cortices (as measured by functional magnetic resonance imaging), and delayed behavioral reaction times were noted, when the participants feigned a hearing loss or responded randomly versus when they responded correctly or incorrectly. The results suggest that cortical imaging techniques could play a role in identifying individuals who are feigning hearing loss. Copyright © 2011 Wiley Periodicals, Inc.

Assessment of knowledge of harmful effects and exposure to recreational music in college students of delhi: a cross sectional exploratory study.

PubMed

Gupta, Neelima; Sharma, Arun; Singh, P P; Goyal, Abhishek; Sao, Rahul

2014-09-01

Exposure to loud sounds results in a mild to profound degree of temporary or permanent hearing loss. Though occupational noise exposure remains the most commonly identified cause of noise-induced hearing loss, potentially hazardous noise can be encountered during recreational activities. Unfortunately not much attention is being given to the increasing trend of prolonged exposure to noisy environment, in the younger generation of Indians. The purpose of our study was to know the knowledge of college students about the harmful effects of loud music, prevailing practices with regard to exposure to recreational music and the subjective effects that this exposure is causing if any. Cross Sectional survey of College Students (n = 940), from randomly selected colleges of Delhi University. Majority of students listened to music using music-enabled phones; earphones were preferred and 56.6 % participants listened to music on a loud volume. Effects experienced due to loud sound were headache (58 %), inability to concentrate (48 %), and ringing sensation in the ear (41.8 %). Only 2.7 % respondents used ear protection device in loud volume settings. Twenty-three percent respondents complained of transient decreased hearing and other effects after exposure to loud music. 83.8 % knew that loud sound has harmful effect on hearing but still only 2.7 % used protection device. The survey indicates that we need to generate more such epidemiological data and follow up studies on the high risk group; so as to be able to convincingly sensitize the Indian young generation to take care of their hearing and the policy makers to have more information and education campaigns for this preventable cause of deafness.

Active Duty - U.S. Army Noise Induced Hearing Injury Surveillance Calendar Years 2009-2013

DTIC Science & Technology

2014-06-01

rates for sensorineural hearing loss, significant threshold shift, tinnitus , and Noise-Induced Hearing Loss. The intention is to monitor the morbidity...surveillance. These code groups include sensorineural hearing loss (SNHL), significant threshold shift (STS), noise-induced hearing loss (NIHL) and tinnitus ... Tinnitus ) was analyzed using a regression model to determine the trend of incidence rates from 2007 to the current year. Statistical significance of a

Emergent Literacy Skills in Preschool Children with Hearing Loss Who Use Spoken Language: Initial Findings from the Early Language and Literacy Acquisition (ELLA) Study

ERIC Educational Resources Information Center

Werfel, Krystal L.

2017-01-01

Purpose: The purpose of this study was to compare change in emergent literacy skills of preschool children with and without hearing loss over a 6-month period. Method: Participants included 19 children with hearing loss and 14 children with normal hearing. Children with hearing loss used amplification and spoken language. Participants completed…

Hearing loss and work participation: a cross-sectional study in Norway.

PubMed

Svinndal, Elisabeth Vigrestad; Solheim, Jorunn; Rise, Marit By; Jensen, Chris

2018-04-27

To study work participation of persons with hearing loss, and associations with hearing disabilities, self-reported workability, fatigue and work accommodation. Cross-sectional internet-based survey. A total of 10,679 persons with hearing loss within working-age were invited to answer the survey, where 3330 answered (35.6%). Degree of hearing loss was associated with low workability, fatigue and work place accommodation, while sick leave was associated with fatigue. Degree of hearing loss was positively associated with being unemployed (p < .001) and having part-time work (p < .01) (often combined with disability benefits) for women. Work place accommodation was more frequently provided among respondents working with sedentary postures, high seniority, long-term sick leave or low workability. Additional unfavourable sensory conditions were associated with decreased employment (p < .001) and workability, and an increase in sick leave (p < .01) and fatigue (p < .001). Hearing loss seemed to influence work participation factors negatively; particularly, for moderate hearing loss and for women, even though the degree of employment was high. A lack of work place accommodation when there was a need for such was found. This implies increased attentiveness towards individual needs concerning the experienced disability a hearing loss may produce. A more frequent use of hearing disability assessment is suggested.

Audiologic and otologic phenotype in children with Duane's Retraction Syndrome: A rare ophthalmologic disorder.

PubMed

Weir, Forest W; Kreicher, Kathryn L; Hatch, Jonathan L; Nguyen, Shaun A; Meyer, Ted A

2016-10-01

The focus of this study was to evaluate the prevalence, type, and severity of hearing impairment in patients with Duane's Retraction Syndrome and to relate these measures to patient demographics and other otologic and audiologic factors. Retrospective AudGen Database analysis Tertiary academic referral center Pediatric patients in AudGenDB with a diagnosis of Duane's Retraction Syndrome (DRS). Appropriate audiologic, otologic, and demographic data were recorded. Seventy-nine patients (n = 79) met inclusion criteria. The first encounter with available audiometric data or the first encounter with hearing loss were documented. Audiograms were stratified by type and severity of hearing loss, and common associated medical issues were documented. 57 children had normal hearing; 22 had hearing loss. 9 ears had pure conductive hearing loss, 1 had pure sensorineural, 14 ears had components of both, and 79 had hearing loss that could not be specified. Multivariate regression revealed episodes of chronic otitis and craniofacial anomalies are associated with worse hearing loss. This study presents a detailed characterization of hearing loss in patients with Duane's retraction syndrome. Conductive and sensorineural hearing loss are both prevalent among these children. Careful and early audiologic evaluation of all patients with DRS is important. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

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

PubMed

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

2009-12-01

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

Hearing loss is associated with decreased nonverbal intelligence in rural Nepal.

PubMed

Emmett, Susan D; Schmitz, Jane; Pillion, Joseph; Wu, Lee; Khatry, Subarna K; Karna, Sureshwar L; LeClerq, Steven C; West, Keith P

2015-01-01

To evaluate the association between adolescent and young-adult hearing loss and nonverbal intelligence in rural Nepal. Cross-sectional assessment of hearing loss among a population cohort of adolescents and young adults. Sarlahi District, southern Nepal. Seven hundred sixty-four individuals aged 14 to 23 years. Evaluation of hearing loss, defined by World Health Organization criteria of pure-tone average greater than 25 decibels (0.5, 1, 2, 4 kHz), unilaterally and bilaterally. Nonverbal intelligence, as measured by the Test of Nonverbal Intelligence, 3rd Edition standardized score (mean, 100; standard deviation, 15). Nonverbal intelligence scores differed between participants with normal hearing and those with bilateral (p = 0.04) but not unilateral (p = 0.74) hearing loss. Demographic and socioeconomic factors including male sex; higher caste; literacy; education level; occupation reported as student; and ownership of a bicycle, watch, and latrine were strongly associated with higher nonverbal intelligence scores (all p < 0.001). Subjects with bilateral hearing loss scored an average of 3.16 points lower (95% confidence interval, -5.56 to -0.75; p = 0.01) than subjects with normal hearing after controlling for socioeconomic factors. There was no difference in nonverbal intelligence score based on unilateral hearing loss (0.97; 95% confidence interval, -1.67 to 3.61; p = 0.47). Nonverbal intelligence is adversely affected by bilateral hearing loss even at mild hearing loss levels. Socio economic well-being appears compromised in individuals with lower nonverbal intelligence test scores.

Pilot study of cognition in children with unilateral hearing loss.

PubMed

Ead, Banan; Hale, Sandra; DeAlwis, Duneesha; Lieu, Judith E C

2013-11-01

The objective of this study was to obtain preliminary data on the cognitive function of children with unilateral hearing loss in order to identify, quantify, and interpret differences in cognitive and language functions between children with unilateral hearing loss and with normal hearing. Fourteen children ages 9-14 years old (7 with severe-to-profound sensorineural unilateral hearing loss and 7 sibling controls with normal hearing) were administered five tests that assessed cognitive functions of working memory, processing speed, attention, and phonological processing. Mean composite scores for phonological processing were significantly lower for the group with unilateral hearing loss than for controls on one composite and four subtests. The unilateral hearing loss group trended toward worse performance on one additional composite and on two additional phonological processing subtests. The unilateral hearing loss group also performed worse than the control group on the complex letter span task. Analysis examining performance on the two levels of task difficulty revealed a significant main effect of task difficulty and an interaction between task difficulty and group. Cognitive function and phonological processing test results suggest two related deficits associated with unilateral hearing loss: (1) reduced accuracy and efficiency associated with phonological processing, and (2) impaired executive control function when engaged in maintaining verbal information in the face of processing incoming, irrelevant verbal information. These results provide a possible explanation for the educational difficulties experienced by children with unilateral hearing loss. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

Hearing Loss is Associated with Decreased Nonverbal Intelligence in Rural Nepal

PubMed Central

Emmett, Susan D.; Schmitz, Jane; Pillion, Joseph; Wu, Lee; Khatry, Subarna K.; Karna, Sureshwar L.; LeClerq, Steven C.; West, Keith P.

2014-01-01

Objective Evaluate the association between adolescent and young adult hearing loss and nonverbal intelligence in rural Nepal Study Design Cross-sectional assessment of hearing loss among a population cohort of adolescents and young adults Setting Sarlahi District, southern Nepal Patients 764 individuals aged 14–23 years Intervention Evaluation of hearing loss, defined by WHO criteria of pure-tone average (PTA) >25 decibels (0.5, 1, 2, 4 kHz), unilaterally and bilaterally Main Outcome Measure Nonverbal intelligence, measured by the Test of Nonverbal Intelligence, 3rd Edition (TONI-3) standardized score (mean 100; standard deviation (SD) 15) Results Nonverbal intelligence scores differed between participants with normal hearing and those with bilateral (p =0.04) but not unilateral (p =0.74) hearing loss. Demographic and socioeconomic factors including male sex, higher caste, literacy, education level, occupation reported as student, and ownership of a bicycle, watch, and latrine were strongly associated with higher nonverbal intelligence scores (all p <0.001). Subjects with bilateral hearing loss scored an average of 3.16 points lower (95% CI: −5.56, −0.75; p =0.01) than subjects with normal hearing after controlling for socioeconomic factors. There was no difference in nonverbal intelligence score based on unilateral hearing loss (0.97; 95% CI: −1.67, 3.61; p =0.47). Conclusions Nonverbal intelligence is adversely affected by bilateral hearing loss, even at mild hearing loss levels. Social and economic well being appear compromised in individuals with lower nonverbal intelligence test scores. PMID:25299832

Hearing loss in very preterm and very low birthweight infants at the age of 5 years in a nationwide cohort.

