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Sample records for non-syndromic hearing loss

  1. 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

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

    PubMed

    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

  3. 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

  4. Recessive TBC1D24 Mutations Are Frequent in Moroccan Non-Syndromic Hearing Loss Pedigrees

    PubMed Central

    Bakhchane, Amina; Charif, Majida; Salime, Sara; Boulouiz, Redouane; Nahili, Halima; Roky, Rachida

    2015-01-01

    Mutations in the TBC1D24 gene are responsible for four neurological presentations: infantile epileptic encephalopathy, infantile myoclonic epilepsy, DOORS (deafness, onychodystrophy, osteodystrophy, mental retardation and seizures) and NSHL (non-syndromic hearing loss). For the latter, two recessive (DFNB86) and one dominant (DFNA65) mutations have so far been identified in consanguineous Pakistani and European/Chinese families, respectively. Here we report the results of a genetic study performed on a large Moroccan cohort of deaf patients that identified three families with compound heterozygote mutations in TBC1D24. Four novel mutations were identified, among which, one c.641G>A (p.Arg214His) was present in the three families, and has a frequency of 2% in control Moroccan population with normal hearing, suggesting that it acts as an hypomorphic variant leading to restricted deafness when combined with another recessive severe mutation. Altogether, our results show that mutations in TBC1D24 gene are a frequent cause (>2%) of NSHL in Morocco, and that due to its possible compound heterozygote recessive transmission, this gene should be further considered and screened in other deaf cohorts. PMID:26371875

  5. Genetics of Non Syndromic Hearing Loss in the Republic of Macedonia

    PubMed Central

    Sukarova Stefanovska, E; Cakar, M; Filipce, I; Plaseska Karanfilska, D

    2012-01-01

    Hearing impairment is the most common sensory deficit in humans affecting 1 in 1000 newborns. When present in an infant, deafness may have dramatic effects on language acquisition, seriously compromising the quality of their life. Deafness is influenced by both genetic and environmental factors, with inherited causes as the most prominent etiological factor in deafness in developed countries. The genetic basis of hearing loss is complex with numerous loci and genes underlying hereditary sensoryneural non syndromic hearing loss (NSHL) in humans. Despite the wide functional heterogeneity of the genes, mutations in the GJB2 gene are found to be the most common cause of sporadic and recessive NSHL in many populations worldwide. Molecular characterization of deafness in the Republic of Macedonia was performed in 130 NSHL profoundly deaf children from different ethnic origins. Molecular studies included direct sequencing of the GJB2 gene and specific polymerase chain reaction (PCR) analyses for the del(GJB6-D13S1830) mutation. Five common mitochondrial DNA (mtDNA) mutations [A1555G, 961delT+ C(n), T1095C, C1494T and A827G] were also analyzed using the SNaPShot method. In preliminary studies, GJB2 gene mutations were found in 36.4% of analyzed patients, with predominance of 35delG in Macedonian and Albanian patients and W24X in Gypsy patients, respectively. No del(GJB6-D13S1830) mutation was found. None of the analyzed deafness-associated mutations in mtDNA were identified in the studied patients. PMID:24052745

  6. 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.

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

    PubMed Central

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

    2014-01-01

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

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

    PubMed Central

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

    2015-01-01

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

  9. Identification of I411K, a novel missense EYA4 mutation causing autosomal dominant non-syndromic hearing loss

    PubMed Central

    TAN, MINXING; SHEN, XIAOFEI; YAO, JUN; WEI, QINJUN; LU, YAJIE; CAO, XIN; XING, GUANGQIAN

    2014-01-01

    Hearing loss is the most common sensory deficit in humans and gaining a better understanding of the underlying causes is necessary to improve counseling and rehabilitation. In the present study, a genetic analysis of a Chinese family with autosomal dominant non-syndromic progressive hearing impairment was conducted and assessed. Whole-exome sequencing in combination with a co-segregation analysis identified a novel missense mutation in EYA4 exon 15 (c.T1301A; p.I411K). The mutation segregated with the hearing loss of the family. This mutation was not identified in the databases of 1000 Genome Project, dbSNP 130, HapMap and YH project or in matched controls. Bioinformatic analysis confirmed the pathogenic effects of this mutation. To the best of our knowledge, this is the first report to provide a description of a missense mutation in the EYA4 gene resulting in non-syndromic hearing loss. Our results provide additional molecular and clinical information in order to gain improved understanding of the pathogenesis of EYA4 mutations and the genotype-phenotype correlations of DFNA10 hearing loss. PMID:25242383

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

    PubMed Central

    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

  11. Novel mutations confirm that COL11A2 is responsible for autosomal recessive non-syndromic hearing loss DFNB53.

    PubMed

    Chakchouk, Imen; Grati, M'hamed; Bademci, Guney; Bensaid, Mariem; Ma, Qi; Chakroun, Amine; Foster, Joseph; Yan, Denise; Duman, Duygu; Diaz-Horta, Oscar; Ghorbel, Abdelmonem; Mittal, Rahul; Farooq, Amjad; Tekin, Mustafa; Masmoudi, Saber; Liu, Xue Zhong

    2015-08-01

    Hearing loss (HL) is a major public health issue. It is clinically and genetically heterogeneous.The identification of the causal mutation is important for early diagnosis, clinical follow-up, and genetic counseling. HL due to mutations in COL11A2, encoding collagen type XI alpha-2, can be non-syndromic autosomal-dominant or autosomal-recessive, and also syndromic as in Otospondylomegaepiphyseal Dysplasia, Stickler syndrome type III, and Weissenbacher-Zweymuller syndrome. However, thus far only one mutation co-segregating with autosomal recessive non-syndromic hearing loss (ARNSHL) in a single family has been reported. In this study, whole exome sequencing of two consanguineous families with ARNSHL from Tunisia and Turkey revealed two novel causative COL11A2 mutations, c.109G > T (p.Ala37Ser) and c.2662C > A (p.Pro888Thr). The variants identified co-segregated with deafness in both families. All homozygous individuals in those families had early onset profound hearing loss across all frequencies without syndromic findings. The variants are predicted to be damaging the protein function. The p.Pro888Thr mutation affects a -Gly-X-Y- triplet repeat motif. The novel p.Ala37Ser is the first missense mutation located in the NC4 domain of the COL11A2 protein. Structural model suggests that this mutation will likely obliterate, or at least partially compromise, the ability of NC4 domain to interact with its cognate ligands. In conclusion, we confirm that COL11A2 mutations cause ARNSHL and broaden the mutation spectrum that may shed new light on genotype-phenotype correlation for the associated phenotypes and clinical follow-up. PMID:25633957

  12. Novel mutations confirm that COL11A2 is responsible for autosomal recessive non-syndromic hearing loss DFNB53

    PubMed Central

    Chakchouk, Imen; Grati, M’hamed; Bademci, Guney; Bensaid, Mariem; Ma, Qi; Chakroun, Amine; Foster, Joseph; Yan, Denise; Duman, Duygu; Diaz-Horta, Oscar; Ghorbel, Abdelmonem; Mittal, Rahul; Farooq, Amjad; Tekin, Mustafa

    2015-01-01

    Hearing loss (HL) is a major public health issue. It is clinically and genetically heterogeneous. The identification of the causal mutation is important for early diagnosis, clinical follow-up, and genetic counseling. HL due to mutations in COL11A2, encoding collagen type XI alpha-2, can be non-syndromic autosomal-dominant or autosomal-recessive, and also syndromic as in Otospondylomegaepiphyseal Dysplasia, Stickler syndrome type III, and Weissenbacher–Zweymuller syndrome. However, thus far only one mutation co-segregating with autosomal recessive non-syndromic hearing loss (ARNSHL) in a single family has been reported. In this study, whole exome sequencing of two consanguineous families with ARNSHL from Tunisia and Turkey revealed two novel causative COL11A2 mutations, c.109G > T (p.Ala37Ser) and c.2662C > A (p.Pro888Thr). The variants identified co-segregated with deafness in both families. All homozygous individuals in those families had early onset profound hearing loss across all frequencies without syndromic findings. The variants are predicted to be damaging the protein function. The p.Pro888Thr mutation affects a -Gly-X–Y- triplet repeat motif. The novel p.Ala37Ser is the first missense mutation located in the NC4 domain of the COL11A2 protein. Structural model suggests that this mutation will likely obliterate, or at least partially compromise, the ability of NC4 domain to interact with its cognate ligands. In conclusion, we confirm that COL11A2 mutations cause ARNSHL and broaden the mutation spectrum that may shed new light on genotype–phenotype correlation for the associated phenotypes and clinical follow-up. PMID:25633957

  13. Mutation in the zonadhesin-like domain of alpha-tectorin associated with autosomal dominant non-syndromic hearing loss.

