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Sample records for nonsyndromic hearing impairment

  1. [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.

  2. 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.

  3. A new nonsyndromic X-linked sensorineural hearing impairment linked to Xp21.2

    SciTech Connect

    Lalwani, A.K.; Brister, J.R.; Fex, J.; Grundfast, K.M.; Pikus, A.T.; Ploplis, B.; San Agustin, T.; Skarka, H.; Wilcox, E.R.

    1994-10-01

    X-linked deafness is a rare cause of hereditary hearing impairment. We have identified a family with X-linked dominant sensorineural hearing impairment, characterized by incomplete penetrance and variable expressivity in carrier females, that is linked to the Xp21.2, which contains the Duchenne muscular dystrophy (DMD) locus. The auditory impairment in affected males was congenital, bilateral, profound, sensorineural, affecting all frequencies, and without evidence of radiographic abnormality of the temporal bone. Adult carrier females manifested bilateral, mild-to-moderate high-frequency sensorineural hearing impairment of delayed onset during adulthood. Eighteen commercially available polymorphic markers from the X chromosome, generating a 10-15-cM map, were initially used for identification of a candidate region. DXS997, located within the DMD gene, generated a two-point LOD score of 2.91 at {theta} = 0, with every carrier mother heterozygous at this locus. Recombination events at DXS992 (located within the DMD locus, 3{prime} to exon 50 of the dystrophin gene) and at DXS1068 (5{prime} to the brain promoter of the dystrophin gene) were observed. No recombination events were noted with the following markers within the DMD locus: 5{prime}DYS II, intron 44, DXS997, and intron 50. There was no clinical evidence of Duchenne or Becker muscular dystrophy in any family member. It is likely that this family represents a new locus on the X chromosome, which when mutated results in nonsyndromic sensorineural hearing loss and is distinct from the heterogeneous group of X-linked hearing losses that have been previously described. 57 refs., 6 figs., 1 tab.

  4. Genetic and clinical analysis of nonsyndromic hearing impairment in pediatric and adult cases

    PubMed Central

    Xing, J; Tian, Y; Tan, J; Zhao, H

    2016-01-01

    Abstract Previous studies have linked GJB2 gene and mitochondrial DNA (mtDNA) mutations to nonsyndromic hearing impairment (NSHI), but no study in China has yet investigated these mutations across all age groups. To fill the gap, this study ascertained 263 patients with NSHI between ages 2 months and 60 years and analyzed the presence of GJB2 gene and mtDNA A1555G/C1494T mutations by polymerase chain reaction (PCR) and DNA sequencing. A total of 20 types of mutations were detected for the GJB2 gene. The GJB2 gene and mtDNA A1555G/C1494T mutations were detected in 18.63 and 11.41% cases, respectively. At the first hospital visit, GJB2 gene mutations were detected in 5.97% of adult patients (>18 years) and 22.96% pediatric patients (<18 years) (χ2 =9.506, p = 0.002), and mtDNA A1555G/C1494T mutations were detected in 31.34% of adult patients and 4.59% of pediatric patients (χ2 = 35.359, p <0.001). When patients were classified by age at onset of deafness, significantly more (20.68%) pediatric patients had GJB2 gene mutations than did adult patients (0.0%) (χ2 = 4.685; p = 0.006). Mitochondrial DNA A1555G/C1494T mutations were detected in 15.38% of adult-onset and 8.86% pediatric-onset patients, respectively. Interestingly, most GJB2 gene mutation carriers experienced NSHI onset within the first year of life (65.31%), while mtDNA A1555G/C1494T mutation carriers experienced onset at any age. Therefore, GJB2 gene mutations appear to contribute to congenital deafness, while mtDNAA1555G/C1494T mutations contribute mainly to acquired deafness in Chinese individuals. Both newborn hearing screening and genetic testing are important to diagnose and treat deafness. PMID:27785406

  5. Genetics of Nonsyndromic Congenital Hearing Loss

    PubMed Central

    Egilmez, Oguz Kadir; Kalcioglu, M. Tayyar

    2016-01-01

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

  6. Mutations of the gene encoding otogelin are a cause of autosomal-recessive nonsyndromic moderate hearing impairment.

    PubMed

    Schraders, Margit; Ruiz-Palmero, Laura; Kalay, Ersan; Oostrik, Jaap; del Castillo, Francisco J; Sezgin, Orhan; Beynon, Andy J; Strom, Tim M; Pennings, Ronald J E; Zazo Seco, Celia; Oonk, Anne M M; Kunst, Henricus P M; Domínguez-Ruiz, María; García-Arumi, Ana M; del Campo, Miguel; Villamar, Manuela; Hoefsloot, Lies H; Moreno, Felipe; Admiraal, Ronald J C; del Castillo, Ignacio; Kremer, Hannie

    2012-11-02

    Already 40 genes have been identified for autosomal-recessive nonsyndromic hearing impairment (arNSHI); however, many more genes are still to be identified. In a Dutch family segregating arNSHI, homozygosity mapping revealed a 2.4 Mb homozygous region on chromosome 11 in p15.1-15.2, which partially overlapped with the previously described DFNB18 locus. However, no putative pathogenic variants were found in USH1C, the gene mutated in DFNB18 hearing impairment. The homozygous region contained 12 additional annotated genes including OTOG, the gene encoding otogelin, a component of the tectorial membrane. It is thought that otogelin contributes to the stability and strength of this membrane through interaction or stabilization of its constituent fibers. The murine orthologous gene was already known to cause hearing loss when defective. Analysis of OTOG in the Dutch family revealed a homozygous 1 bp deletion, c.5508delC, which leads to a shift in the reading frame and a premature stop codon, p.Ala1838ProfsX31. Further screening of 60 unrelated probands from Spanish arNSHI families detected compound heterozygous OTOG mutations in one family, c.6347C>T (p.Pro2116Leu) and c. 6559C>T (p.Arg2187X). The missense mutation p.Pro2116Leu affects a highly conserved residue in the fourth von Willebrand factor type D domain of otogelin. The subjects with OTOG mutations have a moderate hearing impairment, which can be associated with vestibular dysfunction. The flat to shallow "U" or slightly downsloping shaped audiograms closely resembled audiograms of individuals with recessive mutations in the gene encoding α-tectorin, another component of the tectorial membrane. This distinctive phenotype may represent a clue to orientate the molecular diagnosis.

  7. 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.

  8. 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.

  9. 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.

  10. 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

  11. 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.

  12. Hearing Impairment

    MedlinePlus

    ... up of invisible waves of energy, causes these vibrations. Hearing begins when sound waves that travel through ... When the eardrum vibrates, the ossicles amplify these vibrations and carry them to the inner ear. The ...

  13. Hearing Impairment

    MedlinePlus

    ... known as noise-induced hearing loss (NIHL) . Personal music players are among the chief culprits of NIHL ... exposure to high noise levels (such as loud music) over time can cause permanent damage to the ...

  14. 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 ...

  15. 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

  16. 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

  17. Evaluation of hearing impairment.

    PubMed

    Zadeh, M H; Selesnick, S H

    2001-01-01

    Hearing impairment is among the most common medical condition presenting to health care professionals. Ear anatomy, physiology, and pathology resulting in hearing loss are discussed. A systematic approach to evaluation, diagnosis, and treatment is presented.

  18. 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.

  19. Hearing or speech impairment - resources

    MedlinePlus

    Resources - hearing or speech impairment ... The following organizations are good resources for information on hearing impairment or speech impairment: Alexander Graham Bell Association for the Deaf and Hard of Hearing -- www.agbell. ...

  20. A Genomic and Protein-Protein Interaction Analyses of Nonsyndromic Hearing Impairment in Cameroon Using Targeted Genomic Enrichment and Massively Parallel Sequencing.

    PubMed

    Lebeko, Kamogelo; Manyisa, Noluthando; Chimusa, Emile R; Mulder, Nicola; Dandara, Collet; Wonkam, Ambroise

    2017-02-01

    Hearing impairment (HI) is one of the leading causes of disability in the world, impacting the social, economic, and psychological well-being of the affected individual. This is particularly true in sub-Saharan Africa, which carries one of the highest burdens of this condition. Despite this, there are limited data on the most prevalent genes or mutations that cause HI among sub-Saharan Africans. Next-generation technologies, such as targeted genomic enrichment and massively parallel sequencing, offer new promise in this context. This study reports, for the first time to the best of our knowledge, on the prevalence of novel mutations identified through a platform of 116 HI genes (OtoSCOPE(®)), among 82 African probands with HI. Only variants OTOF NM_194248.2:c.766-2A>G and MYO7A NM_000260.3:c.1996C>T, p.Arg666Stop were found in 3 (3.7%) and 5 (6.1%) patients, respectively. In addition and uniquely, the analysis of protein-protein interactions (PPI), through interrogation of gene subnetworks, using a custom script and two databases (Enrichr and PANTHER), and an algorithm in the igraph package of R, identified the enrichment of sensory perception and mechanical stimulus biological processes, and the most significant molecular functions of these variants pertained to binding or structural activity. Furthermore, 10 genes (MYO7A, MYO6, KCTD3, NUMA1, MYH9, KCNQ1, UBC, DIAPH1, PSMC2, and RDX) were identified as significant hubs within the subnetworks. Results reveal that the novel variants identified among familial cases of HI in Cameroon are not common, and PPI analysis has highlighted the role of 10 genes, potentially important in understanding HI genomics among Africans.

  1. 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.

  2. Assessment of Hearing Impaired Youth.

    ERIC Educational Resources Information Center

    Hicks, Doin E., Ed.; And Others

    1980-01-01

    The issue of Directions contains 11 articles on assessment of hearing impaired individuals. Entries have the following titles and authors: "Classroom Assessment Techniques for Hearing Impaired Students--A Literature Review" (B. McKee, M. Hausknecht); "Informal Assessment of Hearing Impaired Students In the Classroom" (B. Culhane, R. Hein);…

  3. Diagnostic application of targeted resequencing for familial nonsyndromic hearing loss.

    PubMed

    Choi, Byung Yoon; Park, Gibeom; Gim, Jungsoo; Kim, Ah Reum; Kim, Bong-Jik; Kim, Hyo-Sang; Park, Joo Hyun; Park, Taesung; Oh, Seung-Ha; Han, Kyu-Hee; Park, Woong-Yang

    2013-01-01

    Identification of causative genes for hereditary nonsyndromic hearing loss (NSHL) is important to decide treatment modalities and to counsel the patients. Due to the genetic heterogeneity in sensorineural genetic disorders, the high-throughput method can be adapted for the efficient diagnosis. To this end, we designed a new diagnostic pipeline to screen all the reported candidate genes for NSHL. For validation of the diagnostic pipeline, we focused upon familial NSHL cases that are most likely to be genetic, rather than to be infectious or environmental. Among the 32 familial NSHL cases, we were able to make a molecular genetic diagnosis from 12 probands (37.5%) in the first stage by their clinical features, characteristic inheritance pattern and further candidate gene sequencing of GJB2, SLC26A4, POU3F4 or mitochondrial DNA. Next we applied targeted resequencing on 80 NSHL genes in the remaining 20 probands. Each proband carried 4.8 variants that were not synonymous and had the occurring frequency of less than three among the 20 probands. These variants were then filtered out with the inheritance pattern of the family, allele frequency in normal hearing 80 control subjects, clinical features. Finally NSHL-causing candidate mutations were identified in 13(65%) of the 20 probands of multiplex families, bringing the total solve rate (or detection rate) in our familial cases to be 78.1% (25/32) Damaging mutations discovered by the targeted resequencing were distributed in nine genes such as WFS1, COCH, EYA4, MYO6, GJB3, COL11A2, OTOF, STRC and MYO3A, most of which were private. Despite the advent of whole genome and whole exome sequencing, we propose targeted resequencing and filtering strategy as a screening and diagnostic tool at least for familial NSHL to find mutations based upon its efficacy and cost-effectiveness.

