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Sample records for non-syndromic hearing loss

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

    PubMed Central

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

    2014-01-01

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

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

    PubMed

    Schrijver, Iris

    2004-11-01

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

  3. 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

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

    PubMed

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

    2016-11-01

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

  5. 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

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

    PubMed

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

    2014-02-01

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

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

    PubMed

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

    2008-01-01

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

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

    PubMed

    Onsori, Habib; Rahmati, Mohammad; Fazli, Davood

    2014-01-01

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

  10. [Non-syndromic hereditary hearing impairment].

    PubMed

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

    2007-04-01

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

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

    PubMed

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

    2015-01-01

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

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

    PubMed

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

    2011-06-01

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

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

    SciTech Connect

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

    1994-09-01

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

  14. 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.

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

    PubMed

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

    2015-01-01

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

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

    PubMed Central

    Grossheim, J. M.; Hegde, Rashima S.; Delio, Maria; Samanich, Joy; Riazuddin, Saima; Frolenkov, Gregory I.; Cai, Jinlu; Ahmed, Zubair M.; Morrow, Bernice E.

    2015-01-01

    Hearing loss is a complex disorder caused by both genetic and environmental factors. Previously, mutations in CIB2 have been identified as a common cause of genetic hearing loss in Pakistani and Turkish populations. Here we report a novel (c.556C>T; p.(Arg186Trp)) transition mutation in the CIB2 gene identified through whole exome sequencing (WES) in a Caribbean Hispanic family with non-syndromic hearing loss. CIB2 belongs to the family of calcium-and integrin-binding (CIB) proteins. The carboxy-termini of CIB proteins are associated with calcium binding and intracellular signaling. The p.(Arg186Trp) mutation is localized within predicted type II PDZ binding ligand at the carboxy terminus. Our ex vivo studies revealed that the mutation did not alter the interactions of CIB2 with Whirlin, nor its targeting to the tips of hair cell stereocilia. However, we found that the mutation disrupts inhibition of ATP-induced Ca2+ responses by CIB2 in a heterologous expression system. Our findings support p.(Arg186Trp) mutation as a cause for hearing loss in this Hispanic family. In addition, it further highlights the necessity of the calcium binding property of CIB2 for normal hearing. PMID:26426422

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

    PubMed

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

    2015-08-01

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

  18. 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

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

    PubMed Central

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

    2016-01-01

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

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

    PubMed Central

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

    2014-01-01

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

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

    PubMed

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

    2014-07-01

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

  2. Hearing Loss: Hearing Augmentation.

    PubMed

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

    2015-07-01

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

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

    PubMed Central

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

    2016-01-01

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

  4. 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.

  5. 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

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

    PubMed Central

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

    2014-01-01

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

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

    PubMed

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

    2005-09-01

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

  8. Hearing Loss

    MedlinePlus

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

  9. 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

  10. 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)

  11. Hearing Loss

    MedlinePlus

    ... and heart screening. If your baby doesn’t pass his newborn hearing screening, it doesn’t always ... be screened again. If your baby doesn’t pass a second time, it’s very important that he ...

  12. Hearing Loss

    MedlinePlus

    ... turn the volume higher when you listen to music, the radio or television Some causes of hearing ... levels include snowmobiling, motorcycling or listening to loud music. Some medications. Drugs, such as the antibiotic gentamicin ...

  13. Canine hearing loss management.

    PubMed

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

    2012-11-01

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

  14. 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 ...

  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. Managing Hearing Loss

    MedlinePlus

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

  17. Hearing loss - infants

    MedlinePlus

    ... loss. Two common tests are used to screen newborn infants for hearing loss: Auditory brain stem response (ABR) ... Over 30 states in the United States require newborn hearing screenings. Treating hearing loss early can allow many infants to develop normal language skills without delay. In ...

  18. [Hearing loss in adults].

    PubMed

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

    2009-05-20

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

  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. What's Hearing Loss?

    MedlinePlus

    ... pathologists to help them develop their hearing and speaking skills. Some people with hearing loss may need to use special techniques like these to communicate: speechreading (also ... without speaking. Remember: American Sign Language (ASL) is different from ...

  1. 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.

  2. 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…

  3. Congenital hearing loss.

    PubMed

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

    2017-01-12

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

  4. [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.

  5. Hearing Loss in Children

    MedlinePlus

    ... Español (Spanish) Recommend on Facebook Tweet Share Compartir Hearing loss can affect a child’s ability to develop communication, language, and social skills. The earlier children with hearing loss start getting services, the more likely they are ...

  6. 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…

  7. 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…

  8. Hearing loss and music

    MedlinePlus

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

  9. 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

  10. Sudden sensorineural hearing loss.

    PubMed

    Stew, B T; Fishpool, S J C; Williams, H

    2012-02-01

    Sudden onset sensorineural hearing loss is a medical emergency that continues to be poorly understood despite being recognized in the literature since 1944 (De Kleyn, 1944). A commonly used criterion to qualify for this diagnosis is a sensorineural hearing loss over three contiguous pure-tone frequencies of 30 dB or more that develops within 72 hours. The vast majority of cases are unilateral and the estimated annual incidence is 20 per 100 000 persons (Nosrati-Zarenoe et al, 2007). A cause for the hearing loss is only identified in up to 10% of cases but 50% of patients will improve spontaneously (Penido et al, 2009).

  11. 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,…

  12. What's Hearing Loss?

    MedlinePlus

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

  13. 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 Military jobs that involve combat, aircraft ...

  14. 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 ...

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

    PubMed

    del Castillo, Francisco J; del Castillo, Ignacio

    2011-06-01

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

  16. 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

  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. Could Anemia Cause Hearing Loss?

    MedlinePlus

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

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

    PubMed

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

    2007-11-01

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

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

    PubMed

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

    2014-10-01

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

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

    PubMed Central

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

    2015-01-01

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

  2. Sudden sensorineural hearing loss.

    PubMed

    Schreiber, Benjamin E; Agrup, Charlotte; Haskard, Dorian O; Luxon, Linda M

    2010-04-03

    Sudden sensorineural hearing loss is usually unilateral and can be associated with tinnitus and vertigo. In most cases the cause is not identified, although various infective, vascular, and immune causes have been proposed. A careful examination is needed to exclude life threatening or treatable causes such as vascular events and malignant diseases, and patients should be referred urgently for further assessment. About half of patients completely recover, usually in about 2 weeks. Many treatments are used, including corticosteroids, antiviral drugs, and vasoactive and oxygen-based treatments. Although no treatment is proven, we recommend a short course of oral high-dose corticosteroids. There is much to learn about pathogenesis of sudden sensorineural hearing loss, and more clinical trials are needed to establish evidence-based management.

  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.

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

    PubMed

    Lenz, Danielle R; Avraham, Karen B

    2011-11-01

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

  5. Hereditary hearing loss with thyroid abnormalities.

    PubMed

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

    2011-01-01

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

  6. 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…

  7. 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…

  8. 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 ...

  9. 10 Ways to Identify Hearing Loss

    MedlinePlus

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

  10. 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.

  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. 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…

  13. 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

  14. Occupational hearing loss in Korea.

    PubMed

    Kim, Kyoo Sang

    2010-12-01

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

  15. 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

  16. Sudden hearing loss in children.

    PubMed

    Ječmenica, Jovana; Bajec-Opančina, Aleksandra

    2014-08-01

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

  17. 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.

  18. Use of Hearing Aids by Adults with Hearing Loss

    MedlinePlus

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

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

    PubMed

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

    2015-06-01

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

  20. Sudden Hearing Loss.

    ERIC Educational Resources Information Center

    Vaughan, John C.

    1997-01-01

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

  1. 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.

  2. 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 ...

  3. 10 Ways to Identify Hearing Loss

    MedlinePlus

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

  4. Genetics Home Reference: nonsyndromic hearing loss

    MedlinePlus

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

  5. 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.

  6. 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.

  7. 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,…

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

    PubMed

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

    2012-01-01

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

  9. Hearing: Noise-Induced Hearing Loss

    MedlinePlus

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

  10. Sudden bilateral hearing loss after organophosphate inhalation.

    PubMed

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

    2016-12-01

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

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

    PubMed Central

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

    2015-01-01

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

  12. Idiopathic Sudden Sensorineural Hearing Loss With Minimal Hearing Impairment

    PubMed Central

    Cho, Chin Saeng

    2015-01-01

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

  13. 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…

  14. Reading Recovery for Children with Hearing Loss

    ERIC Educational Resources Information Center

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

    2006-01-01

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

  15. Clinical aspects of hereditary hearing loss.

    PubMed

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

    2007-07-01

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

  16. Identifying hearing loss by means of iridology.

    PubMed

    Stearn, Natalie; Swanepoel, De Wet

    2006-11-13

    Isolated reports of hearing loss presenting as markings on the iris exist, but to date the effectiveness of iridology to identify hearing loss has not been investigated. This study therefore aimed to determine the efficacy of iridological analysis in the identification of moderate to profound sensorineural hearing loss in adolescents. A controlled trial was conducted with an iridologist, blind to the actual hearing status of participants, analyzing the irises of participants with and without hearing loss. Fifty hearing impaired and fifty normal hearing subjects, between the ages of 15 and 19 years, controlled for gender, participated in the study. An experienced iridologist analyzed the randomised set of participants' irises. A 70% correct identification of hearing status was obtained by iridological analyses with a false negative rate of 41% compared to a 19% false positive rate. The respective sensitivity and specificity rates therefore came to 59% and 81%. Iridological analysis of hearing status indicated a statistically significant relationship to actual hearing status (P < 0.05). Although statistically significant sensitivity and specificity rates for identifying hearing loss by iridology were not comparable to those of traditional audiological screening procedures.

  17. Hearing Loss in Children: Types of Hearing Loss

    MedlinePlus

    ... happen when any part of the ear or auditory (hearing) system is not working in the usual way. Outer ... sound information from the ear to the brain. Auditory (Hearing) System The auditory pathway processes sound information as it ...

  18. 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.

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

    Code of Federal Regulations, 2010 CFR

    2010-04-01

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

  20. Cleidocranial dysplasia with hearing loss

    PubMed Central

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

    2013-01-01

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

  1. 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.

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

    PubMed

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

    1997-03-01

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

  3. 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…

  4. Sudden sensorineural hearing loss after heroin injection.

    PubMed

    Schrock, Andreas; Jakob, Mark; Wirz, Stefan; Bootz, Friedrich

    2008-05-01

    Sudden sensorineural hearing loss is a symptom of cochlear injury. Potential aetiologies are vascular diseases, viral infections, allergic reactions, autoimmune disorders, and traumatic rupture of the intralabyrinthe membrane. Unlike in unilateral cases bilateral sensorineural hearing loss is often associated with specific disease entities. We report a case of sudden bilateral deafness after intravenous heroin abuse. The putative pathophysiological mechanisms are discussed.

  5. Hearing loss and dementia: new insights.

    PubMed

    Albers, Kristi

    2012-01-01

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

  6. Hearing Loss in Children: Data and Statistics

    MedlinePlus

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

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

    PubMed

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

    2016-10-01

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

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

    PubMed

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

    1994-07-01

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

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

    PubMed Central

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

    1994-01-01

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

  10. Age-Related Hearing Loss

    MedlinePlus

    ... hearing aids Source: NIH/NIDCD Hearing aids are electronic instruments you wear in or behind your ear ( ... implants. Cochlear (COKE-lee-ur) implants are small electronic devices surgically implanted in the inner ear that ...

  11. Chikungunya virus induced sudden sensorineural hearing loss.

    PubMed

    Bhavana, Kranti; Tyagi, Isha; Kapila, Rajeev Kumar

    2008-02-01

    The aim of this study is to demonstrate the association of Chikungunya virus and sudden sensorineural hearing loss. In the case report described we had a case which developed sudden unilateral sensorineural hearing loss following chikungunya fever. A 15-year-old female presented to us with the complains of unilateral sudden onset of hearing loss following an episode of fever, arthralgia and rashes 1 month ago. At the time of these symptoms there were many cases of chikungunya fever in the city, three being in her locality. Clinically Chikungunya fever was suspected and a positive serological test further confirmed our diagnosis. The hearing loss could thus be attributed to Chikungunya virus. Viruses have always been implicated in causing sudden sensorineural hearing loss but Chikungunya virus as a cause has not been documented earlier making this case report a unique one.

  12. Non-syndromic, autosomal-recessive deafness.

    PubMed

    Petersen, M B; Willems, P J

    2006-05-01

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

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

    Code of Federal Regulations, 2011 CFR

    2011-04-01

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

  14. 20 CFR 702.441 - Claims for loss of hearing.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

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

  15. 20 CFR 702.441 - Claims for loss of hearing.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

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

  16. 20 CFR 702.441 - Claims for loss of hearing.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

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

  17. Sudden hearing loss associated with methylphenidate therapy.

    PubMed

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

    2014-01-01

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

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

    PubMed

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

    2000-09-01

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

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

    PubMed

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

    2012-10-01

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

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

    PubMed

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

    2015-09-01

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

  1. Unilateral sudden hearing loss due to otosyphilis.

    PubMed

    de Goffau, Maaike J; Doelman, Jan C; van Rijswijk, Jeroen B

    2011-09-28

    The differential diagnosis of sudden hearing loss is very extensive. However, in most patients, no underlying pathology will be found and the patient is consequently diagnosed with idiopathic sudden hearing loss. Nevertheless, it is important that one stays alert in everyday routine practice to less common but potentially treatable pathology. We report a case of a 53-year old patient with sudden unilateral hearing loss and tinnitus caused by otosyphilis. Diagnosis, therapy and the incidence of (oto)syphilis are discussed. Also, as ENT-surgeons, it is important to realise that syphilis is still with us, and that its prevalence might well increase in the near future.

  2. [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.

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

    PubMed

    Kalatzis, V; Petit, C

    1998-01-01

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

  4. 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…

  5. Vibrant Soundbridge rehabilitation of sensorineural hearing loss.

    PubMed

    Khan, Andleeb; Hillman, Todd; Chen, Douglas

    2014-12-01

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

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

    PubMed

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

    2013-01-01

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

  7. Acoustic Trauma - Hearing Loss in Teenagers

    MedlinePlus

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

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

    PubMed

    Gimsing, Steen

    2014-07-28

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

  9. Sudden sensorineural hearing loss associated with vardenafil.

    PubMed

    Snodgrass, Amanda J; Campbell, Heather M; Mace, David L; Faria, Victoria L; Swanson, Kathleen M; Holodniy, Mark

    2010-01-01

    The phosphodiesterase type 5 (PDE-5) inhibitors-sildenafil, vardenafil, and tadalafil-are used primarily in erectile dysfunction, but sildenafil is also indicated for pulmonary hypertension. Common adverse effects of vardenafil include headache, flushing, nasal congestion, dyspepsia, and nausea. Recently, PDE-5 inhibitors have been associated with adverse vision effects, and emerging evidence now indicates that they may also be responsible for hearing changes and hearing loss. We describe a patient who developed unilateral sudden sensorineural hearing loss possibly related to the use of vardenafil for erectile dysfunction. To our knowledge, only one other case of hearing loss related to this drug class has been published. Our patient was a 57-year-old man who came to the emergency department with right-sided mild-to-moderate hearing loss in the 500-3000-Hz range, confirmed by audiogram, that occurred after ingestion of vardenafil. The patient was hospitalized 2 days later for administration of intravenous dexamethasone, followed by oral prednisone. He reported that his hearing had improved on the fourth hospital day and was discharged 3 days later, continuing to taper the prednisone on an outpatient basis. A repeat audiogram after 10 days of corticosteroid therapy confirmed that his hearing in the 500-3000-Hz range was within normal limits. Use of the Naranjo adverse drug reaction probability scale indicated a possible (score of 3) adverse reaction of sudden sensorineural hearing loss associated with vardenafil consumption. We also performed an analysis of hearing loss cases related to PDE-5 inhibitors in the United States Food and Drug Administration's Adverse Event Reporting System database to compare the characteristics of our patient with those of other reported adverse event cases. Based on the temporal relation of the sudden sensorineural hearing loss to this patient's drug consumption, we propose that the vardenafil is a likely cause of the hearing loss

  10. 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.

  11. [Sensorineural hearing loss due to neonatal hyperbilirubinemia].

    PubMed

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

    2003-01-01

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

  12. Hearing Loss: Symptoms, Diagnosis & Treatment

    MedlinePlus

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

  13. Hearing Loss and Older Adults

    MedlinePlus

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

  14. Medulloblastoma manifesting as sudden sensorineural hearing loss.

    PubMed

    Terakawa, Yuzo; Tsuyuguchi, Naohiro; Takami, Toshihiro; Ohata, Kenji

    2011-07-01

    We present a rare case of medulloblastoma which presented with unilateral sudden sensorineural hearing loss as an initial symptom. A 19-year-old man was admitted to our hospital with a chief complaint of dizziness and facial numbness on the right side. His illness had begun two years previously with sudden hearing loss on the right side, for which he had been treated as an idiopathic sudden hearing loss. Magnetic resonance imaging demonstrated abnormal signals located mainly in the right middle cerebellar peduncle. We performed partial resection of the tumor by suboccipital craniotomy. The histopathological diagnosis was medulloblastoma. Intrinsic brain tumor is an extremely rare cause of sudden sensorineural hearing loss and is therefore easily overlooked as was in the present case. The present case highlights not only the need to evaluate patients with sudden sensorineural hearing loss by magnetic resonance imaging but also the importance of paying attention to intrinsic lesions involving the brainstem. Although this condition like the presented case might be rare, intrinsic brain tumor should be considered as a potential cause of sudden sensorineural hearing loss, as it may be easily missed leading to a delay in appropriate treatment.

  15. Gender effects in hearing loss in Dalmatians.

    PubMed

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

    2001-01-17

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

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

    ERIC Educational Resources Information Center

    Lass, Norman J.; And Others

    1990-01-01

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

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

    PubMed

    Saunders, Gabrielle H; Griest, Susan E

    2009-01-01

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

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

    PubMed

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

    1992-03-01

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

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

    PubMed Central

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

    2015-01-01

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

  20. 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.

  1. Uncovering effective strategies for hearing loss prevention.

    PubMed

    Morata, Thais C; Meinke, Deanna

    2016-04-01

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

  2. Uncovering effective strategies for hearing loss prevention

    PubMed Central

    Morata, Thais C.; Meinke, Deanna

    2016-01-01

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

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

    ERIC Educational Resources Information Center

    Garstecki, Dean C.; Erler, Susan F.

    1998-01-01

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

  4. 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

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

    PubMed

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

    2016-04-01

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

  6. 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

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

    PubMed

    Wingard, Jeffrey C; Zhao, Hong-Bo

    2015-01-01

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

  8. 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

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

    PubMed

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

    2016-04-01

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

  10. Dichotic listening in adults with sensorineural hearing loss.

    PubMed

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

    1976-01-01

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

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

    PubMed

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

    2016-01-01

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

  12. 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

  13. Sensorineural hearing loss in Kawasaki disease.

    PubMed

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

    2016-01-01

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

  14. Auditory neuroplasticity, hearing loss and cochlear implants.

    PubMed

    Ryugo, David

    2015-07-01

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

  15. 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.

  16. 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…

  17. 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.

  18. 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.

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

    PubMed

    Noben-Trauth, Konrad; Johnson, Kenneth R

    2009-06-24

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

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

    PubMed

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

    2013-08-01

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

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

    PubMed

    Lüers, Jan-Christoffer; Hüttenbrink, Karl-Bernd

    2014-12-01

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

  2. Noise and Hearing Loss Prevention: Facts and Statistics

    MedlinePlus

    ... NIOSHNoise Noise and Hearing Loss on the NIOSH Science Blog Smartphone Sound Apps Measuring the Impact of ... NIOSHNoise Noise and Hearing Loss on the NIOSH Science Blog Smartphone Sound Apps Measuring the Impact of ...

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

    PubMed

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

    2015-03-01

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

  4. 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.

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

    PubMed

    Doroszewska, Grazyna; Kaźmierczak, Henryk

    2002-01-01

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

  6. Genetics of Hearing Loss – Syndromic

    PubMed Central

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

    2015-01-01

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

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

    PubMed Central

    Kerr, Leslie Dubin

    2016-01-01

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

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

    PubMed

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

    2015-08-01

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

  9. 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

  10. 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.

  11. Personal Sound Amplifiers for Adults with Hearing Loss

    PubMed Central

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

    2015-01-01

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

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

    PubMed Central

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

    2017-01-01

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

  13. 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.

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

    PubMed

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

    2015-04-01

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

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

    PubMed Central

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

    2014-01-01

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

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

    PubMed

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

    2006-05-01

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

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

    PubMed Central

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

    2012-01-01

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

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

    PubMed Central

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

    2014-01-01

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

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

    PubMed

    Taiji, Hidenobu; Morimoto, Noriko

    2012-07-01

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

  1. Laterality of sudden sensorineural hearing loss.

    PubMed

    Reiss, Michael; Reiss, Gilfe

    2014-08-01

    It is known that sudden sensorineural hearing loss and other otoneurologic diseases, such as tinnitus or Ménière disease, occur more frequently in the left ear than in the right. We studied lateralization of sudden deafness in 489 patients treated at Radebeul Hospital from January 2004 to December 2009. The male-to-female ratio was 1:1.24; we found a predominance of the left side only in female patients. The cause for this predominance is unclear. The slight asymmetry might indicate a greater vulnerability of the left inner ear in women, suggesting hormonal factors in the genesis of sudden deafness.

  2. 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

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

    PubMed

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

    2015-03-09

    Children with hearing loss are at risk for lower self-esteem due to differences from hearing peers relative to communication skills, physical appearance, and social maturity. This study examines the influence of generic factors unrelated to hearing loss (e.g., age, gender, temperament) and specific factors associated with hearing loss (e.g., age at identification, communication skills) on how children with hearing loss wearing cochlear implants or hearing aids appraise self-esteem. Fifty children with hearing loss wearing cochlear implants or hearing aids participated (Mean age: 12.88 years; mean duration of device use: 3.43 years). Participants independently completed online questionnaires to assess communication skills, social engagement, self-esteem, and temperament. Children with hearing loss rated global self-esteem significantly more positively than hearing peers, t = 2.38, p = .02. Self-esteem ratings attained significant positive correlations with affiliation (r = .42, p = .002) and attention (r = .45, p = .001) temperaments and a significant negative association with depressive mood (r = - .60, p < .0001). No significant correlations emerged between self-esteem and demographic factors, communication skills, or social engagement. Because successful communication abilities do not always co-occur with excellent quality of life, clinicians and professionals working with children with hearing loss need to understand components contributing to self-esteem to improve identification, counseling, and external referrals for children in this population.

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

    PubMed

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

    2014-07-29

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

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

    PubMed Central

    Cohen, Brandon E.; Durstenfeld, Anne

    2014-01-01

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

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

    PubMed Central

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

    2016-01-01

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

  7. 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

  8. Sensorineural hearing loss and auditory perceptual organization

    NASA Astrophysics Data System (ADS)

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

    2004-05-01

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

  9. Noise-induced hearing loss in Asia.

    PubMed

    Fuente, Adrian; Hickson, Louise

    2011-03-01

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

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

    PubMed

    Iossa, Sandra; Marciano, Elio; Franzé, Annamaria

    2011-11-01

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

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

    PubMed Central

    Iossa, Sandra; Marciano, Elio; Franzé, Annamaria

    2011-01-01

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

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

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-06-01

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

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

    ERIC Educational Resources Information Center

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

    2008-01-01

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

  15. Noise Exposure and Hearing Loss in Rural Children.

    ERIC Educational Resources Information Center

    Woodford, Charles M.; Lass, Norman J.

    1993-01-01

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

  16. 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).…

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

    PubMed

    Page, Joshua Cody; Peters, Bob

    2015-01-01

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

  18. Sudden hearing loss following non-petrous craniotomy.

    PubMed

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

    2010-01-01

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

  19. 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.

  20. Prevalence of minimal hearing loss in South Korea

    PubMed Central

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

    2017-01-01

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

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

    PubMed

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

    2016-12-15

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

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

    PubMed

    Bobsin, Lori L; Houston, K Todd

    2015-12-01

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

  3. [Sudden unilateral sensorineural hearing loss after carbon monoxide intoxication].

    PubMed

    Michalska-Piechowiak, Teresa; Miarzyńska, Maria; Perlik-Gattner, Irena

    2004-01-01

    A case of unilateral sensorineural hearing loss of the left ear after carbon monoxide intoxication was presented. The diagnosis was based upon an interview, medical examinations and audiometric investigations. Results of diagnostic evaluations, clinical presentation and treatment were discussed. Hearing improvement was obtained after 6 days of treatment and normal hearing returned after 14 days. Patients who suffer from CO intoxication are at risk of hearing impairment, therefore, there is a need for audiometric follow up in these patients.

  4. Hearing loss and tinnitus in adolescents and young adults

    NASA Astrophysics Data System (ADS)

    Holmes, Alice

    2004-05-01

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

  5. 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.

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

    PubMed Central

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

    2015-01-01

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

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

    PubMed Central

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

    2016-01-01

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

  8. 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.

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

    PubMed

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

    2017-02-01

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

  10. 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

  11. 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…

  12. 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.

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

    PubMed

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

    2014-06-01

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

  14. 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)

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

    ERIC Educational Resources Information Center

    Lyon, David J.; And Others

    1986-01-01

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

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

    PubMed

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

    2016-12-07

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

  17. 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.

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

    PubMed

    Crysdale, W S

    1978-08-01

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

  19. Teaching Vocabulary to Preschool Children with Hearing Loss

    ERIC Educational Resources Information Center

    Lund, Emily; Douglas, W. Michael

    2016-01-01

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

  20. 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…

  1. Noise Induced Hearing Loss among School Band Directors.

    ERIC Educational Resources Information Center

    Cutietta, Robert A.; And Others

    1989-01-01

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

  2. Call centers and noise-induced hearing loss

    PubMed Central

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

    2016-01-01

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

  3. Hearing loss and employment in the United States.

    PubMed

    Kooser, Cathy

    2013-01-01

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

  4. 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…

  5. Hearing Loss Signals Need for Diagnosis

    MedlinePlus

    ... FDA strongly encourages a medical evaluation before the purchase of a hearing aid. Hearing aid sellers are ... feeds Follow FDA on Twitter Follow FDA on Facebook View FDA videos on YouTube View FDA photos ...

