[Application of MALDI-TOF-MS in gene testing for non-syndromic hearing loss].

PubMed

Zeng, Yun; Jiang, Dan; Feng, Da-fei; Jin, Dong-dong; Wu, Xiao-hui; Ding, Yan-li; Zou, Jing

2013-12-01

To investigate the feasibility of Matrix-Assisted Laser Desorption-Ionization Time of Flight Mass Spectrometry (MALDI-TOF-MS) , according to the genetic test of non-syndromic hearing loss (NSHL), and check using the direct sequencing. Peripheral blood was collected from 454 NSHL patients. DNA samples were extracted and 20 loci of the four common disease-causing genes were analysed by MALDI-TOF-MS, including GJB2 (35delG, 167delT, 176_191del16, 235delC, 299_300delAT ), GJB3 (538C→T, 547G→A), SLC26A4 (281C→T, 589G→A, IVS7-2A→G, 1174A→T, 1226G→A, 1229C→T, IVS15+5G→A, 1975G→C, 2027T→A, 2162C→T, 2168A→G), and mitochondrial 12S rRNA (1494C→T, 1555A→G). Direct sequencing was also used to analyse the aforementioned 20 loci in order to validate the accuracy of MALDI-TOF-MS. Among the 454 patients, 166 cases (36.56%) of disease-causing mutations were detected, which included 69 cases (21.15%) of GJB2 gene mutation, four cases (0.88%) of GJB3 gene mutation, 64 cases (14.10%) of SLC26A4 gene mutation, and three cases (0.66%) of mitochondrial 12S rRNA gene mutation. Moreover, the results obtained from direct sequencing and MALDI-TOF-MS were consistent, and the results showed that the two methods were consistent. The MALDI-TOF-MS detection method was designed based on the hearing loss-related mutation hotspots seen in the Chinese population, and it has a high detection rate for NSHL related mutations. In comparison to the conventional detection methods, MALDI-TOF-MS has the following advantages: more detection sites, greater coverage, accurate, high throughput and low cost. Therefore, this method is capable of satisfying the needs of clinical detection for hearing impairment and it is suitable for large-scale implementation.

Cochlear hearing loss in patients with Laron syndrome.

PubMed

Attias, Joseph; Zarchi, Omer; Nageris, Ben I; Laron, Zvi

2012-02-01

The aim of this prospective clinical study was to test auditory function in patients with Laron syndrome, either untreated or treated with insulin-like growth factor I (IGF-I). The study group consisted of 11 patients with Laron syndrome: 5 untreated adults, 5 children and young adults treated with replacement IGF-I starting at bone age <2 years, and 1 adolescent who started replacement therapy at bone age 4.6 years. The auditory evaluation included pure tone and speech audiometry, tympanometry and acoustic reflexes, otoacoustic emissions, loudness dynamics, auditory brain stem responses and a hyperacusis questionnaire. All untreated patients and the patient who started treatment late had various degrees of sensorineural hearing loss and auditory hypersensitivity; acoustic middle ear reflexes were absent in most of them. All treated children had normal hearing and no auditory hypersensitivity; most had recordable middle ear acoustic reflexes. In conclusion, auditory defects seem to be associated with Laron syndrome and may be prevented by starting treatment with IGF-I at an early developmental age.

Natural history of hearing loss in children with enlarged vestibular aqueduct syndrome.

PubMed

Mori, Tyler; Westerberg, Brian D; Atashband, Shahnaz; Kozak, Frederick K

2008-02-01

To determine the natural history of hearing loss in children with enlarged vestibular aqueduct (EVA) syndrome. (1) Retrospective cohort study and (2) systematic literature review. Tertiary pediatric centre. (1) Charts of children assessed by one physician between 1993 and 2000 were reviewed. (2) Source articles were identified by a search of Medline, Embase, and the Cochrane Library of the English-language literature through January 2006, with manual review of references. The search was limited to English, human, and age less than 18 years. Pure-tone average. Hearing was classified as stable, progressive and fluctuating. (1) Twenty-one children (39 ears) with EVA were identified. Eighty-two percent of ears had stable hearing, and 18% of ears demonstrated progressive hearing loss. (2) Seven source articles were identified and combined with the present data for a total of 310 ears with a mean follow-up of 4 years. Bilateral EVA was found to be six times more common than unilateral EVA, and there was an equal male to female ratio. Stable hearing was found in 67% of ears and progressive hearing loss in 33% of ears. Subgroup analysis demonstrated hearing fluctuations in 50% of progressive hearing loss ears and 34% of stable ears. Stable hearing is observed in 67% of ears with EVA of which 34% will demonstrate fluctuations in hearing. Progression of hearing loss is seen in 33% of ears of which half will demonstrate fluctuations.

Expressivity of hearing loss in cases with Usher syndrome type IIA.

PubMed

Sadeghi, André M; Cohn, Edward S; Kimberling, William J; Halvarsson, Glenn; Möller, Claes

2013-12-01

The purpose of this study was to compare the genotype/phenotype relationship between siblings with identical USH2A pathologic mutations and the consequent audiologic phenotypes, in particular degree of hearing loss (HL). Decade audiograms were also compared among two groups of affected subjects with different mutations of USH2A. DNA samples from patients with Usher syndrome type II were analysed. The audiological features of patients and affected siblings with USH2A mutations were also examined to identify genotype-phenotype correlations. Genetic and audiometric examinations were performed in 18 subjects from nine families with Usher syndrome type IIA. Three different USH2A mutations were identified in the affected subjects. Both similarities and differences of the auditory phenotype were seen in families with several affected siblings. A variable degree of hearing loss, ranging from mild to profound, was observed among affected subjects. No significant differences in hearing thresholds were found the group of affected subjects with different pathological mutations. Our results indicate that mutations in the USH2A gene and the resulting phenotype are probably modulated by other variables, such as modifying genes, epigenetics or environmental factors which may be of importance for better understanding the etiology of Usher syndrome.

[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

Munchausen syndrome by proxy presenting as hearing loss.

PubMed

Ashraf, N; Thevasagayam, M S

2014-06-01

To review the diagnosis of Munchausen syndrome by proxy, a factitious disorder, in which symptoms are induced or feigned, usually in a child, by the caregiver. The involved caregiver seeks to gain attention or sympathy and often has a psychological need to maintain the sick role. We highlight the diagnostic difficulties and factors that may help with diagnosis in an otolaryngology setting. We present the case of Munchausen syndrome by proxy presenting with hearing loss in a five-year old boy, who was diagnosed eight years after his initial presentation. A literature review of Munchausen syndrome by proxy cases presenting with ENT symptoms is provided. Munchausen syndrome by proxy is a diagnosis that otolaryngologists should be aware of, particularly where recurrent or persistent illnesses in children, especially those involving otological symptoms, are refractory to the usual treatments.

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.

Audiological findings in Usher syndrome types IIa and II (non-IIa).

PubMed

Sadeghi, Mehdi; Cohn, Edward S; Kelly, William J; Kimberling, William J; Tranebjoerg, Lisbeth; Möller, Claes

2004-03-01

The aim was to define the natural history of hearing loss in Usher syndrome type IIa compared to non-IIa. People with Usher syndrome type II show moderate-to-severe hearing loss, normal balance and retinitis pigmentosa. Several genes cause Usher syndrome type II. Our subjects formed two genetic groups: (1) subjects with Usher syndrome type IIa with a mutation and/or linkage to the Usher IIa gene; (2) subjects with the Usher II phenotype with no mutation and/or linkage to the Usher IIa gene. Four hundred and two audiograms of 80 Usher IIa subjects were compared with 435 audiograms of 87 non-IIa subjects. Serial audiograms with intervals of > or = 5 years were examined for progression in 109 individuals Those with Usher syndrome type IIa had significantly worse hearing thresholds than those with non-IIa Usher syndrome after the second decade. The hearing loss in Usher syndrome type IIa was found to be more progressive, and the progression started earlier than in non-IIa Usher syndrome. This suggests an auditory phenotype for Usher syndrome type IIa that is different from that of other types of Usher syndrome II. Thus, this is to our knowledge one of the first studies showing a genotype-phenotype auditory correlation.

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.

Three novel GJB2 (connexin 26) variants associated with autosomal dominant syndromic and nonsyndromic hearing loss.

PubMed

DeMille, Desiree; Carlston, Colleen M; Tam, Oliver H; Palumbos, Janice C; Stalker, Heather J; Mao, Rong; Zori, Roberto T; Viskochil, David H; Park, Albert H; Carey, John C

2018-04-01

Connexin 26 (Cx26), encoded by the GJB2 gene, is a key protein involved in the formation of gap junctions in epithelial organs including the inner ear and palmoplantar epidermis. Pathogenic variants in GJB2 are responsible for approximately 50% of inherited sensorineural deafness. The majority of these variants are associated with autosomal recessive inheritance; however, rare reports of dominantly co-segregating variants have been published. Since we began offering GJB2 testing in 2003, only about 2% of detected GJB2 variants from our laboratory have been classified as dominant. Here we report three novel dominant GJB2 variants (p.Thr55Ala, p.Gln57_Pro58delinsHisSer, and p.Trp44Gly); two associated with syndromic sensorineural hearing loss and one with nonsyndromic hearing loss. In the kindred with the p.Thr55Ala variant, the proband and his father present with only leukonychia as a cutaneous finding of their syndromic hearing loss. This phenotype has been previously documented in conjunction with palmoplantar hyperkeratosis, but isolated leukonychia is a novel finding likely associated with the unique threonine to alanine change at codon 55 (other variants at this codon have been reported in cases of nonsyndromic hearing loss). This report contributes to the short list of GJB2 variants associated with autosomal dominant hearing loss, highlights the variability of skin and nail findings associated with such cases, and illustrates the occurrence of both syndromic and nonsyndromic presentations with changes in the same gene. © 2018 Wiley Periodicals, Inc.

[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.

Ectrodactyly, Ectodermal dysplasia, and Cleft Lip-Palate Syndrome; Its Association with Conductive Hearing Loss

ERIC Educational Resources Information Center

Robinson, Geoffrey C.; And Others

1973-01-01

Conductive hearing loss associated with the ectrodactyly, ectodermal dysplasia, and cleft lip palate syndrome was reported in one sporadic case and in a pedigree with four cases in three generations. (GW)

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.

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.

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

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.

Pseudoexfoliation Syndrome and Sensorineural Hearing Loss

PubMed Central

Zojaji, Ramin; Alesheykh, Ali; Sedaghat, Mohammad Reza; Navia, Kiamarz; Mazloom Farsi Baf, Morteza; Khaki, Masoud; Raouf, Aliasghar

2011-01-01

Introduction: Pseudoexfoliation syndrome (PXS) occurs due to the deposition of extracellular fibrillar materials on the anterior chamber of the eye. This syndrome has been considered to be part of a systemic disease with the potential involvement of the inner ear called sensoroneural hearing loss (SNHL). In this study, we aimed on evaluating SNHL within PXS patients in Iran to compare them with other international reports. Materials and Methods: In total, 33 patients with PXS and 33 age and sex matched controls were enrolled prospectively in a case-control study. Both groups underwent complete ophthalmologic and otorhinolaryngologic examinations and pure tone audiometry (PTA) testing. Six frequencies (0.25, 0.5, 1, 2, 3, 4 and 6 KHz) were evaluated for PTA in the same ethnic group in order to select the case and control individuals. Data were analyzed using t-test and chi-square test. Results: Forty-nine out of 66 ears (75.2%) in the PXS group and 27 ears (40.9%) in the control group had SNHL (P<0.001). No significant difference was found between the existence of exfoliative glaucoma (EXG) and SNHL in the PXS patients (P=0.768). Conclusion: Our results indicate a significant association between PXS and SNHL and may support the systemic nature of this disease. PMID:24303375

The Hearing Outcomes of Cochlear Implantation in Waardenburg Syndrome.

PubMed

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

2016-01-01

Objectives. This study aimed to determine the feasibility of cochlear implantation for sensorineural hearing loss in patients with Waardenburg syndrome. Method. A retrospective chart review was performed on patients who underwent cochlear implantation at the University of Tokyo Hospital. Clinical classification, genetic mutation, clinical course, preoperative hearing threshold, high-resolution computed tomography of the temporal bone, and postoperative hearing outcome were assessed. Result. Five children with Waardenburg syndrome underwent cochlear implantation. The average age at implantation was 2 years 11 months (ranging from 1 year 9 months to 6 years 3 months). Four patients had congenital profound hearing loss and one patient had progressive hearing loss. Two patients had an inner ear malformation of cochlear incomplete partition type 2. No surgical complication or difficulty was seen in any patient. All patients showed good hearing outcome postoperatively. Conclusion. Cochlear implantation could be a good treatment option for Waardenburg syndrome.

The Hearing Outcomes of Cochlear Implantation in Waardenburg Syndrome

PubMed Central

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

2016-01-01

Objectives. This study aimed to determine the feasibility of cochlear implantation for sensorineural hearing loss in patients with Waardenburg syndrome. Method. A retrospective chart review was performed on patients who underwent cochlear implantation at the University of Tokyo Hospital. Clinical classification, genetic mutation, clinical course, preoperative hearing threshold, high-resolution computed tomography of the temporal bone, and postoperative hearing outcome were assessed. Result. Five children with Waardenburg syndrome underwent cochlear implantation. The average age at implantation was 2 years 11 months (ranging from 1 year 9 months to 6 years 3 months). Four patients had congenital profound hearing loss and one patient had progressive hearing loss. Two patients had an inner ear malformation of cochlear incomplete partition type 2. No surgical complication or difficulty was seen in any patient. All patients showed good hearing outcome postoperatively. Conclusion. Cochlear implantation could be a good treatment option for Waardenburg syndrome. PMID:27376080

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.

Inner ear anomalies and conductive hearing loss in children with Apert syndrome: an overlooked otologic aspect.

PubMed

Zhou, Guangwei; Schwartz, Lynn Thomas; Gopen, Quinton

2009-02-01

To identify the occurrence of inner ear structural anomalies and conductive hearing loss (CHL) in children with Apert syndrome. Retrospective review. Pediatric tertiary referral center. Twenty pediatric patients with Apert syndrome were found; all patients (38/40 ears) had inner ear anomalies. Computerized tomography of the head/temporal bone, pure-tone (including air and bone conduction) audiometry, and tympanometry. Imaging demonstrating inner ear anomalies, including malformations of the cochlea, dilated vestibule, and/or semicircular canal; audiologic findings of air-bone gap(s). Hearing loss was found in 90% of the patients with Apert syndrome, and 80% of them had CHL. Air-bone gaps were found at all frequencies, with larger gaps at low frequencies. Fifty percent (20/40) of the ears had better than 0 dB hearing level bone conduction thresholds at 250 and/or 500 Hz. Normal middle ear pressure and mobility were found in all ears with intact eardrum. Inner ear anomalies were found in all patients, and 90% of them had bilateral involvement. Most frequently observed inner ear anomalies were dilated vestibule, malformed lateral semicircular canal, and cochlear dysplasia. Children with Apert syndrome may present with significant CHL that cannot be explained by minor middle ear pathologies alone. This conductive loss may be, at least partially, attributed to the inner ear anomalies; however, these structural anomalies are usually not recognized in these patients. Failure to close air-bone gap after surgical intervention may raise the suspicion of inner ear anomalies, and computed tomographic scan of the temporal bone can provide definitive proof.

Novel missense mutations in MYO7A underlying postlingual high- or low-frequency non-syndromic hearing impairment in two large families from China.

PubMed

Sun, Yi; Chen, Jing; Sun, Hanjun; Cheng, Jing; Li, Jianzhong; Lu, Yu; Lu, Yanping; Jin, Zhanguo; Zhu, Yuhua; Ouyang, Xiaomei; Yan, Denise; Dai, Pu; Han, Dongyi; Yang, Weiyan; Wang, Rongguang; Liu, Xuezhong; Yuan, Huijun

2011-01-01

The myosin VIIA (MYO7A) gene encodes a protein classified as an unconventional myosin. Mutations within MYO7A can lead to both syndromic and non-syndromic hearing impairment in humans. Among different mutations reported in MYO7A, only five led to non-syndromic sensorineural deafness autosomal dominant type 11 (DFNA11). Here, we present the clinical, genetic and molecular characteristics of two large Chinese DFNA11 families with either high- or low-frequency hearing loss. Affected individuals of family DX-J033 have a sloping audiogram at young ages with high frequency are most affected. With increasing age, all test frequencies are affected. Affected members of family HB-S037 present with an ascending audiogram affecting low frequencies at young ages, and then all frequencies are involved with increasing age. Genome-wide linkage analysis mapped the disease loci within the DFNA11 interval in both families. DNA sequencing of MYO7A revealed two novel nucleotide variations, c.652G > A (p.D218N) and c.2011G > A (p.G671S), in the two families. It is for the first time that the mutations identified in MYO7A in the present study are being implicated in DFNA11 in a Chinese population. For the first time, we tested electrocochleography (ECochG) in a DFNA11 family with low-frequency hearing loss. We speculate that the low-frequency sensorineural hearing loss in this DFNA11 family was not associated with endolymphatic hydrops.

Prevalence and Nature of Hearing Loss in 22q11.2 Deletion Syndrome

ERIC Educational Resources Information Center

Van Eynde, Charlotte; Swillen, Ann; Lambeens, Elien; Verhaert, Nicolas; Desloovere, Christian; Luts, Heleen; Vander Poorten, Vincent; Devriendt, Koenraad; Hens, Greet

2016-01-01

Purpose: The purpose of this study was to clarify the prevalence, type, severity, and age-dependency of hearing loss in 22q11.2 deletion syndrome. Method: Extensive audiological measurements were conducted in 40 persons with proven 22q11.2 deletion (aged 6-36 years). Besides air and bone conduction thresholds in the frequency range between 0.125…

Usher syndrome: hearing loss, retinal degeneration and associated abnormalities

PubMed Central

Mathur, Pranav; Yang, Jun

2014-01-01

Usher syndrome (USH), clinically and genetically heterogeneous, is the leading genetic cause of combined hearing and vision loss. USH is classified into three types, based on the hearing and vestibular symptoms observed in patients. Sixteen loci have been reported to be involved in the occurrence of USH and atypical USH. Among them, twelve have been identified as causative genes and one as a modifier gene. Studies on the proteins encoded by these USH genes suggest that USH proteins interact among one another and function in multiprotein complexes in vivo. Although their exact functions remain enigmatic in the retina, USH proteins are required for the development, maintenance and function of hair bundles, which are the primary mechanosensitive structure of inner ear hair cells. Despite the unavailability of a cure, progress has been made to develop effective treatments for this disease. In this review, we focus on the most recent discoveries in the field with an emphasis on USH genes, protein complexes and functions in various tissues as well as progress toward therapeutic development for USH. PMID:25481835

Usher syndrome: Hearing loss, retinal degeneration and associated abnormalities.

PubMed

Mathur, Pranav; Yang, Jun

2015-03-01

Usher syndrome (USH), clinically and genetically heterogeneous, is the leading genetic cause of combined hearing and vision loss. USH is classified into three types, based on the hearing and vestibular symptoms observed in patients. Sixteen loci have been reported to be involved in the occurrence of USH and atypical USH. Among them, twelve have been identified as causative genes and one as a modifier gene. Studies on the proteins encoded by these USH genes suggest that USH proteins interact among one another and function in multiprotein complexes in vivo. Although their exact functions remain enigmatic in the retina, USH proteins are required for the development, maintenance and function of hair bundles, which are the primary mechanosensitive structure of inner ear hair cells. Despite the unavailability of a cure, progress has been made to develop effective treatments for this disease. In this review, we focus on the most recent discoveries in the field with an emphasis on USH genes, protein complexes and functions in various tissues as well as progress toward therapeutic development for USH. Copyright © 2014 Elsevier B.V. All rights reserved.

A novel pathogenic variant in the MARVELD2 gene causes autosomal recessive non-syndromic hearing loss in an Iranian family.

PubMed

Taghipour-Sheshdeh, Afsaneh; Nemati-Zargaran, Fatemeh; Zarepour, Narges; Tahmasebi, Parisa; Saki, Nader; Tabatabaiefar, Mohammad Amin; Mohammadi-Asl, Javad; Hashemzadeh-Chaleshtori, Morteza

2018-05-09

Hearing loss (HL) is the most common sensorineural disorder and one of the most common human defects. HL can be classified according to main criteria, including: the site (conductive, sensorineural and mixed), onset (pre-lingual and post-lingual), accompanying signs and symptoms (syndromic and non-syndromic), severity (mild, moderate, severe and profound) and mode of inheritance (Autosomal recessive, autosomal dominant, X-linked and mitochondrial). Autosomal recessive non-syndromic HL (ARNSHL) forms constitute a major share of the HL cases. In the present study, next-generation sequencing (NGS) was applied to investigate the underlying etiology of HL in a multiplex ARNSHL family from Khuzestan province, southwest Iran. In this descriptive study, 20 multiplex ARNSHL families from Khuzestan province, southwest of Iran were recruited. After DNA extraction, genetic linkage analysis (GLA) was applied to screen for a panel of more prevalent loci. One family, which was not linked to these loci, was subjected to Otogenetics deafness Next Generation Sequencing (NGS) panel. NGS results showed a novel deletion-insertion variant (c.1555delinsAA) in the MARVELD2 gene. The variant which is a frameshift in the seventh exon of the MARVELD2 gene fulfills the criteria of being categorized as pathogenic according to the American College of Medical Genetics and Genomics (ACMG) guideline. NGS is very promising to identify the molecular etiology of highly heterogeneous diseases such as HL. MARVELD2 might be important in the etiology of HL in this region of Iran. Copyright © 2017. Published by Elsevier Inc.

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

Identification of a novel homozygous mutation in MYO3A in a Chinese family with DFNB30 non-syndromic hearing impairment.

PubMed

Qu, Ronggui; Sang, Qing; Xu, Yao; Feng, Ruizhi; Jin, Li; He, Lin; Wang, Lei

2016-05-01

Hearing loss is a common sensory impairment. Several genetic loci or genes responsible for non-syndrome hearing loss have been identified, including the well-known deafness genes GJB2, MT-RNR1 and SLC26A4. MYO3A belongs to the myosin superfamily. Previously only three mutations in this gene have been found in an Isreali family with DFNB30, in which patients demonstrated progressive hearing loss. In this study, we characterized a consanguineous Kazakh family with congenital hearing loss. By targeted sequence capture and next-generation sequencing, we identified a homozygous mutation and did bioinformatics analysis to this mutation. A homozygous mutation, MYO3A:c.1841C>T (p.S614F), was identified to be responsible for the disease. Ser614 is located in the motor domain of MYO3A that is highly conserved among different species. Molecular modeling predicts that the conserved Ser614 may play an important role in maintaining the stability of β-sheet and the interaction between neighboring β-strand. This is the second report on MYO3A mutations in deafness and the first report in China. The finding help facilitate establishing a better relationship between MYO3A mutation and hearing phenotypes. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Hair phenotype in non-syndromic deafness.

PubMed

Volo, T; Sathiyaseelan, T; Astolfi, L; Guaran, V; Trevisi, P; Emanuelli, E; Martini, A

2013-08-01

The GJB2 gene is located on chromosome 13q12 and it encodes the connexin 26, a transmembrane protein involved in cell-cell attachment of almost all tissues. GJB2 mutations cause autosomal recessive (DFNB1) and sometimes dominant (DFNA3) non-syndromic sensorineural hearing loss. Moreover, it has been demonstrated that connexins are involved in regulation of growth and differentiation of epidermal tissues. Hence, mutations in GJB2 gene, which is responsible for non-syndromic deafness, may be associated with an abnormal skin and hair phenotype. We analyzed hair samples from 96 subjects: a study group of 42 patients with hearing impairments of genetic origin (38 with a non-syndromic form, 4 with a syndromic form), and a control group including 54 people, i.e. 43 patients with other, non-genetic hearing impairments and 11 healthy volunteers aged up to 10 years old. The surface structure of 49 hair samples was normal, whereas in 45 cases it was altered, with a damaged appearance. Two hair samples were considered unclassifiable: one from the patient heterozygotic for the pendrin mutation (Fig. 2C), the other from a patient from Ghana with a R134W mutation (Fig. 2D). Among the 43 altered hair samples, 31 belonged to patients with connexin mutations and the other 12 came from patients without connexin mutations. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

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.

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)

Hearing loss in Waardenburg syndrome: a systematic review.

PubMed

Song, J; Feng, Y; Acke, F R; Coucke, P; Vleminckx, K; Dhooge, I J

2015-06-22

Waardenburg syndrome (WS) is a rare genetic disorder characterized by hearing loss (HL) and pigment disturbances of hair, skin and iris. Classifications exist based on phenotype and genotype. The auditory phenotype is inconsistently reported among the different Waardenburg types and causal genes, urging the need for an up-to-date literature overview on this particular topic. We performed a systematic review in search for articles describing auditory features in WS patients along with the associated genotype. Prevalences of HL were calculated and correlated with the different types and genes of WS. Seventy-three articles were included, describing 417 individual patients. HL was found in 71.0% and was predominantly bilateral and sensorineural. Prevalence of HL among the different clinical types significantly differed (WS1: 52.3%, WS2: 91.6%, WS3: 57.1%, WS4: 83.5%). Mutations in SOX10 (96.5%), MITF (89.6%) and SNAI2 (100%) are more frequently associated with hearing impairment than other mutations. Of interest, the distinct disease-causing genes are able to better predict the auditory phenotype compared with different clinical types of WS. Consequently, it is important to confirm the clinical diagnosis of WS with molecular analysis in order to optimally inform patients about the risk of HL. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Congenital hearing loss

PubMed Central

Korver, Anna M. H.; Smith, Richard J. H.; Van Camp, Guy; Schleiss, Mark R.; Bitner-Glindzicz, Maria A. K.; Lustig, Lawrence R.; Usami, Shin-ichi; Boudewyns, An N.