  4. VOT and hearing impairment

    NASA Astrophysics Data System (ADS)

    Lane, Harlan; Perkell, Joseph

    2001-05-01

    When deafened adults recover some hearing after receiving a cochlear implant, numerous changes in their speech occur at both phonemic and suprasegmental levels. If a change toward normative values is observed for some phonemic parameter, it may be attributed to the restored hearing; however, it may be a by-product of a suprasegmental change. Consistent with results reported for speakers with normal hearing, Lane et al. [J. Acoust. Soc. Am. 98, 3096-3106 (1995)] observed in implant users that VOT varies approximately linearly with syllable duration. Therefore, in comparing pre- and postimplant measures of VOT in five speakers, each token's VOT was adjusted for the change in syllable duration of that token relative to the mean syllable duration in a baseline session (called VOTc). Preimplant, the deaf speakers characteristically uttered plosives with abnormally short VOTc. With some hearing restored, four of the five lengthened VOTc. Changes in voiced plosives' VOTc with restored hearing were correlated with changes in SPL. Some of the reliable VOTc increases that were not correlated with SPL may have been caused by auditory validation of an internal model for phoneme production. Recent studies of VOT in hearing-impaired speakers will be reviewed in this light. [Work supported by NIDCD, NIH.

  5. Autosomal dominant hearing loss resulting from p.R75Q mutation in the GJB2 gene: nonsyndromic presentation in a South Indian family.

    PubMed

    Pavithra, Amritkumar; Selvakumari, Mathiyalagan; Nityaa, Venkatesan; Sharanya, Narasimhan; Ramakrishnan, Rajagopalan; Narasimhan, Murali; Srisailapathy, C R Srikumari

    2015-01-01

    Mutations in the GJB2 gene encoding the gap junction protein Connexin 26 have been associated with autosomal recessive as well as dominant nonsyndromic hearing loss. Owing to the involvement of connexins in skin homeostasis, GJB2 mutations have also been associated with syndromic forms of hearing loss showing various skin manifestations. We report an assortatively mating hearing impaired family of south Indian origin with three affected members spread over two generations, having p.R75Q mutation in the GJB2 gene in the heterozygous condition. The inheritance pattern was autosomal dominant with mother and son being affected. Dermatological and histopathologic examinations showed absence of palmoplantar keratoderma. To the best of our knowledge, this is the first report from India on p.R75Q mutation in the GJB2 gene with nonsyndromic hearing loss.

  6. Conceptions of Hearing Impairment in Students.

    ERIC Educational Resources Information Center

    Alm, Irma; Ronnberg, Jerker

    1991-01-01

    Twelve students (ages 10-18) with hearing impairment and 12 normal hearing students were interviewed to determine attitudes about hearing impairment and self-concept. Results showed that school-integrated hearing-impaired students possessed a positive self-perception, but they shared normal hearing students' negative view of hearing-impaired…

  7. Rapid and Reliable Detection of Nonsyndromic Hearing Loss Mutations by Multicolor Melting Curve Analysis

    PubMed Central

    Wang, Xudong; Hong, Yongjun; Cai, Peihong; Tang, Ning; Chen, Ying; Yan, Tizhen; Liu, Yinghua; Huang, Qiuying; Li, Qingge

    2017-01-01

    Hearing loss is a common birth defect worldwide. The GJB2, SLC26A4, MT-RNR1 and MT-TS1 genes have been reported as major pathogenic genes in nonsyndromic hearing loss. Early genetic screening is recommended to minimize the incidence of hearing loss. We hereby described a multicolor melting curve analysis (MMCA)-based assay for simultaneous detection of 12 prevalent nonsyndromic hearing loss-related mutations. The three-reaction assay could process 30 samples within 2.5 h in a single run on a 96-well thermocycler. Allelic types of each mutation could be reproducibly obtained from 10 pg ~100 ng genomic DNA per reaction. For the mitochondrial mutations, 10% ~ 20% heteroplasmic mutations could be detected. A comparison study using 501 clinical samples showed that the MMCA assay had 100% concordance with both SNaPshot minisequencing and Sanger sequencing. We concluded that the MMCA assay is a rapid, convenient and cost-effective method for detecting the common mutations, and can be expectedly a reliable tool in preliminary screening of nonsyndromic hearing loss in the Chinese Han population. PMID:28225033

  8. Education for the Hearing Impaired (Auditorily Impaired).

    ERIC Educational Resources Information Center

    World Federation of the Deaf, Rome (Italy).

    Education for the hearing impaired is discussed in nine conference papers. J. N. Howarth describes "The Education of Deaf Children in Schools for Hearing Pupils in the United Kingdom" and A.I.Dyachkov of the U.S.S.R. outlines Didactical Principles of Educating the Deaf in the Light of their Rehabilitation Goal." Seven papers from…

  9. Visual impairment in the hearing impaired students

    PubMed Central

    Gogate, Parikshit; Rishikeshi, Nikhil; Mehata, Reshma; Ranade, Satish; Kharat, Jitesh; Deshpande, Madan

    2009-01-01

    Background: Ocular problems are more common in children with hearing problems than in normal children. Neglected visual impairment could aggravate educational and social disability. Aim: To detect and treat visual impairment, if any, in hearing-impaired children. Setting and Design: Observational, clinical case series of hearing-impaired children in schools providing special education. Materials and Methods: Hearing-impaired children in selected schools underwent detailed visual acuity testing, refraction, external ocular examination and fundoscopy. Ocular motility testing was also performed. Teachers were sensitized and trained to help in the assessment of visual acuity using Snellen's E charts. Refractive errors and squint were treated as per standard practice. Statistical Analysis: Excel software was used for data entry and SSPS for analysis. Results: The study involved 901 hearing-impaired students between four and 21 years of age, from 14 special education schools. A quarter of them (216/901, 24%) had ocular problems. Refractive errors were the most common morbidity 167(18.5%), but only 10 children were using appropriate spectacle correction at presentation. Fifty children had visual acuity less than 20/80 at presentation; after providing refractive correction, this number reduced to three children, all of whom were provided low-vision aids. Other common conditions included strabismus in 12 (1.3%) children, and retinal pigmentary dystrophy in five (0.6%) children. Conclusion: Ocular problems are common in hearing-impaired children. Screening for ocular problems should be made mandatory in hearing-impaired children, as they use their visual sense to compensate for the poor auditory sense. PMID:19861747

  10. 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

  11. Clinical and molecular analysis of a four-generation Chinese family with aminoglycoside-induced and nonsyndromic hearing loss associated with the mitochondrial 12S rRNA C1494T mutation

    SciTech Connect

    Wang Qiuju; Li Qingzhong; Han Dongyi . E-mail: hdy301@263.net; Zhao Yali; Zhao Lidong; Qian Yaping; Yuan Hu; Li Ronghua; Zhai Suoqiang; Young Wieyen . E-mail: ywy301@263.net; Guan Minxin . E-mail: min-xin.guan@chmcc.org

    2006-02-10

    We report here the clinical, genetic, and molecular characterization of a four-generation Chinese family with aminoglycoside-induced and nonsyndromic hearing loss. Five of nine matrilineal relatives had aminoglycoside-induced hearing loss. These matrilineal relatives exhibited variable severity and audiometric configuration of hearing impairment, despite sharing some common features: being bilateral and having sensorineural hearing impairment. Sequence analysis of mitochondrial DNA (mtDNA) in the pedigree identified 16 variants and the homoplasmic 12S rRNA C1494T mutation, which was associated with hearing loss in the other large Chinese family. In fact, the occurrence of the C1494T mutation in these genetically unrelated pedigrees affected by hearing impairment strongly indicated that this mutation is involved in the pathogenesis of aminoglycoside-induced and nonsyndromic hearing loss. However, incomplete penetrance of hearing loss indicated that the C1494T mutation itself is not sufficient to produce a clinical phenotype but requires the involvement of modifier factors for the phenotypic expression. Those mtDNA variants, showing no evolutional conservation, may not have a potential modifying role in the pathogenesis of the C1494T mutation. However, nuclear background seems to contribute to the phenotypic variability of matrilineal relatives in this family. Furthermore, aminoglycosides modulate the expressivity and penetrance of deafness associated with the C1494T mutation in this family.

  12. Etiological diagnosis of bilateral, sensorineural hearing impairment in a pediatric Greek population.

    PubMed

    Riga, M; Psarommatis, I; Lyra, Ch; Douniadakis, D; Tsakanikos, M; Neou, P; Apostolopoulos, N

    2005-04-01

    Early diagnosis, evaluation and treatment of childhood deafness are essential for a child's normal growth. Etiological diagnosis of hearing loss makes prevention, family scheduling and more effective therapy feasible goals. Etiological assessment of sensorineural deafness still remains difficult although recently with the progress of genetics it has become more efficient. In this retrospective study, the etiology of bilateral, sensorineural hearing loss with indication for hearing aids has been studied in 153 hearing impaired children. Etiological diagnosis was based on family and patient record, physical, audiological and laboratory examinations. Among the 94 children who completed the diagnostic protocol etiological groups revealed the following distribution: non-hereditary acquired hearing impairment was present in 36 children (38%) and hereditary was present in 44 (47%) children. The etiology remained unknown in 14 (15%) children. Non-syndromic autosomal dominant type accounted for 13 (29% of hereditary hearing loss) children, non-syndromic autosomal recessive type for 21 (48%) children and syndromic deafness for 10 (23%) children. Modern diagnostic methods, such as genetic testing, help diminish the number of cases with hearing impairment of unknown etiology, for the benefit of children who receive early and appropriate medical, audiologic, genetic and educational counseling based on the etiology of their hearing loss.

  13. 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.

  14. Identification of a novel pathogenic OTOF variant causative of nonsyndromic hearing loss with high frequency in the Ashkenazi Jewish population

    PubMed Central

    Fedick, Anastasia M; Jalas, Chaim; Swaroop, Ananya; Smouha, Eric E; Webb, Bryn D

    2016-01-01

    Mutations in the OTOF gene have previously been shown to cause nonsyndromic prelingual deafness (DFNB9, OMIM 601071) as well as auditory neuropathy/dys-synchrony. In this study, the OTOF NM_194248.2 c.5332G>T, p.Val1778Phe variant was identified in a large Ashkenazi Jewish family as the causative variant in four siblings with hearing loss. Our analysis reveals a carrier frequency of the OTOF c.5332G>T, p.Val1778Phe variant of 1.27% in the Ashkenazi Jewish population, suggesting that this variant may be a significant contributor to nonsyndromic sensorineural hearing loss and should be considered for inclusion in targeted hearing loss panels for this population. Of note, the degree of hearing loss associated with this phenotype ranged from mild to moderately severe, with two of the four siblings not known to have hearing loss until they were genotyped and underwent pure tone audiometry and auditory brainstem response testing. The phenotypic variability along with the auditory neuropathy/dys-synchrony, which allows for the production of otoacoustic emissions, supports that nonsyndromic hearing loss caused by OTOF mutations may be much more common in the Ashkenazi Jewish population than currently appreciated due to a lack of diagnosis. PMID:27621663

  15. Pegasus Project for the Hearing-Impaired.

    ERIC Educational Resources Information Center

    Krahe, Jane M.