  6. Head Position Comparison between Students with Normal Hearing and Students with Sensorineural Hearing Loss

    PubMed Central

    Melo, Renato de Souza; Amorim da Silva, Polyanna Waleska; Souza, Robson Arruda; Raposo, Maria Cristina Falcão; Ferraz, Karla Mônica

    2013-01-01

    Introduction Head sense position is coordinated by sensory activity of the vestibular system, located in the inner ear. Children with sensorineural hearing loss may show changes in the vestibular system as a result of injury to the inner ear, which can alter the sense of head position in this population. Aim Analyze the head alignment in students with normal hearing and students with sensorineural hearing loss and compare the data between groups. Methods This prospective cross-sectional study examined the head alignment of 96 students, 48 with normal hearing and 48 with sensorineural hearing loss, aged between 7 and 18 years. The analysis of head alignment occurred through postural assessment performed according to the criteria proposed by Kendall et al. For data analysis we used the chi-square test or Fisher exact test. Results The students with hearing loss had a higher occurrence of changes in the alignment of the head than normally hearing students (p < 0.001). Forward head posture was the type of postural change observed most, occurring in greater proportion in children with hearing loss (p < 0.001), followed by the side slope head posture (p < 0.001). Conclusion Children with sensorineural hearing loss showed more changes in the head posture compared with children with normal hearing. PMID:25992037

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

    PubMed

    Keppler, Hannah; Dhooge, Ingeborg; Vinck, Bart

    2015-01-01

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

  8. 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

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

    PubMed

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

    2014-01-01

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

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

    PubMed

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

    2014-01-01

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

  11. 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

  12. Sensorineural hearing loss in hemodialysis patients.

    PubMed

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

    2010-03-01

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

  13. 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

  14. 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…

  15. 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

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

    PubMed Central

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

    2016-01-01

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

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

    PubMed

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

    2015-04-01

    This study describes procedures for constructing equal-loudness contours (ELCs) in units of phons from categorical loudness scaling (CLS) data and characterizes the impact of hearing loss on these estimates of loudness. Additionally, this study developed a metric, level-dependent loudness loss, which uses CLS data to specify the deviation from normal loudness perception at various loudness levels and as function of frequency for an individual listener with hearing loss. CLS measurements were made in 87 participants with hearing loss and 61 participants with normal hearing. An assessment of the reliability of CLS measurements was conducted on a subset of the data. CLS measurements were reliable. There was a systematic increase in the slope of the low-level segment of the CLS functions with increase in the degree of hearing loss. ELCs derived from CLS measurements were similar to standardized ELCs (International Organization for Standardization, ISO 226:2003). The presence of hearing loss decreased the vertical spacing of the ELCs, reflecting loudness recruitment and reduced cochlear compression. Representing CLS data in phons may lead to wider acceptance of CLS measurements. Like the audiogram that specifies hearing loss at threshold, level-dependent loudness loss describes deficit for suprathreshold sounds. Such information may have implications for the fitting of hearing aids.

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

    PubMed

    Garstecki, D C; Erler, S F

    2001-12-01

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

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

    PubMed

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

    2013-01-01

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

  20. 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.

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

    PubMed

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

    2013-11-01

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

  2. 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…

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

    PubMed

    Onsori, Habib; Rahmati, Mohammad; Fazli, Davood

    2014-12-01

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

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

    PubMed

    Christensen, Vibeke Tornhøj; Datta Gupta, Nabanita

    2017-02-01

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

  5. 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

  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.

  7. 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

  8. 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

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

    PubMed

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

    2011-11-01

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

  10. 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

  11. Course of hearing loss and occurrence of tinnitus.

    PubMed

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

    2006-11-01

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

  12. 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…

  13. 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,…

  14. Screening for Hearing Loss in Early Childhood Programs

    ERIC Educational Resources Information Center

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

    2007-01-01

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

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

    ERIC Educational Resources Information Center

    Partington, Gary; Galloway, Ann

    2005-01-01

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

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

    PubMed

    Puschmann, Sebastian; Thiel, Christiane M

    2017-01-01

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

  17. Risk factors for sensorineural hearing loss in children.

    PubMed

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

    2012-01-01

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

  18. 38 CFR 20.717 - Rule 717. Loss of hearing tapes or transcripts-motion for new hearing.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

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

  19. 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 tapes or transcripts-motion for new hearing. 20.717 Section 20.717 Pensions, Bonuses, and Veterans... on Appeal § 20.717 Rule 717. Loss of hearing tapes or transcripts—motion for new hearing. (a)...

  20. 38 CFR 20.717 - Rule 717. Loss of hearing tapes or transcripts-motion for new hearing.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 2 2014-07-01 2014-07-01 false Rule 717. Loss of hearing tapes or transcripts-motion for new hearing. 20.717 Section 20.717 Pensions, Bonuses, and Veterans... on Appeal § 20.717 Rule 717. Loss of hearing tapes or transcripts—motion for new hearing. (a)...

  1. 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 tapes or transcripts-motion for new hearing. 20.717 Section 20.717 Pensions, Bonuses, and Veterans... on Appeal § 20.717 Rule 717. Loss of hearing tapes or transcripts—motion for new hearing. (a)...

  2. 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 tapes or transcripts-motion for new hearing. 20.717 Section 20.717 Pensions, Bonuses, and Veterans... on Appeal § 20.717 Rule 717. Loss of hearing tapes or transcripts—motion for new hearing. (a)...

  3. Hearing Loss in Children: Treatment and Intervention Services

    MedlinePlus

    ... that early intervention services can greatly improve a child’s development. Babies that are diagnosed with hearing loss should ... just a supportive listener. Related Links Birth Defects Child Development Developmental Disabilities Learn the Signs. Act Early. Campaign ...

  4. [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.

  5. 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

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

    PubMed

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

    2009-09-01

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

  7. Evaluation of Jet Fuel Induced Hearing Loss in Rats

    DTIC Science & Technology

    2011-10-13

    evaluate the potency of JP-8 jet fuel to enhance noise-induced hearing loss ( NIHL ) using inhalation exposure to fuel and simultaneous exposure to...of the current work was to evaluate the potency of JP-8 jet fuel to enhance noise-induced hearing loss ( NIHL ) using inhalation exposure to fuel and...and the characterization of JP-8 exposure able to increase susceptibility to NIHL . Once these objectives were met using a continuous noise exposure

  8. Noise-Induced Hearing Loss in Canadian Military Personnel

    DTIC Science & Technology

    2005-04-01

    results in permanent hearing loss, generally referred to as either noise-induced hearing loss ( NIHL ) or noise-induced permanent threshold shift...to Franks et al. [6], NIHL is among the most prevalent work-related medical conditions in both the United States and Europe. Biological changes from... NIHL from small firearms and large-calibre weapons was investigated by Christiansson and Wintzell [15]. The subjects were 204 infantry officers who

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

    PubMed

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

    2015-01-01

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

  10. Enlarged Vestibular Aqueducts and Childhood Hearing Loss

    MedlinePlus

    ... EVA. However, this is a rare event in commercial aircraft with pressurized cabins. If you have EVA, ... of communication, such as sign language or cued speech, or using assistive devices, such as a hearing ...

  11. 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.

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

    PubMed

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

    2014-10-01

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

  13. 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…

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

    ERIC Educational Resources Information Center

    Kumar, Prawin; Yathiraj, Asha

    2009-01-01

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

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

    ERIC Educational Resources Information Center

    Stegman, Robin Fern

    2016-01-01

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

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

    ERIC Educational Resources Information Center

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

    2013-01-01

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

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

    PubMed

    Foss, Katherine A

    2014-01-01

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

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

    PubMed Central

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

    2016-01-01

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

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

    PubMed Central

    Sanchez, Helmuth A.; Verselis, Vytas K.

    2014-01-01

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

  20. Minocycline attenuates noise-induced hearing loss in rats.

    PubMed

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

    2017-02-03

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

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

    PubMed

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

    2009-01-01

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

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

    PubMed Central

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

    2013-01-01

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

  3. Severe hearing loss in Dlxl mutant mice.

    PubMed

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

    2006-04-01

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

  4. 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.

  5. 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

  6. 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)

  7. 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

  8. 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

  9. 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.

  10. 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.

  11. Severe hearing loss in Pallister-Killian syndrome.

    PubMed

    Schuster, Maria; Hoppe, U; Eysholdt, U; Rosanowski, F

    2002-01-01

    Pallister-Killian syndrome is a rare disorder characterised by a specific combination of anatomic anomalies, mental retardation and lack of speech acquisition due to tetrasomy 12p. Hearing loss does not seem to be characteristic for this syndrome, although it was reported in several cases. We present the case of a girl first seen in our department at the age of 6 months. A severe sensory hearing loss was confirmed by subjective and objective audiometry. The child was successfully equipped with hearing aids. In the literature almost all children with Pallister-Killian syndrome are described as not developing verbal speech. Surprisingly their hearing abilities were not examined systematically. We advise audiological testing of children with Pallister-Killian syndrome.

  12. 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

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

    PubMed Central

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

    2015-01-01

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

  14. Clinical Study on 136 Children with Sudden Sensorineural Hearing Loss

    PubMed Central

    Li, Feng-Jiao; Wang, Da-Yong; Wang, Hong-Yang; Wang, Li; Yang, Feng-Bo; Lan, Lan; Guan, Jing; Yin, Zi-Fang; Rosenhall, Ulf; Yu, Lan; Hellstrom, Sten; Xue, Xi-Jun; Duan, Mao-Li; Wang, Qiu-Ju

    2016-01-01

    Background: The prevalence of sudden sensorineural hearing loss in children (CSSNHL) is consistently increasing. However, the pathology and prognosis of CSSNHL are still poorly understood. This retrospective study evaluated clinical characteristics and possible associated factors of CSSNHL. Methods: One hundred and thirty-six CSSNHL patients treated in Department of Otolaryngology-Head and Neck Surgery and Institute of Otolaryngology at Chinese PLA General Hospital between July 2008 and August 2015 were included in this study. These patients were analyzed for clinical characteristics, audiological characteristics, laboratory examinations, and prognostic factors. Results: Among the 136 patients (151 ears), 121 patients (121 ears, 80.1%) were diagnosed with unilaterally CSSNHL, and 15 patients (30 ears, 19.9%) with bilateral CSSNHL. The complete recovery rate of CSSNHL was 9.3%, and the overall recovery rate was 37.7%. We found that initial degree of hearing loss, onset of treatment, tinnitus, the ascending type audiogram, gender, side of hearing loss, the recorded auditory brainstem response (ABR), and distortion product otoacoustic emissions (DPOAEs) had prognostic significance. Age, ear fullness, and vertigo had no significant correlation with recovery. Furthermore, the relevant blood tests showed 30.8% of the children had abnormal white blood cell (WBC) counts, 22.1% had elevated homocysteine levels, 65.8% had high alkaline phosphatase (ALP), 33.8% had high IgE antibody levels, and 86.1% had positive cytomegalovirus (CMV) IgG antibodies. Conclusions: CSSNHL commonly occurs unilaterally and results in severe hearing loss. Initial severe hearing loss and bilateral hearing loss are negative prognostic factors for hearing recovery, while positive prognostic factors include tinnitus, gender, the ascending type audiogram, early treatment, identifiable ABR waves, and DPOAEs. Age, vertigo, and ear fullness are not correlated with the recovery. Some serologic indicators

  15. 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.

  16. 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.

  17. Neural Alterations in Acquired Age-Related Hearing Loss.

    PubMed

    Mudar, Raksha A; Husain, Fatima T

    2016-01-01

    Hearing loss is one of the most prevalent chronic health conditions in older adults. Growing evidence suggests that hearing loss is associated with reduced cognitive functioning and incident dementia. In this mini-review, we briefly examine literature on anatomical and functional alterations in the brains of adults with acquired age-associated hearing loss, which may underlie the cognitive consequences observed in this population, focusing on studies that have used structural and functional magnetic resonance imaging, diffusion tensor imaging, and event-related electroencephalography. We discuss structural and functional alterations observed in the temporal and frontal cortices and the limbic system. These neural alterations are discussed in the context of common cause, information-degradation, and sensory-deprivation hypotheses, and we suggest possible rehabilitation strategies. Although, we are beginning to learn more about changes in neural architecture and functionality related to age-associated hearing loss, much work remains to be done. Understanding the neural alterations will provide objective markers for early identification of neural consequences of age-associated hearing loss and for evaluating benefits of intervention approaches.

  18. Sudden onset unilateral sensorineural hearing loss after rabies vaccination.

    PubMed

    Okhovat, Saleh; Fox, Richard; Magill, Jennifer; Narula, Antony

    2015-12-15

    A 33-year-old man developed profound sudden onset right-sided hearing loss with tinnitus and vertigo, within 24 h of pretravel rabies vaccination. There was no history of upper respiratory tract infection, systemic illness, ototoxic medication or trauma, and normal otoscopic examination. Pure tone audiograms (PTA) demonstrated right-sided sensorineural hearing loss (thresholds 90-100 dB) and normal left-sided hearing. MRI internal acoustic meatus, viral serology (hepatitis B, C, HIV and cytomegalovirus) and syphilis screen were normal. Positive Epstein-Barr virus IgG, viral capsid IgG and anticochlear antibodies (anti-HSP-70) were noted. Initial treatment involved a course of high-dose oral prednisolone and acyclovir. Repeat PTAs after 12 days of treatment showed a small improvement in hearing thresholds. Salvage intratympanic steroid injections were attempted but failed to improve hearing further. Sudden onset sensorineural hearing loss (SSNHL) is an uncommon but frightening experience for patients. This is the first report of SSNHL following rabies immunisation in an adult.

  19. Influences of Working Memory and Audibility on Word Learning in Children with Hearing Loss

    ERIC Educational Resources Information Center

    Stiles, Derek Jason

    2010-01-01

    As a group, children with hearing loss demonstrate delays in language development relative to their peers with normal hearing. Early intervention has a profound impact on language outcomes in children with hearing loss. Data examining the relationship between degree of hearing loss and language outcomes are variable. Two approaches are used in the…

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

    ERIC Educational Resources Information Center

    Pinquart, Martin; Pfeiffer, Jens P.

    2014-01-01

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

  1. A Survey of Classroom Teachers' and Special Educators' Knowledge of and Exposure to Hearing Loss.

    ERIC Educational Resources Information Center

    Lass, Norman J.; And Others

    1985-01-01

    A questionnaire on aspects of hearing loss, including prevalence, etiology, hearing aids, testing prevention, and treatment of hearing loss, was completed by 98 classroom teachers and 77 special educators. Results indicate teachers have limited knowledge of and exposure to hearing loss. (Author/CL)

  2. Hearing loss features in GJB2 biallelic mutations and GJB2/GJB6 digenic inheritance in a large Italian cohort.

    PubMed

    Cama, Elona; Melchionda, Salvatore; Palladino, Teresa; Carella, Massimo; Santarelli, Rosamaria; Genovese, Elisabetta; Benettazzo, Filippo; Zelante, Leopoldo; Arslan, Edoardo

    2009-01-01

    The aim of this study was to describe the clinical features of hearing loss due to mutations on connexin 26/30 coding genes (GJB2/GJB6). Mutations in the GJB2 gene are found to account for approximately 50% of cases of autosomal recessive non-syndromic deafness. Several European studies have estimated that the GJB2 healthy carrier condition involves about 2-4% of the population, with the 35delG mutations being the most common. A 342-kb deletion truncating the GJB6 gene (encoding connexin-30) has been associated with autosomal recessive non-syndromic deafness, mostly as digenic inheritance of the Cx30 deletion/Cx26 mutation. The following retrospective study describes audiological features and genotypes of a large cohort of 376 Italian hearing-impaired patients who underwent genetic screening for the GJB2/GJB6 genes and received follow-up care at our centre between January 2002 and October 2006. Sixteen different genotypes causing deafness in more than 27% of patients with either biallelic mutations or digenic inheritance GJB2/GJB6 were identified. The most frequent mutations were 35delG, M34T, L90P, and R184P.

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

    PubMed

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

    2015-07-01

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

  4. Bilateral sudden sensorineural hearing loss in Staphylococcus aureus endocarditis.

    PubMed

    Lau, Joanne Wai Ling; Ceranic, Borka; Harris, Robert; Timehin, Elwina

    2015-09-14

    This case highlights the diagnostic challenges in patients presenting with bilateral sudden sensorinueral hearing loss (SNHL). The aetiology of bilateral sudden SNHL may span several medical disciplines. Therefore, clinicians should be mindful of such presentations, and consider aetiologies beyond otological and neurological causes. We present a case of a previously healthy 51-year-old woman who presented with coryzal symptoms and sudden audiovestibular failure. Examination revealed fever, tachycardia, bilateral profound hearing loss and nystagmus. Following investigations, an initial working diagnosis of vasculitis was made. Later, blood cultures revealed methicillin-sensitive Staphylococcus aureus (MSSA) and a transoesophageal echocardiogram confirmed endocarditis. The patient made a good recovery, but the hearing loss was permanent and managed with a cochlear implant.

  5. Protection from noise-induced hearing loss with Src inhibitors.

    PubMed

    Bielefeld, Eric C

    2015-06-01

    Noise-induced hearing loss is a major cause of acquired hearing loss around the world and pharmacological approaches to protecting the ear from noise are under investigation. Noise results in a combination of mechanical and metabolic damage pathways in the cochlea. The Src family of protein tyrosine kinases could be active in both pathways and Src inhibitors have successfully prevented noise-induced cochlear damage and hearing loss in animal models. The long-term goal is to optimize delivery methods into the cochlea to reduce invasiveness and limit side-effects before human clinical testing can be considered. At their current early stage of research investigation, Src inhibitors represent an exciting class of compounds for inclusion in a multifaceted pharmacological approach to protecting the ear from noise.

  6. The prevention of noise induced hearing loss in children.

    PubMed

    Harrison, Robert V

    2012-01-01

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

  7. Traumatic Labyrinthine Concussion in a Patient with Sensorineural Hearing Loss

    PubMed Central

    Chiaramonte, R.; Bonfiglio, M.; D'Amore, A.; Viglianesi, A.; Cavallaro, T.; Chiaramonte, I.

    2013-01-01

    Blunt head trauma without any temporal bone fracture or longitudinal temporal bone fracture, with an associated fracture of the labyrinth may cause labyrinthine injury with sensor neural hearing loss and vertigo because of a concussive injury to the membranous labyrinth. Sudden sensory neural hearing loss is relatively frequent. In most cases, the etiology is not discovered. One of the possible causes for sudden deafness is inner labyrinth bleeding or concussion, which were difficult to diagnose before the advent of magnetic resonance imaging. Vertigo without a demonstrable fracture may also be the result of labyrinthine concussion, cupololithiasis and perilymphatic fistula. We describe the clinical case of a patient with acute traumatic hearing loss and vertigo, without skull base fracture detected on computed tomography. Magnetic resonance study was also performed. We have integrated the discussion with features that allow the differential diagnosis from other similar conditions. PMID:23859168

  8. The Prevention of Noise Induced Hearing Loss in Children

    PubMed Central

    Harrison, Robert V.

    2012-01-01

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

  9. Hearing Loss in Osteogenesis Imperfecta: Characteristics and Treatment Considerations

    PubMed Central

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

    2011-01-01

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

  10. Frequency selectivity in canaries with a hereditary hearing loss

    NASA Astrophysics Data System (ADS)

    Lauer, Amanda M.; Dooling, Robert J.

    2002-05-01

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

  11. Secondhand Smoke Exposure and the Risk of Hearing Loss

    PubMed Central

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

    2011-01-01

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

  12. Hearing Loss as a Function of Aging and Diabetes Mellitus: A Cross Sectional Study

    PubMed Central

    Park, Dong Choon; Kim, MyungGu; Chung, Ji Hyun; Kim, Sang Hoon; Yeo, Seung Geun

    2014-01-01

    Background Although hearing loss may be caused by various factors, it is also a natural phenomenon associated with the aging process. This study was designed to assess the contributions of diabetes mellitus (DM) and hypertension, both chronic diseases associated with aging, as well as aging itself, to hearing loss in health screening examinees. Methods This study included 37,773 individuals who underwent health screening examinations from 2009 to 2012. The relationships between hearing threshold and subject age, hearing threshold at each frequency based on age group, the degree of hearing loss and the presence or absence of hypertension and DM were evaluated. Results The prevalence of hearing loss increased with age, being 1.6%, 1.8%, 4.6%, 14.0%, 30.8%, and 49.2% in subjects in their twenties, thirties, forties, fifties, sixties, and seventies, respectively (p<0.05). Hearing value per frequency showed aging-based changes, in the order of 6000, 4000, 2000, 1000 and 500 Hz, indicating greater hearing losses at high frequencies. The degree of hearing loss ranged from mild to severe. Aging and DM were correlated with the prevalence of hearing loss (p<0.05). There was no statistically significant association between hearing loss and hypertension after adjusting for age and DM. Conclusions The prevalence of hearing loss increases with age and the presence of DM. Hearing loss was greatest at high frequencies. In all age groups, mild hearing loss was the most common form of hearing loss. PMID:25549095

  13. Effects of hearing aid amplification on voice F0 variability in speakers with prelingual hearing loss.

    PubMed

    Lee, Guo-She; Liu, Chialin; Lee, Shao-Hsuan

    2013-08-01

    To investigate the audio-vocal feedback responses of (F0) to hearing amplification in severe-to-profound prelingual hearing loss (SPHL) using power spectral analysis of F0 contour of sustained vowels. Sustained phonations of vowel/a/of seventeen participants with SPHL were acquired with and without hearing-aid amplifications. The vocal intensity was visually fed back to the participants to help controlling the vocal intensity at 65-75 dBA and 85-95 dBA. The F0 contour of the phonations was extracted and submitted to spectral analysis to measure the extent of F0 fluctuations at different frequency ranges. The results showed that both high vocal intensity and hearing-aid amplification significantly improved voice F0 control by reducing the low-frequency fluctuations (low-frequency power, LFP, 0.2-3 Hz) in F0 spectrum. However, the enhanced feedback from higher vocal intensity and/or hearing amplification was not adequate to reduce the LFP to the level of a normal hearing person. Moreover, we found significant and negative correlations between LFP and supra-threshold feedback intensity (phonation intensity - hearing threshold level) for the frequencies of 500-2000 Hz. Increased vocal intensity, as well as hearing-aid amplification, improved voice F0 control by reducing the LFP of F0 spectrum, and the subtle changes in voices could be well explored using spectral analysis of F0.

  14. Hearing loss in stranded odontocete dolphins and whales.

    PubMed

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

    2010-11-03

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

  15. Cochlear implantation for severe sensorineural hearing loss caused by lightning.

    PubMed

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

    2012-01-01

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

  16. Implications of Sensorineural Hearing Loss With Hydrocodone/Acetaminophen Abuse

    PubMed Central

    Novac, Andrei; Iosif, Anamaria M.; Groysman, Regina; Bota, Robert G.

    2015-01-01

    Sensorineural hearing loss is an infrequently recognized side effect of pain medication abuse. Chronic pain patients treated with opiates develop different degrees of tolerance to pain medications. In many cases, the tolerance becomes the gateway to a variety of cycles of overuse and unmasking of significant psychiatric morbidity and mortality. An individualized approach utilizing combined treatment modalities (including nonopiate pharmaceuticals) is expected to become the norm. Patients can now be provided with multidisciplinary care that addresses an individual’s psychiatric, social, and medical needs, which requires close cooperation between physicians of varying specialties. This report describes a patient who experienced hearing loss from hydrocodone/acetaminophen abuse. PMID:26835162

  17. Hearing Restoration in Mouse Models with Noise-induced Hearing Loss

    DTIC Science & Technology

    2013-12-19

    aim to develop therapeutics which would restore hearing in navy servicemen who are suffering from noise induced hearing loss ( NIHL ). Our central...critical role in mammalian HC regeneration after NIHL (Cox et al., in preparation). Non-mammalian vertebrates such as birds, fish and amphibians can...phenotypes in pi6n a-null mice after NIHL remain further characterized, and we are therefore putting on hold the studies of transient

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

    PubMed Central

    Tambs, Kristian; Engdahl, Bo

    2015-01-01

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

  19. Lipid Profile among Patients with Sudden Sensorineural Hearing Loss.

    PubMed

    Mohammed, Ali A Muttalib

    2014-12-01

    Associations between hearing and blood lipids have been the focus of scientific inquiry for more than 50 years. The aim of the present study is to evaluate the association between hyperlipidaemia among patients presented with sudden sensorineural hearing loss compared to normal controls. A case control study concerned with 22 patients presented with sudden sensorineural hearing loss who underwent lipid profile evaluation. The lipid profile of these patients was compared with corresponding results of 55 age matched persons (volunteers) with normal hearing. These patients were collected from the Out Patient Department of ENT at Al-Jamhory Teaching Hospital, Mosul/Iraq and private clinic of the author for the period from February 2011 to July 2013. The average age of patients was 44.7 years with a range of 26-65 years. The peak age incidence was in the 5(th) decade of life. The study included 11 male patients (50 %) and 11 females (50 %). Meanwhile, the average age of the control group was 41.7 years with 25 (45.5 %) males and 30 (54.5 %) females. Statistical analysis showed that there was significant difference between the means of lipid profile and blood sugar of the patients and the control group apart from HDL where there was no significant difference. In conclusion, hyperlipidemia seems to be significantly associated with the occurrence of sudden sensorineural hearing loss according to this study.

  20. Options for Auditory Training for Adults with Hearing Loss

    PubMed Central

    Olson, Anne D.

    2015-01-01

    Hearing aid devices alone do not adequately compensate for sensory losses despite significant technological advances in digital technology. Overall use rates of amplification among adults with hearing loss remain low, and overall satisfaction and performance in noise can be improved. Although improved technology may partially address some listening problems, auditory training may be another alternative to improve speech recognition in noise and satisfaction with devices. The literature underlying auditory plasticity following placement of sensory devices suggests that additional auditory training may be needed for reorganization of the brain to occur. Furthermore, training may be required to acquire optimal performance from devices. Several auditory training programs that are readily accessible for adults with hearing loss, hearing aids, or cochlear implants are described. Programs that can be accessed via Web-based formats and smartphone technology are reviewed. A summary table is provided for easy access to programs with descriptions of features that allow hearing health care providers to assist clients in selecting the most appropriate auditory training program to fit their needs. PMID:27587915

  1. Prediction model for hearing outcome in patients with idiopathic sudden sensorineural hearing loss.

    PubMed

    Suzuki, Hideaki; Mori, Takanori; Hashida, Koichi; Shibata, Minori; Nguyen, Khac-Hung; Wakasugi, Tetsuro; Hohchi, Nobusuke

    2011-04-01

    The aim of this study is to develop a regression model for predicting hearing outcome in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). A total of 174 consecutive patients with ISSNHL (average of the hearing levels at 250, 500, 1,000, 2,000, and 4,000 Hz was ≥40 dB; time from onset to treatment was ≤30 days) were retrospectively analyzed. They received steroid administration (400 mg/day of hydrocortisone sodium succinate followed by tapered doses) in combination with hyperbaric oxygen therapy. The hearing improvement rate compared to the unaffected contralateral ear was calculated. Correlations between the hearing improvement rate and four prognostic factors (patient's age, days from onset to treatment, initial hearing level, and the presence of vertigo) were examined by simple and multiple regression analyses. In the simple regression analysis, significant correlations were observed between the hearing improvement rate and all four prognostic factors. In the multiple regression analysis, the correlation was significant for patient's age, days from onset to treatment, and the presence of vertigo with partial correlation coefficients of -0.221, -0.324, and -0.329, respectively, but was not significant for the initial hearing level. We subsequently formulated a multiple regression equation for predicting the hearing improvement rate. The multiple correlation coefficient was 0.495 with a p value of 1.42 × 10(-9). Using this regression model, the hearing improvement rate is still difficult to predict with 95% probability, but is predictable with 70% probability.

