Science.gov

Sample records for non-syndromic hearing loss

  1. An update of common autosomal recessive non-syndromic hearing loss genes in Iranian population.

    PubMed

    Ghasemnejad, Tohid; Shekari Khaniani, Mahmoud; Zarei, Fatemeh; Farbodnia, Mina; Mansoori Derakhshan, Sima

    2017-06-01

    Autosomal-recessive genes are responsible for about 80% of the hereditary non-syndromic hearing loss (NSHL) cases. In Iran, due to consanguineous marriages, NSHL is the second most frequent disability after intellectual disability, occurring one in 16 individuals. Enormous heterogeneity in the genetic pathology of hearing loss causes a major challenge in identification of responsible genes. In Iran, GJB2 is responsible for the most cases of pre-lingual and non-syndromic hearing loss (with frequency of 16.7%) which followed by other genes with lower frequency. Although several studies have indicated that a large proportion of both syndromic and non-syndromic hearing loss in Iranian populations are caused by defects in just a few genes, new detection strategies such as NGS (Next-generation sequencing) have increased the spectrum of responsible mutations. However, by applying this technique in Iran patients screening, the role of lots of novel related genes have been reported. In this review, we aim to describe function of these genes and their contribution to non-syndromic genetic hearing loss in Iranian population and we classify the genes by their functions. Copyright © 2017 Elsevier B.V. All rights reserved.

  2. TBC1D24 Mutation Causes Autosomal Dominant Non-Syndromic Hearing Loss

    PubMed Central

    Azaiez, Hela; Booth, Kevin T.; Bu, Fengxiao; Huygen, Patrick; Shibata, Seiji; Shearer, A. Eliot; Kolbe, Diana; Meyer, Nicole; Black-Ziegelbein, E. Ann; Smith, Richard J.H.

    2014-01-01

    Hereditary hearing loss (HHL) is extremely heterogeneous. Over 70 genes have been identified to date, and with the advent of massively parallel sequencing, the pace of novel gene discovery has accelerated. In a family segregating progressive autosomal dominant non-syndromic hearing loss (ADNSHL) we used OtoSCOPE® to exclude mutations in known deafness genes and then performed segregation mapping and whole exome sequencing (WES) to identify a unique variant, p.Ser178Leu, in TBC1D24 that segregates with the hearing loss phenotype. TBC1D24 encodes a GTPase-activating protein expressed in the cochlea. Ser178 is highly conserved across vertebrates and its change is predicted to be damaging. Other variants in TBC1D24 have been associated with a panoply of clinical symptoms including autosomal recessive NSHL (ARNSHL), syndromic hearing impairment associated with onychodystrophy, osteodystrophy, mental retardation and seizures (DOORS syndrome), and a wide range of epileptic disorders. PMID:24729539

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

    PubMed

    Schrijver, Iris

    2004-11-01

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

  4. Copy number variants are a common cause of non-syndromic hearing loss

    PubMed Central

    2014-01-01

    Background Copy number variants (CNVs) are a well-recognized cause of genetic disease; however, methods for their identification are often gene-specific, excluded as ‘routine’ in screens of genetically heterogeneous disorders, and not implemented in most next-generation sequencing pipelines. For this reason, the contribution of CNVs to non-syndromic hearing loss (NSHL) is most likely under-recognized. We aimed to incorporate a method for CNV identification as part of our standard analysis pipeline and to determine the contribution of CNVs to genetic hearing loss. Methods We used targeted genomic enrichment and massively parallel sequencing to isolate and sequence all exons of all genes known to cause NSHL. We completed testing on 686 patients with hearing loss with no exclusions based on type of hearing loss or any other clinical features. For analysis we used an integrated method for detection of single nucleotide changes, indels and CNVs. CNVs were identified using a previously published method that utilizes median read-depth ratios and a sliding-window approach. Results Of 686 patients tested, 15.2% (104) carried at least one CNV within a known deafness gene. Of the 38.9% (267) of individuals for whom we were able to determine a genetic cause of hearing loss, a CNV was implicated in 18.7% (50). We identified CNVs in 16 different genes including 7 genes for which no CNVs have been previously reported. CNVs of STRC were most common (73% of CNVs identified) followed by CNVs of OTOA (13% of CNVs identified). Conclusion CNVs are an important cause of NSHL and their detection must be included in comprehensive genetic testing for hearing loss. PMID:24963352

  5. Etiology and associated GJB2 mutations in Mauritanian children with non-syndromic hearing loss.

    PubMed

    Moctar, Ely Cheikh Mohamed; Riahi, Zied; El Hachmi, Hala; Veten, Fatimetou; Meiloud, Ghlana; Bonnet, Christine; Abdelhak, Sonia; Errami, Mohammed; Houmeida, Ahmed

    2016-11-01

    Origins of all hearing impairment forms may be divided into genetic mutations and acquired influence. Both carry damage to the inner ear structure resulting in a mild to profound dysfunction of the auditory system. The purpose of this study was to assess the different etiologies of deafness in two reference centers for hearing-impaired children in Nouakchott/Mauritania. Data on gender, age, consanguinity, etiology and family history of deafness were gathered by interviewing the custodians of 139 children with hearing loss. DNA of pupils with hereditary non-syndromic deafness was then screened for GJB2 mutations by sequencing methods. Postnatal hearing loss was found in 36 (25.8 %) out of the 139 children surveyed. The main etiologies of this group were infections caused by meningitis (12.9 %) and measles (2.8 %). Unknown and ototoxic origins accounted for, respectively, 5.7 and 3.5 %. In 103 (74.1 %) children, deafness was identified near after the time of birth and, therefore, presumed as congenital. 56.8 % of deaf children had consanguineous parents. Two GJB2 mutations, c.del35G with an allele frequency of 4.7 % and R32C (3.7 %) were detected. Infections such as meningitis and measles were the most prevalent causes of postnatal deafness. In cases of congenital hearing impairment, two GJB2 allele variants, i.e., del35G and R32C (3.7 %) were detected. Extended genetic testing is recommended for a more comprehensive determination of congenital causes.

  6. Variations in Multiple Syndromic Deafness Genes Mimic Non-syndromic Hearing Loss

    PubMed Central

    Bademci, G.; Cengiz, F. B.; Foster II, J.; Duman, D.; Sennaroglu, L.; Diaz-Horta, O.; Atik, T.; Kirazli, T.; Olgun, L.; Alper, H.; Menendez, I.; Loclar, I.; Sennaroglu, G.; Tokgoz-Yilmaz, S.; Guo, S.; Olgun, Y.; Mahdieh, N.; Bonyadi, M.; Bozan, N.; Ayral, A.; Ozkinay, F.; Yildirim-Baylan, M.; Blanton, S. H.; Tekin, M.

    2016-01-01

    The genetics of both syndromic (SHL) and non-syndromic hearing loss (NSHL) is characterized by a high degree of genetic heterogeneity. We analyzed whole exome sequencing data of 102 unrelated probands with apparently NSHL without a causative variant in known NSHL genes. We detected five causative variants in different SHL genes (SOX10, MITF, PTPN11, CHD7, and KMT2D) in five (4.9%) probands. Clinical re-evaluation of these probands shows that some of them have subtle syndromic findings, while none of them meets clinical criteria for the diagnosis of the associated syndrome (Waardenburg (SOX10 and MITF), Kallmann (CHD7 and SOX10), Noonan/LEOPARD (PTPN11), CHARGE (CHD7), or Kabuki (KMT2D). This study demonstrates that individuals who are evaluated for NSHL can have pathogenic variants in SHL genes that are not usually considered for etiologic studies. PMID:27562378

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

    SciTech Connect

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

    2010-06-04

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

  8. Study of a Brazilian family presenting non-syndromic hearing loss with mitochondrial inheritance.

    PubMed

    Pupo, Altair Cadrobbi; Pirana, Sulene; Spinelli, Mauro; Lezirovitz, Karina; Mingroni Netto, Regina C; Macedo, Lisandra S

    2008-01-01

    We hereby report on the audiological and genetic findings in individuals from a Brazilian family, with the following mitochondrial mutation A1555G in the 12SrRNA gene (MT-RNR-1). Nine individuals underwent speech, audiologic (tonal audiometry and logoaudiometry) and genetic evaluations. Eight individuals among the A1555G carriers were affected by hearing impairment and one person had normal hearing thresholds till the end of the present study. The audiologic evaluation results indicated normal hearing thresholds all the way to bilateral profound hearing loss with post-lingual onset and variable configuration. Two affected siblings presented progressive hearing loss. It was impossible to precise the time of hearing loss onset; however, the impairment was present in both children and adults. The genetic study revealed the A1555G mitochondrial mutation in the 12SrRNA gene. Given the prevalence of mitochondrial mutations as a cause of hearing loss, it is fundamental to perform the etiopathologic diagnosis in order to postpone the onset or avoid hearing impairment progression. This kind of hearing impairment represents a challenge to the professionals since there are no physical traits that indicate genetic transmission.

  9. A novel compound heterozygous mutation in the GJB2 gene causing non-syndromic hearing loss in a family.

    PubMed

    Wei, Qinjun; Liu, Youguo; Wang, Shuai; Liu, Tingting; Lu, Yajie; Xing, Guangqian; Cao, Xin

    2014-02-01

    Mutations in the GJB2 gene are responsible for up to 50% of cases of non-syndromic recessive hearing loss, with c.35delG, c.167delT and c.235delC being the predominant mutations in many world populations. However, a large number of rare mutations in this gene may also contribute to hearing loss. The aim of the present study was to conduct a clinical and molecular characterization of a Chinese family with non-syndromic hearing loss. Sequence analysis of the GJB2 gene led to the identification of a novel compound heterozygous mutation c.257C>G (p.T86R)/c.605ins46 in two profoundly deaf siblings whose hearing parents were each heterozygous, either for the c.257C>G (paternal) or for the c.605ins46 (maternal) mutations. Both c.257C>G and c.605ins46 are rare GJB2 mutations that have previously been reported to segregate with autosomal recessive hearing loss exclusively in East Asian populations. To study the pathogenic effect of the compound heterozygous mutation, a three-dimensional model was constructed and Anolea mean force potential energy was predicted for a bioinformatic structural analysis. HEK293 cells were used to study the pathogenic effect of mutant connexin 26 proteins. The results suggested that the c.257C>G (p.T86R)/c.605ins46 mutations in the GJB2 gene provides a novel molecular explanation for the role of the GJB2 gene in hearing loss.

  10. A novel compound heterozygous mutation (35delG, 363delC) in the Connexin 26 gene causes non-syndromic autosomal recessive hearing loss.

    PubMed

    Onsori, Habib; Rahmati, Mohammad; Fazli, Davood

    2014-01-01

    Mutations in the Connexin 26 (Cx26) gene are a common cause of hereditary hearing loss in different populations. In the present study, an Iranian patient with bilateral hearing loss underwent molecular analysis for the causative mutation. DNA studies were performed for the Cx26 gene by PCR and sequencing methods. We describe a novel compound heterozygous mutation (35delG, 363delC) in the Cx26 gene that is strongly associated with congenital non-syndromic hearing loss (NSHL).

  11. [Non-syndromic hereditary hearing impairment].

    PubMed

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

    2007-04-01

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

  12. GJB2 Mutation Spectrum and Genotype-Phenotype Correlation in 1067 Han Chinese Subjects with Non-Syndromic Hearing Loss.

    PubMed

    Zheng, Jing; Ying, Zhengbiao; Cai, Zhaoyang; Sun, Dongmei; He, Zheyun; Gao, Yinglong; Zhang, Ting; Zhu, Yi; Chen, Ye; Guan, Min-Xin

    2015-01-01

    Mutations in Gap Junction Beta 2 (GJB2) have been reported to be a major cause of non-syndromic hearing loss in many populations worldwide. The spectrums and frequencies of GJB2 variants vary substantially among different ethnic groups, and the genotypes among these populations remain poorly understood. In the present study, we carried out a systematic and extended mutational screening of GJB2 gene in 1067 Han Chinese subjects with non-syndromic hearing loss, and the resultant GJB2 variants were evaluated by phylogenetic, structural and bioinformatic analysis. A total of 25 (23 known and 2 novel) GJB2 variants were identified, including 6 frameshift mutations, 1 nonsense mutation, 16 missense mutations and 2 silent mutations. In this cohort, c.235delC is the most frequently observed pathogenic mutation. The phylogenetic, structural and bioinformatic analysis showed that 2 novel variants c.127G>T (p.V43L), c.293G>C (p.R98P) and 2 known variants c. 107T>C (p.L36P) and c.187G>T (p.V63L) are localized at highly conserved amino acids. In addition, these 4 mutations are absent in 203 healthy individuals, therefore, they are probably the most likely candidate pathogenic mutations. In addition, 66 (24 novel and 42 known) genotypes were identified, including 6 homozygotes, 20 compound heterozygotes, 18 single heterozygotes, 21 genotypes harboring only polymorphism(s) and the wild type genotype. Among these, 153 (14.34%) subjects were homozygous for pathogenic mutations, 63 (5.91%) were compound heterozygotes, and 157 (14.71%) carried single heterozygous mutation. Furthermore, 65.28% (141/216) of these cases with two pathogenic mutations exhibited profound hearing loss. These data suggested that mutations in GJB2 gene are responsible for approximately 34.96% of non-syndromic hearing loss in Han Chinese population from Zhejiang Province in eastern China. In addition, our results also strongly supported the idea that other factors such as alterations in regulatory regions

  13. The association between GJB2 mutation and GJB6 gene in non syndromic hearing loss school children.

    PubMed

    Asma, A; Ashwaq, A; Norzana, A G; Atmadini, A Maizaton; Ruszymah, B H I; Saim, L; Wahida, I Farah

    2011-06-01

    Recently, molecular testing for GJB2 mutations has become the standard of care for the diagnosis of patients with non syndromic hearing impairment of unknown cause. The aims of this study are to determine the association between GJB2 mutation and GJB6 and to report the variation of mutations in deaf students who have heterozygous GJB2. This retrospective study was conducted at Universiti Kebangsaan Malaysia Medical Center (UKMMC). Data was collected from previous files and records from Tissue Engineering and Human Genetic Research Group Laboratory. Approval from Ethical Committee was obtained prior to the study. A total of 138 students have been screened in previous studies in UKMMC for the presence of GJB2 mutations as a cause for hearing loss. Thirty four of the 138 subjects have GJB2 mutations; 2 showed homozygous mutations whereas another 32 were heterozygous for GJB2 gene mutation. Only 31 DNA samples of students presented with sensorineural hearing loss with heterozygous mutation in GJB2 gene were included in this study. The sequencing results obtained were analyzed. The degree of hearing loss of those students with association between GJB2 mutation and GJB6 mutation will be discussed. Five out of 31 subjects (16.2%) have mutations in their GJB6 gene, suggesting a digenic inheritance of GJB2/GJB6 mutation. In total, four novel mutations were identified; E137D (n=1), R32Q (n=1), E101K (n=1) and Y156H (n=1) and one mutation deletion; 366delT (n=1). All students with association GJB2 mutation and GJB6 showed severe to profound hearing loss in both ears. Interestingly this study not detected the large deletion of 342 kb in GJB6 gene suggesting that the mutation is very rare in this region compared to certain parts of the world.

  14. Investigation of LRTOMT gene (locus DFNB63) mutations in Iranian patients with autosomal recessive non-syndromic hearing loss

    PubMed Central

    Taghizadeh, Seyyed Hossein; Kazeminezhad, Seyyed Reza; Sefidgar, Seyyed Ali Asghar; Yazdanpanahi, Nasrin; Tabatabaeifar, Mohammad Amin; Yousefi, Ahmad; Lesani, Seyyed Mohammad; Abolhasani, Marziyeh; Hashemzadeh Chaleshtori, Morteza

    2013-01-01

    Hearing loss (HL) is the most frequent sensory defect affecting 1 in 1000 neonates. This can occur due to genetic or environmental causes or both. The genetic causes are very heterogenous and over 100 loci have been identified to cause autosomal recessive non - syndromic hearing loss (ARNSHL). The aim of this study was to determine the contribution of the LRTOMT gene mutations in causing ARNSHL. One hundred fifty seven pupils affected with ARNSHL from Azarbaijan Sharghi, Kordestan, Gilan and Golestan provinces, north and west of Iran, were ascertained. In this descriptive - laboratory study, the presence of LRTOMT mutations were initially checked using PCR – Single - strand conformation polymorphism (SSCP) and heteroduplex analysis (HA) strategy. Samples with shifted bands on the gel were confirmed by DNA sequencing method. The PCR-SSCP/HA and the subsequent direct DNA sequencing showed no mutation in the population studied. We conclude that LRTOMT mutations have no role in causing sporadic deafness in the studied population. Further studies on other populations and samples could clarify the exact role of LRTOMT mutations. PMID:24551789

  15. Investigation of LRTOMT gene (locus DFNB63) mutations in Iranian patients with autosomal recessive non-syndromic hearing loss.

    PubMed

    Taghizadeh, Seyyed Hossein; Kazeminezhad, Seyyed Reza; Sefidgar, Seyyed Ali Asghar; Yazdanpanahi, Nasrin; Tabatabaeifar, Mohammad Amin; Yousefi, Ahmad; Lesani, Seyyed Mohammad; Abolhasani, Marziyeh; Hashemzadeh Chaleshtori, Morteza

    2013-01-01

    Hearing loss (HL) is the most frequent sensory defect affecting 1 in 1000 neonates. This can occur due to genetic or environmental causes or both. The genetic causes are very heterogenous and over 100 loci have been identified to cause autosomal recessive non - syndromic hearing loss (ARNSHL). The aim of this study was to determine the contribution of the LRTOMT gene mutations in causing ARNSHL. One hundred fifty seven pupils affected with ARNSHL from Azarbaijan Sharghi, Kordestan, Gilan and Golestan provinces, north and west of Iran, were ascertained. In this descriptive - laboratory study, the presence of LRTOMT mutations were initially checked using PCR - Single - strand conformation polymorphism (SSCP) and heteroduplex analysis (HA) strategy. Samples with shifted bands on the gel were confirmed by DNA sequencing method. The PCR-SSCP/HA and the subsequent direct DNA sequencing showed no mutation in the population studied. We conclude that LRTOMT mutations have no role in causing sporadic deafness in the studied population. Further studies on other populations and samples could clarify the exact role of LRTOMT mutations.

  16. Screening for gap junction protein beta-2 gene mutations in Malays with autosomal recessive, non-syndromic hearing loss, using denaturing high performance liquid chromatography.

    PubMed

    Aishah, Z Siti; Khairi, M D Mohd; Normastura, A R; Zafarina, Z; Zilfalil, B A

    2008-12-01

    To determine the frequency and type of gap junction protein beta-2 gene mutations in Malay patients with autosomal recessive, non-syndromic hearing loss. A total of 33 Malay patients with autosomal recessive, non-syndromic hearing loss were screened for mutations in the Cx26 coding region. Deoxyribonucleic acid was extracted from buccal swab samples and subjected to polymerase chain reaction. Slow-reannealing was performed, followed by screening using denaturing high performance liquid chromatography. Eight of the samples (24.2 per cent) showed heterozygous peaks, and further sequencing of these samples revealed four patients (50.0 per cent) with the W24X mutation, two (25.0 per cent) with the V37I mutation and another two (25.0 per cent) with the G4D mutation. Analysis of buccal swab samples by denaturing high performance liquid chromatography is noninvasive and suitable for rapid and reliable screening of gap junction protein beta-2 gene mutations in patients with autosomal recessive, non-syndromic hearing loss. Malay patients with autosomal recessive, non-syndromic hearing loss have different kinds of gap junction protein beta-2 gene mutations which are rarely found in other populations.

  17. Autozygosity mapping of autosomal recessive non-syndromic sensorineural hearing loss (ARNSSNHL)

    SciTech Connect

    Brown, K.A.; Nobel, A.; Markham, A.F.

    1994-09-01

    Congenital deafness affects about 1 in 2000 persons and is of genetic origin in approximately half these cases. The majority of congenital deafness is non-syndromic and over 75% of cases are compatible with autosomal recessive inheritance. Mapping of the loci responsible for ARNSSNHL will be complicated by genetic heterogeneity. Our approach to isolating genes involved in ARNSSNHL is by autozygosity mapping which involves the genetic analysis of children resulting from consanguineous marriages with the aim of identifying regions of homozygosity unique to the genomes of affected individuals which have been inherited from a common ancestor. The population employed in this study is the Pakistani community of Leeds, Bradford and Manchester in the UK which originated from the Mirpur region of Pakistan. Microsatellite analysis of the genome with markers spaced, on average, 10 cM apart is in progress and the investigation of 15 consanguineous families has identified one family which shows linkage to human chromosome 13q. This family appears to be linked to the same autosomal recessive deafness locus as two Tunisian families recently described and confirms that this chromosome 13q locus is also responsible, although as a minor contributor, to the deafness observed in the Pakistani population.

  18. Correlation analysis of phenotype and genotype of GJB2 in patients with non-syndromic hearing loss in China.

    PubMed

    Dai, Zhi-Yao; Sun, Bao-Chun; Huang, Sha-Sha; Yuan, Yong-Yi; Zhu, Yu-Hua; Su, Yu; Dai, Pu

    2015-10-10

    Disease-associated mutations in GJB2 gene are one of the major reasons that can cause non-syndromic sensorineural hearing loss (NSHL). GJB2 gene deafness has various clinical phenotypes. This study aims to analyze characteristics and relationships of clinical phenotypes through analyzing 1481 NSHL cases and 190 GJB2 deafness patients (with dual gene mutations). All the patients diagnosed as deaf disease molecular diagnostics were obtained from the people's liberation army general hospital from March 2007 to March 2011. The accession number of GJB2 was NM_004004 in GenBank, and sequence alignment and annotation were performed using GeneTool software. In NSHL patients, mutated allele frequency in GJB2 was 20.57%, and the preponderant type was c.235delC (11.84%) followed by c.109G>A (3.75%). Mutation rate of double allelic gene was 16.18%, including 8.43% of homozygous mutation rate and 7.75% of recombination heterozygosis mutation. Moreover, auditory threshold of GJB2 biallelic marker was associated with ages of onset, while no significant correlation was detected with disease time and whether the inner ear malformation. Similar clinical phenotype could be detected between patients with c.109G>A dual gene mutation and dual gene mutation. However, in the aspect of hearing impairment, the phenomenon of pathopoiesia caused by mutation of c.109G>A was poorer than the other mutations, and even near those patients without pathogenic mutations. Our study further shows the definite relationship of clinical phenotype and genotype in GJB2 gene correlated deafness, and these results can provide basis for revealing pathogenesis, gene diagnosis and consult of deafness. The level of evidence in the study is level 4 (case series). Copyright © 2015 Elsevier B.V. All rights reserved.

  19. New polymorphic mtDNA restriction site in the 12S rRNA gene detected in Tunisian patients with non-syndromic hearing loss

    SciTech Connect

    Mkaouar-Rebai, Emna Tlili, Abdelaziz; Masmoudi, Saber; Charfeddine, Ilhem; Fakhfakh, Faiza

    2008-05-09

    The 12S rRNA gene was shown to be a hot spot for aminoglycoside-induced and non-syndromic hearing loss since several deafness-associated mtDNA mutations were identified in this gene. Among them, we distinguished the A1555G, the C1494T and the T1095C mutations and C-insertion or deletion at position 961. One hundred Tunisian patients with non-syndromic hearing loss and 100 hearing individuals were analysed in this study. A PCR-RFLP analysis with HaeIII restriction enzyme showed the presence of the A1555G mutation in the 12S rRNA gene in only one out of the 100 patients. In addition, PCR-RFLP and radioactive PCR revealed the presence of a new HaeIII polymorphic restriction site in the same gene of 12S rRNA site in 4 patients with non-syndromic hearing loss. UVIDOC-008-XD analyses showed the presence of this new polymorphic restriction site with a variable heteroplasmic rates at position +1517 of the human mitochondrial genome. On the other hand, direct sequencing of the entire mitochondrial 12S rRNA gene in the 100 patients and in 100 hearing individuals revealed the presence of the A750G and A1438G polymorphisms and the absence of the C1494T, T1095C and 961insC mutations in all the tested individuals. Sequencing of the whole mitochondrial genome in the 4 patients showing the new HaeIII polymorphic restriction site revealed only the presence of the A8860G transition in the MT-ATP6 gene and the A4769G polymorphism in the ND2 gene.

  20. A Novel C-Terminal CIB2 (Calcium and Integrin Binding Protein 2) Mutation Associated with Non-Syndromic Hearing Loss in a Hispanic Family.

    PubMed

    Patel, Kunjan; Giese, Arnaud P; Grossheim, J M; Hegde, Rashmi S; Hegde, Rashima S; Delio, Maria; Samanich, Joy; Riazuddin, Saima; Frolenkov, Gregory I; Cai, Jinlu; Ahmed, Zubair M; Morrow, Bernice E

    2015-01-01

    Hearing loss is a complex disorder caused by both genetic and environmental factors. Previously, mutations in CIB2 have been identified as a common cause of genetic hearing loss in Pakistani and Turkish populations. Here we report a novel (c.556C>T; p.(Arg186Trp)) transition mutation in the CIB2 gene identified through whole exome sequencing (WES) in a Caribbean Hispanic family with non-syndromic hearing loss. CIB2 belongs to the family of calcium-and integrin-binding (CIB) proteins. The carboxy-termini of CIB proteins are associated with calcium binding and intracellular signaling. The p.(Arg186Trp) mutation is localized within predicted type II PDZ binding ligand at the carboxy terminus. Our ex vivo studies revealed that the mutation did not alter the interactions of CIB2 with Whirlin, nor its targeting to the tips of hair cell stereocilia. However, we found that the mutation disrupts inhibition of ATP-induced Ca2+ responses by CIB2 in a heterologous expression system. Our findings support p.(Arg186Trp) mutation as a cause for hearing loss in this Hispanic family. In addition, it further highlights the necessity of the calcium binding property of CIB2 for normal hearing.

  1. Mutations of GJB2 encoding connexin 26 contribute to non-syndromic moderate and severe hearing loss in Pakistan.

    PubMed

    Salman, Midhat; Bashir, Rasheeda; Imtiaz, Ayesha; Maqsood, Azra; Mujtaba, Ghulam; Iqbal, Muddassar; Naz, Sadaf

    2015-08-01

    Mutations of GJB2 which encode connexin 26, contribute to 6-7 % of profound deafness in Pakistan. We investigated the involvement of GJB2 mutations in a cohort of 84 pedigrees and 86 sporadic individuals with moderate or severe hearing loss. Individuals in eight consanguineous families and four sporadic cases (9.52 and 4.65 %, respectively) were homozygous or compound heterozygous for p.W24X or p.W77X mutations in GJB2. These two variants are also among the most common mutations known to cause profound deafness in South Asia. The association of identical mutations with both profound and less severe phenotype of hearing loss suggests that alleles of other genes modify the phenotype due to these GJB2 nonsense mutations. Our study demonstrates that GJB2 mutations are an important contributor to aetiology of moderate to severe hearing loss in Pakistan.

  2. Mutation Screening of Exons 7 and 13 of the TMC1 Gene in Autosomal Recessive Non-syndromic Hearing Loss (ARNSHL) in Iran

    PubMed Central

    Moradipour, Negar; Ghasemi-Dehkordi, Payam; Heibati, Fatemeh; Parchami-Barjui, Shahrbanuo; Abolhasani, Marziyeh; Rashki, Ahmad; Hashemzadeh-Chaleshtori, Morteza

    2016-01-01

    Background: Non-syndromic hearing loss (NSHL) is the most common birth defect and occurs in approximately 1/1,000 newborns. NSHL is a heterogeneous trait and can arise due to both genetic and environmental factors. Mutations of the transmembrane channel-like 1 (TMC1) gene cause non-syndromic deafness in humans and mice. Objectives: The aim of the present study was to investigate the association of TMC1 gene mutations of the locus DFNB7/11 in exons 7 and 13 in a cohort of 100 patients with hearing loss in Iran using polymerase chain reaction–single-stranded conformation polymorphism (PCR-SSCP), heteroduplex analysis (HA), and DNA sequencing. Patients and Methods: In this experimental study, the blood samples of 100 NSHL patients were collected from 10 provinces in Iran. These patients had a mean age of 16.5 ± 2.01 years and 74.15% of their parents had consanguinity. DNA was extracted from specimens and mutations of exons 7 and 13 of the TMC1 gene were investigated using PCR-SSCP. All samples were checked via HA reaction and suspected specimens with shift bands were subjected to DNA sequencing for investigation of any gene variation. Results: In this study, no mutation was found in the two exons of TMC1 gene. It was concluded from these results that mutations of the TMC1 gene’s special exons 7 and 13 have a low contribution in patients and are not great of clinical importance in these Iranian provinces. Conclusions: More studies are needed to investigate the relationship between other parts of this gene with hearing loss in different populations through the country. More research could clarify the role of this gene and its relation with deafness and provide essential information for the prevention and management of auditory disorders caused by genetic factors in the Iranian population. PMID:27247785

  3. A common SLC26A4-linked haplotype underlying non-syndromic hearing loss with enlargement of the vestibular aqueduct.

    PubMed

    Chattaraj, Parna; Munjal, Tina; Honda, Keiji; Rendtorff, Nanna D; Ratay, Jessica S; Muskett, Julie A; Risso, Davide S; Roux, Isabelle; Gertz, E Michael; Schäffer, Alejandro A; Friedman, Thomas B; Morell, Robert J; Tranebjærg, Lisbeth; Griffith, Andrew J

    2017-10-01

    Enlargement of the vestibular aqueduct (EVA) is the most common radiological abnormality in children with sensorineural hearing loss. Mutations in coding regions and splice sites of the SLC26A4 gene are often detected in Caucasians with EVA. Approximately one-fourth of patients with EVA have two mutant alleles (M2), one-fourth have one mutant allele (M1) and one-half have no mutant alleles (M0). The M2 genotype is correlated with a more severe phenotype. We performed genotype-haplotype analysis and massively parallel sequencing of the SLC26A4 region in patients with M1 EVA and their families. We identified a shared novel haplotype, termed CEVA (Caucasian EVA), composed of 12 uncommon variants upstream of SLC26A4. The presence of the CEVA haplotype on seven of ten 'mutation-negative' chromosomes in a National Institutes of Health M1 EVA discovery cohort and six of six mutation-negative chromosomes in a Danish M1 EVA replication cohort is higher than the observed prevalence of 28 of 1006 Caucasian control chromosomes (p<0.0001 for each EVA cohort). The corresponding heterozygous carrier rate is 28/503 (5.6%). The prevalence of CEVA (11 of 126) is also increased among M0 EVA chromosomes (p=0.0042). The CEVA haplotype causally contributes to most cases of Caucasian M1 EVA and, possibly, some cases of M0 EVA. The CEVA haplotype of SLC26A4 defines the most common allele associated with hereditary hearing loss in Caucasians. The diagnostic yield and prognostic utility of sequence analysis of SLC26A4 exons and splice sites will be markedly increased by addition of testing for the CEVA haplotype. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  4. Genetic Linkage Analysis of DFNB3, DFNB9 and DFNB21 Loci in GJB2 Negative Families with Autosomal Recessive Non-syndromic Hearing Loss

    PubMed Central

    MASOUDI, Marjan; AHANGARI, Najmeh; POURSADEGH ZONOUZI, Ali Akbar; POURSADEGH ZONOUZI, Ahmad; NEJATIZADEH, Azim

    2016-01-01

    Background: Autosomal recessive non-syndromic hearing loss (ARNSHL) is the most common hereditary form of deafness, and exhibits a great deal of genetic heterogeneity. So far, more than seventy various DFNB loci have been mapped for ARNSHL by linkage analysis. The contribution of three common DFNB loci including DFNB3, DFNB9, DFNB21 and gap junction beta-2 (GJB2) gene mutations in ARNSHL was investigated in south of Iran for the first time. Methods: In this descriptive study, we investigated sixteen large families with at least two affected individuals. After DNA extraction, GJB2 gene mutations were analyzed using direct sequencing method. Negative samples for GJB2 gene mutations were analyzed for the linkage to DFNB3, DFNB9 and DFNB21 loci by genotyping the corresponding short tandem repeat (STR) markers using polymerase chain reaction (PCR) and polyacrylamide gel electrophoresis (PAGE) methods. Results: GJB2 mutations (283G>A and 29delT) were causes of hearing loss in 12.5% of families with ARNSHL and no evidence of linkage were found for any of DFNB3, DFNB9 and DFNB21 loci. Conclusion: GJB2 mutations are associated with ARNSHL. We failed to find linkage of the DFNB3, DFNB9 and DFNB21 loci among GJB2 negative families. Therefore, further studies on large-scale population and other loci will be needed to find conclusively linkage of DFNB loci and ARNSHL in the future. PMID:27398341

  5. 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.

  6. 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

  7. A novel missense variant in the nuclear localization signal of POU4F3 causes autosomal dominant non-syndromic hearing loss.

    PubMed

    Lin, Yin-Hung; Lin, Yi-Hsin; Lu, Ying-Chang; Liu, Tien-Chen; Chen, Chien-Yu; Hsu, Chuan-Jen; Chen, Pei-Lung; Wu, Chen-Chi

    2017-08-08

    Autosomal dominant non-syndromic hearing loss (ADNSHL) is genetically heterogeneous with more than 35 genes identified to date. Using a massively parallel sequencing panel targeting 159 deafness genes, we identified a novel missense variant of POU4F3 (c.982A>G, p.Lys328Glu) which co-segregated with the deafness phenotype in a three-generation Taiwanese family with ADNSHL. This variant could be classified as a "pathogenic variant" according to the American College of Medical Genetics and Genomics guidelines. We then performed subcellular localization experiments and confirmed that p.Lys328Glu compromised transportation of POU4F3 from the cytoplasm to the nucleus. POU3F4 p.Lys328Glu was located within a bipartite nuclear localization signal (NLS), and was the first missense variant in bipartite NLS of POU4F3 validated in functional studies. These findings expanded the mutation spectrum of POU4F3 and provided insight into the pathogenesis associated with aberrant POU4F3 localization.

  8. Hearing Loss: Hearing Augmentation.

    PubMed

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

    2015-07-01

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

  9. Screening of DFNB3 in Iranian families with autosomal recessive non-syndromic hearing loss reveals a novel pathogenic mutation in the MyTh4 domain of the MYO15A gene in a linked family

    PubMed Central

    Reiisi, Somayeh; Tabatabaiefar, Mohammad Amin; Sanati, Mohammad Hosein; Chaleshtori, Morteza Hashemzadeh

    2016-01-01

    Objective(s): Non-syndromic sensorineural hearing loss (NSHL) is a common disorder affecting approximately 1 in 500 newborns. This type of hearing loss is extremely heterogeneous and includes over 100 loci. Mutations in the GJB2 gene have been implicated in about half of autosomal recessive non-syndromic hearing loss (ARNSHL) cases, making this the most common cause of ARNSHL. For the latter form of deafness, most frequent genes proposed include GJB2, SLC26A4, MYO15A, OTOF, and CDH23 worldwide. Materials and Methods: The aim of the present study was to define the role and frequency of MYO15A gene mutation in Iranian families. In this study 30 Iranian families were enrolled with over three deaf children and negative for GJB2. Then linkage analysis was performed by six DFNB3 short tandem repeat markers. Following that, mutation detection accomplished using DNA sequencing. Results: One family (3.33%) showed linkage to DFNB3 and a novel mutation was identified in the MYO15A gene (c.6442T>A): as the disease-causing mutation. Mutation co-segregated with hearing loss in the family but was not present in the 100 ethnicity-matched controls. Conclusion: Our results confirmed that the hearing loss of the linked Iranian family was caused by a novel missense mutation in the MYO15A gene. This mutation is the first to be reported in the world and affects the first MyTH4 domain of the protein. PMID:27635202

  10. Hearing Loss

    MedlinePlus

    ... earphone in your baby’s ear. The earphone is connected to a computer. Your baby’s provider sends a ... treatment can help children with hearing loss develop speech, language and social skills. Without early treatment, hearing ...

  11. Aminoglycoside-induced and non-syndromic hearing loss is associated with the G7444A mutation in the mitochondrial COI/tRNA{sup Ser(UCN)} genes in two Chinese families

    SciTech Connect

    Zhu Yi; Liao Zhisu; Li Zhiyuan; Chen Jianfu; Qian Yaping; Tang Xiaowen; Wang Jindan; Yang Li; Li Ronghua; Ji Jinzhang; Choo, Daniel I. |; Lu Jianxin . E-mail: jx@mail.wz.zj.cn; Guan Minxin |||. E-mail: min-xin.guan@chmcc.org

    2006-04-14

    We report here the clinical, genetic, and molecular characterization of two Chinese families with aminoglycoside induced and non-syndromic hearing impairment. Clinical and genetic evaluations revealed the variable severity and age-of-onset in hearing impairment in these families. Strikingly, there were extremely low penetrances of hearing impairment in these Chinese families. Sequence analysis of the complete mitochondrial genomes in these pedigrees showed the distinct sets of mtDNA polymorphism, in addition to the identical G7444A mutation associated with hearing loss. Indeed, the G7444A mutation in the CO1 gene and the precursor of tRNA{sup Ser(UCN)} gene is present in homoplasmy only in the maternal lineage of those pedigrees but not other members of these families and 164 Chinese controls. Their mitochondrial genomes belong to the Eastern Asian haplogroups C5a and D4a, respectively. In fact, the occurrence of the G7444A mutation in these several genetically unrelated subjects affected by hearing impairment strongly indicates that this mutation is involved in the pathogenesis of hearing impairment. However, there was the absence of other functionally significant mtDNA mutations in two Chinese pedigrees carrying the G7444A mutation. Therefore, nuclear modifier gene(s) or aminoglycoside(s) may play a role in the phenotypic expression of the deafness-associated G7444A mutation in these Chinese pedigrees.

  12. Identification of a Novel Homozygous Mutation, TMPRSS3: c.535G>A, in a Tibetan Family with Autosomal Recessive Non-Syndromic Hearing Loss

    PubMed Central

    Fan, Dongyan; Zhu, Wei; Li, Dejun; Ji, De; Wang, Ping

    2014-01-01

    Different ethnic groups have distinct mutation spectrums associated with inheritable deafness. In order to identify the mutations responsible for congenital hearing loss in the Tibetan population, mutation screening for 98 deafness-related genes by microarray and massively parallel sequencing of captured target exons was conducted in one Tibetan family with familiar hearing loss. A homozygous mutation, TMPRSS3: c.535G>A, was identified in two affected brothers. Both parents are heterozygotes and an unaffected sister carries wild type alleles. The same mutation was not detected in 101 control Tibetan individuals. This missense mutation results in an amino acid change (p.Ala179Thr) at a highly conserved site in the scavenger receptor cysteine rich (SRCR) domain of the TMPRSS3 protein, which is essential for protein-protein interactions. Thus, this mutation likely affects the interactions of this transmembrane protein with extracellular molecules. According to our bioinformatic analyses, the TMPRSS3: c.535G>A mutation might damage protein function and lead to hearing loss. These data suggest that the homozygous mutation TMPRSS3: c.535G>A causes prelingual hearing loss in this Tibetan family. This is the first TMPRSS3 mutation found in the Chinese Tibetan population. PMID:25474651

  13. c.G2114A MYH9 mutation (DFNA17) causes non-syndromic autosomal dominant hearing loss in a Brazilian family

    PubMed Central

    Dantas, Vitor G.L.; Lezirovitz, Karina; Yamamoto, Guilherme L.; Moura de Souza, Carolina Fischinger; Ferreira, Simone Gomes; Mingroni-Netto, Regina C.

    2014-01-01

    We studied a family presenting 10 individuals affected by autosomal dominant deafness in all frequencies and three individuals affected by high frequency hearing loss. Genomic scanning using the 50k Affymetrix microarray technology yielded a Lod Score of 2.1 in chromosome 14 and a Lod Score of 1.9 in chromosome 22. Mapping refinement using microsatellites placed the chromosome 14 candidate region between markers D14S288 and D14S276 (8.85 cM) and the chromosome 22 near marker D22S283. Exome sequencing identified two candidate variants to explain hearing loss in chromosome 14 [PTGDR – c.G894A:p.R298R and PTGER2 – c.T247G:p.C83G], and one in chromosome 22 [MYH9, c.G2114A:p.R705H]. Pedigree segregation analysis allowed exclusion of the PTGDR and PTGER2 variants as the cause of deafness. However, the MYH9 variant segregated with the phenotype in all affected members, except the three individuals with different phenotype. This gene has been previously described as mutated in autosomal dominant hereditary hearing loss and corresponds to DFNA17. The mutation identified in our study is the same described in the prior report. Thus, although linkage studies suggested a candidate gene in chromosome 14, we concluded that the mutation in chromosome 22 better explains the hearing loss phenotype in the Brazilian family. PMID:25505834

  14. High prevalence of the W24X mutation in the gene encoding connexin-26 (GJB2) in Spanish Romani (gypsies) with autosomal recessive non-syndromic hearing loss.

    PubMed

    Alvarez, Araceli; del Castillo, Ignacio; Villamar, Manuela; Aguirre, Luis A; González-Neira, Anna; López-Nevot, Alicia; Moreno-Pelayo, Miguel A; Moreno, Felipe

    2005-09-01

    Molecular testing for mutations in the gene encoding connexin-26 (GJB2) at the DFNB1 locus has become the standard of care for genetic diagnosis and counseling of autosomal recessive non-syndromic hearing impairment (ARNSHI). The spectrum of mutations in GJB2 varies considerably among the populations, different alleles predominating in different ethnic groups. A cohort of 34 families of Spanish Romani (gypsies) with ARNSHI was screened for mutations in GJB2. We found that DFNB1 deafness accounts for 50% of all ARNSHI in Spanish gypsies. The predominating allele is W24X (79% of the DFNB1 alleles), and 35delG is the second most common allele (17%). An allele-specific PCR test was developed for the detection of the W24X mutation. By using this test, carrier frequencies were determined in two sample groups of gypsies from different Spanish regions (Andalusia and Catalonia), being 4% and 0%, respectively. Haplotype analysis for microsatellite markers closely flanking the GJB2 gene revealed five different haplotypes associated with the W24X mutation, all sharing the same allele from marker D13S141, suggesting that a founder effect for this mutation is responsible for its high prevalence among Spanish gypsies. (c) 2005 Wiley-Liss, Inc.

  15. Hearing Loss

    MedlinePlus

    ... topic was provided by the National Institute on Deafness and Other Communication Disorders Topic last reviewed: December ... a total loss of hearing. It can be hereditary or it can result from disease, trauma, certain ...

  16. Frequency of mitochondrial mutations in non-syndromic hearing loss as well as possibly responsible variants found by whole mitochondrial genome screening

    PubMed Central

    Yano, Takuya; Nishio, Shin-ya; Usami, Shin-ichi

    2014-01-01

    Mutations in mitochondrial DNA (mtDNA) are reported to be responsible for the pathogenesis of maternally inherited hearing loss. Complete mtDNA sequencing may detect pathogenic mutations, but whether they are indeed pathogenic can be difficult to interpret because of normal ethnic-associated haplogroup variation and other rare variations existing among control populations. In this study, we performed systemic mutational analysis of mtDNA in 394 Japanese patients with hearing loss. Two different cohorts were analyzed in this study: Cohort 1, 254 maternally inherited patients; and Cohort 2, 140 patients with various inheritance modes. After screening of the entire mtDNA genome with direct sequencing, we evaluated the frequency of previously reported mutations and the frequency and pathogenicity of the novel variants. As a result, the ‘Confirmed' mitochondrial mutations were found predominantly in Cohort 1 rather than in Cohort 2 (14.6 vs 0.7%). 1555A>G (n=23) is the most common mutation, followed by the 3243A>G (n=11) mutations. On the basis of prediction analysis, we detected 10 novel homoplasmic mitochondrial variants. After further classification, the 3595A>G and 6204A>G variants were found to be new candidate mutations possibly associated with hearing loss. PMID:24401907

  17. 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)

  18. 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)

  19. Hearing Loss

    MedlinePlus

    ... regular part of the working environment, such as farming, construction or factory work, can lead to damage ... Diagnosis and management. Primary Care: Clinics in Office Practice. 2014;41:19. Weber PC. Etiology of hearing ...

  20. Canine hearing loss management.

    PubMed

    Scheifele, Lesa; Clark, John Greer; Scheifele, Peter M

    2012-11-01

    Dog owners and handlers are naturally concerned when suspicion of hearing loss arises for their dogs. Questions frequently asked of the veterinarian center on warning signs of canine hearing loss and what can be done for the dog if hearing loss is confirmed. This article addresses warning signs of canine hearing loss, communication training and safety awareness issues, and the feasibility of hearing aid amplification for dogs.

  1. Hearing Loss

    MedlinePlus

    ... unit (NICU) Birth defects & other health conditions Loss & grief Tools & Resources Frequently asked health questions Ask our ... experts Calculating your due date Ovulation calendar Order bereavement materials News Moms Need Blog News & Media News ...

  2. Genetics of Hearing Loss

    MedlinePlus

    ... in Latin America Information For… Media Policy Makers Genetics of Hearing Loss Recommend on Facebook Tweet Share ... of hearing loss in babies is due to genetic causes. There are also a number of things ...