    The Pegasus Project offered nine gifted hearing impaired students (11-15 years old) a summer enrichment experience with hearing peers. Courses included computer programming, literature, fine arts, physical and biological sciences, math enrichment, and sign language. All hearing impaired students also attended a special class on issues for the…

  16. 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

  17. 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

  18. 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.

  19. The Genetic Basis of Nonsyndromic Hearing Loss in Indian and Pakistani Populations.

    PubMed

    Yan, Denise; Kannan-Sundhari, Abhiraami; Vishwanath, Subramanian; Qing, Jie; Mittal, Rahul; Kameswaran, Mohan; Liu, Xue Zhong

    2015-09-01

    Deafness encompasses a series of etiologically heterogeneous disorders with mutations in more than 400 independent genes. However, several studies indicate that a large proportion of both syndromic and nonsyndromic forms of deafness in the racially diverse Indian and Pakistani populations are caused by defects in just a few genes. In these countries, there is a strong cultural preference for consanguineous marriage and an associated relatively high prevalence of genetic disorders. The current Indian population is approximately 1.2 billion and it is estimated that 30,000 infants are born with congenital sensorineural hearing loss (HL) each year. The estimated rate of profound bilateral HL is 1.6 per 1000 in Pakistan and 70% of this HL arises in consanguineous families. Knowledge of the genetic cause of deafness within a distinct population is important for accurate genetic counseling and early diagnosis for timely intervention and treatment options. Many sources and technologies are now available for the testing of hearing efficiency. Population-based screening has been proposed as one of the major strategies for translating genetic and genomic advances into population health gains. This review of the genetics of deafness in Indian and Pakistani populations deals with the major causes of deafness in these countries and prospectives for reducing the incidence of inherited deafness.

  20. Comprehensive Analysis of Deafness Genes in Families with Autosomal Recessive Nonsyndromic Hearing Loss

    PubMed Central

    Atik, Tahir; Onay, Huseyin; Aykut, Ayca; Bademci, Guney; Kirazli, Tayfun; Tekin, Mustafa; Ozkinay, Ferda

    2015-01-01

    Comprehensive genetic testing has the potential to become the standard of care for individuals with hearing loss. In this study, we investigated the genetic etiology of autosomal recessive nonsyndromic hearing loss (ARNSHL) in a Turkish cohort including individuals with cochlear implant, who had a pedigree suggestive of an autosomal recessive inheritance. A workflow including prescreening of GJB2 and a targeted next generation sequencing panel (Illumına TruSightTM Exome) covering 2761 genes that we briefly called as mendelian exome sequencing was used. This panel includes 102 deafness genes and a number of genes causing Mendelian disorders. Using this approach, we identified causative variants in 21 of 29 families. Three different GJB2 variants were present in seven families. Remaining 14 families had 15 different variants in other known NSHL genes (MYO7A, MYO15A, MARVELD2, TMIE, DFNB31, LOXHD1, GPSM2, TMC1, USH1G, CDH23). Of these variants, eight are novel. Mutation detection rate of our workflow is 72.4%, confirming the usefulness of targeted sequencing approach in NSHL. PMID:26561413

  1. Comprehensive Analysis of Deafness Genes in Families with Autosomal Recessive Nonsyndromic Hearing Loss.

    PubMed

    Atik, Tahir; Onay, Huseyin; Aykut, Ayca; Bademci, Guney; Kirazli, Tayfun; Tekin, Mustafa; Ozkinay, Ferda

    2015-01-01

    Comprehensive genetic testing has the potential to become the standard of care for individuals with hearing loss. In this study, we investigated the genetic etiology of autosomal recessive nonsyndromic hearing loss (ARNSHL) in a Turkish cohort including individuals with cochlear implant, who had a pedigree suggestive of an autosomal recessive inheritance. A workflow including prescreening of GJB2 and a targeted next generation sequencing panel (Illumına TruSightTM Exome) covering 2761 genes that we briefly called as mendelian exome sequencing was used. This panel includes 102 deafness genes and a number of genes causing Mendelian disorders. Using this approach, we identified causative variants in 21 of 29 families. Three different GJB2 variants were present in seven families. Remaining 14 families had 15 different variants in other known NSHL genes (MYO7A, MYO15A, MARVELD2, TMIE, DFNB31, LOXHD1, GPSM2, TMC1, USH1G, CDH23). Of these variants, eight are novel. Mutation detection rate of our workflow is 72.4%, confirming the usefulness of targeted sequencing approach in NSHL.

  2. The Genetic Basis of Nonsyndromic Hearing Loss in Indian and Pakistani Populations

    PubMed Central

    Yan, Denise; Kannan-Sundhari, Abhiraami; Vishwanath, Subramanian; Qing, Jie; Mittal, Rahul; Kameswaran, Mohan

    2015-01-01

    Deafness encompasses a series of etiologically heterogeneous disorders with mutations in more than 400 independent genes. However, several studies indicate that a large proportion of both syndromic and nonsyndromic forms of deafness in the racially diverse Indian and Pakistani populations are caused by defects in just a few genes. In these countries, there is a strong cultural preference for consanguineous marriage and an associated relatively high prevalence of genetic disorders. The current Indian population is approximately 1.2 billion and it is estimated that 30,000 infants are born with congenital sensorineural hearing loss (HL) each year. The estimated rate of profound bilateral HL is 1.6 per 1000 in Pakistan and 70% of this HL arises in consanguineous families. Knowledge of the genetic cause of deafness within a distinct population is important for accurate genetic counseling and early diagnosis for timely intervention and treatment options. Many sources and technologies are now available for the testing of hearing efficiency. Population-based screening has been proposed as one of the major strategies for translating genetic and genomic advances into population health gains. This review of the genetics of deafness in Indian and Pakistani populations deals with the major causes of deafness in these countries and prospectives for reducing the incidence of inherited deafness. PMID:26186295

  3. Maternally inherited hearing impairment in a family with the mitochondrial DNA A7445G mutation.

    PubMed

    Hutchin, T P; Lench, N J; Arbuzova, S; Markham, A F; Mueller, R F

    2001-01-01

    Despite the increasing number of reports of families with hearing impairment and mitochondrial DNA (mtDNA) mutations, the frequency of these mutations as causes of non-syndromic sensorineural hearing impairment (NSSHI) remains unknown. Mutations such as A1555G, A7445G and 7472insC have been found in several unrelated families implying they are more frequent than initially thought. We describe a family with NSSHI due to the presence of the homoplasmic mtDNA A7445G mutation in the tRNASer(UCN) gene. This is the fourth such family described with this mutation, all of different genetic backgrounds. Our study also demonstrates the difficulties sometimes encountered in establishing mitochondrial inheritance of hearing impairment in some families.

  4. Rare compound heterozygosity involving dominant and recessive mutations of GJB2 gene in an assortative mating hearing impaired Indian family.

    PubMed

    Pavithra, Amritkumar; Chandru, Jayasankaran; Jeffrey, Justin Margret; Karthikeyen, N P; Srisailapathy, C R Srikumari

    2017-01-01

    Connexin 26 (Cx-26), a gap junction protein coded by GJB2 gene, plays a very important role in recycling of potassium ions, one of the vital steps in the mechanotransduction process of hearing. Mutations in the GJB2 gene have been associated with both autosomal recessive as well as dominant nonsyndromic hearing loss. As Cx-26 is linked with skin homeostasis, mutations in this gene are sometimes associated with syndromic forms of hearing loss showing skin anomalies. We report here a non consanguineous assortatively mating hearing impaired family with one of the hearing impaired partners, their hearing impaired sibling and hearing impaired offspring showing compound heterozygosity in the GJB2 gene, involving a dominant mutation p.R184Q and two recessive mutations p.Q124X and c.IVS 1+1G>A in a unique triallelic combination. To the best of our knowledge, this is the first report from India on p.R184Q mutation in the GJB2 gene associated with rare compound heterozygosity showing nonsyndromic presentation.

  5. Babbling Development of Hearing-Impaired and Normally Hearing Subjects.

    ERIC Educational Resources Information Center

    Stoel-Gammon, Carol; Otomo, Kiyoshi

    1986-01-01

    Phonetic transcriptions of babbling samples from 11 normally hearing subjects, age 4-18 months, were compared with samples for 11 hearing-impaired students, age 4-28 months. Findings suggested both qualitative and quantitative differences in the babbling of the two groups. (Author/CL)

  6. Prevalence of GBJ2 mutations in patients with severe to profound congenital nonsyndromic sensorineural hearing loss in Bulgarian population.

    PubMed

    Popova, Diana P; Kaneva, Radka; Varbanova, Sonya; Popov, Todor M

    2012-06-01

    Objective of the study is to assess the prevalence of Connexin 26 (GJB2) mutation in patients with congenital nonsyndromic sensorineural hearing loss in Bulgarian population. Study design is done prospectively. Patient inclusion criteria for this study were diagnosis of congenital nonsyndromic hearing loss, and absence of potential sibling relationships between patients included in the study (anamnestic pedigree for at least three generations). Patients were excluded from the study group if one of the following conditions were present: secondary hearing loss (cytomegalovirus, rubella, meningo-encephalitis, mastoiditis, other infections, posterior fossa tumors, etc.), exposure to drugs or other prenatal or perinatal etiology of deafness, and congenital syndromic hearing loss. Genomic DNA samples from whole blood were tested with sequence analysis for mutations in the coding region of the GJB2. Results state that 51 patients were analyzed for GJB2 mutations. Twenty of the patients (39%) with mutant alleles were homozygous for the c.35delG mutation (c.35delG/c.35delG) and four patients (8%) presented as heterozygotes (c.35delG/WT). In one patient, who carried a heterozygous mutation c.35delG, a second mutation was found-312del114. Additionally, in two other patients were discovered the mutations Trp24X (W24X) and, respectively, Arg127His(R127H), both in heterozygous states. From the whole study group there was only one patient with compound heterozygous genotype-p.Leu90Pro(L90P)/p.Ile121Asn. The latter one has never been reported in the literature so far. In conclusion, this study determines the importance of connexin 26 mutations in Bulgarian children with severe to profound congenital nonsyndromic sensorineural hearing loss, the prevalence of the different mutation variants and their relationship with the ethnical background of the patients. In addition, we report for the first time a novel mutation in the GJB2 gene.

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

    SciTech Connect

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

    2006-04-14

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

  8. Classroom Teachers' Impressions of Hearing Impaired and Deaf Children.

    ERIC Educational Resources Information Center

    Blood, Ingrid M.; Blood, Gordon W.