2017-01-01

Congenital hearing loss (hearing loss present at birth) is one of the most prevalent chronic conditions in children. In the majority of developed countries, neonatal hearing-screening programmes enable early detection; early intervention will prevent delays in speech and language development and have long-lasting beneficial effects on social and emotional development and quality of life. A hearing loss diagnosis is usually followed by a search for an underlying aetiology. Congenital hearing loss might be attributed to environmental and prenatal factors, which prevail in low-income settings; congenital infections, particularly cytomegalovirus, are also a common risk factor for hearing loss. Genetic causes probably account for the majority of cases in developed countries; mutations can affect any component of the hearing pathway, in particular inner ear homeostasis (endolymph production and maintenance) and mechano-electrical transduction (conversion of a mechanical stimulus into electrochemical activity). Once the underlying cause of hearing loss is established, it might direct therapeutic decision-making and guide prevention and (genetic) counseling. Management options include specific antimicrobial therapies, surgical treatment of cranio-facial abnormalities and hearing aids. An improved understanding of the pathophysiology and molecular mechanisms underlying hearing loss and increased awareness of recent advances in genetic testing will promote the development of new treatment and screening strategies. PMID:28079113

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

Familial leuconychia, knuckle pads, hearing loss, and palmoplantar hyperkeratosis: an additional family with Bart-Pumphrey syndrome.

PubMed

Ramer, J C; Vasily, D B; Ladda, R L

1994-01-01

A family with five members who have variable findings of leuconychia, knuckle pads, hearing loss, and palmoplantar hyperkeratosis is described. The findings in these subjects are compared with those noted in previously reported patients with Bart-Pumphrey syndrome. The range of disorders which include knuckle pads as part of the phenotype is reviewed.

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

PubMed Central

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

2016-01-01

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

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

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.

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 ...

Familial leuconychia, knuckle pads, hearing loss, and palmoplantar hyperkeratosis: an additional family with Bart-Pumphrey syndrome.

PubMed Central

Ramer, J C; Vasily, D B; Ladda, R L

1994-01-01

A family with five members who have variable findings of leuconychia, knuckle pads, hearing loss, and palmoplantar hyperkeratosis is described. The findings in these subjects are compared with those noted in previously reported patients with Bart-Pumphrey syndrome. The range of disorders which include knuckle pads as part of the phenotype is reviewed. Images PMID:8151643

Hearing loss

MedlinePlus

... eardrum) Ringing or buzzing sound in the ears ( tinnitus ) Causes Conductive hearing loss (CHL) occurs because of ... severe hearing loss or ringing in the ears (tinnitus). You have other symptoms, such as ear pain , ...

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.

Saethre-Chotzen syndrome, Pro136His TWIST mutation, hearing loss, and external and middle ear structural anomalies: report on a Brazilian family.

PubMed

Lamônica, Dionísia A C; Maximino, Luciana P; Feniman, Mariza Ribeiro; Silva, Greyce K; Zanchetta, Sthella; Abramides, Dagma V M; Passos-Bueno, Maria Rita; Rocha, Kátia; Richieri-Costa, Antonio

2010-09-01

To describe the clinical, speech, hearing, and imaging findings in three members of a Brazilian family with Saethre-Chotzen syndrome (SCS) who presented some unusual characteristics within the spectrum of the syndrome. Clinical evaluation was performed by a multidisciplinary team. Direct sequencing of the polymerase chain reaction-amplified coding region of the TWIST1 gene, routine and electrophysiological hearing evaluation, speech evaluation, and imaging studies through computed tomography (CT) scan and magnetic resonance imaging (MRI) were performed. TWIST1 gene analysis revealed a Pro136His mutation in all patients. Hearing evaluation showed peripherial and mixed hearing loss in two of the patients, one of them with severe unilateral microtia. Computed tomography scan showed structural middle ear anomalies, and MRI showed distortion of the skull contour as well as some of the brain structures. We report a previously undescribed TWIST1 gene mutation in patients with SCS. There is evidence that indicates hearing loss (conductive and mixed) can be related both with middle ear (microtia, high jugular bulb, and enlarged vestibules) as well as with brain stem anomalies. Here we discuss the relationship between the gene mutation and the clinical, imaging, speech, and hearing findings.

Vestibular function in families with inherited autosomal dominant hearing loss

PubMed Central

Street, Valerie A.; Kallman, Jeremy C.; Strombom, Paul D.; Bramhall, Naomi F.; Phillips, James O.

2008-01-01

The inner ear contains the developmentally related cochlea and peripheral vestibular labyrinth. Given the similar physiology between these two organs, hearing loss and vestibular dysfunction may be expected to occur simultaneously in individuals segregating mutations in inner ear genes. Twenty-two different genes have been discovered that when mutated lead to non-syndromic autosomal dominant hearing loss. A review of the literature indicates that families segregating mutations in 13 of these 22 genes have undergone formal clinical vestibular testing. Formal assessment revealed vestibular dysfunction in families with mutations in ten of these 13 genes. Remarkably, only families with mutations in the COCH and MYO7A genes self-report considerable vestibular challenges. Families segregating mutations in the other eight genes do not self-report significant balance problems and appear to compensate well in everyday life for vestibular deficits discovered during formal clinical vestibular assessment. An example of a family (referred to as the HL1 family) with progressive hearing loss and clinically-detected vestibular hypofunction that does not report vestibular symptoms is described in this review. Notably, one member of the HL1 family with clinically-detected vestibular hypofunction reached the summit of Mount Kilimanjaro. PMID:18776598

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…

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

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

Hearing loss and paid employment: Australian population survey findings.

PubMed

Hogan, Anthony; O'Loughlin, Kate; Davis, Adrian; Kendig, Hal

2009-03-01

This paper provides an analysis of participation in paid employment for people with a hearing loss over the full span of adult ages. The paper is based on original analysis of the 2003 Australian survey of disability, aging and carers (SDAC). This analysis shows that hearing loss was associated with an increased rate of non-participation in employment of between 11.3% and 16.6%. Advancing age and the existence of co-morbidities contribute significantly to reduced participation in employment. A disproportionate impact is evident for women and for those having low education and communication difficulties. Controlling for co-morbidities, hearing loss was associated with a 2.1% increase of non-participation in employment, a proportional difference of 1.4 times the population. People with hearing loss were less likely to be found in highly skilled jobs and were over-represented among low income earners. The SDAC data set provides self-report findings on the experience of disability rather than hearing impairment. As such, these findings serve as a conservative estimate of the impact of hearing loss on accessing well-paid employment.

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.

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.

Hearing loss and enlarged internal auditory canal in children.

PubMed

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

2014-01-01

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

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.

[Characteristics of audiology and clinical genetics of a Chinese family with the DFNA5 genetic hearing loss].

PubMed

Jin, Zhanguo; Cheng, Jing; Han, Bing; Li, Hongbo; Lu, Yu; Li, Zhengyue; Han, Dongyi

2011-05-01

To analysis the characteristics of audiology and clinical genetics of a Chinese family with the DFNA5 genetic hearing loss in detail. A detailed family history and clinical data were collected. The Chinese pedigree is an autosomal-dominant inherited hearing loss. The data of audiological examination about genetic characteristics was analysed. The relationship between the hearing-impaired of this family and age was contrasted. This Chinese family spanned five generations and comprised 42 members. The mode of inheritance of the families should be autosomal dominant according to the pedigree. Pure-tone audiograms showed a so-called Z shape curve. The hearing loss is sensorineural, progressive and beginning at the high frequencies. The audiograms were fairly symmetric. Whole frequencies became involved with increasing age. The Chinese family with the DFNA5 mutation was an autosomal dominant pedigree. In this family, non-syndromic symmetric hearing impairment was severest at the high frequencies early, and gradually accumulated all frequencies of hearing. A mutation in DFNA5 leads to a type of hearing loss that closely resembles the frequently observed age-related hearing impairment. It should take into account DFNA5 mutation which the audiogram of a genetic hearing impaired has the same feature.

Johnson-McMillin syndrome, a neuroectodermal syndrome with conductive hearing loss and microtia: report of a new case.

PubMed

Schweitzer, Daniela N; Yano, Shoji; Earl, Dawn L; Graham, John M

2003-07-30

In 1983, Johnson et al. described 16 related individuals with alopecia, anosmia or hyposmia, conductive hearing loss, microtia and/or atresia of the external auditory canal, and hypogonadotrophic hypogonadism inherited in an autosomal dominant pattern. Other less constant manifestations included facial asymmetry, mental retardation, congenital heart defect, cleft palate, and choanal stenosis. An isolated case was reported later (Johnston et al. [1987: Am J Med Genet 26: 925-927]) and thereafter an affected mother and son (Hennekam and Holtus [1993: Am J Med Genet 47: 714-716]). We describe an additional unrelated female patient with features resembling those of the previously reported cases. She presented with intrauterine growth deficiency, microcephaly, alopecia, bilateral microtia with canal atresia, conductive hearing loss, partial left facial palsy, posterior cleft palate, left choanal stenosis, tetralogy of Fallot, developmental delay, and right thumb polydactyly. Because the phenotypic abnormalities in this syndrome affect the brain, facial structures, ectoderm and its derivatives, outflow tract of the heart, and Rathke's pouch derivatives, this has suggested to previous authors etiologic involvement of the ectoderm and neuroectoderm of the first and second branchial arches, Rathke's pouch, and the diencephalon. Microtia with conductive hearing loss differentiates the condition from other ectodermal dysplasias. In the initial report, females appeared somewhat less affected than males, and there was male-to-male transmission. The mother of our patient manifests subtle features, which suggest she may be a mildly affected female. Additionally, there is a family history of early-onset alopecia in the maternal grandfather's relatives. Copyright 2003 Wiley-Liss, Inc.

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

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 dysfunction in heterozygous Mitf(Mi-wh) /+ mice, a model for Waardenburg syndrome type 2 and Tietz syndrome.

PubMed

Ni, Christina; Zhang, Deming; Beyer, Lisa A; Halsey, Karin E; Fukui, Hideto; Raphael, Yehoash; Dolan, David F; Hornyak, Thomas J

2013-01-01

The human deafness-pigmentation syndromes, Waardenburg syndrome (WS) type 2a, and Tietz syndrome are characterized by profound deafness but only partial cutaneous pigmentary abnormalities. Both syndromes are caused by mutations in MITF. To illuminate differences between cutaneous and otic melanocytes in these syndromes, their development and survival in heterozygous Microphthalmia-White (Mitf(Mi-wh) /+) mice were studied and hearing function of these mice characterized. Mitf(Mi-wh) /+ mice have a profound hearing deficit, characterized by elevated auditory brainstem response thresholds, reduced distortion product otoacoustic emissions, absent endocochlear potential, loss of outer hair cells, and stria vascularis abnormalities. Mitf(Mi-wh) /+ embryos have fewer melanoblasts during embryonic development than their wild-type littermates. Although cochlear melanocytes are present at birth, they disappear from the Mitf(Mi-wh) /+ cochlea between P1 and P7. These findings may provide insight into the mechanism of melanocyte and hearing loss in human deafness-pigmentation syndromes such as WS and Tietz syndrome and illustrate differences between otic and follicular melanocytes. © 2012 John Wiley & Sons A/S.

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.

Hearing Loss in a Mouse Model of 22q11.2 Deletion Syndrome

PubMed Central

Fuchs, Jennifer C.; Zinnamon, Fhatarah A.; Taylor, Ruth R.; Ivins, Sarah; Scambler, Peter J.; Forge, Andrew; Tucker, Abigail S.; Linden, Jennifer F.

2013-01-01

22q11.2 Deletion Syndrome (22q11DS) arises from an interstitial chromosomal microdeletion encompassing at least 30 genes. This disorder is one of the most significant known cytogenetic risk factors for schizophrenia, and can also cause heart abnormalities, cognitive deficits, hearing difficulties, and a variety of other medical problems. The Df1/+ hemizygous knockout mouse, a model for human 22q11DS, recapitulates many of the deficits observed in the human syndrome including heart defects, impaired memory, and abnormal auditory sensorimotor gating. Here we show that Df1/+ mice, like human 22q11DS patients, have substantial rates of hearing loss arising from chronic middle ear infection. Auditory brainstem response (ABR) measurements revealed significant elevation of click-response thresholds in 48% of Df1/+ mice, often in only one ear. Anatomical and histological analysis of the middle ear demonstrated no gross structural abnormalities, but frequent signs of otitis media (OM, chronic inflammation of the middle ear), including excessive effusion and thickened mucosa. In mice for which both in vivo ABR thresholds and post mortem middle-ear histology were obtained, the severity of signs of OM correlated directly with the level of hearing impairment. These results suggest that abnormal auditory sensorimotor gating previously reported in mouse models of 22q11DS could arise from abnormalities in auditory processing. Furthermore, the findings indicate that Df1/+ mice are an excellent model for increased risk of OM in human 22q11DS patients. Given the frequently monaural nature of OM in Df1/+ mice, these animals could also be a powerful tool for investigating the interplay between genetic and environmental causes of OM. PMID:24244619

Hearing loss in a mouse model of 22q11.2 Deletion Syndrome.

PubMed

Fuchs, Jennifer C; Zinnamon, Fhatarah A; Taylor, Ruth R; Ivins, Sarah; Scambler, Peter J; Forge, Andrew; Tucker, Abigail S; Linden, Jennifer F

2013-01-01

22q11.2 Deletion Syndrome (22q11DS) arises from an interstitial chromosomal microdeletion encompassing at least 30 genes. This disorder is one of the most significant known cytogenetic risk factors for schizophrenia, and can also cause heart abnormalities, cognitive deficits, hearing difficulties, and a variety of other medical problems. The Df1/+ hemizygous knockout mouse, a model for human 22q11DS, recapitulates many of the deficits observed in the human syndrome including heart defects, impaired memory, and abnormal auditory sensorimotor gating. Here we show that Df1/+ mice, like human 22q11DS patients, have substantial rates of hearing loss arising from chronic middle ear infection. Auditory brainstem response (ABR) measurements revealed significant elevation of click-response thresholds in 48% of Df1/+ mice, often in only one ear. Anatomical and histological analysis of the middle ear demonstrated no gross structural abnormalities, but frequent signs of otitis media (OM, chronic inflammation of the middle ear), including excessive effusion and thickened mucosa. In mice for which both in vivo ABR thresholds and post mortem middle-ear histology were obtained, the severity of signs of OM correlated directly with the level of hearing impairment. These results suggest that abnormal auditory sensorimotor gating previously reported in mouse models of 22q11DS could arise from abnormalities in auditory processing. Furthermore, the findings indicate that Df1/+ mice are an excellent model for increased risk of OM in human 22q11DS patients. Given the frequently monaural nature of OM in Df1/+ mice, these animals could also be a powerful tool for investigating the interplay between genetic and environmental causes of OM.

Whole mitochondrial genome screening in maternally inherited non-syndromic hearing impairment using a microarray resequencing mitochondrial DNA chip.

PubMed

Lévêque, Marianne; Marlin, Sandrine; Jonard, Laurence; Procaccio, Vincent; Reynier, Pascal; Amati-Bonneau, Patrizia; Baulande, Sylvain; Pierron, Denis; Lacombe, Didier; Duriez, Françoise; Francannet, Christine; Mom, Thierry; Journel, Hubert; Catros, Hélène; Drouin-Garraud, Valérie; Obstoy, Marie-Françoise; Dollfus, Hélène; Eliot, Marie-Madeleine; Faivre, Laurence; Duvillard, Christian; Couderc, Remy; Garabedian, Eréa-Noël; Petit, Christine; Feldmann, Delphine; Denoyelle, Françoise

2007-11-01

Mitochondrial DNA (mtDNA) mutations have been implicated in non-syndromic hearing loss either as primary or as predisposing factors. As only a part of the mitochondrial genome is usually explored in deafness, its prevalence is probably under-estimated. Among 1350 families with non-syndromic sensorineural hearing loss collected through a French collaborative network, we selected 29 large families with a clear maternal lineage and screened them for known mtDNA mutations in 12S rRNA, tRNASer(UCN) and tRNALeu(UUR) genes. When no mutation could be identified, a whole mitochondrial genome screening was performed, using a microarray resequencing chip: the MitoChip version 2.0 developed by Affymetrix Inc. Known mtDNA mutations was found in nine of the 29 families, which are described in the article: five with A1555G, two with the T7511C, one with 7472insC and one with A3243G mutation. In the remaining 20 families, the resequencing Mitochip detected 258 mitochondrial homoplasmic variants and 107 potentially heteroplasmic variants. Controls were made by direct sequencing on selected fragments and showed a high sensibility of the MitoChip but a low specificity, especially for heteroplasmic variations. An original analysis on the basis of species conservation, frequency and phylogenetic investigation was performed to select the more probably pathogenic variants. The entire genome analysis allowed us to identify five additional families with a putatively pathogenic mitochondrial variant: T669C, C1537T, G8078A, G12236A and G15077A. These results indicate that the new MitoChip platform is a rapid and valuable tool for identification of new mtDNA mutations in deafness.

Interventions to prevent occupational noise-induced hearing loss.

PubMed

Tikka, Christina; Verbeek, Jos H; Kateman, Erik; Morata, Thais C; Dreschler, Wouter A; Ferrite, Silvia

2017-07-07

This is the second update of a Cochrane Review originally published in 2009. Millions of workers worldwide are exposed to noise levels that increase their risk of hearing disorders. There is uncertainty about the effectiveness of hearing loss prevention interventions. To assess the effectiveness of non-pharmaceutical interventions for preventing occupational noise exposure or occupational hearing loss compared to no intervention or alternative interventions. We searched the CENTRAL; PubMed; Embase; CINAHL; Web of Science; BIOSIS Previews; Cambridge Scientific Abstracts; and OSH UPDATE to 3 October 2016. We included randomised controlled trials (RCT), controlled before-after studies (CBA) and interrupted time-series (ITS) of non-clinical interventions under field conditions among workers to prevent or reduce noise exposure and hearing loss. We also collected uncontrolled case studies of engineering controls about the effect on noise exposure. Two authors independently assessed study eligibility and risk of bias and extracted data. We categorised interventions as engineering controls, administrative controls, personal hearing protection devices, and hearing surveillance. We included 29 studies. One study evaluated legislation to reduce noise exposure in a 12-year time-series analysis but there were no controlled studies on engineering controls for noise exposure. Eleven studies with 3725 participants evaluated effects of personal hearing protection devices and 17 studies with 84,028 participants evaluated effects of hearing loss prevention programmes (HLPPs). Effects on noise exposure Engineering interventions following legislationOne ITS study found that new legislation in the mining industry reduced the median personal noise exposure dose in underground coal mining by 27.7 percentage points (95% confidence interval (CI) -36.1 to -19.3 percentage points) immediately after the implementation of stricter legislation. This roughly translates to a 4.5 dB(A) decrease in

Association Between Hearing Loss And Cauliflower Ear in Wrestlers, a Case Control Study Employing Hearing Tests.

PubMed

Noormohammadpour, Pardis; Rostami, Mohsen; Nourian, Ruhollah; Mansournia, Mohammad Ali; Sarough Farahani, Saeed; Farahbakhsh, Farzin; Kordi, Ramin

2015-06-01

According to anecdotal findings, some wrestling coaches and wrestlers believe that cauliflower ear might lead to hearing loss. Our preliminary study showed that the prevalence of hearing loss reported by the wrestlers with cauliflower ear is significantly higher than this rate among wrestlers without cauliflower ear. To the best of our knowledge, no other study has confirmed this finding employing hearing tests. To evaluate and to compare the prevalence of hearing loss among wrestlers with and without cauliflower ears employing hearing tests. The subjects were randomly selected form 14 wrestling clubs in Tehran. Subjects were 201 wrestlers with cauliflower ears (100 wrestlers with one cauliflower ear and 101 wrestlers with two cauliflower ears) and 139 wrestlers without cauliflower ears. All the participants in this study were interviewed to collect information on demographic factors and medical history of risk factors and diseases related to hearing loss. The subjects in both groups underwent otoscopic and audiologic examinations. Audiometric examination results at the frequency range of 0.5 - 8 KHz showed that the prevalence of hearing loss among cauliflower ears was higher than this rate among non-cauliflower ears. Also, the percentage of positive history of ear infections among cauliflower ears (8.4%) was about two times more than this finding among non-cauliflower ears (4.9%). This difference tended to be significant (OR: 1.86, P = 0.06, 95% CI: 0.98 - 3.53). To the best of our knowledge, this is the first study showing that the prevalence of hearing loss among cauliflower ears is higher than this rate among non-cauliflower ears confirmed by audiological tests. This emphasizes that, more preventive measures such as mandatory ear gear for wrestlers are required.

Association Between Hearing Loss And Cauliflower Ear in Wrestlers, a Case Control Study Employing Hearing Tests

PubMed Central

Noormohammadpour, Pardis; Rostami, Mohsen; Nourian, Ruhollah; Mansournia, Mohammad Ali; Sarough Farahani, Saeed; Farahbakhsh, Farzin; Kordi, Ramin

2015-01-01

Background: According to anecdotal findings, some wrestling coaches and wrestlers believe that cauliflower ear might lead to hearing loss. Our preliminary study showed that the prevalence of hearing loss reported by the wrestlers with cauliflower ear is significantly higher than this rate among wrestlers without cauliflower ear. To the best of our knowledge, no other study has confirmed this finding employing hearing tests. Objectives: To evaluate and to compare the prevalence of hearing loss among wrestlers with and without cauliflower ears employing hearing tests. Patients and Methods: The subjects were randomly selected form 14 wrestling clubs in Tehran. Subjects were 201 wrestlers with cauliflower ears (100 wrestlers with one cauliflower ear and 101 wrestlers with two cauliflower ears) and 139 wrestlers without cauliflower ears. All the participants in this study were interviewed to collect information on demographic factors and medical history of risk factors and diseases related to hearing loss. The subjects in both groups underwent otoscopic and audiologic examinations. Results: Audiometric examination results at the frequency range of 0.5 - 8 KHz showed that the prevalence of hearing loss among cauliflower ears was higher than this rate among non-cauliflower ears. Also, the percentage of positive history of ear infections among cauliflower ears (8.4%) was about two times more than this finding among non-cauliflower ears (4.9%). This difference tended to be significant (OR: 1.86, P = 0.06, 95% CI: 0.98 - 3.53). Conclusions: To the best of our knowledge, this is the first study showing that the prevalence of hearing loss among cauliflower ears is higher than this rate among non-cauliflower ears confirmed by audiological tests. This emphasizes that, more preventive measures such as mandatory ear gear for wrestlers are required. PMID:26448842

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

Targeted surveillance for postnatal hearing loss: a program evaluation.

PubMed

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

2012-07-01

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

The circling mutant Pcdh15roda is a new mouse model for hearing loss.

PubMed

Torres, Adriana Amorim; Rzadzinska, Agnieszka K; Ribeiro, Andrea Frozino; Silva, Daniel Almeida da Silva E; Guénet, Jean-Louis; Massironi, Sílvia Maria Gomes; Godard, Ana Lúcia Brunialti

2013-01-01

Mouse mutagenesis is a key tool for studying gene function and several mutant alleles have been described and constitute mouse models for human hereditary diseases. Genetic hearing loss represents over 50% of all hearing loss cases in children and, due to the heterogeneity of the disorder, there is still a demand for the isolation and characterization of new genes and alleles. Here we report phenotypic and molecular characterization of a new mouse model for hereditary hearing loss. The mutant rodador, isolated by Massironi and colleagues in 2006, presents an autosomal recessive disorder characterized by deafness and balance dysfunction associated with abnormal stereocilia in the inner ear. The mutation was mapped to mouse chromosome 10, and characterization of the gene Pcdh15 revealed an AT-to-GC transition in intron 23 of mutant animals. The alteration led to the switch of a dinucleotide ApA for ApG, creating a novel intronic acceptor splice site, which leads to incorporation of eight intronic bases into the processed mRNA and alteration of the downstream reading frame. In silico analysis indicated that the mutated protein is truncated and lacks two cadherin domains, and the transmembrane and cytoplasmic domains. Real Time PCR analyses revealed a significantly reduced Pcdh15 mRNA level in the brain of mutant mice, which might be due to the mechanism of non-sense mediated decay. In man, mutations in the orthologue PCDH15 cause non-syndromic deafness and Usher Syndrome Type 1F, a genetic disorder characterized by hearing loss and retinitis pigmentosa. Rodador mouse constitutes a new model for studying deafness in these conditions and may help in the comprehension of the pathogeneses of the disease, as well as of the mechanisms involved in the morphogenesis and function of inner ear stereocilia. This is a new ENU-induced allele and the first isolated in a BALB/c background. Copyright © 2013 Elsevier B.V. All rights reserved.