    PubMed

    Alloisio, N; Morlé, L; Bozon, M; Godet, J; Verhoeven, K; Van Camp, G; Plauchu, H; Muller, P; Collet, L; Lina-Granade, G

    1999-01-01

    A gene responsible for autosomal dominant non-syndromic hearing impairment in two families (DFNA8 and DFNA12) has recently been identified as TECTA encoding alpha-tectorin, a major component of the tectorial membrane. In these families, missense mutations within the zona pellucida domain of alpha-tectorin were associated with stable severe mid-frequency hearing loss. The present study reports linkage to DFNA12 in a new family with autosomal dominant high frequency hearing loss progressing from mild to moderate severity. The candidate region refined to 3.8 cM still contained the TECTA gene. A missense mutation (C1619S) was identified in the zonadhesin-like domain. This mutation abolishes the first of the vicinal cysteines (1619Cys-Gly-Leu- 1622Cys) present in the D4 von Willebrand factor (vWf) type D repeat. These results further support the involvement of TECTA mutations in autosomal dominant hearing impairment, and suggest that vicinal cysteines are involved in tectorial membrane matrix assembly. PMID:10196713

  14. Exome Sequencing Identifies a Mutation in EYA4 as a Novel Cause of Autosomal Dominant Non-Syndromic Hearing Loss.

    PubMed

    Liu, Fei; Hu, Jiongjiong; Xia, Wenjun; Hao, Lili; Ma, Jing; Ma, Duan; Ma, Zhaoxin

    2015-01-01

    Autosomal dominant non-syndromic hearing loss is highly heterogeneous, and eyes absent 4 (EYA4) is a disease-causing gene. Most EYA4 mutations founded in the Eya-homologous region, however, no deafness causative missense mutation in variable region of EYA4 have previously been found. In this study, we identified a pathogenic missense mutation located in the variable region of the EYA4 gene for the first time in a four-generation Chinese family with 57 members. Whole-exome sequencing (WES) was performed on samples from one unaffected and two affected individuals to systematically search for deafness susceptibility genes, and the candidate mutations and the co-segregation of the phenotype were verified by polymerase chain reaction amplification and by Sanger sequencing in all of the family members. Then, we identified a novel EYA4 mutation in exon 8, c.511G>C; p.G171R, which segregated with postlingual and progressive autosomal dominant sensorineural hearing loss (SNHL). This report is the first to describe a missense mutation in the variable region domain of the EYA4 gene, which is not highly conserved in many species, indicating that the potential unconserved role of 171G>R in human EYA4 function is extremely important. PMID:25961296

  15. Exome Sequencing Identifies a Mutation in EYA4 as a Novel Cause of Autosomal Dominant Non-Syndromic Hearing Loss

    PubMed Central

    Xia, Wenjun; Hao, Lili; Ma, Jing; Ma, Duan; Ma, Zhaoxin

    2015-01-01

    Autosomal dominant non-syndromic hearing loss is highly heterogeneous, and eyes absent 4 (EYA4) is a disease-causing gene. Most EYA4 mutations founded in the Eya-homologous region, however, no deafness causative missense mutation in variable region of EYA4 have previously been found. In this study, we identified a pathogenic missense mutation located in the variable region of the EYA4 gene for the first time in a four-generation Chinese family with 57 members. Whole-exome sequencing (WES) was performed on samples from one unaffected and two affected individuals to systematically search for deafness susceptibility genes, and the candidate mutations and the co-segregation of the phenotype were verified by polymerase chain reaction amplification and by Sanger sequencing in all of the family members. Then, we identified a novel EYA4 mutation in exon 8, c.511G>C; p.G171R, which segregated with postlingual and progressive autosomal dominant sensorineural hearing loss (SNHL). This report is the first to describe a missense mutation in the variable region domain of the EYA4 gene, which is not highly conserved in many species, indicating that the potential unconserved role of 171G>R in human EYA4 function is extremely important. PMID:25961296

  16. 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.

  17. 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

  18. Hearing Loss

    MedlinePlus

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

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

    PubMed

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

    2016-05-01

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

  20. Molecular Epidemiology and Functional Assessment of Novel Allelic Variants of SLC26A4 in Non-Syndromic Hearing Loss Patients with Enlarged Vestibular Aqueduct in China

    PubMed Central

    Tang, Jie; Zhang, Guozheng; Wang, Guojian; Han, Mingyu; Zhang, Xun; Yang, Shiming; He, David Z. Z.; Dai, Pu

    2012-01-01

    Background Mutations in SLC26A4, which encodes pendrin, are a common cause of deafness. SLC26A4 mutations are responsible for Pendred syndrome and non-syndromic enlarged vestibular aqueduct (EVA). The mutation spectrum of SLC26A4 varies widely among ethnic groups. To investigate the incidence of EVA in Chinese population and to provide appropriate genetic testing and counseling to patients with SLC26A4 variants, we conducted a large-scale molecular epidemiological survey of SLC26A4. Methods A total of 2352 unrelated non-syndromic hearing loss patients from 27 different regions of China were included. Hot spot regions of SLC26A4, exons 8, 10 and 19 were sequenced. For patients with one allelic variant in the hot spot regions, the other exons were sequenced one by one until two mutant alleles had been identified. Patients with SLC26A4 variants were then examined by temporal bone computed tomography scan for radiological diagnosis of EVA. Ten SLC26A4 variants were cloned for functional study. Confocal microscopy and radioisotope techniques were used to examine the membrane expression of pendrin and transporter function. Results Of the 86 types of variants found, 47 have never been reported. The ratio of EVA in the Chinese deaf population was at least 11%, and that in patients of Han ethnicity reached at least 13%. The mutational spectrum and mutation detection rate of SLC26A4 are distinct among both ethnicities and regions of Mainland China. Most of the variants caused retention of pendrin in the intracellular region. All the mutant pendrins showed significantly reduced transport capability. Conclusion An overall description of the molecular epidemiological findings of SLC26A4 in China is provided. The functional assessment procedure can be applied to identification of pathogenicity of variants. These findings are valuable for genetic diagnosis, genetic counseling, prenatal testing and pre-implantation diagnosis in EVA families. PMID:23185506

  1. DFNA8/12 Caused by TECTA Mutations is the Most Identified Subtype of Non-syndromic Autosomal Dominant Hearing Loss

    PubMed Central

    Hildebrand, Michael S.; Morín, Matías; Meyer, Nicole C.; Mayo, Fernando; Modamio-Hoybjor, Silvia; Mencía, Angeles; Olavarrieta, Leticia; Morales-Angulo, Carmelo; Nishimura, Carla J.; Workman, Heather; DeLuca, Adam P.; del Castillo, Ignacio; Taylor, Kyle R.; Tompkins, Bruce; Goodman, Corey W.; Schrauwen, Isabelle; Van Wesemael, Maarten; Lachlan, K.; Shearer, A. Eliot; Braun, Terry A.; Huygen, Patrick L.M.; Kremer, Hannie; Van Camp, Guy; Moreno, Felipe; Casavant, Thomas L.; Smith, Richard J.H.; Moreno-Pelayo, Miguel A.

    2012-01-01

    The prevalence of DFNA8/DFNA12 (DFNA8/12), a type of autosomal dominant non-syndromic hearing loss (ADNSHL), is unknown as comprehensive population-based genetic screening has not been conducted. We therefore completed unbiased screening for TECTA mutations in a Spanish cohort of 372 probands from ADNSHL families. Three additional families (Spanish, Belgian and English) known to be linked to DFNA8/12 were also included in the screening. In an additional cohort of 835 American ADNSHL families, we preselected 73 probands for TECTA screening based on audiometric data. In aggregate, we identified 23 TECTA mutations in this process. Remarkably 20 of these mutations are novel, more than doubling the number of reported TECTA ADNSHL mutations from 13 to 33. Mutations lie in all domains of the α-tectorin protein, including those for the first time identified in the entactin domain, the vWFD1, vWFD2 and vWFD3 repeats, and the D1-D2 and TIL2 connectors. While the majority are private mutations, four of them – p.Cys1036Tyr, p.Cys1837Gly, p.Thr1866Met and p.Arg1890Cys – were observed in more than one unrelated family. For two of these mutations founder effects were also confirmed. Our data validate previously observed genotype-phenotype correlations in DFNA8/12 and introduce new correlations. Specifically, mutations in the N-terminal region of α-tectorin (entactin domain, vWFD1 and vWFD2) lead to mid frequency NSHL, a phenotype previously associated only with mutations in the ZP domain. Collectively, our results indicate that DFNA8/12 hearing loss is a frequent type of ADNSHL. PMID:21520338

  2. Evaluation of the contribution of the EYA4 and GRHL2 genes in Korean patients with autosomal dominant non-syndromic hearing loss.

    PubMed

    Kim, Ye-Ri; Kim, Min-A; Sagong, Borum; Bae, Seung-Hyun; Lee, Hyo-Jeong; Kim, Hyung-Jong; Choi, Jae Young; Lee, Kyu-Yup; Kim, Un-Kyung

    2015-01-01

    EYA4 and GRHL2 encode transcription factors that play an important role in regulating many developmental stages. Since EYA4 and GRHL2 were identified as the transcription factors for the DFNA10 and DFNA28, 8 EYA4 mutations and 2 GRHL2 mutations have been reported worldwide. However, these genes have been reported in few studies of the Korean population. In this study, we performed a genetic analysis of EYA4 and GRHL2 in 87 unrelated Korean patients with autosomal dominant non-syndromic hearing loss (NSHL). A total of 4 genetic variants in the EYA4 gene were identified, including the 2 nonsense mutations p.S288X and p.Q393X. The novel mutation p.Q393X (c.1177C>T) resulted in a change in the codon at amino acid position 393 from a glutamine to a stop codon. The p.Q393X allele was predicted to encode a truncated protein lacking the entire C-terminal Eya homolog region (Eya HR), which is essential for the interaction with the transcription factor SIX3. The p.S288X (c.863C>A) mutation was found in a Korean family from a previous study. We analyzed p.S288X-linked microsatellite markers and determined that p.S288X might be a founder mutation and a hotspot mutation in Koreans. In GRHL2, a total of 4 genetic variants were identified, but none were associated with hearing loss in Korean patients. This suggests that GRHL2 may not be a main causal gene for autosomal dominant NSHL in Korean patients. In conclusion, our data provide fundamental information to predict the genotypes of Korean patients diagnosed with autosomal dominant NSHL. PMID:25781927

  3. Evaluation of the Contribution of the EYA4 and GRHL2 Genes in Korean Patients with Autosomal Dominant Non-Syndromic Hearing Loss

    PubMed Central

    Sagong, Borum; Bae, Seung-Hyun; Lee, Hyo-Jeong; Kim, Hyung-Jong; Choi, Jae Young; Lee, Kyu-Yup; Kim, Un-Kyung

    2015-01-01

    EYA4 and GRHL2 encode transcription factors that play an important role in regulating many developmental stages. Since EYA4 and GRHL2 were identified as the transcription factors for the DFNA10 and DFNA28, 8 EYA4 mutations and 2 GRHL2 mutations have been reported worldwide. However, these genes have been reported in few studies of the Korean population. In this study, we performed a genetic analysis of EYA4 and GRHL2 in 87 unrelated Korean patients with autosomal dominant non-syndromic hearing loss (NSHL). A total of 4 genetic variants in the EYA4 gene were identified, including the 2 nonsense mutations p.S288X and p.Q393X. The novel mutation p.Q393X (c.1177C>T) resulted in a change in the codon at amino acid position 393 from a glutamine to a stop codon. The p.Q393X allele was predicted to encode a truncated protein lacking the entire C-terminal Eya homolog region (Eya HR), which is essential for the interaction with the transcription factor SIX3. The p.S288X (c.863C>A) mutation was found in a Korean family from a previous study. We analyzed p.S288X-linked microsatellite markers and determined that p.S288X might be a founder mutation and a hotspot mutation in Koreans. In GRHL2, a total of 4 genetic variants were identified, but none were associated with hearing loss in Korean patients. This suggests that GRHL2 may not be a main causal gene for autosomal dominant NSHL in Korean patients. In conclusion, our data provide fundamental information to predict the genotypes of Korean patients diagnosed with autosomal dominant NSHL. PMID:25781927

  4. 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.