    1982-01-01

    Classroom teachers rated achievement and appearance characteristics of nine school-age hearing-impaired and deaf children on a semantic differential. A significant difference between normally hearing and hearing-impaired subjects was noted. Hearing aids and the speech of the hearing-impaired and deaf subjects elicit more negative responses.…

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

    PubMed Central

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

    2016-01-01

    Hearing loss is a common sensory disorder, and at least 50% of cases are due to a genetic etiology. Although hundreds of genes have been reported to be associated with nonsyndromic hearing loss, GJB2, SLC26A4, and mtDNA12SrRNA are the major contributors. However, the mutation spectrum of these common deafness genes varies among different ethnic groups. The present work summarized mutations in these three genes and their prevalence in 339 patients with nonsyndromic hearing loss at three different special education schools and one children's hospital in Linyi, China. A new multiplex genetic screening system “SNPscan assay” was employed to detect a total of 115 mutations of the above three genes. Finally, 48.67% of the patients were identified with hereditary hearing loss caused by mutations in GJB2, SLC26A4, and mtDNA12SrRNA. The carrying rate of mutations in the three genes was 37.76%, 19.75%, and 4.72%, respectively. This mutation profile in our study is distinct from other parts of China, with high mutation rate of GJB2 suggesting a unique mutation spectrum in this area. PMID:27247933

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

    PubMed

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

    2016-01-01

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

  11. Comprehensive Hearing Impaired Reception Program; Spring, 1975.

    ERIC Educational Resources Information Center

    Oxman, Wendy G.

    This document presents a description and evaluation of the Comprehensive Hearing Impaired Reception Program (CHIRP). This program was designed to improve effective communication skills for hearing handicapped students whose native language was not English, and whose language deficiencies prevented them from effective participation in the learning…

  12. Loss-of-Function Mutations of ILDR1 Cause Autosomal-Recessive Hearing Impairment DFNB42

    PubMed Central

    Borck, Guntram; Rehman, Atteeq Ur; Lee, Kwanghyuk; Pogoda, Hans-Martin; Kakar, Naseebullah; von Ameln, Simon; Grillet, Nicolas; Hildebrand, Michael S.; Ahmed, Zubair M.; Nürnberg, Gudrun; Ansar, Muhammad; Basit, Sulman; Javed, Qamar; Morell, Robert J.; Nasreen, Nabilah; Shearer, A. Eliot; Ahmad, Adeel; Kahrizi, Kimia; Shaikh, Rehan S.; Ali, Rana A.; Khan, Shaheen N.; Goebel, Ingrid; Meyer, Nicole C.; Kimberling, William J.; Webster, Jennifer A.; Stephan, Dietrich A.; Schiller, Martin R.; Bahlo, Melanie; Najmabadi, Hossein; Gillespie, Peter G.; Nürnberg, Peter; Wollnik, Bernd; Riazuddin, Saima; Smith, Richard J.H.; Ahmad, Wasim; Müller, Ulrich; Hammerschmidt, Matthias; Friedman, Thomas B.; Riazuddin, Sheikh; Leal, Suzanne M.; Ahmad, Jamil; Kubisch, Christian

    2011-01-01

    By using homozygosity mapping in a consanguineous Pakistani family, we detected linkage of nonsyndromic hearing loss to a 7.6 Mb region on chromosome 3q13.31-q21.1 within the previously reported DFNB42 locus. Subsequent candidate gene sequencing identified a homozygous nonsense mutation (c.1135G>T [p.Glu379X]) in ILDR1 as the cause of hearing impairment. By analyzing additional consanguineous families with homozygosity at this locus, we detected ILDR1 mutations in the affected individuals of 10 more families from Pakistan and Iran. The identified ILDR1 variants include missense, nonsense, frameshift, and splice-site mutations as well as a start codon mutation in the family that originally defined the DFNB42 locus. ILDR1 encodes the evolutionarily conserved immunoglobulin-like domain containing receptor 1, a putative transmembrane receptor of unknown function. In situ hybridization detected expression of Ildr1, the murine ortholog, early in development in the vestibule and in hair cells and supporting cells of the cochlea. Expression in hair cell- and supporting cell-containing neurosensory organs is conserved in the zebrafish, in which the ildr1 ortholog is prominently expressed in the developing ear and neuromasts of the lateral line. These data identify loss-of-function mutations of ILDR1, a gene with a conserved expression pattern pointing to a conserved function in hearing in vertebrates, as underlying nonsyndromic prelingual sensorineural hearing impairment. PMID:21255762

  13. Development of Communicative Function in Young Hearing-Impaired and Normally Hearing Children.

    ERIC Educational Resources Information Center

    Nicholas, Johanna G.; And Others

    1994-01-01

    This study found that, although normally hearing children produced more communicative acts than 9 agemates (age 14-34 months) with severe hearing impairments, the hearing-impaired children produced more than hearing children matched for verbal language age. Results reveal that preverbal hearing-impaired children make significant strides that can…

  14. 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).

  15. Noise-induced hearing impairment and handicap

    NASA Technical Reports Server (NTRS)

    1984-01-01

    A permanent, noise-induced hearing loss has doubly harmful effect on speech communications. First, the elevation in the threshold of hearing means that many speech sounds are too weak to be heard, and second, very intense speech sounds may appear to be distorted. The whole question of the impact of noise-induced hearing loss upon the impairments and handicaps experienced by people with such hearing losses was somewhat controversial partly because of the economic aspects of related practical noise control and workmen's compensation.

  16. 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.

  17. Acquisition of spoken and signed English by hearing-impaired children of hearing-impaired or hearing parents.

    PubMed

    Geers, A E; Schick, B

    1988-05-01

    This study examines the degree to which hearing-impaired children of hearing-impaired parents (HIP) demonstrate an advantage in their acquisition of signed and spoken English over hearing-impaired children of hearing parents (HP). A subset from the normative sample of the Grammatical Analysis of Elicited Language, 50 HIP children and 50 HP children, were matched in terms of their educational program, hearing level, and age. Results indicate that both groups had comparably poor expressive English language ability at 5 and 6 years of age. However, at age 7 and 8 HIP children demonstrated a significant linguistic advantage in both their spoken and signed English over HP children. Because the production of English by HIP children closely resembled that of orally educated hearing-impaired children of hearing parents, consistent language stimulation throughout the child's early years may be a critical factor in the development of English, regardless of the language or mode of expression.

  18. 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.

  19. 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

  20. 38 CFR 4.86 - Exceptional patterns of hearing impairment.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... the Roman numeral designation for hearing impairment from either Table VI or Table VIa, whichever... determine the Roman numeral designation for hearing impairment from either Table VI or Table VIa,...

  1. 38 CFR 4.86 - Exceptional patterns of hearing impairment.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... the Roman numeral designation for hearing impairment from either Table VI or Table VIa, whichever... determine the Roman numeral designation for hearing impairment from either Table VI or Table VIa,...

  2. 38 CFR 3.385 - Disability due to impaired hearing.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... Considerations Relative to Specific Diseases § 3.385 Disability due to impaired hearing. For the purposes of applying the laws administered by VA, impaired hearing will be considered to be a disability when...

  3. A Novel Splice-Site Mutation in the GJB2 Gene Causing Mild Postlingual Hearing Impairment

    PubMed Central

    Gandía, Marta; del Castillo, Francisco J.; Rodríguez-Álvarez, Francisco J.; Garrido, Gema; Villamar, Manuela; Calderón, Manuela; Moreno-Pelayo, Miguel A.; Moreno, Felipe; del Castillo, Ignacio

    2013-01-01

    The DFNB1 subtype of autosomal recessive, nonsyndromic hearing impairment, caused by mutations affecting the GJB2 (connection-26) gene, is highly prevalent in most populations worldwide. DFNB1 hearing impairment is mostly severe or profound and usually appears before the acquisition of speech (prelingual onset), though a small number of hypomorphic missense mutations result in mild or moderate deafness of postlingual onset. We identified a novel GJB2 splice-site mutation, c. -22-2A>C, in three siblings with mild postlingual hearing impairment that were compound heterozygous for c. -22-2A>C and c.35delG. Reverse transcriptase-PCR experiments performed on total RNA extracted from saliva samples from one of these siblings confirmed that c. -22-2A>C abolished the acceptor splice site of the single GJB2 intron, resulting in the absence of normally processed transcripts from this allele. However, we did isolate transcripts from the c. -22-2A>C allele that keep an intact GJB2 coding region and that were generated by use of an alternative acceptor splice site previously unknown. The residual expression of wild-type connection-26 encoded by these transcripts probably underlies the mild severity and late onset of the hearing impairment of these subjects. PMID:24039984

  4. Initiations of social interactions by young hearing impaired preschoolers.

    PubMed

    Weisel, Amatzia; Most, Tova; Efron, Clara

    2005-01-01

    This study examined strategies for initiating social interactions with peers, among 4 children with hearing impairment, aged 33 to 36 months, attending a special early education center or a regular kindergarten. The study investigated initiation type (related to partner's hearing status) and rates of initiation success/failure vis-a-vis hearing and deaf partners. Results revealed (a) more initiations in the regular program than in the special program; (b) in the special program, much more successful initiations toward children with hearing impairment than toward hearing children; (c) vocalization as the most frequent strategy used with both hearing and hearing-impaired partners; and (d) referential decisions about their initiations even among young children with hearing impairment (made by changing frequencies of various strategies according to partner's hearing status). The discussion addressed implications regarding integration of children with hearing impairment into regular educational settings.

  5. Evaluating Motor Profiles of the Hearing Impaired.

    ERIC Educational Resources Information Center

    Brunt, Denis; Dearmond, Dorothy A.

    1981-01-01

    A test for the evaluation of the motor ability of the hearing impaired is described, and illustrations of selected sign language directions used in its administration are presented. Among the skills assessed are running speed and agility, balance, and coordination. (JN)

  6. Communication Methods for the Hearing Impaired.

    ERIC Educational Resources Information Center

    World Federation of the Deaf, Rome (Italy).

    Communication methods for the hearing impaired are discussed in 12 conference papers. Papers from the United States are "Adjustment through Oralism" by G. Fellendorf, "Prospectus of Patterning" (a method of teaching speech to deaf children) by M.S. Buckler, and "Visual Monitoring of Speech by the Deaf" by W.…

  7. The Very Young Hearing-Impaired Child.

    ERIC Educational Resources Information Center

    World Federation of the Deaf, Rome (Italy).

    Five conference papers are presented on deaf preschool children and infants. "The Very Young Hearing-Impaired Child" by G.M. Harris of Canada; "The Organisation and Methods of Educational Work for Deaf Children at the Preschool Age" by K. Lundstrom of Sweden; "Speech Formation in the Young Deaf Child" by B.…

  8. Predicting Language Performance in Hearing Impaired Children.

    ERIC Educational Resources Information Center

    Monsees, Edna K.

    The 2-year study evaluated the language performance of 69 hearing impaired, preschool children born following the rubella epidemic of the early 1960's in order to develop an instrument for objectively assessing language achievement and a predictive index of language achievement. Two language rating scales were developed which were tied to the…

  9. Study Guide for TCT in Hearing Impaired.

    ERIC Educational Resources Information Center

    McCarthy, Patricia S.