  3. Managing Hearing Loss

    MedlinePlus

    ... a total loss of hearing. It can be hereditary or it can result from disease, trauma, certain ... build-up, fluid, or a punctured eardrum. Medical treatment or surgery can usually restore conductive hearing loss. ...

  4. [Hearing loss in adults].

    PubMed

    Eshraghi, Adrien A; Frachet, Bruno; Van De Water, Tom R; Eter, Elias

    2009-05-20

    The management of hearing loss in adults depends of etiology and its severity. It can be as simple as treating an external otitis, removing an impacted cerumen or a more complex one such as a surgery for otosclerosis. The hearing loss is managed mainly by new advances in hearing aids technology and implantable hearing devices which include BAHA, middle ear implant and cochlear implants. The research is focused on developing new molecules for intracochlear drug therapy to treat noise induced hearing loss, drug ototoxicity as well as hearing loss related to cochlear implant insertion trauma. Antioxidant molecules, molecules against apoptosis are at this time the most promising molecules than need further investigations.

  5. 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.

  6. 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.

  7. Coexistence of mitochondrial 12S rRNA C1494T and CO1/tRNA{sup Ser(UCN)} G7444A mutations in two Han Chinese pedigrees with aminoglycoside-induced and non-syndromic hearing loss

    SciTech Connect

    Yuan Huijun; Chen Jing; Liu Xin; Cheng Jing; Wang Xinjian; Yang Li; Yang Shuzhi; Cao Juyang; Kang Dongyang; Dai Pu; Zha, Suoqiang; Han Dongyi Young Wieyen Guan Minxin

    2007-10-12

    Mutations in mitochondrial DNA are one of the important causes of hearing loss. We report here the clinical, genetic, and molecular characterization of two Han Chinese pedigrees with maternally transmitted aminoglycoside-induced and nonsyndromic bilateral hearing loss. Clinical evaluation revealed the wide range of severity, age-at-onset, and audiometric configuration of hearing impairment in matrilineal relatives in these families. The penetrances of hearing loss in these pedigrees were 20% and 18%, when aminoglycoside-induced deafness was included. When the effect of aminoglycosides was excluded, the penetrances of hearing loss in these seven pedigrees were 10% and 15%. Sequence analysis of the complete mitochondrial genomes in these pedigrees showed the presence of the deafness-associated 12S rRNA C1494T and CO1/tRNA{sup Ser(UCN)} G7444A mutations. Their distinct sets of mtDNA polymorphism belonged to Eastern Asian haplogroup C4a1, while other previously identified six Chinese mitochondrial genomes harboring the C1494T mutation belong to haplogroups D5a2, D, R, and F1, respectively. This suggested that the C1494T or G7444A mutation occurred sporadically and multiplied through evolution of the mitochondrial DNA (mtDNA). The absence of functionally significant mutations in tRNA and rRNAs or secondary LHON mutations in their mtDNA suggest that these mtDNA haplogroup-specific variants may not play an important role in the phenotypic expression of the 12S rRNA C1494T and CO1/tRNA{sup Ser(UCN)} G7444A mutations in those Chinese families. However, aminoglycosides and other nuclear modifier genes play a modifying role in the phenotypic manifestation of the C1494T mutation in these Chinese families.

  8. 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…

  9. Congenital hearing loss.

    PubMed

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

    2017-01-12

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

  10. [Inner Ear Hearing Loss].

    PubMed

    Hesse, G

    2016-06-01

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

  11. Hearing Loss and Cytomegalovirus.

    ERIC Educational Resources Information Center

    Strauss, Melvin

    1997-01-01

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

  12. Hereditary Hearing Loss.

    ERIC Educational Resources Information Center

    Tran, LenhAnh P.; Grundfast, Kenneth M.

    1997-01-01

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

  13. Hearing Loss and Cytomegalovirus.

    ERIC Educational Resources Information Center

    Strauss, Melvin

    1997-01-01

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

  14. Hearing loss and music

    MedlinePlus

    ... repeated exposure to loud noise and music can cause hearing loss. Decibels of Sound and Hearing Loss The decibel (dB) is a ... can make you unaware of the pain louder sounds can cause. Rest your ears for 24 hours after exposure ...

  15. Hereditary Hearing Loss.

    ERIC Educational Resources Information Center

    Tran, LenhAnh P.; Grundfast, Kenneth M.

    1997-01-01

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

  16. Sudden Sensorineural Hearing Loss

    PubMed Central

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

    2011-01-01

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

  17. 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,…

  18. The DFNB1 subtype of autosomal recessive non-syndromic hearing impairment.

    PubMed

    del Castillo, Francisco J; del Castillo, Ignacio

    2011-06-01

    Inherited hearing impairment is a frequent and highly heterogeneous condition. Among the different subtypes of autosomal recessive non-syndromic hearing impairment, DFNB1 is remarkable for its high frequency in most populations. It is caused by mutations in the coding region or splice-sites of the GJB2 gene, or by mutations affecting regulatory sequences that are essential for the expression of this gene. GJB2 encodes connexin-26, a protein component of intercellular gap junctions, which play crucial physiological roles in the cochlea. Because of its high frequency, DFNB1 hearing impairment has received continued attention from researchers along the years, resulting in a wealth of data that is unparalleled among these disorders. Here we review our current knowledge on the genetic, molecular, and phenotypic aspects of this subtype of hearing impairment.

  19. Living with Hearing Loss

    MedlinePlus

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

  20. Hearing loss - infants

    MedlinePlus

    ... may not startle when there is a loud noise nearby. Older infants, who should respond to familiar ... hearing loss, such as not reacting to loud noises, not making or mimicking noises, or not speaking ...

  1. What's Hearing Loss?

    MedlinePlus

    ... regular school, or be part of a regular classroom. Depending on how severe their hearing loss is, ... read along to follow the action. Technology is changing all the time, and you will probably see ...

  2. Individual Hearing Loss

    PubMed Central

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

    2016-01-01

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

  3. Congenital sensorineural hearing loss

    SciTech Connect

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

    1984-02-01

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

  4. Could Anemia Cause Hearing Loss?

    MedlinePlus

    ... fullstory_162793.html Could Anemia Cause Hearing Loss? Iron deficiency might keep ear cells from getting oxygen ... HealthDay News) -- Hearing loss may be linked to iron deficiency anemia -- a combination of low levels of ...

  5. Age-related hearing loss

    MedlinePlus

    ... grow older. Your genes and loud noise (from rock concerts or music headphones) may play a large role. The following factors contribute to age-related hearing loss: Family history (age-related hearing loss tends to run in ...

  6. 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.

  7. [Hereditary hearing loss: Part 2: Syndromic forms of hearing loss].

    PubMed

    Burke, W F; Lenarz, T; Maier, H

    2014-10-01

    Syndromic hearing loss is responsible for approximately 30% of cases of inherited hearing loss. The syndromic form can be differentiated from nonsyndromic hearing loss by the presence of associated symptoms in other organ systems. While for many forms of syndromic hearing loss the individual genes responsible have been identified, the etiology of other associated symptoms remains unclear. The role of the ENT physician is to select appropriate clinical and genetic diagnostic tools based on the presentation of the patient and to subsequently initiate and perform the required hearing loss therapy.

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

    PubMed Central

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

    2015-01-01

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

  9. Noise and Hearing Loss Prevention

    MedlinePlus

    ... on Facebook Tweet Share Compartir NIOSH Sound Level Meter App Buy Quiet Program Compendium of Hearing Protection ... Publications by Topic and Type Multimedia Sound Level Meter App Search NIOSH Publications Noise and Hearing Loss ...

  10. Noise-Induced Hearing Loss

    MedlinePlus

    ... Health Cycle Recommendations & Guidelines Free Materials Parent’s Guide Multimedia & ... Hearing plays an essential role in communication, speech and language development, and learning. Even a small amount of hearing loss can ...

  11. Drug Induced Hearing Loss: Researchers Study Strategies to Preserve Hearing

    MedlinePlus

    ... JavaScript on. Feature: Drug-Induced Hearing Loss Researchers Study Strategies to Preserve Hearing Past Issues / Spring 2016 Table ... Read More "Drug Induced Hearing Loss" Articles Researchers Study Strategies to Preserve Hearing / What Is Ototoxicity? Spring 2016 ...

  12. Pediatric Sudden Sensorineural Hearing Loss.

    PubMed

    Kizilay, Ahmet; Koca, Çiğdem Firat

    2016-06-01

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

  13. Hereditary hearing loss: from human mutation to mechanism.

    PubMed

    Lenz, Danielle R; Avraham, Karen B

    2011-11-01

    The genetic heterogeneity of hereditary hearing loss is thus far represented by hundreds of genes encoding a large variety of proteins. Mutations in these genes have been discovered for patients with different modes of inheritance and types of hearing loss, ranging from syndromic to non-syndromic and mild to profound. In many cases, the mechanisms whereby the mutations lead to hearing loss have been partly elucidated using cell culture systems and mouse and other animal models. The discovery of the genes has completely changed the practice of genetic counseling in this area, providing potential diagnosis in many cases that can be coupled with clinical phenotypes and offer predictive information for families. In this review we provide three examples of gene discovery in families with hereditary hearing loss, all associated with elucidation of some of the mechanisms leading to hair cell degeneration and pathology of deafness.

  14. Hereditary hearing loss with thyroid abnormalities.

    PubMed

    Choi, Byung Yoon; Muskett, Julie; King, Kelly A; Zalewski, Christopher K; Shawker, Thomas; Reynolds, James C; Butman, John A; Brewer, Carmen C; Stewart, Andrew K; Alper, Seth L; Griffith, Andrew J

    2011-01-01

    Mutations in SLC26A4 can cause deafness and goiter in Pendred syndrome (PDS) or isolated non-syndromic enlargement of the vestibular aqueduct (NSEVA). PDS is one of the most common hereditary causes of deafness. It is characterized by autosomal-recessive inheritance of sensorineural hearing loss, enlarged vestibular aqueducts (EVA), and an iodide organification defect with or without goiter. The diagnosis is confirmed by detection of two mutant alleles of SLC26A4 in a patient with EVA. The perchlorate discharge test can detect the underlying thyroid biochemical defect and is useful for the evaluation of goiter or for the clinical diagnosis of PDS in a patient with a non-diagnostic SLC26A4 genotype. SLC26A4 encodes the pendrin polypeptide, an anion exchanger that, in recombinant expression systems, transports chloride, bicarbonate, and iodide. Investigation of pendrin function in the inner ear has been facilitated by the Slc26a4(Δ) (knockout) mouse model, but the exact mechanism of its hearing loss remains unclear, as does pendrin's principal transport function in the inner ear. Treatment of PDS is focused upon rehabilitation of hearing loss, and surveillance and management of goiter and, less commonly, hypothyroidism.

  15. 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…

  16. Genes and Syndromic Hearing Loss.

    ERIC Educational Resources Information Center

    Keats, Bronya J. B.

    2002-01-01

    This article provides a description of the human genome and patterns of inheritance and discusses genes that are associated with some of the syndromes for which hearing loss is a common finding, including: Waardenburg, Stickler, Jervell and Lange-Neilsen, Usher, Alport, mitochondrial encephalomyopathy, and sensorineural hearing loss. (Contains…

  17. Genes and Syndromic Hearing Loss.

    ERIC Educational Resources Information Center

    Keats, Bronya J. B.

    2002-01-01

    This article provides a description of the human genome and patterns of inheritance and discusses genes that are associated with some of the syndromes for which hearing loss is a common finding, including: Waardenburg, Stickler, Jervell and Lange-Neilsen, Usher, Alport, mitochondrial encephalomyopathy, and sensorineural hearing loss. (Contains…

  18. 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…

  19. 10 Ways to Identify Hearing Loss

    MedlinePlus

    ... page please turn JavaScript on. Feature: Hearing Loss 10 Ways to Identify Hearing Loss Past Issues / Spring ... Research / Screening Newborns / How Loud Is Too Loud? / 10 Ways to Identify Hearing Loss Spring 2015 Issue: ...

  20. Vertigo and hearing loss.

    PubMed

    Newman-Toker, David E; Della Santina, Charles C; Blitz, Ari M

    2016-01-01

    Symptoms referable to disorders affecting the inner ear and vestibulocochlear nerve (eighth cranial nerve) include dizziness, vertigo, tinnitus, and hearing loss, in various combinations. Similar symptoms may occur with involvement of the central nervous system, principally the brainstem and cerebellum, to which the vestibular and auditory systems are connected. Imaging choices should be tailored to patient symptoms and the clinical context. Computed tomography (CT) should be used primarily to assess bony structures. Magnetic resonance imaging (MRI) should be used primarily to assess soft-tissue structures. Vascular imaging by angiography or venography should be obtained when vascular lesions are suspected. No imaging should be obtained in patients with typical presentations of common peripheral vestibular or auditory disorders. In current clinical practice, neuroimaging is often overused, especially CT in the assessment of acute dizziness and vertigo in the emergency department. Despite low sensitivity for ischemic strokes, CT is often used to rule out neurologic causes. When ischemic stroke is the principal concern in acute vestibular presentations, imaging should almost always be by MRI with diffusion-weighted images, rather than CT. In this chapter, we describe recommended strategies for audiovestibular imaging based on patient symptoms and signs.

  1. Noise-induced hearing loss

    SciTech Connect

    Catlin, F.I.

    1986-03-01

    Hearing loss affects 30 million people in the United States; of these, 21 million are over the age of 65 years. This disorder may have several causes: heredity, noise, aging, and disease. Hearing loss from noise has been recognized for centuries but was generally ignored until some time after the Industrial Revolution. Hearing loss from occupational exposure to hazardous noise was identified as a compensable disability by the United States courts in 1948 to 1959. Development of noisy jet engines and supersonic aircraft created additional claims for personal and property damage in the 1950s and 1960s. These conditions led to legislation for noise control in the form of the Occupational Safety and Health Act of 1970 and the Noise Control Act of 1972. Protection of the noise-exposed employee was also an objective of the Hearing Conservation Act of 1971. Subsequent studies have confirmed the benefits of periodic hearing tests for workers exposed to hazardous noise and of otologic evaluation as part of the hearing conservation process. Research studies in laboratory animals, using scanning electron microscopical techniques, have demonstrated that damage to the inner ear and organ of hearing can occur even though subjective (conditioned) response to sound stimuli remains unaffected. Some investigators have employed an epidemiologic approach to identify risk factors and to develop profiles to susceptibility to noise-induced hearing loss. The need for joint involvement of workers and employers in the reduction and control of occupational noise hazards is evident. 19 references.

  2. Noise-induced hearing loss.

    PubMed

    Catlin, F I

    1986-03-01

    Hearing loss affects 30 million people in the United States; of these, 21 million are over the age of 65 years. This disorder may have several causes: heredity, noise, aging, and disease. Hearing loss from noise has been recognized for centuries but was generally ignored until some time after the Industrial Revolution. Hearing loss from occupational exposure to hazardous noise was identified as a compensable disability by the United States courts in 1948 to 1959. Development of noisy jet engines and supersonic aircraft created additional claims for personal and property damage in the 1950s and 1960s. These conditions led to legislation for noise control in the form of the Occupational Safety and Health Act of 1970 and the Noise Control Act of 1972. Protection of the noise-exposed employee was also an objective of the Hearing Conservation Act of 1971. Subsequent studies have confirmed the benefits of periodic hearing tests for workers exposed to hazardous noise and of otologic evaluation as part of the hearing conservation process. Research studies in laboratory animals, using scanning electron microscopical techniques, have demonstrated that damage to the inner ear and organ of hearing can occur even though subjective (conditioned) response to sound stimuli remains unaffected. Some investigators have employed an epidemiologic approach to identify risk factors and to develop profiles to susceptibility to noise-induced hearing loss. The need for joint involvement of workers and employers in the reduction and control of occupational noise hazards is evident.

  3. 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…

  4. 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…

  5. Occupational hearing loss in Korea.

    PubMed

    Kim, Kyoo Sang

    2010-12-01

    In this article, current status of noise exposure in workplaces, trend of workers with noise-induced hearing loss (NIHL), and prevalence of NIHL in workers by industry and job category in Korea were reviewed. In addition, trends of research on the audiological effects such as hearing loss from noise and occupational hearing loss from non-noise in Korea were addressed through reports in industrial audiology. Though noise exposure level has improved, noise still shows the highest rate of cases exceeding exposure limit among workplace hazards. NIHL is the most common occupational disease except work-related disease such as musculoskeletal disorders and cerebrovascular diseases, and NIHL prevalence is thought to be much higher than reported in official publications. Noise affecting hearing comes from various sources such as workplaces, military settings, areas with exposure to high noise, and specific noise sources. There is also occupational hearing loss by non-noise including chemicals such as organic solvents and heavy metals, barotrauma, and trauma due to welding spark. Noise affects daily life through audiological effects such as hearing loss and tinnitus, non-audiological physical effects (e.g., cardiovascular), and psychosocial and behavioral effects. Development of systematic and comprehensive hearing conservation programs for lowering the noise level in workplaces and preventing the NIHL, and preparation of technological, administrative system for its settlement at workplace are urgently needed.

  6. Occupational Hearing Loss in Korea

    PubMed Central

    2010-01-01

    In this article, current status of noise exposure in workplaces, trend of workers with noise-induced hearing loss (NIHL), and prevalence of NIHL in workers by industry and job category in Korea were reviewed. In addition, trends of research on the audiological effects such as hearing loss from noise and occupational hearing loss from non-noise in Korea were addressed through reports in industrial audiology. Though noise exposure level has improved, noise still shows the highest rate of cases exceeding exposure limit among workplace hazards. NIHL is the most common occupational disease except work-related disease such as musculoskeletal disorders and cerebrovascular diseases, and NIHL prevalence is thought to be much higher than reported in official publications. Noise affecting hearing comes from various sources such as workplaces, military settings, areas with exposure to high noise, and specific noise sources. There is also occupational hearing loss by non-noise including chemicals such as organic solvents and heavy metals, barotrauma, and trauma due to welding spark. Noise affects daily life through audiological effects such as hearing loss and tinnitus, non-audiological physical effects (e.g., cardiovascular), and psychosocial and behavioral effects. Development of systematic and comprehensive hearing conservation programs for lowering the noise level in workplaces and preventing the NIHL, and preparation of technological, administrative system for its settlement at workplace are urgently needed. PMID:21258593

  7. Hearing Loss in Palliative Care

    PubMed Central

    Jain, Nelia; Wallhagen, Margaret L.

    2015-01-01

    Abstract Background: Age-related hearing loss is remarkably common, affecting more than 60% of adults over the age of 75. Moreover, hearing loss has detrimental effects on quality of life and communication, outcomes that are central to palliative care. Despite its high prevalence, there is remarkably little written on the impact of hearing loss in the palliative care literature. Objective: The objective was to emphasize its importance and the need for further study. We use a case as a springboard for discussing what is known and unknown about the epidemiology, presentation, screening methodologies, and treatment strategies for age-related hearing loss in palliative care. Discussion: The case describes a 65-year-old man with acute myelogenous leukemia (AML) that has progressed despite treatment. No concerns are raised about communication challenges during conversations between the palliative care team and the patient in his quiet room. However, in the midst of a family meeting, shortly after discussing prognosis, the patient reports that he cannot hear what anyone is saying. Conclusion: We describe simple methods of screening patients for hearing loss, and suggest that practical approaches should be used universally in patient encounters. These include facing the patient, pitching one's voice low, using a pocket talker, and creating a hearing-friendly environment when planning a family or group meeting. PMID:25867966

  8. Sudden hearing loss in children.

    PubMed

    Ječmenica, Jovana; Bajec-Opančina, Aleksandra

    2014-08-01

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

  9. Occupational hearing loss

    MedlinePlus

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

  10. Non-organic hearing loss.

    PubMed

    Nelson, Sarah C

    2012-12-01

    Annual hearing tests are compulsory in the British Armed Forces. This case report presents a 24-year old soldier who was found to have severe deterioration on her annual audiogram without any significant noise exposure. After two years of specialist audiological investigations she was diagnosed with non-organic hearing loss; further interrogation of her social circumstances suggested potential psychosocial triggers. This diagnosis should be considered early in military primary care in those with objective hearing loss on audiogram where there has been no exposure to significant noise.

  11. [Congenital hearing loss in children. 2: Genetic hearing loss].

    PubMed

    Gross, M; Lange, K; Spormann-Lagodzinski, M

    2001-08-01

    Molecular genetic tests enable a differentiated view of patho-physiological correlation of sensori-neural hearing impairment. On the basis of 4791 records of the German Central Registry on Childhood Hearing Loss (Deutsches Zentralregister für kindliche Hörstörungen (DZH)) it was shown that 35% of all permanent hearing impairment in Germany is caused genetically and 20% are acquired. The proportion of indistinct etiology remains at 45%. The number of children who have a hearing impairment by birth is also vague. It has to be expected that approximately 5% of these children develop a hearing impairment afterwards albeit its genetic determination. In 4791 cases a syndromal phenotype was assumed in 8.9%. Practical advice is given for clinical routine diagnostics with pragmatic suggestions and some distinct syndromes are described. Hitherto identified deafness genes reveal their role in pathological auditory function. Future diagnostic and therapeutic strategies are outlined on the basis of molecular genetic methods.

  12. Use of Hearing Aids by Adults with Hearing Loss

    MedlinePlus

    ... Health Info » Statistics and Epidemiology Use of Hearing Aids by Adults with Hearing Loss [text version] Note: ... displays time trends in the use of hearing aids for adults (20–69 years) and older adults ( ...

  13. [Hearing loss and Alzheimer's disease].

    PubMed

    Bakhos, David; Villeuneuve, Alexandre; Kim, Soo; Hammoudi, Karim; Hommet, Caroline

    2015-06-01

    Recent studies suggest that subjects with hearing loss are more likely to develop Alzheimer's disease. Hearing loss can be consecutive to presbycusis and/or to central auditory dysfunction. Standard audiometric measures (pure tone and speech intelligibility) allow the diagnosis of presbycusis. However, to demonstrate central auditory dysfunction, specific audiometric tests are needed such as noisy and/or dichotic tests. Actually, no consensus exists to investigate hearing loss in people with Alzheimer's disease though hearing loss may be an early manifestation of Alzheimer's disease. Until now, investigations and clinical procedure related to the diagnosis of Alzheimer's disease ignored the hearing ability of the patient. However, the major part of care management and investigations implies the patient's communication ability with the caregivers. Hearing loss may be one of the most unrecognized deficit in subjects with Alzheimer's disease. Auditory rehabilitation could benefit to the patient in order to lessen cognitive decline, but this must be investigated during longitudinal studies in order to clearly demonstrate their efficiency.

  14. Sudden Hearing Loss.

    ERIC Educational Resources Information Center

    Vaughan, John C.

    1997-01-01

    Patients with a sudden dramatic decline in hearing usually require rapid diagnosis and treatment. Unfortunately, the treatment of this condition continues to be controversial and an exact etiology in most cases has been inconclusive. Nevertheless, physicians have reached a consensus regarding several broad principles, which are presented in this…

  15. Genes and Hearing Loss

    MedlinePlus

    ... a rare allele of a gene by a single heterozygous parent is sufficient to generate an affected child. A heterozygous parent has two types of the ... help them determine their risk of having a child with hearing problems. Patient Health Home Copyright © 2017 ... Development Practice Management ENT Careers Marketplace Privacy Policy Terms ...

  16. Sudden Hearing Loss.

    ERIC Educational Resources Information Center

    Vaughan, John C.

    1997-01-01

    Patients with a sudden dramatic decline in hearing usually require rapid diagnosis and treatment. Unfortunately, the treatment of this condition continues to be controversial and an exact etiology in most cases has been inconclusive. Nevertheless, physicians have reached a consensus regarding several broad principles, which are presented in this…

  17. By the Numbers: Hearing Loss Affects Millions

    MedlinePlus

    ... loss affects millions Follow us By the Numbers: Hearing Loss Affects Millions Approximately 15 percent of American adults ( ... to 74). Below are some interesting facts about hearing loss provided by the National Institute on Deafness and ...

  18. Genetic Counseling for Hearing Loss.

    ERIC Educational Resources Information Center

    Arnos, Kathleen S.

    1997-01-01

    This article addresses epidemiologic and demographic characteristics of hereditary hearing loss and genetic evaluation and counseling. It discusses who should have genetic counseling, the genetic counseling process, and effects of genetics technology on the genetic counseling process. A case study of a 2-year-old with severe sensorineural hearing…

  19. 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.

  20. 10 Ways to Identify Hearing Loss

    MedlinePlus

    Skip Navigation Bar Home Current Issue Past Issues 10 Ways to Identify Hearing Loss Past Issues / Spring ... due to excessive noise exposure and other causes. 10 Ways to Identify Hearing Loss Take the following ...

  1. Talking to someone with hearing loss

    MedlinePlus

    ... ency/patientinstructions/000361.htm Talking to someone with hearing loss To use the sharing features on this page, ... It may be hard for a person with hearing loss to understand a conversation with another person. Being ...

  2. Genetics Home Reference: nonsyndromic hearing loss

    MedlinePlus

    ... age. Hearing loss that is present before a child learns to speak is classified as prelingual or congenital. Hearing loss that occurs ... or Free article on PubMed Central GeneReview: Deafness and Hereditary ...

  3. Radiation Therapy and Hearing Loss

    SciTech Connect

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

    2010-03-01

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

  4. Psychosocial Aspects of Hearing Loss in Children.

    PubMed

    Sorkin, Donna L; Gates-Ulanet, Patricia; Mellon, Nancy K

    2015-12-01

    Pediatric hearing loss changed more in the past two decades than it had in the prior 100 years with children now identified in the first weeks of life and fit early with amplification. Dramatic improvements in hearing technology allow children the opportunity to listen, speak and read on par with typically hearing peers. National laws mandate that public and private schools, workplaces, and anywhere people go must be accessible to individuals with disabilities. In 2015, most children with hearing loss attended mainstream schools with typically hearing peers. Psychosocial skills still present challenges for some children with hearing loss.

  5. 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,…

  6. 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,…

  7. Spectrum of genetic changes in patients with non-syndromic hearing impairment and extremely high carrier frequency of 35delG GJB2 mutation in Belarus.

    PubMed

    Danilenko, Nina; Merkulava, Elena; Siniauskaya, Marina; Olejnik, Olga; Levaya-Smaliak, Anastasia; Kushniarevich, Alena; Shymkevich, Andrey; Davydenko, Oleg

    2012-01-01

    The genetic nature of sensorineural hearing loss (SNHL) has so far been studied for many ethnic groups in various parts of the world. The single-nucleotide guanine deletion (35delG) of the GJB2 gene coding for connexin 26 was shown to be the main genetic cause of autosomal recessive deafness among Europeans. Here we present the results of the first study of GJB2 and three mitochondrial mutations among two groups of Belarusian inhabitants: native people with normal hearing (757 persons) and 391 young patients with non-syndromic SNHL. We have found an extremely high carrier frequency of 35delG GJB2 mutation in Belarus -5.7%. This point deletion has also been detected in 53% of the patients with SNHL. The 312del14 GJB2 was the second most common mutation in the Belarus patient cohort. Mitochondrial A1555G mt-RNR1 substitution was found in two SNHL patients (0.55%) but none were found in the population cohort. No individuals carried the A7445G mutation of mitochondrial mt-TS1. G7444A as well as T961G substitutions were detected in mitochondrial mt-RNR1 at a rate of about 1% both in the patient and population cohorts. A possible reason for Belarusians having the highest mutation carrier frequency in Europe 35delG is discussed.

  8. Hearing Loss Signals Need for Diagnosis

    MedlinePlus

    ... Products For Consumers Home For Consumers Consumer Updates Hearing Loss Signals Need for Diagnosis Share Tweet Linkedin ... you’re talking loudly? Thinking about ordering a hearing aid or sound amplifier from a magazine or ...

  9. Sensorineural hearing loss in hemorrhagic dengue?

    PubMed Central

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

    2014-01-01

    Introduction 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. Presentation of case 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. Discussion and conclusion 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? PMID:25625489

  10. Sudden bilateral hearing loss after spinal anaesthesia.

    PubMed

    Sahin, C; Terzioglu, U; Yigit, G

    2015-04-01

    Spinal anaesthesia is one of the most widely used regional anaesthesia techniques. Sudden bilateral hearing loss following spinal anaesthesia has only been reported in a few cases. This paper reports the case of a 50-year-old woman who developed sudden bilateral hearing loss following spinal anaesthesia for hallux valgus orthopaedic surgery. This is followed by a literature review. The patient's hearing improved almost completely on the morning of the 3rd day following surgery. No recurrence of hearing loss, tinnitus or vertigo was reported during the six-month follow-up period. Some complications regarding hearing may emerge after spinal anaesthesia. The possibility of hearing loss after spinal anaesthesia should be taken into consideration. Complaints such as hearing loss, tinnitus or vertigo should be taken seriously when reported, and the patient should be referred to an ENT clinic. This will ensure early diagnosis and treatment.

  11. Hearing: Noise-Induced Hearing Loss

    MedlinePlus

    ... a cochlear implant. A cochlear implant is an electronic hearing device that replaces the damaged inner ear ... by listening to parents, teachers, television, and radio. Music, the sounds of nature, and the voices of ...

  12. Sudden bilateral hearing loss after organophosphate inhalation.

    PubMed

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

    2016-12-01

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

  13. De novo mutation in X-linked hearing loss-associated POU3F4 in a sporadic case of congenital hearing loss

    PubMed Central

    Moteki, Hideaki; Shearer, A Eliot; Izumi, Shuji; Kubota, Yamato; Azaiez, Hela; Booth, Kevin T; Sloan, Christina M; Kolbe, Diana L; Smith, Richard JH; Usami, Shin-ichi

    2015-01-01

    Objective In this report, we present a male patient with no family history of hearing loss, in whom we identified a novel de novo mutation in the POU3F4 gene. Methods One hundred ninety-four (194) Japanese subjects from unrelated and families were enrolled in this study. We used targeted genomic enrichment and massively parallel sequencing of all known non-syndromic hearing loss genes for identifying the genetic causes of hearing loss. Results A novel de novo frameshift mutation of POU3F4, to c.727_728insA (p.N244KfsX26) was identified. The patient was a 7-year-old male with congenital progressive hearing loss and inner ear deformity. Although the patient had received a cochlear implant, auditory skills were still limited. The patient also exhibited developmental delays similar to those previously associated with POU3F4 mutation. Conclusion This is the first report of a mutation in POU3F4 causing hearing loss in a Japanese patient without a family history of hearing loss. This study underscores the importance of comprehensive genetic testing of patients with hearing loss for providing accurate prognostic information and guiding the optimal management of patient rehabilitation. PMID:25792666

  14. Reading Recovery for Children with Hearing Loss

    ERIC Educational Resources Information Center

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

    2006-01-01

    This study investigated the application of Reading Recovery for children with hearing loss by measuring and comparing the literacy achievement of 12 children who are deaf or hard of hearing and 12 children with typical hearing who were participating in Reading Recovery interventions in the second year of primary school. Progress was measured by…

  15. 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…

  16. 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…

  17. Idiopathic Sudden Sensorineural Hearing Loss With Minimal Hearing Impairment

    PubMed Central

    Cho, Chin Saeng

    2015-01-01

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

  18. Clinical aspects of hereditary hearing loss.

    PubMed

    Kochhar, Amit; Hildebrand, Michael S; Smith, Richard J H

    2007-07-01

    Hearing loss is an etiologically diverse condition with many disease-related complications and major clinical, social, and quality of life implications. As the rate of acquired hearing loss secondary to environmental causes decreases and improvements in the diagnosis of abnormalities occur, the significance of genetic factors that lead to deafness increases. Advancements in molecular biology have led to improved detection and earlier intervention in patients with hearing loss. Subsequently, earlier implementation of educational services and cochlear implant technology in patients with profound hearing loss now results in superior communication skills and enhanced language development. The aim of this review is to provide a comprehensive framework underlying the causes of hearing impairment and to detail the clinical management for patients with hereditary hearing loss.

  19. 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.

  20. Hearing aids for mild to moderate hearing loss in adults.

    PubMed

    Ferguson, Melanie A; Kitterick, Pádraig T; Chong, Lee Yee; Edmondson-Jones, Mark; Barker, Fiona; Hoare, Derek J

    2017-09-25

    The main clinical intervention for mild to moderate hearing loss is the provision of hearing aids. These are routinely offered and fitted to those who seek help for hearing difficulties. By amplifying and improving access to sounds, and speech sounds in particular, the aim of hearing aid use is to reduce the negative consequences of hearing loss and improve participation in everyday life. To evaluate the effects of hearing aids for mild to moderate hearing loss in adults. The Cochrane ENT Information Specialist searched the ENT Trials Register; the Cochrane Register of Studies Online; MEDLINE; PubMed; EMBASE; CINAHL; Web of Science; ClinicalTrials.gov; ICTRP and additional sources for published and unpublished trials. The date of the search was 23 March 2017. Randomised controlled trials (RCTs) of hearing aids compared to a passive or active control in adults with mild to moderate hearing loss. We used the standard methodological procedures expected by Cochrane. The primary outcomes in this review were hearing-specific health-related quality of life and the adverse effect pain. Secondary outcomes were health-related quality of life, listening ability and the adverse effect noise-induced hearing loss. We used GRADE to assess the quality of the evidence for each outcome; this is indicated in italics. We included five RCTs involving 825 participants. The studies were carried out in the USA and Europe, and were published between 1987 and 2017. Risk of bias across the studies varied. Most had low risk for selection, reporting and attrition bias, and a high risk for performance and detection bias because blinding was inadequate or absent.All participants had mild to moderate hearing loss. The average age across all five studies was between 69 and 83 years. The duration of the studies ranged between six weeks and six months.There was a large beneficial effect of hearing aids on hearing-specific health-related quality of life associated with participation in daily life as

  1. Non-syndromic autosomal-dominant deafness.

    PubMed

    Petersen, M B

    2002-07-01

    Non-syndromic deafness is a paradigm of genetic heterogeneity. More than 70 loci have been mapped, and 25 of the nuclear genes responsible for non-syndromic deafness have been identified. Autosomal-dominant genes are responsible for about 20% of the cases of hereditary non-syndromic deafness, with 16 different genes identified to date. In the present article we review these 16 genes, their function and their contribution to deafness in different populations. The complexity is underlined by the fact that several of the genes are involved in both dominant and recessive non-syndromic deafness or in both non-syndromic and syndromic deafness. Mutations in eight of the genes have so far been detected in only single dominant deafness families, and their contribution to deafness on a population base might therefore be limited, or is currently unknown. Identification of all genes involved in hereditary hearing loss will help in the understanding of the basic mechanisms underlying normal hearing, will facilitate early diagnosis and intervention and might offer opportunities for rational therapy.

  2. Vestibular function in families with inherited autosomal dominant hearing loss.

    PubMed

    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.

  3. 20 CFR 702.441 - Claims for loss of hearing.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 20 Employees' Benefits 3 2010-04-01 2010-04-01 false Claims for loss of hearing. 702.441 Section... and Supervision Hearing Loss Claims § 702.441 Claims for loss of hearing. (a) Claims for hearing loss... regulations. (b) An audiogram shall be presumptive evidence of the amount of hearing loss on the date...

  4. Cleidocranial dysplasia with hearing loss

    PubMed Central

    Candamourty, Ramesh; Venkatachalam, Suresh; Yuvaraj, Vaithilingam; Kumar, Ganesan Suresh

    2013-01-01

    Cleidocranial dysplasia is an inherited skeletal anomaly that affects primarily the skull, clavicle, and dentition, which can occur spontaneously, but most are inherited in autosomal dominant mode. The skull findings are brachycephaly, delayed or failed closure of the fontanelles, presence of open skull sutures and multiple wormian bones with pronounced frontal bossing. The syndrome is notable for aplasia or hypoplasia of the clavicles. The neck appears long and narrow and the shoulders markedly droop. Oral manifestations exhibit a hypoplastic maxilla with high-arched palate. Crowding of teeth is produced by retention of deciduous teeth, delayed eruption of permanent teeth, and the presence of a large number of unerupted supernumerary teeth. We report a case of CCD in a 12-year-old girl who presented with an unaesthetic facial appearance, unerupted permanent dentition with hearing loss. PMID:23633875

  5. Hearing Loss in Children: Data and Statistics

    MedlinePlus

    ... epilepsy, or vision loss) were employed. [ Read summary ] Economic Cost During the 1999 - 2000 school year, the ... and family out-of-pocket expenses. The actual economic costs of hearing loss, therefore, will be even ...

  6. Hearing loss and dementia: new insights.

    PubMed

    Albers, Kristi

    2012-01-01

    The aging brain is a hot topic among researchers and health professionals worldwide. Although our knowledge of the way the brain changes with age is in its infancy, research into hearing loss and dementia has gained momentum. Recent studies suggest that persons with hearing loss are more likely to develop Alzheimer's disease or other forms of dementia over time. This article discusses recent findings.

  7. 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…

  8. 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…

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

    PubMed

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

    1997-03-01

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

  10. A gene for autosomal dominant hearing loss on the short arm of chromosome 1

    SciTech Connect

    Van Camp, G.; Coucke, P.; Willems, P.J.

    1994-09-01

    Hearing loss is the most common form of sensory impairment and many cases are attributable to genetic causes. The genetic defects underlying several syndromic forms of deafness have been identified, but little is known about the causes of non-syndromic hereditary deafness which accounts for the majority of inherited hearing loss. We report here a large Indonesian family with non-syndromal postlingual hearing loss starting in the high frequencies and showing autosomal dominant inheritance. To locate the gene responsible for the hearing loss in this family, we performed a genome search by genetic linkage analysis with microsatellite markers distributed over the whole genome. We have mapped the gene causing deafness in an extended Indonesian family to chromosome 1p with a multipoint lod score higher than 7. Two other smaller families, showing a similar hereditary hearing loss, were also tested for linkage with chromosome 1p. One family originating from the U.S. was linked to this new locus with a multipoint lod score exceeding 5. In another family from the Netherlands this locus was excluded. The flanking markers D1S255 and D1S211 define a region of 6 cM on chromosome 1p which is likely to contain the deafness gene present in the Indonesian and American family.

  11. Heterogeneity of Hereditary Hearing Loss in Iran: a Comprehensive Review.

    PubMed

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

    2016-10-01

    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.

  12. 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

  13. Restaurant noise, hearing loss, and hearing aids.

    PubMed Central

    Lebo, C P; Smith, M F; Mosher, E R; Jelonek, S J; Schwind, D R; Decker, K E; Krusemark, H J; Kurz, P L

    1994-01-01

    Our multidisciplinary team obtained noise data in 27 San Francisco Bay Area restaurants. These data included typical minimum, peak, and average sound pressure levels; digital tape recordings; subjective noise ratings; and on-site unaided and aided speech discrimination tests. We report the details and implications of these noise measurements and provide basic information on selecting hearing aids and suggestions for coping with restaurant noise. Images PMID:7941506

  14. Restaurant noise, hearing loss, and hearing aids.

    PubMed

    Lebo, C P; Smith, M F; Mosher, E R; Jelonek, S J; Schwind, D R; Decker, K E; Krusemark, H J; Kurz, P L

    1994-07-01

    Our multidisciplinary team obtained noise data in 27 San Francisco Bay Area restaurants. These data included typical minimum, peak, and average sound pressure levels; digital tape recordings; subjective noise ratings; and on-site unaided and aided speech discrimination tests. We report the details and implications of these noise measurements and provide basic information on selecting hearing aids and suggestions for coping with restaurant noise.

  15. Imaging of post-traumatic hearing loss.

    PubMed

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

    2017-09-21

    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.

  16. Non-syndromic, autosomal-recessive deafness.

    PubMed

    Petersen, M B; Willems, P J

    2006-05-01

    Non-syndromic deafness is a paradigm of genetic heterogeneity with 85 loci and 39 nuclear disease genes reported so far. Autosomal-recessive genes are responsible for about 80% of the cases of hereditary non-syndromic deafness of pre-lingual onset with 23 different genes identified to date. In the present article, we review these 23 genes, their function, and their contribution to genetic deafness in different populations. The wide range of functions of these DFNB genes reflects the heterogeneity of the genes involved in hearing and hearing loss. Several of these genes are involved in both recessive and dominant deafness, or in both non-syndromic and syndromic deafness. Mutations in the GJB2 gene encoding connexin 26 are responsible for as much as 50% of pre-lingual, recessive deafness. By contrast, mutations in most of the other DFNB genes have so far been detected in only a small number of families, and their contribution to deafness on a population scale might therefore be limited. Identification of all genes involved in hereditary hearing loss will help in our understanding of the basic mechanisms underlying normal hearing, in early diagnosis and therapy.

  17. Noise and Hearing Loss Prevention: Facts and Statistics

    MedlinePlus

    ... hearing loss were reported among workers in the manufacturing sector. Fact Sheets Occupationally-Induced Hearing Loss DHHS ( ... 2010-136 Statistics on hearing loss in the manufacturing industry. Surveys National Health and Nutrition Examination Survey ( ...

  18. 20 CFR 702.441 - Claims for loss of hearing.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 20 Employees' Benefits 4 2013-04-01 2013-04-01 false Claims for loss of hearing. 702.441 Section... Care and Supervision Hearing Loss Claims § 702.441 Claims for loss of hearing. (a) Claims for hearing... regulations. (b) An audiogram shall be presumptive evidence of the amount of hearing loss on the date...

  19. 20 CFR 702.441 - Claims for loss of hearing.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 20 Employees' Benefits 4 2014-04-01 2014-04-01 false Claims for loss of hearing. 702.441 Section... Care and Supervision Hearing Loss Claims § 702.441 Claims for loss of hearing. (a) Claims for hearing... regulations. (b) An audiogram shall be presumptive evidence of the amount of hearing loss on the date...

  20. 20 CFR 702.441 - Claims for loss of hearing.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 20 Employees' Benefits 4 2012-04-01 2012-04-01 false Claims for loss of hearing. 702.441 Section... Care and Supervision Hearing Loss Claims § 702.441 Claims for loss of hearing. (a) Claims for hearing... regulations. (b) An audiogram shall be presumptive evidence of the amount of hearing loss on the date...

  1. 20 CFR 702.441 - Claims for loss of hearing.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 20 Employees' Benefits 3 2011-04-01 2011-04-01 false Claims for loss of hearing. 702.441 Section... Care and Supervision Hearing Loss Claims § 702.441 Claims for loss of hearing. (a) Claims for hearing... regulations. (b) An audiogram shall be presumptive evidence of the amount of hearing loss on the date...

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

    PubMed

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

    2000-09-01

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

  3. Sudden hearing loss associated with methylphenidate therapy.

    PubMed

    Karapinar, Ugur; Saglam, Omer; Dursun, Engin; Cetin, Bilal; Salman, Nergis; Sahan, Murat

    2014-01-01

    An 8-year-old child diagnosed with attention deficit/hyperactivity disorder presented to our Department of Otolaryngology 4 days after suffering hearing loss, loss of balance, tinnitus, and fullness sensation of the left ear. Her symptoms occured with the first dose of methylphenidate. The medical history and physical examination revealed no other diseases associated with sudden hearing loss. The audiogram revealed a total hearing loss on the left ear. Stapedial reflexes, distortion product and transient-evoked otoacoustic emissions were absent in left ear. The absence of clinical, laboratory and radiological evidence of a possible cause for complaints, an association between methylphenidate and sudden hearing loss was suggested. The patient received a standard course of oral corticosteroid and hyperbaric oxygen therapy. Weekly otological and audiological examinations were performed. Conservative and medical treatments offered no relief from hearing loss. Sudden hearing loss is a serious and irreversible adverse effect of methylphenidate. Therefore, the risk of hearing loss should be taken into consideration when initiating methylphenidate therapy.

  4. Sudden hearing loss after dental treatment.

    PubMed

    Kansu, Leyla; Yilmaz, Ismail

    2013-08-01

    A 66-year-old man presented with impaired balance, tinnitus, sensation of blockage, and hearing loss in his left ear, which developed after dental treatment for dental pain 4 days previously. Treatment of the carious left upper second molar tooth had included pulp extirpation, canal expansion, and tooth filling under local anesthesia with articaine and epinephrine. Impaired balance decreased spontaneously within 3 days of dental treatment, but tinnitus and hearing loss persisted. Pure tone audiogram showed profound sensorineural hearing loss in the left ear, with a downslope from 40 to 100 dB, and an abnormal speech discrimination score (50%). Treatment included intravenous prednisolone, intratympanic dexamethasone, and oral betahistine and trimetazidine. The patient had improved hearing and resolution of tinnitus. Sudden hearing loss is rare after dental treatment, and awareness of this complication may prompt early referral for treatment and may improve recovery and prognosis. Copyright © 2013 American Association of Oral and Maxillofacial Surgeons. All rights reserved.

  5. [Hearing loss in leprosarium: Current status].