  2. Pediatric vestibular evaluation: two children with sensorineural hearing loss.

    PubMed

    Valente, L Maureen; Goebel, Joel A; Sinks, Belinda

    2012-04-01

    These two cases illustrate several important areas of vestibular evaluation with children. The two case reports represent two children who display very different vestibular findings despite having significant sensorineural hearing loss. These case reports highlight that pediatric findings can differ significantly from adult findings, stressing the importance of comparing pediatric results with pediatric normative data. These two cases also highlight that vestibular techniques may successfully be adapted for use with hearing-impaired children. That is, rotary chair, computerized dynamic posturography, and vestibular evoked myogenic potentials can be adapted to use with children, including those who demonstrate significant sensorineural hearing loss. Although there is a paucity of research and clinical work in this area, some investigators (Eviatar and Eviatar, 1977; Buchman et al, 2004; Jacot et al, 2009) have reported very rapid recovery from pediatric vestibular deficits. However, it is important for audiologists to be aware that techniques may successfully be adapted for children and that many children should undergo thorough vestibular evaluation.

  3. Outcomes of Children with Hearing Loss: Data Collection and Methods

    PubMed Central

    Tomblin, J. Bruce; Walker, Elizabeth A.; McCreery, Ryan W.; Arenas, Richard M.; Harrison, Melody; Moeller, Mary Pat

    2015-01-01

    Objectives The primary objective of this article was to describe recruitment, data collection, and methods for a longitudinal, multicenter study involving children with bilateral mild-severe hearing loss. The goals of this research program were to characterize the developmental outcomes of children with mild to severe bilateral hearing loss during infancy and the preschool years. Furthermore, the researchers examined how these outcomes were associated with the child’s hearing loss and how home background and clinical interventions mediated and moderated these outcomes. Design The participants in this study were children who are hard of hearing (CHH) and children with normal hearing (CNH) who provided comparison data. CHH were eligible for participation if (1) their chronological age was between 6 months and 7 years of age at the time of recruitment, (2) they had a better-ear pure tone average of 25 dB HL through 75 dB HL, (3) they had not received a cochlear implant, (4) they were from homes where English was the primary language, and (5) they did not demonstrate significant cognitive or motor delays. Across the time span of recruitment, 430 parents of potential children with hearing loss made contact with the research group. This resulted in 317 CHH who qualified at enrollment. In addition, 117 CNH qualified for enrollment. An accelerated longitudinal design was used, in which multiple age cohorts were followed long enough to provide overlap. Specifically, children were recruited and enrolled continuously across an age span of 6.5 years and were followed for at least 3 years. This design allowed for tests of time (period) versus cohort age effects that could arise by changes in services and technology over time, yet still allowed for examination of important developmental relationships. Results The distribution of degree of hearing loss for the CHH showed that the majority of CHH had moderate or moderate to severe hearing losses, indicating that the sample

  4. Effects of substance P during the recovery of hearing function after noise-induced hearing loss.

    PubMed

    Kanagawa, Eiju; Sugahara, Kazuma; Hirose, Yoshinobu; Mikuriya, Takefumi; Shimogori, Hiroaki; Yamashita, Hiroshi

    2014-09-25

    Substance P (SP) is a widely distributed neurotransmitter in living tissues and is involved in various repair processes. We investigated the possibility that SP may ameliorate cochlear hair cell damage produced by noise exposure. The present study examined the effect of SP in protecting the cochlea from noise damage in guinea pigs exposed to noise after an infusion of SP into the inner ear. Changes in the hearing threshold (auditory brain response, ABR), number of synaptic ribbons, and the appearance of the outer hair cells after noise exposure were analyzed at 2 severity levels of noise-induced hearing loss. The moderate noise-induced hearing loss (110dB, 3h) group showed recovery in the ABR threshold over time, finally reaching a level slightly above pre-exposure levels, with only slight injury to the synaptic ribbons and minimal changes in the appearance of the outer hair cells. Our results indicated that in moderate hearing loss, SP exhibited a protective effect on the inner ear, both functionally and structurally. While the final magnitude of ABR threshold elevation was greater in severe noise-induced hearing loss, the synaptic ribbons and outer hair cells showed signs of severe damage.

  5. Masking Release in Children and Adults with Hearing Loss When Using Amplification

    ERIC Educational Resources Information Center

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

    2016-01-01

    Purpose: This study compared masking release for adults and children with normal hearing and hearing loss. For the participants with hearing loss, masking release using simulated hearing aid amplification with 2 different compression speeds (slow, fast) was compared. Method: Sentence recognition in unmodulated noise was compared with recognition…

  6. Identification of Hearing Loss after Age 18 Months Is Not Early Enough.

    ERIC Educational Resources Information Center

    Yoshinaga-Itano, Christine; Apuzzo, Mah-rya L.

    1998-01-01

    This study compared the development (at 40 months) of 40 deaf and hard-of-hearing infants whose hearing loss was identified either before 6 months or after 18 months. Infants whose hearing loss was identified earlier scored significantly on tests of expressive language and comprehension/concepts. Results support early hearing screening for…

  7. Incorporating Mythic and Interpretive Analysis in the Investigation of Hearing Loss on the Family Farm

    ERIC Educational Resources Information Center

    Meister, Mark; Hest, Theresa; Burnett, Ann

    2010-01-01

    Despite knowing about the dangers of hearing loss, farmers typically choose not to protect their hearing. Examining the myth of farm life, this study aims to discern whether rhetorical myths influence farmers' decisions to wear hearing protection. Researchers conducted 40 interviews with farmers regarding farm life and hearing loss. Results…

  8. Early Detection of Hearing Loss: The Case for Listening to Mothers

    ERIC Educational Resources Information Center

    Marchbank, Alison Margaret

    2011-01-01

    This article is drawn from a larger doctoral study that explored hearing mothers' experiences of discovering that their babies had a permanent hearing loss in Australia in 2008. The particular focus for this paper is the period in time after a concern is flagged, either by a newborn hearing screener or the mother herself, until a hearing loss is…

  9. 76 FR 62093 - Preventing Occupational Hearing Loss: Stakeholder Meeting

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-10-06

    ....osha.gov/pls/oshaweb/owadisp.show_document?p_table=NEWS_RELEASES&p_id=19119 ). OSHA decided to suspend... occupational hearing loss. DATES: The date for the stakeholder meeting is November 03, 2011, from 9 a.m. to 1 p... station is Judiciary Square (Red Line). Photo ID is required to enter the building. Registration to...

  10. Auditory Deprivation and Early Conductive Hearing Loss from Otitis Media.

    ERIC Educational Resources Information Center

    Gunnarson, Adele D.; And Others

    1990-01-01

    This article reviews auditory deprivation effects on anatomy, physiology, and behavior in animals and discusses the sequelae of otitis media with effusion (OME) in children. Focused on are central auditory processing disorders associated with early fluctuating hearing loss from OME. (DB)

  11. Wordlikeness and Word Learning in Children with Hearing Loss

    ERIC Educational Resources Information Center

    Stiles, Derek J.; McGregor, Karla K.; Bentler, Ruth A.

    2013-01-01

    Background: The more a novel word conforms to the phonotactics of the language, the more wordlike it is and the easier it is to learn. It is unknown to what extent children with hearing loss (CHL) take advantage of phonotactic cues to support word learning. Aims: This study investigated whether CHL had similar sensitivities to wordlikeness during…

  12. Sudden Sensorineural Hearing Loss: The Question of Perilymph Fistula.

    ERIC Educational Resources Information Center

    Backous, Douglas D.; Niparko, John K.

    1997-01-01

    Perilymph fistula (PLF) is an abnormal communication between the fluid-containing spaces of the inner ear and the air-containing spaces of the temporal bone that can cause hearing loss, tinnitus, aural fullness, vertigo, and postural instability. Diagnosis of PLF and management of those with presumed PLF are discussed. (Contains extensive…

  13. The Challenge of Assessing Language in Children with Hearing Loss.

    ERIC Educational Resources Information Center

    Yoshinaga-Itano, Christine

    1997-01-01

    Challenges current practices in language evaluation for school-aged children with significant hearing loss. It argues for an interactive approach to assessment and offers specific recommendations for the interpretation of test results. Application of current connectionist and competition theories of language acquisition suggests the concept of…

  14. Is There a Silent Hearing Loss among Children in Jordan?

    ERIC Educational Resources Information Center

    Alaqrabawi, Wala' S.; Alshawabka, Amneh Z.; Al-Addasi, Zainab M.

    2016-01-01

    This study measured the prevalence of hearing loss among school children in Jordan. A random sample of 1649 children (990 males and 659 females) was collected from randomly chosen 40 schools in Amman. Screening was conducted between November 2010 and October 2014. Otoscopic examination, tympanometry, and audiometry were used for screening. Based…

  15. Management of Young Children with Unilateral Hearing Loss

    ERIC Educational Resources Information Center

    McKay, Sarah

    2006-01-01

    Children with unilateral hearing loss (UHL) are at risk for academic, speech and language and social-emotional difficulties. To date, most of the evidence documented in the literature has been obtained from school-age children, most of whom were diagnosed with UHL after enrollment in school. Following the widespread institution of universal…

  16. Sensory Temporal Processing in Adults with Early Hearing Loss

    ERIC Educational Resources Information Center

    Heming, Joanne E.; Brown, Lenora N.

    2005-01-01

    This study examined tactile and visual temporal processing in adults with early loss of hearing. The tactile task consisted of punctate stimulations that were delivered to one or both hands by a mechanical tactile stimulator. Pairs of light emitting diodes were presented on a display for visual stimulation. Responses consisted of YES or NO…

  17. Considerations in Diagnosing Usher's Syndrome: RP and Hearing Loss.

    ERIC Educational Resources Information Center

    Vernon, McCay

    1982-01-01

    The association of hearing loss and retinitis pigmentosa has been generally recognized as the genetic disorder of Usher's syndrome. The article reviews findings of this syndrome and suggests strategies for dealing with the clinical and psychological problems displayed by Usher's syndrome patients. (Author/SW)

  18. Facilitating Emergent Literacy Skills in Children with Hearing Loss

    ERIC Educational Resources Information Center

    Zupan, Barbra; Dempsey, Lynn

    2013-01-01

    Purpose: To (a) familiarize readers with the components of emergent literacy and the impact hearing loss may have on the development of these skills; (b) demonstrate the importance of parent-professional collaboration and show how specific literacy-based activities can be integrated into existing daily routines and intervention programming; and…

  19. Increasing Communication in Children with Concurrent Vision and Hearing Loss

    ERIC Educational Resources Information Center

    Brady, Nancy C.; Bashinski, Susan M.

    2008-01-01

    Nine children with complex communication needs and concurrent vision and hearing losses participated in an intervention program aimed at increasing intentional prelinguistic communication. The intervention constituted a pilot, descriptive study of an adapted version of prelinguistic milieu teaching, hence referred to as A-PMT. In A-PMT, natural…

  20. Professional Preparation: Developing Language in Children with Hearing Loss

    ERIC Educational Resources Information Center

    Muma, John; Perigoe, Christina

    2010-01-01

    Children with hearing loss are increasingly being identified at an early age and receiving family-centered intervention from infancy. This means that they are more likely to follow typical developmental sequences of learning. Therefore, professionals working with them need to be aware of developments in the scholarly literature that drive…

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

    PubMed

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

    2016-10-18

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

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

    PubMed Central

    Charles, Lauren; Street, Nicole Drakopoulos

    2015-01-01

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

  3. Association of TMTC2 With Human Nonsyndromic Sensorineural Hearing Loss

    PubMed Central

    Runge, Christina L.; Indap, Amit; Zhou, Yifan; Kent, Jack W.; King, Ericka; Erbe, Christy B.; Cole, Regina; Littrell, Jack; Merath, Kate; James, Roland; Rüschendorf, Franz; Kerschner, Joseph E.; Marth, Gabor; Hübner, Norbert; Göring, Harald H. H.; Friedland, David R.; Kwok, Wai-Meng; Olivier, Michael

    2016-01-01

    IMPORTANCE Sensorineural hearing loss (SNHL) is commonly caused by conditions that affect cochlear structures or the auditory nerve, and the genes identified as causing SNHL to date only explain a fraction of the overall genetic risk for this debilitating disorder. It is likely that other genes and mutations also cause SNHL. OBJECTIVE To identify a candidate gene that causes bilateral, symmetric, progressive SNHL in a large multigeneration family of Northern European descent. DESIGN, SETTING, AND PARTICIPANTS In this prospective genotype and phenotype study performed from January 1, 2006, through April 1, 2016, a 6-generation family of Northern European descent with 19 individuals having reported early-onset hearing loss suggestive of an autosomal dominant inheritance were studied at a tertiary academic medical center. In addition, 179 unrelated adult individuals with SNHL and 186 adult individuals reporting nondeafness were examined. MAIN OUTCOMES AND MEASURES Sensorineural hearing loss. RESULTS Nine family members (5 women [55.6%]) provided clinical audiometric and medical records that documented hearing loss. The hearing loss is characterized as bilateral, symmetric, progressive SNHL that reached severe to profound loss in childhood. Audiometric configurations demonstrated a characteristic dip at 1000 to 2000 Hz. All affected family members wear hearing aids or have undergone cochlear implantation. Exome sequencing and linkage and association analyses identified a fully penetrant sequence variant (rs35725509) on chromosome 12q21 (logarithm of odds, 3.3) in the TMTC2 gene region that segregates with SNHL in this family. This gene explains the SNHL occurrence in this family. The variant is also associated with SNHL in a cohort of 363 unrelated individuals (179 patients with confirmed SNHL and 184 controls, P = 7 × 10−4). CONCLUSIONS AND RELEVANCE A previously uncharacterized gene, TMTC2, has been identified as a candidate for causing progressive SNHL in humans

  4. Bilateral sudden sensorineural hearing loss as an initial presentation of myelodysplastic syndrome.

    PubMed

    Lee, Eun Jung; Yoon, Yong Joo

    2012-01-01

    This study reports an unusual case in which myelodysplastic syndrome presented bilateral sudden sensorineural hearing loss as the first symptom of the disease. The aural symptoms and signs such as tinnitus, dizziness, and hearing impairment of a hematologic disease are common. However, sudden hearing loss as the first manifestation of a hematologic disease is extremely rare. A 76-year-old woman presented with bilateral sudden hearing loss. The patient was found to have myelodysplastic syndrome during a workup for her hearing loss. Unfortunately, the patient's hearing loss did not improve after the medical treatment.

  5. ‘Ecstasy’ Enhances Noise-Induced Hearing Loss

    PubMed Central

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

    2013-01-01

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

  6. Classification of Hearing Loss Disorders Using Teoae-Based Descriptors

    NASA Astrophysics Data System (ADS)

    Hatzopoulos, Stavros Dimitris

    Transiently Evoked Otoacoustic Emissions (TEOAE) are signals produced by the cochlea upon stimulation by an acoustic click. Within the context of this dissertation, it was hypothesized that the relationship between the TEOAEs and the functional status of the OHCs provided an opportunity for designing a TEOAE-based clinical procedure that could be used to assess cochlear function. To understand the nature of the TEOAE signals in the time and the frequency domain several different analyses were performed. Using normative Input-Output (IO) curves, short-time FFT analyses and cochlear computer simulations, it was found that for optimization of the hearing loss classification it is necessary to use a complete 20 ms TEOAE segment. It was also determined that various 2-D filtering methods (median and averaging filtering masks, LP-FFT) used to enhance of the TEOAE S/N offered minimal improvement (less than 6 dB per stimulus level). Higher S/N improvements resulted in TEOAE sequences that were over-smoothed. The final classification algorithm was based on a statistical analysis of raw FFT data and when applied to a sample set of clinically obtained TEOAE recordings (from 56 normal and 66 hearing-loss subjects) correctly identified 94.3% of the normal and 90% of the hearing loss subjects, at the 80 dB SPL stimulus level. To enhance the discrimination between the conductive and the sensorineural populations, data from the 68 dB SPL stimulus level were used, which yielded a normal classification of 90.2%, a hearing loss classification of 87.5% and a conductive-sensorineural classification of 87%. Among the hearing-loss populations the best discrimination was obtained in the group of otosclerosis and the worst in the group of acute acoustic trauma.

  7. Use of hearing protection and perceptions of noise exposure and hearing loss among construction workers.

    PubMed

    Lusk, S L; Kerr, M J; Kauffman, S A

    1998-07-01

    The purpose of this study was to describe construction workers' use of hearing protection devices (HPDs) and determine their perceptions of noise exposure and hearing loss. Operating engineers, carpenters, and plumbers/pipe fitters in the Midwest (n = 400) completed a written questionnaire regarding their use of HPDs and their perceptions of noise exposure and hearing loss. Subjects were recruited through their trade union groups. Mean reported use of HPDs and mean perceived noise exposure were compared across trade groups. Bivariate and multivariate analysis techniques were used to assess relationships between use of HPDs and trade category, education, age, years of employment, noise exposure, and hearing loss. Bivariate analyses identified significant differences in mean use of HPDs by age, years of employment, and trade group. Multivariate logistic regression assessing the independent effects of these variables found significant differences only by trade group. Results indicate a need for significant improvement in all three trade groups' use of HPDs, and suggest a need to consider use and exposure levels, demographics, and trade group membership in designing hearing conservation programs.

  8. Congenital inner ear malformations without sensorineural hearing loss in children.

    PubMed

    Ozeki, Michio; Kato, Zenichiro; Sasai, Hideo; Kubota, Kazuo; Funato, Michinori; Orii, Kenji; Kaneko, Hideo; Fukao, Toshiyuki; Kondo, Naomi

    2009-10-01

    Inner ear malformations are frequently found in patients with congenital hearing loss. It has been reported that normal hearing is rare in patients with severe inner ear vestibular malformations. A 9-year-old boy had had complained of recurrent dizziness and disequilibrium for 2 months. Clinical and neuro-otological examinations showed peripheral involvement of the vestibular system, while audiological investigation was normal. High-resolution magnetic resonance imaging, with three-dimensional reconstruction, showed dysplasia of the bilateral lateral semicircular canals (LSCCs). Isolated vestibular malformation might not be as rare as previously thought, and should be examined by imaging of the temporal bone.

  9. Economic Impact of Hearing Loss and Reduction of Noise-Induced Hearing Loss in the United States

    ERIC Educational Resources Information Center

    Neitzel, Richard L.; Swinburn, Tracy K.; Hammer, Monica S.; Eisenberg, Daniel

    2017-01-01

    Purpose: Hearing loss (HL) is pervasive and debilitating, and noise-induced HL is preventable by reducing environmental noise. Lack of economic analyses of HL impacts means that prevention and treatment remain a low priority for public health and environmental investment. Method: This article estimates the costs of HL on productivity by building…

  10. [Multicenter trial for sudden hearing loss therapy - planning and concept].

    PubMed

    Plontke, S K; Girndt, M; Meisner, C; Probst, R; Oerlecke, I; Richter, M; Steighardt, J; Dreier, G; Weber, A; Baumann, I; Plößl, S; Löhler, J; Laszig, R; Werner, J A; Rahne, T

    2016-04-01

    Systemic steroids are widely used worldwide as a standard of care for primary therapy of idiopathic sudden sensorineural hearing loss (ISSHL). The German ISSHL guideline recommends high-dose steroids for primary therapy of ISSHL, without evidence from randomized controlled trials (RCTs). The rationale for the treatment of ISSHL using high dose steroids is only based on retrospective cohort studies.This article describes the planning and initiation of a multicenter, national, randomized, controlled clinical trial entitled Efficacy and safety of high dose glucocorticosteroid treatment for idiopathic sudden sensorineural hearing loss - a three-armed, randomized, triple-blind, multicenter trial (HODOKORT). This clinical trial aims to compare standard dose with two types of high-dose steroids for primary systemic therapy with respect to their efficacy in improving hearing, and thus communication ability, in patients with idiopathic sudden sensorineural hearing loss.This study is funded by the "Clinical Trials with High Patient Relevance" research program in the health research framework of the German Federal Ministry of Education and Research. It is one of two studies by the German Study Center of Clinical Trials of the German Society of Otorhinolaryngology, Head and Neck Surgery (DSZ-HNO). Planning and initiation was done in cooperation with the DSZ-HNO, the Coordination Center of Clinical Trials of the Martin-Luther-University Halle-Wittenberg, and the Study Center of the University Hospital Freiburg.

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

    PubMed

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

    2016-01-25

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

  12. Genetic disorders with both hearing loss and cardiovascular abnormalities.

    PubMed

    Belmont, John W; Craigen, William; Martinez, Hugo; Jefferies, John Lynn

    2011-01-01

    There has been a growing appreciation for conditions that affect hearing and which are accompanied by significant cardiovascular disorders. In this chapter we consider several broad classes of conditions including deafness due to abnormal structural development of the inner ear, those with physiological abnormalities in the inner ear sensory apparatus, and conditions with progressive loss of function of sensory cells or middle ear functions. Because of shared developmental controls, inner ear malformations are often associated with congenital heart defects and can be part of complex syndromes that affect other organs and neurodevelopmental outcome. Physiological disorders of the hair cells can lead to hearing loss and can be associated with cardiac arrhythmias, especially long QT syndrome. In addition, cellular energy defects such as mitochondrial disorders can affect maintenance of hair cells and are often associated with cardiomyopathy. Lysosomal storage diseases and other disorders affecting connective tissue can lead to chronic middle ear disease, with conductive hearing loss and also cause cardiac valve disease and/or cardiomyopathy. The genetic basis for these conditions is heterogeneous and includes chromosomal/genomic disorders, de novo dominant mutations, and familial dominant, autosomal-recessive, and mitochondrial (matrilineal) inheritance. Taken together, there are more than 100 individual genes implicated in genetic hearing impairment that are also associated with congenital and/or progressive cardiac abnormalities. These genes encode transcription factors, chromatin remodeling factors, components of signal transduction pathways, ion channels, mitochondrial proteins and assembly factors, extracellular matrix proteins, and enzymes involved in lysosomal functions.

  13. A founder TMIE mutation is a frequent cause of hearing loss in southeastern Anatolia.

    PubMed

    Sirmaci, A; Oztürkmen-Akay, H; Erbek, S; Incesulu, A; Duman, D; Taşir-Yilmaz, S; Ozdağ, H; Tekin, M

    2009-06-01

    Using Affymetrix 10K arrays, we searched for regions of homozygosity in 51 Turkish families including at least three members with either congenital or prelingual autosomal recessive non-syndromic sensorineural hearing loss (ARNSSNHL), and identified four families whose deafness mapped to the DFNB6 locus on 3p21 containing the TMIE gene. Mutation analysis revealed the p.R84W mutation in all four families. Screening of this mutation in 254 families with ARNSSNHL, without GJB2 mutations, revealed four additional affected families. A novel mutation was found in a non-complementary marriage between a deaf couple who were homozygous for p.R84W and p.W57X, respectively with two affected children who were compound heterozygotes. Six of the TMIE families originated from southeastern Anatolia, making p.R84W a common cause of hearing loss in that region with a relative frequency of 10.3% (95% CI is 2.5-18.1%). The overall prevalence of the p.R84W mutation in ARNSSNHL in Turkey is 2.4% (95% CI is 0.7-4.0%). Genotyping of single-nucleotide polymorphisms flanking the TMIE gene revealed a conserved haplotype, suggesting a single origin for p.R84W from a common ancestor 1250 years ago (95% CI is 650-2500 years). We conclude that p.R84W could be a common mutation in other Middle Eastern populations and should be included in mutation screening offered to individuals with ARNSSNHL.

  14. Noise-induced hearing loss in children: A ‘less than silent’ environmental danger

    PubMed Central

    Harrison, Robert V

    2008-01-01

    A review of the problems of noise-induced hearing loss in children, especially related to recreational music and the use of personal entertainment devices. The pathophysiology of noise-induced hearing loss and its associated problems (eg, tinnitus) are discussed. The evidence for an increase in noise-induced hearing loss in children and young people is reviewed. Some practical advice (for clinicians, caregivers and children) on hearing loss prevention is provided. PMID:19412364

  15. The influence of hearing aids on the speech and language development of children with hearing loss.

    PubMed

    Tomblin, J Bruce; Oleson, Jacob J; Ambrose, Sophie E; Walker, Elizabeth; Moeller, Mary Pat

    2014-05-01

    IMPORTANCE Hearing loss (HL) in children can be deleterious to their speech and language development. The standard of practice has been early provision of hearing aids (HAs) to moderate these effects; however, there have been few empirical studies evaluating the effectiveness of this practice on speech and language development among children with mild-to-severe HL. OBJECTIVE To investigate the contributions of aided hearing and duration of HA use to speech and language outcomes in children with mild-to-severe HL. DESIGN, SETTING, AND PARTICIPANTS An observational cross-sectional design was used to examine the association of aided hearing levels and length of HA use with levels of speech and language outcomes. One hundred eighty 3- and 5-year-old children with HL were recruited through records of Universal Newborn Hearing Screening and referrals from clinical service providers in the general community in 6 US states. INTERVENTIONS All but 4 children had been fitted with HAs, and measures of aided hearing and the duration of HA use were obtained. MAIN OUTCOMES AND MEASURES Standardized measures of speech and language ability were obtained. RESULTS Measures of the gain in hearing ability for speech provided by the HA were significantly correlated with levels of speech (ρ179 = 0.20; P = .008) and language: ρ155 = 0.21; P = .01) ability. These correlations were indicative of modest levels of association between aided hearing and speech and language outcomes. These benefits were found for children with mild and moderate-to-severe HL. In addition, the amount of benefit from aided hearing interacted with the duration of HA experience (Speech: F4,161 = 4.98; P < .001; Language: F4,138 = 2.91; P < .02). Longer duration of HA experience was most beneficial for children who had the best aided hearing. CONCLUSIONS AND RELEVANCE The degree of improved hearing provided by HAs was associated with better speech and language development in children

  16. Characteristics of hearing loss in patients with herpes zoster oticus

    PubMed Central

    Kim, Chang-Hee; Choi, Hyerang; Shin, Jung Eun

    2016-01-01

    Abstract Patients with herpes zoster oticus (HZO) may commonly show symptoms associated with 7th and 8th cranial nerve (CN VII and CN VIII) dysfunction. The aim of this study is to investigate the characteristics of hearing loss in patients with HZO and discuss possible mechanisms. Ninety-five HZO patients who showed at least one of the symptoms of CN VII and CN VIII dysfunction between January 2007 and October 2014 were included in this study. Hearing loss was defined when the mean thresholds of pure tone audiometry (PTA) in speech frequency (0.5 kHz + 1 kHz + 2 kHz/3) or isolated high frequency (4 kHz + 8 kHz/2) were greater than 10 dB in the affected ear compared with the healthy ear, and a total of 72 patients were classified as the hearing loss group. The difference of mean PTA thresholds between affected and healthy ears was significantly greater in the high frequency range than in low range (20.0 ± 11.5 dB vs. 12.9 ± 15.7 dB, P = 0.0026) in patients with hearing loss (n = 72). The difference between affected and healthy ear was significantly greater in patients with vertigo (n = 34) than those without vertigo (n = 38) in both the high (P = 0.033) and low (P = 0.024) frequency ranges. In contrast, the differences between affected and healthy ears were not significantly different between patients with facial palsy (n = 50) and those without facial palsy (n = 22) in both the high (P = 0.921) and low (P = 0.382) frequency ranges. In patients with HZO, hearing loss is more severe in the high frequency range than in the low frequency range. Hearing impairment is more severe in patients with vertigo than in those without vertigo in both the high and low frequency ranges, even though the degree of hearing impairment is not significantly different between patients with and without facial palsy. These findings indicate that the mechanisms of viral spread from CN VII to CN VIII may differ

  17. Consequences of congenital hearing loss - a longterm view.

    PubMed

    Rapin, I

    1978-12-01

    Responsibility for detection of hearing loss at the earliest possible age rests on the shoulders of the medical profession. Early detection and presentation of language through all available sensory channels, most notably the visual, are essential since deaf children of hearing parents, the vast majority of the deaf, characteristically remain grossly deficient linguistically despite years of special schooling. The deaf children of deaf parents, who are not as deprived linguistically since they learn a manual language at the normal age of language acquisition, tend to be less severely handicapped than the deaf children of hearing parents. Deafness is a hidden handicap in infancy although lack of vestibular function may delay motor milestones like sitting and walking but does not constitute a serious problem after they are achieved. The average 18 year old deaf student achieves a reading level comparable to that of a hearing fourth grader and the mathematical skills of a sixth grader. He is also deficient in science, social studies, and general information despite his average scores on nonverbal intelligence tests. Although many deaf students drop out of school before graduation and few go on to higher education, congenitally deaf adults are usually self-supporting and independent. Eighty per cent of the deaf marry hearing impaired persons. The deaf are greatly assisted by the cohesive deaf community existing in every sizable city which provides a social, recreational, and economic framework to their lives. As a result, delinquency and psychiatric illness are not higher among the deaf than the hearing, despite the difficulties they have dealing with the complexities of the hearing society.