Secondhand Smoke Exposure and the Risk of Hearing Loss

PubMed Central

Fabry, David A.; Davila, Evelyn P.; Arheart, Kristopher L.; Serdar, Berrin; Dietz, Noella A.; Bandiera, Frank C.; Lee, David J.

2011-01-01

Background Hearing loss has been associated with tobacco smoking, but its relationship with secondhand smoke is not known. We sought to investigate the association between secondhand smoke exposure and hearing loss in a nationally representative sample of adults. Methods The National Health and Nutrition Examination Survey, a nationally representative cross-sectional dataset, was utilized to investigate the association between secondhand smoke exposure and hearing loss. Data collected from non-smoking participants aged 20-69 years were included in the analysis if they had completed audiometric testing, had a valid serum cotinine value, and provided complete smoking, medical co-morbidity and noise exposure histories (n=3,307). Hearing loss was assessed from averaged pure-tone thresholds over low- or mid-frequencies (500, 1,000, and 2,000 Hz) and high-frequencies (3,000, 4,000, 6,000, and 8,000 Hz), and was defined as mild or greater severity (pure-tone average in excess of 25 dB HL). Results SHS exposure was significantly associated with increased risk of hearing loss for low-/mid-frequencies (Adjusted Odds Ratio = 1.14; 95% CI = 1.02-1.28 for never smokers and 1.30; 1.10-1.54 for former smokers) and high-frequencies (1.40; 1.22-1.81 for former smokers), after controlling for potential confounders. Conclusions Findings from the present analysis indicate that SHS exposure is associated with hearing loss in non-smoking adults. PMID:21081307

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

ERIC Educational Resources Information Center

Kahn, Gerri; Goldman, Ellen

1991-01-01

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

Hearing rehabilitation with single-stage bilateral vibroplasty in a child with Franceschetti syndrome.

PubMed

Sargsyan, Sona; Rahne, Torsten; Kösling, Sabrina; Eichler, Gerburg; Plontke, Stefan K

2014-05-01

Hearing is of utmost importance for normal speech and social development. Even children who have mild or unilateral permanent hearing loss may experience difficulties with understanding speech, as well as problems with educational and psycho-social development. The increasing advantages of middle-ear implant technologies are opening new perspectives for restoring hearing. Active middle-ear implants can be used in children and adolescents with hearing loss. In addition to the well-documented results for improving speech intelligibility and quality of hearing in sensorineural hearing loss active middle-ear implants are now successfully used in patients with conductive and mixed hearing loss. In this article we present a case of successful, single-stage vibroplasty, on the right side with the fixation of the FMT on the stapes and PORP CLiP vibroplasty on the left side in a 6-year-old girl with bilateral mixed hearing loss and multiple dyslalia associated with Franceschetti syndrome (mandibulofacial dysostosis). CT revealed bilateral middle-ear malformations as well as an atretic right and stenotic left external auditory canal. Due to craniofacial dysmorphia airway and (post)operative, management is significantly more difficult in patients with a Franceschetti syndrome which in this case favoured a single-stage bilateral procedure. No intra- or postoperative surgical complications were reported. The middle-ear implants were activated 4 weeks after surgery. In the audiological examination 6 months after surgery, the child showed 100% speech intelligibility with activated implants on each side.

A mutation in SLC22A4 encoding an organic cation transporter expressed in the cochlea strial endothelium causes human recessive non-syndromic hearing loss DFNB60

PubMed Central

Ben Said, Mariem; Grati, M’hamed; Ishimoto, Takahiro; Zou, Bing; Chakchouk, Imen; Ma, Qi; Yao, Qi; Hammami, Bouthaina; Yan, Denise; Mittal, Rahul; Nakamichi, Noritaka; Ghorbel, Abdelmonem; Neng, Lingling; Tekin, Mustafa; Shi, Xiao Rui; Kato, Yukio; Masmoudi, Saber; Lu, Zhongmin; Hmani, Mounira; Liu, Xuezhong

2016-01-01

The high prevalence/incidence of hearing loss (HL) in humans makes it the most common sensory defect. The majority of the cases are of genetic origin. Non-syndromic hereditary HL is extremely heterogeneous. Genetic approaches have been instrumental in deciphering genes that are crucial for auditory function. In this study, we first used NADf chip to exclude the implication of known North-African mutations in HL in a large consanguineous Tunisian family (FT13) affected by autosomal recessive non-syndromic HL (ARNSHL). We then performed genome-wide linkage analysis and assigned the deafness gene locus to ch:5q23.2-31.1, corresponding to DFNB60 ARNSHL locus. Moreover, we performed whole-exome sequencing on FT13 patient DNA and uncovered aminoacid substitution p.Cys113Tyr in SLC22A4, a transporter of organic cations, cosegregating with HL in FT13 and therefore the cause of ARNSHL DFNB60. We also screened a cohort of small Tunisian HL families and uncovered an additional deaf proband of consanguineous parents that is homozygous for p.Cys113Tyr carried by the same microsatellite marker haplotype as in FT13, indicating that this mutation is ancestral. Using immunofluorescence, we found that Slc22a4 is expressed in stria vascularis (SV) endothelial cells of rodent cochlea and targets their apical plasma membrane. We also found Slc22a4 transcripts in our RNA-seq library from purified primary culture of mouse SV endothelial cells. Interestingly, p.Cys113Tyr mutation affects the trafficking of the transporter and severely alters Ergothioneine uptake. We conclude that SLC22A4 is an organic cation transporter of the SV endothelium that is essential for hearing, and its mutation causes DFNB60 form of HL. PMID:27023905

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

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

PubMed Central

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

2012-01-01

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

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.

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

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.

Iloprost-induced sudden hearing loss.

PubMed

Dursun, E; Dogru, S; Cincik, H; Cekin, E; Gungor, A; Poyrazoglu, E

2007-06-01

We report a patient who developed sudden, bilateral, sensorineural hearing loss during therapeutic use of iloprost for Raynaud's phenomenon. The sudden hearing loss was attributed to iloprost use and completely reversed in eight days with conservative therapy. Iloprost may be a potentially ototoxic drug, causing sudden hearing loss.

Hearing rehabilitation in Treacher Collins Syndrome with bone anchored hearing aid

PubMed Central

Polanski, José Fernando; Plawiak, Anna Clara; Ribas, Angela

2015-01-01

Objective: To describe a case of hearing rehabilitation with bone anchored hearing aid in a patient with Treacher Collins syndrome. Case description: 3 years old patient, male, with Treacher Collins syndrome and severe complications due to the syndrome, mostly related to the upper airway and hearing. He had bilateral atresia of external auditory canals, and malformation of the pinna. The initial hearing rehabilitation was with bone vibration arch, but there was poor acceptance due the discomfort caused by skull compression. It was prescribed a model of bone-anchored hearing aid, in soft band format. The results were evaluated through behavioral hearing tests and questionnaires Meaningful Use of Speech Scale (MUSS) and Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS). Comments: The patient had a higher acceptance of the bone-anchored hearing aid compared to the traditional bone vibration arch. Audiological tests and the speech and auditory skills assessments also showed better communication and hearing outcomes. The bone-anchored hearing aid is a good option in hearing rehabilitation in this syndrome. PMID:26298651

Electroretinogram assessment of children with sensorineural hearing loss: implications for screening.

PubMed

West, Stephanie K; Hindocha, Maya; Hogg, Chris R; Holder, Graham E; Moore, Anthony T; Reddy, M Ashwin

2015-10-01

The guidelines of the National Deaf Children's Society recommend that children with sensorineural hearing loss (SNHL) be routinely screened for ophthalmological problems and suggest electroretinography (ERG) to exclude Usher syndrome. The present study reports the nature and prevalence of abnormal ERG findings in a cohort of children with SNHL undergoing ERG with the aim of identifying risk factors for the diagnosis of Usher syndrome. The medical records of children (<18 years of age) with SNHL referred for ERG at Moorfields Eye Hospital, London, between January 2009 and December 2011 were retrospectively reviewed. Patients were included if they had been referred with SNHL by an audiological medicine consultant and the primary indication for electrodiagnostic testing was possible Usher syndrome. A total of 84 cases met inclusion criteria of which 13 (15%) had ERG findings showing rod-cone dysfunction consistent with a diagnosis of Usher syndrome. Two patients with retinal pigmentary changes had normal ERGs and were diagnosed with rubella retinopathy based on the clinical findings. Risk factor analysis showed that age of ≥8 years at the time of ERG, sex, and bilateral hearing loss were not predictive of a diagnosis of Usher syndrome. However, the presence of or referral for cochlear implants, having relevant symptoms and/or clinical signs consistent with a retinal dystrophy, and profound hearing loss were all highly predictive. ERG is a useful diagnostic tool in children with SNHL and should be performed in children with SNHL who have cochlear implants and/or have signs or symptoms of retinal dystrophy. A focused approach could have potential cost-saving benefit. Copyright © 2015 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.

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

PubMed

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

2010-10-01

An objective investigation is needed to verify the existence and severity of hearing impairments resulting from work-related, noise-induced hearing loss in arbitration of medicolegal aspects. We investigated the accuracy of multiple-frequency auditory steady-state responses (Mf-ASSRs) between subjects with sensorineural hearing loss (SNHL) with and without occupational noise exposure. Cross-sectional study. Tertiary referral medical centre. Pure-tone audiometry and Mf-ASSRs were recorded in 88 subjects (34 patients had occupational noise-induced hearing loss [NIHL], 36 patients had SNHL without noise exposure, and 18 volunteers were normal controls). Inter- and intragroup comparisons were made. A predicting equation was derived using multiple linear regression analysis. ASSRs and pure-tone thresholds (PTTs) showed a strong correlation for all subjects (r = .77 ≈ .94). The relationship is demonstrated by the equationThe differences between the ASSR and PTT were significantly higher for the NIHL group than for the subjects with non-noise-induced SNHL (p < .001). Mf-ASSR is a promising tool for objectively evaluating hearing thresholds. Predictive value may be lower in subjects with occupational hearing loss. Regardless of carrier frequencies, the severity of hearing loss affects the steady-state response. Moreover, the ASSR may assist in detecting noise-induced injury of the auditory pathway. A multiple linear regression equation to accurately predict thresholds was shown that takes into consideration all effect factors.

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)

Effects of long-term non-traumatic noise exposure on the adult central auditory system. Hearing problems without hearing loss.

PubMed

Eggermont, Jos J

2017-09-01

It is known that hearing loss induces plastic changes in the brain, causing loudness recruitment and hyperacusis, increased spontaneous firing rates and neural synchrony, reorganizations of the cortical tonotopic maps, and tinnitus. Much less in known about the central effects of exposure to sounds that cause a temporary hearing loss, affect the ribbon synapses in the inner hair cells, and cause a loss of high-threshold auditory nerve fibers. In contrast there is a wealth of information about central effects of long-duration sound exposures at levels ≤80 dB SPL that do not even cause a temporary hearing loss. The central effects for these moderate level exposures described in this review include changes in central gain, increased spontaneous firing rates and neural synchrony, and reorganization of the cortical tonotopic map. A putative mechanism is outlined, and the effect of the acoustic environment during the recovery process is illustrated. Parallels are drawn with hearing problems in humans with long-duration exposures to occupational noise but with clinical normal hearing. Copyright © 2016 Elsevier B.V. All rights reserved.

Treatment-induced hearing loss and adult social outcomes in survivors of childhood CNS and non-CNS solid tumors: Results from the St. Jude Lifetime Cohort Study.

PubMed

Brinkman, Tara M; Bass, Johnnie K; Li, Zhenghong; Ness, Kirsten K; Gajjar, Amar; Pappo, Alberto S; Armstrong, Gregory T; Merchant, Thomas E; Srivastava, Deo Kumar; Robison, Leslie L; Hudson, Melissa M; Gurney, James G

2015-11-15

Survivors of childhood cancer who are treated with platinum-based chemotherapy and/or cranial radiation are at risk of treatment-induced hearing loss. However, the effects of such hearing loss on adult social attainment have not been well elucidated. Adult survivors of pediatric central nervous system (CNS) solid tumors (180 survivors) and non-CNS solid tumors (226 survivors) who were treated with potentially ototoxic cancer therapy completed audiologic evaluations and questionnaires assessing their perception of social functioning and social attainment (ie, independent living, marriage, and employment). Audiograms were graded with the Chang ototoxicity grading scale. Analyses were stratified by tumor type (ie, CNS vs non-CNS). Multivariable logistic regression models were conducted with adjustment for age; sex; chronic health conditions; and, for the CNS group, IQ. Adjusted odds ratios (ORs) and 95% confidence intervals (95% CIs) were reported. Serious hearing loss (that requiring a hearing aid or deafness) was detected in 36% of survivors of CNS tumors and 39% of survivors of non-CNS tumors. Serious hearing loss was associated with an increased risk of perceived negative impact in ≥1 areas of social functioning (survivors of non-CNS tumors: OR, 1.83 [95% CI, 1.00-3.34]). Among survivors of non-CNS tumors, serious hearing loss was associated with 2-fold increased risk of nonindependent living (OR, 2.19; 95% CI, 1.19-4.04) and unemployment or not graduating from high school (OR, 1.85; 95% CI, 1.00-3.34). A substantial proportion of adult survivors of childhood cancer treated with potentially ototoxic therapy have serious hearing loss. Treatment-induced hearing loss was found to be associated with reduced social attainment, both perceived and actual, in this study sample. © 2015 American Cancer Society.

Mobile phone usage does not affect sudden sensorineural hearing loss.

PubMed

Sagiv, D; Migirov, L; Madgar, O; Nakache, G; Wolf, M; Shapira, Y

2018-01-01

Recent studies found that mobile phone users had a significantly greater risk of having elevated thresholds in speech frequencies. This study investigated the correlation between the laterality of sudden sensorineural hearing loss, handedness and the preferred ear for mobile phone use. The study included all patients who presented with sudden sensorineural hearing loss to the Department of Otolaryngology - Head and Neck Surgery in our tertiary referral medical centre between 2014 and 2016. Patients were asked to indicate their dominant hand and preferred ear for mobile phone use. The study comprised 160 patients. No correlation was found between the dominant hand or preferred ear for mobile phone use and the side of sudden sensorineural hearing loss. There was no correlation between the side of the sudden sensorineural hearing loss (preferable or non-preferable for mobile phone use) and audiometric characteristics. No correlation was found between the laterality of ears used for mobile phone and sudden sensorineural hearing loss.

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.

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

PubMed

Church, M W; Kaltenbach, J A

1997-05-01

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

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.

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.

Heterogeneity in phenotype of usher-congenital hyperinsulinism syndrome: hearing loss, retinitis pigmentosa, and hyperinsulinemic hypoglycemia ranging from severe to mild with conversion to diabetes.

PubMed

Al Mutair, Angham N; Brusgaard, Klaus; Bin-Abbas, Bassam; Hussain, Khalid; Felimban, Naila; Al Shaikh, Adnan; Christesen, Henrik T

2013-03-01

To evaluate the phenotype of 15 children with congenital hyperinsulinism (CHI) and profound hearing loss, known as Homozygous 11p15-p14 Deletion syndrome (MIM #606528). Prospective clinical follow-up and genetic analysis by direct sequencing, multiplex ligation-dependent probe amplification, and microsatellite markers. Genetic testing identified the previous described homozygous deletion in 11p15, USH1C:c.(90+592)_ABCC8:c.(2694-528)del. Fourteen patients had severe CHI demanding near-total pancreatectomy. In one patient with mild, transient neonatal hypoglycemia and nonautoimmune diabetes at age 11 years, no additional mutations were found in HNF1A, HNF4A, GCK, INS, and INSR. Retinitis pigmentosa was found in two patients aged 9 and 13 years. No patients had enteropathy or renal tubular defects. Neuromotor development ranged from normal to severe delay with epilepsy. The phenotype of Homozygous 11p15-p14 Deletion syndrome, or Usher-CHI syndrome, includes any severity of neonatal-onset CHI and severe, sensorineural hearing loss. Retinitis pigmentosa and nonautoimmune diabetes may occur in adolescence.

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.

[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

Cochlear Implantation in Adults with Asymmetric Hearing Loss

PubMed Central

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

2012-01-01

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

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.

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.

Asymmetric Hearing During Development: The Aural Preference Syndrome and Treatment Options.

PubMed

Gordon, Karen; Henkin, Yael; Kral, Andrej

2015-07-01

Deafness affects ∼2 in 1000 children and is one of the most common congenital impairments. Permanent hearing loss can be treated by fitting hearing aids. More severe to profound deafness is an indication for cochlear implantation. Although newborn hearing screening programs have increased the identification of asymmetric hearing loss, parents and caregivers of children with single-sided deafness are often hesitant to pursue therapy for the deaf ear. Delayed intervention has consequences for recovery of hearing. It has long been reported that asymmetric hearing loss/single-sided deafness compromises speech and language development and educational outcomes in children. Recent studies in animal models of deafness and in children consistently show evidence of an "aural preference syndrome" in which single-sided deafness in early childhood reorganizes the developing auditory pathways toward the hearing ear, with weaker central representation of the deaf ear. Delayed therapy consequently compromises benefit for the deaf ear, with slow rates of improvement measured over time. Therefore, asymmetric hearing needs early identification and intervention. Providing early effective stimulation in both ears through appropriate fitting of auditory prostheses, including hearing aids and cochlear implants, within a sensitive period in development has a cardinal role for securing the function of the impaired ear and for restoring binaural/spatial hearing. The impacts of asymmetric hearing loss on the developing auditory system and on spoken language development have often been underestimated. Thus, the traditional minimalist approach to clinical management aimed at 1 functional ear should be modified on the basis of current evidence. Copyright © 2015 by the American Academy of Pediatrics.

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…

Prevention of the Evolution of Workers' Hearing Loss from Noise-Induced Hearing Loss in Noisy Environments through a Hearing Conservation Program

PubMed Central

Fonseca, Vinicius Ribas; Marques, Jair; Panegalli, Flavio; Gonçalves, Claudia Giglio de Oliveira; Souza, Wesley

2015-01-01

Introduction Noise-induced hearing loss (NIHL) is a serious problem for workers and therefore for businesses. The hearing conservation program (HCP) is a set of coordinated measures to prevent the development or evolution of occupational hearing loss, which involves a continuous and dynamic process of implementation of hearing conservation routines through anticipation, recognition, evaluation, and subsequent control of the occurrence of existing environmental risks or of those that may exist in the workplace and lead to workers' hearing damage. Objective The aim of this study was to evaluate the effectiveness of the HCP in preventing further hearing loss in workers with audiograms suggestive of NIHL. The audiometric tests and medical records of 28 furniture company workers exposed to noise were reviewed and monitored for 2 years. Methods This retrospective, cross-sectional study examined five audiometric tests in the medical records (on admission and every semester) of 28 workers in a furniture company (totaling 140 audiometric exams) following the introduction of the HCP. Results Data analysis showed no differences between the audiometric tests conducted on admission and those performed every semester. Conclusions The HCP implemented was effective in preventing the worsening of hearing loss in workers already with NIHL when exposed to occupational noise. Therefore, such a measure could be useful for the employment of workers with hearing loss in job sectors that have noise exposure. PMID:26722345

"Gun-shooting hearing loss": A pilot study.

PubMed

Sataloff, Joseph; Hawkshaw, Mary J; Sataloff, Robert T

2010-01-01

Gun-shooting deafness is the common terminology applied to sensorineural hearing loss caused by shooting firearms. Many characteristics of gun-shooting hearing loss have been proposed, but they have not been defined clearly or established conclusively. We studied 37 users of recreational firearms to obtain pilot data to help determine if it is true that right-handed gun shooters develop more hearing loss in the left ear and vice versa, whether everyone who frequently shoots guns develops sensorineural hearing loss, and whether significant hearing loss is typically prevented by wearing commercially available ear protectors while shooting.

The effect of novel mutations on the structure and enzymatic activity of unconventional myosins associated with autosomal dominant non-syndromic hearing loss.

PubMed

Kwon, Tae-Jun; Oh, Se-Kyung; Park, Hong-Joon; Sato, Osamu; Venselaar, Hanka; Choi, Soo Young; Kim, SungHee; Lee, Kyu-Yup; Bok, Jinwoong; Lee, Sang-Heun; Vriend, Gert; Ikebe, Mitsuo; Kim, Un-Kyung; Choi, Jae Young

2014-07-01

Mutations in five unconventional myosin genes have been associated with genetic hearing loss (HL). These genes encode the motor proteins myosin IA, IIIA, VI, VIIA and XVA. To date, most mutations in myosin genes have been found in the Caucasian population. In addition, only a few functional studies have been performed on the previously reported myosin mutations. We performed screening and functional studies for mutations in the MYO1A and MYO6 genes in Korean cases of autosomal dominant non-syndromic HL. We identified four novel heterozygous mutations in MYO6. Three mutations (p.R825X, p.R991X and Q918fsX941) produce a premature truncation of the myosin VI protein. Another mutation, p.R205Q, was associated with diminished actin-activated ATPase activity and actin gliding velocity of myosin VI in an in vitro analysis. This finding is consistent with the results of protein modelling studies and corroborates the pathogenicity of this mutation in the MYO6 gene. One missense variant, p.R544W, was found in the MYO1A gene, and in silico analysis suggested that this variant has deleterious effects on protein function. This finding is consistent with the results of protein modelling studies and corroborates the pathogenic effect of this mutation in the MYO6 gene.

The effect of novel mutations on the structure and enzymatic activity of unconventional myosins associated with autosomal dominant non-syndromic hearing loss

PubMed Central

Kwon, Tae-Jun; Oh, Se-Kyung; Park, Hong-Joon; Sato, Osamu; Venselaar, Hanka; Choi, Soo Young; Kim, SungHee; Lee, Kyu-Yup; Bok, Jinwoong; Lee, Sang-Heun; Vriend, Gert; Ikebe, Mitsuo; Kim, Un-Kyung; Choi, Jae Young

2014-01-01

Mutations in five unconventional myosin genes have been associated with genetic hearing loss (HL). These genes encode the motor proteins myosin IA, IIIA, VI, VIIA and XVA. To date, most mutations in myosin genes have been found in the Caucasian population. In addition, only a few functional studies have been performed on the previously reported myosin mutations. We performed screening and functional studies for mutations in the MYO1A and MYO6 genes in Korean cases of autosomal dominant non-syndromic HL. We identified four novel heterozygous mutations in MYO6. Three mutations (p.R825X, p.R991X and Q918fsX941) produce a premature truncation of the myosin VI protein. Another mutation, p.R205Q, was associated with diminished actin-activated ATPase activity and actin gliding velocity of myosin VI in an in vitro analysis. This finding is consistent with the results of protein modelling studies and corroborates the pathogenicity of this mutation in the MYO6 gene. One missense variant, p.R544W, was found in the MYO1A gene, and in silico analysis suggested that this variant has deleterious effects on protein function. This finding is consistent with the results of protein modelling studies and corroborates the pathogenic effect of this mutation in the MYO6 gene. PMID:25080041

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.

Noise-induced hearing loss and combined noise and vibration exposure.

PubMed

Turcot, A; Girard, S A; Courteau, M; Baril, J; Larocque, R

2015-04-01

While there is a wide body of literature addressing noise-induced hearing loss (NIHL) and hand-arm vibration syndrome (HAVS) independently, relatively few studies have considered the combined effects of noise and vibration. These studies have suggested an increased risk of NIHL in workers with vibration white finger (VWF), though the relationship remains poorly understood. To determine whether hearing impairment is worse in noise-exposed workers with VWF than in workers with similar noise exposures but without VWF. The Quebec National Institute of Public Health audiometric database was used in conjunction with work-related accident and occupational diseases data from the Quebec workers' compensation board to analyse differences in audiometry results between vibration-exposed workers in the mining and forestry industries and the overall source population, and between mining and forestry workers with documented VWF and those without VWF. The International Organization for Standardization (ISO) 7029 standards were used to calculate hearing loss not attributable to age. 15751 vibration-exposed workers were identified in an overall source population of 59339. Workers with VWF (n = 96) had significantly worse hearing at every frequency studied (500, 1000, 2000 4000 Hz) compared with other mining and forestry workers without VWF. This study confirms previous findings of greater hearing loss at higher frequencies in workers with VWF, but also found a significant difference in hearing loss at low frequencies. It therefore supports the association between combined noise and hand-arm vibration (HAV) exposure and NIHL. © The Author 2015. Published by Oxford University Press on behalf of the Society of Occupational Medicine. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Leupeptin reduces impulse noise induced hearing loss

PubMed Central

2011-01-01

Background Exposure to continuous and impulse noise can induce a hearing loss. Leupeptin is an inhibitor of the calpains, a family of calcium-activated proteases which promote cell death. The objective of this study is to assess whether Leupeptin could reduce the hearing loss resulting from rifle impulse noise. Methods A polyethelene tube was implanted into middle ear cavities of eight fat sand rats (16 ears). Following determination of auditory nerve brainstem evoked response (ABR) threshold in each ear, the animals were exposed to the noise of 10 M16 rifle shots. Immediately after the exposure, saline was then applied to one (control) ear and non-toxic concentrations of leupeptin determined in the first phase of the study were applied to the other ear, for four consecutive days. Results Eight days after the exposure, the threshold shift (ABR) in the control ears was significantly greater (44 dB) than in the leupeptin ears (27 dB). Conclusion Leupeptin applied to the middle ear cavity can reduce the hearing loss resulting from exposure to impulse noise. PMID:22206578

Hearing Abilities of Down Syndrome and Other Mentally Handicapped Adolescents.