    PubMed

    Kosugiyama, Reiri; Kasai, Norio; Etani, Tsutomu; Oshima, Akio

    2016-01-01

    Due to aging and aftereffects of leprosy, many of them are now struggling with hearing loss and visual impairment. The purpose of this study was to investigate and analyze the present situation of hearing level and hearing aid among residents in leprosy sanatorium. We have been carrying out hearing screening for the all ex-patients in three national sanatoriums, Nagashima-Aisein (since 2011), Oku-Komyo-en (since 2012), and Matsuoka-Hoyo-en (since 2014). The results of audiometry and questionnaire in 2014 were analyzed. Eligible persons who took pure tone audiometry were 198, and their age ranged between 59 and 99, mean 82.95. Their average hearing level of better hearing ear was 39.3 ± 17.2 dB. Since this result is not different from previous reports, which mentioned about average hearing level in elder persons, history of leprosy may not affect with hearing. According to the questionnaire, not only hearing level but also the awareness of hearing disability may lead to need for hearing aid. Also, it was considered that each communicative condition in sanatorium could have effect on self-assessment of hearing disability.

  6. 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.

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

    PubMed

    Hesse, Gerhard

    2016-07-01

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

  8. Hearing loss screening tests for adults.

    PubMed

    Becerril-Ramírez, Perla B; González-Sánchez, Dina F; Gómez-García, Angélica; Figueroa-Moreno, Rafael; Bravo-Escobar, Gerardo A; García de la Cruz, Miguel A

    2013-01-01

    The early detection of hearing loss has been studied widely in newborns due to the emerging technologies for diagnosis and treatment. There are detailed protocols for this goal. Nonetheless, hearing loss screening in adults has become more important lately with the increase of the life expectancy, an expected change in the Mexican population pyramid towards a rectangular shape in the next 50 years (with increased hearing loss prevalence) and the creation of public policies for social security such as the "Seguro Popular". There are no Mexican studies about hearing loss screening in adults. The aim of this work was to assess a tone emission and a questionnaire as screening tools for hearing loss in adults. A sample size of 500 individuals without otology pathology from the outpatient clinics at a general hospital. An otoscopy, 2 screening tests (tone emission and questionnaire) and tonal audiometry were performed on all subjects. The questionnaire turned out to be a sensitive test but with low specificity, while the tone emission was less sensitive but more specific with a higher rate of precision. In this study, the best result was achieved by a combined strategy using the two tests above, with a precision of 90%. The best screening strategy proposed by this study for hearing loss in adults is a questionnaire and tone emission test, which guarantees complete hearing assessment in objective and subjective manners, performed quickly and without special training. Copyright © 2012 Elsevier España, S.L. All rights reserved.

  9. Bilateral sudden sensorineural hearing loss: review.

    PubMed

    Sara, S A; Teh, B M; Friedland, P

    2014-01-01

    Unilateral and bilateral sudden sensorineural hearing loss represent different disease entities. The unilateral condition is more common and predominantly idiopathic, and up to 65 per cent of patients spontaneously recover hearing. Conversely, the bilateral condition is rare, mostly associated with serious systemic conditions, and has a higher prevalence of morbidity and mortality. A literature search using the PubMed database was conducted using the MeSH terms 'sudden', 'bilateral' and 'sensorineural hearing loss'. One hundred and three reported cases of bilateral sudden sensorineural hearing loss were identified. The condition is most often associated with toxic, autoimmune, neoplastic and vascular conditions. A younger age of onset, with a bimodal age distribution, was seen for bilateral sudden sensorineural hearing loss, compared with the unilateral condition. Patients with the bilateral condition had more profound hearing loss, with poorer recovery and a 35 per cent mortality rate. Vestibular symptoms were also less common than in the unilateral condition. The presentation of bilateral sudden onset sensorineural hearing loss is a medical emergency requiring thorough and urgent investigation to exclude life-threatening and reversible conditions.

  10. Cancer treatment in determination of hearing loss.

    PubMed

    Oliveira, Priscila Feliciano de; Oliveira, Camila Silva; Andrade, Joice Santos; Santos, Tamara Figueiredo do Carmo; Oliveira-Barreto, Aline Cabral de

    2016-01-01

    Chemotherapy and radiotherapy in oncology have repercussions in hearing health, and can damage structures of the inner ear. These repercussions usually, result in a bilateral and irreversible hearing loss. To identify sensorineural hearing loss cases with complaints of tinnitus and difficulty in speech understanding and investigate their relationship with the types of chemotherapy and radiotherapy the patients received. Cross-sectional, clinical, observational, analytical, historical cohort study of 58 subjects treated in a public hospital in the state of Sergipe, diagnosed with neoplasia. The subjects were submitted to anamnesis, conventional pure tone audiometry, and speech recognition threshold. Of the 116 ears, 25.9% presented sensorioneural hearing loss characterized by changes in high frequencies. There was a positive correlation between hearing loss and the association of chemotherapy and radiotherapy (p=0.035; R=0.196). The auditory complaint analysis shows that most of the subjects had tinnitus and speech understanding difficulty, even with a normal auditory threshold. Cancer treatment causes hearing loss, associated with the administration of chemotherapy and radiotherapy. Cyclophosphamide increased the risk of causing hearing loss. Complaints of tinnitus and speech understanding difficulty were observed. Copyright © 2015 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  11. Recovery of Idiopathic Sudden Sensorineural Hearing Loss.

    PubMed

    Edizer, Deniz Tuna; Çelebi, Özlem; Hamit, Bahtiyar; Baki, Ahmet; Yiğit, Özgür

    2015-08-01

    The objective was to identify and evaluate factors that may influence the recovery rate in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). A retrospective analysis was performed for patients with sudden sensorineural hearing loss between 2009 and 2013. Those with an identified etiology were excluded. The patients were divided into four treatment groups: (i) systemic corticosteroids (SC) only, (ii) SC+low-molecular-weight heparin (LMWH), (iii) SC+hyperbaric oxygen (HBO), and (iv) SC+LMWH+HBO. Recovery was evaluated according to Siegel's criteria. Age, initial hearing level, onset, treatment and audiogram types, comorbidities, and associated tinnitus and vestibular symptoms were investigated for their impact on prognosis. Two hundred five patients with ISSNHL were included. Recovery was seen in 59% of the patients. The complete recovery rate was significantly lower in patients older than 60 years and in patients presenting with profound hearing loss. Different audiogram curves had no significant effect on recovery. Sudden hearing loss was accompanied by tinnitus in 107 (52.1%) patients and vestibular symptoms in 55 (26.8%); however, neither was noted to affect prognosis. Different treatment combinations did not significantly affect prognosis. However, hypertension and a delay in treatment by more than 10 days from the onset of hearing loss were associated with a worse prognosis. Profound hearing loss, older than 60 years, a delay in treatment by more than 10 days, and hypertension were negative prognostic factors in this study, whereas, the type of audiogram curve and addition of HBO to SC did not affect prognosis.

  12. The fundamental and medical impacts of recent progress in research on hereditary hearing loss.

    PubMed

    Kalatzis, V; Petit, C

    1998-01-01

    What would define real progress in the field of deafness research in fundamental and medical terms? In fundamental terms, progress would be measured by an improvement in our knowledge of the development and physiology of the ear. In medical terms, progress would lead to the division of the broad category of hearing defects into distinct clinical entities or subclasses, the collection of epidemiological data, the creation of molecular diagnostic tests, the improvement of genetic counselling services and the development of new therapeutics. In this review, we will introduce some general considerations on hereditary hearing loss and on the structure and function of the ear, present the rapidly emerging data on the molecular basis of syndromic and non-syndromic forms of hearing loss and comment on relevant recent progress in this field of research. Generally speaking, the isolation of genes underlying hereditary hearing loss has, as yet, had little impact on our understanding of the biology of the ear, whereas it has made major contributions to the medical field, in particular due to the recognition of two genes, Cx26 and mitochondrial 12S rRNA , as frequently underlying cases of non-syndromic hearing impairment.

  13. Coping with Hearing Loss and High School

    ERIC Educational Resources Information Center

    Exceptional Parent, 2010

    2010-01-01

    High school can be a bumpy road for teenagers, especially since most teenagers are trying to fit in and start to define their own individuality and future. Now imagine if a teenager has hearing loss. Besides not being able to hear their instructors or friends as well as their classmates, self image problems can be magnified if they need to wear…

  14. Coping with Hearing Loss and High School

    ERIC Educational Resources Information Center

    Exceptional Parent, 2010

    2010-01-01

    High school can be a bumpy road for teenagers, especially since most teenagers are trying to fit in and start to define their own individuality and future. Now imagine if a teenager has hearing loss. Besides not being able to hear their instructors or friends as well as their classmates, self image problems can be magnified if they need to wear…

  15. Vibrant Soundbridge rehabilitation of sensorineural hearing loss.

    PubMed

    Khan, Andleeb; Hillman, Todd; Chen, Douglas

    2014-12-01

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

  16. [Genetic sensorineural hearing loss in childhood].

    PubMed

    Turan, Onur; Apaydin, Fazil

    2002-01-01

    This study aimed to determine the severity, age of presentation, and genetic etiology in hearing handicapped children. Three groups of children were studied: children who received treatment for hearing loss at the Otolaryngology Department of Ege University Hospital between 1993 and 1999 (1645 children) and throughout 2000 (212 children), and 443 students of Izmir Tülay Aktaş Deaf School during 1998 and 1999. All the children underwent otolaryngologic examination and a detailed history taking, audiometric examinations were undertaken in the second and third groups, as well. In children suspected of having a genetic etiology, the pedigrees were drawn and the mode of inheritance was determined. In the first group, awareness of the families to hearing loss and the presentation were at ages two and four, respectively. The most common accompanying anomaly was Down syndrome. Consanguinity was found in 27%, 8%, and 33%, respectively. Moderate to severe hearing loss was detected in 44% of the second group, and in 28% of the third group. Of the pedigrees obtained from 123 families, 86 were nonsyndromic and 27 were syndromic. Genetic inheritance plays a substantial role in sensorineural hearing loss in children, with consanguinity being the major culprit. Detection of hearing losses at younger ages will improve the success of hearing rehabilitation programs.

  17. 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.

  18. [Diagnostics on noise-induced hearing loss].

    PubMed

    Gimsing, Steen

    2014-07-28

    Occupational noise-induced hearing loss (NIHL) may qualify to compensation and must be distinguished from the ski slope hearing loss (SKI) which is a hereditary type of hearing loss. By combining the NIHL and presbyacusis tables of ISO 1999 it was concluded that in a hearing loss caused solely by noise and ageing the median threshold at 2 kHz is never poorer than 35 dB, and that the ratio between the actual 3 kHz threshold and the average 2-4 kHz threshold does not exceed 1.3. Thus, the audiometric characteristics of the 2-3 kHz segment can distinguish between NIHL and SKI.

  19. Sudden sensorineural hearing loss: an autoimmune disease?

    PubMed

    Greco, A; Fusconi, M; Gallo, A; Marinelli, C; Macri, G F; De Vincentiis, M

    2011-10-01

    To review our current knowledge of the pathogenesis of sudden sensorineural hearing loss, including viral infection, vascular occlusion and immune system-mediated mechanisms, and to discuss the pathogenesis as it relates to pharmacotherapy. Relevant publications on the pathogenesis of sudden sensorineural hearing loss from 1944 to 2010 were analysed. Sudden sensorineural hearing loss is defined as hearing loss of 30 dB in three sequential frequencies over 3 days or less. It can be an isolated symptom or the presenting symptom of a systemic disease. The aetiology and pathogenesis remain unknown. Detailed investigation typically reveals a specific cause in about 10% of patients. Proposed theories of causation include viral infections, vascular occlusion and immune system-mediated mechanisms. A variety of therapies have been proposed based on the various proposed aetiologies. Copyright © 2011 Elsevier B.V. All rights reserved.

  20. Acoustic Trauma - Hearing Loss in Teenagers

    MedlinePlus

    ... hearing loss. Kids expose themselves to noise through electronic media that often is louder than what is ... caused by exposure to loud noises, such as music played through headphones. The authors of another study ...

  1. Research progress in pathogenic genes of hereditary non-syndromic mid-frequency deafness.

    PubMed

    Xia, Wenjun; Liu, Fei; Ma, Duan

    2016-06-01

    Hearing impairment is considered as the most prevalent impairment worldwide. Almost 600 million people in the world suffer from mild or moderate hearing impairment, an estimated 10% of the human population. Genetic factors play an important role in the pathogenesis of this disorder. Hereditary hearing loss is divided into syndromic hearing loss (associated with other anomalies) and non-syndromic hearing loss (not associated with other anomalies). Approximately 80% of genetic deafness is non-syndromic. On the basis of the frequency of hearing loss, hereditary non-syndromic hearing loss can be divided into high-, mid-, low-, and total-frequency hearing loss. An audiometric finding of mid-frequency sensorineural hearing loss, or a "bowl-shaped" audiogram, is uncommon. Up to now, merely 7 loci have been linked to mid-frequency hearing loss. Only four genetic midfrequency deafness genes, namely, DFNA10 (EYA4), DFNA8/12 (TECTA), DFNA13 (COL11A2), DFNA44 (CCDC50), have been reported to date. This review summarizes the research progress of the four genes to draw attention to mid-frequency deafness genes.

  2. [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.

  3. Preventing noise-induced hearing loss in firefighters.

    PubMed

    Chalupka, Stephanie

    2013-09-01

    NIOSH recommends the use of quieter equipment, better work practices, and hearing protection devices and implementation of effective hearing loss prevention programs to prevent noise-induced hearing loss in firefighters. Copyright 2013, SLACK Incorporated.

  4. Hearing Loss: Symptoms, Diagnosis & Treatment

    MedlinePlus

    ... head Loud noise Assistive Devices Hearing aids —Small electronic devices worn in or behind the ear to ... fully in daily life Cochlear implants —Small, complex electronic devices that can help to provide a sense ...

  5. Hearing Loss and Older Adults

    MedlinePlus

    ... are the most common ones: Hearing aids are electronic instruments you wear in or behind your ear. ... information.) Cochlear (COKE-lee-ur) implants are small electronic devices surgically implanted in the inner ear that ...

  6. Age-Related Hearing Loss

    MedlinePlus

    ... potential sources of damaging noises , such as loud music, firearms, snowmobiles, lawn mowers, and leaf blowers. Avoiding ... near the kitchen or near a band playing music. Background noise makes it hard to hear people ...

  7. [Hearing loss in patients with Fabry disease].

    PubMed

    Limberger, A; Beck, M; Delgado-Sanchez, S; Keilmann, A

    2007-03-01

    Fabry disease is an X-linked lysosomal storage disease involving deficient activity of alpha-galactosidase A, which leads initially to pain, and later to renal insufficiency, cardiomyopathy and stroke. Until now few details are available on hearing impairment in patients with Fabry disease, and especially few relating to female patients. We examined 43 female and 29 male patients. In this study we looked into the question of whether and to what extent patients of both genders are affected by hearing impairment. Hearing loss is characteristic being more severe at high frequencies frequencies. Overall, 22 female and 15 male patients were found to have suffered a hearing loss. Patients with severe symptoms of Fabry disease usually demonstrate more prominent hearing losses. Both men and women with Fabry disease are affected by hearing impairment. It seems that the hearing loss is less marked in female than in male patients. Children with Fabry disease complain of tinnitus more frequently than other children and quite early in the course of the disease.

  8. Gender effects in hearing loss in Dalmatians.

    PubMed

    Famula, T R; Oberbauer, A M; Williams, D C

    2001-01-17

    Brainstem auditory-evoked-response data were collected from 3101 Dalmatian dogs from 1984 to 1998 at the Veterinary Medicine Teaching Hospital at the University of California, Davis. Also collected were data on eye color and the presence or absence of a color-patch at birth. Our objective was to evaluate the role of gender in hearing loss, including the possibility that the probability of suffering unilateral or bilateral deafness was greater if the dam was hearing impaired than if the sire was hearing impaired. Results of a multiple-trait threshold-model analysis support the commonly held observation that females were more likely to be deaf than males. In addition, females were also more likely to have two blue eyes (a condition associated with an increased prevalence of deafness). However, gender differences in hearing loss were limited to these direct observations. There was no detectable difference in the prevalence of hearing loss between offspring of deaf mothers and the offspring of deaf fathers. Finally, there was no detectable decrease in the prevalence of hearing loss over the years covered in the data set - suggesting that Dalmatian breeders are not yet selecting against hearing problems.

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

    PubMed

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

    1992-03-01

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

  10. Health Educators' Knowledge of Hearing, Hearing Loss, and Hearing Health Practices.

    ERIC Educational Resources Information Center

    Lass, Norman J.; And Others

    1990-01-01

    A questionnaire on hearing, hearing loss, and hearing health practices was developed and administered to 89 school health educators in 6 states. Analysis of responses indicates some deficiencies in their knowledge level suggesting the need for preservice and continuing education programs. The questionnaire is appended. (Author/DB)

  11. Hearing loss in veterans and the need for hearing loss prevention programs.

    PubMed

    Saunders, Gabrielle H; Griest, Susan E

    2009-01-01

    Currently, there are more than 445,000 veterans receiving compensation for hearing loss associated with military service, and 395,000 receiving compensation for service-related tinnitus. In addition to compensation payments, service-related hearing disorders cost the US Department of Veterans Affairs in terms of provision of hearing aids, hearing aid-related services, and clinical services at its 220 facilities nationwide. It is imperative that hearing conservation among military personnel and veterans be addressed. In this paper, we describe the rationale for and the development of a multimedia Hearing Loss Prevention Program aimed at preventing the progression of hearing loss among veterans associated with social, recreational, and nonmilitary occupational noise exposure. The program was developed based on the principles outlined in the Health Belief Model of Rosenstock (1966) and the Health Promotion Model of Pender et al. (2002).

  12. Sensorineural hearing loss in sporadic congenital hypothyroidism.

    PubMed Central

    Vanderschueren-Lodeweyckx, M; Debruyne, F; Dooms, L; Eggermont, E; Eeckels, R

    1983-01-01

    Hearing acuity was assessed in 45 children with sporadic congenital hypothyroidism during adequate long-term treatment. Otoscopy was performed in each and additional tympanometry in some of them. Secretory otitis media was found in 6 and was treated medically or by inserting grommets in the eardrum. In these children, hearing acuity was assessed after the otitis had been cured. Hearing acuity was measured either by conventional monoaural pure-tone audiometry (125-8000 Hz) or by binaural free field testing depending on the child's age (above and below 4 years respectively). Hearing was normal in 36 (80%) children. In the remaining 9, sensorineural hearing loss to some degree was detected affecting the higher frequencies in particular. Perceptive deafness required the use of a hearing aid in 4 children. No relationship could be found between hearing acuity and chronological age or bone age at diagnosis of congenital hypothyroidism or type of hypothyroidism. Sensorineural hearing loss is common in children with congenital hypothyroidism and should be searched for carefully and systematically to avoid difficulties related to speech and language development. PMID:6859932

  13. Mutations in LOXHD1 gene cause various types and severities of hearing loss

    PubMed Central

    Mori, Kentaro; Moteki, Hideaki; Kobayashi, Yumiko; Azaiez, Hela; Booth, Kevin T; Nishio, Shin-ya; Sato, Hiroaki; Smith, Richard J H; Usami, Shin-ichi

    2015-01-01

    Objective We present two families that were identified with novel mutations in LOXHD1, as a cause of non-progressive hearing loss. Methods One thousand three hundred fourteen (1,314) Japanese subjects with sensorineural hearing loss from unrelated families were enrolled in the study. Targeted genomic enrichment and massively parallel sequencing of all known non-syndromic hearing loss genes were performed to identify the genetic cause of hearing loss. Results Two patients in one family affected with homozygous mutation; c.879+1G>A in LOXHD1, showed profound congenital hearing loss, whereas two patients in the other family with compound heterozygous mutations; c.5869G>T (p.E1957X) and c.4480C>T (p.R1494X) showed moderate to severe hearing loss. Conclusion Mutations in LOXHD1 are extremely rare, and these cases are the first identified in a Japanese population. The genotype-phenotype correlation in LOXHD1 is still unclear. The differences of phenotypes in each patient might be the result of the nature of the mutations, or the location at the gene, or be influenced by genetic modifier. PMID:25792669

  14. Uncovering effective strategies for hearing loss prevention.

    PubMed

    Morata, Thais C; Meinke, Deanna

    2016-04-01

    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.

  15. 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

  16. 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.

  17. Early Hearing Detection and Vocabulary of Children With Hearing Loss.

    PubMed

    Yoshinaga-Itano, Christine; Sedey, Allison L; Wiggin, Mallene; Chung, Winnie

    2017-08-01

    To date, no studies have examined vocabulary outcomes of children meeting all 3 components of the Early Hearing Detection and Intervention (EHDI) guidelines (hearing screening by 1 month, diagnosis of hearing loss by 3 months, and intervention by 6 months of age). The primary purpose of the current study was to examine the impact of the current EHDI 1-3-6 policy on vocabulary outcomes across a wide geographic area. A secondary goal was to confirm the impact of other demographic variables previously reported to be related to language outcomes. This was a cross-sectional study of 448 children with bilateral hearing loss between 8 and 39 months of age (mean = 25.3 months, SD = 7.5 months). The children lived in 12 different states and were participating in the National Early Childhood Assessment Project. The combination of 6 factors in a regression analysis accounted for 41% of the variance in vocabulary outcomes. Vocabulary quotients were significantly higher for children who met the EHDI guidelines, were younger, had no additional disabilities, had mild to moderate hearing loss, had parents who were deaf or hard of hearing, and had mothers with higher levels of education. Vocabulary learning may be enhanced with system improvements that increase the number of children meeting the current early identification and intervention guidelines. In addition, intervention efforts need to focus on preventing widening delays with chronological age, assisting mothers with lower levels of education, and incorporating adults who are deaf/hard-of-hearing in the intervention process. Copyright © 2017 by the American Academy of Pediatrics.

  18. 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.

  19. Cochlear implantation for progressive hearing loss

    PubMed Central

    Gray, R; Jones, S; Court, I

    2003-01-01

    This paper shows that a subgroup of congenitally deaf children exists, who, having made good progress with conventional hearing aids, suffer a sudden or progressive hearing deterioration which arrests the speech development. Sixty children have been implanted in the Cambridge Programme, half for meningitis or other acquired losses and half for congenital prelingual deafness. Six of this latter group were congenital but progressive; their progress, deterioration, and improvement after implantation are summarised. PMID:12876170

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

    ERIC Educational Resources Information Center

    Garstecki, Dean C.; Erler, Susan F.

    1998-01-01

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

  1. Prognostic Factors in Sudden Sensorineural Hearing Loss

    PubMed Central

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

    2016-01-01

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

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

    PubMed

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

    2016-04-01

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

  3. 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

  4. Acoustic hearing implants for mixed hearing loss: a systematic review.

    PubMed

    Verhaert, Nicolas; Desloovere, Christian; Wouters, Jan

    2013-09-01

    A systematic review of literature to determine the clinical outcome and safety of the range of acoustic hearing implants (AHIs) in adults with mixed hearing loss (MHL). Databases MEDLINE, Embase, and Cochrane were searched with no language restrictions between 1950, or the start date of each database, up to March 1, 2013. Initial search found 1,794 studies, of which, 19 met the inclusion criteria of AHI for adults with MHL where safety, coupling strategies to the inner ear, hearing outcome, and patient-reported outcome measures (PROMs) were analyzed, preferably compared with a conventional hearing aid or a bone-conduction implant. A study quality assessment based on different parameters was included: specification of eligibility criteria, prospective study, ethical approval gained, appropriate controls, power calculation, outcome measures, and analysis performed. Comparisons between studies were made based on structured review as meta-analysis was not feasible because of the heterogeneity of outcome measures and reports. The current systematic review shows that AHI and their different coupling strategies in the treatment of MHL were beneficial in terms of speech in quiet, PROM, and safety regarding residual hearing. Overall, the level of evidence and the quality of the included studies were judged to be moderate to low. More comprehensive data on coupling to the inner ear and the comparison with conventional hearing aids or alternatives for speech in noise is mandatory. Long-term follow-up data are also needed.

  5. Genetics of Nonsyndromic Congenital Hearing Loss

    PubMed Central

    Egilmez, Oguz Kadir; Kalcioglu, M. Tayyar

    2016-01-01

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

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

    PubMed

    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.

  7. Dichotic listening in adults with sensorineural hearing loss.

    PubMed

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

    1976-01-01

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

  8. Comprehensive genetic testing in the clinical evaluation of 1119 patients with hearing loss.

    PubMed

    Sloan-Heggen, Christina M; Bierer, Amanda O; Shearer, A Eliot; Kolbe, Diana L; Nishimura, Carla J; Frees, Kathy L; Ephraim, Sean S; Shibata, Seiji B; Booth, Kevin T; Campbell, Colleen A; Ranum, Paul T; Weaver, Amy E; Black-Ziegelbein, E Ann; Wang, Donghong; Azaiez, Hela; Smith, Richard J H

    2016-04-01

    Hearing loss is the most common sensory deficit in humans, affecting 1 in 500 newborns. Due to its genetic heterogeneity, comprehensive diagnostic testing has not previously been completed in a large multiethnic cohort. To determine the aggregate contribution inheritance makes to non-syndromic hearing loss, we performed comprehensive clinical genetic testing with targeted genomic enrichment and massively parallel sequencing on 1119 sequentially accrued patients. No patient was excluded based on phenotype, inheritance or previous testing. Testing resulted in identification of the underlying genetic cause for hearing loss in 440 patients (39%). Pathogenic variants were found in 49 genes and included missense variants (49%), large copy number changes (18%), small insertions and deletions (18%), nonsense variants (8%), splice-site alterations (6%), and promoter variants (<1%). The diagnostic rate varied considerably based on phenotype and was highest for patients with a positive family history of hearing loss or when the loss was congenital and symmetric. The spectrum of implicated genes showed wide ethnic variability. These findings support the more efficient utilization of medical resources through the development of evidence-based algorithms for the diagnosis of hearing loss.

  9. Identification of a nonsense mutation in the STRC gene in a Korean family with moderate hearing loss.

    PubMed

    Sagong, Borum; Baek, Jeong-In; Bok, Jinwoong; Lee, Kyu-Yup; Kim, Un-Kyung

    2016-01-01

    Hereditary hearing loss is a heterogeneous disorder that results in a common sensorineural disorder. To date, more than 150 loci and 89 genes have been reported for non-syndromic hearing loss. Next generation sequencing has recently been developed as a powerful genetic strategy for identifying pathogenic mutations in heterogeneous disorders with various causative genes. In this study, we performed targeted sequencing to identify the causative mutation in a Korean family that had moderate hearing loss. We targeted 64 genes associated with non-syndromic hearing loss and sorted the homozygous variations according to the autosomal recessive inheritance pattern of the family. Implementing a bioinformatic platform for filtering and detecting variations allowed for the identification of two variations within different genes (c.650G>A in TRIOBP and c.4057C>T in STRC). These variants were selected for further analysis. Among these, c.4057C>T (p.Q1353X) was a divergent sequence variation between the STRC gene and the STRC pseudogene. This was the critical difference that resulted in loss of the protein-coding ability of the pseudogene. Therefore, we hypothesized that the p.Q1353X variation in the STRC gene is the causative mutation for hearing loss. This result suggests that application of targeted sequencing will be valuable for the diagnosis of heterogeneous disorders.

  10. Sensorineural hearing loss in postmeningitic children.

    PubMed

    Wellman, Mark B; Sommer, Doron D; McKenna, Joseph

    2003-11-01

    To establish the proportion of children who develop sensorineural hearing loss after bacterial meningitis and to correlate such loss with patient factors. Retrospective case review. McMaster University Children's Hospital, a tertiary referral center. Children between the ages of 1 day and 18 years admitted to McMaster University Children's Hospital with a confirmed diagnosis of bacterial meningitis between January 1, 1991 and December 30, 2000. Audiological assessment including auditory brainstem responses and cortical electric-response audiometry or standard audiometry. The nature of sensorineural hearing loss was assessed according to the degree (mild to profound) and course (transient versus permanent). Correlations between sensorineural hearing loss and the patient's age, sex, duration of illness before admission, use of dexamethasone, concurrent neurologic complications, and types of pathogens were evaluated. Patterns of inpatient and outpatient audiological assessment were determined. Seventy-nine children had confirmed bacterial meningitis. Streptococcus pneumoniae accounted for 36.7 percent of all cases, followed-up by Neisseria meningitides (16.5%), group B Streptococcus (15.2%), and Hemophilus influenzae (13.9%). Sixty-eight (86.1%) children underwent hearing assessment, either as inpatients or after discharge. Of the remaining 11 (13.9%) in whom audiological evaluation could not be confirmed, only two made mention of a referral. As such, a nonreferral rate of 11.4 percent was identified. Abnormal auditory brainstem response findings were present in 22 cases (32.3%), with 11 cases (13.9%) of permanent sensorineural hearing loss identified. A statistically significant association between sensorineural hearing loss and Streptococcus pneumoniae was found (p < 0.001). No association between age, sex, duration of illness before admission, use of dexamethasone, and number of concurrent neurologic complications could be established. In our series, 11 children

  11. Hearing loss in diffuse cutaneous systemic scleroderma.

    PubMed

    Monteiro, T A; Christmann, R B; Bonfá, E; Bento, R F; Novalo-Goto, E S; Vasconcelos, L G E

    2011-11-01

    Patients with diffuse cutaneous systemic sclerosis (dcSSc) have distinct clinical manifestations, usually with more aggressive organ involvement and highermortality than limited cutaneous systemic sclerosis (lcSSC). Hearing loss had not yet been evaluated in this subtype, so our aim was to assess the auditory system of dcSSc patients in a controlled study. Twenty-six patients with dcSSc, according to American College of Rheumatology (ACR) criteria, were evaluated. For each dcSSc patient included in the study, two healthy controls were matched for sex and age (± 2 years). All patients responded to a structured questionnaire regarding otological symptoms and risk factors with regard to hearing loss. A complete ear/nose/throat physical examination was performed followed by speech/pure tone audiometry and impedance measurements. The mean age of the dcSSc patients was 47.0 ± 11.9 years; the mean disease duration was 9.4 ± 9.1 years, and there was a clear female predominance (83%). The questionnaire revealed auditory complaints in 54% of the dcSSc patients (25% hearing loss, 21% aural fullness, 21% tinnitus, and 21% dizziness) and normal otoscopy. Nearly half (46%) of the dcSSc patients were diagnosed as having hearing loss on audiogram compared to only 19% in the control group (p = 0.01). All dcSSc with hearing loss had a sensorineural component, eight (73%) with excellent discrimination on the Percentage Index of Speech Recognition (PISR). Descending configuration and Metz recruitment were observed in 54% of these patients, suggesting cochlear involvement. Patients with dcSSc have a high prevalence of sensorineural audiometric hearing impairment and otological complaints, suggesting that the cochlea is an additional target organ in this disease.

  12. Sensorineural hearing loss in Kawasaki disease.

    PubMed

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

    2016-01-01

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

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

    PubMed

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

    2012-10-01

    Sensorineural hearing loss is a complex disease state influenced by genetics, age, noise, and many other factors. This article reviews our current knowledge regarding the causes of sensorineural hearing loss and reviews the more challenging clinical presentations of sensorineural hearing loss. We have reviewed the latest medical literature in an attempt to provide an evidence-based strategy for the assessment and management of sudden sensorineural hearing loss, rapidly progressive sensorineural hearing loss, and asymmetric/unilateral sensorineural hearing loss. Copyright © 2012 Elsevier Inc. All rights reserved.

  14. Hyperbaric oxygen therapy for sudden sensorineural hearing loss in divers.

    PubMed

    Van Der Wal, A W; Van Ooij, P J A M; De Ru, J A

    2016-11-01

    Sudden sensorineural hearing loss in divers may be caused by either inner-ear barotrauma or inner-ear decompression sickness. There is no consensus on the best treatment option. This study aimed to evaluate the therapeutic value of hyperbaric oxygen therapy for sudden sensorineural hearing loss in divers. A literature review and three cases of divers with sudden sensorineural hearing loss treated with hyperbaric oxygen therapy are presented. Hyperbaric oxygen therapy resulted in hearing improvement in 80 per cent of patients: 39 per cent had hearing improvement and 41 per cent had full recovery. Hyperbaric oxygen therapy improved hearing in divers with sudden sensorineural hearing loss.

  15. Auditory neuroplasticity, hearing loss and cochlear implants.

    PubMed

    Ryugo, David

    2015-07-01

    Data from our laboratory show that the auditory brain is highly malleable by experience. We establish a base of knowledge that describes the normal structure and workings at the initial stages of the central auditory system. This research is expanded to include the associated pathology in the auditory brain stem created by hearing loss. Utilizing the congenitally deaf white cat, we demonstrate the way that cells, synapses, and circuits are pathologically affected by sound deprivation. We further show that the restoration of auditory nerve activity via electrical stimulation through cochlear implants serves to correct key features of brain pathology caused by hearing loss. The data suggest that rigorous training with cochlear implants and/or hearing aids offers the promise of heretofore unattained benefits.

  16. 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.

  17. 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…

  18. 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.

  19. 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…

  20. Survey of commercial airline pilots' hearing loss

    NASA Technical Reports Server (NTRS)

    Begault, D. R.; Wenzel, E. M.; Tran, L. L.; Anderson, M. R.

    1998-01-01

    64 commercial airline pilots (ages 35-64 yr, Mdn: 53) were surveyed regarding hearing loss and tinnitus. Within specific age groups, the proportions responding positively exceed the corresponding proportions in the general population reported by the National Center for Health Statistics.

  1. Sensorineural hearing loss due to secondary syphilis.

    PubMed

    Jeans, A R; Wilkins, E G L; Bonington, A

    2008-05-01

    We present a case of sudden sensorineural hearing loss occurring as a complication of secondary syphilis. Syphilis affecting the inner ear, or otosyphilis, is well described in historical literature, but has rarely been reported in recent times. However, following the resurgence of syphilis in the UK, unusual manifestations such as otosyphilis are likely to be seen increasingly commonly.

  2. Survey of commercial airline pilots' hearing loss.

    PubMed

    Begault, D R; Wenzel, E M; Tran, L L; Anderson, M R

    1998-02-01

    64 commercial airline pilots (ages 35-64 yr, Mdn: 53) were surveyed regarding hearing loss and tinnitus. Within specific age groups, the proportions responding positively exceed the corresponding proportions in the general population reported by the National Center for Health Statistics.

  3. Survey of commercial airline pilots' hearing loss

    NASA Technical Reports Server (NTRS)

    Begault, D. R.; Wenzel, E. M.; Tran, L. L.; Anderson, M. R.

    1998-01-01

    64 commercial airline pilots (ages 35-64 yr, Mdn: 53) were surveyed regarding hearing loss and tinnitus. Within specific age groups, the proportions responding positively exceed the corresponding proportions in the general population reported by the National Center for Health Statistics.

  4. 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.

  5. Inheritance patterns of progressive hearing loss in laboratory strains of mice.

    PubMed

    Noben-Trauth, Konrad; Johnson, Kenneth R

    2009-06-24

    Positional cloning of mouse deafness mutations uncovered a plethora of proteins that have important functions in the peripheral auditory system in particular in the cochlear organ of Corti and stria vascularis. Most of these mutant variants follow a monogenic form of inheritance and are rare, highly penetrant, and deleterious alleles. Inbred and heterogenous strains of mice, in contrast, present with non-syndromic hearing impairment due to the effects of multiple genes and hypomorphic and less penetrant alleles that are often transmitted in a non-Mendelian manner. Here we review hearing loss inheritance patterns as they were discovered in different strains of mice and discuss the relevance of candidate genes to late-onset progressive hearing impairment in mouse and human.

  6. 'Ecstasy' enhances noise-induced hearing loss.

    PubMed

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

    2013-08-01

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

  7. Exome sequencing and genome-wide copy number variant mapping reveal novel associations with sensorineural hereditary hearing loss.

    PubMed

    Haraksingh, Rajini R; Jahanbani, Fereshteh; Rodriguez-Paris, Juan; Gelernter, Joel; Nadeau, Kari C; Oghalai, John S; Schrijver, Iris; Snyder, Michael P

    2014-12-20

    The genetic diversity of loci and mutations underlying hereditary hearing loss is an active area of investigation. To identify loci associated with predominantly non-syndromic sensorineural hearing loss, we performed exome sequencing of families and of single probands, as well as copy number variation (CNV) mapping in a case-control cohort. Analysis of three distinct families revealed several candidate loci in two families and a single strong candidate gene, MYH7B, for hearing loss in one family. MYH7B encodes a Type II myosin, consistent with a role for cytoskeletal proteins in hearing. High-resolution genome-wide CNV analysis of 150 cases and 157 controls revealed deletions in genes known to be involved in hearing (e.g. GJB6, OTOA, and STRC, encoding connexin 30, otoancorin, and stereocilin, respectively), supporting CNV contributions to hearing loss phenotypes. Additionally, a novel region on chromosome 16 containing part of the PDXDC1 gene was found to be frequently deleted in hearing loss patients (OR=3.91, 95% CI: 1.62-9.40, p=1.45×10(-7)). We conclude that many known as well as novel loci and distinct types of mutations not typically tested in clinical settings can contribute to the etiology of hearing loss. Our study also demonstrates the challenges of exome sequencing and genome-wide CNV mapping for direct clinical application, and illustrates the need for functional and clinical follow-up as well as curated open-access databases.

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

    PubMed

    Lüers, Jan-Christoffer; Hüttenbrink, Karl-Bernd

    2014-12-01

    The Vibrant Soundbridge is the world's most often implanted active middle ear implant or hearing aid. During the last few years, the device indications have expanded from sensorineural hearing loss to conductive and mixed hearing loss. Titanium couplers have led to improved contact of the floating mass transducer with the middle ear structures. The resulting hearing gain is satisfying for most patients, but so far, there is no clear audiologic advantage over conventional hearing aids. Currently, the indications are mainly related to intolerance of conventional hearing aids (eg, chronic otitis externa), severe mixed hearing loss with a destructed middle ear and certain medical diagnosis (eg, congenital atresia).

  9. Syndromes of hearing loss associated with visual loss.

    PubMed

    Abou-Elhamd, Kamal Ahmed; ElToukhy, Hesham Mohamed; Al-Wadaani, Fahad Abdullah

    2014-04-01

    Patients with hearing loss depend more on their vision on their communication and vice versa. ENT physician faces difficult situation when he or she consults a patient complaining of hearing loss with a problem of vision. The prevalence of ophthalmic abnormalities in deaf population is also higher compared to their hearing peers. This paper is to summarize the common syndromes associated with visual defect according to the site of affection in the eye. Review article. Syndromes which result in deaf blind person are different among adults compared to among children. Rubella syndrome and Down syndrome are the main causes of deaf blind people above 18 years of age. While in children, CHARGE syndrome is the main cause of deaf blind children. ENT physician should be aware of these syndromes for earlier detection of deaf blind persons. Deaf blind people need a great deal of help. Communication and education are crucial for them and need to be properly supported.

  10. Hear here: children with hearing loss learn words by listening.

    PubMed

    Lew, Joyce; Purcell, Alison A; Doble, Maree; Lim, Lynne H

    2014-10-01

    Early use of hearing devices and family participation in auditory-verbal therapy has been associated with age-appropriate verbal communication outcomes for children with hearing loss. However, there continues to be great variability in outcomes across different oral intervention programmes and little consensus on how therapists should prioritise goals at each therapy session for positive clinical outcomes. This pilot intervention study aimed to determine whether therapy goals that concentrate on teaching preschool children with hearing loss how to distinguish between words in a structured listening programme is effective, and whether gains in speech perception skills impact on vocabulary and speech development without them having to be worked on directly in therapy. A multiple baseline across subjects design was used in this within-subject controlled study. 3 children aged between 2:6 and 3:1 with moderate-severe to severe-profound hearing loss were recruited for a 6-week intervention programme. Each participant commenced at different stages of the 10-staged listening programme depending on their individual listening skills at recruitment. Speech development and vocabulary assessments were conducted before and after the training programme in addition to speech perception assessments and probes conducted throughout the intervention programme. All participants made gains in speech perception skills as well as vocabulary and speech development. Speech perception skills acquired were noted to be maintained a week after intervention. In addition, all participants were able to generalise speech perception skills learnt to words that had not been used in the intervention programme. This pilot study found that therapy directed at listening alone is promising and that it may have positive impact on speech and vocabulary development without these goals having to be incorporated into a therapy programme. Although a larger study is necessary for more conclusive findings, the

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

    PubMed

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

    2015-03-01

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

  12. Risk factors for hearing loss after pediatric meningitis in Japan.

    PubMed

    Adachi, Nodoka; Ito, Ken; Sakata, Hideaki

    2010-05-01

    We sought to identify predictors for hearing loss in Japanese children with meningitis. We analyzed 155 cases of pediatric meningitis without other entities causing hearing loss in children admitted to Saitama Children's Medical Center between 1990 and 2005 for potential risk factors for hearing loss, using multiple logistic regression. Auditory brain stem response tests were performed to evaluate hearing loss. Of 155 children, 35 (23%) developed hearing loss (21 unilaterally and 14 bilaterally). Profound hearing loss (greater than 90 dB normal hearing level) occurred in 15 patients (9.7%; 4 unilaterally and 11 bilaterally). Of 112 patients with positive cerebrospinal fluid cultures, 27 (24%) developed hearing loss and 13 (12%) showed profound loss. Of 22 patients with Streptococcus pneumoniae meningitis, 11 (50%) developed hearing loss and 7 (32%) showed profound loss. Of 54 patients with Haemophilus influenzae meningitis, 11 (20%) developed hearing loss and 4 (7.4%) showed profound loss. High serum C-reactive protein levels and cerebrospinal fluid cultures positive for Streptococcus pneumoniae were identified as significant risk factors for hearing loss. A high serum C-reactive protein level was first identified as a risk factor for hearing impairment after pediatric meningitis.

  13. [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.

  14. 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.

  15. Update of the spectrum of GJB2 gene mutations in 152 Moroccan families with autosomal recessive nonsyndromic hearing loss.

    PubMed

    Bakhchane, Amina; Bousfiha, Amale; Charoute, Hicham; Salime, Sara; Detsouli, Mustapha; Snoussi, Khalid; Nadifi, Sellama; Kabine, Mostafa; Rouba, Hassan; Dehbi, Hind; Roky, Rachida; Charif, Majida; Barakat, Abdelhamid

    2016-06-01

    Deafness is one of the most common genetic diseases in humans and is subject to important genetic heterogeneity. The most common cause of non syndromic hearing loss (NSHL) is mutations in the GJB2 gene. This study aims to update and evaluate the spectrum of GJB2 allele variants in 152 Moroccan multiplex families with non syndromic hearing loss. Seven different mutations were detected: c.35delG, p.V37I, p.E47X, p.G200R, p.Del120E, p.R75Q, the last three mutations were described for the first time in Moroccan deaf patients, in addition to a novel nonsense mutation, the c.385G>T which is not referenced in any database. Sixty six families (43.42%) have mutations in the coding region of GJB2, while the homozygous c.35delG mutation still to date the most represented 51/152 (33.55%). The analysis of the geographical distribution of mutations located in GJB2 gene showed more allelic heterogeneity in the north and center compared to the south of Morocco. Our results showed that the GJB2 gene is a major contributor to non syndromic hearing loss in Morocco. Thus, this report of the GJB2 mutations spectrum all over Morocco has an important implication for establishing a suitable molecular diagnosis.

  16. Bilateral simultaneous sudden sensorineural hearing loss.

    PubMed

    Chen, Yen-Hung; Young, Yi-Ho

    2016-03-15

    This study adopted an inner ear test battery and MR imaging in patients with bilateral sudden sensorineural hearing loss (SSNHL) to investigate their causes, disease extent, and evaluate hearing outcome. From 1995 to 2014, 16 patients with bilateral SSNHL received audiometry, caloric test and MR imaging. Vestibular-evoked myogenic potential (VEMP) test was added to the test battery after 2000. Percentages of abnormal mean hearing level (MHL), cervical VEMP test, ocular VEMP test, and caloric test in patients with bilateral SSNHL were 100% (32/32), 100% (12/12), 100% (4/4), and 81% (26/32), respectively, implying that not only the cochlear part but also the vestibular part was severely affected in both ears. Causes of bilateral SSNHL were neoplasm in 5 patients, stroke in 5, meningitis in 1, and unknown in 5. Post-treatment MHL did not significantly differ from pre-treatment MHL indicating poor hearing outcome. Seven patients (44%) had passed away within 5years after onset, 2 patients were lost, and 7 patients survived. Via MR imaging, causes of bilateral SSNHL were identified for 69% of cases. Both cochlear and vestibular endorgans/afferents were identified to be severely affected bilaterally by the vestibular test battery and resulted in poor hearing outcome. A high mortality rate (44%) indicates that bilateral SSNHL is an ominous sign for a more sinister underlying disease. Copyright © 2016 Elsevier B.V. All rights reserved.

  17. [Genetic study of hearing loss in families from Argentina].

    PubMed

    Reynoso, Raúl A; Hendl, Silvia; Barteik, Marìa E; Curet, Carlos A; Nicemboin, Luis; Moreno Barral, José; Rodríguez Ballesteros, Montserrat; Del Castillo, Ignacio; Moreno, Felipe

    2004-01-01

    Recent advances in molecular genetics as well as improved strategies for the prevention and control of non-syndromic hearing loss (NSHL) have contributed to the rising importance of their inherited causes. In this study we report 32 families from Argentine with one (sporadic) or more (familial) individuals affected. All the families were initially screened for mutations in three autosomal nuclear genes and one mutation in mitochondrial DNA. These genes have been found in a great number of familial or sporadic cases of congenital deafness in Caucasians. The mutant allele 35 del G of connexin 26 (GJB2, locus DFNB1 on 13q12) was present in three families. We have investigated the gene encoding otoferlin (OTOF, locus DFNB9 on 2p22-p23) and we found the Q829X mutation in heterocigosity in two families. We have also identified in heterocigosity the 342-kb deletion of connexin 30 (GJB6, locus DFNB1 on 13q12) in one family. On the other hand, we have not found any patient with mitochondrial mutation. Since the screening for other mutations is very expensive, our main goal is to investigate the most frequent mutations in each separate gene in the argentine population and to develop simple and specific tests for each frequent mutations.