  5. Hearing Loss

    MedlinePlus

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

  6. Cloning genes for non-syndromal hearing impairment.

    PubMed

    Smith, R J; Van Camp, G

    1999-10-01

    Over 45 genes that cause autosomal non-syndromic hearing impairment (NSHI) have been localized and many more are predicted to exist. To clone these genes, a number of different strategies can be used. This paper focuses on four general approaches: functional cloning, positional cloning, position-dependent candidate gene cloning, and position-independent candidate gene cloning. PMID:10890140

  7. 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.

  8. 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

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

    PubMed

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

    2014-02-01

    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

  10. 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

  11. 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)

  12. Living with Hearing Loss

    MedlinePlus

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

  13. Hearing Loss and Older Adults

    MedlinePlus

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

  14. Hearing: Noise-Induced Hearing Loss

    MedlinePlus

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

  15. Genetics of Hearing Loss

    MedlinePlus

    ... in Latin America Information For... Media Policy Makers Genetics of Hearing Loss Language: English Español (Spanish) Recommend ... of hearing loss in babies is due to genetic causes. There are also a number of things ...

  16. Genes and Hearing Loss

    MedlinePlus

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

  17. Living with hearing loss

    MedlinePlus

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

  18. Hearing Loss: Screening Newborns

    MedlinePlus

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

  19. 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.

  20. 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.

  1. 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…

  2. [Inner Ear Hearing Loss].

    PubMed

    Hesse, G

    2016-06-01

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

  3. Noise-Induced Hearing Loss

    MedlinePlus

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

  4. 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…

  5. 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…

  6. Hearing Loss in Children: Types of Hearing Loss

    MedlinePlus

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

  7. Sudden Sensorineural Hearing Loss

    PubMed Central

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

    2011-01-01

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

  8. SOX10 mutations mimic isolated hearing loss.

    PubMed

    Pingault, V; Faubert, E; Baral, V; Gherbi, S; Loundon, N; Couloigner, V; Denoyelle, F; Noël-Pétroff, N; Ducou Le Pointe, H; Elmaleh-Bergès, M; Bondurand, N; Marlin, S

    2015-10-01

    Ninety genes have been identified to date that are involved in non-syndromic hearing loss, and more than 300 different forms of syndromic hearing impairment have been described. Mutations in SOX10, one of the genes contributing to syndromic hearing loss, induce a large range of phenotypes, including several subtypes of Waardenburg syndrome and Kallmann syndrome with deafness. In addition, rare mutations have been identified in patients with isolated signs of these diseases. We used the recent characterization of temporal bone imaging aspects in patients with SOX10 mutations to identify possible patients with isolated hearing loss due to SOX10 mutation. We selected 21 patients with isolated deafness and temporal bone morphological defects for mutational screening. We identified two SOX10 mutations and found that both resulted in a non-functional protein in vitro. Re-evaluation of the two affected patients showed that both had previously undiagnosed olfactory defects. Diagnosis of anosmia or hyposmia in young children is challenging, and particularly in the absence of magnetic resonance imaging (MRI), SOX10 mutations can mimic non-syndromic hearing impairment. MRI should complete temporal bones computed tomographic scan in the management of congenital deafness as it can detect brain anomalies, cochlear nerve defects, and olfactory bulb malformation in addition to inner ear malformations. PMID:25256313

  9. Occupational hearing loss

    MedlinePlus

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

  10. 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 ...

  11. Hearing loss and music

    MedlinePlus

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

  12. Hearing loss - infants

    MedlinePlus

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

  13. 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

  14. Noise and Hearing Loss Prevention

    MedlinePlus

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

  15. Diagnosis of Hearing Loss.

    ERIC Educational Resources Information Center

    World Federation of the Deaf, Rome (Italy).

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

  16. RNA Interference Prevents Autosomal-Dominant Hearing Loss.

    PubMed

    Shibata, Seiji B; Ranum, Paul T; Moteki, Hideaki; Pan, Bifeng; Goodwin, Alexander T; Goodman, Shawn S; Abbas, Paul J; Holt, Jeffrey R; Smith, Richard J H

    2016-06-01

    Hearing impairment is the most common sensory deficit. It is frequently caused by the expression of an allele carrying a single dominant missense mutation. Herein, we show that a single intracochlear injection of an artificial microRNA carried in a viral vector can slow progression of hearing loss for up to 35 weeks in the Beethoven mouse, a murine model of non-syndromic human deafness caused by a dominant gain-of-function mutation in Tmc1 (transmembrane channel-like 1). This outcome is noteworthy because it demonstrates the feasibility of RNA-interference-mediated suppression of an endogenous deafness-causing allele to slow progression of hearing loss. Given that most autosomal-dominant non-syndromic hearing loss in humans is caused by this mechanism of action, microRNA-based therapeutics might be broadly applicable as a therapy for this type of deafness. PMID:27236922

  17. 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.

  18. [Progressive hearing loss].

    PubMed

    Reiss, M; Reiss, G

    2000-01-01

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

  19. Age-Related Hearing Loss

    MedlinePlus

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

  20. [Presbycusis - Age Related Hearing Loss].

    PubMed

    Fischer, N; Weber, B; Riechelmann, H

    2016-07-01

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

  1. Autism and Hearing Loss.

    ERIC Educational Resources Information Center

    Rosenhall, Ulf; Nordin, Viviann; Sandstrom, Mikael; Ahlsen, Gunilla; Gillberg, Christopher

    1999-01-01

    Children and adolescents (N=199) with autistic disorder were audiologically evaluated. Mild to moderate hearing loss was diagnosed in 7.9 percent, with deafness diagnosed in 3.5 percent of all cases, which represented a prevalence considerably above that in the general population and comparable to the prevalence found in populations with mental…

  2. Hearing Loss: Diagnosis and Evaluation.

    PubMed

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

    2015-07-01

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

  3. Pediatric Sudden Sensorineural Hearing Loss.

    PubMed

    Kizilay, Ahmet; Koca, Çiğdem Firat

    2016-06-01

    Sudden sensorineural hearing loss is defined as sudden unilateral or bilateral sensorineural hearing loss with at least 30 dB decrease in threshold in 3 contiguous test frequencies occurring over 72 hours or less. It is rare among children. The mechanism of the process and prognosis of the disorder remains unclear. The current incidence of sudden sensorineural hearing loss among pediatric population is unknown. The authors carried out a retrospective chart analysis of patients under 15 years of age from 2004 to 2015, who consulted to the Otolaryngology Head and Neck Surgery Department of Inonu University Medical Faculty. Age, sex, number of affected ear and side, audiometric evaluations, medical follow-up, treatment method, duration of treatment recovery, associated complaints; tinnitus and/or vertigo, presence of mumps disease were recorded for each patient. A 4-frequency pure-tone average (500, 1000, 2000, and 4000 Hz) was calculated for each ear. Complete recovery, defined as some hearing level compared with the nonaffected ear, was observed in 3 patients (21.4 %) and there was no partial hearing recovery. The hearing loss of 11 patient remained unchanged after prednisolone treatment. Two of the 11 patients had bilaterally total sensorineural hearing loss and evaluated as appropriate for cochlear implantation. Sex of patient and laterality of hearing loss were not correlated with hearing recovery. Sensorineural hearing loss among pediatrics has been the issue of otolaryngologists. The incidence, etiology, and treatment methods should be more studied. PMID:27171971

  4. 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…

  5. 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…

  6. Hearing Loss in Children: Screening and Diagnosis

    MedlinePlus

    ... Form Controls NCBDDD Cancel Submit Search The CDC Hearing Loss in Children Note: Javascript is disabled or is ... message, please visit this page: About CDC.gov . Hearing Loss Homepage Facts Noise-Induced Hearing Loss Genetics of ...

  7. Use of Hearing Aids by Adults with Hearing Loss

    MedlinePlus

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

  8. 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. PMID:27430449

  9. 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. PMID:2938482

  10. 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…

  11. 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.

  12. 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

  13. 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

  14. Resounding Facts on Hearing Loss

    ERIC Educational Resources Information Center

    Apfel, Robert E.

    1977-01-01

    Provides a brief description of the physiology of the human ear. The effect of sustained noise levels on hearing loss is discussed, as well as the establishment of maximum noise levels for American industries. (CP)

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

    PubMed

    Wu, Kaiwen; Wang, Hongyang; Wang, Qiuju

    2016-03-01

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

  16. 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 ...