ERIC Educational Resources Information Center

Marcell, Michael M.; And Others

This study explored the hearing capabilities of Down Syndrome (DS) adolescents and young adults relative to a matched sample of non-DS trainable mentally handicapped (MH) individuals, and examined the relationship between hearing ability and performance on several cognitive tasks. Samples of 26 DS and 26 MH individuals were matched on intelligence…

Hearing loss in shipyard employees

PubMed Central

Alexopoulos, Evangelos C.; Tsouvaltzidou, Thomaella

2015-01-01

Background: Noise-induced hearing loss (NIHL) is one of the most prevalent occupational illnesses, with a higher incidence in the heavy industry. Objectives of the Study: The aim of this study is to investigate the prevalence of NIHL in Greece and explore its correlations with other job and individual-related factors. Materials and Methods: Questionnaires were administered, and audiograms were conducted to 757 employees of a shipyard company in Greece, both white- and blue-collar, during the period 2006–2009. A modification of the 1979' equation of the American Academy of Otolaryngology was used to calculate hearing loss. Statistical analysis was conducted by means of the SPSS v. 17. Results: A 27.1% of the employees were hearing handicap. Hearing loss was correlated with age, past medical history of ear disease (Meniere's disease, acoustic neuroma, otosclerosis) or injury, hyperlipidemia, job title and level of education. A few questions on subjective hearing ability and symptoms showed strong discriminatory power of hearing pathology. Conclusions: The results of this study emphasize the burden of disease in the shipyard industry, and the need for continuous monitoring, implementation of preventive measures and hearing conservation programs. PMID:26023266

[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.

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

Age-related hearing loss

MedlinePlus

... both physical (not hearing a fire alarm) and psychological (social isolation) problems. The hearing loss may lead ... accountability. A.D.A.M. is among the first to achieve this important distinction for online health ...

"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.

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.

Psychosocial Development in 5-Year-Old Children With Hearing Loss Using Hearing Aids or Cochlear Implants.

PubMed

Wong, Cara L; Ching, Teresa Y C; Cupples, Linda; Button, Laura; Leigh, Greg; Marnane, Vivienne; Whitfield, Jessica; Gunnourie, Miriam; Martin, Louise

2017-01-01

This article reports on the psychosocial development and factors influencing outcomes of 5-year-old children with cochlear implants (CIs) or hearing aids (HAs). It further examines differences between children with CIs and HAs with similar levels of hearing loss. Data were collected as part of the Longitudinal Outcomes of Children with Hearing Impairment study-a prospective, population-based study. Parents/caregivers of children completed the Strengths and Difficulties Questionnaire ( n = 333), the Social Skills subscale from the Child Development Inventory ( n = 317), and questionnaires on functional auditory behavior (Parents' Evaluation of Aural/oral performance of Children), and demographics. Children completed assessments of nonverbal cognitive ability (Wechsler Non-verbal Scale of Ability) and language (Preschool Language Scale - fourth edition). On average, parent-rated Strengths and Difficulties Questionnaire scores on emotional or behavioral difficulties were within 1 SD of the normative mean; however, Child Development Inventory scores on social skills were more than 1 SD below the norm. Children with severe-to-profound hearing losses using HAs had significantly more behavioral problems than children with CIs. Regression analyses showed that non-verbal cognitive ability, language, and functional auditory behavior were significantly associated with psychosocial outcomes for children with HAs, whereas outcomes for children with CIs were associated with functional auditory behavior and the presence of additional disabilities. Age at hearing intervention, severity of hearing loss, and communication mode were not associated with outcomes. The results suggest that even children who develop good language ability with the help of a HA or CI may have psychosocial problems if they exhibit difficulties with listening and communicating in everyday environments. The findings have implications for developing interventions for young children with hearing

Psychosocial Development in 5-Year-Old Children With Hearing Loss Using Hearing Aids or Cochlear Implants

PubMed Central

Ching, Teresa Y. C.; Cupples, Linda; Button, Laura; Leigh, Greg; Marnane, Vivienne; Whitfield, Jessica; Gunnourie, Miriam; Martin, Louise

2017-01-01

This article reports on the psychosocial development and factors influencing outcomes of 5-year-old children with cochlear implants (CIs) or hearing aids (HAs). It further examines differences between children with CIs and HAs with similar levels of hearing loss. Data were collected as part of the Longitudinal Outcomes of Children with Hearing Impairment study—a prospective, population-based study. Parents/caregivers of children completed the Strengths and Difficulties Questionnaire (n = 333), the Social Skills subscale from the Child Development Inventory (n = 317), and questionnaires on functional auditory behavior (Parents’ Evaluation of Aural/oral performance of Children), and demographics. Children completed assessments of nonverbal cognitive ability (Wechsler Non-verbal Scale of Ability) and language (Preschool Language Scale - fourth edition). On average, parent-rated Strengths and Difficulties Questionnaire scores on emotional or behavioral difficulties were within 1 SD of the normative mean; however, Child Development Inventory scores on social skills were more than 1 SD below the norm. Children with severe-to-profound hearing losses using HAs had significantly more behavioral problems than children with CIs. Regression analyses showed that non-verbal cognitive ability, language, and functional auditory behavior were significantly associated with psychosocial outcomes for children with HAs, whereas outcomes for children with CIs were associated with functional auditory behavior and the presence of additional disabilities. Age at hearing intervention, severity of hearing loss, and communication mode were not associated with outcomes. The results suggest that even children who develop good language ability with the help of a HA or CI may have psychosocial problems if they exhibit difficulties with listening and communicating in everyday environments. The findings have implications for developing interventions for young children with hearing

Noise-Induced Hearing Loss

MedlinePlus

... to noise. The NIDCD sponsors It's a Noisy Planet. Protect Their Hearing® , a national public education campaign ... induced hearing loss is 100% preventable. NIDCD's Noisy Planet website Have a question? Information specialists can answer ...

Effectiveness of alternative listening devices to conventional hearing aids for adults with hearing loss: a systematic review protocol

PubMed Central

Barker, Alex B; Xia, Jun

2016-01-01

Introduction Hearing loss is a major public health concern, affecting over 11 million people in the UK. While hearing aids are the most common clinical intervention for hearing loss, the majority of people that would benefit from using hearing aids do not take them up. Recent technological advances have led to a rapid increase of alternative listening devices to conventional hearing aids. These include hearing aids that can be customised using a smartphone, smartphone-based ‘hearing aid’ apps, personal sound amplification products and wireless hearing products. However, no systematic review has been published evaluating whether alternative listening devices are an effective management strategy for people with hearing loss. Methods and analysis The objective of this systematic review is to assess whether alternative listening devices are an effective intervention for adults with hearing loss. Methods are reported according to the Preferred Reporting Items for Systematic reviews and Meta-analyses Protocols (PRISMA-P) 2015 checklist. Retrospective or prospective studies, randomised controlled trials, non-randomised controlled trials, and before-after comparison studies will be eligible for inclusion. We will include studies with adult participants (≥18 years) with a mild or moderate hearing loss. The intervention should be an alternative listening device to a conventional hearing aid (comparison). Studies will be restricted to outcomes associated with the consequences of hearing loss. We will search relevant databases to identify published, completed but unpublished and ongoing trials. The overall quality of included evidence will be evaluated using the GRADE system, and meta-analysis performed if appropriate. Ethics and dissemination No ethical issues are foreseen. The findings will be reported at national and international conferences, primarily audiology, and ear, nose and throat, and in a peer-reviewed journal using the PRISMA guidelines. Review

‘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

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.

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.

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.

The Stigma of Hearing Loss

ERIC Educational Resources Information Center

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…

Noise-Induced Hearing Loss (NIHL).

ERIC Educational Resources Information Center

Seidman, Michael D.

1999-01-01

This article provides an overview of noise-induced hearing loss (NIHL), the leading cause of occupationally induced hearing loss in industrialized countries. It discusses causes of NIHL and compelling evidence that reactive oxygen metabolites and cochlear hypoprefusion are responsible for the destruction of cochlear hair cells. Prevention is also…

The hearing benefit of cochlear implantation for individuals with unilateral hearing loss, but no tinnitus.

PubMed

Skarzynski, Henryk; Lorens, Artur; Kruszynska, Marika; Obrycka, Anita; Pastuszak, Dorota; Skarzynski, Piotr Henryk

2017-07-01

Cochlear implants improve the hearing abilities of individuals with unilateral hearing loss and no tinnitus. The benefit is no different from that seen in patients with unilateral hearing loss and incapacitating tinnitus. To evaluate hearing outcomes after cochlear implantation in individuals with unilateral hearing loss and no tinnitus and compare them to those obtained in a similar group who had incapacitating tinnitus. Six cases who did not experience tinnitus before operation and 15 subjects with pre-operative tinnitus were evaluated with a structured interview, a monosyllabic word test under difficult listening situations, a sound localization test, and an APHAB (abbreviated profile of hearing aid benefit) questionnaire. All subjects used their cochlear implant more than 8 hours a day, 7 days a week. In 'no tinnitus' patients, mean benefit of cochlear implantation was 19% for quiet speech, 15% for speech in noise (with the same signal-to-noise ratio in the implanted and non-implanted ear), and 16% for a more favourable signal-to-noise ratio at the implanted ear. Sound localization error improved by an average of 19°. The global score of APHAB improved by 16%. The benefits across all evaluations did not differ significantly between the 'no tinnitus' and 'tinnitus' groups.

Sociodemographic correlates of hearing loss and hearing aid use in Hispanic adults.

PubMed

Lee, D J; Gómez-Marín, O; Lee, H M

1996-07-01

We examined sociodemographic correlates of hearing loss and hearing aid use in adult Hispanics using data from the Hispanic Health and Nutrition Examination Survey. After adjustment for age and gender, odds ratios (ORs) of hearing impairment for non-married vs married participants were 1.6 [95% confidence interval (CI) = 1.0-2.5] for Mexican-Americans and 2.2 (95% CI = 1.2-4.2) for Puerto Ricans. ORs ranged from 1.6 to 3.2 for unemployed vs employed, and from 1.7 to 2.1 for uninsured vs insured Hispanics. For Mexican-Americans, the OR of hearing aid use for those living below vs above the poverty line was 9.1 (95% CI = 1.1-100.0). These results are consistent with those reported for other ethnic groups.

Noise-Induced Hearing Loss

MedlinePlus

... signals Identify sources of loud sounds (such as gas-powered lawnmowers, snowmobiles, power tools, gunfire, or music) that can contribute to hearing loss and try to reduce exposure Adopt behaviors to protect their hearing: Avoid or limit exposure ...

Hearing loss caused by a P2RX2 mutation identified in a MELAS family with a coexisting mitochondrial 3243AG mutation

PubMed Central

Moteki, Hideaki; Azaiez, Hela; Booth, Kevin T; Hattori, Mitsuru; Sato, Ai; Sato, Yoshihiko; Motobayashi, Mitsuo; Sloan, Christina M; Kolbe, Diana L; Shearer, A Eliot; Smith, Richard J H; Usami, Shin-ichi

2015-01-01

Objective We present a family with a mitochondrial DNA 3243A>G mutation resulting in MELAS, of which some members have hearing loss where a novel mutation in the P2RX2 gene was identified. Methods One hundred ninety-four (194) Japanese subjects from unrelated families were enrolled in the study. Targeted genomic enrichment and massively parallel sequencing of all known non-syndromic hearing loss genes were performed to identify the genetic causes of hearing loss. Results A novel mutation in the P2RX2 gene, that corresponded to c.601G>A (p.Asp201Tyr) was identified. Two patients carried the mutation, and had severe SNHL, while other members with MELAS (who did not carry the P2RX2 mutation) had normal hearing. Conclusion This is the first case report of a diagnosis of hearing loss caused by P2RX2 mutation in patients with MELAS. A potential explanation is that decreasing ATP production due to MELAS with mitochondrial 3243A>G mutation might suppress activation of P2X2 receptors. We also suggest that hearing loss caused by the P2RX2 mutation might be influenced by the decrease in ATP production due to MELAS, and that nuclear genetic factors may play a modifying role in mitochondrial dysfunction. PMID:25788561

Navigating Your Child's Hearing Loss Diagnosis

ERIC Educational Resources Information Center

Trapp Petty, Melissa A.

2011-01-01

For hearing parents, receiving a hearing loss diagnosis for their child can be a shocking event. For some parents, the diagnosis is the fulfillment of a hunch; confirmation of the suspected, but still scary verdict. Recent research finds that the period directly after hearing loss diagnosis is the most stressful and burdensome for parents,…

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.

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.

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.

Hearing-loss-associated gene detection in neonatal intensive care unit.

PubMed

Yang, S M; Liu, Ying; Liu, C; Yin, A H; Wu, Y F; Zheng, X E; Yang, H M; Yang, J

2018-02-01

To investigate the frequency and mutation spectrum of hearing loss-associated gene mutation in Neonatal Intensive Care Unit (NICU). Neonates (n=2305) admitted to NICU were enrolled in this study. Nine prominent hearing loss-associated genes, GJB2 (35 del G, 176 del 16,235 del C, 299 del AT), GJB3 (538 C > T), SLC26A4 (IVS7-2A > G, 2168 A > G) and mtDNA 12S rRNA(1555 A > G, 1494 C > T), were detected. There were 73 cases hearing-loss-associated gene mutation among 2305 cases, the mutation frequency was 3.1%, with 40 cases GJB2 (235del C) mutation (54.8%), 6 cases GJB2 (299 del AT) mutation (8.2%), 21 cases SLC26A4 (IVS 7-2 A > G) mutation (28.7%), 4 cases SLC26A4 (2168 A > G) mutation (5.5%), 2 cases of GJB2 (235del C) combined SLC26A4 (IVS 7-2 A > G, 2168 A > G) mutation (2.8%). Among 73 gene mutation cases, preterm neonates presented in 18 cases, accounting for 24.7% (18/73); hyperbilirubinemia in 13 cases, accounting for 17.8% (13/73); Torch Syndrome in 15 cases, with 12 cases CMV, 2 cases rubella, 1 case toxoplasm, respectively, totally accounting for 20.54% (15/73); neonatal pneumonia in 12 cases, accounting for 16.4% (12/73); birth asphyxia in 5 cases, accounting for 6.9% (5/73); sepsis in 5 cases, accounting for 6.9% (5/73); others in 5 cases, accounting for 6.8% (5/73) . The frequency of hearing loss-associated gene mutation was higher in NICU.There were hearing loss-associated gene mutations in the NICU, suggesting this mutation may complicate with perinatal high-risk factors.

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.

Interventions to prevent occupational noise-induced hearing loss: A Cochrane systematic review

PubMed Central

Verbeek, Jos H.; Kateman, Erik; Morata, Thais C.; Dreschler, Wouter A.; Mischke, Christina

2015-01-01

Objective To assess the effectiveness of interventions for preventing occupational noise exposure or hearing loss compared to no intervention or alternative interventions. Design We searched biomedical databases up to 25 January 2012 for randomized controlled trials (RCT), controlled before-after studies and interrupted time-series of hearing loss prevention among workers exposed to noise. Study sample We included 19 studies with 82 794 participants evaluating effects of hearing loss prevention programs (HLPP). The overall quality of studies was low to very low, as rated using the GRADE approach. Results One study of stricter legislation showed a favorable effect on noise levels. Three studies, of which two RCTs, did not find an effect of a HLPP. Four studies showed that better use of hearing protection devices in HLPPs decreased the risk of hearing loss. In four other studies, workers in a HLPP still had a 0.5 dB greater hearing loss at 4 kHz (95% CI – 0.5 to 1.7) than non-exposed workers. In two similar studies there was a substantial risk of hearing loss in spite of a HLPP. Conclusions Stricter enforcement of legislation and better implementation of HLPPs can reduce noise levels in workplaces. Better evaluations of technical interventions and long-term effects are needed. PMID:24564697

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.

Digenic inheritance in autosomal recessive non-syndromic hearing loss cases carrying GJB2 heterozygote mutations: assessment of GJB4, GJA1, and GJC3.

PubMed

Kooshavar, Daniz; Tabatabaiefar, Mohammad Amin; Farrokhi, Effat; Abolhasani, Marziye; Noori-Daloii, Mohammad-Reza; Hashemzadeh-Chaleshtori, Morteza

2013-02-01

Autosomal recessive non-syndromic hearing loss (ARNSHL) can be caused by many genes. However, mutations in the GJB2 gene, which encodes the gap-junction (GJ) protein connexin (Cx) 26, constitute a considerable proportion differing among population. Between 10 and 42 percent of patients with recessive GJB2 mutations carry only one mutant allele. Mutations in GJB4, GJA1, and GJC3 encoding Cx30.3, Cx43, and Cx29, respectively, can lead to HL. Combination of different connexins in heteromeric and heterotypic GJ assemblies is possible. This study aims to determine whether variations in any of the genes GJB4, GJA1 or GJC3 can be the second mutant allele causing the disease in the digenic mode of inheritance in the studied GJB2 heterozygous cases. We examined 34 unrelated GJB2 heterozygous ARNSHL subjects from different geographic and ethnic areas in Iran, using polymerase chain reaction (PCR) followed by direct DNA sequencing to identify any sequence variations in these genes. Restriction fragment length polymorphism (RFLP) assays were performed on 400 normal hearing individuals. Sequence analysis of GJB4 showed five heterozygous variations including c.451C>A, c.219C>T, c.507C>G, c.155_158delTCTG and c.542C>T, with only the latter variation not being detected in any of control samples. There were three heterozygous variations including c.758C>T, c.717G>A and c.3*dupA in GJA1 in four cases. We found no variations in GJC3 gene sequence. Our data suggest that GJB4 c.542C>T variant and less likely some variations of GJB4 and GJA1, but not possibly GJC3, can be assigned to ARNSHL in GJB2 heterozygous mutation carriers providing clues of the digenic pattern. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

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. ...

Characteristics of children with unilateral hearing loss.

PubMed

Fitzpatrick, Elizabeth M; Al-Essa, Rakan S; Whittingham, JoAnne; Fitzpatrick, Jessica

2017-11-01

The purpose of this study was to describe the clinical characteristics of children with unilateral hearing loss (UHL), examine deterioration in hearing, and explore amplification decisions. Population-based data were collected prospectively from time of diagnosis. Serial audiograms and amplification details were retrospectively extracted from clinical charts to document the trajectory and management of hearing loss. The study included all children identified with UHL in one region of Canada over a 13-year period (2003-2015) after implementation of universal newborn hearing screening. Of 537 children with permanent hearing loss, 20.1% (108) presented with UHL at diagnosis. They were identified at a median age of 13.9 months (IQR: 2.8, 49.0). Children with congenital loss were identified at 2.8 months (IQR: 2.0, 3.6) and made up 47.2% (n = 51), reflecting that a substantial portion had late-onset, acquired or late-identified loss. A total of 42.4% (n = 39) showed deterioration in hearing, including 16 (17.4%) who developed bilateral loss. By study end, 73.1% (79/108) of children had received amplification recommendations. Up to 20% of children with permanent HL are first diagnosed with UHL. About 40% are at risk for deterioration in hearing either in the impaired ear and/or in the normal hearing ear.

Does Tinnitus, Hearing Asymmetry or Hearing Loss Predispose to Occupational Injury Risk?

PubMed Central

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

2015-01-01

Objective To determine the relative contributions of tinnitus, asymmetrical hearing loss, low frequency hearing loss (pure tone average of .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. Design and Study Sample This retrospective analysis considered 9,920 workers employed during 2003 to 2008. The cohort consisted of 8,818 workers (89%) whose complete records were available. Results 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. Conclusion 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. PMID:25549168

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

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.

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

Effectiveness of alternative listening devices to conventional hearing aids for adults with hearing loss: a systematic review protocol.

PubMed

Maidment, David W; Barker, Alex B; Xia, Jun; Ferguson, Melanie A

2016-10-27

Hearing loss is a major public health concern, affecting over 11 million people in the UK. While hearing aids are the most common clinical intervention for hearing loss, the majority of people that would benefit from using hearing aids do not take them up. Recent technological advances have led to a rapid increase of alternative listening devices to conventional hearing aids. These include hearing aids that can be customised using a smartphone, smartphone-based 'hearing aid' apps, personal sound amplification products and wireless hearing products. However, no systematic review has been published evaluating whether alternative listening devices are an effective management strategy for people with hearing loss. The objective of this systematic review is to assess whether alternative listening devices are an effective intervention for adults with hearing loss. Methods are reported according to the Preferred Reporting Items for Systematic reviews and Meta-analyses Protocols (PRISMA-P) 2015 checklist. Retrospective or prospective studies, randomised controlled trials, non-randomised controlled trials, and before-after comparison studies will be eligible for inclusion. We will include studies with adult participants (≥18 years) with a mild or moderate hearing loss. The intervention should be an alternative listening device to a conventional hearing aid (comparison). Studies will be restricted to outcomes associated with the consequences of hearing loss. We will search relevant databases to identify published, completed but unpublished and ongoing trials. The overall quality of included evidence will be evaluated using the GRADE system, and meta-analysis performed if appropriate. No ethical issues are foreseen. The findings will be reported at national and international conferences, primarily audiology, and ear, nose and throat, and in a peer-reviewed journal using the PRISMA guidelines. PROSPERO CRD4201502958. Published by the BMJ Publishing Group Limited. For

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

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.

Noise-induced hearing loss.

PubMed

Sliwinska-Kowalska, Mariola; Davis, Adrian

2012-01-01

Noise-induced hearing loss (NIHL) still remains a problem in developed countries, despite reduced occupational noise exposure, strict standards for hearing protection and extensive public health awareness campaigns. Therefore NIHL continues to be the focus of noise research activities. This paper summarizes progress achieved recently in our knowledge of NIHL. It includes papers published between the years 2008-2011 (in English), which were identified by a literature search of accessible medical and other relevant databases. A substantial part of this research has been concerned with the risk of NIHL in the entertainment sector, particularly in professional, orchestral musicians. There are also constant concerns regarding noise exposure and hearing risk in "hard to control" occupations, such as farming and construction work. Although occupational noise has decreased since the early 1980s, the number of young people subject to social noise exposure has tripled. If the exposure limits from the Noise at Work Regulations are applied, discotheque music, rock concerts, as well as music from personal music players are associated with the risk of hearing loss in teenagers and young adults. Several recent research studies have increased the understanding of the pathomechanisms of acoustic trauma, the genetics of NIHL, as well as possible dietary and pharmacologic otoprotection in acoustic trauma. The results of these studies are very promising and offer grounds to expect that targeted therapies might help prevent the loss of sensory hair cells and protect the hearing of noise-exposed individuals. These studies emphasize the need to launch an improved noise exposure policy for hearing protection along with developing more efficient norms of NIHL risk assessment.

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

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.

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.

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.

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

Diagnosis of Hearing Loss.

ERIC Educational Resources Information Center

World Federation of the Deaf, Rome (Italy).

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

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.

What's Hearing Loss?

MedlinePlus

... cells in the cochlea by turning sounds into electrical signals that stimulate the hearing nerve directly. Learning ... loss can read along to follow the action. Technology is changing all the time, and you will ...

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

PubMed

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

2017-07-01

The co-occurrence of hearing impairment and visual dysfunction is devastating. Most deaf-blind etiologies are genetically determined, the commonest being Usher syndrome (USH). While studies of the congenitally deaf population reveal a variable degree of visual problems, there are no effective ophthalmic screening guidelines. We hypothesized that children with congenital sensorineural hearing loss (SNHL) and vestibular impairment were at an increased risk of having USH. A retrospective chart review of 33 cochlear implants recipients for severe to profound SNHL and measured vestibular dysfunction was performed to determine the ocular phenotype. All the cases had undergone ocular examination and electroretinogram (ERG). Patients with an abnormal ERG underwent genetic testing for USH. We found an underlying ocular abnormality in 81.81% (27/33) of cases; of which 75% had refractive errors, and 50% of those patients showed visual improvement with refractive correction. A total of 14 cases (42.42%; 14/33) had generalized rod-cone dysfunction on ERG suggestive of Usher syndrome type 1, confirmed by mutational analysis. This work shows that adding vestibular impairment as a criterion for requesting an eye exam and adding the ERG to detect USH increases the chances of detecting ocular anomalies, when compared with previous literature focusing only on congenital SNHL. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

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

PubMed Central

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

2015-01-01

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

Phonological processes in the speech of school-age children with hearing loss: Comparisons with children with normal hearing.