PubMed

Veen, S; Sassen, M L; Schreuder, A M; Ens-Dokkum, M H; Verloove-Vanhorick, S P; Brand, R; Grote, J J; Ruys, J H

1993-02-01

In a geographically defined population of very preterm and very low birthweight infants (gestational age < 32 weeks and/or birthweight < 1500 g) hearing was evaluated in 890 children by pure-tone audiometry at the age of 5 years. Hearing loss was conductive/unspecified in 123 (13.8%) and sensorineural in 13 (1.5%) children. The prevalence of sensorineural hearing loss was 15 times as high as in 5-7 year old children in the Dutch population at large. The sensorineural hearing loss prevalence in very low birthweight and extremely low birthweight infants was similar. On account of communication disorders 10 (1.1%) children were classified as disabled and 6 (0.7%) as handicapped, following the definitions of the International Classification of Impairments, Disabilities, and Handicaps of the World Health Organisation. Children with conductive hearing loss had a higher risk of impairments, disabilities and handicaps of language and speech development, than children with normal hearing, the difference being statistically significant. The same holds for children with sensorineural hearing loss; moreover they had a significantly higher risk of impairments, disabilities and handicaps of mental development. Overall comparison of children with and without sensorineural hearing loss proved that the children with sensorineural hearing loss had a significantly less favourable outcome, based on 15 perinatal factors simultaneously. The age at which sensorineural hearing loss in very preterm and/or very low birthweight infants is detected has to be improved.

Hearing loss in Behçet syndrome.

PubMed

Bakhshaee, Mehdi; Mahdi, Bakhshaee; Ghasemi, Mohammad Mehdi; Mehdi, Ghasemi Mohammad; Hatef, Mohammad Reza; Reza, Hatef Mohammad; Talebmehr, Mahdieh; Mahdieh, Talebmehr; Shakeri, Mohammad Taghi; Taghi, Shakeri Mohammad

2007-09-01

To determine the prevalence and characteristics of hearing loss in Behçet syndrome. This study included 27 patients with Behçet syndrome and 35 sex-and age-matched controls. A complete audiological evaluation was performed. The average pure-tone audiograms from both groups showed a statistically significant hearing loss in the Behçet group. Sixteen patients (59.26%) showed some degrees of sensorineural hearing loss (SNHL), with the high-frequency type (4, 8, 10, and 12 kHz) being the most common pattern (93.75%). Hearing loss was the fourth most common manifestation. Although the patient's age, sex, and the duration of the disease were not related to hearing loss, there was a significant correlation between a negative pathergy test and hearing loss in patients with Behçet syndrome. We should consider audiovestibular involvement in Behçet syndrome as a common finding.

Preliminary comparison of infants speech with and without hearing loss

NASA Astrophysics Data System (ADS)

McGowan, Richard S.; Nittrouer, Susan; Chenausky, Karen

2005-04-01

The speech of ten children with hearing loss and ten children without hearing loss aged 12 months is examined. All the children with hearing loss were identified before six months of age, and all have parents who wish them to become oral communicators. The data are from twenty minute sessions with the caregiver and child, with their normal prostheses in place, in semi-structured settings. These data are part of a larger test battery applied to both caregiver and child that is part of a project comparing the development of children with hearing loss to those without hearing loss, known as the Early Development of Children with Hearing Loss. The speech comparisons are in terms of number of utterances, syllable shapes, and segment type. A subset of the data was given a detailed acoustic analysis, including formant frequencies and voice quality measures. [Work supported by NIDCD R01 006237 to Susan Nittrouer.

Otitis media and hearing loss among 12-16-year-old Inuit of Inukjuak, Quebec, Canada.

PubMed

Ayukawa, Hannah; Bruneau, Suzanne; Proulx, Jean-François; Macarthur, Judy; Baxter, James

2004-01-01

Chronic otitis media (COM) and associated hearing loss is a frequent problem for many Inuit children in Canada. In this study, we evaluated individuals aged 12-16 years living in Inukjuak, to determine the prevalence of middle ear disease and hearing loss, and the effect of hearing loss on academic performance. Otological examination, hearing test, medical and school file review were performed in November 1997. 88 individuals were seen. Otological examination revealed maximal scarring in 1.8%, minimal scarring in 34.9%, normal eardrums in 49.1% and chronic otitis media in 16.9%. There were 62 individuals whose ear exams could be directly compared with a previous exam done in 1987. Of those, there were three ears that had developed COM and 4/13 ears with COM in 1987 that had healed. Hearing tests found bilateral normal hearing in 80% (PTA <20dB), unilateral loss in 15% and bilateral loss in 5%. Hearing loss was associated with poorer academic performance in Language (p<.05). A similar trend was found in Mathematics but not in Inuttitut. Chronic otitis media remains a significant problem among the Inuit, with a prevalence of 16.9% in individuals aged 12-16 years. One in five in this age group has hearing loss, and this hearing loss impacts on academic performance.

Emergent Literacy Skills in Preschool Children With Hearing Loss Who Use Spoken Language: Initial Findings From the Early Language and Literacy Acquisition (ELLA) Study.

PubMed

Werfel, Krystal L

2017-10-05

The purpose of this study was to compare change in emergent literacy skills of preschool children with and without hearing loss over a 6-month period. Participants included 19 children with hearing loss and 14 children with normal hearing. Children with hearing loss used amplification and spoken language. Participants completed measures of oral language, phonological processing, and print knowledge twice at a 6-month interval. A series of repeated-measures analyses of variance were used to compare change across groups. Main effects of time were observed for all variables except phonological recoding. Main effects of group were observed for vocabulary, morphosyntax, phonological memory, and concepts of print. Interaction effects were observed for phonological awareness and concepts of print. Children with hearing loss performed more poorly than children with normal hearing on measures of oral language, phonological memory, and conceptual print knowledge. Two interaction effects were present. For phonological awareness and concepts of print, children with hearing loss demonstrated less positive change than children with normal hearing. Although children with hearing loss generally demonstrated a positive growth in emergent literacy skills, their initial performance was lower than that of children with normal hearing, and rates of change were not sufficient to catch up to the peers over time.

Clinical Study to Evaluate the Association Between Sensorineural Hearing Loss and Diabetes Mellitus in Poorly Controlled Patients Whose HbA1c >8.

PubMed

Srinivas, C V; Shyamala, V; Shiva Kumar, B R

2016-06-01

The relationship between sensorineural hearing loss (SNHL) and Diabetes mellitus has been known since more than 150 years. The pathophysiology of diabetes related hearing loss is speculative. Hearing loss is usually, bilateral, gradual onset, affecting higher frequencies. This study aims at knowing the prevalence of SNHL in DM and its relation to age, sex, duration of DM and control of DM. A total of 50 type 2 diabetics of age group 30-65 years were involved in the study. FBS, PPBS, HbA1c of all the subjects were done and later subjected to PTA. The type and severity of hearing loss was noted. Occurrence of SNHL was later compared with age, sex, duration, and control of DM. Sensorineural hearing loss was found in 66 % of type II diabetic patients and 34 % were found normal. Out of 50 diabetes mellitus patients, 33 patients had SNHL. All cases of SNHL detected were of gradual in onset and no one had hearing loss of sudden onset. Normal hearing was found in 34 % of patients, whereas 54 % of patients had mild hearing loss and 12 % of patients had moderate hearing loss. Association of hearing loss of DM patients with sex of the patient is insignificant. However there is significant association between older age group, longer duration and uncontrolled DM with that of SNHL. In subjects with HbA1c more than 8 and duration of diabetes mellitus more than 10 years prevalence of SNHL is more than 85 %, which is statistically significant. Sensorineural hearing loss in diabetes mellitus is gradually progressive involving high frequency thresholds. Hearing threshold increases with increasing age duration of diabetes and also high level of HbA1c greater than 8 %.

Masking Release in Children and Adults with Hearing Loss When Using Amplification

ERIC Educational Resources Information Center

Brennan, Marc; McCreery, Ryan; Kopun, Judy; Lewis, Dawna; Alexander, Joshua; Stelmachowicz, Patricia

2016-01-01

Purpose: This study compared masking release for adults and children with normal hearing and hearing loss. For the participants with hearing loss, masking release using simulated hearing aid amplification with 2 different compression speeds (slow, fast) was compared. Method: Sentence recognition in unmodulated noise was compared with recognition…

Alcohol Use among Students with and without Hearing Loss

ERIC Educational Resources Information Center

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…

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 high frequency sounds, as demonstrated by their preference for either the extended bandwidth or nonlinear frequency compression conditions over the restricted bandwidth condition. Preference for extended bandwidth can be limited for those with greater degrees of hearing loss, but participants with greater hearing loss may be more likely to prefer nonlinear frequency compression. Further investigation using participants with more severe hearing loss may be warranted. PMID:25514451

The Natural History and Rehabilitative Outcomes of Hearing Loss in Congenital Cytomegalovirus: A Systematic Review.

PubMed

Fletcher, Kyle T; Horrell, Erin M Wolf; Ayugi, John; Irungu, Catherine; Muthoka, Maria; Creel, Liza M; Lester, Cathy; Bush, Matthew L

2018-06-15

The purpose of this study was to examine the literature regarding the natural history and rehabilitative outcomes of sensorineural hearing loss from congenital cytomegalovirus infections. A systematic search was performed in PubMed, PsychINFO, CINAHL, and Web of Science to identify peer-reviewed research. Eligible studies were those containing original peer-reviewed research in English addressing either the natural history or rehabilitative outcomes of sensorineural hearing loss (SNHL) in congenital cytomegalovirus (cCMV). Two investigators independently reviewed all articles and extracted data. Bias was assessed using the Cochrane Collaboration's tool and the Newcastle-Ottawa Assessment Scale. Thirty-six articles were reviewed. Universal screening identifies 0.2 to 1% of newborns with cCMV infection. SNHL ranged from 8 to 32% of infants and was more prevalent in symptomatic versus asymptomatic cases. Nine to 68% of hearing loss occurs in a late or delayed fashion. In 7 to 71% of cases hearing loss is progressive. Cochlear implantation (CI) is a viable option for patients with cCMV associated hearing loss and leads to improvements in hearing and language. There is limited literature comparing rehabilitation outcomes in cCMV and non-cCMV CI recipients. Late onset and progressive hearing loss is seen in children who develop hearing loss from cCMV. Frequent audiologic follow-up is necessary considering the natural history of cCMV hearing loss. Universal screening should be pursued due to the number of asymptomatic children, at birth, who develop late onset/delayed hearing loss. CI is an effective means of improving speech and language in this population.

Demographic Characteristics and Impairments of Louisiana Students with Usher's Syndrome.

ERIC Educational Resources Information Center

Brown, S. C.

1987-01-01

Of 51 Louisiana students with Usher's Syndrome (a genetic condition characterized by hearing loss and progressive blindness), 71 percent manifested visual impairment and hearing loss, 9 percent had neither, 10 percent had visual impairments but a less-than-profound hearing loss, and 10 percent had profound hearing loss and no visual impairment.…

Phonological Awareness and Vocabulary Performance of Monolingual and Bilingual Preschool Children with Hearing Loss

ERIC Educational Resources Information Center

Lund, Emily; Werfel, Krystal L.; Schuele, C. Melanie

2015-01-01

This pilot study compared the phonological awareness skills and vocabulary performance of English monolingual and Spanish-English bilingual children with and without hearing loss. Preschool children with varying degrees of hearing loss (n = 18) and preschool children without hearing loss (n = 19) completed measures of phonological awareness and…

Commentary: Listening Can Be Exhausting--Fatigue in Children and Adults with Hearing Loss

ERIC Educational Resources Information Center

Bess, Fred H.; Hornsby, Benjamin W.Y.