  17. Devices for hearing loss

    MedlinePlus

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

  18. Pigmentary anomalies and hearing loss.

    PubMed

    Toriello, Helga V

    2011-01-01

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

  19. Noise-induced hearing loss.

    PubMed

    McReynolds, Michael C

    2005-01-01

    Noise-induced hearing loss is a major hazard in many workplaces and in society. The National Institute for Occupational Safety and Health estimates that more than 30 million workers (almost 1 in 10) are exposed to unsafe noise levels on the job. Helicopter emergency medical services crews work in an environment in which exposure to aviation noise makes the issue of hearing loss and prevention strategies salient. Applying the Occupational Safety and Health Administration standard for a regulated hearing conservation program through controlling noise exposure will benefit helicopter emergency medical services professionals. PMID:15741953

  20. 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. PMID:23402072

  1. Occupational hearing loss in farmers.

    PubMed Central

    Plakke, B L; Dare, E

    1992-01-01

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

  2. 10 Ways to Identify Hearing Loss

    MedlinePlus

    ... of this page please turn JavaScript on. Feature: Hearing Loss 10 Ways to Identify Hearing Loss Past Issues / Spring 2015 Table of Contents If ... gov Internet: www.nidcd.nih.gov Read More "Hearing Loss" Articles Managing Hearing Loss / Symptoms, Devices, Prevention & Research / ...

  3. Hearing loss in shipyard employees

    PubMed Central

    Alexopoulos, Evangelos C.; Tsouvaltzidou, Thomaella

    2015-01-01

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

  4. Genetics of Hearing Loss: Syndromic.

    PubMed

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

    2015-12-01

    Hearing loss (HL) is one of the most common birth defects in developed countries and is a diverse pathologic condition with different classifications. One of these is based on the association with other clinical features, defined as syndromic hearing loss (SHL). Determining the cause 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. The advancement of high-throughput sequencing technology is facilitating rapid and low-cost diagnostics for patients with SHL. PMID:26443487

  5. Age-related hearing loss

    MedlinePlus

    ... is no known single cause of age-related hearing loss. Most commonly, it is caused by changes in the inner ear that occur as you grow older. Your genes and loud noise (from rock concerts or music headphones) may play a large role. The following ...

  6. 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. PMID:23257577

  7. Middle Ear Infection (Chronic Otitis Media) and Hearing Loss

    MedlinePlus

    ... You Middle Ear Infection (Chronic Otitis Media) and Hearing Loss Middle Ear Infection (Chronic Otitis Media) and Hearing ... learning important speech and language skills. Types of hearing loss Conductive hearing loss is a form of hearing ...

  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 Pin ... or serious ear condition and lead to further hearing loss or other complications. “The problem might be as ...

  9. 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 ...

  10. Drug Induced Hearing Loss: What Is Ototoxicity?

    MedlinePlus

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

  11. 10 Ways to Identify Hearing Loss

    MedlinePlus

    ... Current Issue Past Issues 10 Ways to Identify Hearing Loss Past Issues / Spring 2007 Table of Contents For ... exposure and other causes. 10 Ways to Identify Hearing Loss Take the following quiz to help determine if ...

  12. Acoustic Trauma - Hearing Loss in Teenagers

    MedlinePlus

    ... Issues Listen Español Text Size Email Print Share Acoustic Trauma - Hearing Loss in Teenagers Page Content Article ... temporary or permanent hearing loss. This is called acoustic trauma. How loud is 85 decibels? Surprisingly, not ...

  13. 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.

  14. 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. PMID:26429333

  15. 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,…

  16. Hearing loss among high school farm students.

    PubMed Central

    Broste, S K; Hansen, D A; Strand, R L; Stueland, D T

    1989-01-01

    To study the prevalence of hearing loss among teen-aged farm children in central Wisconsin, audiometric threshold testing of 872 vocational agriculture students was carried out over a three-year period. The results indicate an increased prevalence of hearing loss among students actively involved in farm work, as compared to their peers not involved in farm work. Findings also suggest that use of hearing protection may reduce the risk of hearing loss among students who work on the farm, although few students report the use of such devices. These data also suggest that the hearing loss often observed in adult farmers may begin in childhood. PMID:2784948

  17. 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. PMID:12123480

  18. Enlarged Vestibular Aqueducts and Childhood Hearing Loss

    MedlinePlus

    ... Health Info » Hearing, Ear Infections, and Deafness Enlarged Vestibular Aqueducts and Childhood Hearing Loss On this page: ... more information about enlarged vestibular aqueducts? What are vestibular aqueducts? The inner ear Credit: NIH Medical Arts ...

  19. Hearing Loss in the Elderly: An Overview

    PubMed Central

    Blakley, Brian W.

    1989-01-01

    Loss of hearing is tragic, yet most of those afflicted can be helped. Surgery is sometimes appropriate, but for the majority a hearing aid is the best answer. This article reviews what happens in different hearing losses, and gives an overview of hearing aids for the physician who lacks an extensive technical background but wishes to understand these devices a little better. Simple tips for investigation of hearing-aid complaints are included, as well as some tips for communication with hard-of-hearing persons. PMID:21249000

  20. Familial hearing loss and cisplatin therapy.

    PubMed

    Kennedy, B J; Torkelson, J L

    1998-01-01

    Familial high-tone hearing loss in males is a recessive trait often unrecognized. Cisplatin chemotherapy may be associated with hearing loss. A review was made of audiograms in 85 patients with testicular carcinoma prior to cisplatin chemotherapy to determine the extent of preexisting familial hearing loss. Clinical histories defined patients exposed to high noise levels and other common causes of hearing loss. Audiometric findings were classified according to normal hearing or mild, moderate, and severe hearing impairment. Pretreatment audiograms were normal in 51 patients and abnormal in 19 patients with histories of high-level noise exposure, and in 15 patients with high-frequency hearing loss there was no history of noise exposure, ear infection, or other potential causes of hearing loss. These last 15 patients were judged to have recessive familial hearing loss. Awareness of familial hearing loss is important in male patients in whom cisplatin chemotherapy is planned. Pretreatment hearing assessment, including audiograms, is recommended for such male patients. PMID:9589029

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

    PubMed

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

    1995-11-01

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

  2. 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

  3. 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…

  4. Loss-of-Function CNKSR2 Mutation Is a Likely Cause of Non-Syndromic X-Linked Intellectual Disability

    PubMed Central

    Houge, G.; Rasmussen, I.H.; Hovland, R.

    2012-01-01

    In a non-dysmorphic 5-year-old boy with developmental delay, well-controlled epilepsy, and microcephaly, a 234-kb deletion of Xp22.12 was detected by copy number analysis. The maternally inherited deletion removed the initial 15 of the 21 exons of the connector enhancer of KSR-2 gene called CNKSR2 or CNK2. Our finding suggests that loss of CNKSR2 is a novel cause of non-syndromic X-linked mental retardation, an assumption supported by high gene expression in the brain, localization to the post-synaptic density, and a role in RAS/MAPK-dependent signal transduction. PMID:22511892

  5. Loss-of-Function CNKSR2 Mutation Is a Likely Cause of Non-Syndromic X-Linked Intellectual Disability.

    PubMed

    Houge, G; Rasmussen, I H; Hovland, R

    2012-01-01

    In a non-dysmorphic 5-year-old boy with developmental delay, well-controlled epilepsy, and microcephaly, a 234-kb deletion of Xp22.12 was detected by copy number analysis. The maternally inherited deletion removed the initial 15 of the 21 exons of the connector enhancer of KSR-2 gene called CNKSR2 or CNK2. Our finding suggests that loss of CNKSR2 is a novel cause of non-syndromic X-linked mental retardation, an assumption supported by high gene expression in the brain, localization to the post-synaptic density, and a role in RAS/MAPK-dependent signal transduction. PMID:22511892

  6. 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.

  7. Hearing Loss in Children: Data and Statistics

    MedlinePlus

    ... hearing loss is intellectual disability (23%), followed by cerebral palsy (10%), autism spectrum disorder (7%), and/or vision ... without other related conditions (such as intellectual disability, cerebral palsy, epilepsy, or vision loss) were employed. [ Read summary ] ...

  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. Amplification considerations for children with minimal or mild bilateral hearing loss and unilateral hearing loss.

    PubMed

    McKay, Sarah; Gravel, Judith S; Tharpe, Anne Marie

    2008-03-01

    Children with minimal or mild bilateral hearing loss and unilateral hearing loss are at higher risk for academic, speech-language, and social-emotional difficulties than their normal hearing peers. The choice to fit infants with moderate or greater degrees of bilateral hearing loss has been standard practice for most clinicians, but for those with minimal or mild bilateral hearing loss or unilateral hearing loss, the fitting of hearing technology must be based on limited data. Evidence does not yet exist to support all the management decisions that an audiologist must make upon identifying an infant with minimal or mild bilateral hearing loss or unilateral hearing loss. It is not yet known which children are at the greatest risk for educational problems nor is it known if the provision of early amplification in this population will help a child avoid later difficulties. Some of these considerations and current hearing technology options for children with minimal or mild bilateral hearing loss or unilateral hearing loss are reviewed in this article. PMID:18270178

  10. Asymmetric and Unilateral Hearing Loss in Children

    PubMed Central

    Vila, Peter; Lieu, Judith E. C.

    2015-01-01

    Asymmetric and unilateral hearing losses in children have traditionally been underappreciated, but health care practitioners are now beginning to understand their effect on development and the underlying pathophysiologic mechanisms. The common wisdom among medical and educational professionals has been that at least one normal hearing or near-normal hearing ear was sufficient for typical speech and language development in children. The objective of this review is to illustrate to the non-otolaryngologist the consequences of asymmetric and unilateral hearing loss in children on developmental and educational outcomes. In the process, etiology, detection, and management are discussed. Lastly, implications for further research are considered. PMID:26004144

  11. Managing Hearing Loss | NIH MedlinePlus the Magazine

    MedlinePlus

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

  12. Sudden hearing loss after dental treatment.

    PubMed

    Kansu, Leyla; Yilmaz, Ismail

    2013-08-01

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

  13. 20 CFR 702.441 - Claims for loss of hearing.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 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... report must set forth the testing standards used and describe the method of evaluating the hearing...