PubMed

Asad, Areej Nimer; Purdy, Suzanne C; Ballard, Elaine; Fairgray, Liz; Bowen, Caroline

2018-04-27

In this descriptive study, phonological processes were examined in the speech of children aged 5;0-7;6 (years; months) with mild to profound hearing loss using hearing aids (HAs) and cochlear implants (CIs), in comparison to their peers. A second aim was to compare phonological processes of HA and CI users. Children with hearing loss (CWHL, N = 25) were compared to children with normal hearing (CWNH, N = 30) with similar age, gender, linguistic, and socioeconomic backgrounds. Speech samples obtained from a list of 88 words, derived from three standardized speech tests, were analyzed using the CASALA (Computer Aided Speech and Language Analysis) program to evaluate participants' phonological systems, based on lax (a process appeared at least twice in the speech of at least two children) and strict (a process appeared at least five times in the speech of at least two children) counting criteria. Developmental phonological processes were eliminated in the speech of younger and older CWNH while eleven developmental phonological processes persisted in the speech of both age groups of CWHL. CWHL showed a similar trend of age of elimination to CWNH, but at a slower rate. Children with HAs and CIs produced similar phonological processes. Final consonant deletion, weak syllable deletion, backing, and glottal replacement were present in the speech of HA users, affecting their overall speech intelligibility. Developmental and non-developmental phonological processes persist in the speech of children with mild to profound hearing loss compared to their peers with typical hearing. The findings indicate that it is important for clinicians to consider phonological assessment in pre-school CWHL and the use of evidence-based speech therapy in order to reduce non-developmental and non-age-appropriate developmental processes, thereby enhancing their speech intelligibility. Copyright © 2018 Elsevier Inc. All rights reserved.

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.

Uncovering effective strategies for hearing loss prevention

PubMed Central

Morata, Thais C.; Meinke, Deanna

2016-01-01

Occupational health agencies, researchers and policy makers have recognized the need for evidence on the effectiveness of interventions designed to reduce or prevent workplace injuries and illnesses. While many workplaces comply with legal or obligatory requirements and implement recommended interventions, few publications exist documenting the effectiveness of these actions. Additionally, some workplaces have discovered through their own processes, novel ways to reduce the risk of injury. Peer-reviewed information on the effectiveness of the many strategies and approaches currently in use could help correct weaknesses, or further encourage their adoption and expansion. The evaluation of intervention effectiveness would certainly contribute to improved worker health and safety. This need is particularly relevant regarding noise exposure in the workplace and hearing loss prevention interventions. In a 2006 review of the U.S. National Institute for Occupational Safety and Health (NIOSH) Hearing Loss Research Program, the independent National Academies of Sciences recommended that NIOSH place greater emphasis on identifying the effectiveness of hearing loss prevention measures on the basis of outcomes that are as closely related as possible to reducing noise exposure and work related hearing loss (http://www.nap.edu/openbook.php?record_id=11721). NIOSH used two different approaches to address that recommendation: the first one was to conduct research, including broad systematic reviews on the effectiveness of interventions to prevent occupational noise-induced hearing loss. The second was to create an award program, the Safe-In-Sound Excellence in Hearing Loss Prevention Award™, to identify and honor excellent real-world examples of noise control and other hearing loss prevention practices and innovations. PMID:27397968

Strategies for genetic study of hearing loss in the Brazilian northeastern region

PubMed Central

Melo, Uirá S; Santos, Silvana; Cavalcanti, Hannalice G; Andrade, Wagner T; Dantas, Vitor G; Rosa, Marine RD; Mingroni-Netto, Regina C

2014-01-01

The overall aim of this study was to estimate the contribution of genetic factors to the etiology of hearing loss (HL) in two counties in the Brazilian northeastern region. A cross-sectional study, based on the key informant approach (KI) was conducted in Queimadas and Gado Bravo counties (Paraíba, Northeast Brazil). The sample consisted of 182 patients with HL. Genetic screening of the most frequent mutations associated with HL was performed for all samples. DFNB1 mutations were the most frequently found in both counties. The c.35delG mutation was detected in homozygosis in seven non-syndromic probands in Queimadas (7/76, 9.2%) and only a single homozygote with this mutation was found in Gado Bravo (1/44, 2.3%). We also detected the del(GJB6-D13S1854) mutation in non-syndromic probands from Gado Bravo (2/44, 4.5%). The c.189C>A (p.TyrY63*) mutation in the CLRN1 gene was detected in homozygosis in 21/23 Usher syndrome patients from Gado Bravo and it was not found in Queimadas. Cases with probable genetic etiology contributed approximately to half of HL probands in each county (54.6% in Gado Bravo and 45.7% in Queimadas). We confirm the importance of DFNB1 locus to non-syndromic HL but we show that the frequency of mutations in the northeastern region differs somewhat from those reported in southeastern Brazil and other populations. In addition, the extremely high frequency of individuals with Usher syndrome with c.189C>A variation in CLRN1 indicates the need for a specific screening of this mutation. PMID:24596593

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.

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

Drug Induced Hearing Loss: What Is Ototoxicity?

MedlinePlus

... page please turn JavaScript on. Feature: Drug-Induced Hearing Loss What Is Ototoxicity? Past Issues / Spring 2016 ... of patients taking these drugs." "Antibiotics Caused My Hearing Loss..." Gulab Lalwani Photo Courtesy of: Gulab Lalwani ...

Hearing Loss: Issues in the Deaf and Hard of Hearing Communities.

PubMed

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

2015-07-01

Hearing loss can lead to impairments in language and speech acquisition, educational attainment, social development, and reading achievement. More than 90% of deaf and hard of hearing (DHH) children are born to hearing parents who may lack the knowledge or experience to effectively care for a child with hearing loss. Family involvement is crucial for teaching self-advocacy and global communication skills, optimizing social development, and helping DHH individuals understand and manage external attitudes about deafness and hearing loss. American Sign Language is a naturally developed language with an always-expanding lexicon and grammatical structures different from those of English. Teaching spoken English and American Sign Language equally, often called bilingual bimodal education, can enhance academic and reading achievement as well as language and psychosocial development. Formal schooling options for a DHH child include enrollment in a public or private school system (often called inclusion, integration, or mainstreaming), a school for the deaf, or a bilingual school. Individuals with hearing loss experience stereotypes and biases that create disparities in health insurance coverage, health care access, and outcomes of mental and physical conditions. Family physicians should recognize and minimize biases to improve health care in the DHH community. Written permission from the American Academy of Family Physicians is required for reproduction of this material in whole or in part in any form or medium.

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

PubMed

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

2015-01-01

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

Impact of OSHA final rule--recording hearing loss: an analysis of an industrial audiometric dataset.

PubMed

Rabinowitz, Peter M; Slade, Martin; Dixon-Ernst, Christine; Sircar, Kanta; Cullen, Mark

2003-12-01

The 2003 Occupational Safety and Health Administration (OSHA) Occupational Injury and Illness Recording and Reporting Final Rule changed the definition of recordable work-related hearing loss. We performed a study of the Alcoa Inc. audiometric database to evaluate the impact of this new rule. The 2003 rule increased the rate of potentially recordable hearing loss events from 0.2% to 1.6% per year. A total of 68.6% of potentially recordable cases had American Academy of Audiology/American Medical Association (AAO/AMA) hearing impairment at the time of recordability. On average, recordable loss occurred after onset of impairment, whereas the non-age-corrected 10-dB standard threshold shift (STS) usually preceded impairment. The OSHA Final Rule will significantly increase recordable cases of occupational hearing loss. The new case definition is usually accompanied by AAO/AMA hearing impairment. Other, more sensitive metrics should therefore be used for early detection and prevention of hearing loss.

Impact of OSHA Final Rule—Recording Hearing Loss: An Analysis of an Industrial Audiometric Dataset

PubMed Central

Rabinowitz, Peter M.; Slade, Martin; Dixon-Ernst, Christine; Sircar, Kanta; Cullen, Mark

2013-01-01

The 2003 Occupational Safety and Health Administration (OSHA) Occupational Injury and Illness Recording and Reporting Final Rule changed the definition of recordable work-related hearing loss. We performed a study of the Alcoa Inc. audiometric database to evaluate the impact of this new rule. The 2003 rule increased the rate of potentially recordable hearing loss events from 0.2% to 1.6% per year. A total of 68.6% of potentially recordable cases had American Academy of Audiology/American Medical Association (AAO/AMA) hearing impairment at the time of recordability. On average, recordable loss occurred after onset of impairment, whereas the non-age-corrected 10-dB standard threshold shift (STS) usually preceded impairment. The OSHA Final Rule will significantly increase recordable cases of occupational hearing loss. The new case definition is usually accompanied by AAO/AMA hearing impairment. Other, more sensitive metrics should therefore be used for early detection and prevention of hearing loss. PMID:14665813

Acquired hearing loss and brain plasticity.

PubMed

Eggermont, Jos J

2017-01-01

Acquired hearing loss results in an imbalance of the cochlear output across frequency. Central auditory system homeostatic processes responding to this result in frequency specific gain changes consequent to the emerging imbalance between excitation and inhibition. Several consequences thereof are increased spontaneous firing rates, increased neural synchrony, and (in adults) potentially restricted to the auditory thalamus and cortex a reorganization of tonotopic areas. It does not seem to matter much whether the hearing loss is acquired neonatally or in adulthood. In humans, no clear evidence of tonotopic map changes with hearing loss has so far been provided, but frequency specific gain changes are well documented. Unilateral hearing loss in addition makes brain activity across hemispheres more symmetrical and more synchronous. Molecular studies indicate that in the brainstem, after 2-5 days post trauma, the glutamatergic activity is reduced, whereas glycinergic and GABAergic activity is largely unchanged. At 2 months post trauma, excitatory activity remains decreased but the inhibitory one is significantly increased. In contrast protein assays related to inhibitory transmission are all decreased or unchanged in the brainstem, midbrain and auditory cortex. Comparison of neurophysiological data with the molecular findings during a time-line of changes following noise trauma suggests that increases in spontaneous firing rates are related to decreases in inhibition, and not to increases in excitation. Because noise-induced hearing loss in cats resulted in a loss of cortical temporal processing capabilities, this may also underlie speech understanding in humans. Copyright © 2016 Elsevier B.V. All rights reserved.

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

Novel and recurrent non-truncating mutations of the MITF basic domain: genotypic and phenotypic variations in Waardenburg and Tietz syndromes

PubMed Central

Léger, Sandy; Balguerie, Xavier; Goldenberg, Alice; Drouin-Garraud, Valérie; Cabot, Annick; Amstutz-Montadert, Isabelle; Young, Paul; Joly, Pascal; Bodereau, Virginie; Holder-Espinasse, Muriel; Jamieson, Robyn V; Krause, Amanda; Chen, Hongsheng; Baumann, Clarisse; Nunes, Luis; Dollfus, Hélène; Goossens, Michel; Pingault, Véronique

2012-01-01

The microphthalmia-associated transcription factor (MITF) is a basic helix-loop-helix leucine zipper transcription factor, which regulates melanocyte development and the biosynthetic melanin pathway. A notable relationship has been described between non-truncating mutations of its basic domain and Tietz syndrome, which is characterized by albinoid-like hypopigmentation of the skin and hair, rather than the patchy depigmentation seen in Waardenburg syndrome, and severe hearing loss. Twelve patients with new or recurrent non-truncating mutations of the MITF basic domain from six families were enrolled in this study. We observed a wide range of phenotypes and some unexpected features. All the patients had blue irides and pigmentation abnormalities that ranged from diffuse hypopigmentation to Waardenburg-like patches. In addition, they showed congenital complete hearing loss, diffuse hypopigmentation of the skin, freckling and ocular abnormalities, more frequently than patients with MITF mutations outside the basic domain. In conclusion, the non-truncating mutations of the basic domain do not always lead to Tietz syndrome but rather to a large range of phenotypes. Sun-exposed freckles are interestingly observed more frequently in Asian populations. This variability argues for the possible interaction with modifier loci. PMID:22258527

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

PubMed

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

2018-02-01

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

Intellectual Disabilities and Hearing Loss

ERIC Educational Resources Information Center

Herer, Gilbert R.

2012-01-01

Undetected/untreated hearing loss imposes significant limitations upon individuals with intellectual disabilities (ID). It can interfere with cognitive development, impede communicative and social interactions, and limit vocational aspirations. Over the past decade, the hearing of 9961 people with ID was evaluated at Special Olympics sports…

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

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.

Growth hormone treatment does not affect incidences of middle ear disease or hearing loss in infants and toddlers with Turner syndrome.

PubMed

Davenport, Marsha L; Roush, Jackson; Liu, Chunhua; Zagar, Anthony J; Eugster, Erica; Travers, Sharon; Fechner, Patricia Y; Quigley, Charmian A

2010-01-01

No randomized, controlled, prospective study has evaluated the effect of growth hormone (GH) on the rates of middle ear (ME) disease and hearing loss in girls with Turner syndrome (TS). A 2-year, prospective, randomized, controlled, open-label, multicenter, clinical trial ('Toddler Turner Study'; August 1999 to August 2003) was carried out. The study was conducted at 11 US pediatric endocrine centers. Eighty-eight girls with TS, aged 9 months to 4 years, were enrolled. The interventions comprised recombinant GH (50 microg/kg/day, n = 45) or no treatment (n = 43) for 2 years. The outcome measures included occurrence rates of ear-related problems, otitis media (OM) and associated antibiotic treatments, tympanometric assessment of ME function and hearing assessment by audiology. At baseline, 57% of the girls (mean age = 1.98 +/- 1.00 years) had a history of recurrent OM, 33% had undergone tympanostomy tube (t-tube) insertion and 27% had abnormal hearing. There was no significant difference between the treatment groups for annual incidence of OM episodes (untreated control: 1.9 +/- 1.4; GH-treated: 1.5 +/- 1.6, p = 0.17). A quarter of the subjects underwent ear surgeries (mainly t-tube insertions) during the study. Recurrent or persistent abnormality of ME function on tympanometry was present in 28-45% of the girls without t-tubes at the 6 postbaseline visits. Hearing deficits were found in 19-32% of the girls at the annual postbaseline visits. Most of these were conductive deficits, however, 2 girls had findings consistent with sensorineural hearing loss, which was evident before 3 years of age. Ear and hearing problems are common in infants and toddlers with TS and are not significantly influenced by GH treatment. Girls with TS need early, regular and thorough ME monitoring by their primary care provider and/or otolaryngologist, and at least annual hearing evaluations by a pediatric audiologist. Copyright 2010 S. Karger AG, Basel.

Suppression of Tinnitus in a Patient with Unilateral Sudden Hearing Loss: A Case Report

PubMed Central

Fioretti, Alessandra; Peri, Giorgia; Eibenstein, Alberto

2012-01-01

We describe a case of a 67-year-old woman with severe disabling right-sided tinnitus, mild hyperacusis, and headache. The tinnitus was associated with sudden right hearing loss and vertigo, which occurred about 18 months before. Magnetic resonance imaging (MRI) resulted in normal anatomical structures of the cochlea and of the cranial nerves showing a partial empty sella syndrome with suprasellar cistern hernia. Angio-MR revealed a bilateral contact between the anterior-inferior cerebellar artery (AICA) and the acoustic-facial nerve with a potential neurovascular conflict. Surgery was considered unnecessary after further evaluations. The right ear was successfully treated with a combination device (hearing aid plus sound generator). Shortly after a standard fitting procedure, the patient reported a reduction of tinnitus, hyperacusis, and headache which completely disappeared at the follow-up evaluation after 3, 6, and 12 months. This paper demonstrates that the combination device resulted in a complete tinnitus and hyperacusis suppression in a patient with unilateral sensorineural sudden hearing loss. Our paper further supports the restoration of peripheral sensory input for the treatment of tinnitus associated with hearing loss in selected patients. PMID:23227400

Cochlear implantation in adults with asymmetric hearing loss.

PubMed

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

2012-01-01

Bilateral severe to profound sensorineural hearing loss is a standard criterion for cochlear implantation. Increasingly, patients are implanted in one ear and continue to use a hearing aid in the nonimplanted ear to improve abilities such as sound localization and speech understanding in noise. Patients with severe to profound hearing loss in one ear and a more moderate hearing loss in the other ear (i.e., asymmetric hearing) are not typically considered candidates for cochlear implantation. Amplification in the poorer ear is often unsuccessful because of limited benefit, restricting the patient to unilateral listening from the better ear alone. The purpose of this study was to determine whether patients with asymmetric hearing loss could benefit from cochlear implantation in the poorer ear with continued use of a hearing aid in the better ear. Ten adults with asymmetric hearing between ears participated. In the poorer ear, all participants met cochlear implant candidacy guidelines; seven had postlingual onset, and three had pre/perilingual onset of severe to profound hearing loss. All had open-set speech recognition in the better-hearing ear. Assessment measures included word and sentence recognition in quiet, sentence recognition in fixed noise (four-talker babble) and in diffuse restaurant noise using an adaptive procedure, localization of word stimuli, and a hearing handicap scale. Participants were evaluated preimplant with hearing aids and postimplant with the implant alone, the hearing aid alone in the better ear, and bimodally (the implant and hearing aid in combination). Postlingual participants were evaluated at 6 mo postimplant, and pre/perilingual participants were evaluated at 6 and 12 mo postimplant. Data analysis compared the following results: (1) the poorer-hearing ear preimplant (with hearing aid) and postimplant (with cochlear implant); (2) the device(s) used for everyday listening pre- and postimplant; and (3) the hearing aid-alone and bimodal

Dentinogenesis imperfecta associated with short stature, hearing loss and mental retardation: a new syndrome with autosomal recessive inheritance?

PubMed

Cauwels, R G E C; De Coster, P J; Mortier, G R; Marks, L A M; Martens, L C

2005-08-01

The follow-up history and oral findings in two brothers from consanguineous parents suggest that the association of dentinogenesis imperfecta (DI), delayed tooth eruption, mild mental retardation, proportionate short stature, sensorineural hearing loss and dysmorphic facies may represent a new syndrome with autosomal recessive inheritance. Histological examination of the dentin matrix of a permanent molar from one of the siblings reveals morphological similarities with defective dentinogenesis as presenting in patients affected with Osteogenesis Imperfecta (OI), a condition caused by deficiency of type I collagen. A number of radiographic and histological characteristics, however, are inconsistent with classical features of DI. These findings suggest that DI may imply greater genetical heterogeneity than currently assumed.

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

[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.

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

Autism and peripheral hearing loss: a systematic review.

PubMed

Beers, Alison N; McBoyle, Melanie; Kakande, Emily; Dar Santos, Rachelle C; Kozak, Frederick K

2014-01-01

To systematically review the literature describing the relationship between autism spectrum disorder (ASD) and peripheral hearing loss including literature recommendations for audiological assessment and auditory habilitation in cases where peripheral hearing loss and ASD coexist. Published studies indexed in MEDLINE (1948-2011). The search strategy identified 595 potential studies. After a review of the titles, 115 abstracts were reviewed and 39 articles were retrieved and assessed independently by at least two authors for possible inclusion. 22 articles pertained to children with ASD and peripheral hearing loss, hearing assessment in children with ASD, audiological habilitation for children with ASD or hyper-responsiveness in children with ASD. 17 further studies were garnered from the reference section of the 22 papers. Controversy exists in the literature regarding prevalence of hearing impairment among individuals with ASD. In cases where ASD and hearing impairment co-exist, diagnosis of one condition often leads to a delay in diagnosing the other. Audiological assessment can be difficult in children with ASD and test-retest reliability of behavioural thresholds can be poor. In cases where hearing impairment exists and hearing aids or cochlear implantation are recommended, devices are often fit with special considerations for the child with ASD. Hyper-responsiveness to auditory stimuli may be displayed by individuals with ASD. Evidence or the suspicion of hyper-responsiveness may be taken into consideration when fitting amplification and planning behavioural intervention. Prevalence rates of hearing impairment among individuals with ASD continue to be debated. At present there is no conclusive evidence that children with ASD are at increased risk of peripheral hearing loss. A complete audiological assessment is recommended in all cases where ASD is suspected so as not to delay the diagnosis of hearing impairment in the event that hearing loss and ASD co

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…

Hearing loss associated with US military combat deployment

PubMed Central

Wells, Timothy S.; Seelig, Amber D.; Ryan, Margaret A. K.; Jones, Jason M.; Hooper, Tomoko I.; Jacobson, Isabel G.; Boyko, Edward J.

2015-01-01

The objective of this study was to define the risk of hearing loss among US military members in relation to their deployment experiences. Data were drawn from the Millennium Cohort Study. Self-reported data and objective military service data were used to assess exposures and outcomes. Among all 48,540 participants, 7.5% self-reported new-onset hearing loss. Self-reported hearing loss showed moderate to substantial agreement (k = 0.57-0.69) with objective audiometric measures. New-onset hearing loss was associated with combat deployment (adjusted odds ratio [AOR] = 1.63, 95% confidence interval [CI] = 1.49-1.77), as well as male sex and older age. Among deployers, new-onset hearing loss was also associated with proximity to improvised explosive devices (AOR = 2.10, 95% CI = 1.62-2.73) and with experiencing a combat-related head injury (AOR = 6.88, 95% CI = 3.77-12.54). These findings have implications for health care and disability planning, as well as for prevention programs. PMID:25599756

(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.

P300 in individuals with sensorineural hearing loss.

PubMed

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

2015-01-01

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

Teaching Children with Hearing Loss in Reading Recovery

ERIC Educational Resources Information Center

Charlesworth, Ann; Charlesworth, Robert; Raban, Bridie; Rickards, Field

2006-01-01

This study quantitatively analyzed the structure of Reading Recovery lessons for children with hearing loss by examining and comparing the supportive interactions of three Reading Recovery teachers of 12 children with hearing loss and three Reading Recovery teachers of 12 hearing children. All of the children were in the second year of primary…

Applications of direct-to-consumer hearing devices for adults with hearing loss: a review.

PubMed

Manchaiah, Vinaya; Taylor, Brian; Dockens, Ashley L; Tran, Nicole R; Lane, Kayla; Castle, Mariana; Grover, Vibhu

2017-01-01

This systematic literature review is aimed at investigating applications of direct-to-consumer hearing devices for adults with hearing loss. This review discusses three categories of direct-to-consumer hearing devices: 1) personal sound amplification products (PSAPs), 2) direct-mail hearing aids, and 3) over-the-counter (OTC) hearing aids. A literature review was conducted using EBSCOhost and included the databases CINAHL, MEDLINE, and PsycINFO. After applying prior agreed inclusion and exclusion criteria, 13 reports were included in the review. Included studies fell into three domains: 1) electroacoustic characteristics, 2) consumer surveys, and 3) outcome evaluations. Electroacoustic characteristics of these devices vary significantly with some meeting the stringent acoustic criteria used for hearing aids, while others producing dangerous output levels (ie, over 120-dB sound pressure level). Low-end (or low-cost) devices were typically poor in acoustic quality and did not meet gain levels necessary for most adult and elderly hearing loss patterns (eg, presbycusis), especially in high frequencies. Despite direct-mail hearing aids and PSAPs being associated with lower satisfaction when compared to hearing aids purchased through hearing health care professionals, consumer surveys suggest that 5%-19% of people with hearing loss purchase hearing aids through direct-mail or online. Studies on outcome evaluation suggest positive outcomes of OTC devices in the elderly population. Of note, OTC outcomes appear better when a hearing health care professional supports these users. While some direct-to-consumer hearing devices have the capability to produce adverse effects due to production of dangerously high sound levels and internal noise, the existing literature suggests that there are potential benefits of these devices. Research of direct-to-consumer hearing devices is limited, and current published studies are of weak quality. Much effort is needed to understand the

Decreased postural control in people with moderate hearing loss

PubMed Central

Thomas, Ewan; Martines, Francesco; Bianco, Antonino; Messina, Giuseppe; Giustino, Valerio; Zangla, Daniele; Iovane, Angelo; Palma, Antonio

2018-01-01

Abstract Balance is a complex process that involves multiple sensory integrations. The auditory, visual, and vestibular systems are the main contributors. Hearing loss or hearing impairment may induce inappropriate postural strategies that could affect balance and therefore increase the risk of falling. The aim of this study was to understand whether hearing loss could influence balance, cervical posture, and muscle activation in the cervical region. Thirteen patients (61 ± 13 years; 161.8 ± 11.0 cm; 70.5 ± 15.9 kg) with moderate hearing loss (Right ear −60 ± 21 dB; Left ear −61 ± 24 dB) underwent: an audiometric examination, a postural examination (with open and closed eyes) through a stabilometric platform, a cervical ROM examination through a head accelerometer, and a sternocleidomastoid electromyography (EMG) examination. A linear regression analysis has shown a regression coefficient (R2) 0.76 and 0.69 between hearing loss and the posturographic parameters, on the sagittal sway, with open and closed eyes, respectively. The combination of frontal and sagittal sway is able to explain up to 84% of the variance of the audiometric assessment. No differences were found between right and left hemibody between the audiometric, posturographic, cervical ROM parameters, and in EMG amplitude. ROM and EMG parameters have not shown any significant associations with hearing loss, for both right and left head rotation. Hearing loss is associated to increased posturographic measures, especially the sagittal sway, underlining a reduced postural control in people with hearing impairments. No association was found between the heads posture and neck activation with hearing loss. Hearing loss may be associated with an increased risk of falls. PMID:29620637

Decreased postural control in people with moderate hearing loss.