2014-01-01

Anecdotal reports of fatigue after sustained speech-processing demands are common among adults with hearing loss; however, systematic research examining hearing loss-related fatigue is limited, particularly with regard to fatigue among children with hearing loss (CHL). Many audiologists, educators, and parents have long suspected that CHL…

Hearing Loss and Deafness. An Annotated Bibliography of Children's Books about Hearing Loss, Deafness, and Hearing Impaired People. Have You Ever Wondered About...?

ERIC Educational Resources Information Center

Oldman-Brown, Deborah

The annotated bibliography lists children's books about hearing loss, deafness, and hearing-impaired persons. The first section lists books about Helen Keller and Anne Sullivan, Keller's teacher. In section 2, each of the fiction entries features at least one major character with hearing impairment. Section 3 contains non-fiction books about…

Communication strategies and accommodations utilized by health care providers with hearing loss: a pilot study.

PubMed

Trotter, Alanna R; Matt, Susan B; Wojnara, Danuta

2014-03-01

Poor communication between health care providers and patients may negatively impact patient outcomes, and enhancing communication is one way to improve outcomes. Effective communication is particularly important for health care providers who have hearing loss. The authors found that a systematic survey of the communication strategies and experiences of health care providers with hearing loss had not yet been conducted. In this pilot study, 32 health care professionals with hearing loss were recruited via the Association of Medical Professionals With Hearing Losses and were asked to complete a 28-question survey. Health care providers with hearing loss already employ strategies that all health care providers are encouraged to use in order to enhance patient–provider communication, and survey participants have found the strategies to be effective. The communication techniques and assistive technologies used by individuals with hearing loss seem to be effective: All participants reported feeling able to communicate effectively with patients at least most of the time. More research is needed to determine if use of these communication techniques has similar results for health care providers without hearing loss.

Effects of hearing loss on speech recognition under distracting conditions and working memory in the elderly.

PubMed

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

2017-01-01

The current study aimed to evaluate hearing-related changes in terms of speech-in-noise processing, fast-rate speech processing, and working memory; and to identify which of these three factors is significantly affected by age-related hearing loss. One hundred subjects aged 65-84 years participated in the study. They were classified into four groups ranging from normal hearing to moderate-to-severe hearing loss. All the participants were tested for speech perception in quiet and noisy conditions and for speech perception with time alteration in quiet conditions. Forward- and backward-digit span tests were also conducted to measure the participants' working memory. 1) As the level of background noise increased, speech perception scores systematically decreased in all the groups. This pattern was more noticeable in the three hearing-impaired groups than in the normal hearing group. 2) As the speech rate increased faster, speech perception scores decreased. A significant interaction was found between speed of speech and hearing loss. In particular, 30% of compressed sentences revealed a clear differentiation between moderate hearing loss and moderate-to-severe hearing loss. 3) Although all the groups showed a longer span on the forward-digit span test than the backward-digit span test, there was no significant difference as a function of hearing loss. The degree of hearing loss strongly affects the speech recognition of babble-masked and time-compressed speech in the elderly but does not affect the working memory. We expect these results to be applied to appropriate rehabilitation strategies for hearing-impaired elderly who experience difficulty in communication.

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.

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

PubMed

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

2017-02-01

Tinnitus is a common condition and there is a need to evaluate effects of tinnitus management in relation to moderating factors such as degree of hearing loss. As it is possible that tinnitus influences concentration, and thus is likely to disturb cognitive processing, the role of cognitive functioning also needs to be investigated. To compare a group of patients with sensorineural hearing loss and tinnitus to a control group with only sensorineural hearing loss (and no tinnitus). To investigate working memory, sleep, and hearing problems measured before and after hearing rehabilitation. A prospective study. The sample consisted of 100 patients, 50 with hearing loss and tinnitus, and 50 controls with hearing loss but no tinnitus. All patients were between 40 and 82 yr old and had a pure-tone average (PTA; average of 0.5, 1, 2, and 4 kHz) <70 dB HL. Patients were tested before and after rehabilitation with hearing aids with regard to their working memory capacity, sleep quality, hearing problems, speech recognition, and tinnitus annoyance. Eight patients dropped out of the study. Thus, a total of 92 patients were included for analysis, with 46 in each group. As a consequence of unplanned age and PTA differences between the groups, an age-matched subsample (n = 30 + 30) was selected for further analysis. Tests including the Reading Span, Hearing-in-Noise Test (HINT), Tinnitus Handicap Inventory (THI), Hearing Handicap Inventory for the Elderly (HHIE), and Pittsburgh Sleep Quality Index (PSQI) were administered before and after hearing aid rehabilitation. There were no between-group differences at baseline in the full sample (n = 92), with the exception of the THI (p < 0.001) and the PSQI (p < 0.002), on which the hearing loss and tinnitus group had significantly higher scores. Pre/post changes were significant for both groups on the Reading Span, and HHIE. However, these improvements were significantly larger for the patients in the hearing loss and tinnitus group on the Reading Span test (p < 0.001) and the PSQI (p < 0.001). Patients with tinnitus and hearing loss also exhibited significantly improved THI scores at follow-up, compared to baseline (p < 0.001). We conducted the same analyses for the age-matched subsample (n = 30 + 30). For the baseline data, only the THI (p < 0.001) and the PSQI (p < 0.015) difference remained significant. With regard to the pre/post changes, we found the same differences in improvement in Reading Span (p < 0.001) and the PSQI (p < 0.015) as in the full sample. Patients with tinnitus benefited from hearing aid rehabilitation. The observed differences in cognitive function were unexpected, and there were larger score improvements on the Reading Span test in the hearing loss and tinnitus group than in the hearing loss group. Patients with tinnitus and hearing loss may receive extra benefit in terms of cognitive function following hearing aid rehabilitation. American Academy of Audiology

Delays in Diagnosis of Congenital Hearing Loss in Rural Children

PubMed Central

Bush, Matthew L.; Bianchi, Kristin; Lester, Cathy; Shinn, Jennifer B.; Gal, TJ; Fardo, David W.; Schoenberg, Nancy

2013-01-01

Objective To examine the incidence of pediatric congenital hearing loss and the timing of diagnosis in a rural region of hearing healthcare disparity. Study design Data from the Kentucky newborn hearing-screening program was accessed to determine the incidence of congenital hearing loss in Kentucky, both in the extremely rural region of Appalachia and non-Appalachian region of Kentucky. We also performed a retrospective review of records of children with congenital hearing loss at our institution to determine the timing of diagnostic testing. Results In Kentucky, during 2009–2011, there were 6,970 newborns who failed hearing screening; the incidence of newborn hearing loss was 1.71 per 1000 births (1.28/1000 in Appalachia and 1.87/1000 in non-Appalachia). 23.8% of Appalachian newborns compared with 17.3% of non-Appalachian children failed to obtain follow-up diagnostic testing. Children from Appalachia were significantly delayed in obtaining a final diagnosis of hearing loss compared with children from non-Appalachian regions (p=0.04). Conclusion Congenital hearing loss in children from rural regions with hearing healthcare disparities is a common problem and these children are at risk for a delay in the timing of diagnosis, which has the potential to limit language and social development. It is important to further assess the causative factors and develop interventions that can address this hearing healthcare disparity issue. PMID:24183213

Audio-visual speech processing in age-related hearing loss: Stronger integration and increased frontal lobe recruitment.

PubMed

Rosemann, Stephanie; Thiel, Christiane M

2018-07-15

Hearing loss is associated with difficulties in understanding speech, especially under adverse listening conditions. In these situations, seeing the speaker improves speech intelligibility in hearing-impaired participants. On the neuronal level, previous research has shown cross-modal plastic reorganization in the auditory cortex following hearing loss leading to altered processing of auditory, visual and audio-visual information. However, how reduced auditory input effects audio-visual speech perception in hearing-impaired subjects is largely unknown. We here investigated the impact of mild to moderate age-related hearing loss on processing audio-visual speech using functional magnetic resonance imaging. Normal-hearing and hearing-impaired participants performed two audio-visual speech integration tasks: a sentence detection task inside the scanner and the McGurk illusion outside the scanner. Both tasks consisted of congruent and incongruent audio-visual conditions, as well as auditory-only and visual-only conditions. We found a significantly stronger McGurk illusion in the hearing-impaired participants, which indicates stronger audio-visual integration. Neurally, hearing loss was associated with an increased recruitment of frontal brain areas when processing incongruent audio-visual, auditory and also visual speech stimuli, which may reflect the increased effort to perform the task. Hearing loss modulated both the audio-visual integration strength measured with the McGurk illusion and brain activation in frontal areas in the sentence task, showing stronger integration and higher brain activation with increasing hearing loss. Incongruent compared to congruent audio-visual speech revealed an opposite brain activation pattern in left ventral postcentral gyrus in both groups, with higher activation in hearing-impaired participants in the incongruent condition. Our results indicate that already mild to moderate hearing loss impacts audio-visual speech processing accompanied by changes in brain activation particularly involving frontal areas. These changes are modulated by the extent of hearing loss. Copyright © 2018 Elsevier Inc. All rights reserved.

[Tinnitus: algorithm of diagnostics and clinical management].

PubMed

Boiko, N V

Hearing of sound, or tinnitus, can be a symptom of different diseases. The differential diagnosis should be based on the identification of subgroups with confirmed causes of the disease. Subjective and objective tinnitus groups should be isolated. Objective tinnitus can be vascular or muscular. In making a diagnosis of tinnitus, it is important to know its characteristics, laterality, circumstances of onset, duration, comorbidity with other symptoms: headache, hearing decline, dizziness, depression, etc. Urgent diagnostic and treatment measures are needed after the identification of 'red flags': acute pulsatile tinnitus, in particular after the brain injury, combination of tinnitus with acute hearing loss and depression.

The search of a genetic basis for noise-induced hearing loss (NIHL).

PubMed

Abreu-Silva, Ronaldo Serafim; Rincon, Daniel; Horimoto, Andréa Roseli Vançan Russo; Sguillar, Ary Papa; Ricardo, Luiz Artur Costa; Kimura, Lilian; Batissoco, Ana Carla; Auricchio, Maria Teresa Balester De Mello; Otto, Paulo Alberto; Mingroni-Netto, Regina Célia

2011-03-01

Knowledge about the genetic factors responsible for noise-induced hearing loss (NIHL) is still limited. This study investigated whether genetic factors are associated or not to susceptibility to NIHL. The family history and genotypes were studied for candidate genes in 107 individuals with NIHL, 44 with other causes of hearing impairment and 104 controls. Mutations frequently found among deaf individuals were investigated (35delG, 167delT in GJB2, Δ(GJB6- D13S1830), Δ(GJB6- D13S1854) in GJB6 and A1555G in MT-RNR1 genes); allelic and genotypic frequencies were also determined at the SNP rs877098 in DFNB1, of deletions of GSTM1 and GSTT1 and sequence variants in both MTRNR1 and MTTS1 genes, as well as mitochondrial haplogroups. When those with NIHL were compared with the control group, a significant increase was detected in the number of relatives affected by hearing impairment, of the genotype corresponding to the presence of both GSTM1 and GSTT1 enzymes and of cases with mitochondrial haplogroup L1. The findings suggest effects of familial history of hearing loss, of GSTT1 and GSTM1 enzymes and of mitochondrial haplogroup L1 on the risk of NIHL. This study also described novel sequence variants of MTRNR1 and MTTS1 genes.