  14. 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. PMID:27142990

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

    PubMed

    Hesse, Gerhard

    2016-07-01

    The great majority of hearing disorders generates from pathologies in the inner ear, mainly the outer hair cells, as mentioned in the first part of this review. Very often, however, hearing loss appears suddenly and even without external causes like noise exposure. This sudden hearing loss is mostly unilateral, recovers very often spontaneously and should be treated, if persisting. Only in this acute stage there are therapeutic options available. If the inner ear hearing loss is chronic there is no curative therapy, an effective management of the hearing disorder is only possible through rehabilitation. This is due to the fact, that hair cells of all mammals, incl. humans, have no regenerative capacity and neither pharmaceutic agents nor other means can induce regeneration and recovery of hair cells. Even a gen-therapy is not available yet. In the second part of this review the main focus lies in sudden hearing loss and general therapeutic options for inner ear hearing loss. PMID:27392187

  16. 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…

  17. 20 CFR 702.441 - Claims for loss of hearing.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 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... report must set forth the testing standards used and describe the method of evaluating the hearing...

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

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 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... report must set forth the testing standards used and describe the method of evaluating the hearing...

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

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 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... report must set forth the testing standards used and describe the method of evaluating the hearing...

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

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 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... report must set forth the testing standards used and describe the method of evaluating the hearing...

  1. Individual Hearing Loss: Characterization, Modelling, Compensation Strategies.

    PubMed

    Santurette, Sébastien; 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

  2. 76 FR 62093 - Preventing Occupational Hearing Loss: Stakeholder Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-10-06

    ... Occupational Safety and Health Administration Preventing Occupational Hearing Loss: Stakeholder Meeting AGENCY... hearing loss. Every year, between 20,000 and 25,000 workers suffer from preventable hearing loss due to... occupational hearing loss. DATES: The date for the stakeholder meeting is November 03, 2011, from 9 a.m. to 1...

  3. Noise and Hearing Loss Prevention: Facts and Statistics

    MedlinePlus

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

  4. 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.

  5. [Extensive hearing loss and deafness in adults].

    PubMed

    Laszig, R

    1993-09-01

    Hearing and understanding are two related, yet different processes. Hearing is the perception of sound. It can be of enormous value to patients with severely impaired hearing, as it facilitates acoustic orientation. An understanding of speech, however, remains virtually impossible for most of these patients. Nevertheless, early habituation to their acoustic situation makes lip-reading much easier, thus enabling conversations to be possible in good listening environments. Severely impaired patients, however, are still not in a position to follow conversation in larger groups. Even with hearing aids and the deployment of the latest technology, sufficient help is not always given. Before making a decision on the use of these technical aids, the ENT specialist should discuss the needs of the particular individual with the hearing-aid specialist. Provided that residual hearing can be used to understand speech with the help of a hearing aid, intracochlear implantation of an electronic prosthesis is not indicated. A cochlear implant is indicated when there is a complete hearing loss on both sides. Such a profound loss means that sufficient understanding of speech is no longer possible, even with the assistance of the latest hearing aids. For most adults, deafness is a postlingual phenomenon. Adults who were born deaf or who lost their hearing in childhood tend to be unsuitable for cochlear implantation. Up to the age of six years, however, children born deaf can benefit considerably from a cochlear implant. Children who are provided with a cochlear implant shortly after becoming deaf also have a good chance of being capable of learning and understanding speech. PMID:8273025

  6. Fluctuating sensorineural hearing loss in children.

    PubMed

    Brookhouser, Patrick E

    2002-08-01

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

  7. 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

  8. Childhood hearing health: educating for prevention of hearing loss.

    PubMed

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

    2015-01-01

    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

  9. 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

  10. Managing Hearing Loss | NIH MedlinePlus the Magazine

    MedlinePlus

    ... the nerve pathways from the ear to the brain. Many people may have a combination of both noise-induced hearing loss and hearing loss from aging. Noise-induced hearing loss can be prevented by lowering the volume, moving away from the noise, or wearing hearing ...

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

    PubMed Central

    Wingard, Jeffrey C.; Zhao, Hong-Bo

    2015-01-01

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

  12. Sudden Sensorineural Hearing Loss; Prognostic Factors

    PubMed Central

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

    2015-01-01

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

  13. Sensorineural hearing loss in chronic otitis media.

    PubMed

    MacAndie, C; O'Reilly, B F

    1999-06-01

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

  14. 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

  15. Genetics of Nonsyndromic Congenital Hearing Loss.

    PubMed

    Egilmez, Oguz Kadir; Kalcioglu, M Tayyar

    2016-01-01

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

  16. Sudden Sensorineural Hearing Loss: Primary Care Update.

    PubMed

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

    2016-06-01

    The primary care physician's role in recognizing sudden sensorineural hearing (SSNHL) loss and delivering initial treatment is critical in the management of the syndrome. This role involves recognizing its clinical symptoms, distinguishing it from conductive hearing loss with the Weber tuning fork or the Rauch hum test, and urgent administration of high dose oral corticosteroids. Diagnosis and treatment should not be delayed for audiometric testing or referral to otolaryngology. This paper provides an update on the initial evaluation and treatment of this syndrome based on the literature and clinical guideline recommendations. PMID:27413627

  17. Sudden Sensorineural Hearing Loss: Primary Care Update

    PubMed Central

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

    2016-01-01

    The primary care physician's role in recognizing sudden sensorineural hearing (SSNHL) loss and delivering initial treatment is critical in the management of the syndrome. This role involves recognizing its clinical symptoms, distinguishing it from conductive hearing loss with the Weber tuning fork or the Rauch hum test, and urgent administration of high dose oral corticosteroids. Diagnosis and treatment should not be delayed for audiometric testing or referral to otolaryngology. This paper provides an update on the initial evaluation and treatment of this syndrome based on the literature and clinical guideline recommendations. PMID:27413627

  18. Hearing Loss in Children: Treatment and Intervention Services

    MedlinePlus

    ... coordinator to find available services in your state. Technology Many people who are deaf or hard-of- ... of-hearing person has is called "residual hearing". Technology does not "cure" hearing loss, but may help ...

  19. Sensorineural hearing loss in Kawasaki disease.

    PubMed

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

    2016-01-01

    Kawasaki disease is a common nonspecific vasculitis seen in childhood. The most significant long-term sequela is coronary artery aneurysm. However, the spectrum of complications involves not only the heart, but also other organs such as the eyes, skin, kidneys, gallbladder, liver, and central nervous system. Sensorineural hearing loss (SNHL) is a relatively unrecognized complication of the disease. Although most of the complications (except coronary artery aneurysm) are self-limiting, SNHL can be persistent. It is, especially important in infants and young children who might not be able to report the hearing deficits and are most likely to have cognitive and speech delays if this hearing loss is not addressed in a timely manner. We report a child with Kawasaki disease who had SNHL during the 2(nd) week of the illness. The aim of this article is to briefly review the pathophysiology behind this hearing loss and strongly emphasize the importance of universal hearing evaluation in all children diagnosed with Kawasaki disease. This screening in children with Kawasaki disease may provide some timely intervention if needed. Since most Kawasaki disease patients will be seen by cardiologists, we hope to create more awareness about this complication to the cardiology community as well. PMID:27011703

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

    PubMed

    Pinquart, Martin; Pfeiffer, Jens P

    2015-01-01

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

  1. 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.

  2. Screening for Hearing Loss in Adolescents.

    ERIC Educational Resources Information Center

    Holmes, Alice E.; And Others

    1997-01-01

    The results of an auditory screening protocol administered to 342 adolescents (ages 10-20) suggest a high level of noise exposure in the adolescent population. Overall failure rate was 25%. Significant correlations were found between firearm use and hearing loss at 6000 Hertz. Screening and education efforts are urged. (Author/CR)

  3. 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…

  4. 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.

  5. 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. PMID:26611069

  6. 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. PMID:27169813

  7. The Relationship between Nonverbal Cognitive Functions and Hearing Loss

    ERIC Educational Resources Information Center

    Zekveld, Adriana A.; Deijen, Jan Berend; Goverts, S. Theo; Kramer, Sophia E.

    2007-01-01

    Purpose: This study investigated the relationship between hearing loss and memory and attention when nonverbal, visually presented cognitive tests are used. Method: Hearing loss (pure-tone audiometry) and IQ were measured in 30 participants with mild to severe hearing loss. Participants performed cognitive tests of pattern recognition memory,…

  8. Observations From a Musician With Hearing Loss

    PubMed Central

    2012-01-01

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

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

    PubMed Central

    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

    2015-01-01

    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. PMID:25666562

  10. 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

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

    PubMed Central

    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.

    2015-01-01

    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. PMID:25555219

  12. 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. PMID:24565744

  13. Introduction to auditory perception in listeners with hearing losses

    NASA Astrophysics Data System (ADS)

    Florentine, Mary; Buus, Søren

    2003-04-01

    Listeners with hearing losses cannot hear low-level sounds. In addition, they often complain that audible sounds do not have a comfortable loudness, lack clarity, and are difficult to hear in the presence of other sounds. In particular, they have difficulty understanding speech in background noise. The mechanisms underlying these complaints are not completely understood, but hearing losses are known to alter many aspects of auditory processing. This presentation highlights alterations in audibility, loudness, pitch, spectral and temporal processes, and binaural hearing that may result from hearing losses. The changes in these auditory processes can vary widely across individuals with seemingly similar amounts of hearing loss. For example, two listeners with nearly identical thresholds can differ in their ability to process spectral and temporal features of sounds. Such individual differences make rehabilitation of hearing losses complex. [Work supported by NIH/NIDCD.

  14. 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. PMID:26498713

  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. Musical hallucination associated with hearing loss.