PubMed

Thomas, Ewan; Martines, Francesco; Bianco, Antonino; Messina, Giuseppe; Giustino, Valerio; Zangla, Daniele; Iovane, Angelo; Palma, Antonio

2018-04-01

Balance is a complex process that involves multiple sensory integrations. The auditory, visual, and vestibular systems are the main contributors. Hearing loss or hearing impairment may induce inappropriate postural strategies that could affect balance and therefore increase the risk of falling.The aim of this study was to understand whether hearing loss could influence balance, cervical posture, and muscle activation in the cervical region.Thirteen patients (61 ± 13 years; 161.8 ± 11.0 cm; 70.5 ± 15.9 kg) with moderate hearing loss (Right ear -60 ± 21 dB; Left ear -61 ± 24 dB) underwent: an audiometric examination, a postural examination (with open and closed eyes) through a stabilometric platform, a cervical ROM examination through a head accelerometer, and a sternocleidomastoid electromyography (EMG) examination.A linear regression analysis has shown a regression coefficient (R) 0.76 and 0.69 between hearing loss and the posturographic parameters, on the sagittal sway, with open and closed eyes, respectively. The combination of frontal and sagittal sway is able to explain up to 84% of the variance of the audiometric assessment. No differences were found between right and left hemibody between the audiometric, posturographic, cervical ROM parameters, and in EMG amplitude. ROM and EMG parameters have not shown any significant associations with hearing loss, for both right and left head rotation.Hearing loss is associated to increased posturographic measures, especially the sagittal sway, underlining a reduced postural control in people with hearing impairments. No association was found between the heads posture and neck activation with hearing loss. Hearing loss may be associated with an increased risk of falls.

Relationship Between Hair Cell Loss and Hearing Loss in Fishes.

PubMed

Smith, Michael E

2016-01-01

Exposure to intense sound or ototoxic chemicals can damage the auditory hair cells of vertebrates, resulting in hearing loss. Although the relationship between such hair cell damage and auditory function is fairly established for terrestrial vertebrates, there are limited data available to understand this relationship in fishes. Although investigators have measured either the morphological damage of the inner ear or the functional deficits in the hearing of fishes, very few have directly measured both in an attempt to find a relationship between the two. Those studies that have examined both auditory hair cell damage in the inner ear and the resulting hearing loss in fishes are reviewed here. In general, there is a significant linear relationship between the number of hair cells lost and the severity of hearing threshold shifts, although this varies between species and different hair cell-damaging stimuli. After trauma to the fish ear, auditory hair cells are able to regenerate to control level densities. With this regeneration also comes a restoration of hearing. Thus there is also a significant relationship between hair cell recovery and hearing recovery in fishes.

Targeted sequencing identifies novel variants involved in autosomal recessive hereditary hearing loss in Qatari families.

PubMed

Alkowari, Moza K; Vozzi, Diego; Bhagat, Shruti; Krishnamoorthy, Navaneethakrishnan; Morgan, Anna; Hayder, Yousra; Logendra, Barathy; Najjar, Nehal; Gandin, Ilaria; Gasparini, Paolo; Badii, Ramin; Girotto, Giorgia; Abdulhadi, Khalid

2017-08-01

Hereditary hearing loss is characterized by a very high genetic heterogeneity. In the Qatari population the role of GJB2, the worldwide HHL major player, seems to be quite limited compared to Caucasian populations. In this study we analysed 18 Qatari families affected by non-syndromic hearing loss using a targeted sequencing approach that allowed us to analyse 81 genes simultaneously. Thanks to this approach, 50% of these families (9 out of 18) resulted positive for the presence of likely causative alleles in 6 different genes: CDH23, MYO6, GJB6, OTOF, TMC1 and OTOA. In particular, 4 novel alleles were detected while the remaining ones were already described to be associated to HHL in other ethnic groups. Molecular modelling has been used to further investigate the role of novel alleles identified in CDH23 and TMC1 genes demonstrating their crucial role in Ca2+ binding and therefore possible functional role in proteins. Present study showed that an accurate molecular diagnosis based on next generation sequencing technologies might largely improve molecular diagnostics outcome leading to benefits for both genetic counseling and definition of recurrence risk. Copyright © 2017 Elsevier B.V. All rights reserved.

Potential treatments for genetic hearing loss in humans: current conundrums.

PubMed

Minoda, R; Miwa, T; Ise, M; Takeda, H

2015-08-01

Genetic defects are a major cause of hearing loss in newborns. Consequently, hearing loss has a profound negative impact on human daily living. Numerous causative genes for genetic hearing loss have been identified. However, presently, there are no truly curative treatments for this condition. There have been several recent reports on successful treatments in mice using embryonic gene therapy, neonatal gene therapy and neonatal antisense oligonucleotide therapy. Herein, we describe state-of-the-art research on genetic hearing loss treatment through gene therapy and discuss the obstacles to overcome in curative treatments of genetic hearing loss in humans.

The Socioeconomic Impact of Hearing Loss in US Adults

PubMed Central

Emmett, Susan D.; Francis, Howard W.

2014-01-01

Objective To evaluate the associations between hearing loss and educational attainment, income, and unemployment/underemployment in US adults. Study design National cross-sectional survey. Setting Ambulatory examination centers. Patients Adults aged 20-69 years who participated in the 1999-2002 cycles of the National Health and Nutrition Examination Survey (NHANES) audiometric evaluation and income questionnaire (n = 3379). Intervention(s) Pure tone audiometry, with hearing loss defined by World Health Organization criteria of bilateral pure tone average >25 decibels (0.5,1,2,4 kHz). Main outcome measure(s) Low educational attainment, defined as not completing high school; low income, defined as family income less than $20,000/year, and unemployment or underemployment, defined as not having a job or working less than 35 hours per week. Results Individuals with hearing loss had 3.21 times higher odds of low educational attainment (95% CI: 2.20-4.68) compared to normal-hearing individuals. Controlling for education, age, sex, and race, individuals with hearing loss had 1.58 times higher odds of low income (95% CI: 1.16-2.15) and 1.98 times higher odds of being unemployed or underemployed (95% CI: 1.38-2.85) compared to normal-hearing individuals. Conclusions Hearing loss is associated with low educational attainment in US adults. Even after controlling for education and important demographic factors, hearing loss is independently associated with economic hardship, including both low income and unemployment/underemployment. The societal impact of hearing loss is profound in this nationally representative study and should be further evaluated with longitudinal cohorts. PMID:25158616

[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.

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

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

PubMed

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

2007-04-01

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

Quality and readability of information pamphlets on hearing and paediatric hearing loss in the Gauteng Province, South Africa.

PubMed

Joubert, Karin; Githinji, Esther

2014-02-01

The implementation of early hearing detection and intervention (EHDI) programmes is necessary in order to facilitate the early identification of hearing loss. An important component of EHDI is parental education. International and national guidelines stipulating that comprehensive, unbiased and appropriate information pamphlets should be provided to parents as part of EHDI programmes, however little is known about the availability and readability of such materials in South Africa. The objectives of this study were therefore to determine the availability of information pamphlets on hearing and hearing loss in children at public hospitals in the Gauteng Province of South Africa. In addition, the quality and readability levels of these pamphlets were determined. A non-experimental, descriptive research design was employed for this study. Information on the availability of leaflets at public health hospitals was obtained through a telephonic survey. Twenty-one information pamphlets available at these hospitals were then evaluated to determine the quality and readability levels. It was found that 73% of audiology departments at public hospitals in Gauteng had information pamphlets available on hearing and hearing loss in children. Of the pamphlets evaluated, the majority were rated to 'present with serious problems' questioning the quality of the content included. In addition, it was found that on average the readability level of these pamphlets were at a sixth-grade level, much higher that the recommended fourth-grade reading level. The need for development of quality educational material focused on providing parents with unbiased, comprehensive and appropriate information on hearing and hearing loss in children has been highlighted. Proposed guidelines were recommended to assist audiologists in this endeavour. The importance of providing appropriate parental educational materials for the success of EHDI in South Africa should not be underestimated. Copyright © 2013

Quality of Life and Hearing Eight Years After Sudden Sensorineural Hearing Loss.

PubMed

Härkönen, Kati; Kivekäs, Ilkka; Rautiainen, Markus; Kotti, Voitto; Vasama, Juha-Pekka

2017-04-01

To explore long-term hearing results, quality of life (QoL), quality of hearing (QoH), work-related stress, tinnitus, and balance problems after idiopathic sudden sensorineural hearing loss (ISSNHL). Cross-sectional study. We reviewed the audiograms of 680 patients with unilateral ISSNHL on average 8 years after the hearing impairment, and then divided the patients into two study groups based on whether their ISSNHL had recovered to normal (pure tone average [PTA] ≤ 30 dB) or not (PTA > 30 dB). The inclusion criteria were a hearing threshold decrease of 30 dB or more in at least three contiguous frequencies occurring within 72 hours in the affected ear and normal hearing in the contralateral ear. Audiograms of 217 patients fulfilled the criteria. We reviewed their medical records; measured present QoL, QoH, and work-related stress with specific questionnaires; and updated the hearing status. Poor hearing outcome after ISSNHL was correlated with age, severity of hearing loss, and vertigo together with ISSNHL. Quality of life and QoH were statistically significantly better in patients with recovered hearing, and the patients had statistically significantly less tinnitus and balance problems. During the 8-year follow-up, the PTA of the affected ear deteriorated on average 7 dB, and healthy ear deteriorated 6 dB. Idiopathic sudden sensorineural hearing loss that failed to recover had a negative impact on long-term QoL and QoH. The hearing deteriorated as a function of age similarly both in the affected and the healthy ear, and there were no differences between the groups. The cumulative recurrence rate for ISSNHL was 3.5%. 4 Laryngoscope, 127:927-931, 2017. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

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.

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.

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…

Awarding and promoting excellence in hearing loss prevention

PubMed Central

Meinke, Deanna K.; Morata, Thais C.

2015-01-01

Objective To describe the rationale and creation of a national award to recognize and promote hearing loss prevention. Design In 2007, the National Institute for Occupational Safety and Health partnered with the National Hearing Conservation Association to create the Safe-in-Sound Excellence in Hearing Loss Prevention Award™ (www.safeinsound.us). The objectives of this initiative were to recognize organizations that document measurable achievements and to share leading edge information to a broader community. Results An expert committee developed specific and explicit award evaluation criteria of excellence in hearing loss prevention for organizations in different industrial sectors. The general approach toward award criteria was to incorporate current ‘best practices’ and familiar benchmarks of hearing loss prevention programs. This approach was reviewed publicly. In addition, mechanisms were identified to measure the impact of the award itself. Interest in the award was recorded through the monitoring of the visitor traffic registered by the award web site and is increasing yearly. Specific values and strategies common across award winners are presented. Conclusion The Safe-in-Sound Award™ has obtained high quality field data; identified practical solutions, disseminated successful strategies to minimize the risk of hearing loss, generated new partnerships, and shared practical solutions with others in the field. PMID:22264064

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.

Diplacusis, hearing threshold and otoacoustic emissions in an episode of sudden, unilateral cochlear hearing loss.

PubMed

Knight, Richard D

2004-01-01

Limited data are available on the relationship between diplacusis and otoacoustic emissions and sudden hearing threshold changes, and the detail of the mechanism underlying diplacusis is not well understood. Data are presented here from an intensively studied single episode of sudden, non-conductive, mild hearing loss with associated binaural diplacusis, probably due to a viral infection. Treatment with steroids was administered for 1 week. This paper examines the relationships between the hearing loss, diplacusis and otoacoustic emissions during recovery on a day-by-day basis. The hearing thresholds were elevated by up to 20 dB at 4kHz and upwards, and there was an interaural pitch difference up to 12% at 4 and 8 kHz. There was also a frequency-specific change in transient evoked otoacoustic emission (TEOAE) and distortion-product otoacoustic emission (DPOAE) level. DPOAE level was reduced by up to 20 dB. with the greatest change seen when a stimulus with a wide stimulus frequency ratio was used. Frequency shifts in the 2f2-fi DPOAE fine structure corresponded to changes in the diplacusis. Complete recovery to previous levels was observed for TEOAE, DPOAE and hearing threshold. The diplacusis recovered to within normal limits after 4 weeks. The frequency shift seen in the DPOAE fine structure did not quite resolve, suggesting a very slight permanent change. The time-courses of TEOAE. diplacusis and hearing threshold were significantly different: most notably, the hearing threshold was stable over a period when the diplacusis deteriorated. This suggests that the cochlear mechanisms involved in diplacusis, hearing threshold and OAE may not be identical.

Hearing in the elderly. Relation of hearing loss, loneliness, and self-esteem.

PubMed

Chen, H L

1994-06-01

1. The prevalence of hearing impairment increases with age. Hearing handicap resulting from hearing loss may lead to psychosocial problems in elders. 2. Previous studies have shown relations among hearing handicap, loneliness and low self-esteem, but those studies focused on populations other than elders. 3. Early detection of hearing handicap and appropriate interventions promote increased independence and preserve the ability of elders to interact with their environment.

Visual speech alters the discrimination and identification of non-intact auditory speech in children with hearing loss.

PubMed

Jerger, Susan; Damian, Markus F; McAlpine, Rachel P; Abdi, Hervé

2017-03-01

Understanding spoken language is an audiovisual event that depends critically on the ability to discriminate and identify phonemes yet we have little evidence about the role of early auditory experience and visual speech on the development of these fundamental perceptual skills. Objectives of this research were to determine 1) how visual speech influences phoneme discrimination and identification; 2) whether visual speech influences these two processes in a like manner, such that discrimination predicts identification; and 3) how the degree of hearing loss affects this relationship. Such evidence is crucial for developing effective intervention strategies to mitigate the effects of hearing loss on language development. Participants were 58 children with early-onset sensorineural hearing loss (CHL, 53% girls, M = 9;4 yrs) and 58 children with normal hearing (CNH, 53% girls, M = 9;4 yrs). Test items were consonant-vowel (CV) syllables and nonwords with intact visual speech coupled to non-intact auditory speech (excised onsets) as, for example, an intact consonant/rhyme in the visual track (Baa or Baz) coupled to non-intact onset/rhyme in the auditory track (/-B/aa or/-B/az). The items started with an easy-to-speechread/B/or difficult-to-speechread/G/onset and were presented in the auditory (static face) vs. audiovisual (dynamic face) modes. We assessed discrimination for intact vs. non-intact different pairs (e.g., Baa:/-B/aa). We predicted that visual speech would cause the non-intact onset to be perceived as intact and would therefore generate more same-as opposed to different-responses in the audiovisual than auditory mode. We assessed identification by repetition of nonwords with non-intact onsets (e.g.,/-B/az). We predicted that visual speech would cause the non-intact onset to be perceived as intact and would therefore generate more Baz-as opposed to az- responses in the audiovisual than auditory mode. Performance in the audiovisual mode showed more same

Visual Speech Alters the Discrimination and Identification of Non-Intact Auditory Speech in Children with Hearing Loss

PubMed Central

Jerger, Susan; Damian, Markus F.; McAlpine, Rachel P.; Abdi, Hervé

2017-01-01

Objectives Understanding spoken language is an audiovisual event that depends critically on the ability to discriminate and identify phonemes yet we have little evidence about the role of early auditory experience and visual speech on the development of these fundamental perceptual skills. Objectives of this research were to determine 1) how visual speech influences phoneme discrimination and identification; 2) whether visual speech influences these two processes in a like manner, such that discrimination predicts identification; and 3) how the degree of hearing loss affects this relationship. Such evidence is crucial for developing effective intervention strategies to mitigate the effects of hearing loss on language development. Methods Participants were 58 children with early-onset sensorineural hearing loss (CHL, 53% girls, M = 9;4 yrs) and 58 children with normal hearing (CNH, 53% girls, M = 9;4 yrs). Test items were consonant-vowel (CV) syllables and nonwords with intact visual speech coupled to non-intact auditory speech (excised onsets) as, for example, an intact consonant/rhyme in the visual track (Baa or Baz) coupled to non-intact onset/rhyme in the auditory track (/–B/aa or /–B/az). The items started with an easy-to-speechread /B/ or difficult-to-speechread /G/ onset and were presented in the auditory (static face) vs. audiovisual (dynamic face) modes. We assessed discrimination for intact vs. non-intact different pairs (e.g., Baa:/–B/aa). We predicted that visual speech would cause the non-intact onset to be perceived as intact and would therefore generate more same—as opposed to different—responses in the audiovisual than auditory mode. We assessed identification by repetition of nonwords with non-intact onsets (e.g., /–B/az). We predicted that visual speech would cause the non-intact onset to be perceived as intact and would therefore generate more Baz—as opposed to az— responses in the audiovisual than auditory mode. Results

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.

Applications of direct-to-consumer hearing devices for adults with hearing loss: a review

PubMed Central

Manchaiah, Vinaya; Taylor, Brian; Dockens, Ashley L; Tran, Nicole R; Lane, Kayla; Castle, Mariana; Grover, Vibhu

2017-01-01

Background This systematic literature review is aimed at investigating applications of direct-to-consumer hearing devices for adults with hearing loss. This review discusses three categories of direct-to-consumer hearing devices: 1) personal sound amplification products (PSAPs), 2) direct-mail hearing aids, and 3) over-the-counter (OTC) hearing aids. Method A literature review was conducted using EBSCOhost and included the databases CINAHL, MEDLINE, and PsycINFO. After applying prior agreed inclusion and exclusion criteria, 13 reports were included in the review. Results Included studies fell into three domains: 1) electroacoustic characteristics, 2) consumer surveys, and 3) outcome evaluations. Electroacoustic characteristics of these devices vary significantly with some meeting the stringent acoustic criteria used for hearing aids, while others producing dangerous output levels (ie, over 120-dB sound pressure level). Low-end (or low-cost) devices were typically poor in acoustic quality and did not meet gain levels necessary for most adult and elderly hearing loss patterns (eg, presbycusis), especially in high frequencies. Despite direct-mail hearing aids and PSAPs being associated with lower satisfaction when compared to hearing aids purchased through hearing health care professionals, consumer surveys suggest that 5%–19% of people with hearing loss purchase hearing aids through direct-mail or online. Studies on outcome evaluation suggest positive outcomes of OTC devices in the elderly population. Of note, OTC outcomes appear better when a hearing health care professional supports these users. Conclusion While some direct-to-consumer hearing devices have the capability to produce adverse effects due to production of dangerously high sound levels and internal noise, the existing literature suggests that there are potential benefits of these devices. Research of direct-to-consumer hearing devices is limited, and current published studies are of weak quality. Much

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

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

PubMed Central

Noda, Teppei; Meas, Steven J.; Nogami, Jumpei; Amemiya, Yutaka; Uchi, Ryutaro; Ohkawa, Yasuyuki; Nishimura, Koji; Dabdoub, Alain

2018-01-01

Primary auditory neurons (PANs) play a critical role in hearing by transmitting sound information from the inner ear to the brain. Their progressive degeneration is associated with excessive noise, disease and aging. The loss of PANs leads to permanent hearing impairment since they are incapable of regenerating. Spiral ganglion non-neuronal cells (SGNNCs), comprised mainly of glia, are resident within the modiolus and continue to survive after PAN loss. These attributes make SGNNCs an excellent target for replacing damaged PANs through cellular reprogramming. We used the neurogenic pioneer transcription factor Ascl1 and the auditory neuron differentiation factor NeuroD1 to reprogram SGNNCs into induced neurons (iNs). The overexpression of both Ascl1 and NeuroD1 in vitro generated iNs at high efficiency. Transcriptome analyses revealed that iNs displayed a transcriptome profile resembling that of endogenous PANs, including expression of several key markers of neuronal identity: Tubb3, Map2, Prph, Snap25, and Prox1. Pathway analyses indicated that essential pathways in neuronal growth and maturation were activated in cells upon neuronal induction. Furthermore, iNs extended projections toward cochlear hair cells and cochlear nucleus neurons when cultured with each respective tissue. Taken together, our study demonstrates that PAN-like neurons can be generated from endogenous SGNNCs. This work suggests that gene therapy can be a viable strategy to treat sensorineural hearing loss caused by degeneration of PANs. PMID:29492404

Profound hearing loss associated with hydrocodone/acetaminophen abuse.

PubMed

Friedman, R A; House, J W; Luxford, W M; Gherini, S; Mills, D

2000-03-01

To describe profound hearing loss associated with hydrocodone overuse and the successful rehabilitation of these patients with cochlear implantation. Retrospective review. A tertiary otologic referral center. Twelve patients with rapidly progressive hearing loss and a concurrent history of hydrocodone overuse. Comprehensive medical histories, physical findings, audiometric tests, and, in those patients undergoing cochlear implantation, postimplantation performance data were reviewed. Clinical characteristics of hydrocodone-related hearing loss and open set word and sentence performance in those patients undergoing cochlear implantation. Hydrocodone overuse was associated with rapidly progressive sensorineural hearing loss in 12 patients. In four patients the initial presentation was unilateral, and two of the patients experienced vestibular symptoms. None of the 12 patients experienced improved thresholds after high-dose prednisone. Seven of the eight patients undergoing cochlear implantation have demonstrated early success with their devices. Hydrocodone is frequently prescribed in combination with acetaminophen for the relief of pain and has a side effects profile similar to other medications in its class. Although not described previously, overuse or abuse can be associated with a rapidly progressive sensorineural hearing loss. These patients can be successfully rehabilitated with cochlear implantation.

Living with hearing loss

MedlinePlus

... independent Be safer wherever you are Managing the Environment Many things in your surroundings can affect how ... hearing loss . References Andrews J. Optimizing the built environment for frail older adults. In: Fillit HM, Rockwood ...

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

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

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.

Enhancing Communication in Adults with Dementia and Age-Related Hearing Loss

PubMed Central

Mamo, Sara K.; Oh, Esther; Lin, Frank R.

2017-01-01

For many adults with dementia, age-related hearing loss is undiagnosed and/or untreated. Untreated hearing loss can exacerbate common dementia-related behavioral symptoms, such as depression, apathy, agitation. Despite the potential benefits to the individual and the family, pursuing and adopting hearing aids for persons with dementia presents with many challenges. As such, this group of vulnerable older adults is well suited for alternative approaches that adopt an interventional audiology framework. This article reviews alternative hearing care models that we have tested when working with older adults with cognitive impairments. We have found that some individuals show improvements in dementia-related problem behaviors and/or in measures of social engagement after brief aural rehabilitation interventions that provide non-custom amplification. We have developed simple training materials to help family and professional caregivers use communication strategies and non-custom amplification. Providing services that can be integrated into the person's broader dementia care has the potential to improve communication and quality of life for individuals and families. There are opportunities in this population to provide basic, simple strategies and make substantial improvements as long as we adopt approaches that bring the services to the people, instead of bringing the people to us in the audiology clinic. PMID:28522892

Novel mutations in the long isoform of the USH2A gene in patients with Usher syndrome type II or non-syndromic retinitis pigmentosa.

PubMed

McGee, Terri L; Seyedahmadi, Babak Jian; Sweeney, Meredith O; Dryja, Thaddeus P; Berson, Eliot L

2010-07-01

Usher syndrome type II (USH2) is an autosomal recessive disorder characterised by retinitis pigmentosa (RP) and mild to moderate sensorineural hearing loss. Mutations in the USH2A gene are the most common cause of USH2 and are also a cause of some forms of RP without hearing loss (ie, non-syndromic RP). The USH2A gene was initially identified as a transcript comprised of 21 exons but subsequently a longer isoform containing 72 exons was identified. The 51 exons unique to the long isoform of USH2A were screened for mutations among a core set of 108 patients diagnosed with USH2 and 80 patients with non-syndromic RP who were all included in a previously reported screen of the short isoform of USH2A. For several exons, additional patients were screened. In total, 35 deleterious mutations were identified including 17 nonsense mutations, 9 frameshift mutations, 5 splice-site mutations, and 4 small in-frame deletions or insertions. Twenty-seven mutations were novel. In addition, 65 rare missense changes were identified. A method of classifying the deleterious effect of the missense changes was developed using the summed results of four different mutation assessment algorithms, SIFT, pMUT, PolyPhen, and AGVGD. This system classified 8 of the 65 changes as 'likely deleterious' and 9 as 'possibly deleterious'. At least one mutation was identified in 57-63% of USH2 cases and 19-23% of cases of non-syndromic recessive RP (calculated without and including probable/possible deleterious changes) thus supporting that USH2A is the most common known cause of RP in the USA.

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.

Impact of peripheral hearing loss on top-down auditory processing.

PubMed

Lesicko, Alexandria M H; Llano, Daniel A

2017-01-01

The auditory system consists of an intricate set of connections interposed between hierarchically arranged nuclei. The ascending pathways carrying sound information from the cochlea to the auditory cortex are, predictably, altered in instances of hearing loss resulting from blockage or damage to peripheral auditory structures. However, hearing loss-induced changes in descending connections that emanate from higher auditory centers and project back toward the periphery are still poorly understood. These pathways, which are the hypothesized substrate of high-level contextual and plasticity cues, are intimately linked to the ascending stream, and are thereby also likely to be influenced by auditory deprivation. In the current report, we review both the human and animal literature regarding changes in top-down modulation after peripheral hearing loss. Both aged humans and cochlear implant users are able to harness the power of top-down cues to disambiguate corrupted sounds and, in the case of aged listeners, may rely more heavily on these cues than non-aged listeners. The animal literature also reveals a plethora of structural and functional changes occurring in multiple descending projection systems after peripheral deafferentation. These data suggest that peripheral deafferentation induces a rebalancing of bottom-up and top-down controls, and that it will be necessary to understand the mechanisms underlying this rebalancing to develop better rehabilitation strategies for individuals with peripheral hearing loss. Copyright © 2016 Elsevier B.V. All rights reserved.