  18. Genetics of Hearing Loss – Syndromic

    PubMed Central

    Koffler, Tal; Ushakov, Kathy; Avraham, Karen B.

    2015-01-01

    Synopsis Hearing loss (HL) is one of the most common birth defects in developed countries and is a diverse pathology with different classifications. One of these is based on the association with other clinical features, defined as syndromic hearing loss (SHL). Determining the etiology of the HL in these patients is extremely beneficial as it enables a personalized approach to caring for the individual. Early screening can further aid in optimal rehabilitation for a child’s development and growth. Pathogenic variants in forty-five genes, encoding proteins functioning as ion channels, transcription factors, molecular motors and more, are known to lead to eleven forms of SHL. The development of high-throughput sequencing technology is facilitating rapid and low-cost diagnostics for patients with SHL. PMID:26443487

  19. [Hyperinsulinemia in vertigo, tinnitus and hearing loss].

    PubMed

    Doroszewska, Grazyna; Kaźmierczak, Henryk

    2002-01-01

    48 patients (25 woman, mean age 42 +/- 9.9 years and 23 men, mean age 46.6 +/- 8.3 years) suffering from vertigo, tinnitus and hearing loss of unknown origin were included into this study. The occurrence and coexistence of symptoms was measured. Insulin levels were measured fasting and at the second hour of OGTT. Results were compared with the control group of 31 healthy persons (16 women, mean age 41.3 +/- 5.6 years and 15 men, mean age 47.6 +/- 9.4 years). The character of vertigo, localization of hearing loss and electronystagmographic findings showed the pathology of the inner ear. The occurrence of hyperinsulinemia was significantly more common in the patients group--43.8%, comparing to the control group--22.6%. Also the insulin levels in the second hour of OGTT, were statistically significantly higher in patients that in the control group.

  20. 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.

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

    PubMed Central

    Kerr, Leslie Dubin

    2016-01-01

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

  2. 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

  3. [Prognostic factors of sudden sensorineural hearing loss in children].

    PubMed

    Li, Fengjiao; Xue, Xijun; Wang, Li; Yang, Fengbo; Wang, Hongyang; Guan, Jing; Du, Wan; Xiong, Wenping; Wu, Kaiwen; Wu, Mukun; Yin, Zifang; Lan, Lan; Wang, Dayong; Wang, Qiuju

    2015-11-01

    The aim of this retrospective study was to analyze the recovery rate of sudden sensorineural hearing loss in children, and explore the prognostic factors in order to guide the clinical diagnosis and treatment. A retrospective review was conducted for the prognosis of children with sudden sensorineural hearing loss during the past 5 years (from November 2010 to May 2015) in Chinese PLA General Hospital. This paper have a complete clinical data of 101 patients (113 ears)with sudden hearing loss, ranging from 0 to 18 years old Patients were divided into four groups according to hearing recovery and eight putative prognostic factors were analyzed. Among 101 patients (113 ears), the ratio of male and female was 60:53. Treatment was initiated from 1 to 183 days after disease onset, with an average of (18.5 ± 22.1) d. Bilateral and unilateral hearing loss were 24 ears and 89 ears, respectively. The proportion of mild hearing loss, moderate hearing loss, severe hearing loss and profound hearing loss were 7.1%, 6.2%, 23.9% and 62.8%, respectively. Vertigo and tinnitus occurred in 54.9% and 77.9% of the patients, respectively. After the treatment, the complete recovery rate was 9.7% and the overall recovery rate was 36.3%. The degree of hearing loss, earlier treatment onset, sex and bilateral involvement were significantly associated with hearing recovery (P < 0.05). Sudden sensorineural hearing loss in children was generally identified as severe and profound hearing loss, but after positive and timely treatment, it can be improved or even cured. The mild hearing loss, earlier treatment onset, unilateral hearing loss and female were positive prognostic factors. The concurrence of tinnitus or vertigo, the results of ABR and DPOAE had no significant influence on prognosis.

  4. The relationship between hearing loss and vestibular dysfunction in patients with sudden sensorineural hearing loss.

    PubMed

    Niu, Xiaorong; Zhang, Yan; Zhang, Qing; Xu, Xinda; Han, Peng; Cheng, Ying; Gao, Ying; Zhang, Rui; Yang, Yintong; Chen, Zichen; Hu, Juan; Chen, Yanfei; Xu, Min

    2016-01-01

    To investigate the relationship between hearing loss and vestibular dysfunction in patients with sudden sensorineural hearing loss (SSHL). Clinical data including the symptom of vertigo of 149 SSHL patients were investigated retrospectively. Pure tone audiometry, ocular vestibular-evoked myogenic potential (oVEMP) and cervical vestibular-evoked myogenic potential (cVEMP) evoked by air-conducted sound (ACS), and caloric test were employed for cochlear and vestibular function assessment. The relationship between hearing level and vestibular dysfunction was analyzed. The pure tone averages (PTAs) (mean ± SD) of SSHL patients with and without vertigo were 88.81 ± 21.74 dB HL and 72.49 ± 21.88 dB HL (Z = -4.411, p = 0.000), respectively. The PTAs of SSHL patients with abnormal and normal caloric test were 84.71 ± 22.54 dB HL and 70.41 ± 24.07 dB HL (t = -2.665, p = 0.009), respectively. Conversely, vertigo and abnormal caloric results also happened more frequently in patients with profound hearing loss. However, no consistent tendency could be found among vestibular evoked myogenic potentials (VEMPs) responses or hearing loss. SSHL patients with vertigo or abnormal caloric test displayed worse hearing loss; and vice versa, vertigo and abnormal caloric results happened more frequently in SSHL patients with profound hearing loss.

  5. 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.

  6. Personal Sound Amplifiers for Adults with Hearing Loss.

    PubMed

    Mamo, Sara K; Reed, Nicholas S; Nieman, Carrie L; Oh, Esther S; Lin, Frank R

    2016-03-01

    Age-related hearing loss is highly prevalent and often untreated. Use of hearing aids has been associated with improvements in communication and quality of life, but such treatment is unaffordable or inaccessible for many adults. The purpose of this review is to provide a practical guide for physicians who work with older adults who are experiencing hearing and communication difficulties. Specifically, we review direct-to-consumer amplification products that can be used to address hearing loss in adults. Helping adults with hearing loss navigate hearing loss treatment options ranging from being professionally fitted with hearing aids to using direct-to-consumer amplification options is important for primary care clinicians to understand given our increasing understanding of the impact of hearing loss on cognitive, social, and physical functioning.

  7. Personal Sound Amplifiers for Adults with Hearing Loss

    PubMed Central

    Mamo, Sara K.; Reed, Nicholas S.; Nieman, Carrie L.; Oh, Esther S.; Lin, Frank R.

    2015-01-01

    Age-related hearing loss is highly prevalent and often untreated. Use of hearing aids has been associated with improvements in communication and quality of life, but such treatment is unaffordable and/or inaccessible for many adults. The purpose of this review is to provide a practical guide for physicians who work with older adults who are experiencing hearing and communication difficulties. Specifically, we review direct-to-consumer amplification products that can be used to address hearing loss in adults. Helping adults with hearing loss navigate hearing loss treatment options ranging from being professionally fit with hearing aids to using direct-to-consumer amplification options is important for primary care clinicians to understand given our increasing understanding of the impact of hearing loss on cognitive, social, and physical functioning. PMID:26498713

  8. Designer aminoglycosides prevent cochlear hair cell loss and hearing loss.

    PubMed

    Huth, Markus E; Han, Kyu-Hee; Sotoudeh, Kayvon; Hsieh, Yi-Ju; Effertz, Thomas; Vu, Andrew A; Verhoeven, Sarah; Hsieh, Michael H; Greenhouse, Robert; Cheng, Alan G; Ricci, Anthony J

    2015-02-01

    Bacterial infections represent a rapidly growing challenge to human health. Aminoglycosides are widely used broad-spectrum antibiotics, but they inflict permanent hearing loss in up to ~50% of patients by causing selective sensory hair cell loss. Here, we hypothesized that reducing aminoglycoside entry into hair cells via mechanotransducer channels would reduce ototoxicity, and therefore we synthesized 9 aminoglycosides with modifications based on biophysical properties of the hair cell mechanotransducer channel and interactions between aminoglycosides and the bacterial ribosome. Compared with the parent aminoglycoside sisomicin, all 9 derivatives displayed no or reduced ototoxicity, with the lead compound N1MS 17 times less ototoxic and with reduced penetration of hair cell mechanotransducer channels in rat cochlear cultures. Both N1MS and sisomicin suppressed growth of E. coli and K. pneumoniae, with N1MS exhibiting superior activity against extended spectrum β lactamase producers, despite diminished activity against P. aeruginosa and S. aureus. Moreover, systemic sisomicin treatment of mice resulted in 75% to 85% hair cell loss and profound hearing loss, whereas N1MS treatment preserved both hair cells and hearing. Finally, in mice with E. coli-infected bladders, systemic N1MS treatment eliminated bacteria from urinary tract tissues and serially collected urine samples, without compromising auditory and kidney functions. Together, our findings establish N1MS as a nonototoxic aminoglycoside and support targeted modification as a promising approach to generating nonototoxic antibiotics.

  9. Vision loss and hearing loss in painting and musical composition.

    PubMed

    Marmor, Michael F

    2014-07-01

    This article considers the impact of vision and hearing loss on great painters and musical composers. The visual work of Mary Cassatt, Georgia O'Keeffe, Edgar Degas, and Claude Monet all showed alterations as their vision failed. In contrast, Gabriel Fauré, Bedřich Smetana, and Ludwig von Beethoven wrote many of their best compositions while totally deaf, and Georg Friedrich Handel and Frederick Delius struggled to compose late in life when they lost their vision (although their hearing remained excellent). There are 2 major distinctions between the role of vision and hearing for these artistic disciplines. First, there is a surrogate means of "hearing" music, through the musical score, which allows composers to write and edit music while totally deaf. The greatest problem with deafness for a skilled composer is interference from internal noise (tinnitus). There is no surrogate for vision to allow a painter to work when the subject is a blur or the colors on the canvas cannot be distinguished. Second, although the appreciation of art is visual and that of music is auditory, the transcription of both art and musical composition is visual. Thus, visual loss does pose a problem for a composer accustomed to working with good sight, because it disrupts habitual methods of writing and editing music. Copyright © 2014 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.

  10. Genetic Predisposition to Sporadic Congenital Hearing Loss in a Pediatric Population

    PubMed Central

    Jung, Jinsei; Lee, Joon Suk; Cho, Kyeong Jee; Yu, Seyoung; Yoon, Joo-Heon; Yung Gee, Heon; Choi, Jae Young

    2017-01-01

    Discriminating between inherited and non-inherited sporadic hearing loss is challenging. Here, we attempted to delineate genetic inheritance in simplex cases of severe-to-profound congenital hearing loss in Korean children. Variations in SLC26A4 and GJB2 in 28 children with bilateral severe-to-profound non-syndromic hearing loss (NSHL) without familial history were analyzed using Sanger sequencing. Genetic analysis of individuals without mutations in SLC26A4 and GJB2 was performed by whole exome sequencing (WES). Bi-allelic mutations in SLC26A4 and GJB2 were identified in 12 and 3 subjects, respectively. Of the 13 individuals without mutations in SLC26A4 and GJB2, 2 and 1 carried compound heterozygous mutations in MYO15A and CDH23, respectively. Thus, 64.3% (18/28) of individuals with NSHL were determined to be genetically predisposed. Individuals with sporadic severe-to-profound NSHL were found to mostly exhibit an autosomal recessive inheritance pattern. Novel causative candidate genes for NSHL were identified by analysis of WES data of 10 families without mutations in known causative genes. Bi-allelic mutations predisposing to NSHL were identified in 64.3% of subjects with sporadic severe-to-profound NSHL. Given that several causative genes for NSHL are still unidentified, genetic inheritance of sporadic congenital hearing loss could be more common than that indicated by our results. PMID:28383030

  11. Molecular genetics of MARVELD2 and clinical phenotype in Pakistani and Slovak families segregating DFNB49 hearing loss.

    PubMed

    Nayak, Gowri; Varga, Lukas; Trincot, Claire; Shahzad, Mohsin; Friedman, Penelope L; Klimes, Iwar; Greinwald, John H; Riazuddin, S Amer; Masindova, Ivica; Profant, Milan; Khan, Shaheen N; Friedman, Thomas B; Ahmed, Zubair M; Gasperikova, Daniela; Riazuddin, Sheikh; Riazuddin, Saima

    2015-04-01

    Pathogenic mutations of MARVELD2, encoding tricellulin, a tricelluar tight junction protein, cause autosomal recessive non-syndromic hearing loss (DFNB49) in families of Pakistan and Czech Roma origin. In fact, they are a significant cause of prelingual hearing loss in the Czech Roma, second only to GJB2 variants. Previously, we reported that mice homozygous for p.Arg497* variant of Marveld2 had a broad phenotypic spectrum, where defects were observed in the inner ear, heart, mandibular salivary gland, thyroid gland and olfactory epithelium. The current study describes the types and frequencies of MARVELD2 alleles and clinically reexamines members of DFNB49 families. We found that MARVELD2 variants are responsible for about 1.5 % (95 % CI 0.8-2.6) of non-syndromic hearing loss in our cohort of 800 Pakistani families. The c.1331+2T>C allele is recurrent. In addition, we identified a novel large deletion in a single family, which appears to have resulted from non-allelic homologous recombination between two similar Alu short interspersed elements. Finally, we observed no other clinical manifestations co-segregating with hearing loss in DFNB49 human families, and hypothesize that the additional abnormalities in the Marveld2 mutant mouse indicates a critical non-redundant function for tricellulin in other organ systems.

  12. Current office-based hearing screening questions fail to identify adolescents at risk for hearing loss.

    PubMed

    Sekhar, Deepa L; Zalewski, Thomas R; King, Tonya S; Paul, Ian M

    2014-12-01

    There is a rising prevalence of hearing loss among adolescents in the United States. Current paediatric preventive care recommendations by the Bright Futures guidelines and the American Academy of Pediatrics suggest that clinicians should ask adolescents ten hearing screening questions to identify those who are at high risk of hearing loss for further objective hearing testing. We assessed the utility of these subjective risk assessment questions to distinguish those adolescents with objectively documented hearing loss. A single public high school in Pennsylvania. We compared results from a prospective study evaluating objective hearing assessments with the use of the ten Bright Futures hearing screening questions plus additional adolescent-specific questions to predict adolescent hearing loss. In relation to the questions used in this study, adolescents who were referred following objective hearing screens were more likely to report "trouble following the conversation when two or more people are talking at the same time" and a "past experience of slight hearing loss". Referrals from sound treated booth testing were more likely to report "trouble hearing over the phone" and have a diagnosis or history of hearing loss. Most Bright Futures questions were not associated with adolescent hearing loss. An objective adolescent hearing screen should be considered in the recommended schedule of preventive care, instead of the current risk-based subjective assessment. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  13. Evaluation of Jet Fuel Induced Hearing Loss in Rats

    DTIC Science & Technology

    2011-10-13

    induced hearing loss by carbon monoxide. Hear Res. 150, 206–214. Sliwinska-Kowalska, M., Zamyslowska-Szmytke, E., Szymczak ,W., Kotylo, P., Fiszer, M...risk of hearing loss. Scand. J. Work Environ. Health 27, 335–342. Sliwinska-Kowalska, M., Zamyslowska-Szmytke, E., Szymczak ,W., Kotylo, P., Fiszer

  14. Reiter's syndrome and hearing loss: a possible association?

    PubMed

    Monsanto, Rafael C; Neto, Arlindo C L; Lorenzetti, Fábio T M

    2014-12-01

    Patient complained of hearing loss and tinnitus after the onset of Reiter's syndrome. Audiometry confirmed the hearing loss on the left ear; blood work showed increased erythrocyte sedimentation rate and C3 fraction of the complement. Genotyping for HLA-B27 was positive. Treatment with prednisolone did not improve the hearing levels.

  15. Reiter's syndrome and hearing loss: a possible association?

    PubMed Central

    Monsanto, Rafael C; Neto, Arlindo C L; Lorenzetti, Fábio T M

    2014-01-01

    Key Clinical Message Patient complained of hearing loss and tinnitus after the onset of Reiter's syndrome. Audiometry confirmed the hearing loss on the left ear; blood work showed increased erythrocyte sedimentation rate and C3 fraction of the complement. Genotyping for HLA-B27 was positive. Treatment with prednisolone did not improve the hearing levels. PMID:25548635

  16. The mapping of DFNB62, a new locus for autosomal recessive non-syndromic hearing impairment, to chromosome 12p13.2-p11.23.

    PubMed

    Ali, G; Santos, R L P; John, P; Wambangco, M A L; Lee, K; Ahmad, W; Leal, Sm

    2006-05-01

    Autosomal recessive non-syndromic hearing impairment (ARNSHI) is the most common form of prelingual inherited hearing impairment (HI). Here is described the mapping of a novel ARNSHI locus in a consanguineous Pakistani family with profound congenital HI. Two-point and multipoint linkage analyses were performed for the genome scan and fine mapping markers. Haplotypes were constructed to determine the region of homozygosity. At theta = 0, the maximum two-point LOD score of 4.0 was obtained at marker AAC040. A maximum multipoint LOD score of 5.3 was derived at marker D12S320, with the three-unit support interval demarcated by D12S89 and D12S1042. The region of homozygosity is flanked by markers D12S358 and D12S1042, which corresponds to 22.4 cM according to the Rutgers combined linkage-physical map of the human genome and spans 15.0 Mb on the sequence-based physical map. A novel ARNSHI locus DFNB62 was mapped to chromosome 12p13.2-p11.23. DFNB62 represents the second ARNSHI locus to map to chromosome 12.

  17. Studies in Pediatric Hearing Loss at the House Research Institute

    PubMed Central

    Eisenberg, Laurie S.; Johnson, Karen C.; Martinez, Amy S.; Visser-Dumont, Leslie; Ganguly, Dianne Hammes; Still, Jennifer F.

    2012-01-01

    Three clinical research projects are described that are relevant to pediatric hearing loss. The three projects fall into two distinct areas. The first area emphasizes clinical studies that track developmental outcomes in children with hearing loss; one project is specific to cochlear implants and the other to hearing aids. The second area addresses speech perception test development for very young children with hearing loss. Although these two lines of research are treated as separate areas, they begin to merge as new behavioral tests become useful in developing protocols for contemporary studies that address longitudinal follow-up of children with hearing loss. PMID:22668762

  18. Reversible hearing loss following cryptococcal meningitis: case study.

    PubMed

    Neo, W L; Durisala, N; Ho, E C

    2016-07-01

    Sensorineural hearing loss is a recognised complication of cryptococcal meningitis. The mechanism of hearing loss in patients with cryptococcal meningitis is different from that in bacterial meningitis. An immune-competent man with cryptococcal meningitis presented with sudden onset, bilateral, severe to profound sensorineural hearing loss and vestibular dysfunction. He was initially evaluated for cochlear implantation. However, he had a significant recovery; he no longer required surgery and was able to cope without a hearing aid. Typically, cochlear implantation is performed with some urgency in patients with hearing loss post-bacterial meningitis, because of the risk of labyrinthitis ossificans. However, this process has not been described in patients with cryptococcal meningitis. Furthermore, patients with hearing loss associated with cryptococcal meningitis have shown varying degrees of reversibility. In this case report, hearing loss from cryptococcal meningitis is compared with that from bacterial meningitis, and the need for cochlear implantation in patients with cryptococcal meningitis is discussed.

  19. Hearing Preservation Electrodes in Veterans and Military Service members with Noise-Induced Hearing Loss

    DTIC Science & Technology

    2016-07-01

    AWARD NUMBER: W81XWH-14-2-0019 TITLE: Hearing Preservation Electrodes in Veterans and Military Servicemembers With Noise-Induced Hearing Loss ...Hearing Loss 5b. GRANT NUMBER W81XWH-14-2-0019 5c. PROGRAM ELEMENT NUMBER 6. AUTHOR(S) Marlan Hansen, MD 5d. PROJECT NUMBER 5e. TASK NUMBER E-Mail...real need to provide rehabilitative options for veterans and service members with severe noise-induced hearing loss (NIHL). Recent studies indicate

  20. Hearing: Its Evolution and Ways of Compensating for Its Loss.

    ERIC Educational Resources Information Center

    De l'Aune, William R.

    1980-01-01

    The article explores the theory that the sense of hearing evolved to help an organism localize its position in space and perform aural analyses of that space and describes two major kinds of hearing loss--conductive and sensorineural. (PHR)

  1. [Siblings with pediatric hearing loss. Data on age at diagnosis and degree of hearing loss].

    PubMed

    Finckh-Krämer, U; Spormann-Lagodzinski, M E; Gross, M

    2001-03-01

    The Deutsches Zentralregister für kindliche Hörstörungen (DZH = German Registry for Hearing Loss in Children) has so far (30.6.2000) entered data records of 4,027 permanently hearing impaired children. These records include data on a total of 151 pairs of siblings. In seven cases a third sibling was registered. An analysis of the data of siblings with different dates of birth shows that the diagnosis for older siblings was delayed 2 years on average compared to the younger siblings (mean 23.6 months, 95% confidence interval 19.3-27.8 months). The degree of hearing loss, which crucially determines the average age at which a child is diagnosed, correlates statistically significantly with pairs of siblings, so that misleading results can be almost totally avoided. In 11 of 17 cases of twins and triplets, the diagnosis was pronounced simultaneously for both siblings; in the remaining 6 cases, one sibling was diagnosed later. It has become apparent that younger siblings of hearing-impaired children profit from the concomitant routine diagnostic investigation of siblings of hearing-impaired children nowadays common in many institutions, but avoidable delays nevertheless still occur, as shown by the data for twins and triplets. Since in approximately 18% of the registered children with one or more siblings one of the brothers or sisters is likewise hearing-impaired, consistent family investigation is recommended.

  2. Hearing loss and PRPS1 mutations: Wide spectrum of phenotypes and potential therapy.

    PubMed

    Liu, Xue Zhong; Xie, Dinghua; Yuan, Hui Jun; de Brouwer, Arjan P M; Christodoulou, John; Yan, Denise

    2013-01-01

    The purpose of this review was to evaluate the current literature on phosphoribosylpyrophosphate synthetase 1 (PRPS1)-related diseases and their consequences on hearing function. A literature search of peer-reviewed, published journal articles was conducted in online bibliographic databases. Three databases for medical research were included in this review. Mutations in PRPS1 are associated with a spectrum of non-syndromic to syndromic hearing loss. Hearing loss in male patients with PRPS1 mutations is bilateral, moderate to profound, and can be prelingual or postlingual, progressive or non-progressive. Audiogram shapes associated with PRPS1 deafness are usually residual and flat. Female carriers can have unilateral or bilateral hearing impairment. Gain of function mutations in PRPS1 cause a superactivity of the PRS-I protein whereas the loss-of-function mutations result in X-linked nonsyndromic sensorineural deafness type 2 (DFN2), or in syndromic deafness including Arts syndrome and X-linked Charcot-Marie-Tooth disease-5 (CMTX5). Lower residual activity in PRS-I leads to a more severe clinical manifestation. Clinical and molecular findings suggest that the four PRPS1 disorders discovered to date belong to the same disease spectrum. Dietary supplementation with S-adenosylmethionine (SAM) appeared to alleviate the symptoms of Arts syndrome patients, suggesting that SAM could compensate for PRS-I deficiency.

  3. The Study of SLC26A4 Gene Causing Autosomal Recessive Hearing Loss by Linkage Analysis in a Cohort of Iranian Populations

    PubMed Central

    Reiisi, Somayeh; Sanati, Mohammad Hosein; Tabatabaiefar, Mohammad Amin; Ahmadian, Shahla; Reiisi, Salimeh; Parchami, Shahrbanoo; Porjafari, Hamid; Shahi, Heshmat; Shavarzi, Afsaneh; Hashemzade Chaleshtori, Morteza

    2014-01-01

    Sensorineural non-syndromic hearing loss is the most common disorder which affects 1 in 500 newborns. Hearing loss is an extremely heterogeneous defect with more than 100 loci identified to date. According to the studies, mutations in GJB2 are estimated to be involved in 50- 80% of autosomal recessive non-syndromic hearing loss cases, but contribution of other loci in this disorder is yet ambiguous. With regard to studies, DFNB4 locus (SLC26A4) can be classified as the second cause of hearing loss. So, this study aimed to determine the contribution of this locus in hearing loss as well as the frequency of SLC26A4 gene mutations in a population in the west of Iran. In this descriptive laboratory study, we included 30 families from the west of Iran with no mutation in GJB2 gene. Linkage analysis was performed by DFNB4 (SLC26A4) molecular markers (STR). The families with hearing loss linked to this locus were further analyzed for mutation detection. SLC26A4 gene exons were amplified and analyzed using direct DNA sequencing. In studied families, 2 families displayed linkage to DFNB4 locus. Identified mutations include mutation in exon 5 (c.416 G>T) and in splicing site of exon 7 (IVS-2 A>G or c.919-2 A>G). PMID:25317404

  4. Reported Causes of Hearing Loss for Hearing Impaired Students; United States 1970-71.

    ERIC Educational Resources Information Center

    Gentile, Augustine; Rambin, J. Bentley

    Reported are causes of hearing loss for 41,109 hearing impaired students enrolled in 555 special educational programs as part of a national annual survey during the 1970-71 school year. Data is provided on the relationship between hearing loss etiology and the following variables: age and sex of students, additional handicapping conditions, family…

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

    PubMed

    Taiji, Hidenobu; Morimoto, Noriko

    2012-07-01

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

  6. 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

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

    PubMed

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

    2014-07-29

    A number of viral infections can cause hearing loss. Hearing loss induced by these viruses can be congenital or acquired, unilateral or bilateral. Certain viral infections can directly damage inner ear structures, others can induce inflammatory responses which then cause this damage, and still others can increase susceptibility or bacterial or fungal infection, leading to hearing loss. Typically, virus-induced hearing loss is sensorineural, although conductive and mixed hearing losses can be seen following infection with certain viruses. Occasionally, recovery of hearing after these infections can occur spontaneously. Most importantly, some of these viral infections can be prevented or treated. For many of these viruses, guidelines for their treatment or prevention have recently been revised. In this review, we outline many of the viruses that cause hearing loss, their epidemiology, course, prevention, and treatment. © The Author(s) 2014.

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

    PubMed Central

    Cohen, Brandon E.; Durstenfeld, Anne

    2014-01-01

    A number of viral infections can cause hearing loss. Hearing loss induced by these viruses can be congenital or acquired, unilateral or bilateral. Certain viral infections can directly damage inner ear structures, others can induce inflammatory responses which then cause this damage, and still others can increase susceptibility or bacterial or fungal infection, leading to hearing loss. Typically, virus-induced hearing loss is sensorineural, although conductive and mixed hearing losses can be seen following infection with certain viruses. Occasionally, recovery of hearing after these infections can occur spontaneously. Most importantly, some of these viral infections can be prevented or treated. For many of these viruses, guidelines for their treatment or prevention have recently been revised. In this review, we outline many of the viruses that cause hearing loss, their epidemiology, course, prevention, and treatment. PMID:25080364

  9. Genetic counseling and prenatal diagnosis for hereditary hearing loss in high-risk families.

    PubMed

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

    2014-08-01

    Genetic counseling and prenatal diagnosis are very necessary and accurate to detect hereditary hearing loss, especially in high-risk families. Prenatal diagnosis is testing for diseases or conditions in fetuses before born, which gives parents the chance to prepare psychologically, financially and medically for the probable health and educational needs of the affected neonates. 54 unrelated families with children affected with non-syndromic sensorineural hearing loss were enrolled in the study and received genetic analysis with microarray and DNA sequencing technologies. Genetic counseling was provided to each participating families, and prenatal diagnosis was given to those at risk and would like to know their fetuses' genotypes and probable hearing statuses. Half the cases in the present study were diagnosed with confirmed pathogenic mutations and clear inheritance patterns. After receiving genetic counseling, 24 carrier couples with pathogenic mutations chose to proceed prenatal diagnosis, the results of which were in accordance with the pregnancy outcomes. Infants prenatally detected to be monoallelic mutation carriers and those harbored neither deafness-causing mutations form their parents passed newborn hearing screening and six-month follow-ups, while neonates prenatally detected to be carriers of diallelic or compound heterozygous mutations developed hearing loss after birth. With appropriate genetic counseling and support services provided, the genetic testing and the prenatal diagnosis of hearing loss were valued by carrier couples for the information provided for future family planning and probably the preparation for the health and educational needs of the affected neonates. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  10. Vowel production of Mandarin-speaking hearing aid users with different types of hearing loss.

    PubMed

    Hung, Yu-Chen; Lee, Ya-Jung; Tsai, Li-Chiun

    2017-01-01

    In contrast with previous research focusing on cochlear implants, this study examined the speech performance of hearing aid users with conductive (n = 11), mixed (n = 10), and sensorineural hearing loss (n = 7) and compared it with the speech of hearing control. Speech intelligibility was evaluated by computing the vowel space area defined by the Mandarin Chinese corner vowels /a, u, i/. The acoustic differences between the vowels were assessed using the Euclidean distance. The results revealed that both the conductive and mixed hearing loss groups exhibited a reduced vowel working space, but no significant difference was found between the sensorineural hearing loss and normal hearing groups. An analysis using the Euclidean distance further showed that the compression of vowel space area in conductive hearing loss can be attributed to the substantial lowering of the second formant of /i/. The differences in vowel production between groups are discussed in terms of the occlusion effect and the signal transmission media of various hearing devices.

  11. Negative consequences of uncorrected hearing loss--a review.

    PubMed

    Arlinger, Stig

    2003-07-01

    Hearing loss gives rise to a number of disabilities. Problems in recognizing speech, especially in difficult environments, give rise to the largest number of complaints. Other kinds of disabilities may concern the reduced ability to detect, identify and localize sounds quickly and reliably. Such sounds may be warning or alarm signals, as well as music and birds singing. The communicative disability affects both hearing-impaired people and other people in their environment--family members, fellow workers, etc. Hearing-impaired people are not always aware of all the consequences of the impairment; they do not always know what they are missing. Several studies have shown that uncorrected hearing loss gives rise to poorer quality of life, related to isolation, reduced social activity, and a feeling of being excluded, leading to an increased prevalence of symptoms of depression. These findings indicate the importance of early identification of hearing loss and offers of rehabilitative support, where the fitting of hearing aids is usually an important component. Several studies also point to a significant correlation between hearing loss and loss of cognitive functions. Most of these studies show such a correlation without being able to show whether the hearing loss caused the reduction in cognitive performance or if both the hearing loss and the cognitive decline are parts of a common, general age-related degeneration. A couple of these studies, however, indicate that the uncorrected hearing loss may be the cause of cognitive decline. Whichever alternative is true, the correlation should be seen as a clear indication for early hearing aid fitting for those needing it. Monaural hearing aid fitting in subjects with bilateral hearing loss may give rise to a reduced ability to recognize speech presented to the unaided ear, the so-called late-onset auditory deprivation effect. This functional decline is reversible in some but not all subjects after fitting of a hearing aid

  12. Prevalence and factors associated with hearing loss and hearing aid use in korean elders.

    PubMed

    Kim, Ji-Su

    2015-03-01

    This study examined hearing loss prevalence and hearing aid usage rates among Korean elders by comparing the differences between those with and without hearing loss, and between those who used and did not use hearing aids. This study was based on data collected during the Korean National Health and Nutrition Examination Survey V (2010-2012). The study sample consisted of 5,447 Koreans aged ≥60 years who received a hearing assessment. Hearing loss was measured using a pure tone audiometry test and classified according to the World Health Organization's criteria. Hearing aid use was assessed by self-report. Multiple logistic regression analyses were performed to determine the associations between hearing loss, hearing aid use, and related variables. Hearing loss was found in 16.8% of the elders and only 15.9% of them used a hearing aid. Male (95% CI: 1.27-2.15), tinnitus (95% CI: 1.58-2.32), dizziness (95% CI: 1.05-1.73), and occupational noise exposure (95% CI: 1.32-2.38) were the variables most strongly associated with hearing loss after multivariate adjustment. Tinnitus (95% CI: 1.34-4.13) and occupational noise exposure (95% CI: 1.01-5.02) were strongly associated with hearing aid use after multivariate adjustment. More than half of South Korean elders aged ≥60 and older have hearing loss but the rate of hearing aid use is very low. An aural public health program should address modifiable risk factors, such as tinnitus and noise exposure, and non-modifiable risk factors associated with hearing loss in the elderly.

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

    PubMed Central

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

    2016-01-01

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

  14. Mouse models of age-related mitochondrial neurosensory hearing loss.

    PubMed

    Han, Chul; Someya, Shinichi

    2013-07-01

    Hearing loss is the most common sensory disorder in the elderly population. Overall, 10% of the population has a hearing loss in the US, and this age-related hearing disorder is projected to afflict more than 28 million Americans by 2030. Age-related hearing loss is associated with loss of sensory hair cells (sensory hearing loss) and/or spiral ganglion neurons (neuronal hearing loss) in the cochlea of the inner ear. Many lines of evidence indicate that oxidative stress and associated mitochondrial dysfunction play a central role in age-related neurodegenerative diseases and are a cause of age-related neurosensory hearing loss. Yet, the molecular mechanisms of how oxidative stress and/or mitochondrial dysfunction lead to hearing loss during aging remain unclear, and currently there is no treatment for this age-dependent disorder. Several mouse models of aging and age-related diseases have been linked to age-related mitochondrial neurosensory hearing loss. Evaluation of these animal models has offered basic knowledge of the mechanism underlying hearing loss associated with oxidative stress, mitochondrial dysfunction, and aging. Here we review the evidence that specific mutations in the mitochondrial DNA or nuclear DNA that affect mitochondrial function result in increased oxidative damage and associated loss of sensory hair cells and/or spiral ganglion neurons in the cochlea during aging, thereby causing hearing loss in these mouse models. Future studies comparing these models will provide further insight into fundamental knowledge about the disordered process of hearing and treatments to improve the lives of individuals with communication disorders. This article is part of a Special Issue entitled 'Mitochondrial function and dysfunction in neurodegeneration'. Copyright © 2012 Elsevier Inc. All rights reserved.

  15. Macrocytosis and sudden sensorineural hearing loss: a case report.

    PubMed

    McMurran, A E L; Adair, R A

    2015-11-01

    Although other blood dyscrasias are known to cause sudden sensorineural hearing loss, macrocytosis has not previously been implicated in the absence of another causative agent. We present a case of bilateral sequential sudden sensorineural hearing loss in a patient with significant macrocytosis (mean corpuscular volume at presentation 124 fl) secondary to alcohol-induced liver dysfunction. A possible pathophysiological mechanism linking macrocytosis and sudden sensorineural hearing loss was identified, suggesting areas for further investigation.

  16. Sensorineural hearing loss and auditory perceptual organization

    NASA Astrophysics Data System (ADS)

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

    2004-05-01

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

  17. Noise-induced hearing loss in Asia.

    PubMed

    Fuente, Adrian; Hickson, Louise

    2011-03-01

    The aim of this manuscript is to summarize the current scenarios encompassing noise exposure in the workplace and the risk of noise-induced hearing loss (NIHL) in Asia. NIHL is the most prevalent and preventable occupational disease in most Asian countries. Sources of noise in these countries include manufacturing and agriculture industries, exploitation of natural resources, and urban traffic. The highest attributable fraction of adult-onset hearing loss resulting from noise exposure in the world comes from Asian countries. NIHL is a serious health problem in Asia, not only because of the number of affected labourers, but also because the majority of Asian countries are still developing economies where access to health services and preventive programmes are limited. Lack of awareness about NIHL among employers, employees, and health care professionals is one of the main barriers for the prevention of NIHL in Asia. In this paper, the sources of noise, NIHL prevalence in different industries, local legislation, and research publications on NIHL from Asia are discussed.

  18. Is Sudden Hearing Loss Associated with Atherosclerosis?

    PubMed Central

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

    2016-01-01

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

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

    PubMed Central

    Iossa, Sandra; Marciano, Elio; Franzé, Annamaria

    2011-01-01

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

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

    PubMed

    Iossa, Sandra; Marciano, Elio; Franzé, Annamaria

    2011-11-01

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

  1. Sudden bilateral sensorineural hearing loss associated with urticarial vasculitis.

    PubMed

    Hall, A C; Leong, A C; Jiang, D; Fitzgerald-O'Connor, A

    2013-07-01

    Bilateral sensorineural hearing loss associated with recurrent urticarial skin lesions may be signs of underlying Muckle-Wells syndrome. Previous reports have described the hearing loss to be progressive in nature. To our knowledge, this paper presents the first published case of sudden onset, bilateral sensorineural hearing loss associated with urticarial vasculitis due to underlying Muckle-Wells syndrome. The patient underwent a cochlear implantation with a modest outcome. Cochlear implantation may help to rehabilitate sudden hearing loss associated with this condition, but early diagnosis may allow treatment with interleukin-1β inhibitors such as anakinra.

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

    PubMed

    Page, Joshua Cody; Peters, Bob

    2015-01-01

    Sudden sensorineural hearing loss following non-otologic surgery is a rare event described in the medical literature. Cardiopulmonary bypass surgery is most commonly associated with this type of hearing loss. Our case report and review of the literature describe two cases with postoperative hearing loss - neither of which are cardiac surgeries - making them exceedingly rare in the medical literature. Regardless of the rarity of this unfortunate event, the possibility for permanent hearing loss is a potentially devastating unanticipated complication and one that all surgeons should be aware. Copyright © 2015 Elsevier Inc. All rights reserved.

  3. Sudden hearing loss following non-petrous craniotomy.

    PubMed

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

    2010-01-01

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

  4. Noise Exposure and Hearing Loss in Rural Children.

    ERIC Educational Resources Information Center

    Woodford, Charles M.; Lass, Norman J.

    1993-01-01

    A high level of work-related and recreational noise has led to a high prevalence of noise-induced hearing loss in rural students. Teachers can help prevent this problem by integrating hearing conservation education with existing curricula. Educators could be trained about hearing conservation by professional audiologists. (LP)

  5. Deafness and Hearing Loss. NICHCY Disability Fact Sheet #3

    ERIC Educational Resources Information Center

    National Dissemination Center for Children with Disabilities, 2010

    2010-01-01

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

  6. Bringing Text Display Digital Radio to Consumers with Hearing Loss

    ERIC Educational Resources Information Center

    Sheffield, Ellyn G.; Starling, Michael; Schwab, Daniel

    2011-01-01

    Radio is migrating to digital transmission, expanding its offerings to include captioning for individuals with hearing loss. Text display radio requires a large amount of word throughput with minimal screen display area, making good user interface design crucial to its success. In two experiments, we presented hearing, hard-of-hearing, and deaf…

  7. Social Engagement, Attention and Competence of Preschoolers with Hearing Loss

    ERIC Educational Resources Information Center

    Brown, P. Margaret; Bortoli, Anna; Remine, Maria D.; Othman, Basyariatul

    2008-01-01

    The social engagement, social attention skills and social competence of 10 hearing preschoolers and 10 preschoolers with hearing loss were investigated during free play in inclusive oral kindergarten settings using a three-level hierarchical model. When comparing the types of opportunities, at the first level, the children with hearing loss…

  8. Social Engagement, Attention and Competence of Preschoolers with Hearing Loss

    ERIC Educational Resources Information Center

    Brown, P. Margaret; Bortoli, Anna; Remine, Maria D.; Othman, Basyariatul

    2008-01-01

    The social engagement, social attention skills and social competence of 10 hearing preschoolers and 10 preschoolers with hearing loss were investigated during free play in inclusive oral kindergarten settings using a three-level hierarchical model. When comparing the types of opportunities, at the first level, the children with hearing loss…

  9. 77 FR 15003 - Passive Activity Losses and Credits Limited; Hearing

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-03-14

    ... Internal Revenue Service 26 CFR Part 1 RIN 1545-BJ33 Passive Activity Losses and Credits Limited; Hearing AGENCY: Internal Revenue Service (IRS), Treasury. ] ACTION: Notice of public hearing on proposed rulemaking. SUMMARY: This document provides notice of public hearing on proposed rulemaking regarding the...

  10. 76 FR 31543 - Controlled Groups; Deferral of Losses; Hearing

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-06-01

    ... Internal Revenue Service 26 CFR Part 1 RIN 1545-BI92 Controlled Groups; Deferral of Losses; Hearing AGENCY: Internal Revenue Service (IRS), Treasury. ACTION: Notice of public hearing on proposed rulemaking. SUMMARY: This document provides notice of public hearing on a notice of proposed rulemaking providing guidance...

  11. Including Children with Hearing Loss in Early Childhood Programs

    ERIC Educational Resources Information Center

    Katz, Laurie; Schery, Teris K.

    2006-01-01

    These are typical scenarios of children with hearing loss who are being included increasingly in early childhood settings. Recent federal legislation encourages states to develop programs to screen the hearing of all infants before they leave the hospital, and currently 39 states have adopted newborn infant hearing screening mandates (ASHA 2005).…

  12. Bringing Text Display Digital Radio to Consumers with Hearing Loss

    ERIC Educational Resources Information Center

    Sheffield, Ellyn G.; Starling, Michael; Schwab, Daniel

    2011-01-01

    Radio is migrating to digital transmission, expanding its offerings to include captioning for individuals with hearing loss. Text display radio requires a large amount of word throughput with minimal screen display area, making good user interface design crucial to its success. In two experiments, we presented hearing, hard-of-hearing, and deaf…

  13. Including Children with Hearing Loss in Early Childhood Programs

    ERIC Educational Resources Information Center

    Katz, Laurie; Schery, Teris K.

    2006-01-01

    These are typical scenarios of children with hearing loss who are being included increasingly in early childhood settings. Recent federal legislation encourages states to develop programs to screen the hearing of all infants before they leave the hospital, and currently 39 states have adopted newborn infant hearing screening mandates (ASHA 2005).…

  14. Immersive simulation of hearing loss and auditory prostheses

    NASA Astrophysics Data System (ADS)

    Zurek, Patrick M.; Desloge, Joseph G.

    2001-05-01

    Simulation of hearing loss is useful for demonstrating the communication challenges facing hearing-impaired people. However, current simulations, most of which are only recordings, do not actually elevate thresholds; i.e., they do not simulate hearing loss, per se. The hearing loss simulator described in this talk is immersive; the user's detection thresh- olds for ambient sounds are shifted by a prescribed degree. This threshold shift is achieved through a combination of passive attenuation (from muff-type hearing protectors) and additive masking noise (introduced by within-muff earphones). Acoustic signals picked up by microphones near each ear are processed through bandpass AGC channels and delivered via the earphones to complete the simulation of frequency-dependent hearing loss and loudness recruitment. Preliminary results validating the accuracy of specified threshold shift will be presented, along with speech-reception data comparing simulated with actual hearing losses. Subjective reactions of users engaged in one-on-one conversation suggest that strong feelings of communication disability are engendered by even moderate degrees of simulated hearing loss. The system, which is capable of simulating any degree of recruiting hearing loss along with hearing aids or cochlear implants, can provide effective interactive demonstrations of both auditory communication handicap and rehabilitation options. [Work supported by NIDCD.

  15. Immersive simulation of hearing loss and auditory prostheses

    NASA Astrophysics Data System (ADS)

    Zurek, Patrick M.; Desloge, Joseph G.

    2004-05-01

    Simulation of hearing loss is useful for demonstrating the communication challenges facing hearing-impaired people. However, current simulations, most of which are only recordings, do not actually elevate thresholds; i.e., they do not simulate hearing loss, per se. The hearing loss simulator described in this talk is immersive; the user's detection thresh- olds for ambient sounds are shifted by a prescribed degree. This threshold shift is achieved through a combination of passive attenuation (from muff-type hearing protectors) and additive masking noise (introduced by within-muff earphones). Acoustic signals picked up by microphones near each ear are processed through bandpass AGC channels and delivered via the earphones to complete the simulation of frequency-dependent hearing loss and loudness recruitment. Preliminary results validating the accuracy of specified threshold shift will be presented, along with speech-reception data comparing simulated with actual hearing losses. Subjective reactions of users engaged in one-on-one conversation suggest that strong feelings of communication disability are engendered by even moderate degrees of simulated hearing loss. The system, which is capable of simulating any degree of recruiting hearing loss along with hearing aids or cochlear implants, can provide effective interactive demonstrations of both auditory communication handicap and rehabilitation options. [Work supported by NIDCD.

  16. Prevalence of minimal hearing loss in South Korea.

    PubMed

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

    2017-01-01

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

  17. Prevalence of minimal hearing loss in South Korea

    PubMed Central

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

    2017-01-01

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

  18. [Universal hearing screening in newborns -- recommendations for organizing and conducting universal hearing screening for congenital hearing loss in Germany].