  18. 29 CFR 1904.10 - Recording criteria for cases involving occupational hearing loss.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... loss. (a) Basic requirement. If an employee's hearing test (audiogram) reveals that the employee has... reflecting the employee's previous recordable hearing loss case). (ii) 25-dB loss. Audiometric test results... audiogram to reflect the effects of aging on hearing? Yes. When you are determining whether an STS...

  19. 29 CFR 1904.10 - Recording criteria for cases involving occupational hearing loss.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... loss. (a) Basic requirement. If an employee's hearing test (audiogram) reveals that the employee has... reflecting the employee's previous recordable hearing loss case). (ii) 25-dB loss. Audiometric test results... audiogram to reflect the effects of aging on hearing? Yes. When you are determining whether an STS...

  20. 29 CFR 1904.10 - Recording criteria for cases involving occupational hearing loss.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... loss. (a) Basic requirement. If an employee's hearing test (audiogram) reveals that the employee has... reflecting the employee's previous recordable hearing loss case). (ii) 25-dB loss. Audiometric test results... audiogram to reflect the effects of aging on hearing? Yes. When you are determining whether an STS...

  1. 29 CFR 1904.10 - Recording criteria for cases involving occupational hearing loss.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... loss. (a) Basic requirement. If an employee's hearing test (audiogram) reveals that the employee has... reflecting the employee's previous recordable hearing loss case). (ii) 25-dB loss. Audiometric test results... audiogram to reflect the effects of aging on hearing? Yes. When you are determining whether an STS...

  2. 29 CFR 1904.10 - Recording criteria for cases involving occupational hearing loss.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... loss. (a) Basic requirement. If an employee's hearing test (audiogram) reveals that the employee has... reflecting the employee's previous recordable hearing loss case). (ii) 25-dB loss. Audiometric test results... audiogram to reflect the effects of aging on hearing? Yes. When you are determining whether an STS...

  3. Phonological Awareness and Vocabulary Performance of Monolingual and Bilingual Preschool Children with Hearing Loss

    ERIC Educational Resources Information Center

    Lund, Emily; Werfel, Krystal L.; Schuele, C. Melanie

    2015-01-01

    This pilot study compared the phonological awareness skills and vocabulary performance of English monolingual and Spanish-English bilingual children with and without hearing loss. Preschool children with varying degrees of hearing loss (n = 18) and preschool children without hearing loss (n = 19) completed measures of phonological awareness and…

  4. Analogous and Distinctive Patterns of Prelinguistic Communication in Toddlers with and without Hearing Loss

    ERIC Educational Resources Information Center

    Zaidman-Zait, Anat; Dromi, Esther

    2007-01-01

    Purpose: This study was conducted to compare the prelinguistic communicative abilities of toddlers with hearing loss and without hearing loss during the 2nd year of life and shortly before the emergence of productive single-word lexicons. Method: The participants were 28 toddlers with hearing loss who participated in an early intervention program…

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

    PubMed

    Böhme, G

    1989-07-01

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

  6. Developmental plasticity of spatial hearing following asymmetric hearing loss: context-dependent cue integration and its clinical implications.

    PubMed

    Keating, Peter; King, Andrew J

    2013-12-27

    Under normal hearing conditions, comparisons of the sounds reaching each ear are critical for accurate sound localization. Asymmetric hearing loss should therefore degrade spatial hearing and has become an important experimental tool for probing the plasticity of the auditory system, both during development and adulthood. In clinical populations, hearing loss affecting one ear more than the other is commonly associated with otitis media with effusion, a disorder experienced by approximately 80% of children before the age of two. Asymmetric hearing may also arise in other clinical situations, such as after unilateral cochlear implantation. Here, we consider the role played by spatial cue integration in sound localization under normal acoustical conditions. We then review evidence for adaptive changes in spatial hearing following a developmental hearing loss in one ear, and show that adaptation may be achieved either by learning a new relationship between the altered cues and directions in space or by changing the way different cues are integrated in the brain. We next consider developmental plasticity as a source of vulnerability, describing maladaptive effects of asymmetric hearing loss that persist even when normal hearing is provided. We also examine the extent to which the consequences of asymmetric hearing loss depend upon its timing and duration. Although much of the experimental literature has focused on the effects of a stable unilateral hearing loss, some of the most common hearing impairments experienced by children tend to fluctuate over time. We therefore propose that there is a need to bridge this gap by investigating the effects of recurring hearing loss during development, and outline recent steps in this direction. We conclude by arguing that this work points toward a more nuanced view of developmental plasticity, in which plasticity may be selectively expressed in response to specific sensory contexts, and consider the clinical implications of this.

  7. Effects of potential neurotoxic pesticides on hearing loss: a review.

    PubMed

    Gatto, M P; Fioretti, M; Fabrizi, G; Gherardi, M; Strafella, E; Santarelli, L

    2014-05-01

    Several pesticides are supposed to be neurotoxic for humans, consequently, they may also affect the auditory system. This review analyzes human and experimental animal studies testing the hypothesis that exposure to pesticides is associated with hearing loss. The literature on this topic is still sparse and methodological limitations of some papers evaluated are identified. As a whole, available data indicate a possible ototoxic action of pesticides, but alternative hypotheses could not be ruled out, also considering some confounders, such as the co-exposure to noise. Therefore, further studies are necessary in order to clarify the association between pesticides exposure and hearing loss. While awaiting more evidence, for precautionary action we recommend considering pesticides as possible ototoxic agents, in particular for vulnerable targets, such as pregnant women and children during early development.

  8. The mitochondrion: a perpetrator of acquired hearing loss.

    PubMed

    Böttger, Erik C; Schacht, Jochen

    2013-09-01

    Age, drugs, and noise are major causes of acquired hearing loss. The involvement of reactive oxygen species (ROS) in hair cell death has long been discussed, but there is considerably less information available as to the mechanisms underlying ROS formation. Most cellular ROS arise in mitochondria and this review will evaluate evidence for mitochondrial pathology in general and dysfunction of the mitochondrial respiratory chain in particular in acquired hearing loss. We will discuss evidence that different pathways can lead to the generation of ROS and that oxidative stress might not necessarily be causal to all three pathologies. Finally, we will detail recent advances in exploiting knowledge of aminoglycoside-mitochondria interactions for the development of non-ototoxic antibacterials. This article is part of a Special Issue entitled "Annual Reviews 2013".

  9. Sensorineural hearing loss associated with a factitious disorder.

    PubMed

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

    2015-12-01

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

  10. Role Salience and Anticipated Work-Family Relations among Young Adults with and without Hearing Loss

    ERIC Educational Resources Information Center

    Cinamon, Rachel Gali; Most, Tova; Michael, Rinat

    2008-01-01

    This study examined the effect of hearing status on role salience and anticipated work-family relations among 101 unmarried young adults aged 20-33 years: 35 with hearing loss (19 hard of hearing and 16 deaf) and 66 hearing. Participants completed the Life Role Salience scale, anticipated conflictual relations scale, anticipated facilitory…

  11. Bilateral sudden sensorineural hearing loss following unilateral temporal bone fracture.

    PubMed

    Hunchaisri, Niran

    2009-06-01

    Temporal bone fractures usually cause unilateral sensorineural hearing loss (SNHL) by fracture that violated otic capsule of that side. Bilateral SNHL from unilateral temporal bone fracture were rarely seen. Labyrinthine concussion was considered to be the pathogenesis in these cases. This article reports an additional case of bilateral SNHL from unilateral temporal bone fracture but in a different pattern of SNHL which may result from an occlusion of the internal auditory artery.

  12. Genetic markers of cisplatin-induced hearing loss in children.

    PubMed

    Carleton, B C; Ross, C J; Pussegoda, K; Bhavsar, A P; Visscher, H; Lee, J W; Brooks, B; Rassekh, S R; Dubé, M-P P; Hayden, M R

    2014-09-01

    This journal recently published a Commentary by Ratain and colleagues at the University of Chicago that criticizes our work on cisplatin-induced hearing loss in children. It is unfortunate that neither the authors nor the editors of Clinical Pharmacology & Therapeutics corresponded with us to provide an earlier opportunity to address these questions. Here we correct the authors' inaccuracies and provide additional analyses that further strengthen our published findings.

  13. Genomic advances for gene discovery in hereditary hearing loss.

    PubMed

    Avraham, Karen B; Kanaan, Moien

    2012-09-07

    High-throughput sequencing is changing the face of genetic diagnosis and counseling. While in the past, it would take on average 1 to 5 years to identify a mutation leading to deafness, today, the genetic basis for deafness can be determined within months in a child or adult with inherited hearing loss. Obstacles and challenges still remain, but the field is changing at a dramatic rate, making gene discovery a much easier and more efficient task than in the past.

  14. Behavioral training promotes multiple adaptive processes following acute hearing loss

    PubMed Central

    Keating, Peter; Rosenior-Patten, Onayomi; Dahmen, Johannes C; Bell, Olivia; King, Andrew J

    2016-01-01

    The brain possesses a remarkable capacity to compensate for changes in inputs resulting from a range of sensory impairments. Developmental studies of sound localization have shown that adaptation to asymmetric hearing loss can be achieved either by reinterpreting altered spatial cues or by relying more on those cues that remain intact. Adaptation to monaural deprivation in adulthood is also possible, but appears to lack such flexibility. Here we show, however, that appropriate behavioral training enables monaurally-deprived adult humans to exploit both of these adaptive processes. Moreover, cortical recordings in ferrets reared with asymmetric hearing loss suggest that these forms of plasticity have distinct neural substrates. An ability to adapt to asymmetric hearing loss using multiple adaptive processes is therefore shared by different species and may persist throughout the lifespan. This highlights the fundamental flexibility of neural systems, and may also point toward novel therapeutic strategies for treating sensory disorders. DOI: http://dx.doi.org/10.7554/eLife.12264.001 PMID:27008181

  15. Metabolic disorders in vertigo, tinnitus, and hearing loss.

    PubMed

    Kaźmierczak, H; Doroszewska, G

    2001-01-01

    Vertigo, tinnitus, and hearing loss are common complaints among populations of industrial countries, especially in persons older than 40 years. Numerous agents are known to incite vertigo, tinnitus, and hearing loss, among them hyperinsulinemia, diabetes mellitus, and hyperlipidemia. In this study, we proposed to assess the occurrence of hyperinsulinemia, diabetes mellitus, and hyperlipidemia in patients suffering from vertigo, tinnitus, or hearing loss of unknown origin. Results of various tests in 48 patients were compared to those in 31 control subjects. Assessments of body mass index, blood pressure, and laryngological, audiometric, and electronystagmographic parameters were performed in all study participants. An oral glucose tolerance test was used to evaluate insulin levels, and lipoprotein phenotyping served to determine cholesterol, triglyceride, and lipoprotein levels. Patients were found to be significantly more overweight (on the basis of body mass index) than were the control subjects. Hypertension was more common among patients than controls, but the difference was significant only between the men in the two groups. Disturbances of glucose metabolism were found in 27.1% of patients but in only 9.7% of controls. Diabetes mellitus was not present in any controls but was identified in four patients. Hyperinsulinemia was almost twice as common in patients as in controls. Only the occurrence of hyperlipoproteinemia seemed not to differ between patients and control subjects. We conclude that such disturbances of glucose metabolism as diabetes mellitus and hyperinsulinemia may be responsible for inner ear diseases, whereas the role of disturbances of lipid metabolism remains vague.

  16. Toward a Differential Diagnosis of Hidden Hearing Loss in Humans

    PubMed Central

    Liberman, M. Charles; Epstein, Michael J.; Cleveland, Sandra S.; Wang, Haobing

    2016-01-01

    Recent work suggests that hair cells are not the most vulnerable elements in the inner ear; rather, it is the synapses between hair cells and cochlear nerve terminals that degenerate first in the aging or noise-exposed ear. This primary neural degeneration does not affect hearing thresholds, but likely contributes to problems understanding speech in difficult listening environments, and may be important in the generation of tinnitus and/or hyperacusis. To look for signs of cochlear synaptopathy in humans, we recruited college students and divided them into low-risk and high-risk groups based on self-report of noise exposure and use of hearing protection. Cochlear function was assessed by otoacoustic emissions and click-evoked electrocochleography; hearing was assessed by behavioral audiometry and word recognition with or without noise or time compression and reverberation. Both groups had normal thresholds at standard audiometric frequencies, however, the high-risk group showed significant threshold elevation at high frequencies (10–16 kHz), consistent with early stages of noise damage. Electrocochleography showed a significant difference in the ratio between the waveform peaks generated by hair cells (Summating Potential; SP) vs. cochlear neurons (Action Potential; AP), i.e. the SP/AP ratio, consistent with selective neural loss. The high-risk group also showed significantly poorer performance on word recognition in noise or with time compression and reverberation, and reported heightened reactions to sound consistent with hyperacusis. These results suggest that the SP/AP ratio may be useful in the diagnosis of “hidden hearing loss” and that, as suggested by animal models, the noise-induced loss of cochlear nerve synapses leads to deficits in hearing abilities in difficult listening situations, despite the presence of normal thresholds at standard audiometric frequencies. PMID:27618300

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

    PubMed Central

    Barker, Alex B; Xia, Jun

    2016-01-01

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

  18. The Importance of Hearing: A Review of the Literature on Hearing Loss for Older People with Learning Disabilities

    ERIC Educational Resources Information Center

    Bent, Sarah; McShea, Lynzee; Brennan, Siobhan

    2015-01-01

    Background: Hearing loss has a significant impact on living well and on communication in all adults, with the numbers affected increasing with age, and adults with learning disabilities being at particular risk. Methods: A review of the literature on hearing loss in older adults with learning disabilities was completed. Results: A significant…

  19. Acute-onset unilateral psychogenic hearing loss in adults: report of six cases and diagnostic pitfalls.

    PubMed

    Oishi, Naoki; Kanzaki, Sho; Kataoka, Chinatsu; Tazoe, Mami; Takei, Yasuhiko; Nagai, Keiichi; Kohno, Naoyuki; Ogawa, Kaoru

    2009-01-01

    We encountered 6 rare cases of acute-onset unilateral psychogenic hearing loss in adults. All were women in their 20s and 30s. Three cases had severe hearing impairment characterized by hearing loss at every frequency; 2 cases had profound hearing impairment, and 1 case had low-frequency hearing impairment. Of the 6 cases, 3 had a history of hearing loss, and 1 had a history of psychogenic visual disturbance. All 6 cases were initially diagnosed as having idiopathic sudden sensorineural hearing loss; all subsequently received steroid therapy. Three cases were not diagnosed as being psychogenic in origin until otoacoustic emissions and auditory brain responses were performed. Although the presence of distinctive clinical features (age, gender, and past history) is important for suspecting psychogenic hearing loss, objective audiological tests such as otoacoustic emissions are essential for diagnosing some cases. Compared to the existing reports of similar cases, our cases had a poorer prognosis (only 2 cases were cured).

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

    PubMed Central

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

    2016-01-01

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

  1. Effects of Hyperlipidemia on Noise Induced Hearing Loss (NIHL).

    PubMed

    Doosti, Afsaneh; Lotfi, Yones; Bakhshi, Enayatollah

    2016-06-01

    Likelihood of developing noise induced hearing loss (NIHL) is affected by some factors such as genes, nutrition and healthy hearing habits. We investigated this study to determine the effect of hyperlipidemia on NIHL. In a case-control study, 144 workers who exposed to continuous noise more than 85 dBA (88-89 dBA) from a textile factory in Tehran were examined. Serum Blood test results as index for dyslipidemi, were collected and audiometry tests were carried out to analyze the association of hyperlipidemian and NIHL. The mean ±SD of Cholesterol level for NIHL and control were 188.19 (28.46) and 159.00 (26.66) mg/dL respectively. Also the mean ±SD of Triglyceride level for NIHL and control were 203.75 (53.92) and 105.00 (30.50) mg/dL consecutively. After adjusting odds ratio (OR) for NIHL, only hypertriglyceridemia had association with NIHL. (aOR = 1.07; 95 % CI 1.04-1.10), and there wasn't any relation between hypercholestomia and NIHL (aOR = 1.02; 95 % CI 0.99-1.05). Workers with hypertriglyceridemia are at increased risk for NIHL. In factories with elevated noise levels, besides hearing conservation programs, attention to blood serum level of workers and healthy diet are proposed to help in preserving hearing threshold.

  2. Developmental Conductive Hearing Loss Reduces Modulation Masking Release

    PubMed Central

    Chen, Yi-Wen; Sanes, Dan H.

    2016-01-01

    Hearing-impaired individuals experience difficulties in detecting or understanding speech, especially in background sounds within the same frequency range. However, normally hearing (NH) human listeners experience less difficulty detecting a target tone in background noise when the envelope of that noise is temporally gated (modulated) than when that envelope is flat across time (unmodulated). This perceptual benefit is called modulation masking release (MMR). When flanking masker energy is added well outside the frequency band of the target, and comodulated with the original modulated masker, detection thresholds improve further (MMR+). In contrast, if the flanking masker is antimodulated with the original masker, thresholds worsen (MMR−). These interactions across disparate frequency ranges are thought to require central nervous system (CNS) processing. Therefore, we explored the effect of developmental conductive hearing loss (CHL) in gerbils on MMR characteristics, as a test for putative CNS mechanisms. The detection thresholds of NH gerbils were lower in modulated noise, when compared with unmodulated noise. The addition of a comodulated flanker further improved performance, whereas an antimodulated flanker worsened performance. However, for CHL-reared gerbils, all three forms of masking release were reduced when compared with NH animals. These results suggest that developmental CHL impairs both within- and across-frequency processing and provide behavioral evidence that CNS mechanisms are affected by a peripheral hearing impairment. PMID:28215119

  3. Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss

    PubMed Central

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

    2016-01-01

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

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

    PubMed Central

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

    2014-01-01

    The Hybrid cochlear implant (CI), also known as Electro- Acoustic Stimulation (EAS), is a new type of CI that preserves residual acoustic hearing and enables combined cochlear implant and hearing aid use in the same ear. However, 30-55% of patients experience acoustic hearing loss within days to months after activation, suggesting that both surgical trauma and electrical stimulation may cause hearing loss. The goals of this study were to: 1) determine the contributions of both implantation surgery and EAS to hearing loss in a normal-hearing guinea pig model; 2) determine which cochlear structural changes are associated with hearing loss after surgery and EAS. Two groups of animals were implanted (n=6 per group), with one group receiving chronic acoustic and electric stimulation for 10 weeks, and the other group receiving no direct acoustic or electric stimulation during this time frame. A third group (n=6) was not implanted, but received chronic acoustic stimulation. Auditory brainstem response thresholds were followed over time at 1, 2, 6, and 16 kHz. At the end of the study, the following cochlear measures were quantified: hair cells, spiral ganglion neuron density, fibrous tissue density, and stria vascularis blood vessel density; the presence or absence of ossification around the electrode entry was also noted. After surgery, implanted animals experienced a range of 0-55 dB of threshold shifts in the vicinity of the electrode at 6 and 16 kHz. The degree of hearing loss was significantly correlated with reduced stria vascularis vessel density and with the presence of ossification, but not with hair cell counts, spiral ganglion neuron density, or fibrosis area. After 10 weeks of stimulation, 67% of implanted, stimulated animals had more than 10 dB of additional threshold shift at 1 kHz, compared to 17% of implanted, non-stimulated animals and 0% of non-implanted animals. This 1-kHz hearing loss was not associated with changes in any of the cochlear measures

  5. Factors associated with hearing loss in a normal-hearing guinea pig model of Hybrid cochlear implants.

    PubMed

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

    2014-10-01

    The Hybrid cochlear implant (CI), also known as Electro-Acoustic Stimulation (EAS), is a new type of CI that preserves residual acoustic hearing and enables combined cochlear implant and hearing aid use in the same ear. However, 30-55% of patients experience acoustic hearing loss within days to months after activation, suggesting that both surgical trauma and electrical stimulation may cause hearing loss. The goals of this study were to: 1) determine the contributions of both implantation surgery and EAS to hearing loss in a normal-hearing guinea pig model; 2) determine which cochlear structural changes are associated with hearing loss after surgery and EAS. Two groups of animals were implanted (n = 6 per group), with one group receiving chronic acoustic and electric stimulation for 10 weeks, and the other group receiving no direct acoustic or electric stimulation during this time frame. A third group (n = 6) was not implanted, but received chronic acoustic stimulation. Auditory brainstem response thresholds were followed over time at 1, 2, 6, and 16 kHz. At the end of the study, the following cochlear measures were quantified: hair cells, spiral ganglion neuron density, fibrous tissue density, and stria vascularis blood vessel density; the presence or absence of ossification around the electrode entry was also noted. After surgery, implanted animals experienced a range of 0-55 dB of threshold shifts in the vicinity of the electrode at 6 and 16 kHz. The degree of hearing loss was significantly correlated with reduced stria vascularis vessel density and with the presence of ossification, but not with hair cell counts, spiral ganglion neuron density, or fibrosis area. After 10 weeks of stimulation, 67% of implanted, stimulated animals had more than 10 dB of additional threshold shift at 1 kHz, compared to 17% of implanted, non-stimulated animals and 0% of non-implanted animals. This 1-kHz hearing loss was not associated with changes in any of the cochlear measures

  6. Talker Differences in Clear and Conversational Speech: Vowel Intelligibility for Older Adults with Hearing Loss

    ERIC Educational Resources Information Center

    Ferguson, Sarah Hargus

    2012-01-01

    Purpose: To establish the range of talker variability for vowel intelligibility in clear versus conversational speech for older adults with hearing loss and to determine whether talkers who produced a clear speech benefit for young listeners with normal hearing also did so for older adults with hearing loss. Method: Clear and conversational vowels…

  7. The Relationship between Language Development and Behaviour Problems in Children with Hearing Loss

    ERIC Educational Resources Information Center

    Stevenson, Jim; McCann, Donna; Watkin, Peter; Worsfold, Sarah; Kennedy, Colin

    2010-01-01

    Background: There are well-replicated findings that link poor development on a range of communication skills with increased behavioural problems. This paper examines this relationship in children with hearing loss. Method: One hundred and twenty children with hearing loss (67 boys, 53 girls) and 63 hearing children (37 boys, 26 girls) with a mean…

  8. Auditory Temporal-Organization Abilities in School-Age Children with Peripheral Hearing Loss

    ERIC Educational Resources Information Center

    Koravand, Amineh; Jutras, Benoit

    2013-01-01

    Purpose: The objective was to assess auditory sequential organization (ASO) ability in children with and without hearing loss. Method: Forty children 9 to 12 years old participated in the study: 12 with sensory hearing loss (HL), 12 with central auditory processing disorder (CAPD), and 16 with normal hearing. They performed an ASO task in which…

  9. Temporal Intraspeech Masking of Plosive Bursts: Effects of Hearing Loss and Frequency Shaping

    ERIC Educational Resources Information Center

    Mackersie, Carol L.

    2007-01-01

    Purpose: The purposes were (a) to compare masking of consonant bursts by adjacent vowels for listeners with and without hearing loss and (b) to determine the extent to which the temporal intraspeech masking can be reduced by a simulated hearing-aid frequency-response shaping. Method: Fourteen adults with sensorineural hearing loss and 10 with…

  10. Comparisons of Social Competence in Young Children with and without Hearing Loss: A Dynamic Systems Framework

    ERIC Educational Resources Information Center

    Hoffman, Michael F.; Quittner, Alexandra L.; Cejas, Ivette

    2015-01-01

    This study compared levels of social competence and language development in 74 young children with hearing loss and 38 hearing peers aged 2.5-5.3 years. This study was the first to examine the relationship between oral language and social competence using a dynamic systems framework in children with and without hearing loss. We hypothesized that,…

  11. The Impact of Hearing Loss on Quality of Life in Older Adults

    ERIC Educational Resources Information Center

    Dalton, Dayna S.; Cruickshanks, Karen J.; Klein, Barbara E. K.; Klein, Ronald; Wiley, Terry L.; Nondahl, David M.

    2003-01-01

    Purpose: The authors investigate the impact of hearing loss on quality of life in a large population of older adults. Design and Methods: Data are from the 5-year follow-up Epidemiology of Hearing Loss Study, a population-based longitudinal study of age-related hearing impairment conducted in Beaver Dam, WI. Participants (N = 2,688) were 53-97…

  12. Children with Mild Bilateral and Unilateral Hearing Loss: Parents' Reflections on Experiences and Outcomes

    ERIC Educational Resources Information Center

    Fitzpatrick, Elizabeth; Grandpierre, Viviane; Durieux-Smith, Andrée; Gaboury, Isabelle; Coyle, Doug; Na, Eunjung; Sallam, Nusaiba

    2016-01-01

    Children with mild bilateral and unilateral hearing loss are now commonly identified early through newborn hearing screening initiatives. There remains considerable uncertainty about how to support parents and about which services to provide for children with mild bilateral and unilateral hearing loss. The goal of this study was to learn about…

  13. Differential pathologies resulting from sound exposure: Tinnitus vs hearing loss

    NASA Astrophysics Data System (ADS)

    Longenecker, Ryan James

    The first step in identifying the mechanism(s) responsible for tinnitus development would be to discover a neural correlate that is differentially expressed in tinnitus-positive compared to tinnitus negative animals. Previous research has identified several neural correlates of tinnitus in animals that have tested positive for tinnitus. However it is unknown whether all or some of these correlates are linked to tinnitus or if they are a byproduct of hearing loss, a common outcome of tinnitus induction. Abnormally high spontaneous activity has frequently been linked to tinnitus. However, while some studies demonstrate that hyperactivity positively correlates with behavioral evidence of tinnitus, others show that when all animals develop hyperactivity to sound exposure, not all exposed animals show evidence of tinnitus. My working hypothesis is that certain aspects of hyperactivity are linked to tinnitus while other aspects are linked to hearing loss. The first specific aim utilized the gap induced prepulse inhibition of the acoustic startle reflex (GIPAS) to monitor the development of tinnitus in CBA/CaJ mice during one year following sound exposure. Immediately after sound exposure, GIPAS testing revealed widespread gap detection deficits across all frequencies, which was likely due to temporary threshold shifts. However, three months after sound exposure these deficits were limited to a narrow frequency band and were consistently detected up to one year after exposure. This suggests the development of chronic tinnitus is a long lasting and highly dynamic process. The second specific aim assessed hearing loss in sound exposed mice using several techniques. Acoustic brainstem responses recorded initially after sound exposure reveal large magnitude deficits in all exposed mice. However, at the three month period, thresholds return to control levels in all mice suggesting that ABRs are not a reliable tool for assessing permanent hearing loss. Input/output functions of

  14. Delayed loss of hearing after hearing preservation cochlear implantation: Human temporal bone pathology and implications for etiology.