    This study guide was specifically designed for individuals preparing to take the Georgia Teacher Certification Test (TCT) in hearing impaired. The test covers four subareas: (1) anatomy, physiology, and physics of sound; (2) audiological interpretation, etiology, and identification; (3) amplification, language, and communications; and (4) other…

  10. New Program Builds Bridges for Hearing Impaired.

    ERIC Educational Resources Information Center

    Johnson, Jeannie

    1998-01-01

    Describes a new approach to teaching English-as-a-Second-Language to New Mexico elementary school students who have hearing impairments and whose dominant language is American Sign Language (ASL). These students must first acquire ASL as a bridge to learning English. Then, using the bilingual approach, they are able to focus on English literacy.…

  11. Computers for the Hearing-Impaired.

    ERIC Educational Resources Information Center

    Auslander, Rudy

    1983-01-01

    Discusses use of microcomputers with the hearing-impaired, focusing on use of Telecommunication Devices for the Deaf (TDD) with a TRS-80 microcomputer. A special modem which handles the TDD's five-level Baudot code and the computer's eight-level ASCII code is needed. (JN)

  12. Psychological Assessment of Hearing Impaired Children.

    ERIC Educational Resources Information Center

    Sullivan, Patricia M.; Vernon, McCay

    1979-01-01

    An ideal assessment of hearing-impaired children includes: case history; medical examination; tests (of intelligence, personality, achievement, communication/language skills, and aptitude); neuropsychological assessment to identify learning strengths and weaknesses; and multidisciplinary team staffing and parent-teacher conferences. Twenty-eight…

  13. A POU3F4 Mutation Causes Nonsyndromic Hearing Loss in a Chinese X-linked Recessive Family

    PubMed Central

    Du, Wan; Han, Ming-Kun; Wang, Da-Yong; Han, Bing; Zong, Liang; Lan, Lan; Yang, Ju; Shen, Qi; Xie, Lin-Yi; Yu, Lan; Guan, Jing; Wang, Qiu-Ju

    2017-01-01

    Background: The molecular genetic research showed the association between X-linked hearing loss and mutations in POU3F4. This research aimed to identify a POU3F4 mutation in a nonsyndromic X-linked recessive hearing loss family. Methods: A series of clinical evaluations including medical history, otologic examinations, family history, audiologic testing, and a high-resolution computed tomography scan were performed for each patient. Bidirectional sequencing was carried out for all polymerase chain reaction products of the samples. Moreover, 834 controls with normal hearing were also tested. Results: The pedigree showed X-linkage recessive inheritance pattern, and pathogenic mutation (c.499C>T) was identified in the proband and his family member, which led to a premature termination prior to the entire POU domains. This mutation co-segregated with hearing loss in this family. No mutation of POU3F4 gene was found in 834 controls. Conclusions: A nonsense mutation is identified in a family displaying the pedigree consistent with X-linked recessive pattern in POU3F4 gene. In addition, we may provide molecular diagnosis and genetic counseling for this family. PMID:28051029

  14. Do You Hear Me? Information for Persons with a Hearing Impairment.

    ERIC Educational Resources Information Center

    Brook, Emily

    1990-01-01

    This compendium of general information for individuals with hearing impairments reports on: the incidence of hearing impairments; methods of communication between hearing and nonhearing persons (fingerspelling, lip reading, cued speech, signing, and telephone devices); 13 titles of books and tapes available; 4 sources of hearing ear dogs; 42…

  15. Career planning for hearing impaired employees

    SciTech Connect

    Ashdown, B.G.; Patterson, J.L.

    1983-06-01

    In recognition of the special needs of hearing-impaired employees, Union Carbide Nuclear Division staff members restructured and rewrote the existing Career Planning Program to accommodate the barriers experienced by deaf people. Consideration for reworking the training program included awareness that hearing-impaired people: learn mostly through their eyes; use sign language, which is grammatically and structurally different than the English language; have a limited understanding of the English language; live in an isolated world influenced mostly by the deaf community; and have sometimes been stigmatized because of their handicap, resulting in the belief by their parents and others in the hearing world that they lack in intelligence and ability. Twelve deaf employees participated in the program, including four from the Oak Ridge Gaseous Diffusion Plant, six from the Oak Ridge National Laboraory, and two from the Department of Energy. All twelve employees completed the 6 1/2 days of sessions spanning over six weeks, evaluating the program overall as very good. Although most did not feel a need for career change, they learned strategies for enhancing and developing their current positions. They also discovered they were not alone in many feelings of isolation or lack of self-confidence, and that many of their problems are shared by hearing people.

  16. 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.

  17. Teacher Strategies in Shared Reading for Children with Hearing Impairment

    ERIC Educational Resources Information Center

    Girgin, Ümit

    2013-01-01

    Problem Statement: Utilization of shared reading practice in hearing impaired children's literacy instruction may yield quite beneficial. However an investigation of Turkish literature revealed no studies regarding application of shared reading within elementary settings for hearing or hearing-impaired children. Furthermore international…

  18. Screening for Vision Problems in Children with Hearing Impairments.

    ERIC Educational Resources Information Center

    Demchak, MaryAnn; Elquist, Marty

    Vision problems occur at higher rates in the deaf and hearing impaired population than in the general population. When an individual has a hearing impairment, vision becomes more significant in the instructional and learning process, as well as in social and communicative exchanges. Regular comprehensive visual screening of hearing impaired…

  19. 38 CFR 4.85 - Evaluation of hearing impairment.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... percentage evaluation by combining the Roman numeral designations for hearing impairment of each ear. The horizontal rows represent the ear having the better hearing and the vertical columns the ear having the...) If impaired hearing is service-connected in only one ear, in order to determine the...

  20. 38 CFR 4.85 - Evaluation of hearing impairment.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... percentage evaluation by combining the Roman numeral designations for hearing impairment of each ear. The horizontal rows represent the ear having the better hearing and the vertical columns the ear having the...) If impaired hearing is service-connected in only one ear, in order to determine the...

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

    PubMed

    Xia, Wenjun; Liu, Fei; Ma, Duan

    2016-06-01

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

  2. 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.

  3. Identification of a Novel MYO15A Mutation in a Chinese Family with Autosomal Recessive Nonsyndromic Hearing Loss

    PubMed Central

    Xia, Hong; Huang, Xiangjun; Guo, Yi; Hu, Pengzhi; He, Guangxiang; Deng, Xiong; Xu, Hongbo; Yang, Zhijian; Deng, Hao

    2015-01-01

    Autosomal recessive nonsyndromic hearing loss (ARNSHL) is a genetically heterogeneous sensorineural disorder, generally manifested with prelingual hearing loss and absence of other clinical manifestations. The aim of this study is to identify the pathogenic gene in a four-generation consanguineous Chinese family with ARNSHL. A novel homozygous variant, c.9316dupC (p.H3106Pfs*2), in the myoxin XVa gene (MYO15A) was identified by exome sequencing and Sanger sequencing. The homozygous MYO15A c.9316dupC variant co-segregated with the phenotypes in the ARNSHL family and was absent in two hundred normal controls. The variant was predicted to interfere with the formation of the Myosin XVa-whirlin-Eps8 complex at the tip of stereocilia, which is indispensable for stereocilia elongation. Our data suggest that the homozygous MYO15A c.9316dupC variant might be the pathogenic mutation, and exome sequencing is a powerful molecular diagnostic strategy for ARNSHL, an extremely heterogeneous disorder. Our findings extend the mutation spectrum of the MYO15A gene and have important implications for genetic counseling for the family. PMID:26308726

  4. Early intervention programme for hearing impaired children.

    PubMed

    Narayanswamy, S

    1992-01-01

    The School for Young Deaf Children was founded in 1969 when the All India Institute of Speech and Hearing at Mysore and the Christian Medical College Hospital at Vellore started diagnosing hearing impairment in children and prescribing hearing aids. These schools admitted children when they were 5 years old. Bala Vidyalaya was funded as an experimental school to satisfy the needs of younger children. A multi sensory approach based on the Montessori method of teaching with special emphasis on language acquisition was adopted. The School that began with 5 children and 2 teachers had 120 children and 15 teachers in 1992: 50 children were under 3 years old and the rest were between 3 and 6 years. Early auditory management and training is the foundation of the child's linguistic achievement which help the child use the innate ability to develop sophisticated listening skills such as listening to one signal in the presence of competing sounds. Simple games captivate the infants. At the age of 2 1/2 years ideovisual reading is introduced to the child: written sentences are presented to the child about an activity that the child had just experienced. Even before 2 years of age he or she starts scribbling. School lessons are used as tools for writing. The school takes efforts to win the confidence of the parents. So far 97 children have joined the mainstream of education after an initial training the school. Of these, 6 are settled in jobs, 5 are in college or in postgraduate studies, 11 are studying at the university, 8 are in the higher secondary school (classes XI or XII)m 28 are studying in high school (class VI to class X), while the remaining 39 are in primary schools. It has been demonstrated that early educational intervention and involvement of the family into the educational program are very important for the successful integration of hearing-impaired children into the main stream.

  5. Deleterious oral habits in children with hearing impairment

    PubMed Central

    SUHANI, RALUCA DIANA; SUHANI, MIHAI FLAVIU; MUNTEAN, ALEXANDRINA; MESAROS, MICHAELA; BADEA, MINDRA EUGENIA

    2015-01-01

    Background and aims Deleterious oral habits represent a serious public health issue. The information available about this problem in children with hearing impairment is insufficient. The purpose of this study was to investigate the prevalence of deleterious oral habits among children with hearing impairment and comparing results against children without hearing impairment. Method This epidemiological study was carried out in a sample size of 315 children. We used a random sampling technique that included 150 children with hearing impairment and 165 without hearing impairment. All subjects were submitted to a clinical examination. The parents/legal guardians were asked to complete a questionnaire regarding the deleterious habits of their children. Results The data collected indicated a higher prevalence of deleterious oral habits among children with hearing impairment: 53.3% as opposed to 40.6% among children without hearing impairment. There was a higher incidence of malocclusion in children with hearing impairment (79.3%) compared to children without hearing impairment (57%). Conclusions This study highlighted the need to establish protocols for preventive orthodontic treatment at an early age, in order to reduce the deleterious oral habits and prevent malocclusion. Dental institutions/clinicians need to implement oral care programs including proper oral education aiming to promote oral health. PMID:26609277

  6. 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.

  7. Timbre discrimination in normal-hearing and hearing-impaired listeners under different noise conditions.

    PubMed

    Emiroglu, Suzan; Kollmeier, Birger

    2008-07-18

    In an attempt to quantify differences in object separation and timbre discrimination between normal-hearing and hearing-impaired listeners with a moderate sensorineural hearing loss of two different configurations, psychoacoustic measurements were performed with a total of 50 listeners. The experiments determined just noticeable differences (JND) of timbre in normal-hearing and hearing-impaired subjects along continua of "morphed" musical instruments and investigated the variance of JND in silence and different background noise conditions and on different sound levels. The results show that timbre JNDs of subjects with a steep hearing loss are significantly higher than of normal-hearing subjects, both in silence and noise, whereas timbre JNDs of flat/diagonal hearing-impaired subjects are similar to JNDs of normal-hearing subjects for signal levels above 55 dB (plus appropriate amplification for hearing-impaired). In noise (SNR=+10 dB) timbre JNDs of all subject groups are significantly higher than in silence. In the condition testing, transferability from silence to noise (i.e., the ability to imagine how the stimulus heard in silence would sound in noise), no significant JND differences across listener groups were found. The results can be explained by primary factors involved in sensorineural hearing loss and contradict the hypothesis that hearing-impaired people generally have more problems in object discrimination than normal-hearing people.