Comparison of self-reported and audiometrically-measured hearing loss in the Australian Defence Force.

PubMed

Kirk, Katherine M; McGuire, Annabel; Nasveld, Peter E; Treloar, Susan A

2012-04-01

To investigate the relationship between self-reported and audiometrically-measured hearing loss in a sample of Australian Defence Force personnel. Responses to a question regarding hearing problems were compared with contemporaneous audiometric data. 3335 members of the Australian Defence Force for whom anonymised medical records were available. The sensitivity of self-report data to identify higher-frequency hearing loss was lower than sensitivity at other frequencies, and positive predictive values were moderate to poor at all frequencies. Performance characteristics of self-report compared with audiometric data also varied with age, sex, and rank. While self-report hearing loss data have good performance characteristics for estimating prevalence of hearing loss as defined by audiometric criteria, this study indicates that the usefulness of self-report data in identifying individuals with hearing loss may be limited in this population.

Meningococcal ACWY Vaccines - MenACWY and MPSV4: What You Need to Know

MedlinePlus

... disabilities such as hearing loss, brain damage, kidney damage, amputations, nervous system problems, or severe scars from skin grafts. Meningococcal ACWY vaccines can help prevent meningococcal disease caused by serogroups ...

Coping – Late Side Effects

Cancer.gov

Cancer treatment can cause late side effects that may not show up for months or years after treatment. These late effects may include heart and lung problems, bone loss, eye and hearing changes, lymphedema, and other problems

Parental perspectives on adolescent hearing loss risk and prevention.

PubMed

Sekhar, Deepa L; Clark, Sarah J; Davis, Matthew M; Singer, Dianne C; Paul, Ian M

2014-01-01

Data indicate that 1 in 6 adolescents has high-frequency hearing loss, which is typically noise related and preventable. Parental participation improves the success of adolescent behavioral interventions, yet little is known about parental perspectives regarding adolescent noise-induced hearing loss. To perform a survey to determine parental knowledge of adolescent hearing loss and willingness to promote hearing conservation to discern information that is critical to design adolescent hearing loss prevention programs. A cross-sectional, Internet-based survey of a nationally representative online sample of parents of 13- to 17-year-olds. A survey conducted with the C.S. Mott Children's Hospital National Poll on Children's Health, a recurring online survey. Parental knowledge of adolescent hearing loss and willingness to promote hearing conservation. Of 716 eligible respondents, 96.3% of parents reported that their adolescent was slightly or not at all at risk of hearing problems from excessive noise, and 69.0% had not spoken with their adolescent about noise exposure, mainly because of the perceived low risk. Nonetheless, to protect their adolescents' hearing, more than 65.0% of parents are either willing or very willing to consider limiting time listening to music, limiting access to excessively noisy situations, or insisting on the use of hearing protection (earplugs or earmuffs). Higher parental education increased the odds of promoting hearing-protective strategies. Parents were less likely to insist on hearing protection for older adolescents. Parents who understood that both volume and time of exposure affect hearing damage were more likely to have discussed hearing loss with their adolescent (odds ratio [OR], 1.98; 95% CI, 1.29-3.03). The odds of discussing hearing loss were also increased for those who were willing or very willing to limit time listening to music (OR, 1.88; 95% CI, 1.19-2.26) and to insist on hearing protection (OR, 1.92; 95% CI, 1.15-3.18) compared with parents who were very unwilling, unwilling, or neutral. Despite the rising prevalence of acquired adolescent hearing loss, few parents believe their adolescent is at risk. Those with higher education are more willing to promote hearing conservation, especially with younger adolescents. To create effective hearing conservation programs, parents need better education on this subject as well as effective and acceptable strategies to prevent adolescent noise exposure.

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…

Incorporating Mythic and Interpretive Analysis in the Investigation of Hearing Loss on the Family Farm

ERIC Educational Resources Information Center

Meister, Mark; Hest, Theresa; Burnett, Ann

2010-01-01

Despite knowing about the dangers of hearing loss, farmers typically choose not to protect their hearing. Examining the myth of farm life, this study aims to discern whether rhetorical myths influence farmers' decisions to wear hearing protection. Researchers conducted 40 interviews with farmers regarding farm life and hearing loss. Results…

The effects of elevated hearing thresholds on performance in a paintball simulation of individual dismounted combat.

PubMed

Sheffield, Benjamin; Brungart, Douglas; Tufts, Jennifer; Ness, James

2017-01-01

To examine the relationship between hearing acuity and operational performance in simulated dismounted combat. Individuals wearing hearing loss simulation systems competed in a paintball-based exercise where the objective was to be the last player remaining. Four hearing loss profiles were tested in each round (no hearing loss, mild, moderate and severe) and four rounds were played to make up a match. This allowed counterbalancing of simulated hearing loss across participants. Forty-three participants across two data collection sites (Fort Detrick, Maryland and the United States Military Academy, New York). All participants self-reported normal hearing except for two who reported mild hearing loss. Impaired hearing had a greater impact on the offensive capabilities of participants than it did on their "survival", likely due to the tendency for individuals with simulated impairment to adopt a more conservative behavioural strategy than those with normal hearing. These preliminary results provide valuable insights into the impact of impaired hearing on combat effectiveness, with implications for the development of improved auditory fitness-for-duty standards, the establishment of performance requirements for hearing protection technologies, and the refinement of strategies to train military personnel on how to use hearing protection in combat environments.

Reading Intervention to Improve Narrative Production, Narrative Comprehension, and Motivation and Interest of Children with Hearing Loss

ERIC Educational Resources Information Center

Pakulski, Lori A.; Kaderavek, Joan N.

2012-01-01

This study examined the effects of a reading intervention on narrative production, narrative comprehension, and reading motivation interest in children with hearing loss. Seven school children between the ages of 9 and 11 were paired with younger "reading buddies" (without hearing loss). The children with hearing loss read storybooks to…

Temporal Envelope Changes of Compression and Speech Rate: Combined Effects on Recognition for Older Adults

ERIC Educational Resources Information Center

Jenstad, Lorienne M.; Souza, Pamela E.

2007-01-01

Purpose: When understanding speech in complex listening situations, older adults with hearing loss face the double challenge of cochlear hearing loss and deficits of the aging auditory system. Wide-dynamic range compression (WDRC) is used in hearing aids as remediation for the loss of audibility associated with hearing loss. WDRC processing has…

Social Skills in Preschool Children with Unilateral and Mild Bilateral Hearing Loss

ERIC Educational Resources Information Center

Laugen, Nina J.; Jacobsen, Karl H.; Rieffe, Carolien; Wichstrøm, Lars

2017-01-01

Hearing loss may represent a risk for developing social skills difficulties; however, little is known about the potential risk resulting from unilateral or mild bilateral hearing loss (UMHL). We compared the social skills of 14 children with UMHL and 21 children with moderate to severe hearing loss (MSHL) with those of 123 children with typical…

Morphological correlates of hearing loss after cochlear implantation and electro-acoustic stimulation in a hearing-impaired Guinea pig model.

PubMed

Reiss, Lina A J; Stark, Gemaine; Nguyen-Huynh, Anh T; Spear, Kayce A; Zhang, Hongzheng; Tanaka, Chiemi; Li, Hongzhe

2015-09-01

Hybrid or electro-acoustic stimulation (EAS) cochlear implants (CIs) are designed to provide high-frequency electric hearing together with residual low-frequency acoustic hearing. However, 30-50% of EAS CI recipients lose residual hearing after implantation. The objective of this study was to determine the mechanisms of EAS-induced hearing loss in an animal model with high-frequency hearing loss. Guinea pigs were exposed to 24 h of noise (12-24 kHz at 116 dB) to induce a high-frequency hearing loss. After recovery, two groups of animals were implanted (n = 6 per group), with one group receiving chronic acoustic and electric stimulation for 10 weeks, and the other group receiving no stimulation during this time frame. A third group (n = 6) was not implanted, but received chronic acoustic stimulation. Auditory brainstem responses were recorded biweekly to monitor changes in hearing. The organ of Corti was immunolabeled with phalloidin, anti-CtBP2, and anti-GluR2 to quantify hair cells, ribbons and post-synaptic receptors. The lateral wall was immunolabeled with phalloidin and lectin to quantify stria vascularis capillary diameters. Bimodal or trimodal diameter distributions were observed; the number and location of peaks were objectively determined using the Aikake Information Criterion and Expectation Maximization algorithm. Noise exposure led to immediate hearing loss at 16-32 kHz for all groups. Cochlear implantation led to additional hearing loss at 4-8 kHz; this hearing loss was negatively and positively correlated with minimum and maximum peaks of the bimodal or trimodal distributions of stria vascularis capillary diameters, respectively. After chronic stimulation, no significant group changes in thresholds were seen; however, elevated thresholds at 1 kHz in implanted, stimulated animals were significantly correlated with decreased presynaptic ribbon and postsynaptic receptor counts. Inner and outer hair cell counts did not differ between groups and were not correlated with threshold shifts at any frequency. As in the previous study in a normal-hearing model, stria vascularis capillary changes were associated with immediate hearing loss after implantation, while little to no hair cell loss was observed even in cochlear regions with threshold shifts as large as 40-50 dB. These findings again support a role of lateral wall blood flow changes, rather than hair cell loss, in hearing loss after surgical trauma, and implicate the endocochlear potential as a factor in implantation-induced hearing loss. Further, the analysis of the hair cell ribbons and post-synaptic receptors suggest that delayed hearing loss may be linked to synapse or peripheral nerve loss due to stimulation excitotoxicity or inflammation. Further research is needed to separate these potential mechanisms of delayed hearing loss. Copyright © 2015 Elsevier B.V. All rights reserved.

Exposure to low-dose barium by drinking water causes hearing loss in mice.

PubMed

Ohgami, Nobutaka; Hori, Sohjiro; Ohgami, Kyoko; Tamura, Haruka; Tsuzuki, Toyonori; Ohnuma, Shoko; Kato, Masashi

2012-10-01

We continuously ingest barium as a general element by drinking water and foods in our daily life. Exposure to high-dose barium (>100mg/kg/day) has been shown to cause physiological impairments. Direct administration of barium to inner ears by vascular perfusion has been shown to cause physiological impairments in inner ears. However, the toxic influence of oral exposure to low-dose barium on hearing levels has not been clarified in vivo. We analyzed the toxic influence of oral exposure to low-dose barium on hearing levels and inner ears in mice. We orally administered barium at low doses of 0.14 and 1.4 mg/kg/day to wild-type ICR mice by drinking water. The doses are equivalent to and 10-fold higher than the limit level (0.7 mg/l) of WHO health-based guidelines for drinking water, respectively. After 2-week exposure, hearing levels were measured by auditory brain stem responses and inner ears were morphologically analyzed. After 2-month exposure, tissue distribution of barium was measured by inductively coupled plasma mass spectrometry. Low-dose barium in drinking water caused severe hearing loss in mice. Inner ears including inner and outer hair cells, stria vascularis and spiral ganglion neurons showed severe degeneration. The Barium-administered group showed significantly higher levels of barium in inner ears than those in the control group, while barium levels in bone did not show a significant difference between the two groups. Barium levels in other tissues including the cerebrum, cerebellum, heart, liver and kidney were undetectably low in both groups. Our results demonstrate for the first time that low-dose barium administered by drinking water specifically distributes to inner ears resulting in severe ototoxicity with degeneration of inner ears in mice. Copyright © 2012 Elsevier Inc. All rights reserved.