    PubMed

    Gross, M

    2005-11-01

    The Interdisciplinary Consensus Conference for Newborn Hearing Screening (IKKNHS) has worked out joint recommendations for universal hearing screening of newborns. In the consensus paper, 11 professional associations and scientific societies in the fields of gynecology and obstetrics, ENT, pediatrics, and phoniatrics and pedaudiology came to an agreement how to implement newborn hearing screening in Germany. The paper deals with the following topics: goals of universal newborn hearing screening, target group of hearing screening, schedule for screening, personnel involved in the screening program, technologies and framework conditions of hearing screening, documentation, continuous quality control of screening, confirmation diagnostics for conspicuous test subjects, motivation to take part in screening, information on newborn hearing screening, tracking, various infrastructural situations in urban and rural regions, follow-up care, in-patient vs. out-patient screening, cost factors of screening, reporting children with permanent hearing loss to the German Central Registry for hearing loss in children.

  19. Differential effects of pannexins on noise-induced hearing loss.

    PubMed

    Abitbol, Julia M; Kelly, John J; Barr, Kevin; Schormans, Ashley L; Laird, Dale W; Allman, Brian L

    2016-12-15

    Hearing loss, including noise-induced hearing loss, is highly prevalent and severely hinders an individual's quality of life, yet many of the mechanisms that cause hearing loss are unknown. The pannexin (Panx) channel proteins, Panx1 and Panx3, are regionally expressed in many cell types along the auditory pathway, and mice lacking Panx1 in specific cells of the inner ear exhibit hearing loss, suggesting a vital role for Panxs in hearing. We proposed that Panx1 and/or Panx3 null mice would exhibit severe hearing loss and increased susceptibility to noise-induced hearing loss. Using the auditory brainstem response, we surprisingly found that Panx1(-/-) and Panx3(-/-) mice did not harbor hearing or cochlear nerve deficits. Furthermore, while Panx1(-/-) mice displayed no protection against loud noise-induced hearing loss, Panx3(-/-) mice exhibited enhanced 16- and 24-kHz hearing recovery 7 days after a loud noise exposure (NE; 12 kHz tone, 115 dB sound pressure level, 1 h). Interestingly, Cx26, Cx30, Cx43, and Panx2 were up-regulated in Panx3(-/-) mice compared with wild-type and/or Panx1(-/-) mice, and assessment of the auditory tract revealed morphological changes in the middle ear bones of Panx3(-/-) mice. It is unclear if these changes alone are sufficient to provide protection against loud noise-induced hearing loss. Contrary to what we expected, these data suggest that Panx1 and Panx3 are not essential for baseline hearing in mice tested, but the therapeutic targeting of Panx3 may prove protective against mid-high-frequency hearing loss caused by loud NE.

  20. Communication Assessment and Intervention: Implications for Pediatric Hearing Loss.

    PubMed

    Bobsin, Lori L; Houston, K Todd

    2015-12-01

    Historically, children with hearing loss have fallen well behind their hearing peers in the areas of speech and language development, which has often limited their participation in a range of social, educational, and vocational activities. However, with early identification and appropriate intervention coupled with current hearing technology, children with hearing loss can achieve speech and language milestones at rates commensurate with hearing peers. To attain the best outcomes for these children, an early intervention system that provides thorough and unbiased information to families and allows for the efficient and coordinated efforts of qualified professionals must be present. Copyright © 2015 Elsevier Inc. All rights reserved.

  1. 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

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

    PubMed

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

    2015-01-01

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

  3. Hearing loss and tinnitus in adolescents and young adults

    NASA Astrophysics Data System (ADS)

    Holmes, Alice

    2004-05-01

    Little attention has been paid to hearing abilities and the effects of noise on the normal adolescent and young adult population. A series of studies will be presented on the prevalence of hearing loss and reported effects of hearing loss and tinnitus in adolescents and young adults from different cultural backgrounds. Adolescents and young adults from different backgrounds may tend to seek or avoid various noise environments that could be detrimental to their hearing and cause tinnitus. Attitudes and exposures to noise environments were evaluated to see if these may be correlated with their hearing losses and/or tinnitus. In addition, these adolescent and young adult subjects reported how often they used hearing protection in various noise environments. Finally, the issues of quality of life and the need for hearing conservation programs with these populations will be presented.

  4. A Mutation in Synaptojanin 2 Causes Progressive Hearing Loss in the ENU-Mutagenised Mouse Strain Mozart

    PubMed Central

    Manji, Shehnaaz S. M.; Williams, Louise H.; Miller, Kerry A.; Ooms, Lisa M.; Bahlo, Melanie; Mitchell, Christina A.; Dahl, Hans-Henrik M.

    2011-01-01

    Background Hearing impairment is the most common sensory impairment in humans, affecting 1∶1,000 births. We have identified an ENU generated mouse mutant, Mozart, with recessively inherited, non-syndromic progressive hearing loss caused by a mutation in the synaptojanin 2 (Synj2), a central regulatory enzyme in the phosphoinositide-signaling cascade. Methodology/Principal Findings The hearing loss in Mozart is caused by a p.Asn538Lys mutation in the catalytic domain of the inositol polyphosphate 5-phosphatase synaptojanin 2. Within the cochlea, Synj2 mRNA expression was detected in the inner and outer hair cells but not in the spiral ganglion. Synj2N538K mutant protein showed loss of lipid phosphatase activity, and was unable to degrade phosphoinositide signaling molecules. Mutant Mozart mice (Synj2N538K/N538K) exhibited progressive hearing loss and showed signs of hair cell degeneration as early as two weeks of age, with fusion of stereocilia followed by complete loss of hair bundles and ultimately loss of hair cells. No changes in vestibular or neurological function, or other clinical or behavioral manifestations were apparent. Conclusions/Significance Phosphoinositides are membrane associated signaling molecules that regulate many cellular processes including cell death, proliferation, actin polymerization and ion channel activity. These results reveal Synj2 as a critical regulator of hair cell survival that is essential for hair cell maintenance and hearing function. PMID:21423608

  5. A mutation in synaptojanin 2 causes progressive hearing loss in the ENU-mutagenised mouse strain Mozart.

    PubMed

    Manji, Shehnaaz S M; Williams, Louise H; Miller, Kerry A; Ooms, Lisa M; Bahlo, Melanie; Mitchell, Christina A; Dahl, Hans-Henrik M

    2011-03-15

    Hearing impairment is the most common sensory impairment in humans, affecting 1:1,000 births. We have identified an ENU generated mouse mutant, Mozart, with recessively inherited, non-syndromic progressive hearing loss caused by a mutation in the synaptojanin 2 (Synj2), a central regulatory enzyme in the phosphoinositide-signaling cascade. The hearing loss in Mozart is caused by a p.Asn538Lys mutation in the catalytic domain of the inositol polyphosphate 5-phosphatase synaptojanin 2. Within the cochlea, Synj2 mRNA expression was detected in the inner and outer hair cells but not in the spiral ganglion. Synj2(N538K) mutant protein showed loss of lipid phosphatase activity, and was unable to degrade phosphoinositide signaling molecules. Mutant Mozart mice (Synj2(N538K/N538K)) exhibited progressive hearing loss and showed signs of hair cell degeneration as early as two weeks of age, with fusion of stereocilia followed by complete loss of hair bundles and ultimately loss of hair cells. No changes in vestibular or neurological function, or other clinical or behavioral manifestations were apparent. Phosphoinositides are membrane associated signaling molecules that regulate many cellular processes including cell death, proliferation, actin polymerization and ion channel activity. These results reveal Synj2 as a critical regulator of hair cell survival that is essential for hair cell maintenance and hearing function.

  6. Characteristics of Patients with Hearing Aids according to the Degree and Pattern of Hearing Loss

    PubMed Central

    Byun, Young Seok; Kim, Sung Su; Park, Sang Hyun; Park, Eun Bin; Kim, Ho Joong; Kim, Sang Hoon

    2016-01-01

    Background and Objectives This study was designed to assess the characteristics of patients according to the degree and audiogram shape of hearing loss and the association of these characteristics with hearing aids (HA) choice, return rate, and cause of return. Subjects and Methods This study included 460 individuals who received HAs from 2011 to 2015. The relationships between type of HA and age, primary and accompanying symptoms, HA choice and return and cause of return were evaluated according to the degree and pattern of hearing loss. Results HA type did not differ significantly according to the degree and pattern of hearing loss. Intensity of hearing loss was greater in male than in female (p<0.05). Open and completely-in-canal types of HA decreased with age (p<0.05). As degree of hearing loss intensified, behind-the-ear and in-the-ear types increased and Open type decreased (p<0.05). The HA return rate was 9.7%, but was not associated with degree or pattern of hearing loss. The main causes of HA return were costs, psychological fears and adaptive failure. Conclusions Choice of HA is affected by age, sex, and degree and pattern of hearing loss. HA for hearing rehabilitation in patients with hearing loss can be personalized according to each patient's characteristics and tendencies. PMID:27942600

  7. Prediction of hearing outcomes by multiple regression analysis in patients with idiopathic sudden sensorineural hearing loss.

    PubMed

    Suzuki, Hideaki; Tabata, Takahisa; Koizumi, Hiroki; Hohchi, Nobusuke; Takeuchi, Shoko; Kitamura, Takuro; Fujino, Yoshihisa; Ohbuchi, Toyoaki

    2014-12-01

    This study aimed to create a multiple regression model for predicting hearing outcomes of idiopathic sudden sensorineural hearing loss (ISSNHL). The participants were 205 consecutive patients (205 ears) with ISSNHL (hearing level ≥ 40 dB, interval between onset and treatment ≤ 30 days). They received systemic steroid administration combined with intratympanic steroid injection. Data were examined by simple and multiple regression analyses. Three hearing indices (percentage hearing improvement, hearing gain, and posttreatment hearing level [HLpost]) and 7 prognostic factors (age, days from onset to treatment, initial hearing level, initial hearing level at low frequencies, initial hearing level at high frequencies, presence of vertigo, and contralateral hearing level) were included in the multiple regression analysis as dependent and explanatory variables, respectively. In the simple regression analysis, the percentage hearing improvement, hearing gain, and HLpost showed significant correlation with 2, 5, and 6 of the 7 prognostic factors, respectively. The multiple correlation coefficients were 0.396, 0.503, and 0.714 for the percentage hearing improvement, hearing gain, and HLpost, respectively. Predicted values of HLpost calculated by the multiple regression equation were reliable with 70% probability with a 40-dB-width prediction interval. Prediction of HLpost by the multiple regression model may be useful to estimate the hearing prognosis of ISSNHL. © The Author(s) 2014.

  8. 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

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

    PubMed

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

    2017-02-01

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

  10. Associated handicaps in children with hearing loss.

    PubMed

    Voutilainen, R; Jauhiainen, T; Linkola, H

    1988-01-01

    In the differential diagnosis as well as in the rehabilitation of hearing impaired children other disorders affecting language aquisition and speech development need to be taken into account. The rehabilitation programme is highly dependent on the early diagnosis of these additional disorders such as dysphasia, mental retardation of various degrees, cognitive disorders such as dyslexia and dysgraphia, dyspractic and dysarthric disorders of speech production, cleft palate and other anomalies of articulatory organs, autism and other abnormal features of psychic and personality development. In addition children with multiple disorders like malformations, visual disorders, epilepsy, CP and other diseases and handicaps, even though they may not influence language and speech development directly, may still be deprived of possibilities to aquire adequate verbal stimulation. The paper presents a material of 200 children whose hearing loss was diagnosed at the preschool age. Major associated handicaps were found in 35.5% of cases and in 26% they were complicating rehabilitation and development of the child. The frequency of associated disorders and their effect on language and speech development, learning ability and social development is being more closely analysed and discussed.

  11. Mechanisms and Treatment of Blast Induced Hearing Loss

    PubMed Central

    2012-01-01

    The main objective of this study is to provide an overview of the basic mechanisms of blast induced hearing loss and review pharmacological treatments or interventions that can reduce or inhibit blast induced hearing loss. The mechanisms of blast induced hearing loss have been studied in experimental animal models mimicking features of damage or injury seen in human. Blast induced hearing loss is characterized by perforation and rupture of the tympanic membrane, ossicular damage, basilar membrane damage, inner and outer hair cell loss, rupture of round window, changes in chemical components of cochlear fluid, vasospasm, ischemia, oxidative stress, excitotoxicity, hematoma, and hemorrhage in both animals and humans. These histopathological consequences of blast exposure can induce hearing loss, tinnitus, dizziness, and headache. The pharmacological approaches to block or inhibit some of the auditory pathological consequences caused by blast exposure have been developed with antioxidant drugs such as 2,4-disulfonyl α-phenyl tertiary butyl nitrone (HXY-059, now called HPN-07) and N-acetylcysteine (NAC). A combination of antioxidant drugs (HPN-07 and NAC) was administered to reduce blast induced cochlear damage and hearing loss. The combination of the antioxidant drugs can prevent or treat blast induced hearing loss by reducing damage to the mechanical and neural component of the auditory system. Although information of the underlying mechanisms and treatment of blast induced hearing loss are provided, further and deep research should be achieved due to the limited and controversial knowledge. PMID:24653882

  12. 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

  13. Towards an Etiologic Diagnosis: Assessing the Patient with Hearing Loss

    PubMed Central

    Lin, Jerry; Oghalai, John S.

    2013-01-01

    This article reviews the clinical approach taken towards identification of the cause of hearing loss in children. A brief overview of the universal newborn hearing screening program is presented. Discussion is then focused on clinical elements of the diagnostic process with emphasis on the importance of the history, physical examination, and audiologic testing. The utility and appropriateness of additional diagnostic testing is considered, particularly with regards to the incorporation of diagnostic radiologic imaging and genetic testing. In the course of these discussions, the genetic and non- genetic causes of pediatric hearing loss are reviewed. Finally, the implications of a definitive identification of hearing loss etiology are considered. PMID:21358182

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

    ERIC Educational Resources Information Center

    Most, Tova; Shina-August, Ella; Meilijson, Sara

    2010-01-01

    This study characterized the profile of pragmatic abilities among 24 children with hearing loss (HL) aged 6.3-9.4 years, 13 using hearing aids (HAs) and 11 using cochlear implants (CIs), in comparison to those of 13 hearing children with similar chronological and language ages. All the children with HL used spoken language, attended regular…

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

    ERIC Educational Resources Information Center

    Most, Tova; Shina-August, Ella; Meilijson, Sara

    2010-01-01

    This study characterized the profile of pragmatic abilities among 24 children with hearing loss (HL) aged 6.3-9.4 years, 13 using hearing aids (HAs) and 11 using cochlear implants (CIs), in comparison to those of 13 hearing children with similar chronological and language ages. All the children with HL used spoken language, attended regular…

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

    PubMed

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

    2014-06-01

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

  17. Hearing Loss following Posterior Fossa Microvascular Decompression: A Systematic Review.

    PubMed

    Bartindale, Matthew; Kircher, Matthew; Adams, William; Balasubramanian, Neelam; Liles, Jeffrey; Bell, Jason; Leonetti, John

    2017-09-01

    Objectives (1) Determine the prevalence of hearing loss following microvascular decompression (MVD) for trigeminal neuralgia (TN) and hemifacial spasm (HFS). (2) Demonstrate factors that affect postoperative hearing outcomes after MVD. Data Sources PubMed-NCBI, Scopus, CINAHL, and PsycINFO databases from 1981 to 2016. Review Methods Systematic review of prospective cohort studies and retrospective reviews in which any type of hearing loss was recorded after MVD for TN or HFS. Three researchers extracted data regarding operative indications, procedures performed, and diagnostic tests employed. Discrepancies were resolved by mutual consensus. Results Sixty-nine references with 18,233 operations met inclusion criteria. There were 7093 patients treated for TN and 11,140 for HFS. The overall reported prevalence of hearing loss after MVD for TN and HFS was 5.58% and 8.25%, respectively. However, many of these studies relied on subjective measures of reporting hearing loss. In 23 studies with consistent perioperative audiograms, prevalence of hearing loss was 13.47% for TN and 13.39% for HFS, with no significant difference between indications ( P = .95). Studies using intraoperative brainstem auditory evoked potential monitoring were more likely to report hearing loss for TN (relative risk [RR], 2.28; P < .001) but not with HFS (RR, 0.88; P = .056). Conclusion Conductive and sensorineural hearing loss are important complications following posterior fossa MVD. Many studies have reported on hearing loss using either subjective measures and/or inconsistent audiometric testing. Routine perioperative audiogram protocols improve the detection of hearing loss and may more accurately represent the true risk of hearing loss after MVD for TN and HFS.

  18. Validity of the National Acoustic Laboratories procedure for determining percentage loss of hearing.

    PubMed

    Macrae, John H

    2012-12-01

    To evaluate the validity of the National Acoustic Laboratories procedure for determining percentage loss of hearing as a measure of hearing disability. The percentage hearing losses of war veterans who had hearing ranging from normal to profound deafness were determined and compared with their scores on two hearing questionnaires. A self-report hearing questionnaire was completed by 282 war veterans and 154 of those veterans were given the hearing measurement scale in the form of a structured interview. A percentage loss of hearing of 0 agreed well with the questionnaire scores representing the limit of normal hearing, and a percentage loss of hearing of 100 agreed well with the questionnaire scores representing total loss of hearing. Percentage loss of hearing accounted for 83% of the variance in scores on the hearing questionnaire and 81% of the variance in scores on the hearing measurement scale. The National Acoustic Laboratories procedure for determining percentage loss of hearing provides a valid measure of hearing disability.

  19. Noise-related hearing loss can be avoided

    SciTech Connect

    Istre, C.O. Jr.

    1982-03-01

    The physiological changes in the auditory organ are described with the protective equipment available for prevention of occupationally related hearing losses. Since most affected employees are unaware of the hearing loss until the damage is irreversible the author stresses that protective equipment usage must be mandatory for supervisory as well as active workers. Mechanical movement, combustion, and high pressure venting are all listed as detrimental to human hearing. (PSB)

  20. 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.

  1. Attention Deficits and Hearing Loss: Meeting the Challenge

    ERIC Educational Resources Information Center

    O'Connell, Joanne; Casale, Kathleen

    2004-01-01

    This article describes attention deficit hyperactivity disorder (ADHD) and examines the criteria that may create a challenge when diagnosing attention deficits in students with hearing loss. Guidelines for differential diagnosis of ADHD in conjunction with hearing loss are provided. A variety of practical intervention strategies for educators and…

  2. Call centers and noise-induced hearing loss

    PubMed Central

    Beyan, Ayse Coskun; Demiral, Yucel; Cimrin, Arif Hikmet; Ergor, Alparslan

    2016-01-01

    Noise is defined as unwelcome sound. It has been estimated that 16% of adult hearing loss in the world is due to noise exposure at the workplace. This report offers a case that diagnosed with hearing loss of whom working as a call center operator at home. Home agent operators should be explored. PMID:26960789

  3. Effective Identification of Functional Hearing Loss Using Behavioral Threshold Measures

    ERIC Educational Resources Information Center

    Schlauch, Robert S.; Koerner, Tess K.; Marshall, Lynne

    2015-01-01

    Purpose: Four functional hearing loss protocols were evaluated. Method: For each protocol, 30 participants feigned a hearing loss first on an audiogram and then for a screening test that began a threshold search from extreme levels (-10 or 90 dB HL). Two-tone and 3-tone protocols compared thresholds for ascending and descending tones for 2 (0.5…

  4. Mild and Unilateral Hearing Loss: Implications for Early Intervention

    ERIC Educational Resources Information Center

    Holstrum, W. June; Biernath, Krista; McKay, Sarah; Ross, Danielle S.

    2009-01-01

    Newborn hearing screening has become a standard practice in most birthing hospitals in the United States. Historically, the primary target for the identification of hearing loss has been infants with permanent bilateral loss of moderate degree or greater (i.e., greater than 40 dB). However, research indicates that without early identification and…

  5. Effective Identification of Functional Hearing Loss Using Behavioral Threshold Measures

    ERIC Educational Resources Information Center

    Schlauch, Robert S.; Koerner, Tess K.; Marshall, Lynne

    2015-01-01

    Purpose: Four functional hearing loss protocols were evaluated. Method: For each protocol, 30 participants feigned a hearing loss first on an audiogram and then for a screening test that began a threshold search from extreme levels (-10 or 90 dB HL). Two-tone and 3-tone protocols compared thresholds for ascending and descending tones for 2 (0.5…

  6. Educational Implications of Conductive Hearing Loss in School Children.

    ERIC Educational Resources Information Center

    Lyon, David J.; And Others

    1986-01-01

    The study investigated specific linguistic abilities/disabilities of 15 children with conductive hearing loss and a history of middle ear dysfunction. Results found significant deficits in verbal intelligence, word recognition, and receptive syntactic skills substantiating the finding that conductive hearing loss due to otitis media is deleterious…

  7. Hearing loss in biotinidase deficiency: genotype-phenotype correlation.

    PubMed

    Sivri, Hatice Serap Kalkanoğlu; Genç, Gülsüm Aydan; Tokatli, Ayşegül; Tokatlý, Ayşegül; Dursun, Ali; Coşkun, Turgay; Aydin, Halil Ibrahim; Aydýn, Halil Ybrahim; Sennaroğlu, Levent; Belgin, Erol; Jensen, Kevin; Wolf, Barry

    2007-04-01

    Children with symptoms of profound biotinidase deficiency with null mutations are more likely to have hearing loss develop than those with missense mutations, even if not treated for a period of time. Hearing loss appears to be preventable in children with null mutations if treatment is initiated soon after birth.

  8. Teaching Vocabulary to Preschool Children with Hearing Loss

    ERIC Educational Resources Information Center

    Lund, Emily; Douglas, W. Michael

    2016-01-01

    Despite poor vocabulary outcomes for children with hearing loss, few studies have evaluated the effectiveness of specific vocabulary teaching methods on vocabulary learning for this group. The authors compared three vocabulary instruction conditions with preschool children with hearing loss: (a) explicit, direct instruction; (b) follow-in…

  9. Abnormal facial appearance and delayed diagnosis of congenital hearing loss.

    PubMed

    Crysdale, W S

    1978-08-01

    Congenital hearing loss occurs in association with cranio-facial anomalies. Lay people and health professionals as well frequently regard individuals with cranio-facial anomalies as "stupid" or of lower than normal intelligence because of their odd appearance. Two case reports illustrate that this erroneous assumption will result in the delayed detection of significant hearing loss.

  10. Educational Implications of Conductive Hearing Loss in School Children.

    ERIC Educational Resources Information Center

    Lyon, David J.; And Others

    1986-01-01

    The study investigated specific linguistic abilities/disabilities of 15 children with conductive hearing loss and a history of middle ear dysfunction. Results found significant deficits in verbal intelligence, word recognition, and receptive syntactic skills substantiating the finding that conductive hearing loss due to otitis media is deleterious…

  11. Call centers and noise-induced hearing loss.

    PubMed

    Beyan, Ayse Coskun; Demiral, Yucel; Cimrin, Arif Hikmet; Ergor, Alparslan

    2016-01-01

    Noise is defined as unwelcome sound. It has been estimated that 16% of adult hearing loss in the world is due to noise exposure at the workplace. This report offers a case that diagnosed with hearing loss of whom working as a call center operator at home. Home agent operators should be explored.

  12. Transient Hearing Loss in Adults Associated with Zika Virus Infection.

    PubMed

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

    2016-12-07

    In 2015, during the outbreak of ZIKAV in Brazil, we identified three cases of acute hearing loss after exanthematous illness. Serology yielded finding compatible with ZIKAV as the cause of a confirmed (1patient) and a probable (2 patients) flavivirus infection, indicating an association between ZIKAV infection and transient hearing loss.

  13. Teaching Vocabulary to Preschool Children with Hearing Loss

    ERIC Educational Resources Information Center

    Lund, Emily; Douglas, W. Michael

    2016-01-01

    Despite poor vocabulary outcomes for children with hearing loss, few studies have evaluated the effectiveness of specific vocabulary teaching methods on vocabulary learning for this group. The authors compared three vocabulary instruction conditions with preschool children with hearing loss: (a) explicit, direct instruction; (b) follow-in…

  14. Mild and Unilateral Hearing Loss: Implications for Early Intervention

    ERIC Educational Resources Information Center

    Holstrum, W. June; Biernath, Krista; McKay, Sarah; Ross, Danielle S.

    2009-01-01

    Newborn hearing screening has become a standard practice in most birthing hospitals in the United States. Historically, the primary target for the identification of hearing loss has been infants with permanent bilateral loss of moderate degree or greater (i.e., greater than 40 dB). However, research indicates that without early identification and…

  15. Noise Induced Hearing Loss among School Band Directors.

    ERIC Educational Resources Information Center

    Cutietta, Robert A.; And Others

    1989-01-01

    Reports on a study that examined noise-induced hearing loss (NIHL) among school band directors. Finds that 41 percent of band directors in the sample had characteristic NIHL curves indicating that their experience as band directors played a role in the hearing loss. Recommends further study into the causes of NIHL among school band directors. (LS)

  16. Hearing loss and cognition: the role of hearing AIDS, social isolation and depression.

    PubMed

    Dawes, Piers; Emsley, Richard; Cruickshanks, Karen J; Moore, David R; Fortnum, Heather; Edmondson-Jones, Mark; McCormack, Abby; Munro, Kevin J

    2015-01-01

    Hearing loss is associated with poor cognitive performance and incident dementia and may contribute to cognitive decline. Treating hearing loss with hearing aids may ameliorate cognitive decline. The purpose of this study was to test whether use of hearing aids was associated with better cognitive performance, and if this relationship was mediated via social isolation and/or depression. Structural equation modelling of associations between hearing loss, cognitive performance, social isolation, depression and hearing aid use was carried out with a subsample of the UK Biobank data set (n = 164,770) of UK adults aged 40 to 69 years who completed a hearing test. Age, sex, general health and socioeconomic status were controlled for as potential confounders. Hearing aid use was associated with better cognition, independently of social isolation and depression. This finding was consistent with the hypothesis that hearing aids may improve cognitive performance, although if hearing aids do have a positive effect on cognition it is not likely to be via reduction of the adverse effects of hearing loss on social isolation or depression. We suggest that any positive effects of hearing aid use on cognition may be via improvement in audibility or associated increases in self-efficacy. Alternatively, positive associations between hearing aid use and cognition may be accounted for by more cognitively able people seeking and using hearing aids. Further research is required to determine the direction of association, if there is any direct causal relationship between hearing aid use and better cognition, and whether hearing aid use results in reduction in rates of cognitive decline measured longitudinally.

  17. Hearing Loss and Cognition: The Role of Hearing Aids, Social Isolation and Depression

    PubMed Central

    Dawes, Piers; Emsley, Richard; Cruickshanks, Karen J.; Moore, David R.; Fortnum, Heather; Edmondson-Jones, Mark; McCormack, Abby; Munro, Kevin J.

    2015-01-01

    Hearing loss is associated with poor cognitive performance and incident dementia and may contribute to cognitive decline. Treating hearing loss with hearing aids may ameliorate cognitive decline. The purpose of this study was to test whether use of hearing aids was associated with better cognitive performance, and if this relationship was mediated via social isolation and/or depression. Structural equation modelling of associations between hearing loss, cognitive performance, social isolation, depression and hearing aid use was carried out with a subsample of the UK Biobank data set (n = 164,770) of UK adults aged 40 to 69 years who completed a hearing test. Age, sex, general health and socioeconomic status were controlled for as potential confounders. Hearing aid use was associated with better cognition, independently of social isolation and depression. This finding was consistent with the hypothesis that hearing aids may improve cognitive performance, although if hearing aids do have a positive effect on cognition it is not likely to be via reduction of the adverse effects of hearing loss on social isolation or depression. We suggest that any positive effects of hearing aid use on cognition may be via improvement in audibility or associated increases in self-efficacy. Alternatively, positive associations between hearing aid use and cognition may be accounted for by more cognitively able people seeking and using hearing aids. Further research is required to determine the direction of association, if there is any direct causal relationship between hearing aid use and better cognition, and whether hearing aid use results in reduction in rates of cognitive decline measured longitudinally. PMID:25760329

  18. 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

  19. 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

  20. 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

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

    PubMed

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

    2014-01-01

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

  2. Sensorineural hearing loss in pediatric patients with celiac disease.

    PubMed

    Urganci, Nafiye; Kalyoncu, Derya; Calis, Asli Batur

    2015-09-01

    Sensorineural hearing loss (SNHL) as an extraintestinal manifestation of celiac disease (CD) has been reported in several studies. The aim of this study was to determine presence of subclinical sensorineural hearing loss associated with CD in pediatric patients. Otoscopy, tympanometry and pure tone audiometry were performed in 44 patients with CD and 20 healthy age and sex-matched controls. Pure tone audiometry did not show significant sensorineural hearing loss over all frequencies in patients with CD compared with controls (P > 0.05). SNHL was detected in only three (6.8 %) patients with CD. In conclusion, subclinical sensorineural hearing loss was demonstrated in adult patients with CD; therefore, we recommend to perform audiometric examinations in pediatric patients for recognizing hearing loss early during the course of the disease.

  3. A Clinical Analysis of Sudden Sensorineural Hearing Loss Cases

    PubMed Central

    Lee, Hyun Soo; Lee, You Jae; Kang, Bo Sung; Lee, Ji Sung

    2014-01-01

    Background and Objectives High-dose systemic steroid therapy is the mainstay treatment for sudden sensorineural hearing loss (SSNHL). Recovery rates from SSNHL range are about 47-63% and are influenced by various prognostic factors. To evaluate the prognostic value of specific clinical parameters, we reviewed 289 cases by clinical and statistical analysis. Subjects and Methods This study included 289 patients with SSNHL who visited the Department of Otolaryngology at Soonchunhyang University Hospital from January 2005 to December 2012. The cases were reviewed retrospectively based on clinical charts. Hearing improvement was evaluated in relation to pure-tone audiogram results, duration between SSNHL onset and time of initial treatment, seasonal incidence, dizziness, patient age, degree of hearing loss, patterns of initial pure-tone audiogram and presence of underlying disease. Results Hearing improvement was observed in 196 of 289 (67.8%) patients; such improvement began within 7 days in most patients, followed by rapid hearing recovery. Cases that failed to show improvement within 14 days were unlikely to achieve hearing recovery. The more severe the hearing loss during the early stage, the lower the hearing recovery rates. Patients aged less than 60 years appear to have better prognosis of hearing improvement compared to those who are over 60 years. Conclusions Important prognostic factors for recovery in patients with SSNHL include the time of initiating treatment after symptom onset, the degree of early-stage hearing loss, and the age of the affected patient. PMID:25279228

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

    PubMed

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

    2014-01-01

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

  5. 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

  6. 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.

  7. Patterns of Long-term Hearing Loss in Hearing Preservation Cochlear Implant Surgery.

    PubMed

    Dedhia, Kavita; Worman, Tina; Meredith, Margaret A; Rubinstein, Jay T

    2016-06-01

    To describe patterns of hearing loss in patients with low-frequency residual hearing after cochlear implantation. Retrospective chart review. Tertiary referral center. Cochlear implant candidates with immediate postoperative residual low-frequency hearing. Hybrid or traditional cochlear implant. Audiograms to measure postoperative hearing. Of the 166 patients reviewed, 17 ears met the inclusion criteria. The age ranged from 3 years 2 months to 86 years. Etiology was unknown (n = 6), presbycusis (n = 5), genetic (n = 4), acoustic trauma (n = 1), and measles virus (n = 1). The Nucleus Hybrid S8 and S12 (n = 7) was the most common electrode array, and then Nucleus 422 (n = 6), Nucleus Contour Advance (n = 2), Med-El Flex 28 (n = 2), and Advanced Bionics Mid Scala (n = 1). Cochleostomy was performed in nine, and round window approach in nine patients. Average follow-up was 28 months (2-68 mo). Postoperative loss was mixed in eight and purely sensorineural in eight. The most common patterns of hearing loss were gradual decline (n = 7), and then fluctuating hearing (n = 6), stable (n = 3), and sudden loss (n = 1). One patient only had one postoperative audiogram. Some long-term hearing preservation was achieved in 94% of patients with immediate postoperative hearing preservation. Patients developed both mixed and sensorineural loss postoperatively. A majority of patients with mixed hearing loss had a supra-preoperative bone curve. Gradual decline and fluctuating hearing loss were the most common patterns of hearing loss; few patients had stable hearing and one had a sudden loss.

  8. Hearing loss in children treated for multidrug-resistant tuberculosis.

    PubMed

    Seddon, James A; Thee, Stephanie; Jacobs, Kayleen; Ebrahim, Adam; Hesseling, Anneke C; Schaaf, H Simon

    2013-04-01

    The aminoglycosides and polypeptides are vital drugs for the management of multidrug-resistant (MDR) tuberculosis (TB). Both classes of drug cause hearing loss. We aimed to determine the extent of hearing loss in children treated for MDR-TB. In this retrospective study, children (<15 years) admitted to Brooklyn Chest Hospital, Cape Town, South Africa, from January 2009 until December 2010, were included if treated for MDR-TB with injectable drugs. Hearing was assessed and classified using audiometry and otoacoustic emissions. Ninety-four children were included (median age: 43 months). Of 93 tested, 28 (30%) were HIV-infected. Twenty-three (24%) children had hearing loss. Culture-confirmed, as opposed to presumed, diagnosis of TB was a risk factor for hearing loss (OR: 4.12; 95% CI: 1.13-15.0; p = 0.02). Seven of 11 (64%) children classified as having hearing loss using audiometry had progression of hearing loss after finishing the injectable drug. Hearing loss is common in children treated for MDR-TB. Alternative drugs are required for the treatment of paediatric MDR-TB. Copyright © 2012 The British Infection Association. Published by Elsevier Ltd. All rights reserved.

  9. Comparison of Carina active middle-ear implant with conventional hearing aids for mixed hearing loss.

    PubMed

    Savaş, V A; Gündüz, B; Karamert, R; Cevizci, R; Düzlü, M; Tutar, H; Bayazit, Y A

    2016-04-01

    To compare the auditory outcomes of Carina middle-ear implants with those of conventional hearing aids in patients with moderate-to-severe mixed hearing loss. The study comprised nine patients (six males, three females) who underwent middle-ear implantation with Carina fully implantable active middle-ear implants to treat bilateral moderate-to-severe mixed hearing loss. The patients initially used conventional hearing aids and subsequently received the Carina implants. The hearing thresholds with implants and hearing aids were compared. There were no significant differences between: the pre-operative and post-operative air and bone conduction thresholds (p > 0.05), the thresholds with hearing aids and Carina implants (p > 0.05), or the pre-operative (mean, 72.8 ± 19 per cent) and post-operative (mean, 69.9 ± 24 per cent) speech discrimination scores (p > 0.05). One of the patients suffered total sensorineural hearing loss three months following implantation despite an initial 38 dB functional gain. All except one patient showed clinical improvements after implantation according to quality of life questionnaire (Glasgow Benefit Inventory) scores. Acceptance of Carina implants is better than with conventional hearing aids in patients with mixed hearing loss, although both yield similar hearing amplification. Cosmetic reasons appear to be critical for patient acceptance.

  10. Screening of Connexin 26 in Nonsyndromic Hearing Loss

    PubMed Central

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

    2014-01-01

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

  11. Positive, Neutral, and Negative Connotations Associated with Social Representation of 'Hearing Loss' and 'Hearing Aids'.

    PubMed

    Manchaiah, Vinaya; Stein, Gretchen; Danermark, Berth; Germundsson, Per

    2015-12-01

    In our previous studies we explored the social representation of hearing loss and hearing aids. In this study we aimed at exploring if the positive, neutral and negative connotations associated with the social representation of 'hearing loss' and 'hearing aids' for the same categories vary across countries. In addition, we also looked at if there is an association between connotations and demographic variables. A total of 404 individuals from four countries were asked to indicate the words and phrases that comes to mind when they think about 'hearing loss' and 'hearing aids'. They also indicated if the words and phrases they reported had positive, neutral or negative association, which were analyzed and reported in this paper. There are considerable differences among the countries in terms of positive, neutral and negative associations report for each category in relation to hearing loss and hearing aids. However, there is limited connection between demographic variables and connotations reported in different countries. These results suggesting that the social representation about the phenomenon hearing loss and hearing aids are relatively stable within respondents of each country.

  12. Changes in auditory perceptions and cortex resulting from hearing recovery after extended congenital unilateral hearing loss

    PubMed Central

    Firszt, Jill B.; Reeder, Ruth M.; Holden, Timothy A.; Burton, Harold; Chole, Richard A.

    2013-01-01

    Monaural hearing induces auditory system reorganization. Imbalanced input also degrades time-intensity cues for sound localization and signal segregation for listening in noise. While there have been studies of bilateral auditory deprivation and later hearing restoration (e.g., cochlear implants), less is known about unilateral auditory deprivation and subsequent hearing improvement. We investigated effects of long-term congenital unilateral hearing loss on localization, speech understanding, and cortical organization following hearing recovery. Hearing in the congenitally affected ear of a 41 year old female improved significantly after stapedotomy and reconstruction. Pre-operative hearing threshold levels showed unilateral, mixed, moderately-severe to profound hearing loss. The contralateral ear had hearing threshold levels within normal limits. Testing was completed prior to, and 3 and 9 months after surgery. Measurements were of sound localization with intensity-roved stimuli and speech recognition in various noise conditions. We also evoked magnetic resonance signals with monaural stimulation to the unaffected ear. Activation magnitudes were determined in core, belt, and parabelt auditory cortex regions via an interrupted single event design. Hearing improvement following 40 years of congenital unilateral hearing loss resulted in substantially improved sound localization and speech recognition in noise. Auditory cortex also reorganized. Contralateral auditory cortex responses were increased after hearing recovery and the extent of activated cortex was bilateral, including a greater portion of the posterior superior temporal plane. Thus, prolonged predominant monaural stimulation did not prevent auditory system changes consequent to restored binaural hearing. Results support future research of unilateral auditory deprivation effects and plasticity, with consideration for length of deprivation, age at hearing correction and degree and type of hearing loss. PMID

  13. mtDNA mutations, hearing loss and aminoglycoside treatment in Mexicans.

    PubMed

    Meza, G; Torres-Ruíz, N M; Tirado-Gutiérrez, C; Aguilera, P

    2011-01-01

    Streptomycin and aminoglycoside derivatives are commonly used to treat tuberculosis and other stubborn infections; these drugs may alter auditory and/or vestibular function. Mutations in mitochondrial DNA have been associated with hypersensitivity to aminoglycosides; no studies have been conducted in Mexicans, which are very prone to such alterations because aminoglycosides have been prescribed carelessly for many years, irrespective of the ailment to be treated. We investigated "hot spot" mutations described previously as causing inner ear alterations. Hot spot mutations at the 12S rRNA gene and the tRNA Serine (UCN) gene were screened by PCR-RFLP and sequencing in 65 subjects undergoing audiological and vestibular testing. Experimental. 32 individuals had healthy auditory and vestibular function, whereas 33 subjects had auditory affections. We found none of the previously reported mutations related to aminoglycoside hypersensitivity, or non-syndromic hearing loss. Two hearing-impaired patients that had been treated with streptomycin had the T1189C variant of the mitochondrial 12S rRNA region. Mutations related to hearing loss in other ethnic backgrounds were not found in Mexicans. However, the T1189C variant is possibly a putative mutation related to aminoglycoside hypersensitivity and was present in 2 patients.

  14. Associations Between TGFA/TGFB3/MSX1 Gene Polymorphisms and Congenital Non-Syndromic Hearing Impairment in a Chinese Population

    PubMed Central

    Du, Jihong; Deng, Jianhua

    2016-01-01

    Background The aim of this study was to investigate whether the TGFA/TGFB3/MSX1 gene polymorphisms and haplotypes lead to individual differences between congenital non-syndromic hearing impairment (NSHI) patients and normal people in a Chinese population and to analyze the risk factors for NSHI. Material/Methods Between December 2010 and September 2014, 343 congenital NSHI patients were recruited as cases, and 272 healthy subjects were recruited as controls. Denaturing high-performance liquid chromatography (DHPLC) was used to identify genotypes, SHEsis software was used to conduct gene linkage disequilibrium and haplotype analyses, and regression analysis was performed to identify risk factors for congenital NSHI. Results The distribution of genotype frequencies and allele frequencies of TGFA rs3771494, TGFB3 rs3917201 and rs2268626, and MSX1 rs3821949 and rs62636562 were significantly different between the case and the control groups (all P<0.05). TGFA/TGFB3/MSX1 gene rs3771494, rs1058213, rs3917201, rs2268626, rs3821949, and rs62636562 haplotype analysis showed that haplotype CCGTAC and TTACGT might be protective factors (both P<0.001), while TTGCGC might be a risk factor for the normal population (P<0.001). The other risk factors include paternal smoking, advanced maternal age, maternal sickness history, maternal contact with pesticides or similar drugs, maternal abortion history, maternal medication history, maternal passive smoking history during pregnancy, rs3771494 CT, rs2268626 CC and TC, and rs3821949 GG and AG genotypes were risk factors (all P<0.05), while maternal vitamin supplements during pregnancy, rs3917201 GA, rs62636562 TT and CT genotypes were protective factors for congenital NSHI (all P<0.05). Conclusions rs3771494, rs3917201, rs2268626, rs3821949 and rs62636562 might be associated with congenital NSHI. PMID:27356075

  15. Sensorineural hearing loss in hemodialysis patients.

    PubMed

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

    2010-03-01

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

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

    PubMed

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

    2016-05-01

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

  17. EPS8L2 is a new causal gene for childhood onset autosomal recessive progressive hearing loss.

    PubMed

    Dahmani, Malika; Ammar-Khodja, Fatima; Bonnet, Crystel; Lefèvre, Gaelle M; Hardelin, Jean-Pierre; Ibrahim, Hassina; Mallek, Zahia; Petit, Christine

    2015-08-19

    More than 70 % of the cases of congenital deafness are of genetic origin, of which approximately 80 % are non-syndromic and show autosomal recessive transmission (DFNB forms). To date, 60 DFNB genes have been identified, most of which cause congenital, severe to profound deafness, whereas a few cause delayed progressive deafness in childhood. We report the study of two Algerian siblings born to consanguineous parents, and affected by progressive hearing loss. After exclusion of GJB2 (the gene most frequently involved in non-syndromic deafness in Mediterranean countries), we performed whole-exome sequencing in one sibling. A frame-shift variant (c.1014delC; p.Ser339Alafs*15) was identified in EPS8L2, encoding Epidermal growth factor receptor Pathway Substrate 8 L2, a protein of hair cells' stereocilia previously implicated in progressive deafness in the mouse. This variant predicts a truncated, inactive protein, or no protein at all owing to nonsense-mediated mRNA decay. It was detected at the homozygous state in the two clinically affected siblings, and at the heterozygous state in the unaffected parents and one unaffected sibling, whereas it was never found in a control population of 150 Algerians with normal hearing or in the Exome Variant Server database. Whole-exome sequencing allowed us to identify a new gene responsible for childhood progressive hearing loss transmitted on the autosomal recessive mode.

  18. 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.