    PubMed

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

    2011-01-01

    In spite of the fact that musical hallucination have a significant impact on patients' lives, they have received very little attention of experts. Some researchers agree on a combination of peripheral and central dysfunctions as the mechanism that causes hallucination. The most accepted physiopathology of musical hallucination associated to hearing loss (caused by cochlear lesion, cochlear nerve lesion or by interruption of mesencephalon or pontine auditory information) is the disinhibition of auditory memory circuits due to sensory deprivation. Concerning the cortical area involved in musical hallucination, there is evidence that the excitatory mechanism of the superior temporal gyrus, as in epilepsies, is responsible for musical hallucination. In musical release hallucination there is also activation of the auditory association cortex. Finally, considering the laterality, functional studies with musical perception and imagery in normal individuals showed that songs with words cause bilateral temporal activation and melodies activate only the right lobe. The effect of hearing aids on the improvement of musical hallucination as a result of the hearing loss improvement is well documented. It happens because auditory hallucination may be influenced by the external acoustical environment. Neuroleptics, antidepressants and anticonvulsants have been used in the treatment of musical hallucination. Cases of improvement with the administration of carbamazepine, meclobemide and donepezil were reported, but the results obtained were not consistent. PMID:21625772

  17. A novel autosomal recessive non-syndromic hearing impairment locus (DFNB47) maps to chromosome 2p25.1-p24.3.

    PubMed

    Hassan, Muhammad Jawad; Santos, Regie Lyn P; Rafiq, Muhammad Arshad; Chahrour, Maria H; Pham, Thanh L; Wajid, Muhammad; Hijab, Nadine; Wambangco, Michael; Lee, Kwanghyuk; Ansar, Muhammad; Yan, Kai; Ahmad, Wasim; Leal, Suzanne M

    2006-01-01

    Hereditary hearing impairment (HI) displays extensive genetic heterogeneity. Autosomal recessive (AR) forms of prelingual HI account for approximately 75% of cases with a genetic etiology. A novel AR non-syndromic HI locus (DFNB47) was mapped to chromosome 2p25.1-p24.3, in two distantly related Pakistani kindreds. Genome scan and fine mapping were carried out using microsatellite markers. Multipoint linkage analysis resulted in a maximum LOD score of 4.7 at markers D2S1400 and D2S262. The three-unit support interval was bounded by D2S330 and D2S131. The region of homozygosity was found within the three-unit support interval and flanked by markers D2S2952 and D2S131, which corresponds to 13.2 cM according to the Rutgers combined linkage-physical map. This region contains 5.3 Mb according to the sequence-based physical map. Three candidate genes, KCNF1, ID2 and ATP6V1C2 were sequenced, and were found to be negative for functional sequence variants. PMID:16261342

  18. A novel autosomal recessive non-syndromic hearing impairment locus (DFNB47) maps to chromosome 2p25.1-p24.3

    PubMed Central

    Hassan, Muhammad Jawad; Santos, Regie Lyn P.; Rafiq, Muhammad Arshad; Chahrour, Maria H.; Pham, Thanh L.; Wajid, Muhammad; Hijab, Nadine; Wambangco, Michael; Lee, Kwanghyuk; Ansar, Muhammad; Yan, Kai; Ahmad, Wasim; Leal, Suzanne M.

    2010-01-01

    Hereditary hearing impairment (HI) displays extensive genetic heterogeneity. Autosomal recessive (AR) forms of prelingual HI account for ~75% of cases with a genetic etiology. A novel AR non-syndromic HI locus (DFNB47) was mapped to chromosome 2p25.1-p24.3, in two distantly related Pakistani kindreds. Genome scan and fine mapping were carried out using microsatellite markers. Multipoint linkage analysis resulted in a maximum LOD score of 4.7 at markers D2S1400 and D2S262. The three-unit support interval was bounded by D2S330 and D2S131. The region of homozygosity was found within the three-unit support interval and flanked by markers D2S2952 and D2S131, which corresponds to 13.2 cM according to the Rutgers combined linkage-physical map. This region contains 5.3 Mb according to the sequence-based physical map. Three candidate genes, KCNF1, ID2 and ATP6V1C2 were sequenced, and were found to be negative for functional sequence variants. PMID:16261342

  19. 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…

  20. 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

  1. Self-esteem in children and adolescents with hearing loss.

    PubMed

    Warner-Czyz, Andrea D; Loy, Betty A; Evans, Christine; Wetsel, Ashton; Tobey, Emily A

    2015-01-01

    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

  2. Normative data for the Attitudes towards Loss of Hearing Questionnaire.

    PubMed

    Saunders, Gabrielle H; Cienkowski, Kathleen M; Forsline, Anna; Fausti, Stephen

    2005-10-01

    Investigations have shown that patient attitudes toward hearing loss and hearing aids impact self-reported handicap and disability, hearing aid benefit, and hearing aid use. The Attitudes towards Loss of Hearing Questionnaire (ALHQ) was developed by Saunders and Cienkowski (1996) to examine some of the psychosocial factors underlying the use and acquisition of hearing aids. Here we report data from a new version of questionnaire (ALHQ v2.1), which examines attitudes towards hearing loss and hearing aids on five scales: Denial of Hearing Loss, Negative Associations, Negative Coping Strategies, Manual Dexterity and Vision, and Hearing-Related Esteem. Reliability values, internal consistency values, and cut points for typical and atypical scores are provided, along with comparison of the scores of women, men, current hearing aid users, non-hearing aid users, and paying versus nonpaying individuals. The ALHQ takes about ten minutes to complete and identifies for the clinician some of the issues that might jeopardize successful hearing aid outcome. PMID:16515136

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

    PubMed

    Harada, Hirofumi; Ichikawa, Daisuke; Imamura, Akihide

    2008-01-01

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

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

    PubMed

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

    2015-06-01

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

  5. Preliminary comparison of infants speech with and without hearing loss

    NASA Astrophysics Data System (ADS)

    McGowan, Richard S.; Nittrouer, Susan; Chenausky, Karen

    2005-04-01

    The speech of ten children with hearing loss and ten children without hearing loss aged 12 months is examined. All the children with hearing loss were identified before six months of age, and all have parents who wish them to become oral communicators. The data are from twenty minute sessions with the caregiver and child, with their normal prostheses in place, in semi-structured settings. These data are part of a larger test battery applied to both caregiver and child that is part of a project comparing the development of children with hearing loss to those without hearing loss, known as the Early Development of Children with Hearing Loss. The speech comparisons are in terms of number of utterances, syllable shapes, and segment type. A subset of the data was given a detailed acoustic analysis, including formant frequencies and voice quality measures. [Work supported by NIDCD R01 006237 to Susan Nittrouer.

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

    PubMed Central

    Fujimoto, Chisato

    2015-01-01

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

  7. Sensorineural hearing loss in otitis media.

    PubMed

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

    1984-01-01

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

  8. 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

  9. Consanguinity and hereditary hearing loss in Qatar.

    PubMed

    Girotto, Giorgia; Mezzavilla, Massimo; Abdulhadi, Khalid; Vuckovic, Dragana; Vozzi, Diego; Khalifa Alkowari, Moza; Gasparini, Paolo; Badii, Ramin

    2014-01-01

    Qatar is a sovereign state located on the Eastern coast of the Arabian Peninsula in the Persian Gulf. Its native population consists of 3 major subgroups: people of Arabian origin or Bedouins, those from an Eastern or Persian ancestry and individuals with African admixture. Historically, all types of consanguineous marriages have been and still are common in the Qatari population, particularly among first and double-first cousins. Thus, there is a higher risk for most inherited diseases including hereditary hearing loss (HHL). In particular, a hearing loss prevalence of 5.2% has been reported in Qatar, with parental consanguinity being more common among affected individuals as compared with unaffected ones. Our recent molecular results confirm a high homogeneity and level of inbreeding in Qatari HHL patients. Among all HHL genes, GJB2, the major player worldwide, accounts for a minor proportion of cases and at least 3 additional genes have been found to be mutated in Qatari patients. Interestingly, one gene, BDP1, has been described to cause HHL only in this country. These results point towards an unexpected level of genetic heterogeneity despite the high level of inbreeding. This review provides an up-to-date picture of HHL in Qatar and of the impact of consanguinity on this disease. PMID:25060281

  10. Prevalence and Factors Associated With Hearing Loss and Hearing Aid Use in Korean Elders

    PubMed Central

    2015-01-01

    Background: 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. Methods: 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. Results: 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. Conclusion: 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. PMID:25905073

  11. 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.

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

    PubMed

    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 AND 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

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

    PubMed

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

    2015-01-01

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

  17. Hearing loss and tinnitus in adolescents and young adults

    NASA Astrophysics Data System (ADS)

    Holmes, Alice

    2001-05-01

    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.

  18. A case of sudden unilateral sensorineural hearing loss with contralateral psychogenic hearing loss induced by gunshot noise.

    PubMed

    Hong, Young-Ho; Mun, Seog-Kyun

    2011-10-01

    The reasons behind sudden sensorineural hearing loss are mostly unknown, but viral infections, blood disorders, ototoxicity, noise trauma, autoimmune disease, acoustic tumor, and even mental stress may be related to the disease. In cases of hearing loss as a result of psychogenic factors, early diagnosis and adequate treatment under collaboration with the psychiatric department are crucial, since failure to take appropriate measures may result in permanent sequela. We report a case, with a review of the literature, of sudden unilateral sensorineural hearing loss with contralateral psychogenic hearing loss induced by gunshot noise. PMID:22128658

  19. Hearing loss severity: impaired processing of formant transition duration.

    PubMed

    Coez, A; Belin, P; Bizaguet, E; Ferrary, E; Zilbovicius, M; Samson, Y

    2010-08-01

    Normal hearing listeners exploit the formant transition (FT) detection to identify place of articulation for stop consonants. Neuro-imaging studies revealed that short FT induced less cortical activation than long FT. To determine the ability of hearing impaired listeners to distinguish short and long formant transitions (FT) from vowels of the same duration, 84 mild to severe hearing impaired listeners and 5 normal hearing listeners were asked to detect 10 synthesized stimuli with long (200 ms) or short (40 ms) FT among 30 stimuli of the same duration without FT. Hearing impaired listeners were tested with and without hearing aids. The effect of the difficulty of the task (short/long FT) was analysed as a function of the hearing loss with and without hearing aids. Normal hearing listeners were able to detect every FT (short and long). For hearing impaired listeners, the detection of long FT was better than that of short ones irrespective of their degree of hearing loss. The use of hearing aids improved detection of both kinds of FT; however, the detection of long FT remained much better than the detection of the short ones. The length of FT modified the ability of hearing impaired patients to detect FT. Short FT had access to less cortical processing than long FT and cochlea damages enhanced this specific deficit in short FT brain processing. These findings help to understand the limit of deafness rehabilitation in the time domain and should be taken into account in future devices development. PMID:20600193

  20. [Hearing loss associated with smoking in male workers].