    PubMed

    Quesnel, Alicia M; Nakajima, Hideko Heidi; Rosowski, John J; Hansen, Marlan R; Gantz, Bruce J; Nadol, Joseph B

    2016-03-01

    After initially successful preservation of residual hearing with cochlear implantation, some patients experience subsequent delayed hearing loss. The etiology of such delayed hearing loss is unknown. Human temporal bone pathology is critically important in investigating the etiology, and directing future efforts to maximize long term hearing preservation in cochlear implant patients. Here we present the temporal bone pathology from a patient implanted during life with an Iowa/Nucleus Hybrid S8 implant, with initially preserved residual hearing and subsequent hearing loss. Both temporal bones were removed for histologic processing and evaluated. Complete clinical and audiologic records were available. He had bilateral symmetric high frequency severe to profound hearing loss prior to implantation. Since he was implanted unilaterally, the unimplanted ear was presumed to be representative of the pre-implantation pathology related to his hearing loss. The implanted and contralateral unimplanted temporal bones both showed complete degeneration of inner hair cells and outer hair cells in the basal half of the cochleae, and only mild patchy loss of inner hair cells and outer hair cells in the apical half. The total spiral ganglion neuron counts were similar in both ears: 15,138 (56% of normal for age) in the unimplanted right ear and 13,722 (51% of normal for age) in the implanted left ear. In the basal turn of the implanted left cochlea, loose fibrous tissue and new bone formation filled the scala tympani, and part of the scala vestibuli. Delayed loss of initially preserved hearing after cochlear implantation was not explained by additional post-implantation degeneration of hair cells or spiral ganglion neurons in this patient. Decreased compliance at the round window and increased damping in the scala tympani due to intracochlear fibrosis and new bone formation might explain part of the post-implantation hearing loss. Reduction of the inflammatory and immune response to

  15. Development of conductive hearing loss due to posterior semicircular canal dehiscence.

    PubMed

    Kubota, Marie; Kubo, Kazuhiko; Yasui, Tetsuro; Matsumoto, Nozomu; Komune, Shizuo

    2015-06-01

    We herein report a case of posterior semicircular canal dehiscence (SCD) syndrome who had been audiologically followed up for eight years. The patient originally had sensorineural hearing loss. The audiogram had gradually transformed to pure conductive hearing loss. The posterior SCD was identified in CT scan. The reported case showed the possibility to distinguish the mechanism at play underlying the typical conductive hearing loss in SCD patients by tracing the transition of the hearing loss pattern. This information is of much help to predict the hearing outcomes if surgical intervention were chosen for the treatment.

  16. [FEDERAL CLINICAL RECOMMENDATIONS IN DIAGNOSIS, TREATMENT AND PREVENTION OF HEARING LOSS DUE TO NOISE].

    PubMed

    Adeninskaya, E E; Bukhtiarov, I V; Bushmanov, A Iu; Dayhes, N A; Denisov, E I; Izmerov, N F; Mazitova, N N; Pankova, V B; Preobrazhenskaya, E A; Prokopenko, L V; Simonova, N I; Tavartkiladze, G A; Fedina, I N

    2016-01-01

    Noise induced hearing loss is a slowly developing hearing impairment, caused by occupational exposure to excessive noise levels, constitutes a lesion of the auditory analyzer and clinically manifested as chronic bilateral sensorineural hearing loss. Currently, there is not a treatment that provide a cure of sensorineural hearing loss. Regular, individually tailored treatment should be directed to the pathogenic mechanisms and specific clinical symptoms of hearing loss, as well as the prevention of complications. We recommend using non-drug therapies that can improve blood flow in labyrinth, tissue and cellular metabolism.

  17. Current aspects of hearing loss from occupational and leisure noise

    PubMed Central

    Plontke, S.; Zenner, H.-P.

    2004-01-01

    Hearing loss from occupational and leisure noise numbers amongst the most frequent causes of an acquired sensorineural hearing loss. Here we present a review of up-to-date findings on the pathophysiology of acoustic injury to the inner ear, with special attention being paid to its molecular-biological and genetic aspects. Epidemiological aspects shall also be dealt with, as shall the roles of lacking recovery from occupational noise due to additional exposure by leisure noise and the combined exposure of noise and chemicals. Based on the epidemiological and pathophysiological findings and against the background of published animal-experimental, pre-clinical and clinical findings, the various approaches for prevention, protection and therapeutic intervention with acoustic trauma are discussed. Pharmacological strategies involving anti-oxidative, anti-excitotoxic and anti-apoptotic substances as well as non-pharmacological strategies like "sound conditioning" are given attention. Furthermore, systemic and local substance application as well as the therapy of acute acoustic trauma and chronic hearing problems (including modern therapy forms for comorbidities such as tinnitus) shall be delved into. PMID:22073048

  18. Minocycline Protection of Neomycin Induced Hearing Loss in Gerbils

    PubMed Central

    Robinson, Alan M.; Vujanovic, Irena; Richter, Claus-Peter

    2015-01-01

    This animal study was designed to determine if minocycline ameliorates cochlear damage is caused by intratympanic injection of the ototoxic aminoglycoside antibiotic neomycin. Baseline auditory-evoked brainstem responses were measured in gerbils that received 40 mM intratympanic neomycin either with 0, 1.2, or 1.5 mg/kg intraperitoneal minocycline. Four weeks later auditory-evoked brainstem responses were measured and compared to the baseline measurements. Minocycline treatments of 1.2 mg/kg and 1.5 mg/kg resulted in significantly lower threshold increases compared to 0 mg/kg, indicating protection of hearing loss between 6 kHz and 19 kHz. Cochleae were processed for histology and sectioned to allow quantification of the spiral ganglion neurons and histological evaluation of organ of Corti. Significant reduction of spiral ganglion neuron density was demonstrated in animals that did not receive minocycline, indicating that those receiving minocycline demonstrated enhanced survival of spiral ganglion neurons, enhanced survival of sensory hairs cells and spiral ganglion neurons, and reduced hearing threshold elevation correlates with minocycline treatment demonstrating that neomycin induced hearing loss can be reduced by the simultaneous application of minocycline. PMID:25950003

  19. Sensorineural hearing loss and celiac disease: A coincidental finding

    PubMed Central

    Volta, Umberto; Ferri, Gian Gaetano; De Giorgio, Roberto; Fabbri, Angela; Parisi, Claudia; Sciajno, Laura; Castellari, Alessandra; Fiorini, Erica; Piscaglia, Maria; Barbara, Giovanni; Granito, Alessandro; Pirodda, Antonio

    2009-01-01

    BACKGROUND Celiac disease (CD) can be associated with a variety of extraintestinal manifestations, including neurological diseases. A new neurological correlation has been found between CD and sensorineural hearing loss (SNHL). OBJECTIVE To verify the association between SNHL and CD, and to establish whether the neurological hearing impairment in CD is related to nonorgan-specific and antineuronal antibodies, as well as the presence of autoimmune disorders. METHODS A sample of 59 consecutive biopsy- and serologically proven CD patients were studied. Among CD patients, 11 were newly diagnosed and 48 were on a gluten-free diet. Hearing function was assessed by audiometric analysis in all CD patients as well as in 59 age- and sex-matched controls. Patients were tested for a panel of immune markers including nonorgan-specific autoantibodies and antineuronal antibodies. RESULTS SNHL was detected in five CD patients (8.5%) and in two controls (3.4%). In one patient, the SNHL was bilateral, whereas the remaining four had a monolateral impairment. The prevalence of SNHL was not significantly different between CD patients and controls. At least one of the antibodies tested for was positive in two of the five CD patients with SNHL and in 12 of the 54 CD patients without SNHL. Antineuronal antibodies to central nervous system antigens were consistently negative in the five CD patients with SNHL. Only one of the five CD patients with SNHL had Hashimoto thyroiditis. CONCLUSIONS SNHL and CD occur coincidentally. Hearing function should be assessed only in CD patients with clinical signs of hearing deficiency. PMID:19668795

  20. Update of the spectrum of GJB2 gene mutations in Tunisian families with autosomal recessive nonsyndromic hearing loss.

    PubMed

    Riahi, Zied; Hammami, Hassen; Ouragini, Houyem; Messai, Habib; Zainine, Rim; Bouyacoub, Yosra; Romdhane, Lilia; Essaid, Donia; Kefi, Rym; Rhimi, Mohsen; Bedoui, Monia; Dhaouadi, Afef; Feldmann, Delphine; Jonard, Laurence; Besbes, Ghazi; Abdelhak, Sonia

    2013-08-01

    Hearing loss is the most frequent sensory disorder. It affects 3 in 1000 newborns. It is genetically heterogeneous with 60 causally-related genes identified to date. Mutations in GJB2 gene account for half of all cases of non-syndromic deafness. The aim of this study was to determine the relative frequency of GJB2 allele variants in Tunisia. In this study, we screened 138 patients with congenital hearing loss belonging to 131 families originating from different parts of Tunisia for mutations in GJB2 gene. GJB2 mutations were found in 39% of families (51/131). The most common mutation was c.35delG accounting for 35% of all cases (46/131). The second most frequent mutation was p.E47X present in 3.8% of families. Four identified mutations in our cohort have not been reported in Tunisia; p.V37I, c.235delC, p.G130A and the splice site mutation IVS1+1G>A (0.76%). These previously described mutations were detected only in families originating from Northern and not from other geographical regions in Tunisia. In conclusion we have confirmed the high frequency of c.35delG in Tunisia which represents 85.4% of all GJB2 mutant alleles. We have also extended the mutational spectrum of GJB2 gene in Tunisia and revealed a more pronounced allelic heterogeneity in the North compared to the rest of the country.

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

    PubMed

    Preminger, Jill E; Meeks, Suzanne

    2010-04-01

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

  2. Noise Induced Hearing Loss in Iran: (1997-2012): Systematic Review Article.

    PubMed

    Soltanzadeh, Ahmad; Ebrahimi, Hossein; Fallahi, Majid; Kamalinia, Mojtaba; Ghassemi, Shadi; Golmohammadi, Rostam

    2014-12-01

    Noise-induced hearing loss, which is one of the 10 leading occupational diseases, is a debilitating and irreversible disease. During the recent 15-years period (1997-2012), several studies have investigated the association between noise, hearing damage and other side effects of noise in Iran. The aim of this study was to review systematically the relevant literature related to noise-induced hearing loss, lead to developing noise exposure limits. In this systematic review, two researchers independently extracted the data from 31 past studies that had considered noise-induced hearing loss (including hearing loss, temporary and permanent hearing threshold shift and auditory trauma). The data were then recorded in a modified form and Statistical analyses were performed using SPSS, version 16.0. In analyzed studies the weighted average equivalent sound pressure level [L Aeq ] was 90.29 dB(A) and average hearing loss was 26.44 dB(A). The Highest degree of hearing loss in the right ear was associated at 4000 Hz, and the highest degree of hearing loss in the left ear was associated to 1000 and 4000 Hz. The majority of the reviewed studies have confirmed that exposure to a noise level above 85 dB (A) can lead to an increased chance of hearing loss. Furthermore, the results of the present review indicated that as L Aeq increased up to 85 dB(A), so did the severity of the hearing loss.

  3. A Taxonomy of Fatigue Concepts and Their Relation to Hearing Loss.

    PubMed

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

    2016-01-01

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

  4. Improvement of sudden bilateral hearing loss after vertebral artery stenting

    PubMed Central

    Kim, Ji Hwa; Roh, Kyung Jin; Suh, Sang Hyun; Lee, Kyung-Yul

    2015-01-01

    Bilateral deafness is a rare but possible symptom of vertebrobasilar ischemia. We report a case of sudden bilateral sensorineural hearing loss caused by bilateral vertebral artery (VA) occlusion which dramatically improved after stenting. A 54-year-old man was admitted with sudden onset of bilateral deafness, vertigo, and drowsy mental status. Brain diffusion-weighted MRI showed acute infarction involving both the posterior inferior cerebellar artery and left posterior cerebral artery territory. Cerebral angiography showed bilateral distal VA occlusion, and emergency intracranial stenting was performed in the left VA. After reperfusion therapy his symptoms gradually improved, including hearing impairment. Endovascular stenting may be helpful in a patient with sudden deafness caused by bilateral VA occlusion. PMID:25697296

  5. [Gene therapy for human hearing loss: challenges and promises].

    PubMed

    Meyer, Anaïs; Petit, Christine; Safieddine, Saaid

    2013-10-01

    Thanks to the advances accomplished in human genomics during the last twenty years, major progress has been made towards understanding the pathogenesis of various forms of congenital or acquired deafness. The identification of deafness genes, which are potential therapeutic targets, and generation and functional characterization of murine models for human deafness forms have advanced the knowledge of the molecular physiology of auditory sensory cells. These milestones have opened the way for the development of new therapeutic strategies, alternatives to conventional prostheses, hearing amplification for mild-to-severe hearing loss, or cochlear implantation for severe-to-profound deafness. In this review, we first summarize the progress made over the last decade in using gene therapy and antisense RNA delivery, including the development of new methods for cochlear gene transfer. We then discuss the potential of gene therapy for curing acquired or inherited deafness and the major obstacles that must be overcome before clinical application can be considered.

  6. Auditory function and hearing loss in children and adults with Williams syndrome: cochlear impairment in individuals with otherwise normal hearing.

    PubMed

    Marler, Jeffrey A; Sitcovsky, Jessica L; Mervis, Carolyn B; Kistler, Doris J; Wightman, Frederic L

    2010-05-15

    Hearing loss is common in school-age individuals with Williams syndrome (WS) and extensive in adults. Prior studies with relatively small sample sizes suggest that hearing loss in WS has an early onset and may be progressive, yet the auditory phenotype and the scope of the hearing loss have not been adequately characterized. We used standard audiometric tools: Otoscopy, tympanometry, air-conduction (bone conduction when available) behavioral testing, and distortion product otoacoustic emissions (DPOAEs) to measure hearing sensitivity and outer hair cell function. We tested 81 individuals with WS aged 5.33-59.50 years. Sixty-three percent of the school-age and 92% of the adult participants had mild to moderately-severe hearing loss. The hearing loss in at least 50% was sensorineural. DPOAE testing corroborated behavioral results. Strikingly, 12 of 14 participants with hearing within normal limits bilaterally had 4,000-Hz DPOAE input/output (DPOAE IO) functions indicative of outer hair cell damage and impaired cochlear compression. Our results indicate that hearing loss is very common in WS. Furthermore, individuals with WS who have "normal" hearing as defined by behavioral thresholds may actually have sub-clinical impairments or undetected cochlear pathology. Our findings suggest outer hair cell dysfunction in otherwise normal hearing individuals. The DPOAE IO in this same group revealed growth functions typically seen in groups with noise-induced damage. Given this pattern of findings, individuals with WS may be at increased risk of noise-induced hearing loss. Recommendations regarding audiological testing for individuals with WS and accommodations for these individuals in both academic and nonacademic settings are provided.

  7. Mechanisms of Hearing Loss after Blast Injury to the Ear

    PubMed Central

    Cho, Sung-Il; Gao, Simon S.; Xia, Anping; Wang, Rosalie; Salles, Felipe T.; Raphael, Patrick D.; Abaya, Homer; Wachtel, Jacqueline; Baek, Jongmin; Jacobs, David; Rasband, Matthew N.; Oghalai, John S.

    2013-01-01

    Given the frequent use of improvised explosive devices (IEDs) around the world, the study of traumatic blast injuries is of increasing interest. The ear is the most common organ affected by blast injury because it is the body’s most sensitive pressure transducer. We fabricated a blast chamber to re-create blast profiles similar to that of IEDs and used it to develop a reproducible mouse model to study blast-induced hearing loss. The tympanic membrane was perforated in all mice after blast exposure and found to heal spontaneously. Micro-computed tomography demonstrated no evidence for middle ear or otic capsule injuries; however, the healed tympanic membrane was thickened. Auditory brainstem response and distortion product otoacoustic emission threshold shifts were found to be correlated with blast intensity. As well, these threshold shifts were larger than those found in control mice that underwent surgical perforation of their tympanic membranes, indicating cochlear trauma. Histological studies one week and three months after the blast demonstrated no disruption or damage to the intra-cochlear membranes. However, there was loss of outer hair cells (OHCs) within the basal turn of the cochlea and decreased spiral ganglion neurons (SGNs) and afferent nerve synapses. Using our mouse model that recapitulates human IED exposure, our results identify that the mechanisms underlying blast-induced hearing loss does not include gross membranous rupture as is commonly believed. Instead, there is both OHC and SGN loss that produce auditory dysfunction. PMID:23840874

  8. Infants and Children with Hearing Loss Need Early Language Access

    PubMed Central

    Kushalnagar, Poorna; Mathur, Gaurav; Moreland, Christopher J.; Napoli, Donna Jo; Osterling, Wendy; Padden, Carol; Rathmann, Christian

    2010-01-01

    Around 96 percent of children with hearing loss are born to parents with intact hearing,1 who may initially know little about deafness or sign language. Therefore, such parents will need information and support in making decisions about the medical, linguistic, and educational management of their child. Some of these decisions are time-sensitive and irreversible and come at a moment of emotional turmoil and vulnerability (when some parents grieve the toss of a normally hearing child).2 Clinical research indicates that a deaf child’s poor communication skills can be made worse by increased level of parental depression.3 Given this, the importance of reliable and up-to-date support for parents’ decisions is critical to the overall well-being of their child.4 In raising and educating a child, parents are often offered an exclusive choice between an oral environment (including assistive technology, speech reading, and voicing) and a signing environment. A heated controversy surrounds this choice, and has since at least the late 19th century, beginning with the International Congress on the Education of the Deaf in Milan, held in 1880.5 While families seek advice from many sources, including, increasingly, the internet,6 the primary care physician (PCP) is the professional medical figure the family interacts with repeatedly.7 The present article aims to help family advisors, particularly the PCP and other medical advisors in this regard. We argue that deaf children need to be exposed regularly and frequently to good language models in both visual and auditory modalities from the time hearing loss is detected and continued throughout their education to ensure proper cognitive, psychological, and educational development. Since there is, unfortunately, a dearth of empirical studies on many of the issues families must confront, professional opinions, backed by what studies do exist, are the only option. We here give our strongly held professional opinions and stress the

  9. Asymmetric Hearing Loss in Chinese Workers Exposed to Complex Noise

    PubMed Central

    Wang, Xiaoxiao; Li, Nan; Zeng, Lin; Tao, Liyuan; Zhang, Hua; Yang, Qiuling; Qiu, Wei; Zhu, Liangliang; Zhao, Yiming

    2015-01-01

    Objectives Evaluate audiometric asymmetry in Chinese industrial workers and investigate the effects of noise exposure, sex, and binaural average thresholds on audiometric asymmetry. Design Data collected from Chinese industrial workers during a cross-sectional study were re-analyzed. Of the 1388 workers, 266 met the inclusion criteria for this study. Each subject underwent a physical examination and an otologic examination and completed a health-related questionnaire. χ2 and t tests were used to examine the differences between the asymmetric and symmetric hearing loss groups. Results One hundred thirty-one (49.2%) subjects had a binaural hearing threshold difference of 15 dB or more for at least one frequency, and there was no statistically significant difference between the left and right ears. The asymmetric hearing loss group was not exposed to higher cumulative noise levels (t = 0.522, p = 0.602), and there was no dose-response relationship between asymmetry and cumulative noise levels (χ2 = 6.502, p = 0.165). Men were 1.849 times more likely to have asymmetry than women were [95% confidence interval (CI), 1.051–3.253]. Among the workers with higher high-frequency hearing thresholds (HFHTs), audiometric asymmetry was 1.024 times more prevalent than that among those with lower HFHTs (95% CI, 1.004–1.044). Conclusions The results indicated that occupational noise exposure contributed minimally to asymmetry, while sex and binaural average thresholds significantly affected audiometric asymmetry. There was no evidence that the left ears were worse than the right ears. PMID:26502292

  10. Occupational hearing loss of market mill workers in the city of Accra, Ghana.

    PubMed

    Kitcher, Emmanuel D; Ocansey, Grace; Abaidoo, Benjamin; Atule, Alidu

    2014-01-01

    Noise induced hearing loss (NIHL) is an irreversible sensorineural hearing loss associated with exposure to high levels of excessive noise. Prevention measures are not well established in developing countries. This comparative cross sectional study aims to determine the prevalence of hearing loss in both a group of high risk workers and a control group and to assess their knowledge of the effects of noise on hearing health. A total of 101 market mill workers and 103 controls employed within markets in the city of Accra, Ghana, were evaluated using a structured questionnaire and pure tone audiometry. The questionnaire assessed factors including self-reported hearing loss, tinnitus, knowledge on the effects of noise on hearing health and the use of hearing protective devices. Pure tone audiometric testing was conducted for both mill workers and controls. Noise levels at the work premises of the mill workers and controls were measured. Symptoms of hearing loss were reported by 24 (23.76%) and 8 (7.7%) mill workers and controls respectively. Fifty-five (54.5%) and fifty-four (52.37%) mill workers and controls exhibited knowledge of the effects of noise on hearing health. Five (5.0%) mill workers used hearing protective devices. There was significant sensorineural hearing loss and the presence of a 4 kHz audiometric notch among mill workers when compared with controls for the mean thresholds of 2 kHz, 3 kHz and 4 kHz (P = 0. 001). The prevalence of hearing loss in the better hearing ears of the mill workers and controls was 24.8% and 4.8% respectively (P < 0.5). The prevalence of hearing loss, which may be characteristic of NIHL in the better hearing ears of the mill workers and controls was 24.8% and 4.8% respectively. The majority of mill workers did not use hearing protection.

  11. Idiopathic sudden sensorineural hearing loss: cardiovascular risk factors do not influence hearing threshold recovery.

    PubMed

    Ciorba, A; Hatzopoulos, S; Bianchini, C; Iannini, V; Rosignoli, M; Skarzynski, H; Aimoni, C

    2015-04-01

    Previous studies have suggested that risk factors for ischaemic vascular disease, such as cigarette smoking, hypertension and hyperlipidaemia, can also be considered risk factors for the development of idiopathic sudden sensorineural hearing loss (ISSNHL). In this study, we have evaluated the hypothesis that these factors can influence hearing threshold recovery in patients affected by ISSNHL. A total of 141 subjects who suffered an episode of ISSNHL were included. All subjects were assessed with tonal audiometry, auditory brainstem responses and MRI to exclude retrocochlear pathology. Hearing tests were conducted at ISSNHL onset (t = 0) and after 30 days. Patients were divided into three classes according to the presence/absence of one or more cardiovascular risk factors including: history of smoking, total serum cholesterol/triglycerides, history of hypertension and diabetes mellitus. Values of hearing threshold recovery were estimated and comparisons were conducted across the three risk factor classes. 75% of patients affected by ISSNHL showed a threshold recovery. However, the threshold recovery was found to be class-independent (average recovery value of 18 dB HL per classes) and also independent of age and gender. Even if cardiovascular risk factors have been found to be involved in the pathogenesis of ISSNHL, the present study suggests that these factors do not have any significant influence on the threshold recovery in ISSNHL.

  12. Audiometric notch as a sign of noise induced hearing loss

    PubMed Central

    McBride, D; Williams, S

    2001-01-01

    OBJECTIVES—To investigate the relation between different types of exposure to noise and a classic sign of noise induced hearing loss (NIHL), the audiometric notch.
METHODS—The study sample had exposure to both continuous and impulse noise and was drawn from a population of electrical transmission workers. Audiograms, taken as part of a hearing conservation programme, were read by three clinicians experienced in the assessment of NIHL. Working independently and using their clinical judgment, they were asked to identify localised increases in the threshold of hearing (audiometric notches) which they would attribute to noise, had a suitable history of exposure been elicited. Prevalent cases of NIHL were identified by the presence of a notch in either ear. Risk factors for NIHL were assessed by a questionnaire which sought information about exposure to air blast circuit breaker noise; firearms; explosions, and continuous noise. The odds of exposure to these factors in those with and without hearing loss were calculated, and odds ratios (ORs) and 95% confidence intervals (95% CIs) were estimated by logistic regression.
RESULTS—Of the 648 questionnaires sent out 357 were returned, a response rate of 55%. Of these, at least two out of the three assessors identified 175 (49%) people with a notch at any audiometric frequency. There was no association between these cases and the NIHL risk factors identified by the questionnaire, but a further frequency specific analysis showed a small proportion of people (15 (4%)) with notches at 4 kHz who had the expected associations with exposure to noise and a significant OR for firearms of 4.25 (95% CI 1.28 to 14.1). The much larger proportion of people with 6 kHz notches (110 (31%)) did not show these associations.
CONCLUSIONS—To diagnose NIHL it is important to elicit a detailed and accurate history of exposure to noise: although the notch at 4 kHz is a well established clinical sign and may be valuable in

  13. GJB2-associated hearing loss undetected by hearing screening of newborns.

    PubMed

    Minami, Shujiro B; Mutai, Hideki; Nakano, Atsuko; Arimoto, Yukiko; Taiji, Hidenobu; Morimoto, Noriko; Sakata, Hideaki; Adachi, Nodoka; Masuda, Sawako; Sakamoto, Hirokazu; Yoshida, Haruo; Tanaka, Fujinobu; Morita, Noriko; Sugiuchi, Tomoko; Kaga, Kimitaka; Matsunaga, Tatsuo

    2013-12-10

    The hearing loss caused by GJB2 mutations is usually congenital in onset, moderate to profound in degree, and non-progressive. The objective of this study was to study genotype/phenotype correlations and to document 14 children with biallelic GJB2 mutations who passed newborn hearing screening (NHS). Genetic testing for GJB2 mutations by direct sequencing was performed on 924 individuals (810 families) with hearing loss, and 204 patients (175 families) were found to carry biallelic GJB2 mutations. NHS results were obtained through medical records. A total of 18 pathological mutations were identified, which were subclassified as eight inactivating and 10 non-inactivating mutations. p.I128M and p.H73Y were identified as novel missense GJB2 mutations. Of the 14 children with biallelic GJB2 mutations who passed NHS, eight were compound heterozygotes and 3 were homozygous for the c.235delC mutation in GJB2, and the other three combinations of non-c.235delC mutations identified were p.Y136X-p.G45E/p.V37I heterozygous, c.512ins4/p.R143W heterozygous, and p.V37I/p.R143W heterozygous. These 14 cases demonstrate that the current NHS does not identify all infants with biallelic GJB2 mutations. They suggest that the frequency of non-penetrance at birth is approximately 6.9% or higher in DFNB1 patients and provide further evidence that GJB2 hearing loss may not always be congenital in onset.