Professionals with hearing loss: maintaining that competitive edge.

PubMed

Tye-Murray, Nancy; Spry, Jacqueline L; Mauzé, Elizabeth

2009-08-01

The goals of this investigation were to gauge how hearing loss affects the self-perceived job performance and psycho-emotional status of professionals in the workforce and to develop a profile of their aural rehabilitation needs. Forty-eight participants who had at least a high school education and who hold salaried positions participated in one of seven focus groups. Participants first answered questions about a hypothetical executive who had hearing loss and considered how she might react to various communication issues. They then addressed questions about their own work-related predicaments. The sessions were audiovideo recorded and later transcribed for analysis. Unlike workers who have occupational hearing loss, the professionals in this investigation seem not to experience an inordinate degree of stigmatization in their workplaces, although most believe that hearing loss has negatively affected their job performance. Some of the participants believe that they have lost their "competitive edge," and some believe that they have been denied promotions because of hearing loss. However, most report that they have overcome their hearing-related difficulties by various means, and many have developed a determination and stamina to remain active in the workforce. The majority of the participants seemed to be unfamiliar with the Americans with Disability Act, Public Law 101-336. The overriding theme to emerge is that professionals desire to maintain their competency to perform their jobs and will do what they have to do to "get the job done." The situations of professionals who have hearing loss can be modeled, with a central theme of maintaining job competency or a competitive edge. It is hypothesized that five factors affect professionals' abilities to continue their optimal work performance in the face of hearing loss: (a) self-concept and sense of internal locus of control, (b) use of hearing assistive technology, (c) supervisor's and co-workers' perceptions and

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.

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.

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.

The relationship between neonatal hyperbilirubinemia and sensorineural hearing loss.

PubMed

Corujo-Santana, Cándido; Falcón-González, Juan Carlos; Borkoski-Barreiro, Silvia Andrea; Pérez-Plasencia, Daniel; Ramos-Macías, Ángel

2015-01-01

Severe jaundice that requires exchange transfusion has become a relatively rare situation today. About 60% of full term neonates and 80% of premature ones will suffer from jaundice within the first week of life. Hyperbilirubinemia at birth is a risk factor associated with hearing loss that is usually further linked to other factors that might have an effect on hearing synergistically. This study aimed to identify the relationship between hyperbilirubinemia at birth as a risk factor for sensorineural hearing loss in children born at Complejo Hospitalario Universitario Insular Materno-Infantil de Gran Canaria, in the 2007-2011 period. This was a retrospective study of 796 newborns that had hyperbilirubinemia at birth, using transient evoked otoacoustic emissions and evoked auditory brainstem response. Hundred eighty-five newborns (23.24%) were referred for evoked auditory brainstem response. Hearing loss was diagnosed for 35 (4.39%): 18 neonates (51.43%) with conductive hearing loss and 17 (48.57%) with sensorineural hearing loss, 3 of which were diagnosed as bilateral profound hearing loss. Half of the children had other risk factors associated, the most frequent being exposure to ototoxic medications. The percentage of children diagnosed with sensorineural hearing loss that suffered hyperbilirubinemia at birth is higher than for the general population. Of those diagnosed, none had levels of indirect bilirubin≥20mg/dl, only 47% had hyperbilirubinemia at birth as a risk factor and 53% had another auditory risk factor associated. Copyright © 2014 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

Risk factors for sensorineural hearing loss in children.

PubMed

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

2012-01-01

In the last decade, tremendous progress has been made very rapidly in the development of Early Hearing Detection and Intervention (EHDI) systems as a major public health initiative. The percentage of infants screened annually in Spain has increased significantly since the EHDI systems have expanded to all autonomic regions. Historically, high risk indicators have been used for the identification of infants who should receive audiological evaluation but who live in geographic locations where universal hearing screening is not yet available, to help identify infants who pass neonatal screening but are at risk of developing delayed-onset hearing loss and to identify infants who may have passed neonatal screening but have mild forms of permanent hearing loss. In this review, the standard risk factors for hearing loss are analysed and the risk factors known to be associated with late onset or progressive hearing loss are identified. The recommendation for infants with a risk factor that may be considered as low risk is to perform at least one audiology assessment by 24-30 months. In contrast, for an infant with risk factors known to be associated with late onset or progressive hearing loss (such as cytomegalovirus infection or family history), early and more frequent assessment is appropriate. All infants should have an objective standardised screening of global development with a validated assessment tool at 9, 18 and 24-30 months of age or at any time if the health care professional or the family is concerned. Copyright © 2011 Elsevier España, S.L. All rights reserved.

Risk factors of sensorineural hearing loss in preterm infants.

PubMed

Borradori, C; Fawer, C L; Buclin, T; Calame, A

1997-01-01

Among 547 preterm infants of < or = 34 weeks gestation born between 1987 and 1991, 8 children (1.46%) developed severe progressive and bilateral sensorineural hearing loss. Perinatal risk factors of infants with hearing loss were compared with those of two control groups matched for gestation and birth weight and for perinatal complications. Our observations demonstrated an association of hearing loss with a higher incidence of perinatal complications. Ototoxicity appeared closely related to a prolonged administration and higher total dose of ototoxic drugs, particularly aminoglycosides and furosemide. Finally, we strongly recommend to prospectively and regularly perform audiologic assessment in sick preterm children as hearing loss is of delayed onset and in most cases bilateral and severe.

[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.

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.

Allelic Mutations of KITLG, Encoding KIT Ligand, Cause Asymmetric and Unilateral Hearing Loss and Waardenburg Syndrome Type 2

PubMed Central

Zazo Seco, Celia; Serrão de Castro, Luciana; van Nierop, Josephine W.; Morín, Matías; Jhangiani, Shalini; Verver, Eva J.J.; Schraders, Margit; Maiwald, Nadine; Wesdorp, Mieke; Venselaar, Hanka; Spruijt, Liesbeth; Oostrik, Jaap; Schoots, Jeroen; van Reeuwijk, Jeroen; Lelieveld, Stefan H.; Huygen, Patrick L.M.; Insenser, María; Admiraal, Ronald J.C.; Pennings, Ronald J.E.; Hoefsloot, Lies H.; Arias-Vásquez, Alejandro; de Ligt, Joep; Yntema, Helger G.; Jansen, Joop H.; Muzny, Donna M.; Huls, Gerwin; van Rossum, Michelle M.; Lupski, James R.; Moreno-Pelayo, Miguel Angel; Kunst, Henricus P.M.; Kremer, Hannie

2015-01-01

Linkage analysis combined with whole-exome sequencing in a large family with congenital and stable non-syndromic unilateral and asymmetric hearing loss (NS-UHL/AHL) revealed a heterozygous truncating mutation, c.286_303delinsT (p.Ser96Ter), in KITLG. This mutation co-segregated with NS-UHL/AHL as a dominant trait with reduced penetrance. By screening a panel of probands with NS-UHL/AHL, we found an additional mutation, c.200_202del (p.His67_Cys68delinsArg). In vitro studies revealed that the p.His67_Cys68delinsArg transmembrane isoform of KITLG is not detectable at the cell membrane, supporting pathogenicity. KITLG encodes a ligand for the KIT receptor. Also, KITLG-KIT signaling and MITF are suggested to mutually interact in melanocyte development. Because mutations in MITF are causative of Waardenburg syndrome type 2 (WS2), we screened KITLG in suspected WS2-affected probands. A heterozygous missense mutation, c.310C>G (p.Leu104Val), that segregated with WS2 was identified in a small family. In vitro studies revealed that the p.Leu104Val transmembrane isoform of KITLG is located at the cell membrane, as is wild-type KITLG. However, in culture media of transfected cells, the p.Leu104Val soluble isoform of KITLG was reduced, and no soluble p.His67_Cys68delinsArg and p.Ser96Ter KITLG could be detected. These data suggest that mutations in KITLG associated with NS-UHL/AHL have a loss-of-function effect. We speculate that the mechanism of the mutation underlying WS2 and leading to membrane incorporation and reduced secretion of KITLG occurs via a dominant-negative or gain-of-function effect. Our study unveils different phenotypes associated with KITLG, previously associated with pigmentation abnormalities, and will thereby improve the genetic counseling given to individuals with KITLG variants. PMID:26522471

Allelic Mutations of KITLG, Encoding KIT Ligand, Cause Asymmetric and Unilateral Hearing Loss and Waardenburg Syndrome Type 2.

PubMed

Zazo Seco, Celia; Serrão de Castro, Luciana; van Nierop, Josephine W; Morín, Matías; Jhangiani, Shalini; Verver, Eva J J; Schraders, Margit; Maiwald, Nadine; Wesdorp, Mieke; Venselaar, Hanka; Spruijt, Liesbeth; Oostrik, Jaap; Schoots, Jeroen; van Reeuwijk, Jeroen; Lelieveld, Stefan H; Huygen, Patrick L M; Insenser, María; Admiraal, Ronald J C; Pennings, Ronald J E; Hoefsloot, Lies H; Arias-Vásquez, Alejandro; de Ligt, Joep; Yntema, Helger G; Jansen, Joop H; Muzny, Donna M; Huls, Gerwin; van Rossum, Michelle M; Lupski, James R; Moreno-Pelayo, Miguel Angel; Kunst, Henricus P M; Kremer, Hannie

2015-11-05

Linkage analysis combined with whole-exome sequencing in a large family with congenital and stable non-syndromic unilateral and asymmetric hearing loss (NS-UHL/AHL) revealed a heterozygous truncating mutation, c.286_303delinsT (p.Ser96Ter), in KITLG. This mutation co-segregated with NS-UHL/AHL as a dominant trait with reduced penetrance. By screening a panel of probands with NS-UHL/AHL, we found an additional mutation, c.200_202del (p.His67_Cys68delinsArg). In vitro studies revealed that the p.His67_Cys68delinsArg transmembrane isoform of KITLG is not detectable at the cell membrane, supporting pathogenicity. KITLG encodes a ligand for the KIT receptor. Also, KITLG-KIT signaling and MITF are suggested to mutually interact in melanocyte development. Because mutations in MITF are causative of Waardenburg syndrome type 2 (WS2), we screened KITLG in suspected WS2-affected probands. A heterozygous missense mutation, c.310C>G (p.Leu104Val), that segregated with WS2 was identified in a small family. In vitro studies revealed that the p.Leu104Val transmembrane isoform of KITLG is located at the cell membrane, as is wild-type KITLG. However, in culture media of transfected cells, the p.Leu104Val soluble isoform of KITLG was reduced, and no soluble p.His67_Cys68delinsArg and p.Ser96Ter KITLG could be detected. These data suggest that mutations in KITLG associated with NS-UHL/AHL have a loss-of-function effect. We speculate that the mechanism of the mutation underlying WS2 and leading to membrane incorporation and reduced secretion of KITLG occurs via a dominant-negative or gain-of-function effect. Our study unveils different phenotypes associated with KITLG, previously associated with pigmentation abnormalities, and will thereby improve the genetic counseling given to individuals with KITLG variants. Copyright © 2015 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

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

A comparison of an audiometric screening survey with an in-depth research questionnaire for hearing loss and hearing loss risk factors

PubMed Central

Mosites, Emily; Neitzel, Richard; Galusha, Deron; Trufan, Sally; Dixon-Ernst, Christine; Rabinowitz, Peter

2017-01-01

Objective We assessed the reliability of a hearing risk factor screening survey used by hearing conservation programs for noise-exposed workers. Design We compared workers’ answers from the screening survey to their answers to a confidential research questionnaire regarding hearing loss risk factors. We calculated kappa statistics to test the correlation between yes/no questions in the research questionnaire compared to answers from one and five years of screening surveys. Study Sample We compared the screening survey and research questionnaire answers of 274 aluminum plant workers. Results Most of the questions in the in-company screening survey showed fair to moderate agreement with the research questionnaire (kappa range: −0.02, 0.57). Workers’ answers to the screening survey had better correlation with the research questionnaire when we compared five years of screening answers. For nearly all questions, workers were more likely to respond affirmatively on the research questionnaire than the screening survey. Conclusions Hearing conservation programs should be aware that workers may underreport hearing loss risk factors and functional hearing status on an audiometric screening survey. Validating company screening tools could help provide more accurate information on hearing loss and risk factors. PMID:27609310

A comparison of an audiometric screening survey with an in-depth research questionnaire for hearing loss and hearing loss risk factors.

PubMed

Mosites, Emily; Neitzel, Richard; Galusha, Deron; Trufan, Sally; Dixon-Ernst, Christine; Rabinowitz, Peter

2016-12-01

We assessed the reliability of a hearing risk factor screening survey used by hearing conservation programmes for noise-exposed workers. We compared workers' answers from the screening survey to their answers to a confidential research questionnaire regarding hearing loss risk factors. We calculated kappa statistics to test the correlation between yes/no questions in the research questionnaire compared to answers from 1 and 5 years of screening surveys. We compared the screening survey and research questionnaire answers of 274 aluminum plant workers. Most of the questions in the in-company screening survey showed fair to moderate agreement with the research questionnaire (kappa range: -0.02, 0.57). Workers' answers to the screening survey had better correlation with the research questionnaire when we compared 5 years of screening answers. For nearly all questions, workers were more likely to respond affirmatively on the research questionnaire than the screening survey. Hearing conservation programmes should be aware that workers may underreport hearing loss risk factors and functional hearing status on an audiometric screening survey. Validating company screening tools could help provide more accurate information on hearing loss and risk factors.

External ear anomalies and hearing impairment in Noonan Syndrome.

PubMed

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

2015-06-01

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

Frequency of Usher syndrome in two pediatric populations: Implications for genetic screening of deaf and hard of hearing children.

PubMed

Kimberling, William J; Hildebrand, Michael S; Shearer, A Eliot; Jensen, Maren L; Halder, Jennifer A; Trzupek, Karmen; Cohn, Edward S; Weleber, Richard G; Stone, Edwin M; Smith, Richard J H

2010-08-01

Usher syndrome is a major cause of genetic deafness and blindness. The hearing loss is usually congenital and the retinitis pigmentosa is progressive and first noticed in early childhood to the middle teenage years. Its frequency may be underestimated. Newly developed molecular technologies can detect the underlying gene mutation of this disorder early in life providing estimation of its prevalence in at risk pediatric populations and laying a foundation for its incorporation as an adjunct to newborn hearing screening programs. A total of 133 children from two deaf and hard of hearing pediatric populations were genotyped first for GJB2/6 and, if negative, then for Usher syndrome. Children were scored as positive if the test revealed > or =1 pathogenic mutations in any Usher gene. Fifteen children carried pathogenic mutations in one of the Usher genes; the number of deaf and hard of hearing children carrying Usher syndrome mutations was 15/133 (11.3%). The population prevalence was estimated to be 1/6000. Usher syndrome is more prevalent than has been reported before the genome project era. Early diagnosis of Usher syndrome has important positive implications for childhood safety, educational planning, genetic counseling, and treatment. The results demonstrate that DNA testing for Usher syndrome is feasible and may be a useful addition to newborn hearing screening programs.

Frequency of Usher Syndrome in Two Pediatric Populations: Implications for genetic screening of Deaf and Hard of Hearing Children

PubMed Central

Kimberling, William J.; Hildebrand, Michael S.; Shearer, A. Eliot; Jensen, Maren L.; Halder, Jennifer A.; Cohn, Edward S.; Weleber, Richard G.; Stone, Edwin M.; Smith, Richard J. H.

2011-01-01

Purpose Usher syndrome is a major cause of genetic deafblindness. The hearing loss is usually congenital and the retinitis pigmentosa is progressive and first noticed in early childhood to the middle teenage years. Its frequency may be underestimated. Newly developed molecular technologies can detect the underlying gene mutation of this disorder early in life providing estimation of its prevalence in at risk pediatric populations and laying a foundation for its incorporation as an adjunct to newborn hearing screening programs. Methods A total of 133 children from two deaf and hard of hearing pediatric populations were genotyped first for GJB2/6 and, if negative, then for Usher syndrome. Children were scored as positive if the test revealed ≥1 pathogenic mutations in any Usher gene. Results Fifteen children carried pathogenic mutations in one of the Usher genes; the number of deaf and hard of hearing children carrying Usher syndrome mutations was 15/133 (11.3%). The population prevalence was estimated to be 1/6000. Conclusion Usher syndrome is more prevalent than has been reported prior to the genome project era. Early diagnosis of Usher syndrome has important positive implications for childhood safety, educational planning, genetic counseling, and treatment. The results demonstrate that DNA testing for Usher syndrome is feasible and may be a useful addition to newborn hearing screening programs. PMID:20613545

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.

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.

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.

Hearing loss among older construction workers: Updated analyses.

PubMed

Dement, John; Welch, Laura S; Ringen, Knut; Cranford, Kim; Quinn, Patricia

2018-04-01

A prior study of this construction worker population found significant noise-associated hearing loss. This follow-up study included a much larger study population and consideration of additional risk factors. Data included audiometry, clinical chemistry, personal history, and work history. Qualitative exposure metrics for noise and solvents were developed. Analyses compared construction workers to an internal reference group with lower exposures and an external worker population with low noise exposure. Among participants (n = 19 127) an overall prevalence of hearing loss of 58% was observed, with significantly increased prevalence across all construction trades. Construction workers had significantly increased risk of hearing loss compared to reference populations, with increasing risk by work duration. Noise exposure, solvent exposure, hypertension, and smoking were significant risk factors in multivariate models. Results support a causal relationship between construction trades work and hearing loss. Prevention should focus on reducing exposure to noise, solvents, and cigarette smoke. © 2018 Wiley Periodicals, Inc.

Salivary Cortisol Profiles of Children with Hearing Loss

ERIC Educational Resources Information Center

Bess, Fred H.; Gustafson, Samantha J.; Corbett, Blythe A.; Lambert, E. Warren; Camarata, Stephen M.; Hornsby, Benjamin W. Y.

2016-01-01

Objectives: It has long been speculated that effortful listening places children with hearing loss at risk for fatigue. School-age children with hearing loss experiencing cumulative stress and listening fatigue on a daily basis might undergo dysregulation of hypothalamic-pituitary-adrenal (HPA) axis activity resulting in elevated or flattened…

Perinatal asphyxia, hypoxia, ischemia and hearing loss. An overview.

PubMed

Borg, E

1997-01-01

Birth hypoxia, asphyxia and ischemia have often been thought to be major causes of early hearing loss or deafness. The purpose of the present review is to focus on the role of these particular factors for perinatal auditory disorders. On the whole, only a small proportion of neonatal hearing loss is caused by perinatal factors. The exact etiology of neonatal hearing loss in children with complicated deliveries is difficult to evaluate due to the large number of causative factors that might be involved. After reviewing the literature covering the past 15-20 years, it is not possible to say that we understand the relative importance of different factors and their interactions. However, in the majority of studies, birth asphyxia is not correlated with hearing loss in babies with complicated deliveries Prolonged artificial ventilation, the presence of severe hypoxic ischemic encephalopathy or persistent pulmonary hypertension are important factors. The brain is more susceptible to anoxia than the ear and both are more likely to be damaged after prolonged pre-, peri- and postnatal hypoxia-ischemia than pure hypoxia during delivery. Perinatal hypoxia is more likely to cause a temporary hearing loss than a permanent one. Preterm babies are more vulnerable than term babies. The total number of risk factors, e.g. medicated by total length of stay in the neonatal intensive care unit and length of artificial ventilation, is the best predictor of risk for hearing loss of perinatal origin. The similarities between hearing loss and cerebral palsy are pointed out; only 8% of the cases of cerebral palsy are considered to be caused by conditions during delivery.

Hearing Loss in Osteogenesis Imperfecta: Characteristics and Treatment Considerations

PubMed Central

Pillion, Joseph P.; Vernick, David; Shapiro, Jay

2011-01-01

Osteogenesis imperfecta (OI) is the most common heritable disorder of connective tissue. It is associated with fractures following relatively minor injury, blue sclerae, dentinogenesis imperfecta, increased joint mobility, short stature, and hearing loss. Structures in the otic capsule and inner ear share in the histologic features common to other skeletal tissues. OI is due to mutations involving several genes, the most commonly involved are the COL1A1 or COL1A2 genes which are responsible for the synthesis of the proalpha-1 and proalpha-2 polypeptide chains that form the type I collagen triple helix. A genotype/phenotype relationship to hearing loss has not been established in OI. Hearing loss is commonly found in OI with prevalence rates ranging from 50 to 92% in some studies. Hearing loss in OI may be conductive, mixed, or sensorineural and is more common by the second or third decade. Treatment options such as hearing aids, stapes surgery, and cochlear implants are discussed. PMID:22567374

Speech-evoked auditory brainstem responses in children with hearing loss.

PubMed

Koravand, Amineh; Al Osman, Rida; Rivest, Véronique; Poulin, Catherine

2017-08-01

The main objective of the present study was to investigate subcortical auditory processing in children with sensorineural hearing loss. Auditory Brainstem Responses (ABRs) were recorded using click and speech/da/stimuli. Twenty-five children, aged 6-14 years old, participated in the study: 13 with normal hearing acuity and 12 with sensorineural hearing loss. No significant differences were observed for the click-evoked ABRs between normal hearing and hearing-impaired groups. For the speech-evoked ABRs, no significant differences were found for the latencies of the following responses between the two groups: onset (V and A), transition (C), one of the steady-state wave (F), and offset (O). However, the latency of the steady-state waves (D and E) was significantly longer for the hearing-impaired compared to the normal hearing group. Furthermore, the amplitude of the offset wave O and of the envelope frequency response (EFR) of the speech-evoked ABRs was significantly larger for the hearing-impaired compared to the normal hearing group. Results obtained from the speech-evoked ABRs suggest that children with a mild to moderately-severe sensorineural hearing loss have a specific pattern of subcortical auditory processing. Our results show differences for the speech-evoked ABRs in normal hearing children compared to hearing-impaired children. These results add to the body of the literature on how children with hearing loss process speech at the brainstem level. Copyright © 2017 Elsevier B.V. All rights reserved.

Effect of tractor driving on hearing loss in farmers in India.

PubMed

Kumar, Adarsh; Mathur, N N; Varghese, Mathew; Mohan, Dinesh; Singh, J K; Mahajan, Punnet

2005-04-01

Indian tractor drivers operate their tractors and other implements/equipment in an environment which can have extreme temperatures and high level of suspended particulate matter. In addition, Indian tractors do not have adequate vibration and noise attenuating designs features. This cross-sectional study compares the hearing status of tractor driving farmers (TDFs) (study group) and non-tractor driving farmers (NTDFs) (control group) matched for age, sex, generic/ethnic group, land holding, education levels, and work routines. Two groups of 50 experienced tractor-driving farmers and 50 non-driving farmers were selected from 2 villages, 50 km from Delhi. All participants were interviewed for details of work routine and noise exposures. An audiogram was performed and noise measurements taken on tractors and other machines. Self-reported hearing problems were similar (4 cases each) in both the groups of 50 farmers. However, audiogram analysis showed higher prevalence of abnormalities in TDFs. TDFs (24) had more often high frequency hearing loss when compared to NTDFs (14). The noise levels observed on tractors in different operations were in the range of 90-110 dB (A). Tractor noise levels exceeded the recommended safe limits of OSHA and NIOSH prescribed standards. TDFs had higher high frequency hearing loss than NTDFs. The mechanism of damage and prevention needs to be studied further. Copyright (c) 2005 Wiley-Liss, Inc.

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 socioeconomic impact of hearing loss in U.S. adults.

PubMed

Emmett, Susan D; Francis, Howard W

2015-03-01

To evaluate the associations between hearing loss and educational attainment, income, and unemployment/underemployment in U.S. adults. National cross-sectional survey. Ambulatory examination centers. Adults aged 20 to 69 years who participated in the 1999 to 2002 cycles of the NHANES (National Health and Nutrition Examination Survey) audiometric evaluation and income questionnaire (N = 3,379). Pure-tone audiometry, with hearing loss defined by World Health Organization criteria of bilateral pure-tone average of more than 25 dB (0.5, 1, 2, 4 kHz). Low educational attainment, defined as not completing high school; low income, defined as family income less than $20,000 per year; and unemployment or underemployment, defined as not having a job or working less than 35 hours per week. Individuals with hearing loss had 3.21 times higher odds of low educational attainment (95% confidence interval [95% CI], 2.20-4.68) compared with normal-hearing individuals. Controlling for education, age, sex, and race, individuals with hearing loss had 1.58 times higher odds of low income (95% CI, 1.16-2.15) and 1.98 times higher odds of being unemployed or underemployed (95% CI, 1.38-2.85) compared with normal-hearing individuals. Hearing loss is associated with low educational attainment in U.S. adults. Even after controlling for education and important demographic factors, hearing loss is independently associated with economic hardship, including both low income and unemployment/underemployment. The societal impact of hearing loss is profound in this nationally representative study and should be further evaluated with longitudinal cohorts. Received institutional review board approval (National Center for Health Statistics Institutional Review Board Protocol no. 98-12).