  19. Prelinguistic Vocal Development in Infants with Typical Hearing and Infants with Severe-to-Profound Hearing Loss

    ERIC Educational Resources Information Center

    Iyer, Suneeti Nathani; Oller, D. Kimbrough

    2008-01-01

    Delays in the onset of canonical babbling with hearing loss are extensively documented. Relatively little is known about other aspects of prelinguistic vocal development and hearing loss. Eight infants with typical hearing and eight with severe-to-profound hearing loss were matched with regard to a significant vocal development milestone, the…

  20. Prelinguistic Vocal Development in Infants with Typical Hearing and Infants with Severe-to-Profound Hearing Loss

    ERIC Educational Resources Information Center

    Iyer, Suneeti Nathani; Oller, D. Kimbrough

    2008-01-01

    Delays in the onset of canonical babbling with hearing loss are extensively documented. Relatively little is known about other aspects of prelinguistic vocal development and hearing loss. Eight infants with typical hearing and eight with severe-to-profound hearing loss were matched with regard to a significant vocal development milestone, the…

  1. Noise Exposure and Hearing Loss Among Sand and Gravel Miners

    PubMed Central

    Landen, Deborah; Wilkins, Steve; Stephenson, Mark; McWilliams, Linda

    2017-01-01

    The objectives of this study were to describe workplace noise exposures, risk factors for hearing loss, and hearing levels among sand and gravel miners, and to determine whether full shift noise exposures resulted in changes in hearing thresholds from baseline values. Sand and gravel miners (n = 317) were interviewed regarding medical history, leisure-time and occupational noise exposure, other occupational exposures, and use of hearing protection. Audiometric tests were performed both before the work shift (following a 12-hour noise-free interval) and immediately following the work shift. Full shift noise dosimetry was conducted. Miners' noise exposures exceeded the Recommended Exposure Limit (REL) of the National Institute for Occupational Safety and Health (NIOSH) for 69% of workers, and exceeded the Mine Safety and Health Administration's action level for enrollment in a hearing conservation program for 41% of workers. Significantly higher noise exposures occurred among employees of small companies, among workers with a job classification of truck driver, among males, and among black workers. Hearing protection usage was low, with 48% of subjects reporting that they never used hearing protection. Hearing impairment, as defined by NIOSH, was present among 37% of 275 subjects with valid audiograms. Black male workers and white male workers had higher hearing thresholds than males from a comparison North Carolina population unexposed to industrial noise. Small but statistically significant changes in hearing thresholds occurred following full shift noise exposure among subjects who had good hearing sensitivity at baseline. In a logistic regression model, age and history of a past noisy job were significant predictors of hearing impairment. Overall, sand and gravel workers have excessive noise exposures and significant hearing loss, and demonstrate inadequate use of hearing protection. Well-designed hearing conservation programs, with reduction of noise exposure

  2. Noise exposure and hearing loss among sand and gravel miners.

    PubMed

    Landen, Deborah; Wilkins, Steve; Stephenson, Mark; McWilliams, Linda

    2004-08-01

    The objectives of this study were to describe workplace noise exposures, risk factors for hearing loss, and hearing levels among sand and gravel miners, and to determine whether full shift noise exposures resulted in changes in hearing thresholds from baseline values. Sand and gravel miners (n = 317) were interviewed regarding medical history, leisure-time and occupational noise exposure, other occupational exposures, and use of hearing protection. Audiometric tests were performed both before the work shift (following a 12-hour noise-free interval) and immediately following the work shift. Full shift noise dosimetry was conducted. Miners' noise exposures exceeded the Recommended Exposure Limit (REL) of the National Institute for Occupational Safety and Health (NIOSH) for 69% of workers, and exceeded the Mine Safety and Health Administration's action level for enrollment in a hearing conservation program for 41% of workers. Significantly higher noise exposures occurred among employees of small companies, among workers with a job classification of truck driver, among males, and among black workers. Hearing protection usage was low, with 48% of subjects reporting that they never used hearing protection. Hearing impairment, as defined by NIOSH, was present among 37% of 275 subjects with valid audiograms. Black male workers and white male workers had higher hearing thresholds than males from a comparison North Carolina population unexposed to industrial noise. Small but statistically significant changes in hearing thresholds occurred following full shift noise exposure among subjects who had good hearing sensitivity at baseline. In a logistic regression model, age and history of a past noisy job were significant predictors of hearing impairment. Overall, sand and gravel workers have excessive noise exposures and significant hearing loss, and demonstrate inadequate use of hearing protection. Well-designed hearing conservation programs, with reduction of noise exposure

  3. Hypertension, Diuretic Use, and Risk of Hearing Loss

    PubMed Central

    Lin, Brian M.; Curhan, Sharon G.; Wang, Molin; Eavey, Roland; Stankovic, Konstantina M.; Curhan, Gary C.

    2016-01-01

    Background Hearing loss is highly prevalent among adults in the United States. Hypertension is also common and is often treated with diuretics. Hypertension may increase the risk of hearing loss by decreasing vascular supply to the stria vascularis. Use of thiazides has been anecdotally associated with hearing loss. In small studies, furosemide use has been associated with hearing loss that is usually reversible, but can be permanent. We investigated the relation between hypertension, diuretic use, and hearing loss in a prospective cohort of 54,721 women in Nurses’ Health Study I (NHSI), 1994–2012. Methods Eligible participants included 54,721 female nurses aged 48–73 years in 1994 who provided information on thiazide diuretic and furosemide use in 1994, answered the question on hearing loss over their lifetime in 2012, and did not report hearing loss with date of onset before date of onset of hypertension diagnosis or medication use. The outcome was self-reported hearing loss. Cox proportional hazards regression was used to adjust for potential confounders. Results During 774,096 person-years of follow-up, 19,296 cases of hearing loss were reported (incidence rate: 25 cases per 1000 person-years). At baseline in 1994, the mean age was 57.9 years and mean body mass index was 26.3 kg/m2. 30.8% of participants had a history of hypertension. History of hypertension was independently associated with a modestly higher risk of hearing loss (multivariable adjusted relative risk = 1.04 [1.01, 1.07]). Among women with a history of hypertension, neither thiazide diuretic (multivariable adjusted relative risk = 1.07 [0.99, 1.16]) nor furosemide use (multivariable adjusted relative risk = 0.91 [0.75, 1.09]) was significantly associated with risk of hearing loss, when compared with women not taking anti-hypertensive medications. There was no significant effect modification by age. Conclusions History of hypertension was associated with a small increased risk of hearing

  4. Lyme disease: sudden hearing loss as the sole presentation.

    PubMed

    Espiney Amaro, C; Montalvão, P; Huins, C; Saraiva, J

    2015-02-01

    Lyme disease is an uncommon tick-borne multisystemic infection caused by Borrelia burgdorferi. The most common clinical manifestation is erythema migrans. In this report, a very unusual presentation of this condition is described, in which sudden onset sensorineural hearing loss was the sole presenting symptom. Case report and review of English-language literature. A patient presented with sensorineural hearing loss, with no other symptoms or signs. Acute Lyme infection was detected by laboratory tests. Magnetic resonance imaging showed signs of labyrinthitis of the same inner ear. After hyperbaric oxygen and systemic antibiotic treatment, the patient showed total hearing recovery, and magnetic resonance imaging showed complete resolution of the labyrinthitis. To our knowledge, this is the first reported case of Lyme disease presenting only with sensorineural hearing loss. Borreliosis should be considered as an aetiological factor in sensorineural hearing loss. Adequate treatment may provide total recovery and prevent more severe forms of Lyme disease.

  5. 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

  6. Categorical loudness scaling and equal-loudness contours in listeners with normal hearing and hearing loss.

    PubMed

    Rasetshwane, Daniel M; Trevino, Andrea C; Gombert, Jessa N; Liebig-Trehearn, Lauren; Kopun, Judy G; Jesteadt, Walt; Neely, Stephen T; Gorga, Michael P

    2015-04-01

    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.

  7. Personal and social conditions potentially influencing women's hearing loss management.

    PubMed

    Garstecki, D C; Erler, S F

    2001-12-01

    Little gender-specific data related to hearing loss and hearing loss management are available. The purpose of this investigation was to examine personal and social conditions affecting women at selected stages of the adult life course that may influence hearing loss management. In all, 191 women in three age groups, ranging from 35 to 85 years old, participated. None reported hearing problems. Participants completed a demographic data form and were given a standard audiometric evaluation to confirm age-normal hearing. Each completed assessments of speech understanding in quiet and noise, auditory signal duration discrimination, and binaural processing. Measures of hearing knowledge, behaviors, and attitudes; health-related locus of control; ego strength; and, social support were administered. Results revealed that although some variables deteriorate among subsequent age groups (i.e., hearing thresholds, central auditory processing, and ego strength), the reverse is true for others (i.e., social interaction and satisfaction with income). Age-specific sociodemographic burdens that may interfere with hearing loss management were noted. New psychosocial data are revealed against which women and men with impaired hearing may be compared.

  8. 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.

  9. Subjective Ratings of Fatigue and Vigor in Adults With Hearing Loss Are Driven by Perceived Hearing Difficulties Not Degree of Hearing Loss.

    PubMed

    Hornsby, Benjamin W Y; Kipp, Aaron M

    2016-01-01

    Anecdotal reports and qualitative research suggests that fatigue is a common, but often overlooked, accompaniment of hearing loss which negatively affects quality of life. However, systematic research examining the relationship between hearing loss and fatigue is limited. In this study, the authors examined relationships between hearing loss and various domains of fatigue and vigor using standardized and validated measures. Relationships between subjective ratings of multidimensional fatigue and vigor and the social and emotional consequences of hearing loss were also explored. Subjective ratings of fatigue and vigor were assessed using the profile of mood states and the multidimensional fatigue symptom inventory-short form. To assess the social and emotional impact of hearing loss participants also completed, depending on their age, the hearing handicap inventory for the elderly or adults. Responses were obtained from 149 adults (mean age = 66.1 years, range 22 to 94 years), who had scheduled a hearing test and/or a hearing aid selection at the Vanderbilt Bill Wilkerson Center Audiology clinic. These data were used to explore relationships between audiometric and demographic (i.e., age and gender) factors, fatigue, and hearing handicap scores. Compared with normative data, adults seeking help for their hearing difficulties in this study reported significantly less vigor and more fatigue. Reports of severe vigor/fatigue problems (ratings exceeding normative means by ±1.5 standard deviations) were also increased in the study sample compared with that of normative data. Regression analyses, with adjustments for age and gender, revealed that the subjective percepts of fatigue, regardless of domain, and vigor were not strongly associated with degree of hearing loss. However, similar analyses controlling for age, gender, and degree of hearing loss showed a strong association between measures of fatigue and vigor (multidimensional fatigue symptom inventory-short form

  10. Hearing loss in hydrocephalus: a review, with focus on mechanisms.

    PubMed

    Satzer, David; Guillaume, Daniel J

    2016-01-01

    While neither hydrocephalus nor cerebrospinal fluid (CSF) shunt placement is traditionally considered in the differential diagnosis of hearing loss, there is substantial evidence that CSF circulation and pressure abnormalities can produce auditory dysfunction. Several indirect mechanisms may explain association between hydrocephalus and hearing loss, including mass effect, compromise of the auditory pathway, complications of prematurity, and genetically mediated hydrocephalus and hearing loss. Nevertheless, researchers have proposed a direct mechanism, which we term the hydrodynamic theory. In this hypothesis, the intimate relationship between CSF and inner ear fluids permits relative endolymphatic or perilymphatic hydrops in the setting of CSF pressure disturbances. CSF is continuous with perilymph, and CSF pressure changes are known to produce parallel perilymphatic pressure changes. In support of the hydrodynamic theory, some studies have found an independent association between hydrocephalus and hearing loss. Moreover, surgical shunting of CSF has been linked to both resolution and development of auditory dysfunction. The disease burden of hydrocephalus-associated hearing loss may be large, and because hydrocephalus and over-shunting are reversible, this relationship merits broader recognition. Hydrocephalic patients should be monitored for hearing loss, and hearing loss in a patient with shunted hydrocephalus should prompt further evaluation and possibly adjustment of shunt settings.

  11. Sound preconditioning therapy inhibits ototoxic hearing loss in mice.

    PubMed

    Roy, Soumen; Ryals, Matthew M; Van den Bruele, Astrid Botty; Fitzgerald, Tracy S; Cunningham, Lisa L

    2013-11-01

    Therapeutic drugs with ototoxic side effects cause significant hearing loss for thousands of patients annually. Two major classes of ototoxic drugs are cisplatin and the aminoglycoside antibiotics, both of which are toxic to mechanosensory hair cells, the receptor cells of the inner ear. A critical need exists for therapies that protect the inner ear without inhibiting the therapeutic efficacy of these drugs. The induction of heat shock proteins (HSPs) inhibits both aminoglycoside- and cisplatin-induced hair cell death and hearing loss. We hypothesized that exposure to sound that is titrated to stress the inner ear without causing permanent damage would induce HSPs in the cochlea and inhibit ototoxic drug–induced hearing loss. We developed a sound exposure protocol that induces HSPs without causing permanent hearing loss. We used this protocol in conjunction with a newly developed mouse model of cisplatin ototoxicity and found that preconditioning mouse inner ears with sound has a robust protective effect against cisplatin-induced hearing loss and hair cell death. Sound therapy also provided protection against aminoglycoside-induced hearing loss. These data indicate that sound preconditioning protects against both classes of ototoxic drugs, and they suggest that sound therapy holds promise for preventing hearing loss in patients receiving these drugs.

  12. Symptomatic sensorineural hearing loss in patients with ulcerative colitis.

    PubMed

    Casella, G; Corbetta, D; Zolezzi, M; Di Bella, C; Villanacci, V; Salemme, M; Milanesi, U; Antonelli, E; Baldini, V; Bassotti, G

    2015-12-01

    Sensorineural hearing loss has been reported as an extraintestinal manifestation of inflammatory bowel disease, especially in adult patients with ulcerative colitis. However, to date only a few series have been reported in the literature, and none from Italy. The aim of the present investigation was to assess the prevalence of symptomatic sensorineural hearing loss in Italian patients with ulcerative colitis. We retrospectively assessed the charts of all patients with ulcerative colitis who underwent otolaryngologic investigation in a 10-year period. Complete charts of 57 patients were available for the observation period. Reasons for head and neck investigation were transient, mild hearing loss and sporadic vertigo. Clinical and instrumental head and neck examination was unremarkable in all but one woman who complained of mild hearing loss without vertigo or tinnitus, in whom sensorineural hearing loss was diagnosed. In our series, sensorineural hearing loss was found in less than 2 % of adult patients with ulcerative colitis evaluated in a department of otolaryngology. Systematic evaluation for this extraintestinal manifestation should not be carried out unless hearing loss is present.

  13. [Device-based treatment of mixed hearing loss: An audiological comparison of current hearing systems].

    PubMed

    Rahne, T; Plontke, S K

    2016-02-01

    Various different hearing systems are available for device-supported hearing rehabilitation of patients with mixed hearing loss. Using the recently introduced objective comparison criterion "maximum output" (i.e., the maximum output level of a hearing device), the indications for different hearing devices can be compared. This article reviews important terms such as gain, dynamic range, and maximum output level-all of which are relevant for the selection of a hearing device. The experimental part of this study compares all currently available hearing devices and determines the range of their indication with respect to the maximum bone-conduction hearing threshold. The maximum frequency-specific output levels reported in the literature for the Baha Cordelle 2, the Sophono Alpha 2, and the Bonebridge (measured at the skull simulator), as well as those of the Codacs and the Soundbridge (in-vivo measurements) are compared to the maximum output levels given in the datasheets of the BP110 Power, the Baha Cordelle 2, the Bonebridge, the Codacs, the Ponto Pro Power, and the Sophono Alpha 2. Using appropriate correction factors, the maximum dynamic range and thus the maximum indication based on the bone-conduction threshold was determined. In patients with mild sensorineural hearing loss, passive transcutaneous hearing or Bonebridge implants can achieve good audiological results. In the transition region to moderate hearing loss, percutaneous devices are applicable. Combined hearing loss with more pronounced sensorineural hearing loss is best treated with a Soundbridge or Codacs implant. In the latter case, the cochlear potential for speech recognition has to be explored and, where appropriate, cochlear implants considered as an alternative.

  14. Cytomegalovirus bearing in children with sensorineural hearing losses.

    PubMed

    Devdariani, T; Chibalashvili, N; Tushishvili, M; Gogberashvili, K; Kevanishvili, Z

    2012-05-01

    The rate of bearing of cytomegalovirus, CMV, has been estimated in children suffering from inborn or newborn types of a sensorineural hearing loss of hard-to-severe degrees. The test group comprised 15 hearing-loss children of 3-6 years of age. The control group included accidentally selected 30 healthy children of the same age without any hearing complains. In both groups the CMV-specific IgG antibodies were determined in blood via the enzyme-linked immunosorbent assay, ELISA. The excessive amount of IgG antibodies was found in 14 out of 15 children with sensorineural hearing losses being estimated objectively, via computer registration of auditory brainstem responses, ABRs, and in 14 out of 30 children with normal hearing, being also inspected objectively, via specialized screening procedure. The intergroup difference in CMV bearing rates, 93.3% and 46.7%, respectively, has been confirmed to be statistically significant (p=0.007). CMV bearing happens thus twice as much in sensorineural hearing-loss than in normally-hearing children. Early detection of a hearing loss and early assessment of CMV bearing seem essential for an immediate start and, consequently, for a batter chance of positive outcomes of specific treatment-rehabilitation means.

  15. 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…

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

    PubMed

    Onsori, Habib; Rahmati, Mohammad; Fazli, Davood

    2014-12-01

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

  17. Hearing loss and disability exit: Measurement issues and coping strategies.

    PubMed

    Christensen, Vibeke Tornhøj; Datta Gupta, Nabanita

    2017-02-01

    Hearing loss is one of the most common conditions related to aging, and previous descriptive evidence links it to early exit from the labor market. These studies are usually based on self-reported hearing difficulties, which are potentially endogenous to labor supply. We use unique representative data collected in the spring of 2005 through in-home interviews. The data contains self-reported functional and clinically-measured hearing ability for a representative sample of the Danish population aged 50-64. We estimate the causal effect of hearing loss on early retirement via disability benefits, taking into account the endogeneity of functional hearing. Our identification strategy involves the simultaneous estimation of labor supply, functional hearing, and coping strategies (i.e. accessing assistive devices at work or informing one's employer about the problem). We use hearing aids as an instrument for functional hearing. Our main empirical findings are that endogeneity bias is more severe for men than women and that functional hearing problems significantly increase the likelihood of receiving disability benefits for both men and women. However, relative to the baseline the effect is larger for men (47% vs. 20%, respectively). Availability of assistive devices in the workplace decreases the likelihood of receiving disability benefits, whereas informing an employer about hearing problems increases this likelihood.

  18. Hearing Loss, Dizziness, and Carbohydrate Metabolism

    PubMed Central

    Albernaz, Pedro L. Mangabeira

    2015-01-01

    Introduction  Metabolic activity of the inner ear is very intense, and makes it sensitive to changes in the body homeostasis. This study involves a group of patients with inner ear disorders related to carbohydrate metabolism disturbances, including hearing loss, tinnitus, dizziness, and episodes of vertigo. Objectives  To describe the symptoms of metabolic inner ear disorders and the examinations required to establish diagnoses. These symptoms are often the first to allow for an early diagnosis of metabolic disorders and diabetes. Methods  Retrospective study of 376 patients with inner ear symptoms suggestive of disturbances of carbohydrate metabolism. The authors present patientś clinical symptoms and clinical evaluations, with emphasis on the glucose and insulin essays. Results  Authors based their conclusions on otolaryngological findings, diagnostic procedures and treatment principles. They found that auditory and vestibular symptoms usually occur prior to other manifestations of metabolic changes, leading to an early diagnosis of hyperinsulinemia, intestinal sugar malabsorption or diabetes. Previously undiagnosed diabetes mellitus type II was found in 39 patients. Conclusions  The identification of carbohydrate metabolism disturbances is important not only to minimize the patients' clinical symptoms, but also to help maintain their general health. PMID:27413410

  19. 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.

  20. 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

  1. Reduced Connexin26 in the Mature Cochlea Increases Susceptibility to Noise-Induced Hearing Loss in Mice

    PubMed Central

    Zhou, Xing-Xing; Chen, Sen; Xie, Le; Ji, Yu-Zi; Wu, Xia; Wang, Wen-Wen; Yang, Qi; Yu, Jin-Tao; Sun, Yu; Lin, Xi; Kong, Wei-Jia

    2016-01-01

    Connexin26 (Cx26, encoded by GJB2) mutations are the most common cause of non-syndromic deafness. GJB2 is thought to be involved in noise-induced hearing loss (NIHL). However, the role of Cx26 in NIHL is still obscure. To explore the association between Cx26 and NIHL, we established a Cx26 knockdown (KD) mouse model by conditional knockdown of Cx26 at postnatal day 18 (P18), and then we observed the auditory threshold and morphologic changes in these mice with or without noise exposure. The Cx26 KD mice did not exhibit substantial hearing loss and hair cell degeneration, while the Cx26 KD mice with acoustic trauma experienced higher hearing loss than simple noise exposure siblings and nearly had no recovery. Additionally, extensive outer hair cell loss and more severe destruction of the basal organ of Corti were observed in Cx26 KD mice after noise exposure. These data indicate that reduced Cx26 expression in the mature mouse cochlea may increase susceptibility to noise-induced hearing loss and facilitate the cell degeneration in the organ of Corti. PMID:26927086

  2. Internet access and use in adults with hearing loss.

    PubMed

    Thorén, Elisabet Sundewall; Oberg, Marie; Wänström, Gunilla; Andersson, Gerhard; Lunner, Thomas

    2013-05-09

    The future rehabilitation of adults with hearing loss is likely to involve online tools used by individuals at home. Online tools could also be useful for people who are not seeking professional help for their hearing problems. Hearing impairment is a disability that increases with age, and increased age is still associated with reduced use of the Internet. Therefore, to continue the research on online audiological rehabilitative tools for people with hearing loss, it is important to determine if and to what extent adults with hearing loss use the Internet. To evaluate the use of the Internet and email in a group of adults with hearing loss and to investigate if their use of Internet and email differed between genders, among different age groups, and how it compared with the general population in Sweden. Questionnaires containing multiple-choice questions about Internet access, email use, and educational level were mailed to individuals with hearing loss, who were registered as patients at a hearing aid clinic. Out of the 269 invited participants, 158 returned a completed questionnaire, which was a response rate of 58.7%. The results showed that 60% (94/158) of the participants with hearing loss used computers and the Internet. The degree of hearing loss in the group of participants did not explain the level of Internet usage, while factors of age, gender, and education did (P<.001). More men than women used the Internet (OR 2.54, 95% CI 1.32-4.91, P<.001). Use of the Internet was higher in the youngest age group (25-64 years) compared to the oldest age group (75-96 years, P=.001). A higher usage of the Internet was observed in the participants with hearing loss, especially the elderly, when compared with the general population of Sweden (OR 1.74, 95% CI 1.23-3.17, P=.04). We conclude that the use of computers and the Internet overall is at least at the same level for people with hearing loss as for the general age-matched population in Sweden, but that this use is

  3. [Sudden onset hearing loss: imaging work-up].

    PubMed

    Pons, Y; Ukkola-Pons, E; Kossowski, M

    2011-11-01

    Sudden onset hearing loss is idiopathic. It occurs in less than 24 h and spontaneously resolves within 15 days in two thirds of cases. Imaging is performed to exclude other causes of sudden onset hearing loss (vestibular schwannoma, vertebral artery dissection, stroke) and evaluate the inner ear structures. A few anatomical anomalies have been associated with an increased risk of hearing loss. Morphological anomalies involved the following structures in decreasing order of frequency: lateral semicircular canal (hypoplasia and dilatation), superior semicircular canal, posterior semicircular canal, vestibule and cochlea. Enlargement of the vestibular aqueduct also is frequently observed.

  4. Developmental hearing loss disrupts synaptic inhibition: implications for auditory processing

    PubMed Central

    Takesian, Anne E; Kotak, Vibhakar C; Sanes, Dan H

    2009-01-01

    Hearing loss during development leads to central deficits that persist even after the restoration of peripheral function. One key class of deficits is due to changes in central inhibitory synapses, which play a fundamental role in all aspects of auditory processing. This review focuses on the anatomical and physiological alterations of inhibitory connections at several regions within the central auditory pathway following hearing loss. Such aberrant inhibitory synaptic function may be linked to deficits in encoding binaural and spectral cues. Understanding the cellular changes that occur at inhibitory synapses following hearing loss may provide specific loci that can be targeted to improve function. PMID:20161214

  5. Cisplatin and cranial irradiation-related hearing loss in children.

    PubMed

    Warrier, Rajasekharan; Chauhan, Aman; Davluri, Murali; Tedesco, Sonya L; Nadell, Joseph; Craver, Randall

    2012-01-01

    High doses of cisplatin and cranial radiotherapy (CRT) have been reported to cause irreversible hearing loss. The objective of this study was to examine the influence of cranial irradiation on cisplatin-associated ototoxicity in children with pediatric malignancies. Serial audiograms were obtained for 33 children, age <16 years, treated with cisplatin-based chemotherapy (90-120 mg/m(2) per cycle) with or without CRT. Eligible patients included those with normal baseline audiometric evaluations and without significant exposure to other ototoxic drugs. We defined significant hearing loss as a hearing threshold ≥30 dB at 2,000-8,000 Hz frequencies. The median age of our study population was 4.9 years (range 6 weeks to 16 years), and the male to female ratio was 0.8:1. The study population consisted of 15 Caucasians, 17 African-Americans, and 1 Hispanic. Fourteen patients had brain tumors, and 19 had other solid tumors. Thirteen patients were exposed to CRT, and 20 were not. Bilateral hearing loss was observed in 24/33 (73%) patients, with severe/profound (≥70 dB) impairment in 10/33 (30%) of all patients. Young age (<5 years), CRT, and brain tumors were independent prognostic factors predicting hearing loss. The study demonstrated a high incidence of hearing loss in children treated with cisplatin and CRT. Consequently, we recommend monitoring these children for the early detection of hearing loss.

  6. Cisplatin and Cranial Irradiation-Related Hearing Loss in Children

    PubMed Central

    Warrier, Rajasekharan; Chauhan, Aman; Davluri, Murali; Tedesco, Sonya L.; Nadell, Joseph; Craver, Randall

    2012-01-01

    Background: High doses of cisplatin and cranial radiotherapy (CRT) have been reported to cause irreversible hearing loss. The objective of this study was to examine the influence of cranial irradiation on cisplatin-associated ototoxicity in children with pediatric malignancies. Methods: Serial audiograms were obtained for 33 children, age <16 years, treated with cisplatin-based chemotherapy (90-120 mg/m2 per cycle) with or without CRT. Eligible patients included those with normal baseline audiometric evaluations and without significant exposure to other ototoxic drugs. We defined significant hearing loss as a hearing threshold ≥30 dB at 2,000-8,000 Hz frequencies. Results: The median age of our study population was 4.9 years (range 6 weeks to 16 years), and the male to female ratio was 0.8:1. The study population consisted of 15 Caucasians, 17 African-Americans, and 1 Hispanic. Fourteen patients had brain tumors, and 19 had other solid tumors. Thirteen patients were exposed to CRT, and 20 were not. Bilateral hearing loss was observed in 24/33 (73%) patients, with severe/profound (≥70 dB) impairment in 10/33 (30%) of all patients. Young age (<5 years), CRT, and brain tumors were independent prognostic factors predicting hearing loss. Conclusion: The study demonstrated a high incidence of hearing loss in children treated with cisplatin and CRT. Consequently, we recommend monitoring these children for the early detection of hearing loss. PMID:23049454

  7. Course of hearing loss and occurrence of tinnitus.

    PubMed

    König, Ovidiu; Schaette, Roland; Kempter, Richard; Gross, Manfred

    2006-11-01

    Chronic tinnitus is often accompanied by a hearing impairment, but it is still unknown whether hearing loss can actually cause tinnitus. The association between the pitch of the tinnitus sensation and the audiogram edge in patients with high-frequency hearing loss suggests a functional relation, but a large fraction of patients with hearing loss does not present symptoms of tinnitus. We therefore, investigated how the occurrence of tinnitus is related to the shape of the audiogram. We analyzed a sample where all patients had noise-induced hearing loss, containing 30 patients without tinnitus, 24 patients with tone-like tinnitus, and 17 patients with noise-like tinnitus. All patients had moderate to severe high-frequency hearing loss, and only minor to moderate hearing loss at low frequencies. We found that tinnitus patients had less overall hearing loss than patients without tinnitus. Moreover, the maximum steepness of the audiogram was higher in patients with tinnitus (-52.9+/-1.9 dB/octave) compared to patients without tinnitus (-43.1+/-2.4 dB/octave). Differences in overall hearing loss and maximum steepness between tone-like and noise-like tinnitus were not significant. For tone-like tinnitus, there was a clear association between the tinnitus pitch and the edge of the audiogram, with tinnitus pitch being on average 1.48+/-0.12 octaves above the audiogram edge frequency, and 0.81+/-0.1 octaves above the frequency with the steepest slope. Our results suggest that the occurrence of tinnitus is promoted by a steep audiogram slope. A steep slope leads to abrupt discontinuities in the activity along the tonotopic axis of the auditory system, which could be misinterpreted as sound.

  8. Bimodal Stimulation with Cochlear Implant and Hearing Aid in Cases of Highly Asymmetrical Hearing Loss.

    PubMed

    Sanhueza, Ignacio; Manrique, Raquel; Huarte, Alicia; de Erenchun, Iñigo Ruiz; Manrique, Manuel

    2016-04-01

    Bimodal stimulation is a possible treatment for asymmetrical hearing loss, wherein 1 ear is stimulated with a cochlear implant and the other is stimulated with a hearing aid. This emerging indication has gained significance over the last few years. However, little research has been conducted regarding the performance in different types of asymmetric Hearing loss. This study seeks to prove the bilateral-binaural advantage in a group of patients treated with bimodal stimulation (cochlear implant and hearing aid), with different degrees of hearing loss in their best ear. In total, 31 patients were recruited for the study. They were divided into 3 groups on the basis of the ear with the hearing aid: Group A, pure tone average (PTA) between 41 and 70 dB HL; Group B, PTA between 71 and 80 dB HL; and Group C, PTA between 81 and 90 dB HL. The performance in PTA and disyllabic word recognition were analyzed separately in each ear and then bimodally. The minimum follow-up period was 2 years. There were statistically significant differences between bimodal and monaural conditions both in PTA and in disyllabic word recognition. The better the residual hearing in the ear with the hearing aid, the greater were the benefits obtained with bilateralism-binaurality. Bimodal stimulation provides better results than any monaural hearing mode, regardless of whether it involves the use of a hearing aid alone or a cochlear implant alone.

  9. 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

  10. Hearing loss in singers: a preliminary study.

    PubMed

    Hu, Amanda; Hofmann, Erik; Davis, John; Capo, Joseph; Krane, Natalie; Sataloff, Robert T

    2015-01-01

    Singers need good hearing; however, they may be exposed to loud noises during their musical activities. The objectives of this study were to describe the incidence and type of hearing loss (HL) in singers. Retrospective case cohort. Billing records identified patients who had undergone videostroboscopy and audiogram during the same visit over a 3 year period. A singer was defined as anyone who self-identified as a singer (professional or avocational). Age and gender matched nonsingers were used as controls. Patients with otologic diagnoses, surgery, or complaints were excluded. Retrospective chart review was conducted for the presence of HL, type of HL, and pure tones audiogram results. Statistical analysis included descriptive statistics, Students t test, chi-square test, and Fisher exact test. Of 172 singers (44.7 years, 37.8% male), 31 (17.5%) had HL. Pure tone thresholds for the singers with HL subgroup at 3, 4, and 6 kHz were 21.0, 26.5, and 34.4 dB in the right, and 22.8, 30.3, and 38.8 dB in the left ear, respectively. Older age (P = 0.000000000000001), male gender (P < 0.001), longer number of years of singing (P = 0.0000000003), and baritone voice (P < 0.001) were associated with HL. There was no association with genre of music. When compared with controls, the incidence of HL (19.8%) was not significantly different (χ2 = 0.300, P = 0.58). Pure tones at 3, 4, and 6 kHz were not significantly different than controls with HL. Most common type of HL in singers was bilateral sensorineural (83.9%), which was significantly higher than controls (39.0%, χ2 = 14.6, P < 0.001). The incidence of HL in singers was 17.5%, which was not significantly different from controls. Bilateral sensorineural HL was most common. Copyright © 2015 The Voice Foundation. Published by Elsevier Inc. All rights reserved.

  11. Effective Practices in Teaching Indigenous Students with Conductive Hearing Loss

    ERIC Educational Resources Information Center

    Partington, Gary; Galloway, Ann

    2005-01-01

    Hearing impairment due to conductive hearing loss can have a devastating effect on children's language development, and consequently educational outcomes, especially for Indigenous students, for whom there may be the additional issue of being educated in their second or third language. With appropriate interventions, however, Indigenous students…

  12. Parental stress among parents of toddlers with moderate hearing loss.

    PubMed

    Dirks, Evelien; Uilenburg, Noëlle; Rieffe, Carolien

    2016-08-01

    The purpose of this study was to examine parental stress in parents of toddlers with moderate hearing loss compared to hearing controls. Furthermore, the associations between parental stress and child- and parent-related factors such as language, social-emotional functioning and social support were examined. The study sample consisted of 30 toddlers with moderate hearing loss and 30 hearing children (mean age 27.4 months). The two groups were compared using the Nijmegen Parenting Stress Index (NPSI) and parent-reports to rate the amount of social support and the children's social-emotional functioning. Receptive and expressive language tests were administered to the children to examine their language ability. Parents of toddlers with moderate hearing loss reported comparable levels of parental stress to parents of hearing children. Individual differences in parental stress were related to child- and parent-related factors. Poorer social-emotional functioning and language ability of the child were related to higher stress levels in parents. Parents who experienced less social support reported higher stress levels. Parents of toddlers with moderate hearing loss experience no more parental stress than parents of hearing children on average. Given parental stress was found to be related to poorer child functioning, early interventionists should be aware of signs of elevated stress levels in parents. Copyright © 2016 Elsevier Ltd. All rights reserved.

  13. Hearing Loss in Middle-Age Persons with Down Syndrome.

    ERIC Educational Resources Information Center

    Evenhuis, H. M.; And Others

    1992-01-01

    This study assessed the hearing function of 35 middle-aged adults with Down's syndrome by means of otoscopy, impedance audiometry, brainstem evoked response audiometry, and pure tone audiometry. The study found brainstem evoked response audiometry useful for routine audiological assessment, as it identified hearing losses of 20 to 90 decibels in…

  14. Identifying Hearing Loss in Young Children: Technology Replaces the Bell

    ERIC Educational Resources Information Center

    Eiserman, William; Shisler, Lenore

    2010-01-01

    Hearing loss can too easily be misdiagnosed or overlooked by providers serving young children. Parents and professionals may observe a language delay--an "invisible" condition--while failing to identify the underlying cause. Otoacoustic emissions (OAE) hearing screening technology, used extensively with newborns, is becoming an essential tool,…

  15. Hearing Loss in Middle-Age Persons with Down Syndrome.

    ERIC Educational Resources Information Center

    Evenhuis, H. M.; And Others

    1992-01-01

    This study assessed the hearing function of 35 middle-aged adults with Down's syndrome by means of otoscopy, impedance audiometry, brainstem evoked response audiometry, and pure tone audiometry. The study found brainstem evoked response audiometry useful for routine audiological assessment, as it identified hearing losses of 20 to 90 decibels in…

  16. Identifying Hearing Loss in Young Children: Technology Replaces the Bell

    ERIC Educational Resources Information Center

    Eiserman, William; Shisler, Lenore

    2010-01-01

    Hearing loss can too easily be misdiagnosed or overlooked by providers serving young children. Parents and professionals may observe a language delay--an "invisible" condition--while failing to identify the underlying cause. Otoacoustic emissions (OAE) hearing screening technology, used extensively with newborns, is becoming an essential tool,…

  17. Screening for Hearing Loss in Early Childhood Programs

    ERIC Educational Resources Information Center

    Eiserman, William D.; Shisler, Lenore; Foust, Terry; Buhrmann, Jan; Winston, Randi; White, Karl R.

    2007-01-01

    This study assessed the feasibility of doing hearing screening in Migrant, American Indian and Early Head Start programs using otoacoustic emissions (OAE) technology. Staff members were trained to screen 0-3-year-old children for hearing loss using hand-held OAE equipment and a multi-step screening and referral protocol. Of the 3486 children…

  18. Screening for Hearing Loss in Early Childhood Programs

    ERIC Educational Resources Information Center

    Eiserman, William D.; Shisler, Lenore; Foust, Terry; Buhrmann, Jan; Winston, Randi; White, Karl R.

    2007-01-01

    This study assessed the feasibility of doing hearing screening in Migrant, American Indian and Early Head Start programs using otoacoustic emissions (OAE) technology. Staff members were trained to screen 0-3-year-old children for hearing loss using hand-held OAE equipment and a multi-step screening and referral protocol. Of the 3486 children…

  19. 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.

  20. Features of unilateral hearing loss detected by newborn hearing screening programme in different regions of Turkey.

    PubMed

    Genç, Gülsüm Aydan; Konukseven, Ozlem; Muluk, Nuray Bayar; Kirkim, Günay; Başar, Figen Suren; Tuncer, Ulkü; Kayikci, Maviş Kulak; Bolat, Hilal; Topcu, Cigdem; Dizdar, Handan Turan; Kaynar, Feray; Akar, Funda; Ozdek, Ali; Serbetcioglu, Bülent; Belgin, Erol

    2013-06-01

    Newborn hearing screening (NHS) works well for babies with bilateral hearing loss. However, for those with unilateral loss, it has yet to be established some standard rules like age of diagnose, risk factors, hearing loss degree. The aim of this study is to identify the demographic characteristics of newborns with unilateral hearing loss to obtain evidence based data in order to see what to be done for children with unilateral hearing loss (UHL). Newborn hearing screening data of 123 babies with unilateral hearing loss, 71 (57.7%) male and 52 (42.3%) female, were investigated retrospectively. Data provided from the archives of six referral tertiary audiology centers from four regions in Turkey. Data, including type of hearing loss; age of diagnosis; prenatal, natal and postnatal risk factors; familial HL and parental consanguinity was analyzed in all regions and each of the Regions 1-4 separately. The difference between data obtained in terms of gender and type of hearing loss was detected as statistically significant (p<0.05). While UHL was significantly higher in females at Region 1, and in males at other Regions of 2-4; SNHL was the most detected type of UHL in all regions with the rate of 82.9-100.0%. There were not significant differences between regions in terms of the degree of hearing loss, presence of risk factors, family history of hearing loss, age at diagnosis and parental consanguinity (p>0.05). Diagnosis procedure was completed mostly at 3-6 months in Region 4; whereas, in other regions (Regions 1-3), completion of procedure was delayed until 6 months-1 year. This study indicates that the effect of postnatal risk factors, i.e. curable hyperbilirubinemia, congenital infection and intensive care is relatively high on unilateral hearing loss, precautions should be taken regarding their prevention, as well as physicians and other health personnel should be trained in terms of these risks. For early and timely diagnosis, families will be informed about

  1. Changed crossmodal functional connectivity in older adults with hearing loss.

    PubMed

    Puschmann, Sebastian; Thiel, Christiane M

    2017-01-01

    Previous work compellingly demonstrates a crossmodal plastic reorganization of auditory cortex in deaf individuals, leading to increased neural responses to non-auditory sensory input. Recent data indicate that crossmodal adaptive plasticity is not restricted to severe hearing impairments, but may also occur as a result of high-frequency hearing loss in older adults and affect audiovisual processing in these subjects. We here used functional magnetic resonance imaging (fMRI) to study the effect of hearing loss in older adults on auditory cortex response patterns as well as on functional connectivity between auditory and visual cortex during audiovisual processing. Older participants with a varying degree of high frequency hearing loss performed an auditory stimulus categorization task, in which they had to categorize frequency-modulated (FM) tones presented alone or in the context of matching or non-matching visual motion. A motion only condition served as control for a visual take-over of auditory cortex. While the individual hearing status did not affect auditory cortex responses to auditory, visual, or audiovisual stimuli, we observed a significant hearing loss-related increase in functional connectivity between auditory cortex and the right motion-sensitive visual area MT+ when processing matching audiovisual input. Hearing loss also modulated resting state connectivity between right area MT+ and parts of the left auditory cortex, suggesting the existence of permanent, task-independent changes in coupling between visual and auditory sensory areas with an increasing degree of hearing loss. Our data thus indicate that hearing loss impacts on functional connectivity between sensory cortices in older adults.

  2. 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.

  3. 38 CFR 20.717 - Rule 717. Loss of hearing tapes or transcripts-motion for new hearing.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 2 2010-07-01 2010-07-01 false Rule 717. Loss of hearing tapes or transcripts-motion for new hearing. 20.717 Section 20.717 Pensions, Bonuses, and Veterans... on Appeal § 20.717 Rule 717. Loss of hearing tapes or transcripts—motion for new hearing. (a) Motion...

  4. 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.

  5. [Sudden hearing loss and the cranio-cervical junction].

    PubMed

    Bernal Sprekelsen, M; Hörmann, K; Weh, L

    1990-01-01

    Morphological alterations of the craniocervical junction as basilar impressions, a ponticulus posterior, an atlas assimilation, an intervertebral narrowing and spondylosis deformans, were found radiologically in patients with sudden hearing loss. There were no radiological differences to a healthy population. No relationship could be established between static morphological changes of the craniocervical junction of the upper cervical spine and the sudden hearing loss. However, there was a statistically significant reduction of the mobility in the upper cervical spine in patients suffering from sudden hearing loss. Very high standard deviations in the atlanto-occipital and the atlanto-odontoid joints are interpreted as hyper- as well as hypomobile joints. These results indicate a possible correlation between sudden hearing loss and a functional pathology of the craniocervical junction.

  6. Acute transient sensorineural hearing loss due to Anaplasma phagocytophilum.

    PubMed

    Ghera, Princy; Kasirye, Yusuf; Choudhry, Muhammad Waqas; Shaw, Gene R; Ejercito, Victor S

    2011-12-01

    We report the case of a patient who presented with a 3-month history of random epistaxis and recent onset of acute hearing loss associated with fever, chills, and myalgias. Pure tone audiometry revealed bilateral sensorineural hearing loss. Complete blood cell count showed an abnormal neutrophil count of 700/uL (normal >1900/microL) and platelet count of 25 x 10(3)/microL (normal >175 x 10(3)/microL). Giemsa-stained peripheral blood smear revealed neutrophilic intracytoplasmic inclusion consistent with anaplasma morulae. Polymerase chain reaction confirmed Anaplasma phagocytophilum. The patient was treated with oral doxycycline, and, after 14 days of treatment, the hearing loss had improved markedly. Therefore, we concluded that the patient's acute transient bilateral sensorineural hearing loss was associated with anaplasmosis.

  7. Hearing loss among operating engineers in American construction industry.

    PubMed

    Hong, OiSaeng

    2005-08-01

    Occupational noise exposure and noise-induced hearing loss (NIHL) among construction workers has long been recognized as a problem in the United States, yet little is known about the prevalence of NIHL among American construction workers. The purpose of this study was to determine the prevalence and characteristics of hearing loss among operating engineers (OEs) who operate heavy construction machinery. As a part of hearing protection intervention, an audiometric test was conducted for both ears at frequencies 0.5 through 8 kHz in the soundproof booth. Prior to the audiometric test, a paper-pencil pre-hearing test questionnaire was administered and an otoscopic examination was completed. Prevalence of hearing loss was determined based on hearing threshold levels (HTLs) in the worst ear with a low fence of 25 dB. A total of 623 workers were included in the analysis and they were predominantly middle-aged Caucasian males (mean age = 43 years, Caucasian = 90%, male = 92%). Over 60% of OEs showed hearing loss in the noise-sensitive higher frequencies of 4 and 6 kHz. The rate of hearing loss was particularly higher among workers who reported longer years of working in the construction industry. Workers showed significantly poorer hearing in the left ear, and a typical characteristic of NIHL, a V-notch at 4 or 6 kHz, was not shown in this population. Thirty-eight percent reported ringing/buzzing in the ear and 62% indicated having problems in understanding what people say in loud noise. Average reported use of hearing protection devices (HPDs) was 48% of the time they were required to be used. Significant inverse relationship was found between higher frequency (4-6 kHz) hearing loss and use of HPDs (r =-0.134, p < 0.001). Workers using HPDs more had significantly better hearing than those who did not. The study demonstrated a significant NIHL problem and low use of HPDs in OEs. An effective hearing conservation program, including a periodic audiometric testing and

  8. Use of a hearing loss grading system and an owner-based hearing questionnaire to assess hearing loss in pet dogs with chronic otitis externa or otitis media.

    PubMed

    Mason, Carly L; Paterson, Susan; Cripps, Peter J

    2013-10-01

    Hearing loss is important when assessing the suitability of dogs with otitis externa/media for medical or surgical therapy. To assess an owner-completed questionnaire as an indicator of hearing loss and a canine hearing loss scoring system in chronic canine otitis. One hundred hospital population dogs referred for chronic otitis investigation. Owners completed a questionnaire to assess their dog's response to common household noises. The presence of otitis externa or media was determined and brainstem auditory-evoked response measurements were performed on each dog. The minimal hearing threshold (MHT) in decibels normal hearing level (dB NHL) was recorded and categorized according to the human World Health Organization grading system into five grades from 0 to 4 with cut-off values of ≤25 dB NHL, 26-40 dB NHL, 41-60 dB NHL, 60-80 dB NHL and ≥81 dB NHL. The questionnaire correctly determined normal hearing in grade 0 cases, but did not reliably detect unilateral or grade 1 bilateral hearing loss. For dogs with bilateral hearing loss ≥ grade 2, questionnaire sensitivity was 83% [24 of 29, 95% confidence interval, (CI) 64-94%] and specificity was 94% (67 of 71, 95% CI 86-98%). Higher grades of hearing loss were significantly associated with the presence of otitis media (P < 0.01). The questionnaire may be a useful in-practice screening tool in chronic canine otitis for moderate to severe bilateral hearing deficits (MHT ≥41 dB NHL). The hearing loss grading system may help clinicians make therapeutic decisions. Chronic otitis media may be associated with higher grades of hearing loss. © 2013 ESVD and ACVD.