The relationship between loudness intensity functions and the click-ABR wave V latency.

PubMed

Serpanos, Y C; O'Malley, H; Gravel, J S

1997-10-01

To assess the relationship of loudness growth and the click-evoked auditory brain stem response (ABR) wave V latency-intensity function (LIF) in listeners with normal hearing or cochlear hearing loss. The effect of hearing loss configuration on the intensity functions was also examined. Behavioral and electrophysiological intensity functions were obtained using click stimuli of comparable intensities in listeners with normal hearing (Group I; n = 10), and cochlear hearing loss of flat (Group II; n = 10) or sloping (Group III; n = 10) configurations. Individual intensity functions were obtained from measures of loudness growth using the psychophysical methods of absolute magnitude estimation and production of loudness (geometrically averaged to provide the measured loudness function), and from the wave V latency measures of the ABR. Slope analyses for the behavioral and electrophysiological intensity functions were separately performed by group. The loudness growth functions for the groups with cochlear hearing loss approximated the normal function at high intensities, with overall slope values consistent with those reported from previous psychophysical research. The ABR wave V LIF for the group with a flat configuration of cochlear hearing loss approximated the normal function at high intensities, and was displaced parallel to the normal function for the group with sloping configuration. The relationship between the behavioral and electrophysiological intensity functions was examined at individual intensities across the range of the functions for each subject. A significant relationship was obtained between loudness and the ABR wave V LIFs for the groups with normal hearing and flat configuration of cochlear hearing loss; the association was not significant (p = 0.10) for the group with a sloping configuration of cochlear hearing loss. The results of this study established a relationship between loudness and the ABR wave V latency for listeners with normal hearing, and flat cochlear hearing loss. In listeners with a sloping configuration of cochlear hearing loss, the relationship was not significant. This suggests that the click-evoked ABR may be used to estimate loudness growth at least for individuals with normal hearing and those with a flat configuration of cochlear hearing loss. Predictive equations were derived to estimate loudness growth for these groups. The use of frequency-specific stimuli may provide more precise information on the nature of the relationship between loudness growth and the ABR wave V latency, particularly for listeners with sloping configurations of cochlear hearing loss.

Prevalence of Hearing Loss by Severity in the United States.

PubMed

Goman, Adele M; Lin, Frank R

2016-10-01

To estimate the age- and severity-specific prevalence of hearing impairment in the United States. We conducted cross-sectional analyses of 2001 through 2010 data from the National Health and Nutrition Examination Survey on 9648 individuals aged 12 years or older. Hearing loss was defined as mild (> 25 dB through 40 dB), moderate (> 40 dB through 60 dB), severe (> 60 dB through 80 dB), or profound (> 80 dB). An estimated 25.4 million, 10.7 million, 1.8 million, and 0.4 million US residents aged 12 years or older, respectively, have mild, moderate, severe, and profound better-ear hearing loss. Older individuals displayed a higher prevalence of hearing loss and more severe levels of loss. Across most ages, the prevalence was higher among Hispanic and non-Hispanic Whites than among non-Hispanic Blacks and was higher among men than women. Hearing loss directly affects 23% of Americans aged 12 years or older. The majority of these individuals have mild hearing loss; however, moderate loss is more prevalent than mild loss among individuals aged 80 years or older. Our estimates can inform national public health initiatives on hearing loss and help guide policy recommendations currently being discussed at the Institute of Medicine and the White House.

Occupational Hearing Loss among Chinese Municipal Solid Waste Landfill Workers: A Cross-Sectional Study.

PubMed

Liu, Yuewei; Wang, Haijiao; Weng, Shaofan; Su, Wenjin; Wang, Xin; Guo, Yanfei; Yu, Dan; Du, Lili; Zhou, Ting; Chen, Weihong; Shi, Tingming

2015-01-01

Occupational hearing loss is an increasingly prevalent occupational condition worldwide, and has been reported to occur in a wide range of workplaces; however, its prevalence among workers from municipal solid waste landfills (MSWLs) remains less clear. This study aimed to investigate the occupational hearing loss among Chinese MSWL workers. A cross-sectional study of 247 workers from 4 Chinese MSWLs was conducted. Noise and total volatile organic compounds (TVOCs) levels at worksites were determined. We conducted hearing examinations to determine hearing thresholds. A worker was identified as having hearing loss if the mean threshold at 2000, 3000 and 4000 Hz in either ear was equal to or greater than 25 dB. Prevalence of occupational hearing loss was then evaluated. Using unconditional Logistic regression models, we estimated the odds ratios (ORs) of MSWL work associated with hearing loss. According to the job title for each worker, the study subjects were divided into 3 groups, including group 1 of 63 workers without MSWL occupational hazards exposure (control group), group 2 of 84 workers with a few or short-period MSWL occupational hazards exposure, and group 3 of 100 workers with continuous MSWL occupational hazards exposure. Both noise and TVOCs levels were significantly higher at worksites for group 3. Significantly poorer hearing thresholds at frequencies of 2000, 3000 and 4000 Hz were found in group 3, compared with that in group 1 and group 2. The overall prevalence rate of hearing loss was 23.5%, with the highest in group 3 (36.0%). The OR of MSWL work associated with hearing loss was 3.39 (95% confidence interval [CI]: 1.28-8.96). The results of this study suggest significantly higher prevalence of hearing loss among MSWL workers. Further studies are needed to explore possible exposure-response relationship between MSWL occupational hazards exposure and hearing loss.

[Newborn hearing screening program: association between hearing loss and risk factors].

PubMed

Pereira, Priscila Karla Santana; Martins, Adriana de Souza; Vieira, Márcia Ribeiro; Azevedo, Marisa Frasson de

2007-01-01

Hearing loss in newborns. To verify the prevalence of auditory alterations in newborns of Hospital São Paulo (hospital), observing if there are any correlations with the following variables: birth weight, gestational age, relation weight/gestational age and risk factors for hearing loss. A retrospective analysis of the hospital records of 1696 newborns; 648 records of preterm infants and 1048 records of infants born at term. All of the infants had been submitted to an auditory evaluation consisting of: Transient Otoacoustic Emissions, investigation of the cochleal-palpebral reflexes and acoustic imittance tests, identifying the type and level of hearing loss. Sensorineural hearing loss was identified in .82% of the infants who were born at term and in 3.1% of the preterm infants -- with a statistically significant difference. Conductive hearing loss was the most frequent type of hearing loss in both groups, occurring in 14.6% of the term infants and in 16.3% of the preterm infants. Alteration of the central auditory system was considered as a possible diagnosis for 5.8% of the preterm infants and for 3.3% of the term infants. For the group of infants who were born at term, a significant correlation was observed between failure in the hearing screening test and the presence of risk factors such as family history and presence of a syndrome -- the child who presented a syndrome had 37 times more chances of failing in the hearing screening test and seven times more chances of failing in the right ear when there was a family history for hearing loss. The lower the gestational age (< 30 weeks) and birth weight (< 1500 g), the higher the chances of failing in the hearing screening test (3 times more). Hearing loss had a higher occurrence in preterm infants who remained in the ICU. Gestational age and birth weight were important variables related to the possibility of failure in the hearing screening test. A correlation was observed between the presence of a syndrome and sensorineural hearing loss in infants who were born at term.

Timing and Impact of Hearing Healthcare in Adult Cochlear Implant Recipients: A Rural-Urban Comparison

PubMed Central

Hixon, Brian; Chan, Stephen; Adkins, Margaret; Shinn, Jennifer B.; Bush, Matthew L.

2016-01-01

Objective The purpose of this study is to compare the timing and impact of hearing healthcare of rural and urban adults with severe hearing loss who use cochlear implants (CI). Study Design Cross-sectional questionnaire study Setting Tertiary referral center Patients Adult cochlear implant recipients. Main Outcome Measures Data collected included county of residence, socioeconomic information, impact of hearing loss on education/employment, and timing of hearing loss treatment. The benefits obtained from cochlear implantation were also evaluated. Results There were 91 participants (32 from urban counties, 26 from moderately rural counties, and 33 for extremely rural counties). Rural participants have a longer commute time to the CI center (p<0.001), lower income (p<0.001) and higher percentage of Medicaid coverage (p=0.004). Compared with urban-metro participants, rural participants with gradually progressive hearing loss had a greater time interval from the onset of hearing loss to obtaining hearing aid amplification (10 years versus 5 years, p=0.04). There was also a greater time interval from onset of hearing loss to the time of cochlear implantation in rural participants (p=0.04). Reported job loss was higher in rural participants than in urban participants (p=0.05). Both groups reported comparable benefit from cochlear implantation. Conclusions Rural CI recipients differ from urban residents in socioeconomic characteristics and may be delayed in timely treatment of hearing loss. Further efforts to expand access to hearing healthcare services may benefit rural adult patients. PMID:27636389

Timing and Impact of Hearing Healthcare in Adult Cochlear Implant Recipients: A Rural-Urban Comparison.

PubMed

Hixon, Brian; Chan, Stephen; Adkins, Margaret; Shinn, Jennifer B; Bush, Matthew L

2016-10-01

The purpose of this study is to compare the timing and impact of hearing healthcare of rural and urban adults with severe hearing loss who use cochlear implants (CI). Cross-sectional questionnaire study. Tertiary referral center. Adult cochlear implant recipients. Data collected included county of residence, socioeconomic information, impact of hearing loss on education/employment, and timing of hearing loss treatment. The benefits obtained from cochlear implantation were also evaluated. There were 91 participants (32 from urban counties, 26 from moderately rural counties, and 33 for extremely rural counties). Rural participants have a longer commute time to the CI center (p < 0.001), lower income (p < 0.001), and higher percentage of Medicaid coverage (p = 0.004). Compared with urban-metro participants, rural participants with gradually progressive hearing loss had a greater time interval from the onset of hearing loss to obtaining hearing aid amplification (10 yr versus 5 yr, p = 0.04). There was also a greater time interval from onset of hearing loss to the time of cochlear implantation in rural participants (p = 0.04). Reported job loss was higher in rural participants than in urban participants (p = 0.05). Both groups reported comparable benefit from cochlear implantation. Rural CI recipients differ from urban residents in socioeconomic characteristics and may be delayed in timely treatment of hearing loss. Further efforts to expand access to hearing healthcare services may benefit rural adult patients.

Prosody Perception and Production in Children with Hearing Loss and Age- and Gender-Matched Controls.