  8. Hearing impaired speech in noisy classrooms

    NASA Astrophysics Data System (ADS)

    Shahin, Kimary; McKellin, William H.; Jamieson, Janet; Hodgson, Murray; Pichora-Fuller, M. Kathleen

    2005-04-01

    Noisy classrooms have been shown to induce among students patterns of interaction similar to those used by hearing impaired people [W. H. McKellin et al., GURT (2003)]. In this research, the speech of children in a noisy classroom setting was investigated to determine if noisy classrooms have an effect on students' speech. Audio recordings were made of the speech of students during group work in their regular classrooms (grades 1-7), and of the speech of the same students in a sound booth. Noise level readings in the classrooms were also recorded. Each student's noisy and quiet environment speech samples were acoustically analyzed for prosodic and segmental properties (f0, pitch range, pitch variation, phoneme duration, vowel formants), and compared. The analysis showed that the students' speech in the noisy classrooms had characteristics of the speech of hearing-impaired persons [e.g., R. O'Halpin, Clin. Ling. and Phon. 15, 529-550 (2001)]. Some educational implications of our findings were identified. [Work supported by the Peter Wall Institute for Advanced Studies, University of British Columbia.

  9. Mutations in the lipoma HMGIC fusion partner-like 5 (LHFPL5) gene cause autosomal recessive nonsyndromic hearing loss.

    PubMed

    Kalay, Ersan; Li, Yun; Uzumcu, Abdullah; Uyguner, Oya; Collin, Rob W; Caylan, Refik; Ulubil-Emiroglu, Melike; Kersten, Ferry F J; Hafiz, Gunter; van Wijk, Erwin; Kayserili, Hulya; Rohmann, Edyta; Wagenstaller, Janine; Hoefsloot, Lies H; Strom, Tim M; Nürnberg, Gudrun; Baserer, Nermin; den Hollander, Anneke I; Cremers, Frans P M; Cremers, Cor W R J; Becker, Christian; Brunner, Han G; Nürnberg, Peter; Karaguzel, Ahmet; Basaran, Seher; Kubisch, Christian; Kremer, Hannie; Wollnik, Bernd

    2006-07-01

    In two large Turkish consanguineous families, a locus for autosomal recessive nonsyndromic hearing loss (ARNSHL) was mapped to chromosome 6p21.3 by genome-wide linkage analysis in an interval overlapping with the loci DFNB53 (COL11A2), DFNB66, and DFNB67. Fine mapping excluded DFNB53 and subsequently homozygous mutations were identified in the lipoma HMGIC fusion partner-like 5 (LHFPL5) gene, also named tetraspan membrane protein of hair cell stereocilia (TMHS) gene, which was recently shown to be mutated in the "hurry scurry" mouse and in two DFNB67-linked families from Pakistan. In one family, we found a homozygous one-base pair deletion, c.649delG (p.Glu216ArgfsX26) and in the other family we identified a homozygous transition c.494C>T (p.Thr165Met). Further screening of index patients from 96 Turkish ARNSHL families and 90 Dutch ARNSHL patients identified one additional Turkish family carrying the c.649delG mutation. Haplotype analysis revealed that the c.649delG mutation was located on a common haplotype in both families. Mutation screening of the LHFPL5 homologs LHFPL3 and LHFPL4 did not reveal any disease causing mutation. Our findings indicate that LHFPL5 is essential for normal function of the human cochlea.

  10. Hearing Aids and Hearing Impaired Students in Rural Schools.

    ERIC Educational Resources Information Center

    Woodford, Charles

    This paper describes functions of the components of hearing aids and provides a detailed procedure to detect hearing aid dysfunctions. The most common type of hearing aids for school children are the behind the ear type. Various hearing aid components change sound into an electrical signal, which is amplified and adjusted by a volume control. The…

  11. TOEFL and Hearing Impaired Students: A Feasibility Study.

    ERIC Educational Resources Information Center

    Ragosta, Marjorie; Nelson, Catherine

    The Test of English as a Foreign Language (TOEFL) was administered to 26 hearing impaired college students, in order to test the assumption that the English-language deficiencies of hearing impaired students are similar to those of foreign students. The students were attending Gallaudet College's School of Preparatory Studies and were identified…

  12. 38 CFR 4.85 - Evaluation of hearing impairment.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... and puretone threshold average intersect. (c) Table VIa, “Numeric Designation of Hearing Impairment... XI) for hearing impairment based only on the puretone threshold average. Table VIa will be used when... provisions of § 4.86. (d) “Puretone threshold average,” as used in Tables VI and VIa, is the sum of...

  13. Issues in the Evaluation of Hearing Impaired Children and Adolescents.

    ERIC Educational Resources Information Center

    Ulissi, Stephen Mark

    Intended for mental health professionals who evaluate hearing impaired children and adolescents, the paper discusses issues of general concern. Areas touched upon include psychological and sociological effects from the hearing impaired subculture, complicating medical and physical disorders, and modification of assessment devices to remove the…

  14. The Oral Referential Communication Skills of Hearing-Impaired Children

    ERIC Educational Resources Information Center

    Lloyd, Julian; Lieven, Elena; Arnold, Paul

    2005-01-01

    This paper focuses on the oral referential communication skills of hearing-impaired (HI) children. A task based on that used with language impaired children by Leinonen and Letts (1997) was used to assess the speaking and listening skills of 20 HI children (mean age=10;2 years; mean better ear average hearing loss=88.85 dBHL). Their performance…

  15. Setting of Classroom Environments for Hearing Impaired Children

    ERIC Educational Resources Information Center

    Turan, Zerrin

    2007-01-01

    This paper aims to explain effects of acoustical environments in sound perception of hearing impaired people. Important aspects of sound and hearing impairment are explained. Detrimental factors in acoustic conditions for speech perception are mentioned. Necessary acoustic treatment in classrooms and use of FM systems to eliminate these factors…

  16. 38 CFR 3.385 - Disability due to impaired hearing.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2011-07-01 2011-07-01 false Disability due to... Considerations Relative to Specific Diseases § 3.385 Disability due to impaired hearing. For the purposes of applying the laws administered by VA, impaired hearing will be considered to be a disability when...

  17. 38 CFR 3.385 - Disability due to impaired hearing.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2013-07-01 2013-07-01 false Disability due to... Considerations Relative to Specific Diseases § 3.385 Disability due to impaired hearing. For the purposes of applying the laws administered by VA, impaired hearing will be considered to be a disability when...

  18. 38 CFR 3.385 - Disability due to impaired hearing.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2014-07-01 2014-07-01 false Disability due to... Considerations Relative to Specific Diseases § 3.385 Disability due to impaired hearing. For the purposes of applying the laws administered by VA, impaired hearing will be considered to be a disability when...

  19. 38 CFR 3.385 - Disability due to impaired hearing.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2012-07-01 2012-07-01 false Disability due to... Considerations Relative to Specific Diseases § 3.385 Disability due to impaired hearing. For the purposes of applying the laws administered by VA, impaired hearing will be considered to be a disability when...

  20. Study of modifiers factors associated to mitochondrial mutations in individuals with hearing impairment

    SciTech Connect

    Sousa de Moraes, Vanessa Cristine; Alexandrino, Fabiana; Andrade, Paula Baloni; Camara, Marilia Fontenele; Sartorato, Edi Lucia

    2009-04-03

    Hearing impairment is the most prevalent sensorial deficit in the general population. Congenital deafness occurs in about 1 in 1000 live births, of which approximately 50% has hereditary cause in development countries. Non-syndromic deafness can be caused by mutations in both nuclear and mitochondrial genes. Mutations in mtDNA have been associated with aminoglycoside-induced and non-syndromic deafness in many families worldwide. However, the nuclear background influences the phenotypic expression of these pathogenic mutations. Indeed, it has been proposed that nuclear modifier genes modulate the phenotypic manifestation of the mitochondrial A1555G mutation in the MTRNR1 gene. The both putative nuclear modifiers genes TRMU and MTO1 encoding a highly conserved mitochondrial related to tRNA modification. It has been hypothesizes that human TRMU and also MTO1 nuclear genes may modulate the phenotypic manifestation of deafness-associated mitochondrial mutations. The aim of this work was to elucidate the contribution of mitochondrial mutations, nuclear modifier genes mutations and aminoglycoside exposure in the deafness phenotype. Our findings suggest that the genetic background of individuals may play an important role in the pathogenesis of deafness-associated with mitochondrial mutation and aminoglycoside-induced.

  1. Relative clause reading in hearing impairment: different profiles of syntactic impairment.

    PubMed

    Szterman, Ronit; Friedmann, Naama

    2014-01-01

    Children with hearing impairment show difficulties in sentences derived by Wh-movement, such as relative clauses and Wh-questions. This study examines the nature of this deficit in 48 hearing impaired children aged 9-12 years and 38 hearing controls. The task involved reading aloud and paraphrasing of object relatives that include a noun-verb heterophonic homograph. The correct pronunciation of the homograph in these sentences depended upon the correct construction of the syntactic structure of the sentence. An analysis of the reading and paraphrasing of each participant exposed two different patterns of syntactic impairment. Some hearing-impaired children paraphrased the object relatives incorrectly but could still read the homograph, indicating impaired assignment of thematic roles alongside good syntactic structure building; other hearing-impaired children could neither read the homograph nor paraphrase the sentence, indicating a structural deficit in the syntactic tree. Further testing of these children confirmed the different impairments: some are impaired only in Wh-movement, whereas others have CP impairment. The syntactic impairment correlated with whether or not a hearing device was fitted by the age of 1 year, but not with the type of hearing device or the depth of hearing loss: children who had a hearing device fitted during the first year of life had better syntactic abilities than children whose hearing devices were fitted later.

  2. Occupational performance: comparing normally-hearing and hearing-impaired employees using the Amsterdam Checklist for Hearing and Work.

    PubMed

    Kramer, Sophia E; Kapteyn, Theo S; Houtgast, Tammo

    2006-09-01

    This study compares the occupational performance of employees with and without hearing impairment, and aims to identify occupational difficulties specifically related to hearing loss. The Amsterdam Checklist for Hearing and Work was administered to 150 hearing-impaired employees and 60 normally-hearing colleagues. A multivariate analysis of variance was performed to test group effects, and to examine differences between means. Factors predicting sick-leave were identified by regression analyses. A significant group effect (p < 0.01) was found. Hearing-impaired employees differed from normally-hearing colleagues in their perception of 'environmental noise', 'job control' and the 'necessity to use hearing activities' at work. Also, sick-leave due to distress occurred significantly more often in the hearing impaired group (p < 0.05). 'Hearing impairment', 'job demand', and the requirement to 'recognize/distinguish between sounds' were the strongest risk-factors for stress related sick-leave. The importance of hearing functions besides speech communication is discussed. Implications for rehabilitation are suggested. In future research, hearing loss should be considered as a risk factor for fatigue and mental distress which may lead to sick-leave.