  14. Resolution of sudden sensorineural hearing loss following a roller coaster ride.

    PubMed

    Kumar, Aman; Sinha, Amrita; Al-Waa, Ahmad M

    2011-07-01

    We report a case of sudden unilateral sensorineural hearing loss of sudden onset during an aeroplane flight, which completely resolved during a roller coaster ride at Alton Towers theme park. A review of the literature concerning sudden idiopathic sensorineural hearing loss and spontaneous resolution are discussed. Initially, pure-tone audiometry showed a profound sensorineural hearing loss in the right ear and mild sensorineural hearing loss in the left ear (of note, the hearing was normal prior to the episode). Following resolution of the patient's symptoms during a roller coaster ride, pure-tone audiometry showed normal hearing thresholds in both ears. Sudden sensorineural hearing loss is a symptom of cochlear injury and the mechanism of the patient's symptoms was attributed to a patent cochlear aqueduct.

  15. Towards a comprehensive approach for managing transitions of older workers with hearing loss.

    PubMed

    Fok, Daniel; Shaw, Lynn; Jennings, Mary Beth; Cheesman, Margaret

    2009-01-01

    Demographic and legislative trends suggest that many older workers may remain at work past the traditional retirement age. This extended work trajectory poses new challenges and opportunities for workers with acquired hearing loss as they age. Workplaces require a new approach to enable transitions of older workers with hearing loss to remain safe and productive. A review of the literature on older workers, those with hearing loss, and strategies used to accommodate them suggests that individualized and piecemeal approaches are predominant. While universal design represents a fresh ideology that may help create more accessible and usable products and environments, its application to improve workplaces for older workers with hearing loss is limited. This paper proposes that occupational science be integrated with knowledge in hearing sciences, accessibility, and usability to assist with the transitions faced by older workers with hearing loss. A more comprehensive approach including the following three key components will be posited to examine the nexus of aging, hearing loss and work: (1) the use of an occupational perspective, along with concepts in hearing sciences to examine hearing demands and improve hearing access; (2) the use of contextual processes to promote physical and social change, and (3) the inclusion of Universal Design for Hearing (UDH) considerations as stakeholders develop more hearing friendly workplaces.

  16. The influence of hearing-aid compression on forward-masked thresholds for adults with hearing loss.

    PubMed

    Brennan, Marc A; McCreery, Ryan W; Jesteadt, Walt

    2015-10-01

    This paper describes forward-masked thresholds for adults with hearing loss. Previous research has demonstrated that the loss of cochlear compression contributes to deficits in this measure of temporal resolution. Cochlear compression can be mimicked with fast-acting compression where the normal dynamic range is mapped to the impaired dynamic range. To test the hypothesis that fast-acting compression will most-closely approximate the normal ability to perceive forward-masked pure-tones, forward-masked thresholds were measured for two groups of adults (normal hearing, hearing loss). Adults with normal hearing were tested without amplification. Adults with hearing loss were tested with three different compression speeds and two different prescriptive procedures using a hearing-aid simulator. The two prescriptive procedures differed in the extent to which the normal dynamic range was mapped onto the impaired dynamic range. When using a faster compression speed with the prescriptive procedure that best restored the lost dynamic range, forward-masked thresholds for the listeners with hearing loss approximated those observed for the listeners with normal hearing.

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

    PubMed

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

    2013-01-01

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

  18. Loudness perception affected by early age hearing loss.

    PubMed

    Sun, Wei; Fu, Qiang; Zhang, Chao; Manohar, Senthilvelan; Kumaraguru, Anand; Li, Ji

    2014-07-01

    Tinnitus and hyperacusis, commonly seen in adults, are also reported in children. Although clinical studies found children with tinnitus and hyperacusis often suffered from recurrent otitis media, there is no direct study on how temporary hearing loss in the early age affects the sound loudness perception. In this study, sound loudness changes in rats affected by perforation of the tympanic membranes (TM) have been studied using an operant conditioning based behavioral task. We detected significant increases of sound loudness and susceptibility to audiogenic seizures (AGS) in rats with bilateral TM damage at postnatal 16 days. As increase to sound sensitivity is commonly seen in hyperacusis and tinnitus patients, these results suggest that early age hearing loss is a high risk factor to induce tinnitus and hyperacusis in children. In the TM damaged rats, we also detected a reduced expression of GABA receptor δ and α6 subunits in the inferior colliculus (IC) compared to the controls. Treatment of vigabatrin (60 mg/kg/day, 7-14 days), an anti-seizure drug that inhibits the catabolism of GABA, not only blocked AGS, but also significantly attenuated the loudness response. Administration of vigabatrin following the early age TM damage could even prevent rats from developing AGS. These results suggest that TM damage at an early age may cause a permanent reduction of GABA tonic inhibition which is critical towards the maintenance of normal loudness processing of the IC. Increasing GABA concentration during the critical period may alleviate the impairment in the brain induced by early age hearing loss.

  19. [The active middle ear implant for the rehabilitation of sensorineural, mixed and conductive hearing losses].

    PubMed

    Sprinzl, G M; Wolf-Magele, A; Schnabl, J; Koci, V

    2011-09-01

    Active middle ear implants, such as the Vibrant Soundbridge, are used as an important part in the rehabilitation of sensorineural, conductive hearing, or mixed hearing loss. The attachment of the Vibrant Soundbridge at the round window and the usage of the Vibroplasty couplers strongly expanded the application of the Vibrant Soundbridge.The Vibrant Soundbridge is developed for patients who have an intolerance to hearing aids and a moderate to profound sensorineural hearing loss. The VSB also provides an optimal solution for patients with failed middle ear reconstructions or patients with atresia. To capture the improvement with the VSB Implant with different hearing losses a literature analysis was conducted. The functional gain was analyzed for 107 patients with conductive hearing loss and for 214 patients with sensorineural hearing loss out of 14 studies.Patients with conductive and mixed hearing loss resulted in a functional gain from 30 to 58 dB with the VSB. Patients with a pure sensorineural hearing loss showed a functional gain of 23-30 dB. The VSB bone conduction threshold shift was analyzed for all studies conducted in the years between 2000 and 2009. In 11 of the 16 studies there was no significant (p=0.05) change found. In 5 studies, the pre- to post-surgical bone conduction threshold shift was less than 10 dB. None of these studies measured a threshold shift of more than 10 dB.The flexible attachment at either the long process of the incus with sensorineural hearing loss, with an conductive hearing loss at the round window or the use of Vibroplasty couplers at the oval window, head of the stapes or round window makes the VSB an extremely versatile instrument. If patients can't wear conventional hearing aids, had failed middle ear reconstructions or atresia the VSB presents, due to the significant hearing improvement in any type of hearing loss, an ideal solution.

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

    PubMed Central

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

    2016-01-01

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

  1. Bringing text display digital radio to consumers with hearing loss.

    PubMed

    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 consumers with National Public Radio stories converted to text and examined their preferences for and reactions to midsized and small radio text displays. We focused on physical display attributes such as text color, font style, line length, and scrolling type as well as emergency alert messages and emergency prompts for drivers, announcer identification schemes, and synchronization of audio and text. Results suggest that midsized, Global Positioning System (GPS)-style displays were well liked, synchronization of audio and text was important to comprehension and retrieval of story details, identification of announcers was served best with a combination of name change in parenthesis and color change, and a mixture of color and flashing symbols was preferred for emergency alerting.

  2. G-CSF attenuates noise-induced hearing loss.

    PubMed

    Shi, Ze-tao; Lin, Ying; Wang, Jie; Wu, Jin; Wang, Ren-feng; Chen, Fu-quan; Mi, Wen-juan; Qiu, Jian-hua

    2014-03-06

    In this study, we investigated the effects of granulocyte colony-stimulating factor (G-CSF) for the treatment of noise-induced hearing loss (NIHL) in a guinea pig model. Forty guinea pigs were randomly divided into four groups: control, noise (white noise, 3 h/d for 2 days at 115 dB), noise+G-CSF (350 μg/kg/d for 5 days), and noise+saline. Auditory brainstem response (ABR) and distortion product otoacoustic emission (DPOAE) were used to determine the hearing threshold and outer hair cell function, respectively, in each group. Cochlear morphology was examined to evaluate hair cell injury induced by intense noise exposure. Fourteen days after noise exposure, the noise+G-CSF group had a lower ABR value than the noise group (P<0.05) or the noise+saline group (P<0.01). At most frequencies, the DPOAE value of the noise+G-CSF group showed a significant rise (P<0.05) compared to the noise group or the noise+saline group. Neither the ABR value nor the DPOAE value differed between the noise group and the noise+saline group. The morphology of the phalloidin-stained organ of Corti was consistent with the functional measurements. In conclusion, G-CSF can preserve hearing in an experimental model of NIHL in guinea pigs, by preserving hair cells after intense noise exposure.

  3. The nature of victimization among youths with hearing loss in substance abuse treatment.

    PubMed

    Titus, Janet C

    2010-01-01

    The author profiles the prevalence, severity, and characteristics of victimization among a group of youths with hearing loss presenting to substance abuse treatment. Intake data on 111 deaf and hard of hearing youths (42% female) were analyzed and compared with data from a weighted, gender-matched sample of hearing youths. After gender is controlled, results indicate that the hearing loss group reported more widespread, more severe abuse than that reported by their hearing peers. Physical abuse and weapon attacks were significantly more prevalent among the deaf and hard of hearing youths, while no differences in sexual or emotional abuse were observed. Youths with hearing loss also reported increased rates of abuse by a trusted person and abuse that made them fear for their lives. Substance abuse treatment of deaf and hard of hearing youths should include routine assessment of victimization and trauma-informed methods.

  4. Auditory, Visual, and Auditory-Visual Perceptions of Emotions by Young Children with Hearing Loss versus Children with Normal Hearing

    ERIC Educational Resources Information Center

    Most, Tova; Michaelis, Hilit

    2012-01-01

    Purpose: This study aimed to investigate the effect of hearing loss (HL) on emotion-perception ability among young children with and without HL. Method: A total of 26 children 4.0-6.6 years of age with prelingual sensory-neural HL ranging from moderate to profound and 14 children with normal hearing (NH) participated. They were asked to identify…

  5. Pitch and Loudness from Tinnitus in Individuals with Noise-induced Hearing Loss

    PubMed Central

    Flores, Leticia Sousa; Teixeira, Adriane Ribeiro; Rosito, Leticia Petersen Schmidt; Seimetz, Bruna Macagnin; Dall'Igna, Celso

    2015-01-01

    Introduction  Tinnitus is one of the symptoms that affects individuals suffering from noise induced hearing loss. This condition can be disabling, leading the affected individual to turn away from work. Objective  This literature review aims to analyze the possible association between gender and tinnitus pitch and loudness, the degree of hearing loss and the frequencies affected in subjects with noise-induced hearing loss. Methods  This contemporary cohort study was conducted through a cross-sectional analysis. The study sample consisted of adults with unilateral or bilateral tinnitus, who had been diagnosed with noise-induced hearing loss. The patients under analysis underwent an otorhinolaryngological evaluation, pure tone audiometry, and acuphenometry. Results  The study included 33 subjects with noise-induced hearing loss diagnoses, of which 22 (66.7%) were men. Authors observed no statistical difference between gender and loudness/pitch tinnitus and loudness/pitch in subjects with bilateral tinnitus. Authors found an inverse relation between tinnitus loudness with intensity greater hearing threshold and the average of the thresholds and the grade of hearing loss. The tinnitus pitch showed no association with higher frequency of hearing threshold. Conclusion  Data analysis shows that, among the individuals evaluated, the greater the hearing loss, the lower the loudness of tinnitus. We did not observe an association between hearing loss and tinnitus pitch. PMID:27413408

  6. Evaluating long-latency auditory evoked potentials in the diagnosis of cortical hearing loss in children

    PubMed Central

    Lopez-Soto, Teresa; Postigo-Madueno, Amparo; Nunez-Abades, Pedro

    2016-01-01

    In centrally related hearing loss, there is no apparent damage in the auditory system, but the patient is unable to hear sounds. In patients with cortical hearing loss (and in the absence of communication deficit, either total or partial, as in agnosia or aphasia), some attention-related or language-based disorders may lead to a wrong diagnosis of hearing impairment. The authors present two patients (8 and 11 years old) with no anatomical damage to the ear, the absence of neurological damage or trauma, but immature cortical auditory evoked potentials. Both patients presented a clinical history of multiple diagnoses over several years. Because the most visible symptom was moderate hearing loss, the patients were recurrently referred to audiological testing, with no improvement. This report describes the use of long-latency evoked potentials to determine cases of cortical hearing loss, where hearing impairment is a consequence of underdevelopment at the central nervous system. PMID:27006780

  7. Osteogenesis imperfecta and hearing loss--description of three case reports.

    PubMed

    Pereira da Silva, Ana; Feliciano, Telma; Figueirinhas, Rosário; Almeida E Sousa, Cecília

    2013-01-01

    Osteogenesis imperfecta is the commonest connective tissue hereditary disease. Its clinical presentation has a wide spectrum of characteristics, which includes skeletal deformities and hearing loss. We describe three case reports of individuals carriers of this disease presenting with different patterns of hearing loss. Hearing loss prevalence and patterns are variable and have no clear relation with genotype. Its assessment at initial evaluation and posterior monitoring is essential to provide the best therapeutic alternatives.

  8. Subchronic JP-8 Jet Fuel Exposure Enhances Vulnerability to Noise-Induced Hearing Loss in Rats

    DTIC Science & Technology

    2012-01-01

    with noise. The objective of this study was to evaluate the potency of JP-8 jet fuel to enhance noise-induced hearing loss ( NIHL ) using inhalation...The objective of this study was to evaluate the potency of JP-8 jet fuel to enhance noise-induced hearing loss ( NIHL ) using inhalation exposure to...susceptibility to noise-induced hearing loss ( NIHL ). Once these objectives were met using a continuous noise exposure paradigm, an additional study

  9. Research in Prevention and Treatment of Noise-Induced Hearing Loss (NIHL)

    DTIC Science & Technology

    2014-04-01

    of Noise-Induced Hearing Loss ( NIHL ) PRINCIPAL INVESTIGATOR: Kathleen Campbell, PhD CONTRACTING ORGANIZATION: Southern Illinois...NUMBER W81XWH-12-1-0031 Hearing Loss ( NIHL ) 5c. PROGRAM ELEMENT NUMBER 6. AUTHOR(S) Kathleen Campbell, PhD Betty Diamond 5d. PROJECT...delayed time otoprotective response from steady state and impulse noise-induced hearing loss ( NIHL ). The second year was dedicated to determining

  10. Research in Prevention and Treatment of Noise-Induced Hearing Loss (NIHL)

    DTIC Science & Technology

    2013-04-01

    Noise-Induced Hearing Loss ( NIHL ) PRINCIPAL INVESTIGATOR: Dr. Kathleen Campbell...Prevention and Treatment of Noise-Induced Hearing Loss ( NIHL ) 5a. CONTRACT NUMBER 5b. GRANT NUMBER W81XWH-12-1-0031 5c. PROGRAM ELEMENT NUMBER...methionine Dose Response Curves for Prevention of Noise Induced Hearing Loss ( NIHL ) for Steady State and Impulse Noise Target required for clinical

  11. Development of a FDA-Approved Pharmaceutical to Treat Noise-Induced Hearing Loss

    DTIC Science & Technology

    2014-08-13

    CONTRACT NUMBER: N62645-12-C-403 7 TITLE: Development of a FDA-Approved Pharmaceutical to Treat Noise-Induced Hearing Loss PRINCIPAL INVESTIGATOR...Development of a FDA-Approved Pharmaceutical to Treat Noise-Induced N62645-12-C-4037 Hearing Loss (NIHL) 5b. GRANT NUMBER 5c. PROGRAM ELEMENT...TERMS Noise-induced hearing loss, pharmaceutical , pre-clinical, animal studies 16. SECURITY CLASSIFICATION OF: 17. LIMITATION OF 18. NUMBER a. REPORT

  12. Comprehensive genetic testing with ethnic-specific filtering by allele frequency in a Japanese hearing-loss population

    PubMed Central

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

    2016-01-01

    Recent advances in targeted genomic enrichment with massively parallel sequencing (TGE+MPS) have made comprehensive genetic testing for non-syndromic hearing loss (NSHL) possible. After excluding NSHL subjects with causative mutations in GJB2 and the MT-RNR1 (1555A>G) variant by Sanger sequencing, we completed TGE+MPS on 194 probands with presumed NSHL identified across Japan. We used both publicly available minor allele frequency (MAF) datasets and ethnic-specific MAF filtering against an in-house database of 200 normal-hearing Japanese controls. Ethnic-specific MAF filtering allowed us to re-categorize as common 203 variants otherwise annotated as rare or novel in non-Japanese ethnicities. This step minimizes false-positive results and improves the annotation of identified variants. Causative variants were identified in 27% of probands with solve rates of 35%, 35% and 19% for dominant, recessive and sporadic NSHL, respectively. Mutations in MYO15A and CDH23 follow GJB2 as the frequent causes of recessive NSHL; copy number variations in STRC are a major cause of mild-to-moderate NSHL. Ethnic-specific filtering by allele frequency is essential to optimize the interpretation of genetic data. PMID:26346818

  13. Sonar-induced temporary hearing loss in dolphins.

    PubMed

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

    2009-08-23

    There is increasing concern that human-produced ocean noise is adversely affecting marine mammals, as several recent cetacean mass strandings may have been caused by animals' interactions with naval 'mid-frequency' sonar. However, it has yet to be empirically demonstrated how sonar could induce these strandings or cause physiological effects. In controlled experimental studies, we show that mid-frequency sonar can induce temporary hearing loss in a bottlenose dolphin (Tursiops truncatus). Mild-behavioural alterations were also associated with the exposures. The auditory effects were induced only by repeated exposures to intense sonar pings with total sound exposure levels of 214 dB re: 1 microPa(2) s. Data support an increasing energy model to predict temporary noise-induced hearing loss and indicate that odontocete noise exposure effects bear trends similar to terrestrial mammals. Thus, sonar can induce physiological and behavioural effects in at least one species of odontocete; however, exposures must be of prolonged, high sound exposures levels to generate these effects.

  14. Toward a Diagnostic Test for Hidden Hearing Loss

    PubMed Central

    Léger, Agnès; Prendergast, Garreth; Kluk, Karolina; Guest, Hannah; Munro, Kevin J.

    2016-01-01

    Cochlear synaptopathy (or hidden hearing loss), due to noise exposure or aging, has been demonstrated in animal models using histological techniques. However, diagnosis of the condition in individual humans is problematic because of (a) test reliability and (b) lack of a gold standard validation measure. Wave I of the transient-evoked auditory brainstem response is a noninvasive electrophysiological measure of auditory nerve function and has been validated in the animal models. However, in humans, Wave I amplitude shows high variability both between and within individuals. The frequency-following response, a sustained evoked potential reflecting synchronous neural activity in the rostral brainstem, is potentially more robust than auditory brainstem response Wave I. However, the frequency-following response is a measure of central activity and may be dependent on individual differences in central processing. Psychophysical measures are also affected by intersubject variability in central processing. Differential measures may help to reduce intersubject variability due to unrelated factors. A measure can be compared, within an individual, between conditions that are affected differently by cochlear synaptopathy. Validation of the metrics is also an issue. Comparisons with animal models, computational modeling, auditory nerve imaging, and human temporal bone histology are all potential options for validation, but there are technical and practical hurdles and difficulties in interpretation. Despite the obstacles, a diagnostic test for hidden hearing loss is a worthwhile goal, with important implications for clinical practice and health surveillance. PMID:27604783

  15. Assessing residual vestibular function in adults with congenital hearing loss.

    PubMed

    Lin, Bing-Yi; Young, Yi-Ho

    2016-12-01

    This study adopted an inner ear test battery in adults with congenital hearing loss to assess residual vestibular function. Thirty patients with non-inherited non-syndromatic congenital hearing loss were assigned to two groups based on their pure tone average (PTA). Subjects in Group A (n = 10) had PTA >90 dB, and those with PTA ≤90 dB were assigned to Group B (n = 20). All patients underwent audiometry, and ocular vestibular-evoked myogenic potential (oVEMP), cervical VEMP (cVEMP) and caloric tests. Percentages of abnormal PTA, and cVEMP, oVEMP, and caloric test results showed a significantly sequential decline in inner ear deficits from the cochlea to the saccule, utricle, and semicircular canals. Group A had significantly higher percentages of absent oVEMP and caloric areflexia than Group B. Via receiver operating characteristic curve analysis, the cutoff value of PTA was 65 dB for discriminating between present and absent oVEMP/caloric responses, with a sensitivity of 71 % and a specificity of 88 %. In conclusion, congenitally deaf patients with PTA ≥65 dB may retain less vestibular function than those with PTA <65 dB, as evidenced by higher percentages of absent oVEMP and caloric areflexia. Hence, comprehensive assessment of the residual vestibular function in congenitally deaf patients may help predict the occurrence of vertigo in the future.

  16. Cellular mechanisms of noise-induced hearing loss.

    PubMed

    Kurabi, Arwa; Keithley, Elizabeth M; Housley, Gary D; Ryan, Allen F; Wong, Ann C-Y

    2016-12-02

    Exposure to intense sound or noise can result in purely temporary threshold shift (TTS), or leave a residual permanent threshold shift (PTS) along with alterations in growth functions of auditory nerve output. Recent research has revealed a number of mechanisms that contribute to noise-induced hearing loss (NIHL). The principle cause of NIHL is damage to cochlear hair cells and associated synaptopathy. Contributions to TTS include reversible damage to hair cell (HC) stereocilia or synapses, while moderate TTS reflects protective purinergic hearing adaptation. PTS represents permanent damage to or loss of HCs and synapses. While the substrates of HC damage are complex, they include the accumulation of reactive oxygen species and the active stimulation of intracellular stress pathways, leading to programmed and/or necrotic cell death. Permanent damage to cochlear neurons can also contribute to the effects of NIHL, in addition to HC damage. These mechanisms have translational potential for pharmacological intervention and provide multiple opportunities to prevent HC damage or to rescue HCs and spiral ganglion neurons that have suffered injury. This paper reviews advances in our understanding of cellular mechanisms that contribute to NIHL and their potential for therapeutic manipulation.

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

    PubMed Central

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

    2016-01-01

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

  18. Emerging treatments for noise-induced hearing loss

    PubMed Central

    Oishi, Naoki; Schacht, Jochen

    2011-01-01

    Introduction Approximately 5% of the population worldwide suffer from industrial, military, or recreational noise-induced hearing loss (NIHL) at great economic cost and detriment to the quality of life of affected individuals. This review discusses pharmacological strategies to attenuate NIHL that have been developed in animal models and that are now beginning to be tested in field trials. Areas covered The review describes the epidemiology, pathology and pathophysiology of NIHL in experimental animals and human. The underlying molecular mechanisms of damage are then discussed as a basis for therapeutic approaches to ameliorate the loss of auditory function. Finally, studies in military, industrial, and recreational settings are evaluated. Literature was searched employing the terms “noise-induced hearing loss” and “noise trauma”. Expert opinion NIHL, in principle, can be prevented. With the current pace of development, oral drugs to protect against NIHL should be available within the next 5 to 10 years. Positive results from ongoing trials combined with additional laboratory tests might accelerate the time from the bench to clinical treatment. PMID:21247358

  19. Achieving Developmental Synchrony in Young Children With Hearing Loss

    PubMed Central

    Mellon, Nancy K.; Ouellette, Meredith; Greer, Tracy; Gates-Ulanet, Patricia

    2009-01-01

    Children with hearing loss, with early and appropriate amplification and intervention, demonstrate gains in speech, language, and literacy skills. Despite these improvements many children continue to exhibit disturbances in cognitive, behavioral, and emotional control, self-regulation, and aspects of executive function. Given the complexity of developmental learning, educational settings should provide services that foster the growth of skills across multiple dimensions. Transdisciplinary intervention services that target the domains of language, communication, psychosocial functioning, motor, and cognitive development can promote academic and social success. Educational programs must provide children with access to the full range of basic skills necessary for academic and social achievement. In addition to an integrated curriculum that nurtures speech, language, and literacy development, innovations in the areas of auditory perception, social emotional learning, motor development, and vestibular function can enhance student outcomes. Through ongoing evaluation and modification, clearly articulated curricular approaches can serve as a model for early intervention and special education programs. The purpose of this article is to propose an intervention model that combines best practices from a variety of disciplines that affect developmental outcomes for young children with hearing loss, along with specific strategies and approaches that may help to promote optimal development across domains. Access to typically developing peers who model age-appropriate skills in language and behavior, small class sizes, a co-teaching model, and a social constructivist perspective of teaching and learning, are among the key elements of the model. PMID:20150187

  20. Cochlear Synaptopathy and Noise-Induced Hidden Hearing Loss

    PubMed Central

    Shi, Lijuan; Chang, Ying; Li, Xiaowei; Aiken, Steve

    2016-01-01

    Recent studies on animal models have shown that noise exposure that does not lead to permanent threshold shift (PTS) can cause considerable damage around the synapses between inner hair cells (IHCs) and type-I afferent auditory nerve fibers (ANFs). Disruption of these synapses not only disables the innervated ANFs but also results in the slow degeneration of spiral ganglion neurons if the synapses are not reestablished. Such a loss of ANFs should result in signal coding deficits, which are exacerbated by the bias of the damage toward synapses connecting low-spontaneous-rate (SR) ANFs, which are known to be vital for signal coding in noisy background. As there is no PTS, these functional deficits cannot be detected using routine audiological evaluations and may be unknown to subjects who have them. Such functional deficits in hearing without changes in sensitivity are generally called “noise-induced hidden hearing loss (NIHHL).” Here, we provide a brief review to address several critical issues related to NIHHL: (1) the mechanism of noise induced synaptic damage, (2) reversibility of the synaptic damage, (3) the functional deficits as the nature of NIHHL in animal studies, (4) evidence of NIHHL in human subjects, and (5) peripheral and central contribution of NIHHL. PMID:27738526

  1. Long-term asymmetric hearing affects cochlear implantation outcomes differently in adults with pre- and postlingual hearing loss.