  9. Auditory Evoked Potential Response and Hearing Loss: A Review

    PubMed Central

    Paulraj, M. P; Subramaniam, Kamalraj; Yaccob, Sazali Bin; Adom, Abdul H. Bin; Hema, C. R

    2015-01-01

    Hypoacusis is the most prevalent sensory disability in the world and consequently, it can lead to impede speech in human beings. One best approach to tackle this issue is to conduct early and effective hearing screening test using Electroencephalogram (EEG). EEG based hearing threshold level determination is most suitable for persons who lack verbal communication and behavioral response to sound stimulation. Auditory evoked potential (AEP) is a type of EEG signal emanated from the brain scalp by an acoustical stimulus. The goal of this review is to assess the current state of knowledge in estimating the hearing threshold levels based on AEP response. AEP response reflects the auditory ability level of an individual. An intelligent hearing perception level system enables to examine and determine the functional integrity of the auditory system. Systematic evaluation of EEG based hearing perception level system predicting the hearing loss in newborns, infants and multiple handicaps will be a priority of interest for future research. PMID:25893012

  10. Analysis of Predisposing Factors for Hearing Loss in Adults.

    PubMed

    Lee, Joong Seob; Choi, Hyo Geun; Jang, Jeong Hun; Sim, Songyong; Hong, Sung Kwang; Lee, Hyo-Jeong; Park, Bumjung; Kim, Hyung-Jong

    2015-08-01

    We aimed to estimate the effects of various risk factors on hearing level in Korean adults, using data from the Korea National Health and Nutrition Examination Survey. We examined data from 13,369 participants collected between 2009 and 2011. Average hearing thresholds at low (0.5, 1, and 2 kHz) and high frequencies (3, 4, and 6 kHz), were investigated in accordance with various known risk factors via multiple regression analysis featuring complex sampling. We additionally evaluated data from 4,810 participants who completed a questionnaire concerned with different types of noise exposure. Low body mass index, absence of hyperlipidemia, history of diabetes mellitus, low incomes, low educational status, and smoking were associated with elevated low frequency hearing thresholds. In addition, male sex, low body mass index, absence of hyperlipidemia, low income, low educational status, smoking, and heavy alcohol consumption were associated with elevated high frequency hearing thresholds. Participants with a history of earphone use in noisy circumstances demonstrated hearing thresholds which were 1.024 dB (95% CI: 0.176 to 1.871; P = 0.018) higher, at low-frequencies, compared to participants without a history of earphone use. Our study suggests that low BMI, absence of hyperlipidemia, low household income, and low educational status are related with hearing loss in Korean adults. Male sex, smoking, and heavy alcohol use are related with high frequency hearing loss. A history of earphone use in noisy circumstances is also related with hearing loss.

  11. Vascular risk factors in sudden hearing loss.

    PubMed

    Rudack, Claudia; Langer, Claus; Stoll, Wolfgang; Rust, Stephan; Walter, Michael

    2006-03-01

    Low density lipoprotein (LDL) and fibrinogen apheresis was recently reported to be an effective therapy in sudden hearing loss (SHL). In this study, we investigated whether lipoprotein and/or fibrinogen plasma concentrations, related gene polymorphisms and other cardiovascular risk factors are also risk factors for SHL. Total cholesterol, HDL and LDL cholesterol plasma concentrations, fibrinogen levels, and two functionally relevant fibrinogen polymorphisms were determined in 142 consecutive patients and in 84 age- and sex-matched control subjects of the same ethnic background, using routine laboratory methods and PCR analysis. In addition, we determined the platelet glycoprotein Ia (GPIa) C807T polymorphism, which was recently proposed to be a genetic risk factor for SHL, and we compared the patients' and controls' clinical characteristics. Total and LDL cholesterol concentrations were not different between patients and controls. Fibrinogen plasma levels were significantly increased in SHL patients (260+/-57 vs. 239+/-110 mg/dl, p=0.002). However, fibrinogen was not related to SHL in multivariate analysis, and none of the investigated fibrinogen polymorphisms was associated with SHL. By contrast, T allele carriers of the GPIa 807 polymorphic site had an increased risk to develop SHL (OR 1.81) and were more likely not to recover from SHL, compared to C allele carriers (OR 3.0). Moreover, significantly more SHL patients were current smokers (56.3% vs. 19.3% in the control group, p<0.0001). In conclusion, there is a partial overlap between classical coronary risk factors and risk factors for SHL. Hypercholesterolemia and hypoalphalipoproteinemia (low HDL cholesterol levels) are apparently no major risk factors for SHL, whereas the GPIa C807T polymorphism, elevated fibrinogen levels, and smoking are associated with an increased risk for SHL. Altogether these findings suggest a vascular involvement in the pathogenesis of SHL and may have important implications for the

  12. Automatic hearing loss detection system based on auditory brainstem response

    NASA Astrophysics Data System (ADS)

    Aldonate, J.; Mercuri, C.; Reta, J.; Biurrun, J.; Bonell, C.; Gentiletti, G.; Escobar, S.; Acevedo, R.

    2007-11-01

    Hearing loss is one of the pathologies with the highest prevalence in newborns. If it is not detected in time, it can affect the nervous system and cause problems in speech, language and cognitive development. The recommended methods for early detection are based on otoacoustic emissions (OAE) and/or auditory brainstem response (ABR). In this work, the design and implementation of an automated system based on ABR to detect hearing loss in newborns is presented. Preliminary evaluation in adults was satisfactory.

  13. [Two formulas for exact calculation of hearing loss for numbers].

    PubMed

    Braun, T; Wimmer, M; Hempel, J M

    2012-09-01

    In Germany assessment of hearing loss for numbers is used to calculate the percentage hearing loss from speech audiometry and for plausibility checking with pure tone thresholds. It is common practice to take a graphical reading from the speech audiogram to determine the hearing loss for numbers. This study searches for a mathematical formula for the exact calculation of the hearing loss for numbers from the intelligibility values measured. With analytical methods on the basis of the standard curve following DIN 45626-1, two simple formulas for the calculation of the hearing loss for numbers were developed. The hearing loss for numbers a(1) is calculated as a(1)=p(1)+(50-v(1)) (p(2)-p(1))/(v(2)-v(1))-18,4 for two available measured values and as a(1)=p(1)-0,13 v(1)-11,9 if only one measured value is used, with v(i )being number intelligibility in percent at the level p(i) in decibels (dB) of measurement number i. Number intelligibility of all inserted pairs of values must be between 30% and 70% because the standard curve of DIN 45626-1 runs approximately linearly only in this range. The calculated value for the hearing loss for numbers is subsequently mathematically rounded up to 5 dB as well as for the conventional graphically determined value. With the presented formulas the hearing loss for numbers can be calculated exactly from the measured values of the Freiburg number test especially in matters of expertise if the conventional graphical determination of this value does not seem to be unambiguous.

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

    PubMed

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

    2009-09-01

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

  15. Controlling Mitochondrial Dynamics to Mitigate Noise-Induced Hearing Loss

    DTIC Science & Technology

    2016-10-01

    of excessive ROS by mitochondria in many different tissues of the inner ear is well recognized. Mitochondrial dysfunction, including the...of reactive oxygen species. 2. KEYWORDS Hearing loss, loud sound, mitochondria , reactive oxygen species, dynamin-related protein-1... mitochondria is an underlying mechanism of noise-induced damage to tissues in the inner ear that leads to noise-induced loss of hearing sensitivity. Our

  16. 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

  17. "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.

  18. Hearing Loss: Communicating With the Patient Who Is Deaf or Hard of Hearing.

    PubMed

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

    2015-07-01

    Hearing loss impairs health care communication and adversely affects patient satisfaction, treatment adherence, and use of health services. Hearing loss is the third most common chronic health condition among older patients after hypertension and arthritis, but only 15% to 18% of older adults are screened for hearing loss during health maintenance examinations. Patients with hearing loss may be reluctant to disclose it because of fear of ageism, perceptions of disability, and vanity. Lipreading and note writing often are ineffective ways to communicate with deaf and hard of hearing (DHH) patients who use American Sign Language; use of medical sign language interpreters is preferred. A variety of strategies can improve the quality of health care communication for DHH patients, such as the physician facing the patient, listening attentively, and using visual tools. Physicians should learn what hearing loss means to the DHH patient. Deaf American Sign Language users may not perceive hearing loss as a disability but as a cultural identity. Patients' preferred communication strategies will vary. Relay services, electronic communication, and other telecommunications methods can be helpful, but family physicians and medical staff should learn from each DHH patient about which communication strategies will work best.

  19. Bilateral sensorineural hearing loss and labyrinthitis ossificans secondary to neurosarcoidosis.

    PubMed

    Dhanjal, Hardeep; Rainsbury, James; Irving, Richard M

    2014-11-01

    To report a case of cochlear ossification as a result of neurosarcoidosis in a patient with bilateral profound sensorineural hearing loss. Case report: Setting University teaching hospital, tertiary referral center. Forty-year-old man with neurosarcoidosis and bilateral profound sensorineural hearing loss. Intervention Unilateral cochlear implantation. Aided thresholds speech perception tests. Marked improvement in hearing following implant. Patients with neurosarcoidosis are at risk of labyrinthitis ossificans. Early imaging of these patients is recommended and if early cochlear ossification is identified they should be offered rapid access to rehabilitation with a cochlear implant.

  20. Managing hearing loss in children and adults: Singapore context.

    PubMed

    Low, W K

    2005-05-01

    Hearing loss is common in Singapore, with as many as 360,000 persons affected. With Singapore's ageing population, this prevalence is expected to increase. Modern hearing devices have the potential to restore hearing in most patients. Cochlear implants can restore hearing in those with even profound total hearing loss, by stimulating the auditory nerve directly without the need for viable cochlear hair cells. A holistic approach to managing deafness is necessary, including addressing psychosocial issues. Many of the problems experienced by the hearing-impaired are caused by the ignorance and lack of understanding from other people around him/her. "Reverse education therapy" refers to the effective education of these people, to directly benefit the hearing-impaired. In children, hearing restoration together with early detection and effective rehabilitation enable up to 80% to 90% of those born with significant hearing loss to cope well in mainstream education. The infrastructure to support them and facilitate their integration into mainstream society is necessary, and some of these are in place in Singapore. Leisure time noise-induced hearing loss in children is becoming increasingly important. In adults, societal support and understanding are just as important, particularly towards the elderly and pre-lingually deafened adults. Public education in Singapore is necessary, including that on the recognition of the abilities of successfully treated individuals, as well as that on showing more compassion, support and patience towards those who have not benefited sufficiently from treatment. In the future, there is potential for the clinical application of cochlear hair cell/auditory nerve preservation and regeneration.

  1. Age-related hearing loss increases cross-modal distractibility.

    PubMed

    Puschmann, Sebastian; Sandmann, Pascale; Bendixen, Alexandra; Thiel, Christiane M

    2014-10-01

    Recent electrophysiological studies have provided evidence that changes in multisensory processing in auditory cortex cannot only be observed following extensive hearing loss, but also in moderately hearing-impaired subjects. How the reduced auditory input affects audio-visual interactions is however largely unknown. Here we used a cross-modal distraction paradigm to investigate multisensory processing in elderly participants with an age-related high-frequency hearing loss as compared to young and elderly subjects with normal hearing. During the experiment, participants were simultaneously presented with independent streams of auditory and visual input and were asked to categorize either the auditory or visual information while ignoring the other modality. Unisensory sequences without any cross-modal input served as control conditions to assure that all participants were able to perform the task. While all groups performed similarly in these unisensory conditions, hearing-impaired participants showed significantly increased error rates when confronted with distracting cross-modal stimulation. This effect could be observed in both the auditory and the visual task. Supporting these findings, an additional regression analysis indicted that the degree of high-frequency hearing loss significantly modulates cross-modal visual distractibility in the auditory task. These findings provide new evidence that already a moderate sub-clinical hearing loss, a common phenomenon in the elderly population, affects the processing of audio-visual information.

  2. (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.

  3. Vowel production of Mandarin-speaking hearing aid users with different types of hearing loss

    PubMed Central

    Lee, Ya-Jung; Tsai, Li-Chiun

    2017-01-01

    In contrast with previous research focusing on cochlear implants, this study examined the speech performance of hearing aid users with conductive (n = 11), mixed (n = 10), and sensorineural hearing loss (n = 7) and compared it with the speech of hearing control. Speech intelligibility was evaluated by computing the vowel space area defined by the Mandarin Chinese corner vowels /a, u, i/. The acoustic differences between the vowels were assessed using the Euclidean distance. The results revealed that both the conductive and mixed hearing loss groups exhibited a reduced vowel working space, but no significant difference was found between the sensorineural hearing loss and normal hearing groups. An analysis using the Euclidean distance further showed that the compression of vowel space area in conductive hearing loss can be attributed to the substantial lowering of the second formant of /i/. The differences in vowel production between groups are discussed in terms of the occlusion effect and the signal transmission media of various hearing devices. PMID:28575087

  4. Perception of Speech Simulating Different Configurations of Hearing Loss in Normal Hearing Individuals

    ERIC Educational Resources Information Center

    Kumar, Prawin; Yathiraj, Asha

    2009-01-01

    The present study aimed at assessing perception of filtered speech that simulated different configurations of hearing loss. The simulation was done by filtering four equivalent lists of a monosyllabic test developed by Shivaprasad for Indian-English speakers. This was done using the Adobe Audition software. Thirty normal hearing participants in…

  5. Hearing Parents of Children with Hearing Loss: Perceptions of the IEP Process

    ERIC Educational Resources Information Center

    Stegman, Robin Fern

    2016-01-01

    Under federal guidelines, parents of school-aged children with hearing loss are required to attend an individualized education program (IEP) meeting on behalf of their child. However, it remains unclear how prepared hearing parents are to oversee development of IEPs that guarantee their children the best educational outcomes, as well as how much…

  6. Prevalence of hearing loss among people aged 65 years and over: screening and hearing aid provision.

    PubMed Central

    Wilson, P S; Fleming, D M; Donaldson, I

    1993-01-01

    The aim of this study was to assess the prevalence of hearing loss among people aged 65 years and over and to offer hearing aids where necessary. The study was carried out in a large health centre practice in Birmingham with a computerized record system. All patients aged 65 years, and a 20% random sample of patients aged 66 years and over were invited for interview. They were questioned about hearing loss and examined audiometrically. All patients with a hearing loss in the better ear of 35 decibels or more over the speech frequencies (0.5-4 kHz) were offered a hearing aid. A total of 322 patients attended (72% of those invited) and of these 34 patients already had a hearing aid. A further 142 patients were identified for whom an aid was recommended and 69 of these accepted. The acceptance rate was higher among men than women (57% compared with 43%). Sixty one patients (19% of those screened) had an asymmetrical hearing loss and of these 24 required consultant assessment; none had serious underlying pathology. There is a large unmet need for hearing aid provision. Simple audiometric assessment in health centres provides an opportunity to meet this need. PMID:8260217

  7. Predictors of Hearing Aid Use Time in Children with Mild-to-Severe Hearing Loss

    ERIC Educational Resources Information Center

    Walker, Elizabeth A.; Spratford, Meredith; Moeller, Mary Pat; Oleson, Jacob; Ou, Hua; Roush, Patricia; Jacobs, Shana

    2013-01-01

    Purpose: This study investigated predictors of hearing aid (HA) use time for children with mild-to-severe hearing loss (HL). Barriers to consistent HA use and reliability of parent report measures were also examined. Method: Participants included parents of 272 children with HL. Parents estimated the amount of time the child used HAs daily.…

  8. Hearing Parents of Children with Hearing Loss: Perceptions of the IEP Process

    ERIC Educational Resources Information Center

    Stegman, Robin Fern

    2016-01-01

    Under federal guidelines, parents of school-aged children with hearing loss are required to attend an individualized education program (IEP) meeting on behalf of their child. However, it remains unclear how prepared hearing parents are to oversee development of IEPs that guarantee their children the best educational outcomes, as well as how much…

  9. Predictors of Hearing Aid Use Time in Children with Mild-to-Severe Hearing Loss

    ERIC Educational Resources Information Center

    Walker, Elizabeth A.; Spratford, Meredith; Moeller, Mary Pat; Oleson, Jacob; Ou, Hua; Roush, Patricia; Jacobs, Shana

    2013-01-01

    Purpose: This study investigated predictors of hearing aid (HA) use time for children with mild-to-severe hearing loss (HL). Barriers to consistent HA use and reliability of parent report measures were also examined. Method: Participants included parents of 272 children with HL. Parents estimated the amount of time the child used HAs daily.…

  10. The Influence of Hearing Aid Use on Outcomes of Children with Mild Hearing Loss

    ERIC Educational Resources Information Center

    Walker, Elizabeth A.; Holte, Lenore; McCreery, Ryan W.; Spratford, Meredith; Page, Thomas; Moeller, Mary Pat

    2015-01-01

    Purpose: This study examined the effects of consistent hearing aid (HA) use on outcomes in children with mild hearing loss (HL). Method: Five- or 7-year-old children with mild HL were separated into 3 groups on the basis of patterns of daily HA use. Using analyses of variance, we compared outcomes between groups on speech and language tests and a…

  11. The Influence of Hearing Aid Use on Outcomes of Children with Mild Hearing Loss

    ERIC Educational Resources Information Center

    Walker, Elizabeth A.; Holte, Lenore; McCreery, Ryan W.; Spratford, Meredith; Page, Thomas; Moeller, Mary Pat

    2015-01-01

    Purpose: This study examined the effects of consistent hearing aid (HA) use on outcomes in children with mild hearing loss (HL). Method: Five- or 7-year-old children with mild HL were separated into 3 groups on the basis of patterns of daily HA use. Using analyses of variance, we compared outcomes between groups on speech and language tests and a…

  12. Perception of Speech Simulating Different Configurations of Hearing Loss in Normal Hearing Individuals

    ERIC Educational Resources Information Center

    Kumar, Prawin; Yathiraj, Asha

    2009-01-01

    The present study aimed at assessing perception of filtered speech that simulated different configurations of hearing loss. The simulation was done by filtering four equivalent lists of a monosyllabic test developed by Shivaprasad for Indian-English speakers. This was done using the Adobe Audition software. Thirty normal hearing participants in…

  13. 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.

  14. 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.

  15. [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.

  16. Aberrant Cx26 hemichannels and keratitis-ichthyosis-deafness syndrome: insights into syndromic hearing loss

    PubMed Central

    Sanchez, Helmuth A.; Verselis, Vytas K.

    2014-01-01

    Mutation of the GJB2 gene, which encodes the connexin 26 (Cx26) gap junction (GJ) protein, is the most common cause of hereditary, sensorineural hearing loss. Cx26 is not expressed in hair cells, but is widely expressed throughout the non-sensory epithelial cells of the cochlea. Most GJB2 mutations produce non-syndromic deafness, but a subset produces syndromic deafness in which profound hearing loss is accompanied by a diverse array of infectious and neoplastic cutaneous disorders that can be fatal. Although GJ channels, which are assembled by the docking of two, so-called hemichannels (HCs), have been the main focus of deafness-associated disease models, it is now evident that the HCs themselves can function in the absence of docking and contribute to signaling across the cell membrane as a novel class of ion channel. A notable feature of syndromic deafness mutants is that the HCs exhibit aberrant behaviors providing a plausible basis for disease that is associated with excessive or altered contributions of Cx26 HCs that, in turn, lead to compromised cell integrity. Here we discuss some of the aberrant Cx26 HC properties that have been described for mutants associated with keratitis-ichthyosis-deafness (KID) syndrome, a particularly severe Cx26-associated syndrome, which shed light on genotype-phenotype relationships and causes underlying cochlear dysfunction. PMID:25386120

  17. 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

    2017-05-04

    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.

  18. Application of SNPscan in Genetic Screening for Common Hearing Loss Genes

    PubMed Central

    Ke, Jia; Li, Tao; Hu, Ping; Song, Yu; Xu, Chiyu; Wang, Jie; Cheng, Jing; Zhang, Lei; Duan, Hong; Yuan, Huijun; Ma, Furong

    2016-01-01

    The current study reports the successful application of a fast and efficient genetic screening system for common hearing loss (HL) genes based on SNPscan genotyping technology. Genetic analysis of 115 variants in common genes related to HL, GJB2, SLC26A4 and MT-RNR, was performed on 695 subjects with non-syndromic hearing loss (NSHL) from the Northern China. The results found that 38.7% (269/695) of cases carried bi-allelic pathogenic variants in GJB2 and SLC26A4 and 0.7% (5/695) of cases carried homoplasmic MT-RNR1 variants. The variant allele frequency of GJB2, SLC26A4 and MT-RNR1 was 19.8% (275/1390), 21.9% (304/1390), and 0.86% (6/695), respectively. This approach can explain ~40% of NSHL cases and thus is a useful tool for establishing primary molecular diagnosis of NSHL in clinical genetics. PMID:27792752

  19. [Analyses prognostic factors relevant to sudden sensorineural hearing loss].

    PubMed

    Wang, Jun; Xiao, Shuifang; Zeng, Zhengang; Zhen, Zhen; Zhang, Xuexi; Lin, Feng; Dong, Mingmin; Lu, Wei; Qin, Zhaobing; Zuo, Bin; Bai, Xianfeng

    2015-06-01

    To investigate the prognostic factors relevant to sudden sensorineural hearing loss. The internationally accepted standardized clinical research methods, unified design, and unified program were adopted to conduct the prospective clinical multi-center study. The sudden deafness patients between 18 to 65 years old, with the course of this disorder less than two weeks, and without any medical treatments were collected, and then, divided into four types according to the hearing curve: type A, acute sensorineural hearing loss in low tone frequencies; type B, acute sensorineural hearing loss in high tone frequencies; type C, acute sensorineural hearing loss in all frequencies; and type D, total deafness. The factors, in terms of age, gender, type of initial audiogram, time delay before the first visit, and severity of hearing loss, were included in the analyses. A total of 1 024 cases with single side sudden deafness were collected in the study from 33 hospitals in China from August 2007 to October 2011, inclusive of for 492 males (48.05%) and 532 females (51.95%). The average age was (41.2 ± 12.8) years old. There were 553 cases (54.00%) in left ear, and 471 cases (46.00%) in right ear. The curative effects of different types were shown as follows: the type in low tone frequencies had the highest rate of 90.73%, the type in all frequencies was 82.59%; the type of total deafness was 70.29%; and the type in high tone frequencies had the lowest rate of 65.96%. It had significant difference of the effective rate between different types (χ(2) = 231.58, P = 0.000). Age, time delay before first visit, and severity of initial hearing loss were significantly correlated with hearing improvement. Initial audiogram of SSNHL might predict hearing recovery. The young in age and a short time delay before starting treatment are positive prognostic factors for hearing recovery in SSNHL. The initial severity of hearing loss is negative prognostic factor of hearing recovery.

  20. 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.

  1. Prevalence of hearing loss in the United States by industry.

    PubMed

    Masterson, Elizabeth A; Tak, SangWoo; Themann, Christa L; Wall, David K; Groenewold, Matthew R; Deddens, James A; Calvert, Geoffrey M

    2013-06-01

    Twenty-two million workers are exposed to hazardous noise in the United States. The purpose of this study is to estimate the prevalence of hearing loss among U.S. industries. We examined 2000-2008 audiograms for male and female workers ages 18-65, who had higher occupational noise exposures than the general population. Prevalence and adjusted prevalence ratios (PRs) for hearing loss were estimated and compared across industries. In our sample, 18% of workers had hearing loss. When compared with the Couriers and Messengers industry sub-sector, workers employed in Mining (PR = 1.65, CI = 1.57-1.73), Wood Product Manufacturing (PR = 1.65, CL = 1.61-1.70), Construction of Buildings (PR = 1.52, CI = 1.45-1.59), and Real Estate and Rental and Leasing (PR = 1.61, CL = 1.51-1.71) [corrected] had higher risks for hearing loss. Workers in the Mining, Manufacturing, and Construction industries need better engineering controls for noise and stronger hearing conservation strategies. More hearing loss research is also needed within traditional "low-risk" industries like Real Estate. Copyright © 2012 Wiley Periodicals, Inc.

  2. Hearing loss in patients with systemic lupus erythematosus.

    PubMed

    Abbasi, Mahnaz; Yazdi, Zohreh; Kazemifar, Amir Mohammad; Bakhsh, Zahra Zarin

    2013-06-15

    Systemic lupus erythematosus has its unique complications which warrant careful examination and assessment during follow/up visits of patients. The present study was conducted to evaluate prevalence of hearing loss in patients with SLE. At present a case- control study has been performed on 45 patients with SLE in a clinic of a teaching university hospital, Qazvin city, Iran. The patients were examined and evaluated for auditory and hearing problems as well as parameters related to their disease severity and progression. The control group was selected from the same clinic. Five patients (11.1%) complained from hearing loss, 4 patients s (8.9%) complained from otorrhea, 3 patients (6.7%) had tinnitus in research group, moreover twelve patients (26.7%) in case group and 4 patients (8.9%) in control group had sensorineural hearing loss. The difference was found to be statistically significant. No statistical significant relationship was found between severity, age of onset, and duration of the disease, and the lab tests of the patients with hearing loss. The present study implies that patients with systemic lupus erythematosus may develop sensorineural hearing loss during their course of the disease. It is recommended that audiology examination and/or audiometry become a part of routine follow/up studies of the patients.

  3. Noise-induced hearing loss in French police officers.

    PubMed

    Lesage, François-Xavier; Jovenin, Nicolas; Deschamps, Frederic; Vincent, Samuel

    2009-10-01

    There is a lack of data about police officers' hearing thresholds and the risk of noise-induced hearing loss (NIHL) associated with this occupation. In France, 129,000 national police officers, 96,000 state police force members and 16000 municipal police officers may be affected by occupational noise exposure. To evaluate the association between police employment and NIHL. We undertook a cross-sectional study using review of medical records. Audiometric and otological data and information on potential confounders were extracted from medical records. Global hearing loss and selective 4000 Hz hearing loss were analysed. Of total, 1692 subjects (887 policemen and 805 civil servants) participated in the study. After adjusting for potential cofounders, police officers were 1.4 times more likely to have a selective 4000 Hz hearing loss than civil servants (95% CI 1.1-1.9). This difference was greater between motorcycle police officers and civil servants (OR = 3; 95% CI 1.4-6.3). These data suggest that occupational noise exposure in police work, particularly in motorcycle police officers, may induce hearing loss. Noise sources need to be more accurately defined to confirm high-level noise exposures, to better define significant sources of noise and to identify effective solutions.

  4. Perceptual Consequences of “Hidden” Hearing Loss

    PubMed Central

    Barker, Daphne; Prendergast, Garreth

    2014-01-01

    Dramatic results from recent animal experiments show that noise exposure can cause a selective loss of high-threshold auditory nerve fibers without affecting absolute sensitivity permanently. This cochlear neuropathy has been described as hidden hearing loss, as it is not thought to be detectable using standard measures of audiometric threshold. It is possible that hidden hearing loss is a common condition in humans and may underlie some of the perceptual deficits experienced by people with clinically normal hearing. There is some evidence that a history of noise exposure is associated with difficulties in speech discrimination and temporal processing, even in the absence of any audiometric loss. There is also evidence that the tinnitus experienced by listeners with clinically normal hearing is associated with cochlear neuropathy, as measured using Wave I of the auditory brainstem response. To date, however, there has been no direct link made between noise exposure, cochlear neuropathy, and perceptual difficulties. Animal experiments also reveal that the aging process itself, in the absence of significant noise exposure, is associated with loss of auditory nerve fibers. Evidence from human temporal bone studies and auditory brainstem response measures suggests that this form of hidden loss is common in humans and may have perceptual consequences, in particular, regarding the coding of the temporal aspects of sounds. Hidden hearing loss is potentially a major health issue, and investigations are ongoing to identify the causes and consequences of this troubling condition. PMID:25204468

  5. Effects of shift work on noise-induced hearing loss.

    PubMed

    Chou, Yu-Fung; Lai, Jim-Shoung; Kuo, Hsien-Wen

    2009-01-01

    Evidence has accumulated concerning the adverse effects of noise on hearing acuity, but it is not clear whether working shifts may decelerate the effects of hearing loss. The objective of this study is to assess the effects of shift work on hearing loss in a noisy work environment. A sample of 218 male workers recruited at a semiconductor factory with no known occupational hazards that affected hearing acuity other than noise was chosen. The subjects worked either in an eight-hour or 12-hour shift. A standardized audiometric procedure was performed by a qualified audiologist to measure pure-tone hearing thresholds at 0.5 kHz, 1 kHz, 2 kHz, 3 kHz, 4 kHz, 6 kHz and 8 kHz in both ears. Using multiple linear regression adjusted for age, smoking habits, and work duration, the results showed that the severity of hearing loss in both ears was significantly lower in subjects who worked a 12-hour shift. In conclusion, working a 12-hour shift followed by a day off is best for workers and hearing protection should be provided in high noise areas.

  6. Hearing loss in the elderly: History of occupational noise exposure

    PubMed Central

    Meneses-Barriviera, Caroline Luiz; Melo, Juliana Jandre; Marchiori, Luciana Lozza de Moraes

    2013-01-01

    Summary Introduction: Noise exposure is one of the most common health risk factors, and workers are exposed to sound pressure levels capable of producing hearing loss. Aim: To assess the prevalence of hearing loss in the elderly and its possible association with a history of occupational noise exposure and with sex. Methods: A prospective study in subjects aged over 60 years. The subjects underwent anamnesis and audiological assessment. The Mann–Whitney test and multiple logistic regression, with 95% confidence interval and p < 0.05, were used for statistical analysis. Results: There were 498 subjects from both sexes, and the median age was 69 years. From the comparison between men and women, we obtained the medium hearing I (500, 1000, and 2000 Hz p = 0.8318) and the mean hearing II (3000, 4000, and 6000 Hz; p < 0.0001). Comparing the thresholds of individuals with and without a history of occupational noise exposure, we obtained the medium hearing I (p = 0.9542) and the mean hearing II (p = 0.0007). Conclusion: There was a statistically significant association between hearing loss at high frequencies and the risk factors being male and occupational noise exposure. PMID:25992010

  7. Oral steroid treatment for idiopathic sudden sensorineural hearing loss

    PubMed Central

    Chen, Wei T.; Lee, Jui W.; Yuan, Chien H.; Chen, Rong F.

    2015-01-01

    Objectives: To describe the efficacy of long-term oral steroids in idiopathic sudden sensorineural hearing loss (ISSHL), and to explore potential prognosis factors, the relationship of hearing recovery outcome, and the recovery time-course in ISSHL. Methods: In this retrospective study, we analyzed 215 cases diagnosed with idiopathic unilateral sudden deafness between January 2003 and December 2012 at a regional hospital in southern Taiwan. All of them received oral steroid therapy and were followed for at least 3 months. Results: Young age, the presence of no other disease (diabetes, hypertension, or cardiovascular disease), treatment within 7 days of the onset of ISSHL, mild hearing loss, and audiograms with ascending curves had a statistically significant better hearing recovery. The average pure-tone audiometry (PTA) was 67.8±23.9 dB initially, and was improved between one month (51.6±28.7 dB) and 2 months post treatment (49.7±28.6 dB). The average last-visit PTA was 49.9±29.2 dB. Conclusion: Approximately one third of patients had full recovery in hearing, one third had partial recovery, and approximately one third did not recover from hearing loss. In addition, the hearing level remained relatively stable following 2 months of treatment. More than 2 months of oral steroid therapy for ISSHL is not recommended because no additional benefits were evidenced. PMID:25737170

  8. Prognostic predictors of sudden sensorineural hearing loss in defibrinogenation therapy.

    PubMed

    Oya, Ryohei; Horii, Arata; Akazawa, Hitoshi; Osaki, Yasuhiro; Inohara, Hidenori

    2016-01-01

    Defibrinogenation therapy rather than corticosteroids therapy should be chosen for patients specifically with profound hearing loss and with initial high fibrinogen. Corticosteroids therapy is the standard treatment for sudden sensorineural hearing loss (SSNHL) and prognostic factors by this therapy were reported. Defibrinogenation therapy is one of the treatment options for SSNHL. Aims of this study were to identify prognostic factors and correlative markers with hearing improvement in treating SSNHL by defibrinogenation therapy. During the early phase of the study, consecutive 61 patients were treated by defibrinogenation therapy with batroxobin (50 units), whereas corticosteroids (500 mg/day of hydrocortisone tapered by 9 days) were used for consecutive 64 patients during the late phase. Blood data that could predict a complete recovery were identified. Coagulation/fibrinolysis markers correlated with hearing improvement by defibrinogenation therapy were investigated. Although there were no overall differences in hearing improvement between the two therapies, recovery rate in profound hearing loss patients was better in defibrinogenation therapy. In patients who showed complete recovery, serum fibrinogen level before treatment was significantly higher in the defibrinogenation group than the corticosteroid group. Responses of several fibrinolysis markers to defibrinogenation therapy evaluated by post-/pre-values were negatively correlated with hearing improvement.

  9. Evidence of hearing loss in a 'normally-hearing' college-student population.

    PubMed

    Le Prell, C G; Hensley, B N; Campbell, K C M; Hall, J W; Guire, K

    2011-03-01

    We report pure-tone hearing threshold findings in 56 college students. All subjects reported normal hearing during telephone interviews, yet not all subjects had normal sensitivity as defined by well-accepted criteria. At one or more test frequencies (0.25-8 kHz), 7% of ears had thresholds ≥25 dB HL and 12% had thresholds ≥20 dB HL. The proportion of ears with abnormal findings decreased when three-frequency pure-tone-averages were used. Low-frequency PTA hearing loss was detected in 2.7% of ears and high-frequency PTA hearing loss was detected in 7.1% of ears; however, there was little evidence for 'notched' audiograms. There was a statistically reliable relationship in which personal music player use was correlated with decreased hearing status in male subjects. Routine screening and education regarding hearing loss risk factors are critical as college students do not always self-identify early changes in hearing. Large-scale systematic investigations of college students' hearing status appear to be warranted; the current sample size was not adequate to precisely measure potential contributions of different sound sources to the elevated thresholds measured in some subjects.

  10. Severe hearing loss in Dlxl mutant mice.

    PubMed

    Polley, Daniel B; Cobos, Inma; Merzenich, Michael M; Rubenstein, John L R

    2006-04-01

    The Dlx homeobox gene family participates in regulating middle and inner ear development. A significant role for Dlxl, in particular,has been demonstrated in the development of the middle ear ossicles, but the functional consequences of Dlx.l gene mutation on hearing thresholds has not been assessed. The present study characterizes auditory brainstem responses to click and tonal stimuli in a non-lethal variant of a Dlxl gene knockout. We found that peripheral hearing thresholds for click and tonal stimuli were significantly elevated in homozygous Dlxl knockout (Dlxl-/ ) compared to both heterozygous (Dlxl+/ ) and wild type (Dlxl+/+) mice. Thus, abnormal mor-phogenesis of the incus and stapes that has been documented previously with histological measures is now known to result in a severe peripheral hearing deficit.

  11. [New aspects of hearing aid fitting in noise-induced hearing loss].

    PubMed

    Kiessling, J

    2006-07-01

    In the past hearing aid fitting frequently turned out to be a problem in patients with noise-induced hearing loss. Selective amplification in the high frequency range and at the same time natural sound and appropriate wearing comfort (open fitting) could not be achieved in numerous cases. Today these problems can be tackled by modern hearing aid technology providing us with efficient feedback suppression algorithms making open fittings possible for many more patients. This development is particularly beneficial for patients with noise-induced hearing loss. Unfortunately, open fitting is in opposition to wearing hearing aids at noisy workplaces. Tight fittings, however, can be used at work if a special listening program for noisy conditions is available. This dilemma is discussed and possible solutions are pointed out.

  12. Behavioral counselling for subjects with acquired hearing loss. A new approach to hearing tactics.

    PubMed

    Andersson, G; Melin, L; Scott, B; Lindberg, P

    1994-01-01

    Effects of behavioral counselling of subjects with an acquired hearing loss were evaluated in an experimental design. The study included a group of 20 hearing-impaired subjects who were assessed and randomly allocated to a treatment or control group. Behavioural counselling, individualized according to the results of functional analysis, was then given to 10 subjects and included the teaching of 'hearing tactics'. The groups were measured three times, pre-counselling and post-counselling and at a 15-month follow-up, after which treatment results were analysed. Significant (time x treatment) interactions were followed by post-hoc testing revealing that the treated group had improved in several areas of functioning while controls had not. These results were in agreement with interview data. The findings indicate that a behavioural counselling approach to hearing tactics is beneficial in the understanding and rehabilitation of hearing loss, especially over time.

  13. Monitoring hearing loss at United Airlines.

    PubMed

    Weaver, J H; Harper, R

    1981-08-01

    The airlines are a highly diversified industry. Their major concerns are in the safe transportation of passengers and cargo with on time arrival and departure of its aircraft at various locations throughout the world. The challenge the airlines face is in the development and administration of an effective hearing conservation program that will produce valid audiometry at all of its logistic operations and yet be economically feasible and practical. The methods and techniques used by United Airlines to develop and administer an effective hearing conservation program are presented here. Guidelines employing the OSHA Noise Exposure Standard were incorporated in the program requirements as set forth by the medical department.

  14. 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.

  15. Cellular signaling protective against noise-induced hearing loss – A role for novel intrinsic cochlear signaling involving corticotropin-releasing factor?

    PubMed

    Vetter, Douglas E

    2015-09-01

    Hearing loss afflicts approximately 15% of the world's population, and crosses all socioeconomic boundaries. While great strides have been made in understanding the genetic components of syndromic and non-syndromic hearing loss, understanding of the mechanisms underlying noise-induced hearing loss (NIHL) have come much more slowly. NIHL is not simply a mechanism by which older individuals loose their hearing. Significantly, the incidence of NIHL is increasing, and is now involving ever younger populations. This may predict future increased occurrences of hearing loss. Current research has shown that even short-term exposures to loud sounds generating what was previously considered temporary hearing loss, actually produces an almost immediate and permanent loss of specific populations of auditory nerve fibers. Additionally, recurrent exposures to intense sound may hasten age-related hearing loss. While NIHL is a significant medical concern, to date, few compounds have delivered significant protection, arguing that new targets need to be identified. In this commentary, we will explore cellular signaling processes taking place in the cochlea believed to be involved in protection against hearing loss, and highlight new data suggestive of novel signaling not previously recognized as occurring in the cochlea, that is perhaps protective of hearing. This includes a recently described local hypothalamic-pituitary-adrenal axis (HPA)-like signaling system fully contained in the cochlea. This system may represent a local cellular stress-response system based on stress hormone release similar to the systemic HPA axis. Its discovery may hold hope for new drug therapies that can be delivered directly to the cochlea, circumventing systemic side effects.

  16. Predictors of hearing aid use time in children with mild-severe hearing loss

    PubMed Central

    Walker, Elizabeth A.; Spratford, Meredith; Moeller, Mary Pat; Oleson, Jacob; Ou, Hua; Roush, Patricia; Jacobs, Shana

    2012-01-01

    Purpose This study investigated predictors of hearing aid (HA) use time for children with mild-severe hearing loss. Barriers to consistent HA use and reliability of parent report measures were also examined. Method Participants included parents of 272 children with hearing loss. Parents estimated the amount of time the child used HAs daily. Regression analysis examined the relationships among independent variables and HA use time. To determine parental accuracy of HA use time, datalogging from the HA was compared to parental estimates. Results Longer HA use related to older age, poorer hearing, and higher maternal education. Parental consistency ratings revealed similar findings; younger children and children with milder hearing losses wore HAs less consistently than older children and children with more severe hearing loss. Parents’ estimates and datalogging were significantly correlated; however, results suggested parents overestimate the amount of time their children wear their hearing aids. Conclusions The findings provide evidence that certain variables were significantly related to the amount of time children wore their HAs. Consistency rating scales provided insight into circumstances that were challenging for families. Use of both parental reports and datalogging may allow clinicians and researchers to obtain a general estimate of HA use time. PMID:22869089

  17. 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.

  18. Effects of age and hearing loss on concurrent vowel identification.

    PubMed

    Chintanpalli, Ananthakrishna; Ahlstrom, Jayne B; Dubno, Judy R

    2016-12-01

    Differences in formant frequencies and fundamental frequencies (F0) are important cues for segregating and identifying two simultaneous vowels. This study assessed age- and hearing-loss-related changes in the use of these cues for recognition of one or both vowels in a pair and determined differences related to vowel identity and specific vowel pairings. Younger adults with normal hearing, older adults with normal hearing, and older adults with hearing loss listened to different-vowel and identical-vowel pairs that varied in F0 differences. Identification of both vowels as a function of F0 difference revealed that increased age affects the use of F0 and formant difference cues for different-vowel pairs. Hearing loss further reduced the use of these cues, which was not attributable to lower vowel sensation levels. High scores for one vowel in the pair and no effect of F0 differences suggested that F0 cues are important only for identifying both vowels. In contrast to mean scores, widely varying differences in effects of F0 cues, age, and hearing loss were observed for particular vowels and vowel pairings. These variations in identification of vowel pairs were not explained by acoustical models based on the location and level of formants within the two vowels.

  19. Idiopathic sudden sensorineural hearing loss: vascular or viral?

    PubMed

    Linthicum, Fred H; Doherty, Joni; Berliner, Karen I

    2013-12-01

    To demonstrate that sudden sensorineural hearing loss is possibly of viral origin rather than vascular. The histopathologic morphology in 7 temporal bones with known vascular impairment due to surgical interventions was compared with that of 11 bones with a history of idiopathic sudden sensorineural hearing loss (ISSNHL). Attention was paid to the spiral ligament, stria vascularis, organ of Corti hair cells, tectorial membrane, ganglion cell population, and degree of perilymph fibrosis and the auditory nerve. A temporal bone laboratory that has been in operation for more than 50 years and includes a database consisting of clinical and histopathological information that facilitates quantitative and qualitative analysis. Eight hundred forty-nine individuals who pledged their temporal bones for scientific study, of which 18 were selected for this study by means of the database criteria of sudden sensorineural hearing loss and postmiddle fossa and retro sigmoid sinus tumor removal or vestibular nerve section. Sudden sensorineural hearing loss bones exhibited no perilymph fibrosis compared with 6 of 7 vascular cases with fibrosis (P ≤ .001), exhibited less loss of ganglion cells (P ≤ .026), exhibited greater survival of spiral ligament (P ≤ .029), and averaged twice the survival of hair cells and more widespread tectorial membrane abnormalities. Analysis of human temporal bones from patients with a sudden sensorineural hearing loss does not support a vascular insufficiency but is more suggestive of a viral etiology.

  20. Dose passive smoking induce sensorineural hearing loss in children?

    PubMed

    Talaat, Hossam Sanyelbhaa; Metwaly, Mohamed Akram; Khafagy, Ahmed Hafez; Abdelraouf, Hatem Ragaa

    2014-01-01

    Smoking plays major role in development of vascular and respiratory serious diseases. It has been reported that negative smoker children are prone for conductive hearing impairment due to repeated attacks of Eustachian tube dysfunction and middle ear effusion. This study aims to identify negative smoking as potential risk factor for development of sensorineural hearing loss. This study was done between January 2010 and November 2012. 411 children aged 5-11 years (8.2 ± 1.5) participated in this study; they were children attending the Ear, Nose, and Throat clinic of a tertiary care hospital and their siblings. The inclusion criteria were: (i) normal speech and language, (ii) absence of any disease or condition that may cause sensorineural hearing loss, and (iii) normal middle ear function on the day of hearing assessment. They were divided into three groups according to the exposure to second-hand smoke at home; group of "no exposure" whereas no smoker in the family (131 children), group of 'mild exposure" whereas the father was the only smoking parent and smoking was prohibited at home (155 children), and group of "heavy exposure", whereas the mother was smoking, or the father was freely smoking at home and in the presence of his children (125 children). Audiological evaluation in the form of pure tone and speech audiometry and immitancemetry was done for the study group. Audiological evaluation revealed that the prevalence of hearing loss was 3.8%, 4.5% and 12% in the "no exposure", "mild exposure", and "heavy exposure" groups, respectively. Significant difference was only detected between the high exposure group and the other two groups. All children had minimal sensorineural hearing loss, i.e. threshold of frequencies showing hearing loss was 20 or 25 dB HL. The risk ratios (95% confidence interval) for hearing loss in the study subgroups were 1.18 (0.38, 3.64) for mild exposure group (p>0.05), 3.14 (1.18, 8.3) for heavy exposure group (p<0.05). Passive smoking

  1. Evidence for hearing loss in amblyopsid cavefishes

    PubMed Central

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

    2013-01-01

    The constant darkness of caves and other subterranean habitats imposes sensory constraints that offer a unique opportunity to examine evolution of sensory modalities. Hearing in cavefishes has not been well explored, and here we show that cavefishes in the family Amblyopsidae are not only blind but have also lost a significant portion of their hearing range. Our results showed that cave and surface amblyopsids shared the same audiogram profile at low frequencies but only surface amblyopsids were able to hear frequencies higher than 800 Hz and up to 2 kHz. We measured ambient noise in aquatic cave and surface habitats and found high intensity peaks near 1 kHz for streams underground, suggesting no adaptive advantage in hearing in those frequencies. In addition, cave amblyopsids had lower hair cell densities compared with their surface relative. These traits may have evolved in response to the loud high-frequency background noise found in subterranean pools and streams. This study represents the first report of auditory regression in a subterranean organism. PMID:23536444

  2. Evidence for hearing loss in amblyopsid cavefishes.

    PubMed

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

    2013-06-23

    The constant darkness of caves and other subterranean habitats imposes sensory constraints that offer a unique opportunity to examine evolution of sensory modalities. Hearing in cavefishes has not been well explored, and here we show that cavefishes in the family Amblyopsidae are not only blind but have also lost a significant portion of their hearing range. Our results showed that cave and surface amblyopsids shared the same audiogram profile at low frequencies but only surface amblyopsids were able to hear frequencies higher than 800 Hz and up to 2 kHz. We measured ambient noise in aquatic cave and surface habitats and found high intensity peaks near 1 kHz for streams underground, suggesting no adaptive advantage in hearing in those frequencies. In addition, cave amblyopsids had lower hair cell densities compared with their surface relative. These traits may have evolved in response to the loud high-frequency background noise found in subterranean pools and streams. This study represents the first report of auditory regression in a subterranean organism.