  3. 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.

  4. Genetic analysis of TMPRSS3 gene in the Korean population with autosomal recessive nonsyndromic hearing loss.

    PubMed

    Lee, Jinwook; Baek, Jeong-In; Choi, Jae Young; Kim, Un-Kyung; Lee, Sang-Heun; Lee, Kyu-Yup

    2013-12-15

    The TMPRSS3 gene (DFNB8/10), which encodes a transmembrane serine protease, is a common hearing loss gene in several populations. Accurate functions of TMPRSS3 in the hearing pathway are still unknown, but TMPRSS3 has been reported to play a crucial role in inner ear development or maintenance. To date, 16 pathogenic mutations have been identified in many countries, but no mutational studies of the TMPRSS3 gene have been conducted in the Korean hearing loss population. In this study, we performed genetic analysis of TMPRSS3 in 40 unrelated Korean patients with autosomal recessive hearing loss to identify the aspect and frequency of TMPRSS3 gene mutations in the Korean population. A total of 22 variations were detected, including a novel variant (p.V291L) and a previously reported pathogenic mutation (p.A306T). The p.A306T mutation which has been detected in only compound heterozygous state in previous studies was identified in homozygous state for the first time in this study. Moreover, the clinical evaluation identified bilateral dilated vestibules in the patient with p.A306T mutation, and it suggested that p.A306T mutation of the TMPRSS3 gene might be associated with vestibular anomalies. In conclusion, this study investigated that only 2.5% of patients with autosomal recessive hearing loss were related to TMPRSS3 mutations suggesting low prevalence of TMPRSS3 gene in Korean hearing loss population. Also, it will provide the information of genotype-phenotype correlation to understand definite role of TMPRSS3 in the auditory system.

  5. Satisfaction with Hearing Aids Based on Technology and Style among Hearing Impaired Persons

    PubMed Central

    Faraji- Khiavi, Farzad; Dashti, Rezvan; Sameni, Seyyed-Jalal; Bayat, Arash

    2016-01-01

    Introduction: Hearing loss is one of the most disabling impairments. Using a hearing aid as an attempt to improve the hearing problem can positively affect the quality of life for these people. This research was aimed to assess satisfaction of hearing impaired patients with their hearing aids regarding the employed technology and style. Materials and Methods: This descriptive-analytic cross-sectional research was conducted on 187 subjects with hearing loss who were using a hearing aid. The subjects were over 18 years of age and were using a hearing aid for at least 6 months. The Persian version of Satisfaction with Amplification in Daily Life (SADL) questionnaire was the instrument which was used for assessing satisfaction with the hearing aid. Cronbach’s alpha was calculated to be 0.80 for instrument reliability. Results: A significant difference was observed among satisfaction subscales’ mean scores with hearing aid technology. Also a significant difference was observed between the total satisfaction score and the hearing aid model. With respect to the analysis of satisfaction with the hearing aid and its style, cost and services was the only subscale which showed a significant difference (P=0.005). Conclusion: Respondents using hearing aids with different technology and style were estimated to be quite satisfied. Training audiologists in using more appropriate and fitting hearing aids in addition to using self-reporting questionnaires like SADL for estimating patients’ social condition and participation in their life can essentially change their disability condition and countervail their hearing loss. PMID:27738608

  6. 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…

  7. Visual aid for the hearing impaired

    NASA Astrophysics Data System (ADS)

    Jhabvala, Murzban D.; Lin, Hung C.

    1991-07-01

    A multichannel electronic visual aid device which is able to signal to the user whether sound is coming from the left or right, front or back, or both is presented. For the plurality of channels, which may operate in pairs, the sound is picked up by a respective microphone and amplified and rectified into a DC voltage. The DC voltage is next fed to an analog to digital converter and then to a digital encoder. The binary code from the encoder is coupled into a logic circuit where the binary code is decoded to proved a plurality of output levels which are used to drive an indicator which, in turn, provides a visual indication of the sound level received. The binary codes for each pair of channels are also fed into a digital comparator. The output of the comparator is used to enable the logic circuits of the two channels such that if, for example, the signal coming from the right is louder than that coming from the left, the output of the logic unit of the right channel will be enabled and the corresponding indicator activated, indicating the sound source on the right. An indication of the loudness is also provided. One embodiment of the invention may be carried by the hearing impaired or deaf, as a system which is embedded into eye glasses or a cap. Another embodiment of the invention may be integrated with a vehicle to give a hearing impaired or deaf driver a warning, with a directional indication, that an emergency vehicle is in the vicinity. In this second embodiment, the emergency vehicle transmits a radio frequency signal which would be used as an enabling signal for the visual aid device to avoid false alarms from traffic and other sound sources in the vicinity of the driver's vehicle.

  8. Inflectional morphology in German hearing-impaired children.

    PubMed

    Penke, Martina; Wimmer, Eva; Hennies, Johannes; Hess, Markus; Rothweiler, Monika

    2016-01-01

    Despite modern hearing aids, children with hearing impairment often have only restricted access to spoken language input during the 'critical' years for language acquisition. Specifically, a sensorineural hearing impairment affects the perception of voiceless coronal consonants which realize verbal affixes in German. The aim of this study is to explore if German hearing-impaired children have problems in producing and/or acquiring inflectional suffixes expressed by such phonemes. The findings of two experiments (an elicitation task and a picture-naming task) conducted with a group of hearing-impaired monolingual German children (age 3-4 years) demonstrate that difficulties in perceiving specific phonemes relate to the avoidance of these same sounds in speech production independent of the grammatical function these phonemes have.

  9. Hypo-functional SLC26A4 variants associated with nonsyndromic hearing loss and enlargement of the vestibular aqueduct: genotype-phenotype correlation or coincidental polymorphisms?

    PubMed Central

    Choi, Byung Yoon; Stewart, Andrew K.; Madeo, Anne C.; Pryor, Shannon P.; Lenhard, Suzanne; Kittles, Rick; Eisenman, David; Kim, H. Jeffrey; Niparko, John; Thomsen, James; Arnos, Kathleen S.; Nance, Walter E.; King, Kelly A.; Zalewski, Christopher K.; Brewer, Carmen C.; Shawker, Thomas; Reynolds, James C.; Butman, John A.; Karniski, Lawrence P.; Alper, Seth L.; Griffith, Andrew J.

    2008-01-01

    Hearing loss with enlargement of the vestibular aqueduct (EVA) can be associated with mutations of the SLC26A4 gene encoding pendrin, a transmembrane Cl−/I−/HCO3− exchanger. Pendrin’s critical transport substrates are thought to be I− in the thyroid gland and HCO3− in the inner ear. We previously reported that bi-allelic SLC26A4 mutations are associated with Pendred syndromic EVA whereas one or zero mutant alleles are associated with nonsyndromic EVA. One study proposed a correlation of nonsyndromic EVA with SLC26A4 alleles encoding pendrin with residual transport activity. Here we describe the phenotypes and SLC26A4 genotypes of 47 EVA patients ascertained since our first report of 39 patients. We sought to determine the pathogenic potential of each variant in our full cohort of 86 patients. We evaluated the trafficking of 11 missense pendrin products expressed in COS-7 cells. Products that targeted to the plasma membrane were expressed in Xenopus oocytes for measurement of anion exchange activity. p.F335L, p.C565Y, p.L597S, p.M775T, and p.R776C had Cl−/I− and Cl−/HCO3− exchange rate constants that ranged from 13 to 93% of wild type values. p.F335L, p.L597S, p.M775T and p.R776C are typically found as mono-allelic variants in nonsyndromic EVA. The high normal control carrier rate for p.L597S indicates it is a coincidentally detected nonpathogenic variant in this context. We observed moderate differential effects of hypo-functional variants upon exchange of HCO3− versus I− but their magnitude does not support a causal association with nonsyndromic EVA. However, these alleles could be pathogenic in trans configuration with a mutant allele in Pendred syndrome. PMID:19204907

  10. Visual Field Abnormalities among Adolescent Boys with Hearing Impairments

    PubMed Central

    KHORRAMI-NEJAD, Masoud; HERAVIAN, Javad; SEDAGHAT, Mohamad-Reza; MOMENI-MOGHADAM, Hamed; SOBHANI-RAD, Davood; ASKARIZADEH, Farshad

    2016-01-01

    The aim of this study was to compare the visual field (VF) categorizations (based on the severity of VF defects) between adolescent boys with hearing impairments and those with normal hearing. This cross-sectional study involved the evaluation of the VF of 64 adolescent boys with hearing impairments and 68 age-matched boys with normal hearing at high schools in Tehran, Iran, in 2013. All subjects had an intelligence quotient (IQ) > 70. The hearing impairments were classified based on severity and time of onset. Participants underwent a complete eye examination, and the VFs were investigated using automated perimetry with a Humphrey Visual Field Analyzer. This device was used to determine their foveal threshold (FT), mean deviation (MD), and Glaucoma Hemifield Test (GHT) results. Most (50%) of the boys with hearing impairments had profound hearing impairments. There was no significant between-group difference in age (P = 0.49) or IQ (P = 0.13). There was no between-group difference in the corrected distance visual acuity (P = 0.183). According to the FT, MD, and GHT results, the percentage of boys with abnormal VFs in the hearing impairment group was significantly greater than that in the normal hearing group: 40.6% vs. 22.1%, 59.4% vs. 19.1%, and 31.2% vs. 8.8%, respectively (P < 0.0001). The mean MD in the hearing impairment group was significantly worse than that in the normal hearing group (-0.79 ± 2.04 and -4.61 ± 6.52 dB, respectively, P < 0.0001), and the mean FT was also significantly worse (38.97 ± 1.66 vs. 35.30 ± 1.43 dB, respectively, P <0.0001). Moreover, there was a significant between-group difference in the GHT results (P < 0.0001). Thus, there were higher percentages of boys with VF abnormalities and higher mean MD, FT, and GHT results among those with hearing impairments compared to those with normal hearing. These findings emphasize the need for detailed VF assessments for patients with hearing impairments. PMID:28293650

  11. 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.

  12. Autosomal-Recessive Hearing Impairment Due to Rare Missense Variants within S1PR2

    PubMed Central

    Santos-Cortez, Regie Lyn P.; Faridi, Rabia; Rehman, Atteeq U.; Lee, Kwanghyuk; Ansar, Muhammad; Wang, Xin; Morell, Robert J.; Isaacson, Rivka; Belyantseva, Inna A.; Dai, Hang; Acharya, Anushree; Qaiser, Tanveer A.; Muhammad, Dost; Ali, Rana Amjad; Shams, Sulaiman; Hassan, Muhammad Jawad; Shahzad, Shaheen; Raza, Syed Irfan; Bashir, Zil-e-Huma; Smith, Joshua D.; Nickerson, Deborah A.; Bamshad, Michael J.; Riazuddin, Sheikh; Ahmad, Wasim; Friedman, Thomas B.; Leal, Suzanne M.