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

Minocycline attenuates noise-induced hearing loss in rats.

PubMed

Zhang, Jing; Song, Yong-Li; Tian, Ke-Yong; Qiu, Jian-Hua

2017-02-03

Noise-induced hearing loss (NIHL) is a serious health concern and prevention of hair cell death or therapeutic intervention at the early stage of NIHL is critical to preserve hearing. Minocycline is a semi-synthetic derivative of tetracycline and has been shown to have otoprotective effects in ototoxic drug-induced hearing impairment, however, whether minocycline can protect against NIHL has not been investigated. The present study demonstrated elevated ABR (auditory brainstem response) thresholds and outer hair cell loss following traumatic noise exposure, which was mitigated by intraperitoneal administration of minocycline (45mg/kg/d) for 5 consecutive days. In conclusion, the present study demonstrated that minocycline, a clinically approved drug with a good safety profile, can attenuate NIHL in rats and may potentially be used for treatment of hearing loss in clinic. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

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.

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

Risk factors for hearing loss in infants under universal hearing screening program in Northern Thailand

PubMed Central

Poonual, Watcharapol; Navacharoen, Niramon; Kangsanarak, Jaran; Namwongprom, Sirianong

2016-01-01

Objective To define the risk factors for hearing loss in infants (aged 3 months) under universal hearing screening program. Materials and methods A total of 3,120 infants (aged 3 months) who underwent hearing screening using a universal hearing screening program using automated otoacoustic emission test between November 1, 2010 and May 31, 2012 in Uttaradit Hospital, Buddhachinaraj Hospital, and Sawanpracharuk Hospital (tertiary hospitals) located in Northern Thailand were included in this prospective cohort study. Results Of the 3,120 infants, 135 (4.3%) were confirmed to have hearing loss with the conventional otoacoustic emission test. Five of these 135 infants (3.7%) with hearing loss showed test results consistent with auditory brainstem responses. From the univariable analysis, there were eleven potential risk factors associated with hearing deterioration. On multivariable analysis, the risk factors independently associated with hearing loss at 3 months were birth weight 1,500–2,500 g (risk ratio [RR] 1.6, 95% confidence interval [CI] 1.1–2.6), APGAR score <6 at 5 minutes (RR 2.2, 95% CI 1.1–4.4), craniofacial anomalies (RR 2.5, 95% CI 1.6–4.2), sepsis (RR 1.8, 95% CI 1.0–3.2), and ototoxic exposure (RR 4.1, 95% CI 1.9–8.6). Conclusion This study concluded that low birth weight, APGAR score <6 at 5 minutes, craniofacial anomalies, sepsis, and ototoxic exposure are the risk factors for bilateral hearing loss in infants (aged 3 months) and proper tests should be performed to identify these risk factors. As an outcome, under the present circumstances, it is suggested that infirmary/physicians/general practitioners/health action centers/polyclinics should carry out universal hearing screening in all infants before 36 weeks. The public health policy of Thailand regarding a universal hearing screening program is important for the prevention of disability and to enhance people’s quality of life. PMID:26766912

Middle Ear Infection (Chronic Otitis Media) and Hearing Loss

MedlinePlus

... You Middle Ear Infection (Chronic Otitis Media) and Hearing Loss Middle Ear Infection (Chronic Otitis Media) and ... loss. How does otitis media affect a child’s hearing? All children with middle ear infection or fluid ...

Novel mutations in the long isoform of the USH2A gene in patients with Usher syndrome type II or non-syndromic retinitis pigmentosa

PubMed Central

McGee, Terri L.; Seyedahmadi, Babak Jian; Sweeney, Meredith O.; Dryja, Thaddeus P.; Berson, Eliot L.

2010-01-01

Background Usher syndrome type II (USH2) is an autosomal recessive disorder characterized by retinitis pigmentosa (RP) and mild to moderate sensorineural hearing loss. Mutations in the USH2A gene are the most common cause of USH2 and are also a cause of some forms of RP without hearing loss (ie non-syndromic RP). The USH2A gene was initially identified as a transcript comprised of 21 exons but subsequently a longer isoform containing 72 exons was identified. Methods The 51 exons unique to the long isoform of USH2A were screened for mutations among a core set of 108 patients diagnosed with USH2 and 80 patients with non-syndromic RP who were all included in a previously reported screen of the short isoform of USH2A. For several exons, additional patients were screened. Results In total, 35 deleterious mutations were identified including 17 nonsense mutations, 9 frameshift mutations, 5 splice-site mutations, and 4 small in-frame deletions or insertions. Twenty-seven mutations were novel. In addition, 65 rare missense changes were identified. A method of classifying the deleterious effect of the missense changes was developed using the summed results of 4 different mutation assessment algorithms, SIFT, pMUT, PolyPhen, and AGVGD. This system classified 8 of the 65 changes as “likely deleterious” and 9 as “possibly deleterious”. Conclusion At least one mutation was identified in 57–63% of USH2 cases and 19–23% of cases of non-syndromic recessive RP (calculated without and including probable/possible deleterious changes) thus supporting that USH2A is the most common known cause of RP in the United States. PMID:20507924

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

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

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

Hearing aid fitting for visual and hearing impaired patients with Usher syndrome type IIa.

PubMed

Hartel, B P; Agterberg, M J H; Snik, A F; Kunst, H P M; van Opstal, A J; Bosman, A J; Pennings, R J E

2017-08-01

Usher syndrome is the leading cause of hereditary deaf-blindness. Most patients with Usher syndrome type IIa start using hearing aids from a young age. A serious complaint refers to interference between sound localisation abilities and adaptive sound processing (compression), as present in today's hearing aids. The aim of this study was to investigate the effect of advanced signal processing on binaural hearing, including sound localisation. In this prospective study, patients were fitted with hearing aids with a nonlinear (compression) and linear amplification programs. Data logging was used to objectively evaluate the use of either program. Performance was evaluated with a speech-in-noise test, a sound localisation test and two questionnaires focussing on self-reported benefit. Data logging confirmed that the reported use of hearing aids was high. The linear program was used significantly more often (average use: 77%) than the nonlinear program (average use: 17%). The results for speech intelligibility in noise and sound localisation did not show a significant difference between type of amplification. However, the self-reported outcomes showed higher scores on 'ease of communication' and overall benefit, and significant lower scores on disability for the new hearing aids when compared to their previous hearing aids with compression amplification. Patients with Usher syndrome type IIa prefer a linear amplification over nonlinear amplification when fitted with novel hearing aids. Apart from a significantly higher logged use, no difference in speech in noise and sound localisation was observed between linear and nonlinear amplification with the currently used tests. Further research is needed to evaluate the reasons behind the preference for the linear settings. © 2016 The Authors. Clinical Otolaryngology Published by John Wiley & Sons Ltd.

Interaction of smoking and occupational noise exposure on hearing loss: a cross-sectional study

PubMed Central

Pouryaghoub, Gholamreza; Mehrdad, Ramin; Mohammadi, Saber

2007-01-01

Background Noise is the most common hazardous agent at workplaces. Noise induced hearing loss (NIHL) has been known since the industrial revolution. Although NIHL is permanent, irreversible and frequent, it is preventable. The economic costs of NIHL have been estimated to be about billions of dollars. Besides, cigarette smoking is a common habit worldwide, and according to some recent studies smoking and noise may act in common causal pathways for hearing loss. Methods A cross-sectional study was designed to study the effect of smoking on NIHL in 206 male smoker workers and 206 male non-smoker workers in a large food-producing factory, in which workers were exposed to noise levels exceeding 85dBA. To determine noise exposure level, we used sound level measurements reported by industrial hygienists. A qualified audiologist assessed hearing acuity by using standardized audiometric procedures assuring at least 14 h of noise avoidance. Results We observed that the percentage of workers with hearing threshold differences of greater than or equal to 30 dB between 4000 Hz and 1000 Hz in both ears were 49.5% and 11.2% in smoker and non smoker groups, respectively (Odds ratio = 7.8, 95% CI = 4.7 – 13), and the percentage of workers with a hearing threshold of greater than 25dB at 4000 Hz in the better ear were 63.6% and 18.4% in smoker and non smoker groups, respectively. This difference was statistically significant after adjustment for age and exposure duration. Conclusion It can be concluded that smoking can accelerate noise induced hearing loss, but more research is needed to understand the underlying mechanisms. Accurate follow up of smoker workers who are exposed to noise levels exceeding 85 dBA is suggested. Smokers should periodically attend educational courses on "smoking cessation", especially in noisy workplaces. PMID:17605828

Consensus on Hearing Aid Candidature and Fitting for Mild Hearing Loss, With and Without Tinnitus: Delphi Review

PubMed Central

Hoare, Derek J.; Nicholson, Richard; Smith, Sandra; Hall, Deborah A.

2015-01-01

Objectives: In many countries including the United Kingdom, hearing aids are a first line of audiologic intervention for many people with tinnitus and aidable hearing loss. Nevertheless, there is a lack of high quality evidence to support that they are of benefit for tinnitus, and wide variability in their use in clinical practice especially for people with mild hearing loss. The aim of this study was to identify a consensus among a sample of UK clinicians on the criteria for hearing aid candidature and clinical practice in fitting hearing aids specifically for mild hearing loss with and without tinnitus. This will allow professionals to establish clinical benchmarks and to gauge their practice with that used elsewhere. Design: The Delphi technique, a systematic methodology that seeks consensus amongst experts through consultation using a series of iterative questionnaires, was used. A three-round Delphi survey explored clinical consensus among a panel of 29 UK hearing professionals. The authors measured panel agreement on 115 statements covering: (i) general factors affecting the decision to fit hearing aids, (ii) protocol-driven factors affecting the decision to fit hearing aids, (iii) general practice, and (iv) clinical observations. Consensus was defined as a priori ≥70% agreement across the panel. Results: Consensus was reached for 58 of the 115 statements. The broad areas of consensus were around factors important to consider when fitting hearing aids; hearing aid technology/features offered; and important clinical assessment to verify hearing aid fit (agreement of 70% or more). For patients with mild hearing loss, the greatest priority was given by clinicians to patient-centered criteria for fitting hearing aids: hearing difficulties, motivation to wear hearing aids, and impact of hearing loss on quality of life (chosen as top five by at least 64% of panelists). Objective measures were given a lower priority: degree of hearing loss and shape of the

Hearing loss and use of health services: a population-based cross-sectional study among Finnish older adults.

PubMed

Mikkola, Tuija M; Polku, Hannele; Sainio, Päivi; Koponen, Päivikki; Koskinen, Seppo; Viljanen, Anne

2016-11-08

Older adults with hearing difficulties face problems of communication which may lead to underuse of health services. This study investigated the association of hearing loss and self-reported hearing difficulty with the use of health services and unmet health care needs in older adults. Data on persons aged 65 and older (n = 2144) drawn from a population-based study, Health 2000, were analyzed. Hearing loss was determined with screening audiometry (n = 1680). Structured face-to-face interviews were used to assess self-reported hearing difficulty (n = 1962), use of health services (physician and nurse visits, health examinations, mental health services, physical therapy, health promotion groups, vision test, hearing test, mammography, PSA test) and perceived unmet health care needs. Multivariable logistic regression analyses were used. After adjusting for socio-economic and health-related confounders, persons with hearing loss (hearing level of better ear 0.5-2 kHz > 40 dB) were more likely to have used mental health services than those with non-impaired hearing (OR = 3.2, 95 % CI 1.3-7.9). Self-reported hearing difficulty was also associated with higher odds for mental health service use (OR = 2.1 95 % CI 1.2-3.5). Hearing was not associated with use of the other health services studied, except presenting for a hearing test. Persons with self-reported hearing difficulty were more likely to perceive unmet health care needs than those without hearing difficulty (OR = 1.7, 95 % CI 1.4-2.1). Older adults with hearing loss or self-reported hearing difficulty are as likely to use most health services as those without hearing loss. However, self-reported hearing difficulty is associated with experiencing unmet health care needs. Adequate health services should be ensured for older adults with hearing difficulties.

Hearing loss and employment in the United States.

PubMed

Kooser, Cathy

2013-01-01

This Sounding Board article will briefly review the biopsychosocial impact of hearing loss. It will consider the individual and employment; the laws supporting employment and the current vocational rehabilitation system assisting people with hearing loss remain in the workplace. It concludes with the author's suggestion of three systematic changes to enhance the employee's workplace success.

Familial pattern of large vestibular aqueduct syndrome in a Chinese family

PubMed Central

Hazmi, Mohd; Ab Aziz, A.; Asma, A.

2013-01-01

Large Vestibular Aqueduct Syndrome (LVAS) is the most common radiographic malformation in children with early onset of hearing loss. Usually its occurrence is non-familial, however intriguingly a portion of patients with LVAS is found to have evidence of genetic predisposition. We described cases of LVAS in two siblings of a Chinese family. The elder sister first presented with reduced hearing since childhood and her brother has a similar complaint upon further questioning. Their hearing test showed bilateral sensorineural hearing loss (SNHL) and computed tomography (CT) of temporal bone showed enlarged vestibular aqueduct in both patients. We described an approach to diagnosis of LVAS and highlight the importance of hearing assessment in genetic link hearing loss. PMID:27034633

Detection of Unilateral Hearing Loss by Stationary Wavelet Entropy.

PubMed

Zhang, Yudong; Nayak, Deepak Ranjan; Yang, Ming; Yuan, Ti-Fei; Liu, Bin; Lu, Huimin; Wang, Shuihua

2017-01-01

Sensorineural hearing loss is correlated to massive neurological or psychiatric disease. T1-weighted volumetric images were acquired from fourteen subjects with right-sided hearing loss (RHL), fifteen subjects with left-sided hearing loss (LHL), and twenty healthy controls (HC). We treated a three-class classification problem: HC, LHL, and RHL. Stationary wavelet entropy was employed to extract global features from magnetic resonance images of each subject. Those stationary wavelet entropy features were used as input to a single-hidden layer feedforward neuralnetwork classifier. The 10 repetition results of 10-fold cross validation show that the accuracies of HC, LHL, and RHL are 96.94%, 97.14%, and 97.35%, respectively. Our developed system is promising and effective in detecting hearing loss. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

Hearing loss and tinnitus in rock musicians: A Norwegian survey.

PubMed

Størmer, Carl Christian Lein; Laukli, Einar; Høydal, Erik Harry; Stenklev, Niels Christian

2015-01-01

Our focus in this study was to assess hearing thresholds and the prevalence and characteristics of tinnitus in a large group of rock musicians based in Norway. A further objective was to assess related factors such as exposure, instrument category, and the preventive effect of hearing protection. The study was a cross-sectional survey of rock musicians selected at random from a defined cohort of musicians. A random control group was included for comparison. We recruited 111 active musicians from the Oslo region, and a control group of 40 nonmusicians from the student population at the University of TromsØ. The subjects were investigated using clinical examination, pure tone audiometry, tympanometry, and a questionnaire. We observed a hearing loss in 37.8% of the rock musicians. Significantly poorer hearing thresholds were seen at most pure-tone frequencies in musicians than controls, with the most pronounced threshold shift at 6 kHz. The use of hearing protection, in particular custom-fitted earplugs, has a preventive effect but a minority of rock musicians apply them consistently. The degree of musical performance exposure was inversely related to the degree of hearing loss in our sample. Bass and guitar players had higher hearing thresholds than vocalists. We observed a 20% prevalence of chronic tinnitus but none of the affected musicians had severe tinnitus symptomatology. There was no statistical association between permanent tinnitus and hearing loss in our sample. We observed an increased prevalence of hearing loss and tinnitus in our sample of Norwegian rock musicians but the causal relationship between musical exposure and hearing loss or tinnitus is ambiguous. We recommend the use of hearing protection in rock musicians.

Hearing loss and tinnitus in rock musicians: A Norwegian survey

PubMed Central

Størmer, Carl Christian Lein; Laukli, Einar; Høydal, Erik Harry; Stenklev, Niels Christian

2015-01-01

Our focus in this study was to assess hearing thresholds and the prevalence and characteristics of tinnitus in a large group of rock musicians based in Norway. A further objective was to assess related factors such as exposure, instrument category, and the preventive effect of hearing protection. The study was a cross-sectional survey of rock musicians selected at random from a defined cohort of musicians. A random control group was included for comparison. We recruited 111 active musicians from the Oslo region, and a control group of 40 nonmusicians from the student population at the University of Tromsø. The subjects were investigated using clinical examination, pure tone audiometry, tympanometry, and a questionnaire. We observed a hearing loss in 37.8% of the rock musicians. Significantly poorer hearing thresholds were seen at most pure-tone frequencies in musicians than controls, with the most pronounced threshold shift at 6 kHz. The use of hearing protection, in particular custom-fitted earplugs, has a preventive effect but a minority of rock musicians apply them consistently. The degree of musical performance exposure was inversely related to the degree of hearing loss in our sample. Bass and guitar players had higher hearing thresholds than vocalists. We observed a 20% prevalence of chronic tinnitus but none of the affected musicians had severe tinnitus symptomatology. There was no statistical association between permanent tinnitus and hearing loss in our sample. We observed an increased prevalence of hearing loss and tinnitus in our sample of Norwegian rock musicians but the causal relationship between musical exposure and hearing loss or tinnitus is ambiguous. We recommend the use of hearing protection in rock musicians. PMID:26572701

Subjective Fatigue in Children With Hearing Loss: Some Preliminary Findings

PubMed Central

Hornsby, Benjamin W. Y.; Werfel, Krystal; Camarata, Stephen; Bess, Fred H.

2014-01-01

Purpose In this study, the authors examined the effect of hearing loss on subjective reports of fatigue in school-age children using a standardized measure. Methods As part of a larger ongoing study, the authors obtained subjective ratings of fatigue using the Pediatric Quality of Life Inventory (PedsQL) Multidimensional Fatigue Scale (Varni, Burwinkle, Katz, Meeske, & Dickinson, 2002). This standardized scale provides a measure of general fatigue, sleep/rest fatigue, cognitive fatigue, and an overall composite measure of fatigue. To date, data from 10 children with hearing loss (CHL) and 10 age-matched children with normal hearing (CNH) have been analyzed. Results These preliminary results show that subjective fatigue is increased in school-age children with hearing loss (Cohen's d = 0.78–1.90). In addition, the impact of hearing loss on fatigue in school-age children appears pervasive across multiple domains (general, sleep/rest, and cognitive fatigue). Conclusion School-age CHL reported significantly more fatigue than did CNH. These preliminary data are important given the negative academic and psychosocial consequences associated with fatigue. Further research is needed to determine the underlying mechanisms responsible for this increased fatigue in school-age children with hearing loss, and to identify factors that may modulate (e.g., degree of loss) and mediate (e.g., hearing aid or cochlear implant use) its impact. PMID:23824428

Radiation Therapy and Hearing Loss

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

Bhandare, Niranjan; Jackson, Andrew; Eisbruch, Avraham

2010-03-01

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

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

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

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

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

Prediction of hearing loss among the noise-exposed workers in a steel factory using artificial intelligence approach.

PubMed

Aliabadi, Mohsen; Farhadian, Maryam; Darvishi, Ebrahim

2015-08-01

Prediction of hearing loss in noisy workplaces is considered to be an important aspect of hearing conservation program. Artificial intelligence, as a new approach, can be used to predict the complex phenomenon such as hearing loss. Using artificial neural networks, this study aims to present an empirical model for the prediction of the hearing loss threshold among noise-exposed workers. Two hundred and ten workers employed in a steel factory were chosen, and their occupational exposure histories were collected. To determine the hearing loss threshold, the audiometric test was carried out using a calibrated audiometer. The personal noise exposure was also measured using a noise dosimeter in the workstations of workers. Finally, data obtained five variables, which can influence the hearing loss, were used for the development of the prediction model. Multilayer feed-forward neural networks with different structures were developed using MATLAB software. Neural network structures had one hidden layer with the number of neurons being approximately between 5 and 15 neurons. The best developed neural networks with one hidden layer and ten neurons could accurately predict the hearing loss threshold with RMSE = 2.6 dB and R(2) = 0.89. The results also confirmed that neural networks could provide more accurate predictions than multiple regressions. Since occupational hearing loss is frequently non-curable, results of accurate prediction can be used by occupational health experts to modify and improve noise exposure conditions.

Childhood Hearing Health: Educating for Prevention of Hearing Loss

PubMed Central

Lacerda, Adriana Bender Moreira; Gonçalves, Claudia Giglio de Oliveira; Lacerda, Giselle; Lobato, Diolén Conceição Barros; Santos, Luciana; Moreira, Aline Carlezzo; Ribas, Angela

2014-01-01

Introduction The presence of noise in our society has attracted the attention of health professionals, including speech-language pathologists, who have been charged along with educators with developing hearing conservation programs in schools. Objective To describe the results of three strategies for awareness and hearing preservation in first to fourth grades in public elementary schools. Methods The level of environmental noise in classrooms was assessed, and 638 elementary school students from first to fourth grades, 5 to 10 years of age, were audiologically evaluated. After the evaluations, educational activities were presented to children and educators. Results The noise level in the classroom ranged from 71.8 to 94.8 A-weighted decibels. The environment of the classroom was found to promote sound reverberation, which hinders communication. Thirty-two students (5.1%) presented hearing alterations. Conclusion The application of strategies for a hearing conservation program at the school showed that noise is present in the room, and hearing loss, sometimes silent, affects schoolchildren. Students and teachers were aware that hearing problems can be prevented. Avoiding exposure to noise and improving the acoustics in classrooms are essential. PMID:25992146

Comparison of Various Anthropometric Indices as Risk Factors for Hearing Impairment in Asian Women.

PubMed

Kang, Seok Hui; Jung, Da Jung; Lee, Kyu Yup; Choi, Eun Woo; Do, Jun Young

2015-01-01

The objective of the present study was to examine the associations between various anthropometric measures and metabolic syndrome and hearing impairment in Asian women. We identified 11,755 women who underwent voluntary routine health checkups at Yeungnam University Hospital between June 2008 and April 2014. Among these patients, 2,485 participants were <40 years old, and 1,072 participants lacked information regarding their laboratory findings or hearing and were therefore excluded. In total 8,198 participants were recruited into our study. The AUROC value for metabolic syndrome was 0.790 for the waist to hip ratio (WHR). The cutoff value was 0.939. The sensitivity and specificity for predicting metabolic syndrome were 72.7% and 71.7%, respectively. The AUROC value for hearing loss was 0.758 for WHR. The cutoff value was 0.932. The sensitivity and specificity for predicting hearing loss were 65.8% and 73.4%, respectively. The WHR had the highest AUC and was the best predictor of metabolic syndrome and hearing loss. Univariate and multivariate linear regression analyses showed that WHR levels were positively associated with four hearing thresholds including averaged hearing threshold and low, middle, and high frequency thresholds. In addition, multivariate logistic analysis revealed that those with a high WHR had a 1.347-fold increased risk of hearing loss compared with the participants with a low WHR. Our results demonstrated that WHR may be a surrogate marker for predicting the risk of hearing loss resulting from metabolic syndrome.

Comparison of Ear-Canal Reflectance and Umbo Velocity in Patients with Conductive Hearing Loss

NASA Astrophysics Data System (ADS)

Merchant, Gabrielle R.; Nakajima, Hideko H.; Pisano, Dominic V.; Röösli, Christof; Hamade, Mohamad A.; Mafoud, Lorice; Halpin, Christopher F.; Merchant, Saumil N.; Rosowski, John J.

2011-11-01

Patients who present at hearing clinics with a conductive hearing loss (CHL) in the presence of an intact, healthy tympanic membrane create a unique challenge for otologists. While patient counseling, treatment options, and outcome vary with differing middle-ear pathologies, a non-invasive diagnostic that can differentiate between these pathologies does not currently exist. We evaluated the clinical utility and diagnostic accuracy of two non-invasive measures of middle-ear mechanics: ear-canal reflectance (ECR) and umbo velocity (VU).

Genetic heterogeneity in Usher syndrome.

PubMed

Keats, Bronya J B; Savas, Sevtap

2004-09-15

Mutations in seven different genes have been associated with Usher syndrome, and an additional four loci have been mapped. The identified genes encode myosin VIIa, harmonin (a PDZ-domain protein), cadherin 23, protocadherin 15, sans (a scaffold-like protein), usherin and clarin. Three clinical types of Usher syndrome have been described: USH1 patients have severe to profound congenital hearing loss, vestibular dysfunction, and retinal degeneration beginning in childhood, those with USH2 have moderate to severe congenital hearing loss, normal vestibular function, and later onset of retinitis pigmentosa, and USH3 patients have progressive hearing loss, which distinguishes them from the other two types. The shaker-1, waltzer, Ames waltzer, and Jackson shaker mice provide murine models for four of the genetic forms of Usher syndrome. Ongoing studies are enabling early diagnosis of Usher syndrome in children who present with hearing loss, thus providing time to prepare for the onset of visual loss. Copyright 2004 Wiley-Liss, Inc.

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.

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

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

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.

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

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

[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.

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…

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.

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

Pragmatic Abilities of Children with Hearing Loss Using Cochlear Implants or Hearing Aids Compared to Hearing Children