PubMed

Kalathottukaren, Rose Thomas; Purdy, Suzanne C; Ballard, Elaine

2017-04-01

Auditory development in children with hearing loss, including the perception of prosody, depends on having adequate input from cochlear implants and/or hearing aids. Lack of adequate auditory stimulation can lead to delayed speech and language development. Nevertheless, prosody perception and production in people with hearing loss have received less attention than other aspects of language. The perception of auditory information conveyed through prosody using variations in the pitch, amplitude, and duration of speech is not usually evaluated clinically. This study (1) compared prosody perception and production abilities in children with hearing loss and children with normal hearing; and (2) investigated the effect of age, hearing level, and musicality on prosody perception. Participants were 16 children with hearing loss and 16 typically developing controls matched for age and gender. Fifteen of the children with hearing loss were tested while using amplification (n = 9 hearing aids, n = 6 cochlear implants). Six receptive subtests of the Profiling Elements of Prosody in Speech-Communication (PEPS-C), the Child Paralanguage subtest of Diagnostic Analysis of Nonverbal Accuracy 2 (DANVA 2), and Contour and Interval subtests of the Montreal Battery of Evaluation of Amusia (MBEA) were used. Audio recordings of the children's reading samples were rated using a perceptual prosody rating scale by nine experienced listeners who were blinded to the children's hearing status. Thirty two children, 16 with hearing loss (mean age = 8.71 yr) and 16 age- and gender-matched typically developing children with normal hearing (mean age = 8.87 yr). Assessments were completed in one session lasting 1-2 hours in a quiet room. Test items were presented using a laptop computer through loudspeaker at a comfortable listening level. For children with hearing loss using hearing instruments, all tests were completed with hearing devices set at their everyday listening setting. All PEPS-C subtests and total scores were significantly lower for children with hearing loss compared to controls (p < 0.05). The hearing loss group performed more poorly than the control group in recognizing happy, sad, and fearful emotions in the DANVA 2 subtest. Musicality (composite MBEA scores and musical experience) was significantly correlated with prosody perception scores, but this link was not evident in the regression analyses. Regression modeling showed that age and hearing level (better ear pure-tone average) accounted for 55.4% and 56.7% of the variance in PEPS-C and DANVA 2 total scores, respectively. There was greater variability for the ratings of pitch, pitch variation, and overall impression of prosody in the hearing loss group compared to control group. Prosody perception (PEPS-C and DANVA 2 total scores) and ratings of prosody production were not correlated. Children with hearing loss aged 7-12 yr had significant difficulties in understanding different aspects of prosody and were rated as having more atypical prosody overall than controls. These findings suggest that clinical assessment and speech-language therapy services for children with hearing loss should be expanded to target prosodic difficulties. Future studies should investigate whether musical training is beneficial for improving receptive prosody skills. American Academy of Audiology

Menopause and postmenopausal hormone therapy and risk of hearing loss.

PubMed

Curhan, Sharon G; Eliassen, A Heather; Eavey, Roland D; Wang, Molin; Lin, Brian M; Curhan, Gary C

2017-09-01

Menopause may be a risk factor for hearing loss, and postmenopausal hormone therapy (HT) has been proposed to slow hearing decline; however, there are no large prospective studies. We prospectively examined the independent relations between menopause and postmenopausal HT and risk of self-reported hearing loss. Prospective cohort study among 80,972 women in the Nurses' Health Study II, baseline age 27 to 44 years, followed from 1991 to 2013. Baseline and updated information was obtained from detailed validated biennial questionnaires. Cox proportional-hazards regression models were used to examine independent associations between menopausal status and postmenopausal HT and risk of hearing loss. After 1,410,928 person-years of follow-up, 18,558 cases of hearing loss were reported. There was no significant overall association between menopausal status, natural or surgical, and risk of hearing loss. Older age at natural menopause was associated with higher risk. The multivariable-adjusted relative risk of hearing loss among women who underwent natural menopause at age 50+ years compared with those aged less than 50 years was 1.10 (95% confidence interval [CI] 1.03, 1.17). Among postmenopausal women, oral HT (estrogen therapy or estrogen plus progestogen therapy) was associated with higher risk of hearing loss, and longer duration of use was associated with higher risk (P trend < 0.001). Compared with women who never used HT, the multivariable-adjusted relative risk of hearing loss among women who used oral HT for 5 to 9.9 years was 1.15 (95% CI 1.06, 1.24) and for 10+ years was 1.21 (95% CI 1.07, 1.37). Older age at menopause and longer duration of postmenopausal HT are associated with higher risk of hearing loss.

Associations between cardiovascular disease and its risk factors with hearing loss-A cross-sectional analysis.

PubMed

Tan, H E; Lan, N S R; Knuiman, M W; Divitini, M L; Swanepoel, D W; Hunter, M; Brennan-Jones, C G; Hung, J; Eikelboom, R H; Santa Maria, P L

2018-02-01

To investigate the relationship between hearing loss and cardiovascular disease risk factors. Cross-sectional study. Participants were recruited between May 2010 and December 2015 and answered a health and risk factor questionnaire. Physical and biochemical assessments were performed. A community-based population. A total of 5107 participants born within the years 1946-1964 enrolled in the Busselton Healthy Ageing Study. Hearing was assessed behaviourally through the best ear pure-tone average (500, 1000, 2000, 4000 Hz), low-frequency average (250, 500, 1000 Hz) and high-frequency average (4000, 8000 Hz). Self-reported hearing loss, tinnitus and hyperacusis were assessed via questionnaire. Cardiovascular risk factors were assessed via a patient-completed questionnaire and objective measurements including blood pressure, body mass index, waist circumference, lipid profile and glycated haemoglobin. Of the participants, 54% were female, with the mean age of 58 years (range 45-69 years). Age, sex and family history of hearing loss were consistently strong determinants of hearing loss outcomes. After adjusting for these, obesity, current smoking, peripheral arterial disease and history of cardiovascular disease were significantly associated with pure-tone, low-frequency and high-frequency hearing loss. In addition, high blood pressure, triglyceride and glycated haemoglobin were significantly associated with low-frequency hearing loss. There was a graded association between hearing loss and Framingham Risk Score for cardiovascular risk (P<0.001). Established cardiovascular disease and individual and combined cardiovascular disease risk factors were found to be associated with hearing loss. Future research should prospectively investigate whether targeting cardiovascular disease can prevent hearing loss. © 2017 John Wiley & Sons Ltd.

Platinum-Induced Ototoxicity in Children: A Consensus Review on Mechanisms, Predisposition, and Protection, Including a New International Society of Pediatric Oncology Boston Ototoxicity Scale

PubMed Central

Brock, Penelope R.; Knight, Kristin R.; Freyer, David R.; Campbell, Kathleen C.M.; Steyger, Peter S.; Blakley, Brian W.; Rassekh, Shahrad R.; Chang, Kay W.; Fligor, Brian J.; Rajput, Kaukab; Sullivan, Michael; Neuwelt, Edward A.

2012-01-01

Purpose The platinum chemotherapy agents cisplatin and carboplatin are widely used in the treatment of adult and pediatric cancers. Cisplatin causes hearing loss in at least 60% of pediatric patients. Reducing cisplatin and high-dose carboplatin ototoxicity without reducing efficacy is important. Patients and Methods This review summarizes recommendations made at the 42nd Congress of the International Society of Pediatric Oncology (SIOP) in Boston, October 21-24, 2010, reflecting input from international basic scientists, pediatric oncologists, otolaryngologists, oncology nurses, audiologists, and neurosurgeons to develop and advance research and clinical trials for otoprotection. Results Platinum initially impairs hearing in the high frequencies and progresses to lower frequencies with increasing cumulative dose. Genes involved in drug transport, metabolism, and DNA repair regulate platinum toxicities. Otoprotection can be achieved by acting on several these pathways and generally involves antioxidant thiol agents. Otoprotection is a strategy being explored to decrease hearing loss while maintaining dose intensity or allowing dose escalation, but it has the potential to interfere with tumoricidal effects. Route of administration and optimal timing relative to platinum therapy are critical issues. In addition, international standards for grading and comparing ototoxicity are essential to the success of prospective pediatric trials aimed at reducing platinum-induced hearing loss. Conclusion Collaborative prospective basic and clinical trial research is needed to reduce the incidence of irreversible platinum-induced hearing loss, and optimize cancer control. Wide use of the new internationally agreed-on SIOP Boston ototoxicity scale in current and future otoprotection trials should help facilitate this goal. PMID:22547603

Genetics Evaluation Guidelines for the Etiologic Diagnosis of Congenital Hearing Loss

PubMed Central

2002-01-01

The advent of hearing screening in newborns in many states has led to an increase in the use of genetic testing and related genetic services in the follow-up of infants with hearing loss. A significant proportion of those with congenital hearing loss have genetic etiologies underlying their hearing loss. To ensure that those identified with congenital hearing loss receive the genetic services appropriate to their conditions, the Maternal and Child Health Bureau of the Health Resources and Services Administration funded the American College of Medical Genetics to convene an expert panel to develop guidelines for the genetic evaluation of congential hearing loss. After a brief overview of the current knowledge of hearing loss, newborn screening, and newborn hearing screening, we provide an overview of genetic services and a guideline that describes how best to ensure that patients receive appropriate genetic services. The significant contribution of genetic factors to these conditions combined with the rapid evolution of knowledge about the genetics of these conditions overlaid with the inherently multidisciplinary nature of genetic services provides an example of a condition for which a well-integrated multidisciplinary approach to care is clearly needed. PMID:12180152

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

PubMed

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

2015-02-01

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

Estrogen and hearing from a clinical point of view; characteristics of auditory function in women with Turner syndrome.

PubMed

Hederstierna, Christina; Hultcrantz, Malou; Rosenhall, Ulf

2009-06-01

Turner syndrome is a chromosomal aberration affecting 1:2000 newborn girls, in which all or part of one X chromosome is absent. This leads to ovarial dysgenesis and little or no endogenous estrogen production. These women have, among many other syndromal features, a high occurrence of ear and hearing problems, and neurocognitive dysfunctions, including reduced visual-spatial abilities; it is assumed that estrogen deficiency is at least partially responsible for these problems. In this, study 30 Turner women aged 40-67, with mild to moderate hearing loss, performed a battery of hearing tests aimed at localizing the lesion causing the sensorineural hearing impairment and assessing central auditory function, primarily sound localization. The results of TEOAE, ABR and speech recognition scores in noise were all indicative of cochlear dysfunction as the cause of the sensorineural impairment. Phase audiometry, a test for sound localization, showed mild disturbances in the Turner women compared to the reference group, suggesting that auditory-spatial dysfunction is another facet of the recognized neurocognitive phenotype in Turner women.

The standpoint of persons with hearing loss on work disparities and workplace accommodations.

PubMed

Shaw, Lynn; Tetlaff, Britta; Jennings, Mary Beth; Southall, Kenneth E

2013-01-01

The perspectives of persons who live and work with hearing loss were sought to examine workplace accommodation challenges and strategies. A convenience sample of seven older adults with hearing loss participated in in-depth interviews. A systematic grounded theory approach informed the study design and analysis. Categories of facilitators and challenges in the data were identified through axial coding and clustering. Core categories of social processes emerged through constant comparison and theoretical sampling of the data to reveal the actions and interactions used to negotiate or implement adaptations or workplace accommodations. Persons with hearing loss use a realm of strategies to live and work with a hearing loss. Social processes used to navigate the challenges to working with hearing loss and to manage optimal work performance included: self-accommodation, self-advocacy, self-management of hearing loss, and lobbying. Success in overcoming work disparities for persons with hearing loss requires individuals to take control of identifying their needs within the workplace and at home, and to negotiate for specific accommodations. These strategies and processes draw attention to the need for a repository on contextualized workplace accommodation strategies for improving communication and hearing in the workplace. Further to this a best practice guide for use by workers, employers, and work rehabilitation and health care workers is indicated.