  3. Temporary Hearing Loss and Rock Music.

    ERIC Educational Resources Information Center

    Danenberg, Mary A.; And Others

    1987-01-01

    Pre- and post-exposure testing of normal-hearing secondary school students (N=20) and adult chaperones (N=7) exposed to live rock music indicated that almost all subjects experienced at least a five-decibel threshold shift, with most also reporting tinnitus. Of six subjects retested three days later, four demonstrated partial recovery. (Author/CB)

  4. The HEAR-QL: quality of life questionnaire for children with hearing loss.

    PubMed

    Umansky, Amy M; Jeffe, Donna B; Lieu, Judith E C

    2011-01-01

    Few quality of life (QOL) assessment tools are available for children with specific chronic conditions, and none have been designed specifically for children with hearing loss (HL). A validated hearing-related QOL questionnaire could help clinicians determine whether an intervention is beneficial and whether one intervention is better than another. To examine QOL in children with HL and assess the validity, reliability, and factor structure of a new measure, the Hearing Environments and Reflection on Quality of Life (HEAR-QL) questionnaire. A descriptive and correlational study of a convenience sample of children. Participants included 35 children with unilateral HL, 45 with bilateral HL, and 35 siblings with normal hearing. Children 7-12 yr old were recruited by mail from a tertiary-care pediatric otolaryngology practice and the local county's Special School District. With parent consent, children completed the validated Pediatric Quality of Life Inventory™ (PedsQL) 4.0 and a 35-item HEAR-QL questionnaire. The factor structure of the HEAR-QL was determined through principal components analysis (PCA), and mean scores were computed for each subscale and the total HEAR-QL. Three weeks following the return of the initial questionnaires, a second HEAR-QL questionnaire was sent to participants to assess test-retest reliability. Both PedsQL and HEAR-QL scores were compared between children with and without HL, between children with unilateral and bilateral HL, and between children who used and did not use a hearing device using analysis of variance. Sensitivity and specificity were calculated for both the HEAR-QL and the PedsQL. A multivariable, hierarchical linear regression analysis was conducted with independent variables associated with the HEAR-QL in unadjusted tests. Using exploratory PCA, the 35-item HEAR-QL was reduced to 26 items (Cronbach's α = 0.97, sensitivity of 91% and specificity of 92% at a cutoff score of 93.5) loading on three factors: difficulty

  5. The HEAR-QL: Quality of Life Questionnaire for Children with Hearing Loss

    PubMed Central

    Umansky, Amy M.; Jeffe, Donna B.; Lieu, Judith E.C.

    2012-01-01

    Background Few quality of life (QOL) assessment tools are available for children with specific chronic conditions, and none have been designed specifically for children with hearing loss (HL). A validated hearing-related QOL questionnaire could help clinicians determine whether an intervention is beneficial and whether one intervention is better than another. Purpose To examine QOL in children with HL and assess the validity, reliability, and factor structure of a new measure, the Hearing Environments and Reflection on Quality of Life (HEAR-QL) questionnaire. Research Design A descriptive and correlational study of a convenience sample of children. Study Sample Participants included 35 children with unilateral HL, 45 with bilateral HL, and 35 siblings with normal hearing. Data Collection and Analysis Children 7-12 years old were recruited by mail from a tertiary-care pediatric otolaryngology practice and the local county's Special School District. With parent consent, children completed the validated Pediatric Quality of Life Inventory™ (PedsQL™) 4.0 and a 35-item HEAR-QL questionnaire. The factor structure of the HEAR-QL was determined through principal components analysis (PCA), and mean scores were computed for each subscale and the total HEAR-QL. Three weeks following return of the initial questionnaires, a second HEAR-QL questionnaire was sent to participants to assess test-retest reliability. Both PedsQL and HEAR-QL scores were compared between children with and without HL, between children with unilateral and bilateral HL, and between children who used and did not use a hearing device using analysis of variance. Sensitivity and specificity were calculated for both the HEAR-QL and PedsQL. A multivariable, hierarchical linear regression analysis was conducted with independent variables associated with HEAR-QL in unadjusted tests. Results Using exploratory PCA, the 35-item HEAR-QL was reduced to 26 items (Cronbach's α=0.97; sensitivity 91% and specificity

  6. Hearing handicap in adults with unilateral deafness and bilateral hearing loss.

    PubMed

    Iwasaki, Satoshi; Sano, Hajime; Nishio, Shinya; Takumi, Yutaka; Okamoto, Makito; Usami, Shin-ichi; Ogawa, Kaoru

    2013-06-01

    To assess the perception of hearing handicap in adult patients with unilateral sudden sensorineural hearing loss (SNHL) compared with those with bilateral SNHL or unilateral congenital SNHL. Retrospective chart review. Multicenter department of otolaryngology referrals. Seventy-one subjects in the unilateral severe-profound (>70 dB) sudden SNHL group (Group 1), 17 subjects in the unilateral prelingual or congenital SNHL group (Group 2), and 121 subjects in the bilateral SNHL group (Group 3). Questionnaire. Hearing Handicap Inventory for Adults (HHIA) and visual analogue scale (VAS) measurements of hearing handicap. Average levels of hearing loss were 92 dB in Group 1, 109 dB in Group 2, and 67 dB in Group 3. The relative percentage scores of HHIA and VAS compared with Group 3 were 72.6% and 81.0% in Group 1 and 25.4% and 50.3% in Group 2, respectively. A mild correlation between the HHIA subscale or VAS scores and degree of hearing loss could be found in Group 3. No significant correlation was found between the HHIA subscale or VAS scores and duration of hearing loss in Group 1 or Group 3. Higher scores were obtained in male subjects than in female subjects. Patients in Group 1 who were troubled by tinnitus scored significantly higher in the HHIA. In multiple logistic regression analysis, presence of tinnitus, older age, higher average hearing loss level, and group (bilateral SNHL>unilateral sudden SNHL>unilateral precongenital SNHL) revealed a significant positive association with high score (>42) of HHIA (odds ratio, 3.171, 1.021, 1.031, and 6.690, respectively). The results of HHIA and VAS suggest that not only patients with bilateral SNHL but also those with unilateral sudden SNHL, particularly those who have tinnitus, experience a hearing handicap.

  7. Automated Screening for High-Frequency Hearing Loss

    PubMed Central

    MacKinnon, Robert C.; Jansen, Marije; Moore, David R.

    2014-01-01

    Objective: Hearing loss at high frequencies produces perceptual difficulties and is often an early sign of a more general hearing loss. This study reports the development and validation of two new speech-based hearing screening tests in English that focus on detecting hearing loss at frequencies above 2000 Hz. Design: The Internet-delivered, speech-in noise tests used closed target-word sets of digit triplets or consonant–vowel–consonant (CVC) words presented against a speech-shaped noise masker. The digit triplet test uses the digits 0 to 9 (excluding the disyllabic 7), grouped in quasi-random triplets. The CVC test uses simple words (e.g., “cat”) selected for the high-frequency spectral content of the consonants. During testing, triplets or CVC words were identified in an adaptive procedure to obtain the speech reception threshold (SRT) in noise. For these new, high-frequency (HF) tests, the noise was low-pass filtered to produce greater masking of the low-frequency speech components, increasing the sensitivity of the test for HF hearing loss. Individual test tokens (digits, CVCs) were first homogenized using a group of 10 normal-hearing (NH) listeners by equalizing intelligibility across tokens at several speech-in-noise levels. Both tests were then validated and standardized using groups of 24 NH listeners and 50 listeners with hearing impairment. Performance on the new high frequency digit triplet (HF-triplet) and CVC (HF-CVC) tests was compared with audiometric hearing loss, and with that on the unfiltered, broadband digit triplet test (BB-triplet) test, and the ASL (Adaptive Sentence Lists) speech-in-noise test. Results: The HF-triplet and HF-CVC test results (SRT) both correlated positively and highly with high-frequency audiometric hearing loss and with the ASL test. SRT for both tests as a function of high-frequency hearing loss increased at nearly three times the rate as that of the BB-triplet test. The intraindividual variability (SD) on the

  8. Automated screening for high-frequency hearing loss.

    PubMed

    Vlaming, Marcel S M G; MacKinnon, Robert C; Jansen, Marije; Moore, David R

    2014-01-01

    Hearing loss at high frequencies produces perceptual difficulties and is often an early sign of a more general hearing loss. This study reports the development and validation of two new speech-based hearing screening tests in English that focus on detecting hearing loss at frequencies above 2000 Hz. The Internet-delivered, speech-in noise tests used closed target-word sets of digit triplets or consonant-vowel-consonant (CVC) words presented against a speech-shaped noise masker. The digit triplet test uses the digits 0 to 9 (excluding the disyllabic 7), grouped in quasi-random triplets. The CVC test uses simple words (e.g., "cat") selected for the high-frequency spectral content of the consonants. During testing, triplets or CVC words were identified in an adaptive procedure to obtain the speech reception threshold (SRT) in noise. For these new, high-frequency (HF) tests, the noise was low-pass filtered to produce greater masking of the low-frequency speech components, increasing the sensitivity of the test for HF hearing loss. Individual test tokens (digits, CVCs) were first homogenized using a group of 10 normal-hearing (NH) listeners by equalizing intelligibility across tokens at several speech-in-noise levels. Both tests were then validated and standardized using groups of 24 NH listeners and 50 listeners with hearing impairment. Performance on the new high frequency digit triplet (HF-triplet) and CVC (HF-CVC) tests was compared with audiometric hearing loss, and with that on the unfiltered, broadband digit triplet test (BB-triplet) test, and the ASL (Adaptive Sentence Lists) speech-in-noise test. The HF-triplet and HF-CVC test results (SRT) both correlated positively and highly with high-frequency audiometric hearing loss and with the ASL test. SRT for both tests as a function of high-frequency hearing loss increased at nearly three times the rate as that of the BB-triplet test. The intraindividual variability (SD) on the tests was about 2.1 (HF-triplet) and 1

  9. 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

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

    PubMed

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

    2016-01-01

    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.

  11. Fractionated Stereotactic Radiotherapy of Vestibular Schwannomas Accelerates Hearing Loss

    SciTech Connect

    Rasmussen, Rune; Claesson, Magnus; Stangerup, Sven-Eric; Roed, Henrik; Christensen, Ib Jarle; Caye-Thomasen, Per; Juhler, Marianne

    2012-08-01

    Objective: To evaluate long-term tumor control and hearing preservation rates in patients with vestibular schwannoma treated with fractionated stereotactic radiotherapy (FSRT), comparing hearing preservation rates to an untreated control group. The relationship between radiation dose to the cochlea and hearing preservation was also investigated. Methods and Materials: Forty-two patients receiving FSRT between 1997 and 2008 with a minimum follow-up of 2 years were included. All patients received 54 Gy in 27-30 fractions during 5.5-6.0 weeks. Clinical and audiometry data were collected prospectively. From a 'wait-and-scan' group, 409 patients were selected as control subjects, matched by initial audiometric parameters. Radiation dose to the cochlea was measured using the original treatment plan and then related to changes in acoustic parameters. Results: Actuarial 2-, 4-, and 10-year tumor control rates were 100%, 91.5%, and 85.0%, respectively. Twenty-one patients had serviceable hearing before FSRT, 8 of whom (38%) retained serviceable hearing at 2 years after FSRT. No patients retained serviceable hearing after 10 years. At 2 years, hearing preservation rates in the control group were 1.8 times higher compared with the group receiving FSRT (P=.007). Radiation dose to the cochlea was significantly correlated to deterioration of the speech reception threshold (P=.03) but not to discrimination loss. Conclusion: FSRT accelerates the naturally occurring hearing loss in patients with vestibular schwannoma. Our findings, using fractionation of radiotherapy, parallel results using single-dose radiation. The radiation dose to the cochlea is correlated to hearing loss measured as the speech reception threshold.

  12. Severe hearing loss in Pallister-Killian syndrome.

    PubMed

    Schuster, Maria; Hoppe, U; Eysholdt, U; Rosanowski, F

    2002-01-01

    Pallister-Killian syndrome is a rare disorder characterised by a specific combination of anatomic anomalies, mental retardation and lack of speech acquisition due to tetrasomy 12p. Hearing loss does not seem to be characteristic for this syndrome, although it was reported in several cases. We present the case of a girl first seen in our department at the age of 6 months. A severe sensory hearing loss was confirmed by subjective and objective audiometry. The child was successfully equipped with hearing aids. In the literature almost all children with Pallister-Killian syndrome are described as not developing verbal speech. Surprisingly their hearing abilities were not examined systematically. We advise audiological testing of children with Pallister-Killian syndrome.

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

    PubMed Central

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

    2015-01-01

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

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

    PubMed Central

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

    2016-01-01

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

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

    PubMed

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

    2016-04-20

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

  16. 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

  17. Audiology In The Sudden Hearing Loss Clinical Trial

    PubMed Central

    Halpin, Chris; Shi, Helen; Reda, Domenic; Antonelli, PJ; Babu, S; Carey, JP; Gantz, BJ; Goebel, JA; Hammerschlag, PE; Harris, JP; Isaacson, B; Lee, D; Linstrom, CJ; Parnes, LS; Slattery, WH; Telian, SA; Vrabec, JT; Rauch, Steven

    2012-01-01

    Objective To report the pre- and post treatment population characteristics, and the overall stability of the audiologic outcomes found during the Sudden Hearing Loss Clinical Trial [ClinicalTrials.gov: Identifier NCT00097448]. Study Design Multi-center, prospective randomized non-inferiority trial of oral v. intratympanic (IT) steroid treatment of sudden sensorineural hearing loss (SSNHL). Setting Fifteen academically-based otology practices Patients 250 patients with unilateral SSNHL presenting within 14 days of onset with ≥50 dBHL pure tone average hearing threshold in the affected ear. Intervention Either 60 mg/day oral prednisone for 14 days with a 5-day taper (121 patients) or four IT doses over 14 days of 40mg/ml methylprednisolone (129 patients). Main Outcome Measure Primary endpoint was change in hearing [dB PTA] at 2 months after treatment. Non-inferiority was defined as <10 dB difference in hearing outcome between treatments. In this article, pre- and post treatment hearing findings will be reported in detail. Results A general (and stable) effect of treatment, and a specific effect of greater improvement at low frequencies was found in both treatment groups. Conclusions Hearing improvements are stable and a significantly greater improvement occurs wit lower frequency following either oral or IT steroid treatment of SSNHL. PMID:22805100

  18. 38 CFR 20.717 - Rule 717. Loss of hearing tapes or transcripts-motion for new hearing.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... tapes or transcripts-motion for new hearing. 20.717 Section 20.717 Pensions, Bonuses, and Veterans... on Appeal § 20.717 Rule 717. Loss of hearing tapes or transcripts—motion for new hearing. (a) Motion... only a portion of a hearing tape is unintelligible or only a portion of a transcript has been lost or...

  19. 38 CFR 20.717 - Rule 717. Loss of hearing tapes or transcripts-motion for new hearing.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... tapes or transcripts-motion for new hearing. 20.717 Section 20.717 Pensions, Bonuses, and Veterans... on Appeal § 20.717 Rule 717. Loss of hearing tapes or transcripts—motion for new hearing. (a) Motion... only a portion of a hearing tape is unintelligible or only a portion of a transcript has been lost or...

  20. 38 CFR 20.717 - Rule 717. Loss of hearing tapes or transcripts-motion for new hearing.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... tapes or transcripts-motion for new hearing. 20.717 Section 20.717 Pensions, Bonuses, and Veterans... on Appeal § 20.717 Rule 717. Loss of hearing tapes or transcripts—motion for new hearing. (a) Motion... only a portion of a hearing tape is unintelligible or only a portion of a transcript has been lost or...

  1. 38 CFR 20.717 - Rule 717. Loss of hearing tapes or transcripts-motion for new hearing.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... tapes or transcripts-motion for new hearing. 20.717 Section 20.717 Pensions, Bonuses, and Veterans... on Appeal § 20.717 Rule 717. Loss of hearing tapes or transcripts—motion for new hearing. (a) Motion... only a portion of a hearing tape is unintelligible or only a portion of a transcript has been lost or...

  2. The application of genome editing in studying hearing loss.

    PubMed

    Zou, Bing; Mittal, Rahul; Grati, M'hamed; Lu, Zhongmin; Shu, Yilai; Tao, Yong; Feng, Youg; Xie, Dinghua; Kong, Weijia; Yang, Shiming; Chen, Zheng-Yi; Liu, Xuezhong

    2015-09-01

    Targeted genome editing mediated by clustered, regularly interspaced, short palindromic repeat (CRISPR)/CRISPR-associated nuclease 9 (Cas9) technology has emerged as one of the most powerful tools to study gene functions, and with potential to treat genetic disorders. Hearing loss is one of the most common sensory disorders, affecting approximately 1 in 500 newborns with no treatment. Mutations of inner ear genes contribute to the largest portion of genetic deafness. The simplicity and robustness of CRISPR/Cas9-directed genome editing in human cells and model organisms such as zebrafish, mice and primates make it a promising technology in hearing research. With CRISPR/Cas9 technology, functions of inner ear genes can be studied efficiently by the disruption of normal gene alleles through non-homologous-end-joining (NHEJ) mechanism. For genetic hearing loss, CRISPR/Cas9 has potential to repair gene mutations by homology-directed-repair (HDR) or to disrupt dominant mutations by NHEJ, which could restore hearing. Our recent work has shown CRISPR/Cas9-mediated genome editing can be efficiently performed in the mammalian inner ear in vivo. Thus, application of CRISPR/Cas9 in hearing research will open up new avenues for understanding the pathology of genetic hearing loss and provide new routes in the development of treatment to restore hearing. In this review, we describe major methodologies currently used for genome editing. We will highlight applications of these technologies in studies of genetic disorders and discuss issues pertaining to applications of CRISPR/Cas9 in auditory systems implicated in genetic hearing loss. Copyright © 2015 Elsevier B.V. All rights reserved.

  3. The Application of Genome Editing in Studying Hearing Loss

    PubMed Central

    Zou, Bing; Mittal, Rahul; Grati, M’hamed; Lu, Zhongmin; Shu, Yilai; Tao, Yong; Feng, Youg; Xie, Dinghua; Kong, Weijia; Yang, Shiming; Chen, Zheng-Yi; Liu, Xuezhong

    2015-01-01

    Targeted genome editing mediated by clustered, regularly interspaced, short palindromic repeat (CRISPR)/CRISPR-associated nuclease 9 (Cas9) technology has emerged as one of the most powerful tools to study gene functions, and with potential to treat genetic disorders. Hearing loss is one of the most common sensory disorders, affecting approximately 1 in 500 newborns with no treatment. Mutations of inner ear genes contribute to the largest portion of genetic deafness. The simplicity and robustness of CRISPR/Cas9-directed genome editing in human cells and model organisms such as zebrafish, mice and primates make it a promising technology in hearing research. With CRISPR/Cas9 technology, functions of inner ear genes can be studied efficiently by the disruption of normal gene alleles through non-homologous-end-joining (NHEJ) mechanism. For genetic hearing loss, CRISPR/Cas9 has potential to repair gene mutations by homology-directed-repair (HDR) or to disrupt dominant mutations by NHEJ, which could restore hearing. Our recent work has shown CRISPR/Cas9-mediated genome editing can be efficiently performed in the mammalian inner ear in vivo. Thus, application of CRISPR/Cas9 in hearing research will open up new avenues for understanding the pathology of genetic hearing loss and provide new routes in the development of treatment to restore hearing. In this review, we describe major methodologies currently used for genome editing. We will highlight applications of these technologies in studies of genetic disorders and discuss issues pertaining to applications of CRISPR/Cas9 in auditory systems implicated in genetic hearing loss. PMID:25987504

  4. Noise-induced hearing loss: the family physician's role

    SciTech Connect

    Dobie, R.A.

    1987-12-01

    Noise is an environmental health problem that has not received sufficient attention. Physicians should become knowledgeable about the medical consequences of excessive noise, support legislation to reduce the problem and promote programs aimed at noise control and prevention of hearing loss. Questions about noise and hearing should be incorporated into the medical history, and pure-tone audiometry should be a part of periodic physical evaluations.

  5. Effect of hearing aids on static balance function in elderly with hearing loss.

    PubMed

    Negahban, Hossein; Bavarsad Cheshmeh Ali, Mahtab; Nassadj, Gholamhossein

    2017-07-24

    While a few studies have investigated the relationship between hearing acuity and postural control, little is known about the effect of hearing aids on postural stability in elderly with hearing loss. The aim was to compare static balance function between elderly with hearing loss who used hearing aids and those who did not use. The subjects asked to stand with (A) open eyes on rigid surface (force platform), (B) closed eyes on rigid surface, (C) open eyes on a foam pad, and (D) closed eyes on a foam pad. Subjects in the aided group (n=22) were tested with their hearing aids turned on and hearing aids turned off in each experimental condition. Subjects in the unaided group (n=25) were tested under the same experimental conditions as the aided group. Indicators for postural stability were center of pressure (COP) parameters including; mean velocity, standard deviation (SD) velocity in anteroposterior (AP) and mediolateral (ML) directions, and sway area (95% confidence ellipse). The results showed that within open eyes-foam surface condition, there was greater SD velocity in the off-aided than the on-aided and the unaided than the on-aided (p<0.0001 for SD velocity in AP and ML). Also, no significant differences were found between the off-aided and unaided group (p=0.56 and p=0.77 for SD velocity in AP and ML, respectively). Hearing aids improve static balance function by reducing the SD velocity. Clinical implications may include improving hearing inputs in order to increase postural stability in older adults with hearing loss. Copyright © 2017 Elsevier B.V. All rights reserved.

  6. [Cochlear implant in postlingual adults with progressive hearing loss].

    PubMed

    Padilla Romero, M J; Sainz Quevedo, M; Roldán Segura, C

    2004-12-01

    To analize the results of patients with cohlear implants in postlingual adults with progressive hearing loss and to analize various predictive factors. Prospective study of 42 patients with profound and progressive hearing loss. We analized the outcomes in the first two years of follow-up. We examined the results in regards to the of age at time of implantation, other handicaps, cause of hearing loss, age of onset, cochlear ossification and actives electrodes. We use the logo-auditory protocol developed at the University Spanish of navarra. All tests presented a significative improvement in the first six months (p<0.01 Student t or Wilcoxon test). After six months the results were not statistically significant. The more important predictive factors were percentage of life with hearing loss, percentage of life with profound hearing loss, cochlear ossification and actives electrodes thus the significancy was more relevant at a follow-up period of 3 months (p<0.05 Student t or Mann-Withney test). These patients showed clear benefits in a short period of time and the patients with unfavourable circumstances had a more slow progression but a similar final outcome.

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

    PubMed

    Mudar, Raksha A; Husain, Fatima T

    2016-01-01

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

  8. 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

  9. 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

  10. Age-related hearing loss patterns in Fischer 344/NHsd rats with cisplatin-induced hearing loss.

    PubMed

    Bielefeld, Eric C

    2013-12-01

    The current study was undertaken to explore the impact of cisplatin ototoxicity at a young adult age on the development of age-related hearing loss, both in terms of age of onset and severity of the hearing loss. For the study, 21 Fischer 344/NHsd rats were tested. All rats were tested for auditory brainstem responses (ABRs) at age 7 months and then 15 of the rats were exposed to 7 mg/kg cisplatin by intra-peritoneal infusion. The other 6 rats received saline infusions to serve as controls. Seven of the cisplatin rats were euthanized after an ABR test 7 days after cisplatin exposure to assess acute damage. The other 14 rats were tested monthly until age 18 months. Cisplatin caused acute ABR threshold shift at 30 and 40 kHz, but that acute hearing loss led to less age-related hearing loss at those frequencies. Cisplatin exposure led to a primarily additive interaction with age-related hearing loss at 20 kHz, with some exacerbation of hearing loss at age 16-18 months, along with a larger lesion of missing outer hair cells in the corresponding region of the cochlea. ABR P1 amplitude input-output functions were not significantly affected by the cisplatin exposure when controlling for threshold shift. Results indicate that cisplatin ototoxicity and age-related hearing loss interact antagonistically in the cochlear region damaged by cisplatin, and primarily show an additive interaction in the frequencies lower than the focus of the cisplatin damage. Copyright © 2013 Elsevier B.V. All rights reserved.

  11. Influences of Working Memory and Audibility on Word Learning in Children with Hearing Loss

    ERIC Educational Resources Information Center

    Stiles, Derek Jason

    2010-01-01

    As a group, children with hearing loss demonstrate delays in language development relative to their peers with normal hearing. Early intervention has a profound impact on language outcomes in children with hearing loss. Data examining the relationship between degree of hearing loss and language outcomes are variable. Two approaches are used in the…

  12. A Survey of Classroom Teachers' and Special Educators' Knowledge of and Exposure to Hearing Loss.

    ERIC Educational Resources Information Center

    Lass, Norman J.; And Others

    1985-01-01

    A questionnaire on aspects of hearing loss, including prevalence, etiology, hearing aids, testing prevention, and treatment of hearing loss, was completed by 98 classroom teachers and 77 special educators. Results indicate teachers have limited knowledge of and exposure to hearing loss. (Author/CL)

  13. 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…

  14. Influences of Working Memory and Audibility on Word Learning in Children with Hearing Loss

    ERIC Educational Resources Information Center

    Stiles, Derek Jason

    2010-01-01

    As a group, children with hearing loss demonstrate delays in language development relative to their peers with normal hearing. Early intervention has a profound impact on language outcomes in children with hearing loss. Data examining the relationship between degree of hearing loss and language outcomes are variable. Two approaches are used in the…

  15. 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…

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

    PubMed

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

    2015-12-15

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

  17. Revision operations after previous stapes surgery for persisting hearing loss.

    PubMed

    Skrivan, J; Cada, Z; Kluh, J; Boucek, J; Profant, O; Betka, J

    2014-01-01

    The aim of the study was to find out the reasons of the recurrent or persisting hearing loss after previous stapes surgery indicated for otosclerosis. Revision stapes surgery is a relatively safe surgical method. Recurrent or persisting conductive hearing loss is commonly caused by prosthesis dislocation and adhesions in the oval window. Hearing loss is directly proportional to the number of previous operations. Retrospective analysis of 48 patients after revision stapes surgery was done over a period of 4 years (2005-2008). Improvement of the hearing and the reasons of a previous surgery failure were studied. RESULTS were compared to the other studies. The main reason of the failed surgery was adhesions and dislocation of the prosthesis. The mean postoperative air-bone gap was 12.0 dB. A mean postoperative air-bone gap closure within 10 dB occurred in 24 cases (55.8 %), between 11-20 dB occurred in 11 cases (25.6 %) and above 20 dB in 8 cases (18.6 %). The original prosthesis was replaced with a new one in 41 (95.3 %) cases. In 2 cases (4.7 %), previous prostheses were left in place and fixed by a ionomer glass cement to the long process of incus. Revision stapes surgery is a relatively safe surgical procedure allowing to improve hearing. The number of previous stapes surgery deteriorates hearing (p < 0.05) (Tab. 4, Ref. 20).

  18. Platinum-induced hearing loss after treatment for childhood cancer.

    PubMed

    van As, Jorrit W; van den Berg, Henk; van Dalen, Elvira C

    2016-08-03

    Platinum-based therapy, including cisplatin, carboplatin, oxaliplatin or a combination of these, is used to treat a variety of paediatric malignancies. Unfortunately, one of the most important adverse effects is the occurrence of hearing loss or ototoxicity. There is a wide variation in the reported prevalence of platinum-induced ototoxicity and the associated risk factors. More insight into the prevalence of and risk factors for platinum-induced hearing loss is essential in order to develop less ototoxic treatment protocols for the future treatment of children with cancer and to develop adequate follow-up protocols for childhood cancer survivors treated with platinum-based therapy. To evaluate the existing evidence on the association between childhood cancer treatment including platinum analogues and the occurrence of hearing loss. We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (2015, Issue 8), MEDLINE (PubMed) (1945 to 23 September 2015) and EMBASE (Ovid) (1980 to 23 September 2015). In addition, we searched reference lists of relevant articles and the conference proceedings of the International Society for Paediatric Oncology (2008 to 2014), the American Society of Pediatric Hematology/Oncology (2008 to 2015) and the International Conference on Long-Term Complications of Treatment of Children and Adolescents for Cancer (2010 to 2015). Experts in the field provided information on additional studies. All study designs, except case reports, case series (i.e. a description of non-consecutive participants) and studies including fewer than 100 participants treated with platinum-based therapy who had an ototoxicity assessment, examining the association between childhood cancer treatment including platinum analogues and the occurrence of hearing loss. Two review authors independently performed the study selection. One review author performed data extraction and risk of bias assessment, which was checked by another review author. We identified

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

    PubMed

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

    2015-01-01

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

  20. Hearing loss features in GJB2 biallelic mutations and GJB2/GJB6 digenic inheritance in a large Italian cohort.

    PubMed

    Cama, Elona; Melchionda, Salvatore; Palladino, Teresa; Carella, Massimo; Santarelli, Rosamaria; Genovese, Elisabetta; Benettazzo, Filippo; Zelante, Leopoldo; Arslan, Edoardo

    2009-01-01

    The aim of this study was to describe the clinical features of hearing loss due to mutations on connexin 26/30 coding genes (GJB2/GJB6). Mutations in the GJB2 gene are found to account for approximately 50% of cases of autosomal recessive non-syndromic deafness. Several European studies have estimated that the GJB2 healthy carrier condition involves about 2-4% of the population, with the 35delG mutations being the most common. A 342-kb deletion truncating the GJB6 gene (encoding connexin-30) has been associated with autosomal recessive non-syndromic deafness, mostly as digenic inheritance of the Cx30 deletion/Cx26 mutation. The following retrospective study describes audiological features and genotypes of a large cohort of 376 Italian hearing-impaired patients who underwent genetic screening for the GJB2/GJB6 genes and received follow-up care at our centre between January 2002 and October 2006. Sixteen different genotypes causing deafness in more than 27% of patients with either biallelic mutations or digenic inheritance GJB2/GJB6 were identified. The most frequent mutations were 35delG, M34T, L90P, and R184P.

  1. Binaural Interference and the Effects of Age and Hearing Loss.

    PubMed

    Mussoi, Bruna S S; Bentler, Ruth A

    2017-01-01

    The existence of binaural interference, defined here as poorer speech recognition with both ears than with the better ear alone, is well documented. Studies have suggested that its prevalence may be higher in the elderly population. However, no study to date has explored binaural interference in groups of younger and older adults in conditions that favor binaural processing (i.e., in spatially separated noise). Also, the effects of hearing loss have not been studied. To examine binaural interference through speech perception tests, in groups of younger adults with normal hearing, older adults with normal hearing for their age, and older adults with hearing loss. A cross-sectional study. Thirty-three participants with symmetric thresholds were recruited from the University of Iowa community. Participants were grouped as follows: younger with normal hearing (18-28 yr, n = 12), older with normal hearing for their age (73-87 yr, n = 9), and older with hearing loss (78-94 yr, n = 12). Prior noise exposure was ruled out. The Connected Speech Test (CST) and Hearing in Noise Test (HINT) were administered to all participants bilaterally, and to each ear separately. Test materials were presented in the sound field with speech at 0° azimuth and the noise at 180°. The Dichotic Digits Test (DDT) was administered to all participants through earphones. Hearing aids were not used during testing. Group results were compared with repeated measures and one-way analysis of variances, as appropriate. Within-subject analyses using pre-established critical differences for each test were also performed. The HINT revealed no effect of condition (individual ear versus bilateral presentation) using group analysis, although within-subject analysis showed that 27% of the participants had binaural interference (18% had binaural advantage). On the CST, there was significant binaural advantage across all groups with group data analysis, as well as for 12% of the participants at each of the two

  2. 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.

  3. 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

  4. The prevention of noise induced hearing loss in children.

    PubMed

    Harrison, Robert V

    2012-01-01

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

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

    PubMed

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

    2015-09-14

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

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

    PubMed Central

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

    2013-01-01

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

  7. Magnetic Nanoparticle Mediated Steroid Delivery Mitigates Cisplatin Induced Hearing Loss

    PubMed Central

    Ramaswamy, Bharath; Roy, Soumen; Apolo, Andrea B.; Shapiro, Benjamin; Depireux, Didier A.

    2017-01-01

    Cisplatin (cis-diamminedichloroplatinum) is widely used as a chemotherapeutic drug for genitourinary, breast, lung and head and neck cancers. Though effective in inducing apoptosis in cancer cells, cisplatin treatment causes severe hearing loss among patients. Steroids have been shown to mitigate cisplatin-induced hearing loss. However, steroids may interfere with the anti-cancer properties of cisplatin if administered systemically, or are rapidly cleared from the middle and inner ear and hence lack effectiveness when administered intra-tympanically. In this work, we deliver prednisolone-loaded nanoparticles magnetically to the cochlea of cisplatin-treated mice. This magnetic delivery method substantially reduced hearing loss in treated animals at high frequency compared to control animals or animals that received intra-tympanic methylprednisolone. The method also protected the outer hair cells from cisplatin-mediated ototoxicity. PMID:28955202

  8. Magnetic Nanoparticle Mediated Steroid Delivery Mitigates Cisplatin Induced Hearing Loss.

    PubMed

    Ramaswamy, Bharath; Roy, Soumen; Apolo, Andrea B; Shapiro, Benjamin; Depireux, Didier A

    2017-01-01

    Cisplatin (cis-diamminedichloroplatinum) is widely used as a chemotherapeutic drug for genitourinary, breast, lung and head and neck cancers. Though effective in inducing apoptosis in cancer cells, cisplatin treatment causes severe hearing loss among patients. Steroids have been shown to mitigate cisplatin-induced hearing loss. However, steroids may interfere with the anti-cancer properties of cisplatin if administered systemically, or are rapidly cleared from the middle and inner ear and hence lack effectiveness when administered intra-tympanically. In this work, we deliver prednisolone-loaded nanoparticles magnetically to the cochlea of cisplatin-treated mice. This magnetic delivery method substantially reduced hearing loss in treated animals at high frequency compared to control animals or animals that received intra-tympanic methylprednisolone. The method also protected the outer hair cells from cisplatin-mediated ototoxicity.

  9. Protection from noise-induced hearing loss with Src inhibitors.

    PubMed

    Bielefeld, Eric C

    2015-06-01

    Noise-induced hearing loss is a major cause of acquired hearing loss around the world and pharmacological approaches to protecting the ear from noise are under investigation. Noise results in a combination of mechanical and metabolic damage pathways in the cochlea. The Src family of protein tyrosine kinases could be active in both pathways and Src inhibitors have successfully prevented noise-induced cochlear damage and hearing loss in animal models. The long-term goal is to optimize delivery methods into the cochlea to reduce invasiveness and limit side-effects before human clinical testing can be considered. At their current early stage of research investigation, Src inhibitors represent an exciting class of compounds for inclusion in a multifaceted pharmacological approach to protecting the ear from noise.

  10. Navigating genetic diagnostics in patients with hearing loss.

    PubMed

    Sloan-Heggen, Christina M; Smith, Richard J H

    2016-12-01

    In the age of targeted genomic enrichment and massively parallel sequencing, there is no more efficient genetic testing method for the diagnosis of hereditary hearing loss. More clinical tests are on the market, which can make choosing good tests difficult. More and larger comprehensive genetic studies in patients with hearing loss have been published recently. They remind us of the importance of looking for both single nucleotide variation and copy number variation in all genes implicated in nonsyndromic hearing loss. They also inform us of how a patient's history and phenotype provide essential information in the interpretation of genetic data. Choosing the most comprehensive genetic test improves the chances of a genetic diagnosis and thereby impacts clinical care.

  11. The Prevention of Noise Induced Hearing Loss in Children

    PubMed Central

    Harrison, Robert V.

    2012-01-01

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

  12. Frequency selectivity in canaries with a hereditary hearing loss

    NASA Astrophysics Data System (ADS)

    Lauer, Amanda M.; Dooling, Robert J.

    2002-05-01

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

  13. Hearing loss and acquired immune deficiency syndrome: systematic review.

    PubMed

    Araújo, Eliene da Silva; Zucki, Fernanda; Corteletti, Lilian Cássia Bórnia Jacob; Lopes, Andrea Cintra; Feniman, Mariza Ribeiro; Alvarenga, Kátia de Freitas

    2012-01-01

    To investigate the occurrence of hearing loss in individuals with HIV/AIDS and their characterization regarding type and degree. It was conducted a systematic review of the literature found on the electronic databases PubMed, EMBASE, ADOLEC, IBECS, Web of Science, Scopus, Lilacs and SciELO. The search strategy was directed by a specific question: "Is hearing loss part of the framework of HIV/AIDS manifestations?", and the selection criteria of the studies involved coherence with the proposed theme, evidence levels 1, 2 or 3, and language (Portuguese, English and Spanish). We found 698 studies. After an analysis of the title and abstract, 91 were selected for full reading. Out of these, 38 met the proposed criteria and were included on the review. The studies reported presence of conductive, sensorineural, and mixed hearing loss, of variable degrees and audiometric configurations, in addition to tinnitus and vestibular disorders. The etiology can be attributed to opportunistic infections, ototoxic drugs or to the action of virus itself. The auditory evoked potentials have been used as markers of neurological alterations, even in patients with normal hearing. HIV/AIDS patients may present hearing loss. Thus, programs for prevention and treatment of AIDS must involve actions aimed at auditory health.

  14. Pediatric Idiopathic Intracranial Hypertension Presenting With Sensorineural Hearing Loss.

    PubMed

    Reitsma, Sietze; Stokroos, Robert; Weber, Jacobiene W; van Tongeren, Joost

    2015-12-01

    To present the rare case of a young boy with idiopathic intracranial hypertension presenting with bilateral sensorineural hearing loss developing over several months. This was accompanied by headaches, otalgia, tinnitus, and vertigo. Furthermore, we aim to provide a concise review on this matter, as this report represents the second case in literature of pediatric idiopathic intracranial hypertension presenting with hearing loss. Workup of a 9-year-old boy with bilateral sensorineural hearing loss, including (among others) physical examination, audiometry, diagnostic imaging, and lumbar puncture. Physical examination including fundoscopy as well as imaging showed no abnormalities. At presentation, pure tone audiometry revealed bone conduction thresholds of about 30 dB HL in both ears. Two months later, this declined to about 35 dB HL in both ears. Lumbar puncture revealed an increased intracranial pressure. The boy was thus diagnosed with idiopathic intracranial hypertension. After the lumbar puncture, the otological complaints gradually resolved, and the hearing normalized (bone conduction thresholds of 0-5 dB HL). Although rare, sensorineural hearing loss in the pediatric population together with otalgia, tinnitus, and vertigo can be due to idiopathic intracranial hypertension and as such can be reversible. © The Author(s) 2015.

  15. Efficacy of multimodality approach to sudden hearing loss.

    PubMed

    Naiboğllu, Bariş; Külekçi, Semra; Sürmeli, Mehmet; Verim, Ayşegül; Kalaycik Ertugay, Çiğdem; İhvan, Önder; Şeneldir, Lütfü; Zer Toros, Sema

    2015-01-01

    This study aims to investigate whether addition of intratympanic steroid (ITS) to systemic steroid and hyperbaric oxygen (HBO) is effective in the treatment of sudden hearing loss (SHL). Between January 2008 and October 2011, 58 patients diagnosed with SHL were enrolled in the study. Twenty patients (11 males, 9 females; mean age 45.3±21 years; range 24 to 66 years) who received systemic steroid and HBO composed group 1, while 38 patients (19 males, 19 females; mean age 41.6±16 years; range 25 to 61 years) who received ITS in addition to systemic steroid and HBO composed group 2. Post-treatment hearing improvement was statistically significant in both groups in terms of the mean pure tone according to the Siegel's criteria (p<0.05). Treatment was successful at 55% of patients in group 1 and 63% in group 2. Despite increased success rate with the addition of ITS, it did not indicate statistical significance (p>0.05). However, there was a strong statistically significant difference in terms of profound hearing loss over 90 dB (p<0.05). None of six patients (0%) with profound hearing loss in group 1 benefited treatment, while addition of ITS to the treatment yielded success in six of 12 patients with profound hearing loss (50%) in group 2 (p<0.05). Addition of ITS to systemic steroid and HBO treatment may yield better results in patients with SHL. However, ITS injection seems beneficial for patients with profound SHL.

  16. 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

  17. Noise induced hearing loss and other hearing complaints among musicians of symphony orchestras.

    PubMed

    Jansen, E J M; Helleman, H W; Dreschler, W A; de Laat, J A P M

    2009-01-01

    An investigation of the hearing status of musicians of professional symphony orchestras. Main questions are: (1) Should musicians be treated as a special group with regard to hearing, noise, and noise related hearing problems (2) Do patterns of hearing damage differ for different instrument types (3) Do OAE have an added value in the diagnosis of noise induced hearing loss (NIHL) in musicians. 241 professional musicians, aged between 23-64 participated. A brief medical history and the subjective judgment of their hearing and hearing problems were assessed. Musicians were subjected to an extensive audiological test battery, which contained testing of audiometric thresholds, loudness perception, diplacusis, tinnitus, speech perception in noise, and otoacoustic emissions. Most musicians could be categorized as normal hearing, but their audiograms show notches at 6 kHz, a frequency that is associated with NIHL. Musicians often complained about tinnitus and hyperacusis, while diplacusis was generally not reported as a problem. Tinnitus was most often localized utmost left and this could not be related to the instrument. It was usually perceived in high frequency areas, associated with NIHL. In general, musicians scored very well on the speech-in-noise test. The results of the loudness perception test were within normal limits. Otoacoustic emissions were more intense with better pure-tone thresholds, but due to large individual differences it can still not be used as an objective test for early detection of NIHL. Musicians show more noise induced hearing loss than could be expected on the basis of age and gender. Other indicators, such as complaints and prevalence of tinnitus, complaints about hyperacusis and prevalence of diplacusis suggest that musicians' ears are at risk. Continuing education about the risks of intensive sound exposure to musicians, with the emphasis on the possible development of tinnitus and hyperacusis and the need for good hearing protection is

  18. Can You Hear What I Think? Theory of Mind in Young Children With Moderate Hearing Loss.

    PubMed

    Netten, Anouk P; Rieffe, Carolien; Soede, Wim; Dirks, Evelien; Korver, Anna M H; Konings, Saskia; Briaire, Jeroen J; Oudesluys-Murphy, Anne Marie; Dekker, Friedo W; Frijns, Johan H M

    The first aim of this study was to examine various aspects of Theory of Mind (ToM) development in young children with moderate hearing loss (MHL) compared with hearing peers. The second aim was to examine the relation between language abilities and ToM in both groups. The third aim was to compare the sequence of ToM development between children with MHL and hearing peers. Forty-four children between 3 and 5 years old with MHL (35 to 70 dB HL) who preferred to use spoken language were identified from a nationwide study on hearing loss in young children. These children were compared with 101 hearing peers. Children were observed during several tasks to measure intention understanding, the acknowledgement of the other's desires, and belief understanding. Parents completed two scales of the child development inventory to assess expressive language and language comprehension in all participants. Objective language test scores were available from the medical files of children with MHL. Children with MHL showed comparable levels of intention understanding but lower levels of both desire and belief understanding than hearing peers. Parents reported lower language abilities in children with MHL compared with hearing peers. Yet, the language levels of children with MHL were within the average range compared with test normative samples. A stronger relation between language and ToM was found in the hearing children than in children with MHL. The expected developmental sequence of ToM skills was divergent in approximately one-fourth of children with MHL, when compared with hearing children. Children with MHL have more difficulty in their ToM reasoning than hearing peers, despite the fact that their language abilities lie within the average range compared with test normative samples.

  19. 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

  20. Hearing Loss as a Function of Aging and Diabetes Mellitus: A Cross Sectional Study

    PubMed Central

    Park, Dong Choon; Kim, MyungGu; Chung, Ji Hyun; Kim, Sang Hoon; Yeo, Seung Geun

    2014-01-01

    Background Although hearing loss may be caused by various factors, it is also a natural phenomenon associated with the aging process. This study was designed to assess the contributions of diabetes mellitus (DM) and hypertension, both chronic diseases associated with aging, as well as aging itself, to hearing loss in health screening examinees. Methods This study included 37,773 individuals who underwent health screening examinations from 2009 to 2012. The relationships between hearing threshold and subject age, hearing threshold at each frequency based on age group, the degree of hearing loss and the presence or absence of hypertension and DM were evaluated. Results The prevalence of hearing loss increased with age, being 1.6%, 1.8%, 4.6%, 14.0%, 30.8%, and 49.2% in subjects in their twenties, thirties, forties, fifties, sixties, and seventies, respectively (p<0.05). Hearing value per frequency showed aging-based changes, in the order of 6000, 4000, 2000, 1000 and 500 Hz, indicating greater hearing losses at high frequencies. The degree of hearing loss ranged from mild to severe. Aging and DM were correlated with the prevalence of hearing loss (p<0.05). There was no statistically significant association between hearing loss and hypertension after adjusting for age and DM. Conclusions The prevalence of hearing loss increases with age and the presence of DM. Hearing loss was greatest at high frequencies. In all age groups, mild hearing loss was the most common form of hearing loss. PMID:25549095