    PubMed

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

    2015-01-01

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

  2. Inhibitors of Histone Deacetylases Attenuate Noise-Induced Hearing Loss.

    PubMed

    Chen, Jun; Hill, Kayla; Sha, Su-Hua

    2016-08-01

    Loss of auditory sensory hair cells is the major pathological feature of noise-induced hearing loss (NIHL). Currently, no established clinical therapies for prevention or amelioration of NIHL are available. The absence of treatments is due to our lack of a comprehensive understanding of the molecular mechanisms underlying noise-induced damage. Our previous study indicates that epigenetic modification of histones alters hair cell survival. In this study, we investigated the effect of noise exposure on histone H3 lysine 9 acetylation (H3K9ac) in the inner ear of adult CBA/J mice and determined if inhibition of histone deacetylases by systemic administration of suberoylanilide hydroxamic acid (SAHA) could attenuate NIHL. Our results showed that H3K9ac was decreased in the nuclei of outer hair cells (OHCs) and marginal cells of the stria vascularis in the basal region after exposure to a traumatic noise paradigm known to induce permanent threshold shifts (PTS). Consistent with these results, levels of histone deacetylases 1, 2, and 3 (HDAC1, HDAC2 and HDAC3) were increased predominately in the nuclei of cochlear cells. Silencing of HDAC1, HDAC2, or HDAC3 with siRNA reduced the expression of the target HDAC in OHCs, but did not attenuate noise-induced PTS, whereas treatment with the pan-HDAC inhibitor SAHA, also named vorinostat, reduced OHC loss, and attenuated PTS. These findings suggest that histone acetylation is involved in the pathogenesis of noise-induced OHC death and hearing loss. Pharmacological targeting of histone deacetylases may afford a strategy for protection against NIHL.

  3. The Nature of Victimization among Youths with Hearing Loss in Substance Abuse Treatment

    ERIC Educational Resources Information Center

    Titus, Janet C.

    2010-01-01

    The author profiles the prevalence, severity, and characteristics of victimization among a group of youths with hearing loss presenting to substance abuse treatment. Intake data on 111 deaf and hard of hearing youths (42% female) were analyzed and compared with data from a weighted, gender-matched sample of hearing youths. After gender is…

  4. Coarticulation in Early Vocalizations by Children with Hearing Loss: A Locus Perspective

    ERIC Educational Resources Information Center

    Morrison, Helen Mccaffrey

    2012-01-01

    Locus equations derived from productions by three children with hearing loss revealed sensory and motor influences on anticipatory coarticulation. Participants who received auditory access to speech via hearing aids and cochlear implants at different ages (5-39 months) were recorded at approximately 6 and 12 months after hearing technology…

  5. Assessing Speech Intelligibility in Children with Hearing Loss: Toward Revitalizing a Valuable Clinical Tool

    ERIC Educational Resources Information Center

    Ertmer, David J.

    2011-01-01

    Background: Newborn hearing screening, early intervention programs, and advancements in cochlear implant and hearing aid technology have greatly increased opportunities for children with hearing loss to become intelligible talkers. Optimizing speech intelligibility requires that progress be monitored closely. Although direct assessment of…

  6. 78 FR 53702 - Computation of, and Rules Relating to, Medical Loss Ratio; Hearing Cancellation

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-08-30

    ... Internal Revenue Service 26 CFR Part 1 RIN 1545-BL05 Computation of, and Rules Relating to, Medical Loss Ratio; Hearing Cancellation AGENCY: Internal Revenue Service (IRS), Treasury. ACTION: Cancellation of a notice of public hearing on proposed rulemaking. SUMMARY: This document cancels a public hearing...

  7. Deafferentation-based pathophysiological differences in phantom sound: Tinnitus with and without hearing loss.

    PubMed

    Vanneste, Sven; De Ridder, Dirk

    2016-04-01

    Tinnitus has been considered an auditory phantom percept. Recently a theoretical multiphase compensation mechanism at a cortical level has been hypothesized linking auditory deafferentation to tinnitus. This Bayesian brain model predicts that two very different kinds of tinnitus should exist, depending on the amount of hearing loss: an auditory cortex related form of tinnitus not associated with hearing loss, and a (para)hippocampal form associated with hearing loss, in which the auditory cortex might be of little relevance. In order to verify this model, resting state source analyzed EEG recordings were made in 129 tinnitus patients, and correlated to the mean hearing loss, the range of the hearing loss and the hearing loss at the tinnitus frequency. Results demonstrate that tinnitus can be linked to 2 very different mechanisms. In patients with little or no hearing loss, the tinnitus seems to be more related to auditory cortex activity, but not to (para)hippocampal memory related activity, whereas in tinnitus patients with more severe hearing loss, tinnitus seems to be related to (para)hippocampal mechanisms. Furthermore hearing loss seems to drive the communication between the auditory cortex and the parahippocampus, as measured by functional and effective connectivity.

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

    PubMed

    Cushing, Sharon L; Papsin, Blake C

    2015-12-01

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

  9. [Active electronic hearing implants for middle and inner ear hearing loss--a new era in ear surgery. III: prospects for inner ear hearing loss].

    PubMed

    Zenner, H P; Leysieffer, H

    1997-10-01

    The perspectives for active hearing implants lie in the treatment of patients with sensorineural hearing loss (SNHL). The majority of patients with SNHL suffer from a cochlea amplifier (CA) failure which is discernible by a positive recruitment and loss of otoacoustic emissions (OAE). Therefore, the electronic implant is expected to partially replace functions of the CA. Thus, the implant is thought to function as a CAI (cochlea amplifier implant). An approved implant for routine use is not yet available. Clinical studies have thus far only used the high energy consuming (HEC), narrow-band, electromagnetic floating-mass transducer, as well as the Maniglia-HEC implant. The high energie consuming, yet broadband Canadian Fredrickson implant is soon to be used in humans. Of the piezoelectrical implants, a German CAI (Tübingen implant) at present consisting of a piezoelectrical transducer and a microphone has thus far been acutely implanted in first patient. It is a low energy consuming (LEC), broad-band implantable system for patients with sensorineural hearing loss. Routine surgical treatment of patients with sensorineural hearing loss with a CAI will only be achieved if complete implants (with transducer, microphones, batteries, and control unit) are made available. They combine distinct acoustic superiority with invisibility (end of stigmatization), an open ear canal, and hopefully, the end of feedback whistling. Among the implants mentioned, the German CAI is the only LEC implant. Its energy requirements are so low that with today's technologie implantable batteries (e.g., in pacemakers), the additional implantation of an energy carrier seems feasible. Since the implantable microphone is already available in the German system, the only essential part missing for a totally implantable CAI is the implantable control unit.

  10. [Diagnosis of sensorineural hearing loss by ipsilateral masking of brain stem auditory evoked potentials].

    PubMed

    Motta, G; Tranchino, G; Lombardo, N; Ripa, G; Motta, S

    1991-01-01

    The Authors show their original technique for the differential diagnosis of cochlear and retrocochlear hearing loss which employs ipsilateral masking of the A.B.R. in order to define the difference in V wave latency between a standard technique (clicks at 21 pps without noise) and a sensitized technique (clicks at 21 pps with noise at S/N of +40). This difference in latency has been named Sensitizing Latency Difference (SLD). Sixty normal subjects, 85 patients with cochlear hearing loss and 6 with retrocochlear hearing loss, were studied. The obtained data showed a SLD value: in normal hearing of: a) 0.31 msec. (+/- 0.14 SD) from 18 to 40 years old; b) 0.36 msec. (+/- 0.26 SD) from 41 to 60 years old; c) 0.48 msec. (+/- 0.21 SD) over 60 years old; in patients with cochlear hearing loss 0.12 msec. (+/- 0.14 SD) in 2 subjects affected by retrocochlear hearing loss a value superior to 1.5 msec. in the other 4 cases the V waves disappeared during recording of the sensitized test. A differential value of 1 msec. between cochlear and retrocochlear hearing loss was established by the Authors who with this value correctly identified all the patients with retrocochlear hearing loss and 97.8% of the subjects with cochlear hearing loss. An incorrect diagnosis of the hearing loss origin (false positives) was made in 2.2% of the patients with cochlear hearing loss. In conclusion, the Authors feel that the SLD evaluation, in consideration of its large clinical utility and of the high reliability of obtained data, is a particularly useful test in making a differential diagnosis between cochlear and retrocochlear hearing loss.

  11. Animal Model of Sensorineural Hearing Loss Associated with Lassa Virus Infection

    PubMed Central

    Yun, Nadezhda E.; Ronca, Shannon; Tamura, Atsushi; Koma, Takaaki; Seregin, Alexey V.; Dineley, Kelly T.; Miller, Milagros; Cook, Rebecca; Shimizu, Naoki; Walker, Aida G.; Smith, Jeanon N.; Fair, Joseph N.; Wauquier, Nadia; Bockarie, Bayon; Khan, Sheik Humarr

    2015-01-01

    ABSTRACT Approximately one-third of Lassa virus (LASV)-infected patients develop sensorineural hearing loss (SNHL) in the late stages of acute disease or in early convalescence. With 500,000 annual cases of Lassa fever (LF), LASV is a major cause of hearing loss in regions of West Africa where LF is endemic. To date, no animal models exist that depict the human pathology of LF with associated hearing loss. Here, we aimed to develop an animal model to study LASV-induced hearing loss using human isolates from a 2012 Sierra Leone outbreak. We have recently established a murine model for LF that closely mimics many features of human disease. In this model, LASV isolated from a lethal human case was highly virulent, while the virus isolated from a nonlethal case elicited mostly mild disease with moderate mortality. More importantly, both viruses were able to induce SNHL in surviving animals. However, utilization of the nonlethal, human LASV isolate allowed us to consistently produce large numbers of survivors with hearing loss. Surviving mice developed permanent hearing loss associated with mild damage to the cochlear hair cells and, strikingly, significant degeneration of the spiral ganglion cells of the auditory nerve. Therefore, the pathological changes in the inner ear of the mice with SNHL supported the phenotypic loss of hearing and provided further insights into the mechanistic cause of LF-associated hearing loss. IMPORTANCE Sensorineural hearing loss is a major complication for LF survivors. The development of a small-animal model of LASV infection that replicates hearing loss and the clinical and pathological features of LF will significantly increase knowledge of pathogenesis and vaccine studies. In addition, such a model will permit detailed characterization of the hearing loss mechanism and allow for the development of appropriate diagnostic approaches and medical care for LF patients with hearing impairment. PMID:26719273

  12. Perception of dissonance by people with normal hearing and sensorineural hearing loss.

    PubMed

    Tufts, Jennifer B; Molis, Michelle R; Leek, Marjorie R

    2005-08-01

    The purpose of this study was to determine whether the perceived sensory dissonance of pairs of pure tones (PT dyads) or pairs of harmonic complex tones (HC dyads) is altered due to sensorineural hearing loss. Four normal-hearing (NH) and four hearing-impaired (HI) listeners judged the sensory dissonance of PT dyads geometrically centered at 500 and 2000 Hz, and of HC dyads with fundamental frequencies geometrically centered at 500 Hz. The frequency separation of the members of the dyads varied from 0 Hz to just over an octave. In addition, frequency selectivity was assessed at 500 and 2000 Hz for each listener. Maximum dissonance was perceived at frequency separations smaller than the auditory filter bandwidth for both groups of listners, but maximum dissonance for HI listeners occurred at a greater proportion of their bandwidths at 500 Hz than at 2000 Hz. Further, their auditory filter bandwidths at 500 Hz were significantly wider than those of the NH listeners. For both the PT and HC dyads, curves displaying dissonance as a function of frequency separation were more compressed for the HI listeners, possibly reflecting less contrast between their perceptions of consonance and dissonance compared with the NH listeners.

  13. Perception of dissonance by people with normal hearing and sensorineural hearing loss

    NASA Astrophysics Data System (ADS)

    Tufts, Jennifer B.; Molis, Michelle R.; Leek, Marjorie R.

    2005-08-01

    The purpose of this study was to determine whether the perceived sensory dissonance of pairs of pure tones (PT dyads) or pairs of harmonic complex tones (HC dyads) is altered due to sensorineural hearing loss. Four normal-hearing (NH) and four hearing-impaired (HI) listeners judged the sensory dissonance of PT dyads geometrically centered at 500 and 2000 Hz, and of HC dyads with fundamental frequencies geometrically centered at 500 Hz. The frequency separation of the members of the dyads varied from 0 Hz to just over an octave. In addition, frequency selectivity was assessed at 500 and 2000 Hz for each listener. Maximum dissonance was perceived at frequency separations smaller than the auditory filter bandwidth for both groups of listners, but maximum dissonance for HI listeners occurred at a greater proportion of their bandwidths at 500 Hz than at 2000 Hz. Further, their auditory filter bandwidths at 500 Hz were significantly wider than those of the NH listeners. For both the PT and HC dyads, curves displaying dissonance as a function of frequency separation were more compressed for the HI listeners, possibly reflecting less contrast between their perceptions of consonance and dissonance compared with the NH listeners.

  14. Hearing outcome does not depend on the interval of intratympanic steroid administration in idiopathic sudden sensorineural hearing loss.

    PubMed

    Suzuki, Hideaki; Koizumi, Hiroki; Ohkubo, Jun-Ichi; Hohchi, Nobusuke; Ikezaki, Shoji; Kitamura, Takuro

    2016-10-01

    We studied the effect of intratympanic steroid administration with different intervals on hearing outcomes in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). The subjects were 197 consecutive patients (197 ears) with ISSNHL (hearing level ≥40 dB, interval between onset and treatment ≤30 days). They received systemic administration of prednisolone (100 mg followed by tapered doses) combined with intratympanic injection of dexamethasone (4 mg/ml). Intratympanic injection was performed once a week for 4 weeks in 105 patients (long-interval group), or 4 times in 1 week in 92 patients (short-interval group). The hearing outcomes were evaluated at two points of time: 1 week from the start of treatment, and 1-2 months after the completion of treatment when the hearing level reached a plateau. There was no significant difference in the cure rate, marked-recovery rate, recovery rate, hearing gain, hearing level, or percent hearing improvement between the long- and short-interval groups at either point of time. Multiple regression analysis also showed that the final hearing level did not depend on the interval of intratympanic steroid injection. These results indicate that the hearing outcome of ISSNHL does not improve even if the interval of intratympanic injection is shortened. This implies that a lower total number of intratympanic steroid injections may be as effective as the present protocol.

  15. Musicians change their tune: how hearing loss alters the neural code.

    PubMed

    Parbery-Clark, Alexandra; Anderson, Samira; Kraus, Nina

    2013-08-01

    Individuals with sensorineural hearing loss have difficulty understanding speech, especially in background noise. This deficit remains even when audibility is restored through amplification, suggesting that mechanisms beyond a reduction in peripheral sensitivity contribute to the perceptual difficulties associated with hearing loss. Given that normal-hearing musicians have enhanced auditory perceptual skills, including speech-in-noise perception, coupled with heightened subcortical responses to speech, we aimed to determine whether similar advantages could be observed in middle-aged adults with hearing loss. Results indicate that musicians with hearing loss, despite self-perceptions of average performance for understanding speech in noise, have a greater ability to hear in noise relative to nonmusicians. This is accompanied by more robust subcortical encoding of sound (e.g., stimulus-to-response correlations and response consistency) as well as more resilient neural responses to speech in the presence of background noise (e.g., neural timing). Musicians with hearing loss also demonstrate unique neural signatures of spectral encoding relative to nonmusicians: enhanced neural encoding of the speech-sound's fundamental frequency but not of its upper harmonics. This stands in contrast to previous outcomes in normal-hearing musicians, who have enhanced encoding of the harmonics but not the fundamental frequency. Taken together, our data suggest that although hearing loss modifies a musician's spectral encoding of speech, the musician advantage for perceiving speech in noise persists in a hearing-impaired population by adaptively strengthening underlying neural mechanisms for speech-in-noise perception.

  16. The effects of a hearing education program on recreational noise exposure, attitudes and beliefs toward noise, hearing loss, and hearing protector devices in young adults.

    PubMed

    Keppler, Hannah; Ingeborg, Dhooge; Sofie, Degeest; Bart, Vinck

    2015-01-01

    Excessive recreational noise exposure in young adults might result in noise-induced hearing loss (NIHL) and tinnitus. Inducing behavioral change in young adults is one of the aims of a hearing conservation program (HCP). The goal of the current study was to evaluate the effect of a hearing education program after 6 months in young adults in relation to knowledge regarding their individual hearing status. The results of a questionnaire regarding the weekly equivalent recreational noise exposure, attitudes and beliefs toward noise, and hearing loss and hearing protector devices (HPDs) were compared between both sessions. Seventy-eight young adults completed the questionnaire concerning recreational noise exposure, youth attitude to noise scale (YANS), and beliefs about hearing protection and hearing loss (BAHPHL). Their hearing status was evaluated based on admittance measures, audiometry, transient-evoked otoacoustic emissions (TEOAEs), and distortion-product otoacoustic emissions (DPOAEs). The main analysis consisted of a mixed model analysis of variance with dependent variables of either the noise exposure or the scores on (subscales of) YANS and BAHPHL. The independent variables were hearing status and session one versus session two. There was a significant decrease in recreational noise exposure and several (sub) scales of YANS and BAHPHL between both the sessions. This behavioral change resulted in a more frequent use of HPDs in 12% of the participants. However, the behavioral change was not completely related to the knowledge of young adults' individual hearing status. To prevent hearing damage in young people, investing in HCPs is necessary, apart from regulating sound levels and its compliance at various leisure-time activities. Also, the long-term effect of HCPs and their most cost-efficient repetition rates should be further investigated.

  17. The effects of a hearing education program on recreational noise exposure, attitudes and beliefs toward noise, hearing loss, and hearing protector devices in young adults

    PubMed Central

    Keppler, Hannah; Ingeborg, Dhooge; Sofie, Degeest; Bart, Vinck

    2015-01-01

    Excessive recreational noise exposure in young adults might result in noise-induced hearing loss (NIHL) and tinnitus. Inducing behavioral change in young adults is one of the aims of a hearing conservation program (HCP). The goal of the current study was to evaluate the effect of a hearing education program after 6 months in young adults in relation to knowledge regarding their individual hearing status. The results of a questionnaire regarding the weekly equivalent recreational noise exposure, attitudes and beliefs toward noise, and hearing loss and hearing protector devices (HPDs) were compared between both sessions. Seventy-eight young adults completed the questionnaire concerning recreational noise exposure, youth attitude to noise scale (YANS), and beliefs about hearing protection and hearing loss (BAHPHL). Their hearing status was evaluated based on admittance measures, audiometry, transient-evoked otoacoustic emissions (TEOAEs), and distortion-product otoacoustic emissions (DPOAEs). The main analysis consisted of a mixed model analysis of variance with dependent variables of either the noise exposure or the scores on (subscales of) YANS and BAHPHL. The independent variables were hearing status and session one versus session two. There was a significant decrease in recreational noise exposure and several (sub) scales of YANS and BAHPHL between both the sessions. This behavioral change resulted in a more frequent use of HPDs in 12% of the participants. However, the behavioral change was not completely related to the knowledge of young adults’ individual hearing status. To prevent hearing damage in young people, investing in HCPs is necessary, apart from regulating sound levels and its compliance at various leisure-time activities. Also, the long-term effect of HCPs and their most cost-efficient repetition rates should be further investigated. PMID:26356367

  18. Occupational Hearing Loss among Chinese Municipal Solid Waste Landfill Workers: A Cross-Sectional Study

    PubMed Central

    Liu, Yuewei; Wang, Haijiao; Weng, Shaofan; Su, Wenjin; Wang, Xin; Guo, Yanfei; Yu, Dan; Du, Lili; Zhou, Ting; Chen, Weihong; Shi, Tingming

    2015-01-01

    Background Occupational hearing loss is an increasingly prevalent occupational condition worldwide, and has been reported to occur in a wide range of workplaces; however, its prevalence among workers from municipal solid waste landfills (MSWLs) remains less clear. This study aimed to investigate the occupational hearing loss among Chinese MSWL workers. Methods A cross-sectional study of 247 workers from 4 Chinese MSWLs was conducted. Noise and total volatile organic compounds (TVOCs) levels at worksites were determined. We conducted hearing examinations to determine hearing thresholds. A worker was identified as having hearing loss if the mean threshold at 2000, 3000 and 4000 Hz in either ear was equal to or greater than 25 dB. Prevalence of occupational hearing loss was then evaluated. Using unconditional Logistic regression models, we estimated the odds ratios (ORs) of MSWL work associated with hearing loss. Results According to the job title for each worker, the study subjects were divided into 3 groups, including group 1 of 63 workers without MSWL occupational hazards exposure (control group), group 2 of 84 workers with a few or short-period MSWL occupational hazards exposure, and group 3 of 100 workers with continuous MSWL occupational hazards exposure. Both noise and TVOCs levels were significantly higher at worksites for group 3. Significantly poorer hearing thresholds at frequencies of 2000, 3000 and 4000 Hz were found in group 3, compared with that in group 1 and group 2. The overall prevalence rate of hearing loss was 23. 5%, with the highest in group 3 (36.0%). The OR of MSWL work associated with hearing loss was 3.39 (95% confidence interval [CI]: 1.28-8.96). Conclusion The results of this study suggest significantly higher prevalence of hearing loss among MSWL workers. Further studies are needed to explore possible exposure-response relationship between MSWL occupational hazards exposure and hearing loss. PMID:26042421

  19. In Vitro Models of GJB2-Related Hearing Loss Recapitulate Ca(2+) Transients via a Gap Junction Characteristic of Developing Cochlea.

    PubMed

    Fukunaga, Ichiro; Fujimoto, Ayumi; Hatakeyama, Kaori; Aoki, Toru; Nishikawa, Atena; Noda, Tetsuo; Minowa, Osamu; Kurebayashi, Nagomi; Ikeda, Katsuhisa; Kamiya, Kazusaku

    2016-12-13

    Mutation of the Gap Junction Beta 2 gene (GJB2) encoding connexin 26 (CX26) is the most frequent cause of hereditary deafness worldwide and accounts for up to 50% of non-syndromic sensorineural hearing loss cases in some populations. Therefore, cochlear CX26-gap junction plaque (GJP)-forming cells such as cochlear supporting cells are thought to be the most important therapeutic target for the treatment of hereditary deafness. The differentiation of pluripotent stem cells into cochlear CX26-GJP-forming cells has not been reported. Here, we detail the development of a novel strategy to differentiate induced pluripotent stem cells into functional CX26-GJP-forming cells that exhibit spontaneous ATP- and hemichannel-mediated Ca(2+) transients typical of the developing cochlea. Furthermore, these cells from CX26-deficient mice recapitulated the drastic disruption of GJPs, the primary pathology of GJB2-related hearing loss. These in vitro models should be useful for establishing inner-ear cell therapies and drug screening that target GJB2-related hearing loss.

  20. Usher syndrome: hearing loss, retinal degeneration and associated abnormalities

    PubMed Central

    Mathur, Pranav; Yang, Jun

    2014-01-01

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

  1. Screening infants for hearing loss--an economic evaluation.

    PubMed Central

    Brown, J

    1992-01-01

    STUDY OBJECTIVE--The aim was to carry out an economic evaluation of the programme implemented in one district health authority for the screening of infants for hearing loss. DESIGN--The approach taken was a cost-effectiveness analysis using the methodology of decision analysis to model the options appraised: (1) the conventional screening policy was for a health visitor and colleague to screen at 8-9 months, and at 10 months for each child to be seen again by a clinical medical officer for a developmental assessment plus hearing screen if necessary; (2) the alternative policy was for screening to take place at 10 months only if concern is expressed (or if there is a clinical indication) at the developmental assessment; the introduction of a "clue list" was considered; (3) the third option was no screening. MAIN RESULTS--The annual expected cost per unit output was pounds 20.57 for the conventional screening policy, between pounds 11.13 and pounds 11.23 for the alternative policy, and pounds 11.27 for the third option of no screening. Introducing the "clue list" under the alternative screening policy is likely to raise the cost per unit output, but the effects are uncertain. CONCLUSIONS--The results suggest that the alternative screening policy is more cost-effective than the conventional policy, but has little advantage over not screening at all. The effects of introducing a clue list need further investigation. PMID:1431705

  2. Hearing Loss in Syndromic Craniosynostoses: Introduction and Consideration of Mechanisms

    PubMed Central

    Agochukwu, Nneamaka B.; Solomon, Benjamin D.; Muenke, Maximilian

    2014-01-01

    Purpose There are a number of craniosynostosis syndromes with hearing loss—including Muenke, Apert, Pfeiffer, Crouzon, Beare-Stevenson, Crouzon with acanthosis nigricans, and Jackson-Weiss syndromes—that result from mutations in the fibroblast growth factor receptor (FGFR) genes. Studies of FGFRs and their ligands, fibroblast growth factors (FGFs), have revealed clues to the precise contribution of aberrant FGFR signaling to inner ear morphogenesis and the hearing loss encountered in craniosynostoses. The purpose of this article is to review basic studies of FGFRs with emphasis on their function and expression in the inner ear and surrounding structures. Method A Medline search was performed to find basic science articles regarding FGFR, their ligands, and their expression and relevant mouse models. Additional items searched included clinical descriptions and studies of individuals with FGFR-related craniosynostosis syndromes. Results The FGF signaling pathway is essential for the morphogensis and proper function of the inner ear and auditory sensory epithelium. Conclusion The variable auditory phenotypes seen in individuals with Muenke syndrome may have a genetic basis, likely due to multiple interacting factors in the genetic environment or modifying factors. Further analysis and studies of mouse models of Muenke syndrome, in particular, may provide clues to the specific effects of the defining mutation in FGFR3 in the inner ear not only at birth but also into adulthood. In particular, investigations into these models may give insight into the variable expression and incomplete penetrance of this phenotype. PMID:24686979

  3. Cochlear hearing loss in patients with Laron syndrome.

    PubMed

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

    2012-02-01

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

  4. Neural tracking of attended versus ignored speech is differentially affected by hearing loss.

    PubMed

    Petersen, Eline Borch; Wöstmann, Malte; Obleser, Jonas; Lunner, Thomas

    2017-01-01

    Hearing loss manifests as a reduced ability to understand speech, particularly in multitalker situations. In these situations, younger normal-hearing listeners' brains are known to track attended speech through phase-locking of neural activity to the slow-varying envelope of the speech. This study investigates how hearing loss, compensated by hearing aids, affects the neural tracking of the speech-onset envelope in elderly participants with varying degree of hearing loss (n = 27, 62-86 yr; hearing thresholds 11-73 dB hearing level). In an active listening task, a to-be-attended audiobook (signal) was presented either in quiet or against a competing to-be-ignored audiobook (noise) presented at three individualized signal-to-noise ratios (SNRs). The neural tracking of the to-be-attended and to-be-ignored speech was quantified through the cross-correlation of the electroencephalogram (EEG) and the temporal envelope of speech. We primarily investigated the effects of hearing loss and SNR on the neural envelope tracking. First, we found that elderly hearing-impaired listeners' neural responses reliably track the envelope of to-be-attended speech more than to-be-ignored speech. Second, hearing loss relates to the neural tracking of to-be-ignored speech, resulting in a weaker differential neural tracking of to-be-attended vs. to-be-ignored speech in listeners with worse hearing. Third, neural tracking of to-be-attended speech increased with decreasing background noise. Critically, the beneficial effect of reduced noise on neural speech tracking decreased with stronger hearing loss. In sum, our results show that a common sensorineural processing deficit, i.e., hearing loss, interacts with central attention mechanisms and reduces the differential tracking of attended and ignored speech.