Inner ear involvement in Fabry disease: Clinical and audiometric evaluation of a large cohort of patients followed in a reference centre.

PubMed

Rodrigues, Jorge; Azevedo, Olga; Sousa, Nuno; Cunha, Damião; Mexedo, Alexandre; Fonseca, Rui

2018-06-01

Fabry disease (FD) is a lysosomal storage disorder (LSD) that involves the cochleovestibular system. Tinnitus and progressive sensorineural hearing loss are frequent complains. A stabilization of hearing function has been reported with enzyme replacement therapy (ERT). This study aims to characterize the inner ear involvement, identify factors associated to hearing loss and evaluate the effect of ERT on the hearing function of FD patients. We reviewed the clinical records of patients with confirmed diagnosis of FD followed in a Reference Centre on LSD in the North of Portugal. We included a total of 122 patients with a mean age of 47.1 ± 17.6 years and 48.3% males. Hearing loss was reported by 26.2% of the patients and 23.0% mentioned tinnitus. Pure tone audiometry revealed sensorineural hearing loss in 36.9% of the cases. FD patients presented worse age-adjusted hearing thresholds in all analysed frequencies compared to the normal population (p = .001). Patients with hearing loss presented a significantly higher value of microalbuminuria (p = .001) and a higher frequency of acroparesthesias (p = .032). Patients presented a comparable hearing level one year after starting ERT (p = .384). In FD, hearing loss is common and age-matched hearing thresholds by frequency are worse than in the general population. Hearing loss was associated to the presence of acroparesthesias and higher values of microalbuminuria. Hearing loss stabilized in patients under ERT. A careful cochleo-vestibular evaluation should be part of the clinical assessment of FD. Copyright © 2018 Elsevier Masson SAS. All rights reserved.

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.

Quantitative study of the correlation between cerebellar retraction factors and hearing loss following microvascular decompression for hemifacial spasm.

PubMed

Li, Ning; Zhao, Wei-Guo; Pu, Chun-Hua; Yang, Wen-Lei

2018-01-01

This prospective study quantitatively measured the cerebellar retraction factors, including retraction distance, depth and duration, and evaluated their potential relationship to the development of hearing loss after microvascular decompression (MVD) for hemifacial spasm (HFS). One hundred ten patients with primary HFS who underwent MVD in our department were included into this study. The cerebellar retraction factors were quantitatively measured on preoperative MR and timed during MVD. Associations of cerebellar retraction and other factors to postoperative hearing loss were analyzed. Eleven (10%) patients developed hearing loss after MVD. Compared with the group without hearing loss, the cerebellar retraction distance, depth and duration of the group with hearing loss were significantly greater (p < 0.05). Multivariate regression analysis showed that greater cerebellar retraction depth and longer retraction duration were significantly associated with a higher incidence of postoperative hearing impairment (p < 0.05). This study strongly suggested a correlation between the cerebellar retraction factors, especially retraction depth and duration, and possibility of hearing loss following MVD for HFS.

Heterozygous SSBP1 start loss mutation co-segregates with hearing loss and the m.1555A>G mtDNA variant in a large multigenerational family.

PubMed

Kullar, Peter J; Gomez-Duran, Aurora; Gammage, Payam A; Garone, Caterina; Minczuk, Michal; Golder, Zoe; Wilson, Janet; Montoya, Julio; Häkli, Sanna; Kärppä, Mikko; Horvath, Rita; Majamaa, Kari; Chinnery, Patrick F

2018-01-01

The m.1555A>G mtDNA variant causes maternally inherited deafness, but the reasons for the highly variable clinical penetrance are not known. Exome sequencing identified a heterozygous start loss mutation in SSBP1, encoding the single stranded binding protein 1 (SSBP1), segregating with hearing loss in a multi-generational family transmitting m.1555A>G, associated with mtDNA depletion and multiple deletions in skeletal muscle. The SSBP1 mutation reduced steady state SSBP1 levels leading to a perturbation of mtDNA metabolism, likely compounding the intra-mitochondrial translation defect due to m.1555A>G in a tissue-specific manner. This family demonstrates the importance of rare trans-acting genetic nuclear modifiers in the clinical expression of mtDNA disease. © The Author (2017). Published by Oxford University Press on behalf of the Guarantors of Brain.

Effects of Noise on Speech Recognition and Listening Effort in Children with Normal Hearing and Children with Mild Bilateral or Unilateral Hearing Loss

ERIC Educational Resources Information Center

Lewis, Dawna; Schmid, Kendra; O'Leary, Samantha; Spalding, Jody; Heinrichs-Graham, Elizabeth; High, Robin

2016-01-01

Purpose: This study examined the effects of stimulus type and hearing status on speech recognition and listening effort in children with normal hearing (NH) and children with mild bilateral hearing loss (MBHL) or unilateral hearing loss (UHL). Method Children (5-12 years of age) with NH (Experiment 1) and children (8-12 years of age) with MBHL,…

Smartphone serious games for vision and hearing assessment.

PubMed

Dias, Pedro; Aguiar, Bruno; Sousa, Filipe; Sousa, Augusto

2015-01-01

Falls are the second leading cause of accidental injury deaths worldwide. In this paper, it is intended to define methodologies that permit the evaluation of two potential factors which might have an impact on fall risk, these are: visual and hearing loss. The aim of the work developed is not to replace clinic visits, but to offer the user the means to continue the tracking of his vision and hearing at home, during the long time intervals between clinical tests. Tests conducted in a sample of our target users indicate a good ability to measure vision and hearing using an android smartphone and the proposed methodologies. While some tests require further validation, promising results were achieved in the most common tests for vision and hearing, presenting a good correlation between the system's results when compared to the traditional tests (for distance visual acuity) and the data gathered from the users (for hearing tests).

[Working with hearing impairment: an integrated approach].

PubMed

van Til, Marten J; Kramer, Sophia E; Anema, Johannes R; Goverts, S T Theo

2016-01-01

Patients with hearing impairment are more likely to encounter health problems and difficulties at work than their colleagues with normal hearing. This is often not realised by either patients or professionals. In this article we describe three cases that illustrate how working conditions can influence the health of workers with hearing loss. We have implemented a vocational enablement protocol that follows a multidisciplinary approach in order to meet these patients' needs. Due to a mismatch between the demands of a job and an individual's auditory capacities, even a mild hearing impairment can cause serious problems if a patient works in adverse conditions. In addition, in many workplaces the ability to hear well is a safety issue. Professionals have to be aware of both possibilities. Specialized centres offer good facilities and ensure the optimal transfer of insight into the working environment by involving an occupational physician in their team.

Otologic manifestations of Fanconi anemia and other inherited bone marrow failure syndromes.

PubMed

Kalejaiye, Adedoyin; Giri, Neelam; Brewer, Carmen C; Zalewski, Christopher K; King, Kelly A; Adams, Charleen D; Rosenberg, Philip S; Kim, H Jeffrey; Alter, Blanche P

2016-12-01

The inherited bone marrow failure syndromes (IBMFSs) are diverse disorders with syndrome-specific features; their otologic and audiologic manifestations have not been well described. Our objective was to characterize these in patients with Fanconi anemia (FA), dyskeratosis congenita (DC), Diamond-Blackfan anemia (DBA), and Shwachman-Diamond syndrome (SDS), and to determine the association between physical findings and hearing loss. Patients with an IBMFS underwent comprehensive clinical and laboratory evaluations and testing for syndrome-specific gene mutations. Hearing loss was measured by pure tone audiometry and otologic abnormalities by otomicroscopy. Patients included 33 with FA, 37 with DC, 32 with DBA, and nine with SDS. Hearing loss was most frequent in patients with FA (45%) and DBA (14%). The most common type of hearing loss in FA was conductive (65%). Absent or hypoplastic radius, noted in 21% of the patients with FA, was associated with hearing loss in all cases. Otomicroscopy was abnormal in 66% of patients with FA. Characteristic ear abnormalities included small tympanic membrane (66%), malformed malleus (57%), aberrant tympanic bony island (48%), narrow external auditory canal (EAC) (32%), and abnormal course of chorda tympani (34%). Ear malformations were almost always associated with hearing loss. Hearing loss was rare in patients with DC and SDS. FA is the major IBMFS with associated hearing loss, which is most commonly conductive. Radial hypoplasia or aplasia and characteristic congenital ear malformations are associated with hearing loss in patients with FA. Recognition of these syndrome-specific abnormalities should lead to earlier management of hearing loss. © 2016 Wiley Periodicals, Inc.

Lack of ear care knowledge in nursing homes.

PubMed

Solheim, Jorunn; Shiryaeva, Olga; Kvaerner, Kari J

2016-01-01

Rising life expectancy means an increase in the number of elderly people with hearing loss in the population. Many elderly people live in nursing homes, with varying care needs. A substantial proportion of these people will need help with their hearing aids and other hearing devices. The objective of the study has been to assess the knowledge, experience, skills, competence, and need for information of staff at nursing homes in relation to residents' hearing loss and hearing aids. One hundred and ninety-five employees at seven nursing homes participated in the study. The main approach was a descriptive study, using questionnaires. The main findings are that 73% of informants found that many residents need help with their hearing aids. Only one-tenth report that they know enough about the residents' hearing aids. Almost four out of five informants find that the residents become socially isolated as a result of hearing loss. Seventy-eight percent agree to some extent that more residents would benefit from hearing aids. Staff at nursing homes have insufficient knowledge about hearing loss and hearing aids. Increased focus on the elderly with hearing impairment in nursing homes is needed. Contact between nursing homes and audiological specialists should be improved to best followup hearing loss and hearing aids.

Hearing status in patients with rheumatoid arthritis.

PubMed

Ahmadzadeh, A; Daraei, M; Jalessi, M; Peyvandi, A A; Amini, E; Ranjbar, L A; Daneshi, A

2017-10-01

Rheumatoid arthritis is thought to induce conductive hearing loss and/or sensorineural hearing loss. This study evaluated the function of the middle ear and cochlea, and the related factors. Pure tone audiometry, speech reception thresholds, speech discrimination scores, tympanometry, acoustic reflexes, and distortion product otoacoustic emissions were assessed in rheumatoid arthritis patients and healthy volunteers. Pure tone audiometry results revealed a higher bone conduction threshold in the rheumatoid arthritis group, but there was no significant difference when evaluated according to the sensorineural hearing loss definition. Distortion product otoacoustic emissions related prevalence of conductive or mixed hearing loss, tympanometry values, acoustic reflexes, and speech discrimination scores were not significantly different between the two groups. Sensorineural hearing loss was significantly more prevalent in patients who used azathioprine, cyclosporine and etanercept. Higher bone conduction thresholds in some frequencies were detected in rheumatoid arthritis patients that were not clinically significant. Sensorineural hearing loss is significantly more prevalent in refractory rheumatoid arthritis patients.