    PubMed

    Takata, Yasumitsu

    2011-03-01

    A cross-sectional study was designed to examine the effect of smoking habit on hearing loss at 1000 and 4000 Hz in the workplace. Among 1,875 male workers, including 287 subjects with occupational noise exposure, the ratio of the number with hearing loss at 1000 or 4000 Hz increased with smoking habits and that relation at 4000 Hz was statistically significant. These hearing losses showed a significant relation with age but not with working- duration under occupational noise exposure by multiple regression analysis. The amount of smoking showed a weak but significant association with hearing loss at 4000 Hz. However, among the 287 male subjects with occupational noise exposure, there was no significant relation between smoking habits and hearing loss. Therefore, both hearing loss induced by occupational noise exposure and that related with smoking habit were well controlled in this workplace. These results indicate that hearing check-ups and education to prevent noise-induced hearing impairment in the workplace might be useful to prevent the hearing loss associated with smoking habit among male workers. PMID:21438339

  1. 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

  2. Unilateral sudden hearing loss: a rare symptom of Moyamoya disease.

    PubMed

    Gül, Fatih; Berçin, Sami; Müderris, Togay; Yalçıner, Gökhan; Ünal, Özkan; Kırış, Muzaffer

    2016-01-01

    A 38-year-old female patient experienced a sudden onset of unilateral sensorineural hearing loss due to Moyamoya disease. A detailed summary of audiological and neurological findings indicated that the sudden hearing loss might be due to Moyamoya disease resulting in occlusion of posterior and middle cerebral arteries. Intravenous prednisolone and trimetazidine dihydrochloride may improve hearing thresholds and speech understanding. To our knowledge, this is the first article in the literature reporting a case of sudden hearing loss as the first manifestation of Moyamoya disease in a young adult. PMID:26890714

  3. 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…

  4. Hearing aid fitting in older persons with hearing impairment: the influence of cognitive function, age, and hearing loss on hearing aid benefit

    PubMed Central

    Meister, Hartmut; Rählmann, Sebastian; Walger, Martin; Margolf-Hackl, Sabine; Kießling, Jürgen

    2015-01-01

    Purpose To examine the association of cognitive function, age, and hearing loss with clinically assessed hearing aid benefit in older hearing-impaired persons. Methods 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. Results 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. Conclusion 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

  5. 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)

  6. 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

  7. Arts Education: Diminishing Communications Barriers for Students with Hearing Loss.

    ERIC Educational Resources Information Center

    Ryan, Susan D.

    1996-01-01

    The writer, an individual with progressive hearing loss, describes the isolation and powerlessness experienced by people with hearing loss and comments on the great value that participation in the arts and art education can have in moderating this isolation and empowering individuals to find their own identity. (DB)

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

    PubMed

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

    2016-07-01

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

  9. 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…

  10. 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…

  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. PMID:26960789

  12. 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

  13. 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

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

    PubMed Central

    Stein, Gretchen; Danermark, Berth; Germundsson, Per

    2015-01-01

    Background and Objectives 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. Subjects and Methods 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. Results 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. Conclusions These results suggesting that the social representation about the phenomenon hearing loss and hearing aids are relatively stable within respondents of each country. PMID:26771011

  15. 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

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

    MedlinePlus

    ... dying due to ototoxic drugs. Please describe the benefits of your findings. Our results advance our understanding ... hearing is critical to their developing speech and language skills. Our goal is to improve the lives ...

  18. Hearing Loss and Older Adults’ Perceptions of Access to Care

    PubMed Central

    Schumacher, Jessica R.; Barnett, Steven; Smith, Maureen A.

    2011-01-01

    We investigated whether hard-of-hearing older adults were more likely to report difficulties and delays in accessing care and decreased satisfaction with healthcare access than those without hearing loss. The Wisconsin Longitudinal Study (2003–2006 wave, N = 6,524) surveyed respondents regarding hearing, difficulties/delays in accessing care, satisfaction with healthcare access, socio-demographics, chronic conditions, self-rated health, depression, and length of relationship with provider/site. We used multivariate regression to compare access difficulties/delays and satisfaction by respondents’ hearing status (hard-of-hearing or not). Hard-of-hearing individuals comprised 18% of the sample. Compared to those not hard-of-hearing, hard-of-hearing individuals were significantly more likely to be older, male and separated/divorced. They had a higher mean number of chronic conditions, including atherosclerotic vascular disease, diabetes and depression. After adjustment for potential confounders, hard-of-hearing individuals were more likely to report difficulties in accessing healthcare (Odds Ratio 1.85; 95% Confidence Interval 1.19–2.88). Satisfaction with healthcare access was similar in both groups. Our findings suggest healthcare access difficulties will be heightened for more of the population because of the increasing prevalence of hearing loss. The prevalence of hearing loss in this data is low and our findings from a telephone survey likely underestimate the magnitude of access difficulties experienced by hard-of-hearing older adults. Further research which incorporates accessible surveys is needed. In the meantime, clinicians should pay particular attention to assessing barriers in healthcare access for hard-of-hearing individuals. Resources should be made available to proactively address these issues for those who are hard-of-hearing and to educate providers about the specific needs of this population. PMID:21301940

  19. 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

  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. 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. 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. PMID:26280639

  3. 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

  4. 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…

  5. 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

  6. 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. PMID:26572701

  7. The role of mitochondria in age-related hearing loss.

    PubMed

    Chen, Hengchao; Tang, Jianguo

    2014-02-01

    Age-related hearing loss (ARHL), the hearing loss associated with aging, is a vital problem in present society. The severity of hearing loss is possibly associated with the degeneration of cochlear cells. Mitochondria play a key role in the energy supply, cellular redox homeostasis, signaling, and regulation of programmed cell death. In this review, we focus on the central role of mitochondria in ARHL. The mitochondrial redox imbalance and mitochondrial DNA mutation might collaboratively involve in the process of cochlear senescence in response to the aging stress. Subsequent responses, including alteration of mitochondrial biogenesis, mitophagy, apoptosis and paraptosis, participate in the aging process from different respects. PMID:24202185

  8. Hearing loss and the high speed dental handpiece.

    PubMed Central

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

    1980-01-01

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

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

    PubMed Central

    2009-01-01

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

  10. Hearing loss in speech-language delayed children.

    PubMed

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

    2001-05-11

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

  11. Ear Infection and Hearing Loss Amongst Headphone Users

    PubMed Central

    Mazlan, R.; Saim, L.; Thomas, A.; Said, R.; Liyab, B.

    2002-01-01

    The use of headphone has been thought to cause infection in the ear canal and contribute to hearing loss. In this study, we examined 136 Customer Service Representative from Celcom (Malaysia) Sdn. Bhd. who use headphone throughout their working hours. The purpose of this study was to determine the prevalence of ear canal infection and other related diseases of the ear, nose and throat. Their hearing thresholds were also determined using the Amplaid 309 Clinical Audiometer. We found no incidence of infection of the external ear canal amongst the subjects. There were 4 cases of chronic middle ear infection and 4 cases of impacted wax. Hearing impairment was found in 25 subjects (21.2%). However, there was no significant association between hearing loss and the exposure to sound from headphone usage because the high frequencies were not predominantly affected. There was also no association between hearing loss and duration of service. PMID:22844220

  12. Subjective Fatigue in Children With Hearing Loss: Some Preliminary Findings

    PubMed Central

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

    2014-01-01

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

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

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-06-01

    ... Register on Thursday, April 21, 2011 (76 FR 22336). Persons who wish to present oral comments at the... 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....

  15. 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,…

  16. The Socioeconomic Impact of Hearing Loss in US Adults

    PubMed Central

    Emmett, Susan D.; Francis, Howard W.

    2014-01-01

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

  17. Audiological results with Baha in conductive and mixed hearing loss.

    PubMed

    Pfiffner, Flurin; Caversaccio, Marco-Domenico; Kompis, Martin

    2011-01-01

    The level of improvement in the audiological results of Baha(®) users mainly depends on the patient's preoperative hearing thresholds and the type of Baha sound processor used. This investigation shows correlations between the preoperative hearing threshold and postoperative aided thresholds and audiological results in speech understanding in quiet of 84 Baha users with unilateral conductive hearing loss, bilateral conductive hearing loss and bilateral mixed hearing loss. Secondly, speech understanding in noise of 26 Baha users with different Baha sound processors (Compact, Divino, and BP100) is investigated. Linear regression between aided sound field thresholds and bone conduction (BC) thresholds of the better ear shows highest correlation coefficients and the steepest slope. Differences between better BC thresholds and aided sound field thresholds are smallest for mid-frequencies (1 and 2 kHz) and become larger at 0.5 and 4 kHz. For Baha users, the gain in speech recognition in quiet can be expected to lie in the order of magnitude of the gain in their hearing threshold. Compared to its predecessor sound processors Baha(®) Compact and Baha(®) Divino, Baha(®) BP100 improves speech understanding in noise significantly by +0.9 to +4.6 dB signal-to-noise ratio, depending on the setting and the use of directional microphone. For Baha users with unilateral and bilateral conductive hearing loss and bilateral mixed hearing loss, audiological results in aided sound field thresholds can be estimated with the better BC hearing threshold. The benefit in speech understanding in quiet can be expected to be similar to the gain in their sound field hearing threshold. The most recent technology of Baha sound processor improves speech understanding in noise by an order of magnitude that is well perceived by users and which can be very useful in everyday life. PMID:21389707

  18. [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. PMID:2221307

  19. 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

  20. 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.