  5. Training changes processing of speech cues in older adults with hearing loss

    PubMed Central

    Anderson, Samira; White-Schwoch, Travis; Choi, Hee Jae; Kraus, Nina

    2013-01-01

    Aging results in a loss of sensory function, and the effects of hearing impairment can be especially devastating due to reduced communication ability. Older adults with hearing loss report that speech, especially in noisy backgrounds, is uncomfortably loud yet unclear. Hearing loss results in an unbalanced neural representation of speech: the slowly-varying envelope is enhanced, dominating representation in the auditory pathway and perceptual salience at the cost of the rapidly-varying fine structure. We hypothesized that older adults with hearing loss can be trained to compensate for these changes in central auditory processing through directed attention to behaviorally-relevant speech sounds. To that end, we evaluated the effects of auditory-cognitive training in older adults (ages 55–79) with normal hearing and hearing loss. After training, the auditory training group with hearing loss experienced a reduction in the neural representation of the speech envelope presented in noise, approaching levels observed in normal hearing older adults. No changes were noted in the control group. Importantly, changes in speech processing were accompanied by improvements in speech perception. Thus, central processing deficits associated with hearing loss may be partially remediated with training, resulting in real-life benefits for everyday communication. PMID:24348347

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

    PubMed

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

    2015-02-01

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

  7. Effect of hearing aid release time and presentation level on speech perception in noise in elderly individuals with hearing loss.

    PubMed

    Pottackal Mathai, Jijo; Mohammed, Hasheem

    2017-02-01

    To investigate the effect of compression time settings and presentation levels on speech perception in noise for elderly individuals with hearing loss. To compare aided speech perception performance in these individuals with age-matched normal hearing subjects. Twenty (normal hearing) participants within the age range of 60-68 years and 20 (mild-to-moderate sensorineural hearing loss) in the age range of 60-70 years were randomly recruited for the study. In the former group, SNR-50 was determined using phonetically balanced sentences that were mixed with speech-shaped noise presented at the most comfortable level. In the SNHL group, aided SNR-50 was determined at three different presentation levels (40, 60, and 80 dB HL) after fitting binaural hearing aids that had different compression time settings (fast and slow). In the SNHL group, slow compression time settings showed significantly better SNR-50 compared to fast release time. In addition, the mean of SNR-50 in the SNHL group was comparable to normal hearing participants while using a slow release time. A hearing aid with slow compression time settings led to significantly better speech perception in noise, compared to that of a hearing aid that had fast compression time settings.

  8. Audiological evidence of therapeutic effect of steroid treatment in neuromyelitis optica with hearing loss.

    PubMed

    Takanashi, Yoshitaka; Misu, Tatsuro; Oda, Kazuha; Miyazaki, Hiromitsu; Yahata, Izumi; Hidaka, Hiroshi; Fujihara, Kazuo; Kawase, Tetsuaki; Kobayashi, Toshimitsu; Katori, Yukio

    2014-12-01

    Neuromyelitis optica (NMO) is an autoimmune astrocytopathy caused by anti-aquaporin 4 antibody. Only two patients with NMO have been reported presenting with hearing disorders to our knowledge. We recently treated a 40-year-old woman with NMO complaining of right hearing loss. Audiometry showed minimal asymmetry, but the auditory brainstem responses (ABR) were severely attenuated on the right. The attenuated ABR and her aural symptoms (hearing loss and fullness) improved after steroid treatment. The present case shows that the retrocochlear-type hearing loss may be associated with NMO.

  9. Acute Wernicke encephalopathy and sensorineural hearing loss complicating bariatric surgery.

    PubMed

    Jethava, Ashif; Dasanu, Constantin A

    2012-01-01

    Health-care professionals must be aware of the mandatory vitamin supplementation in patients status post bariatric surgery. A recent increase in the number of gastric bypass surgeries in US has been associated with a proportional increase in Wernicke encephalopathy reports. Subtle or atypical neurologic features are not uncommon. Our report is of a female patient with acute Wernicke encephalopathy accompanied by sensorineural hearing loss six weeks after bariatric surgery. The patient had only a partial recovery of her neurologic symptoms eightweeks after vigorous therapy for this condition. Symptomatic thiamine (vitamin B1) and vitamin B12 deficiencies are particularly concerning effects of bariatric procedures, as neurologic and cognitive deficits may be long lasting or even permanent despite aggressive replacement therapy.

  10. Next-Generation Sequencing in Genetic Hearing Loss

    PubMed Central

    Yan, Denise; Tekin, Mustafa; Blanton, Susan H.

    2013-01-01

    The advent of the $1000 genome has the potential to revolutionize the identification of genes and their mutations underlying genetic disorders. This is especially true for extremely heterogeneous Mendelian conditions such as deafness, where the mutation, and indeed the gene, may be private. The recent technological advances in target-enrichment methods and next generation sequencing offer a unique opportunity to break through the barriers of limitations imposed by gene arrays. These approaches now allow for the complete analysis of all known deafness-causing genes and will result in a new wave of discoveries of the remaining genes for Mendelian disorders. In this review, we describe commonly used genomic technologies as well as the application of these technologies to the genetic diagnosis of hearing loss (HL) and to the discovery of novel genes for syndromic and nonsyndromic HL. PMID:23738631

  11. Predicting the Perceptual Consequences of Hidden Hearing Loss

    PubMed Central

    2016-01-01

    Recent physiological studies in several rodent species have revealed that permanent damage can occur to the auditory system after exposure to a noise that produces only a temporary shift in absolute thresholds. The damage has been found to occur in the synapses between the cochlea’s inner hair cells and the auditory nerve, effectively severing part of the connection between the ear and the brain. This synaptopathy has been termed hidden hearing loss because its effects are not thought to be revealed in standard clinical, behavioral, or physiological measures of absolute threshold. It is currently unknown whether humans suffer from similar deficits after noise exposure. Even if synaptopathy occurs in humans, it remains unclear what the perceptual consequences might be or how they should best be measured. Here, we apply a simple theoretical model, taken from signal detection theory, to provide some predictions for what perceptual effects could be expected for a given loss of synapses. Predictions are made for a number of basic perceptual tasks, including tone detection in quiet and in noise, frequency discrimination, level discrimination, and binaural lateralization. The model’s predictions are in line with the empirical observations that a 50% loss of synapses leads to changes in threshold that are too small to be reliably measured. Overall, the model provides a simple initial quantitative framework for understanding and predicting the perceptual effects of synaptopathy in humans. PMID:28024462

  12. Psychological Assessment of Children with Multiple Handicaps Who Have Hearing Loss

    ERIC Educational Resources Information Center

    Schum, Robert

    2004-01-01

    This article discusses issues involved in psychological assessment of children with hearing loss who have additional disabilities or special needs. It provides recommendations for appropriate methods of assessment that accommodate the communication difficulties associated with hearing loss. This article includes assessment procedures for children…

  13. Identifying Minimal Hearing Loss and Managing Its Effects on Literacy Learning

    ERIC Educational Resources Information Center

    Werfel, Krystal L.; Hendricks, Alison Eisel

    2016-01-01

    It is well established that students who have moderate to profound hearing loss may experience difficulty in learning how to read and write and can benefit from modifications to the classroom environment and curriculum, however, minimal hearing loss often goes undiagnosed, and its negative impact on literacy acquisition is less widely known.…

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

    ERIC Educational Resources Information Center

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

    2010-01-01

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

  15. Authentic Membership: The Experiences of Two Students with Hearing Loss in Instrumental Music

    ERIC Educational Resources Information Center

    Burdett, John Paul

    2012-01-01

    The purpose of this study was to explore the experiences of two students with hearing loss participating in instrumental music. Four orienting questions guided the inquiry: (a) What factors contribute to these students' motivation to continue participating in instrumental music?; (b) How is hearing loss affecting each student's music participation…

  16. Profound Bilateral Sensorineural Hearing Loss in Nigerian Children: Any Shift in Etiology?

    ERIC Educational Resources Information Center

    Dunmade, A. D.; Segun-Busari, S.; Olajide, T. G.; Ologe, F. E.

    2007-01-01

    Deafness, profound hearing loss, is a global problem. However, the causes of, attitudes toward, and management options for deafness differ considerably from region to region. This study seeks to identify the present causes of profound sensorineural hearing loss in Nigeria, which in our environment is almost synonymous to a life sentence of silence…

  17. Vowel Production in 7- to 12-Month-Old Infants with Hearing Loss

    ERIC Educational Resources Information Center

    Nelson, Rebecca; Yoshinaga-Itano, Christine; Rothpletz, Ann; Sedey, Allison

    2007-01-01

    The purpose of this study was to examine vowel production in 7- to 12-month-old infants with hearing loss. Fifty-four infants were divided into three groups according to degree of hearing loss (mild-to-moderate, moderately severe-to-severe, profound), and their vocalizations were phonetically transcribed from 30-minute videotaped samples. These…

  18. The Role of Educators of the Deaf in the Early Identification of Hearing Loss.

    ERIC Educational Resources Information Center

    Arehart, Kathryn Hoberg; Yoshinaga-Itano, Christine

    1999-01-01

    Asserts that, as identification of hearing loss in infancy becomes increasingly common due to universal newborn screening, early interventionists will need to forge links to health-care providers, to have specialized training in deafness and hearing loss, and to have expertise in providing services to very young children and to children with mild…

  19. Professionals' Guidance about Spoken Language Multilingualism and Spoken Language Choice for Children with Hearing Loss

    ERIC Educational Resources Information Center

    Crowe, Kathryn; McLeod, Sharynne

    2016-01-01

    The purpose of this research was to investigate factors that influence professionals' guidance of parents of children with hearing loss regarding spoken language multilingualism and spoken language choice. Sixteen professionals who provide services to children and young people with hearing loss completed an online survey, rating the importance of…

  20. Characteristics of Youths with Hearing Loss Admitted to Substance Abuse Treatment

    ERIC Educational Resources Information Center

    Titus, Janet C.; Schiller, James A.; Guthmann, Debra

    2008-01-01

    The purpose of this study is to provide a profile of youths with hearing loss admitted to substance abuse treatment facilities. Intake data on 4,167 youths (28% female; 3% reporting a hearing loss) collected via the Global Appraisal of Individual Need-I assessment was used for the analyses. Information on demographics, environmental…

  1. Weighting of Acoustic Cues to a Manner Distinction by Children with and without Hearing Loss

    ERIC Educational Resources Information Center

    Nittrouer, Susan; Lowenstein, Joanna H.

    2015-01-01

    Purpose: Children must develop optimal perceptual weighting strategies for processing speech in their first language. Hearing loss can interfere with that development, especially if cochlear implants are required. The three goals of this study were to measure, for children with and without hearing loss: (a) cue weighting for a manner distinction,…

  2. The Cultural and Linguistic Diversity of 3-Year-Old Children with Hearing Loss

    ERIC Educational Resources Information Center

    Crowe, Kathryn; McLeod, Sharynne; Ching, Teresa Y. C.

    2012-01-01

    Understanding the cultural and linguistic diversity of young children with hearing loss informs the provision of assessment, habilitation, and education services to both children and their families. Data describing communication mode, oral language use, and demographic characteristics were collected for 406 children with hearing loss and their…

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

    ERIC Educational Resources Information Center

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

    2015-01-01

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

  4. "Minimal" High-Frequency Hearing Loss and School-Age Children: Speech Recognition in a Classroom.

    ERIC Educational Resources Information Center

    Johnson, Carole E.; And Others

    1997-01-01

    A study 12 children (ages 6-14) with minimal high frequency hearing loss, 12 typical children, and 12 typical young adults (ages 18-28) found the typical children had higher consonant identification scores in quiet than the children with hearing loss, but the performances did not differ in noise. Vowel identification scores did not differ in noise…

  5. Emergent Literacy Skills during Early Childhood in Children with Hearing Loss: Strengths and Weaknesses

    ERIC Educational Resources Information Center

    Easterbrooks, Susan R.; Lederberg, Amy R.; Miller, Elizabeth M.; Bergeron, Jessica P.; Connor, Carol McDonald

    2008-01-01

    The difficulties for students with hearing loss in gaining proficient literacy skills are well documented. However, studies describing the nature and variability of emergent literacy skills for students with hearing loss or the rate at which progress occurs at young ages are limited. We assessed emergent literacy skills and outcomes at the…

  6. Outlining the Concerns of Children Who Have Hearing Loss and Their Families

    ERIC Educational Resources Information Center

    Mathos, Kimberly K.; Broussard, Elsie R.

    2005-01-01

    Nationwide, there are some 1,055,000 young people under the age of 18 who have hearing loss according to statistics from the National Institute on Deafness and Other Communication Disorders (http://www.nidcd.nih.gov). Reported rates of psychiatric disorders for children who have hearing loss range from 15.4% to 54% (Hindley, 1997). Selected…

  7. Mastery Motivation and Expressive Language in Young Children with Hearing Loss.

    ERIC Educational Resources Information Center

    Pipp-Siegel, Sandra; Sedey, Allison L.; VanLeeuwen, Alison M.; Yoshinaga-Itano, Christine

    2003-01-01

    The relation between mastery motivation and expressive language was studied in 200 children (ages 7-67 months) with hearing loss. When demographic and hearing loss variables were entered into a regression equation, increased social/symbolic persistence was significantly related, and increased object-oriented persistence was marginally related, to…

  8. A case report of hearing loss post use of hydroxychloroquine in a HIV-infected patient

    PubMed Central

    2014-01-01

    Objective A case with reversible symmetrical sensorineural hearing loss following hydroxychloroquine therapy is described. Case summary A 57-year-old, human immunodeficiency virus (HIV) positive man was referred to the HIV clinic of Imam Khomeini Hospital, Tehran with chief complaint of bilateral slowly progressive hearing loss starting from two months ago. The man had history of rheumatoid arthritis diagnosed from 3 months ago and was administered hydroxychloroquine 200 mg and prednisolone 5 mg twice daily. Audiometry test showed moderate to severe neuronal hearing loss and reduced speech recognition in both ears of the patient. With suspicion of hydroxychloroquine-induced hearing loss, this drug was discontinued. After 2 months of hydroxychloroquine discontinuation, his audiometry findings were improved. Discussion A few cases of hydroxychloroquine-induced hearing loss have been reported. All of the cases were non-HIV positive individuals. Irreversible hearing loss was developed following long-term therapy with hydroxychloroquine. The present case was a HIV-positive man who developed hearing loss following short course (one month) hydroxychloroquine therapy and his problem was resolved following discontinuation of hydroxychloroquine and continuation of prednisolone. Conclusions Hydroxychloroquine-induced hearing loss may reversibly occur following short term therapy in HIV patients. PMID:24450358

  9. Combining Temporal-Envelope Cues across Channels: Effects of Age and Hearing Loss

    ERIC Educational Resources Information Center

    Souza, Pamela E.; Boike, Kumiko T.

    2006-01-01

    The goal of this study was to examine the ability to combine temporal-envelope information across frequency channels. Three areas were addressed: (a) the effects of hearing loss, (b) the effects of age and (c) whether such effects increase with the number of frequency channels. Twenty adults aged 23-80 years with hearing loss ranging from mild to…

  10. The Risk of Noise-Induced Hearing Loss During Simulated Dives in Canadian Forces Hyperbaric Facilities

    DTIC Science & Technology

    2012-10-01

    The risk of noise-induced hearing loss during simulated dives in Canadian Forces hyperbaric facilities Sharon M...2012-084 October 2012 The risk of noise-induced hearing loss during simulated dives in Canadian Forces hyperbaric ...transferred into the dive chamber of a hyperbaric facility. The mechanism is audible and sufficiently high in level in adjacent areas to warrant the

  11. Vocabulary development in children with hearing loss: the role of child, family, and educational variables.

    PubMed

    Coppens, Karien M; Tellings, Agnes; van der Veld, William; Schreuder, Robert; Verhoeven, Ludo

    2012-01-01

    In the present study we examined the effect of hearing status on reading vocabulary development. More specifically, we examined the change of lexical competence in children with hearing loss over grade 4-7 and the predictors of this change. Therefore, we used a multi-factor longitudinal design with multiple outcomes, measuring the reading vocabulary knowledge in children with hearing loss from grades 4 and 5, and of children without hearing loss from grade 4, for 3 years with two word tasks: a lexical decision task and a use decision task. With these tasks we measured word form recognition and (in)correct usage recognition, respectively. A GLM repeated measures procedure indicated that scores and growth rates on the two tasks were affected by hearing status. Moreover, with structural equation modeling we observed that the development of lexical competence in children with hearing loss is stable over time, and a child's lexical competence can be explained best by his or her lexical competence assessed on a previous measurement occasion. If you look back, differences in lexical competence among children with hearing loss stay unfortunately the same. Educational placement, use of sign language at home, intelligence, use of hearing devices, and onset of deafness can account for the differences among children with hearing loss.

  12. Association of skin color, race/ethnicity, and hearing loss among adults in the USA.

    PubMed

    Lin, Frank R; Maas, Paige; Chien, Wade; Carey, John P; Ferrucci, Luigi; Thorpe, Roland

    2012-02-01

    Epidemiologic studies of hearing loss in adults have demonstrated that the odds of hearing loss are substantially lower in black than in white individuals. The basis of this association is unknown. We hypothesized that skin pigmentation as a marker of melanocytic functioning mediates this observed association and that skin pigmentation is associated with hearing loss independent of race/ethnicity. We analyzed cross-sectional data from 1,258 adults (20-59 years) in the 2003-2004 cycle of the National Health and Nutritional Examination Survey who had assessment of Fitzpatrick skin type and pure-tone audiometric testing. Audiometric thresholds in the worse hearing ear were used to calculate speech- (0.5-4 kHz) and high-frequency (3-8 kHz) pure-tone averages (PTA). Regression models were stratified by Fitzpatrick skin type or race/ethnicity to examine the association of each factor with hearing loss independent of the other. Models were adjusted for potential confounders (demographic, medical, and noise exposure covariates). Among all participants, race/ethnicity was associated with hearing thresholds (black participants with the best hearing followed by Hispanics and then white individuals), but these associations were not significant in analyses stratified by skin color. In contrast, in race-stratified analyses, darker-skinned Hispanics had better hearing than lighter-skinned Hispanics by an average of -2.5 dB hearing level (HL; 95% CI, -4.8 to -0.2) and -3.1 dB HL (95% CI, -5.3 to -0.8) for speech and high-frequency PTA, respectively. Associations between skin color and hearing loss were not significant in white and black participants. Our results demonstrate that skin pigmentation is independently associated with hearing loss in Hispanics and suggest that skin pigmentation as a marker of melanocytic functioning may mediate the strong association observed between race/ethnicity and hearing loss.

  13. The clinical analysis of bilateral successive sudden sensorineural hearing loss.

    PubMed

    Wang, Yaowen; Zhang, Litao; Zhang, Jianhua; Zhang, Xuqun; Zhang, Weimin; Chen, Xing; Tang, Shixiong

    2016-11-01

    The objective of this study is to investigate the clinical characteristics of successive bilateral sudden sensorineural hearing loss (SSNHL) with an interval of more than 1 year to aid the evaluation and management of successive bilateral SSNHL (BSSNHL). 14 successive BSSNHL patients and 118 unilateral SSNHL patients with severe and profound hearing loss were reviewed retrospectively. Information about successive BSSNHL was collected included demographics, the intervals between the attacks of bilateral ears, the past medical history, inducing factors, accompanying symptoms, pure-tone tests, blood tests, b-ultrasound examinations of vertebral artery and carotid artery, and medical interventions. And the comparison of improvement rate was made between successive BSSNHL and unilateral SSNHL. SPSS 15.0 was used to analyze the data. In successive BSSNHL, there were six males and eight females; the average aged was 49.86 ± 15.45 years (20-73 years). The interval of the two attacks was 11.43 ± 12.07 years (1-50 years) on average. The onset of treatment was 18.86 ± 12.71 days. Tinnitus was seen in 100 % of the patients, followed by vertigo in 42.85 %, and ear fullness in 21.43 %. 3 of 14 patients described obvious inducing factor: tiredness. Five patients (35.71 %) had hypertension histories, three (21.43 %) had diabetes histories, two (14.29 %) had surgery histories, one (7.14 %) was with depression history, one (7.14 %) was with coronary heart disease history. 30 % (3/10) patients were with atherosclerotic plaque in carotid artery. 4 (28.57 %) patients were with high blood sugar. 8 patients (57.14 %) were with high blood lipids. Thyroid function tests were positive in 27.27 % (3/11) patients. No abnormality was found in antinuclear antibodies titer. The percentage of profound and severe hearing loss were 71.43 and 78.57 % in the recently affected ear and contralateral ear separately. The PTAs of the recently affected ear were 77.14 ± 27.12

  14. Noise-Induced Hearing Loss (NIHL): literature review with a focus on occupational medicine

    PubMed Central

    Metidieri, Mirella Melo; Rodrigues, Hugo Fernandes Santos; Filho, Francisco José Motta Barros de Oliveira; Ferraz, Daniela Pereira; Neto, Antonio Fausto de Almeida; Torres, Sandro

    2013-01-01

    Summary According to the Ministry of Health (2006), Noise-Induced Hearing Loss (NIHL) is hearing loss caused by prolonged exposure to noise. It is characterized as sensorineural hearing loss and is usually bilateral, irreversible, and progressive while the exposure to noise continues. A NIHL is a predictable and preventable disease with an epidemiologically relevant prevalence in urban communities. The hearing loss begins and predominates in the frequencies of 3, 4, and 6 kHz and eventually progresses to 8, 2, 1, 0.5, and 0.25 kHz. In Brazil, regulatory standard 15 limits the exposure to continuous noise to no more than 4 hours' exposure to 90 dBA and a maximum level of 85 dB for a full 8-hour working period. As NIHL is a preventable and predictable disease, preventive action by professionals may be able to change the prevalence of hearing loss in noisy environments. PMID:25992015

  15. Noise-Induced Hearing Loss (NIHL): literature review with a focus on occupational medicine.

    PubMed

    Metidieri, Mirella Melo; Rodrigues, Hugo Fernandes Santos; Filho, Francisco José Motta Barros de Oliveira; Ferraz, Daniela Pereira; Neto, Antonio Fausto de Almeida; Torres, Sandro

    2013-04-01

    According to the Ministry of Health (2006), Noise-Induced Hearing Loss (NIHL) is hearing loss caused by prolonged exposure to noise. It is characterized as sensorineural hearing loss and is usually bilateral, irreversible, and progressive while the exposure to noise continues. A NIHL is a predictable and preventable disease with an epidemiologically relevant prevalence in urban communities. The hearing loss begins and predominates in the frequencies of 3, 4, and 6 kHz and eventually progresses to 8, 2, 1, 0.5, and 0.25 kHz. In Brazil, regulatory standard 15 limits the exposure to continuous noise to no more than 4 hours' exposure to 90 dBA and a maximum level of 85 dB for a full 8-hour working period. As NIHL is a preventable and predictable disease, preventive action by professionals may be able to change the prevalence of hearing loss in noisy environments.

  16. Impact of a Child's Hearing Loss on the Family.

    ERIC Educational Resources Information Center

    Meadow-Orlans, Kathryn P.

    To examine the effects of hearing impairment on the family, questionnaires were completed by 358 mothers and 184 fathers of hearing impaired children enrolled in special education. Questionnaires measured three areas: (1) family stress; (2) communication with the hearing-impaired child; and (3) relationships with professionals and others outside…

  17. Bullying in Students with and without Hearing Loss

    ERIC Educational Resources Information Center

    Pinquart, Martin; Pfeiffer, Jens P.

    2015-01-01

    While bullying is a common phenomenon at schools in general, very few studies have addressed bullying in students with hearing impairment. This study assessed being bullied and bullying others in 181 adolescents from German schools for students who are deaf and hard of hearing, and in 259 hearing peers from regular schools. Students who are deaf…

  18. [Management of sudden neurosensory hearing loss in a Primary Care Centre].

    PubMed

    Muñoz-Proto, F; Carnevale, C; Bejarano-Panadés, N; Ferrán-de la Cierva, L; Mas-Mercant, S; Sarría-Echegaray, P

    2014-04-01

    Sudden hearing loss is a rapid loss of neurosensory hearing that may occur within hours or days in an apparently healthy patient. Its origins are variable and multifactorial. Most patients do not recover hearing if not treated, and some even develop cophosis (deafness) in the affected ear. It is an otological emergency, as early therapeutic management offers a better hearing prognosis. As there is limited knowledge on this condition, it may be underdiagnosed in Primary Health Care Centers. It should be suspected in patients with abrupt hearing loss or tinnitus. Sophisticated instruments are not required for its diagnosis, just a detailed history, basic otoscopy, and proper interpretation of the hearing test. In this way, an accurate diagnosis is achieved in most cases, which is confirmed by audiometry.

  19. Effects of Aging and Adult-Onset Hearing Loss on Cortical Auditory Regions

    PubMed Central

    Cardin, Velia

    2016-01-01

    Hearing loss is a common feature in human aging. It has been argued that dysfunctions in central processing are important contributing factors to hearing loss during older age. Aging also has well documented consequences for neural structure and function, but it is not clear how these effects interact with those that arise as a consequence of hearing loss. This paper reviews the effects of aging and adult-onset hearing loss in the structure and function of cortical auditory regions. The evidence reviewed suggests that aging and hearing loss result in atrophy of cortical auditory regions and stronger engagement of networks involved in the detection of salient events, adaptive control and re-allocation of attention. These cortical mechanisms are engaged during listening in effortful conditions in normal hearing individuals. Therefore, as a consequence of aging and hearing loss, all listening becomes effortful and cognitive load is constantly high, reducing the amount of available cognitive resources. This constant effortful listening and reduced cognitive spare capacity could be what accelerates cognitive decline in older adults with hearing loss. PMID:27242405

  20. The impact of hyperacusis and hearing loss on tinnitus perception in German teachers

    PubMed Central

    Meuer, Sandra P.; Hiller, Wolfgang

    2015-01-01

    Recent research indicates that a notable number of teachers are concerned with conditions of auditory impairment such as tinnitus, hyperacusis, and hearing loss. Studies focussing on characteristics and interdependencies of single hearing disorders (HD) are rare. This explorative study examines tinnitus, hyperacusis, hearing loss, and all possible combinations (tinnitus + hyperacusis; tinnitus + hearing loss; hyperacusis + hearing loss; tinnitus, hyperacusis + hearing loss) in German teachers. The impact of single HD on perceived distress, depending on the number and kind of comorbid HD, was of special interest. Information was collected via online survey and includes self-reported data as well as data from the Mini-Tinnitus Questionnaire (Mini-TQ). Results show that most of the 1468 participants (45%) suffered from two HD in different combinations, and the fewest (25%) were afflicted with only one HD. Considering the seven HD groups, most teachers (30%) suffered from all three HD. Across all groups, tinnitus was present in 1096, hyperacusis in 988, and hearing loss in 937 teachers. Multiple intergroup comparisons revealed that self-rated tinnitus-related distress rose significantly with the increasing number of HD. No significant differences were found for distress ratings of hyperacusis between the four groups including hyperacusis and between the four groups with hearing loss. In the Mini-TQ, groups including hyperacusis scored considerably higher than those excluding hyperacusis. The frequent prevalence of HD in German teachers points to a need of better noise prevention in German schools as one priority of occupational safety. PMID:26168948