An Introduction to the Outcomes of Children with Hearing Loss Study

PubMed Central

Moeller, Mary Pat; Tomblin, J. Bruce

2015-01-01

The landscape of service provision for young children with hearing loss has shifted in recent years as a result of newborn hearing screening and the early provision of interventions, including hearing technologies. It is expected that early service provision will minimize or prevent linguistic delays that typically accompany untreated permanent childhood hearing loss. The post-newborn hearing screening era has seen a resurgence of interest in empirically examining the outcomes of children with hearing loss to determine if service innovations have resulted in expected improvements in children’s functioning. The Outcomes of Children with Hearing Loss (OCHL) project was among these recent research efforts, and this introductory article provides background in the form of literature review and theoretical discussion to support the goals of the study. The OCHL project was designed to examine the language and auditory outcomes of infants and preschool-aged children with permanent, bilateral, mild-to-severe hearing loss and to identify factors that moderate the relationship between hearing loss and longitudinal outcomes. We propose that children who are hard of hearing experience limitations in access to linguistic input, which lead to a decrease in uptake of language exposure and an overall reduction in linguistic experience. We explore this hypothesis in relation to three primary factors that are proposed to influence children’s access to linguistic input: aided audibility, duration and consistency of hearing aid (HA) use, and characteristics of caregiver input. PMID:26731159

Early Detection of Hearing Loss: The Case for Listening to Mothers

ERIC Educational Resources Information Center

Marchbank, Alison Margaret

2011-01-01

This article is drawn from a larger doctoral study that explored hearing mothers' experiences of discovering that their babies had a permanent hearing loss in Australia in 2008. The particular focus for this paper is the period in time after a concern is flagged, either by a newborn hearing screener or the mother herself, until a hearing loss is…

Physical Education and Sport Adaptations for Students Who Are Hard of Hearing

ERIC Educational Resources Information Center

Reich, Lori M.; Lavay, Barry

2009-01-01

Hearing loss is the number one disability in the United States. It cannot be assumed, however, that all people with hearing loss have similar needs. Most individuals with hearing loss do not use sign language, and people who are hard of hearing (HOH) are often grouped together with people who are Deaf and referred to as "deaf" or "hearing…

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

PubMed

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

2015-01-01

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

The Stigma of Hearing Loss

PubMed Central

Wallhagen, Margaret I.

2010-01-01

Purpose: To explore dimensions of stigma experienced by older adults with hearing loss and those with whom they frequently communicate to target interventions promoting engagement and positive aging. Design and Methods: This longitudinal qualitative study conducted interviews over 1 year with dyads where one partner had hearing loss. Participants were naive to or had not worn hearing aids in the past year. Data were analyzed using grounded theory, constant comparative methodology. Results: Perceived stigma emerged as influencing decision-making processes at multiple points along the experiential continuum of hearing loss, such as initial acceptance of hearing loss, whether to be tested, type of hearing aid selected, and when and where hearing aids were worn. Stigma was related to 3 interrelated experiences, alterations in self-perception, ageism, and vanity and was influenced by dyadic relationships and external societal forces, such as health and hearing professionals and media. Implications: Findings are discussed in relation to theoretical perspectives regarding stigma and ageism and suggest the need to destigmatize hearing loss by promoting its assessment and treatment as well as emphasizing the importance of remaining actively engaged to support positive physical and cognitive functioning. PMID:19592638

Adjusting to Hearing Loss during High School: Preparing Students for Successful Transition to Postsecondary Education or Training. Tipsheet

ERIC Educational Resources Information Center

Brooks, Dianne

2009-01-01

Completion of postsecondary education frequently builds upon a student's successful academic and personal experience during high school. For students with hearing loss, healthy adjustment to hearing loss is a key lifelong developmental process. The vast majority (94%) of approximately 1.1 million K-12 students with hearing loss are educated in…

Effects of Anxiety Sensitivity and Hearing Loss on Tinnitus Symptom Severity

PubMed Central

Moon, Kyung Ray; Park, Subin; Jung, YouJi; Lee, AhReum

2018-01-01

Objective The aim of the present study was to examine the relative role of anxiety sensitivity and hearing loss on the tinnitus symptoms severity in a large clinical sample of patients with tinnitus. Methods A total of 1,705 patients with tinnitus who visited the tinnitus clinic underwent the pure-tone audiometric testing and a battery of self-report questionnaires. Multiple linear regression analyses were performed to identify the relationship of anxiety sensitivity and hearing loss to tinnitus symptoms severity. Results Both anxiety sensitivity and hearing loss were a significant association with of annoyance (anxiety sensitivity β=0.11, p=0.010; hearing loss β=0.09, p=0.005) and THI score (anxiety sensitivity β=0.21, p<0.001; hearing loss β=0.10, p<0.001) after adjusting for confounding factors. Meanwhile, the awareness time (β=0.19, p<0.001) and loudness (β=0.11, p<0.001) of tinnitus was associated with only the hearing loss but not with anxiety sensitivity. Conclusion Our results indicate that both hearing loss and anxiety sensitivity were associated with increased tinnitus symptom severity. Furthermore, these associations could be different according to the characteristics of tinnitus symptoms. PMID:29422923

Auditory-Verbal Music Play Therapy: An Integrated Approach (AVMPT).

PubMed

Mohammad Esmaeilzadeh, Sahar; Sharifi, Shahla; Tayarani Niknezhad, Hamid

2013-09-01

Hearing loss occurs when there is a problem with one or more parts of the ear or ears and causes children to have a delay in the language-learning process. Hearing loss affects children's lives and their development. Several approaches have been developed over recent decades to help hearing-impaired children develop language skills. Auditory-verbal therapy (AVT) is one such approach. Recently, researchers have found that music and play have a considerable effect on the communication skills of children, leading to the development of music therapy (MT) and play therapy (PT). There have been several studies which focus on the impact of music on hearing-impaired children. The aim of this article is to review studies conducted in AVT, MT, and PT and their efficacy in hearing-impaired children. Furthermore, the authors aim to introduce an integrated approach of AVT, MT, and PT which facilitates language and communication skills in hearing-impaired children. In this article we review studies of AVT, MT, and PT and their impact on hearing-impaired children. To achieve this goal, we searched databases and journals including Elsevier, Chor Teach, and Military Psychology, for example. We also used reliable websites such as American Choral Directors Association and Joint Committee on Infant Hearing websites. The websites were reviewed and key words in this article used to find appropriate references. Those articles which are related to ours in content were selected. VT, MT, and PT enhance children's communication and language skills from an early age. Each method has a meaningful impact on hearing loss, so by integrating them we have a comprehensive method in order to facilitate communication and language learning. To achieve this goal, the article offers methods and techniques to perform AVT and MT integrated with PT leading to an approach which offers all advantages of these three types of therapy.

Auditory-Verbal Music Play Therapy: An Integrated Approach (AVMPT)

PubMed Central

Mohammad Esmaeilzadeh, Sahar; Sharifi, Shahla; Tayarani Niknezhad, Hamid

2013-01-01

Introduction: Hearing loss occurs when there is a problem with one or more parts of the ear or ears and causes children to have a delay in the language-learning process. Hearing loss affects children's lives and their development. Several approaches have been developed over recent decades to help hearing-impaired children develop language skills. Auditory-verbal therapy (AVT) is one such approach. Recently, researchers have found that music and play have a considerable effect on the communication skills of children, leading to the development of music therapy (MT) and play therapy (PT). There have been several studies which focus on the impact of music on hearing-impaired children. The aim of this article is to review studies conducted in AVT, MT, and PT and their efficacy in hearing-impaired children. Furthermore, the authors aim to introduce an integrated approach of AVT, MT, and PT which facilitates language and communication skills in hearing-impaired children. Materials and Methods: In this article we review studies of AVT, MT, and PT and their impact on hearing-impaired children. To achieve this goal, we searched databases and journals including Elsevier, Chor Teach, and Military Psychology, for example. We also used reliable websites such as American Choral Directors Association and Joint Committee on Infant Hearing websites. The websites were reviewed and key words in this article used to find appropriate references. Those articles which are related to ours in content were selected. Conclusion: VT, MT, and PT enhance children’s communication and language skills from an early age. Each method has a meaningful impact on hearing loss, so by integrating them we have a comprehensive method in order to facilitate communication and language learning. To achieve this goal, the article offers methods and techniques to perform AVT and MT integrated with PT leading to an approach which offers all advantages of these three types of therapy. PMID:24303441

Sudden sensorineural hearing loss with positional vertigo: Initial findings of positional nystagmus and hearing outcomes.

PubMed

Kim, Chang-Hee; Shin, Jung Eun; Yang, Young Soo; Im, Donghyuk

2016-10-01

To investigate the initial findings of positional nystagmus in patients with sudden sensorineural hearing loss (SSNHL) and positional vertigo, and to compare hearing improvement among patients with different types of positional nystagmus. The characteristics of positional nystagmus upon initial examination were analysed, and the initial mean pure-tone audiometry (PTA) threshold was compared with that at three months after treatment. Forty-four SSNHL patients with concomitant positional vertigo were included. Positional nystagmus was classified into five subgroups; persistent geotropic direction-changing positional nystagmus (DCPN) in head-roll test (HRT) and negative Dix-Hallpike test (DHT), persistent apogeotropic DCPN in HRT and negative DHT, positive DHT and negative HRT, persistent geotropic DCPN in HRT and positive DHT, and persistent apogeotropic DCPN in HRT and positive DHT. PTA threshold improvement was significantly greater in SSNHL patients with negative DHT than with positive DHT (p = 0.027). When geotropic DCPN was elicited by HRT, the nystagmus was persistent, which suggests that alteration of specific gravity of the endolymph, rather than the lateral canal canalolithiasis, may be a cause of this characteristic positional nystagmus. Positive DTH may be a prognostic factor for worse hearing recovery among patients with SSNHL and positional vertigo.

Serogroup B Meningococcal vaccine (MenB) - What you need to know

MedlinePlus

... disabilities such as hearing loss, brain damage, kidney damage, amputations, nervous system problems, or severe scars from skin grafts. Serogroup B meningococcal (MenB) vaccines can help prevent meningococcal disease caused by serogroup ...

Borate transporter SLC4A11 mutations cause both Harboyan syndrome and non‐syndromic corneal endothelial dystrophy

PubMed Central

Desir, Julie; Moya, Graciela; Reish, Orit; Van Regemorter, Nicole; Deconinck, Hilde; David, Karen L; Meire, Françoise M; Abramowicz, Marc J

2007-01-01

Harboyan syndrome, or corneal dystrophy and perceptive deafness (CDPD), consists of congenital corneal endothelial dystrophy and progressive perceptive deafness, and is transmitted as an autosomal recessive trait. CDPD and autosomal recessive, non‐syndromic congenital hereditary endothelial corneal dystrophy (CHED2) both map at overlapping loci at 20p13, and mutations of SLC4A11 were reported recently in CHED2. A genotype study on six families with CDPD and on one family with either CHED or CDPD, from various ethnic backgrounds (in the seventh family, hearing loss could not be assessed because of the proband's young age), is reported here. Novel SLC4A11 mutations were found in all patients. Why some mutations cause hearing loss in addition to corneal dystrophy is presently unclear. These findings extend the implication of the SLC4A11 borate transporter beyond corneal dystrophy to perceptive deafness. PMID:17220209