  1. Hearing Loss in Patients with Shunt-Treated Hydrocephalus.

    PubMed

    Panova, Margarita V; Geneva, Ina E; Madjarova, Kalina I; Bosheva, Miroslava N

    2015-01-01

    Hearing loss is a common manifestation of the long-term complications in patients with shunt treated hydrocephalus along with motor development disturbance, cognitive and visual impairment, epilepsy and endocrine disorders. The aim of the present study was to investigate the alterations of hearing in patients with shunt treated hydrocephalus of non-tumor etiology and at least one year after implantation of ventriculo-peritoneal shunt, as well as their impact on the quality of life of patients. The study included 70 patients (age range 1.25 years - 21.25 years) with shunted non-tumor hydrocephalus and at least one year after placement of the shunt system. Hearing alterations were proved by measuring the brainstem auditory evoked potentials (BAEP) for children up to 5 years of age and children with mental retardation; audiograms was used for children older than 5 years with normal neuro-psychological development (NPD). Of the 70 studied patients 17 (24%) had hearing loss (10 bilateral and 7-unilateral) and all of them had sensorineural hearing loss, which is associated with low weight at birth, posthemorrhagic hydrocephalus and brainstem symptoms at the time of diagnosis of hydrocephalus. Hearing pathology was found more often in shunt-treated patients with NPD retardation, poor functional status and low quality of life. Children with shunt-treated hydrocephalus have hearing loss of sensorineural type. Children with brain stem symptomatology at diagnosing hydrocephalus and children with post-hemorrhagic hydrocephalus show higher risk of hearing loss. Children with shunted hydrocephalus and hearing loss show lower NPD, lower quality of life and lower functional status. PMID:27180348

  2. 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. PMID:26161525

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

    PubMed

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

    2012-03-01

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

  4. 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…

  5. Informational Masking and Spatial Hearing in Listeners with and without Unilateral Hearing Loss

    ERIC Educational Resources Information Center

    Rothpletz, Ann M.; Wightman, Frederic L.; Kistler, Doris J.

    2012-01-01

    Purpose: This study assessed selective listening for speech in individuals with and without unilateral hearing loss (UHL) and the potential relationship between spatial release from informational masking and localization ability in listeners with UHL. Method: Twelve adults with UHL and 12 normal-hearing controls completed a series of monaural and…

  6. CHARACTERIZING THE SPECTRUM OF AUTOSOMAL RECESSIVE HEREDITARY HEARING LOSS IN IRAN

    PubMed Central

    Sloan-Heggen, Christina M; Babanejad, Mojgan; Beheshtian, Maryam; Simpson, Allen C; Booth, Kevin T; Ardalani, Fariba; Frees, Kathy L; Mohseni, Marzieh; Mozafari, Reza; Mehrjoo, Zohreh; Jamali, Leila; Vaziri, Saeideh; Akhtarkhavari, Tara; Bazazzadegan, Niloofar; Nikzat, Nooshin; Arzhangi, Sanaz; Sabbagh, Farahnaz; Otukesh, Hasan; Seifati, Seyed Morteza; Khodaei, Hossein; Taghdiri, Maryam; Meyer, Nicole C; Daneshi, Ahmad; Farhadi, Mohammad; Kahrizi, Kimia; Smith, Richard JH; Azaiez, Hela; Najmabadi, Hossein

    2016-01-01

    Background Countries with culturally accepted consanguinity provide a unique resource for the study of rare recessively inherited genetic diseases. Although hereditary hearing loss (HHL) is not uncommon, it is genetically heterogeneous, with over 85 genes causally implicated in non-syndromic hearing loss (NSHL). This heterogeneity makes many gene-specific types of NSHL exceedingly rare. We sought to define the spectrum of autosomal recessive HHL in Iran by investigating both common and rarely diagnosed deafness-causing genes. Design Using a custom targeted genomic enrichment (TGE) panel we simultaneously interrogating all known genetic causes of NSHL in a cohort of 302 GJB2-negative Iranian families. Results We established a genetic diagnosis for 67% of probands and their families, with over half of all diagnoses attributable to variants in five genes: SLC26A4, MYO15A, MYO7A, CDH23, and PCDH15. As a reflection of the power of consanguinity mapping, 26 genes were identified as causative for NSHL in the Iranian population for the first time. In total, 179 deafness-causing variants were identified in 40 genes in 201 probands, including 110 novel single nucleotide or small insertion-deletion variants and 3 novel copy number variations. Several variants represent founder mutations. Conclusion This study attests to the power of TGE and massively parallel sequencing (TGE+MPS) as a diagnostic tool for the evaluation of hearing loss in Iran, and expands on our understanding of the genetics of HHL in this country. Families negative for variants in the genes represented on this panel represent an excellent cohort for novel gene discovery. PMID:26445815

  7. Awarding and promoting excellence in hearing loss prevention

    PubMed Central

    Meinke, Deanna K.; Morata, Thais C.

    2015-01-01

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

  8. A causal relationship between hearing loss and cognitive impairment.

    PubMed

    Park, So Young; Kim, Min Jung; Sikandaner, Huerxidan; Kim, Dong-Kee; Yeo, Sang Won; Park, Shi Nae

    2016-05-01

    Conclusion Moderate hearing loss in young mice caused decreases in cognition associated with spatial working and recognition memories in 6 months. These results provide evidence for a causal relationship between hearing loss and cognitive impairment. Objectives Hypothesized mechanisms to connect sensory and cognitive functions include the sensory-deprivation, information-degradation, and common-cause hypotheses. This study intended to investigate the effect of hearing loss on cognitive function, as estimated by radial arm maze (RAM) and novel object recognition (NOR) tasks in mice through age- and hearing-matched longitudinal work during a 6-month period. Methods Twenty-four male C57BL/6 mice aged 1 month with normal ABR thresholds were used. Twelve mice in the hearing loss (HL) group were exposed to white noise at 110 dB SPL for 60 min every day for 20 days. At post-noise 6 months, all mice underwent RAM and one-trial NOR test. RAM performance measures and NOR discrimination index were compared between two groups. Results At 6 months after noise exposure, all mice in the experimental group had moderate hearing loss. Most of the RAM performances improved gradually within each group across five trials, probably due to learning effect. The HL group showed lower performance scores than the control group in several trial points in the RAM task. The contact time with the novel object was shorter in the HL group than in the control group. PMID:26808715

  9. Hearing loss in elderly patients in a family practice.

    PubMed Central

    Sangster, J F; Gerace, T M; Seewald, R C

    1991-01-01

    OBJECTIVE: To investigate hearing loss in elderly patients. DESIGN: Cohort study. SETTING: Family practice. PATIENTS: All ambulatory patients 65 years of age or older who attended the practice from June to August 1989. OUTCOME MEASURES: The Hearing Handicap Inventory for the Elderly--Screening Version (HHIE-S) and the Welch-Allyn Audioscope. Patients who failed one or both of the screening tests were referred to a speech and hearing clinic for audiologic assessment and treatment recommendations. Those with hearing aids were excluded from the main study but were given the opportunity to have them assessed at the clinic. MAIN RESULTS: Of 157 eligible patients 42 were excluded: 16 refused to participate, 13 already had hearing aids, and 13 could not be contacted. Of the remaining 115, 34 failed one or both of the tests (14 failed the HHIE-S, 9 failed the audioscope test, and 11 failed both). Of the 34, 25 completed the audiologic assessment at the clinic. Fifteen were found to have severe hearing impairment; the recommendation was hearing aids for 12, further assessment for 2 and no treatment for 1. Of the remaining 10 patients it was thought that 6 would benefit from hearing aids. Ten of the 11 patients with hearing aids who agreed to undergo testing at the clinic were found to need an adjustment or replacement of their devices. CONCLUSIONS: Hearing loss is a significant problem in elderly patients in primary practice. Further study is required to determine which of the two screening tools is most effective. Most elderly patients with hearing aids may require modification or replacement of their devices. PMID:2009476

  10. 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. PMID:26074267

  11. 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

    ... 38 Pensions, Bonuses, and Veterans' Relief 2 2014-07-01 2014-07-01 false Rule 717. Loss of hearing... on Appeal § 20.717 Rule 717. Loss of hearing tapes or transcripts—motion for new hearing. (a) Motion... motion include, but will not be limited to, the extent of the loss of the record in those cases...

  12. 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

    ... 38 Pensions, Bonuses, and Veterans' Relief 2 2011-07-01 2011-07-01 false Rule 717. Loss of hearing... on Appeal § 20.717 Rule 717. Loss of hearing tapes or transcripts—motion for new hearing. (a) Motion... motion include, but will not be limited to, the extent of the loss of the record in those cases...

  13. 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

    ... 38 Pensions, Bonuses, and Veterans' Relief 2 2013-07-01 2013-07-01 false Rule 717. Loss of hearing... on Appeal § 20.717 Rule 717. Loss of hearing tapes or transcripts—motion for new hearing. (a) Motion... motion include, but will not be limited to, the extent of the loss of the record in those cases...

  14. 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... on Appeal § 20.717 Rule 717. Loss of hearing tapes or transcripts—motion for new hearing. (a) Motion... motion include, but will not be limited to, the extent of the loss of the record in those cases...

  15. 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

    ... 38 Pensions, Bonuses, and Veterans' Relief 2 2012-07-01 2012-07-01 false Rule 717. Loss of hearing... on Appeal § 20.717 Rule 717. Loss of hearing tapes or transcripts—motion for new hearing. (a) Motion... motion include, but will not be limited to, the extent of the loss of the record in those cases...

  16. 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)

  17. 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

  18. 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

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

    PubMed

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

    2002-12-01

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

  20. 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.

  1. 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

  2. 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.

  3. 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

  4. 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. PMID:26722345

  5. 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

  6. 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

  7. 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. PMID:25987504

  8. 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.

  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. 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