    2016-01-01

    The sphingosine-1-phosphate receptors (S1PRs) are a well-studied class of transmembrane G protein-coupled sphingolipid receptors that mediate multiple cellular processes. However, S1PRs have not been previously reported to be involved in the genetic etiology of human traits. S1PR2 lies within the autosomal-recessive nonsyndromic hearing impairment (ARNSHI) locus DFNB68 on 19p13.2. From exome sequence data we identified two pathogenic S1PR2 variants, c.323G>C (p.Arg108Pro) and c.419A>G (p.Tyr140Cys). Each of these variants co-segregates with congenital profound hearing impairment in consanguineous Pakistani families with maximum LOD scores of 6.4 for family DEM4154 and 3.3 for family PKDF1400. Neither S1PR2 missense variant was reported among ∼120,000 chromosomes in the Exome Aggregation Consortium database, in 76 unrelated Pakistani exomes, or in 720 Pakistani control chromosomes. Both DNA variants affect highly conserved residues of S1PR2 and are predicted to be damaging by multiple bioinformatics tools. Molecular modeling predicts that these variants affect binding of sphingosine-1-phosphate (p.Arg108Pro) and G protein docking (p.Tyr140Cys). In the previously reported S1pr2−/− mice, stria vascularis abnormalities, organ of Corti degeneration, and profound hearing loss were observed. Additionally, hair cell defects were seen in both knockout mice and morphant zebrafish. Family PKDF1400 presents with ARNSHI, which is consistent with the lack of gross malformations in S1pr2−/− mice, whereas family DEM4154 has lower limb malformations in addition to hearing loss. Our findings suggest the possibility of developing therapies against hair cell damage (e.g., from ototoxic drugs) through targeted stimulation of S1PR2. PMID:26805784

  13. 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

  14. Symptoms of Psychopathology in Hearing-Impaired Children

    PubMed Central

    Rieffe, Carolien; Soede, Wim; Briaire, Jeroen J.; Ketelaar, Lizet; Kouwenberg, Maartje; Frijns, Johan H. M.

    2015-01-01

    Objectives: Children with hearing loss are at risk of developing psychopathology, which has detrimental consequences for academic and psychosocial functioning later in life. Yet, the causes of the extensive variability in outcomes are not fully understood. Therefore, the authors wanted to objectify symptoms of psychopathology in children with cochlear implants or hearing aids, and in normally hearing peers, and to identify various risk and protective factors. Design: The large sample (mean age = 11.8 years) included three subgroups with comparable age, gender, socioeconomic status, and nonverbal intelligence: 57 with cochlear implants, 75 with conventional hearing aids, and 129 children who were normally hearing. Psychopathology was assessed by means of self- and parent-report measures. Results: Children with cochlear implants showed similar levels of symptoms of psychopathology when compared with their normally hearing peers, but children with hearing aids had significantly higher levels of psychopathological symptoms, while their hearing losses were approximately 43 dB lower than those of children with implants. Type of device was related with internalizing symptoms but not with externalizing symptoms. Furthermore, lower age and sufficient language and communication skills predicted less psychopathological symptoms. Conclusions: Children who are deaf or profoundly hearing impaired and have cochlear implants have lower levels of psychopathological symptoms than children with moderate or severe hearing loss who have hearing aids. Most likely, it is not the type of hearing device but rather the intensity of the rehabilitation program that can account for this difference. This outcome has major consequences for the next generation of children with hearing loss because children with profound hearing impairment still have the potential to have levels of psychopathology that are comparable to children who are normally hearing. PMID:25668391

  15. Some Aerodynamic Characteristics of Plosive Consonants Produced by Hearing-Impaired Speakers.

    ERIC Educational Resources Information Center

    Whitehead, Robert; Barefoot, Sidney

    1980-01-01

    The study investigated the rate of oral air flow for plosive consonants produced by 10 normal hearing adult males, 10 hearing impaired adult males who demonstrated intelligible speech, and 10 hearing impaired adult males with semi-intelligible speech. (Author)

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

    ERIC Educational Resources Information Center

    Schildroth, Arthur

    1986-01-01

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

  17. Captions and Reading Rates of Hearing-Impaired Students.

    ERIC Educational Resources Information Center

    Shroyer, Edgar H.; Birch, Jack

    1980-01-01

    By adapting the scoring procedure of the Gates McGinitie Reading Test, speed and accuracy portion, the reading rates of 185 randomly selected hearing impaired students from residential schools for the deaf were obtained. Rates were then compared with the reading rates of hearing students and extempore speech. (Author)

  18. Initiations of Social Interactions by Young Hearing Impaired Preschoolers

    ERIC Educational Resources Information Center

    Weisel, Amatzia; Most, Tova; Efron, Clara

    2005-01-01

    This study examined strategies for initiating social interactions with peers, among 4 children with hearing impairment, aged 33 to 36 months, attending a special early education center or a regular kindergarten. The study investigated initiation type (related to partner's hearing status) and rates of initiation success/failure vis-a-vis hearing…

  19. Hearing Impaired Children's Performance on the Piagetian Liquid Horizontality Test.

    ERIC Educational Resources Information Center

    Murphy-Berman, Virginia; And Others

    A paper-and-pencil test consisting of a series of 24 sketches was administered to assess the performance of hearing impaired students aged 9-12 on a Piagetian horizontality task. This age range among hearing students is the developmental period during which comprehension of the principle of horizontality should begin to emerge, indicating ability…

  20. The Hearing Impaired Student in the Regular Classroom.

    ERIC Educational Resources Information Center

    Alberta Dept. of Education, Edmonton.

    The guide provides strategies for teachers to use with deaf and hearing impaired (HI) students in regular classrooms in the province of Alberta, Canada. An introductory section includes symptoms of a suspected hearing loss and a sample audiogram to aid teachers in recognizing the problem. Ways to meet special needs at different age levels are…

  1. Onset, Cause, and Additional Handicaps in Hearing Impaired Children

    ERIC Educational Resources Information Center

    Jensema, Carl; Mullins, Jane

    1974-01-01

    Some statistics are presented concerning age of onset, cause, and additional handicaps from a nationwide sample (1972-73) of 43,946 hearing impaired students enrolled in 712 special education programs. (Author/LS)

  2. Syntactic and Semantic Characteristics in the Written Language of Hearing Impaired and Normally Hearing School-Aged Children.

    ERIC Educational Resources Information Center

    Yoshinaga, Christine

    To investigate semantic and syntactic variables in the written language of normally hearing and hearing impaired children, 49 hearing impaired and 49 normally hearing children (10-14 years old) were asked to write compositions based on the Accident/Emergency Picture in the Peabody Language Development Kit. In addition, syntactic characteristics…

  3. Citizenship among a Sample of Hearing and Hearing Impaired Kindergarten's Children in Al-Riyadh Saudi Arabia "Comparative Study"

    ERIC Educational Resources Information Center

    Turkestani, Maryam Hafez; Bahatheg, Raja' Omar

    2015-01-01

    This study aimed at identifying statistically significant differences in citizenship between Saudi hearing and hearing impaired children. The study sample consisted of (167) hearing and (42) hearing impaired children at public kindergartens in Al-Riyadh city, (82) of whom were males and (127) were female children. Data was collected using…

  4. Hearing impairment in Stickler syndrome: a systematic review

    PubMed Central

    2012-01-01

    Background Stickler syndrome is a connective tissue disorder characterized by ocular, skeletal, orofacial and auditory defects. It is caused by mutations in different collagen genes, namely COL2A1, COL11A1 and COL11A2 (autosomal dominant inheritance), and COL9A1 and COL9A2 (autosomal recessive inheritance). The auditory phenotype in Stickler syndrome is inconsistently reported. Therefore we performed a systematic review of the literature to give an up-to-date overview of hearing loss in Stickler syndrome, and correlated it with the genotype. Methods English-language literature was reviewed through searches of PubMed and Web of Science, in order to find relevant articles describing auditory features in Stickler patients, along with genotype. Prevalences of hearing loss are calculated and correlated with the different affected genes and type of mutation. Results 313 patients (102 families) individually described in 46 articles were included. Hearing loss was found in 62.9%, mostly mild to moderate when reported. Hearing impairment was predominantly sensorineural (67.8%). Conductive (14.1%) and mixed (18.1%) hearing loss was primarily found in young patients or patients with a palatal defect. Overall, mutations in COL11A1 (82.5%) and COL11A2 (94.1%) seem to be more frequently associated with hearing impairment than mutations in COL2A1 (52.2%). Conclusions Hearing impairment in patients with Stickler syndrome is common. Sensorineural hearing loss predominates, but also conductive hearing loss, especially in children and patients with a palatal defect, may occur. The distinct disease-causing collagen genes are associated with a different prevalence of hearing impairment, but still large phenotypic variation exists. Regular auditory follow-up is strongly advised, particularly because many Stickler patients are visually impaired. PMID:23110709

  5. 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.

  6. The homozygote p.V27I/p.E114G variant of GJB2 is a putative indicator of nonsyndromic hearing loss in Chinese infants.

    PubMed

    Chen, Wen-Xia; Huang, Yue; Yang, Xiao-Lin; Duan, Bo; Lu, Ping; Wang, Yan; Xu, Zheng-Min

    2016-05-01

    The gap junction β2 (GJB2) gene is associated with more than half of the recessive forms of hereditary hearing loss (HHL). However, the correlation between p.V27I and p.E114G variants of GJB2 and hearing phenotype remains controversial. This study aimed to clarify possible roles of these variants in Chinese infants with nonsyndromic hearing loss (NSHL). Hearing and gene tests were conducted in 300 infants (aged 0-3 months) with NSHL and 484 normal infants (aged 0-3 months). The p.V27I and p.E114G variants appeared frequently in both NSHL patients and normal controls. The allele and haplotype frequencies of p.V27I and p.E114G in patients and controls were compared, but no significant difference was observed (p=0.44 and p=0.26, respectively). Moreover, genotype frequencies of the p.V27I variant showed no significant difference between the two groups (p=0.66). Interestingly, more homozygote p.V27I/p.E114G subjects were found in NSHL infants than in controls (5/484 and 13/300, respectively), most of whom (61.54%) had mild or moderate hearing losses. Our results indicate that homozygote p.V27I/p.E114G is associated with mild and moderate HHL.

  7. 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

  8. Cortical Auditory Evoked Potentials in (Un)aided Normal-Hearing and Hearing-Impaired Adults

    PubMed Central

    Van Dun, Bram; Kania, Anna; Dillon, Harvey

    2016-01-01

    Cortical auditory evoked potentials (CAEPs) are influenced by the characteristics of the stimulus, including level and hearing aid gain. Previous studies have measured CAEPs aided and unaided in individuals with normal hearing. There is a significant difference between providing amplification to a person with normal hearing and a person with hearing loss. This study investigated this difference and the effects of stimulus signal-to-noise ratio (SNR) and audibility on the CAEP amplitude in a population with hearing loss. Twelve normal-hearing participants and 12 participants with a hearing loss participated in this study. Three speech sounds—/m/, /g/, and /t/—were presented in the free field. Unaided stimuli were presented at 55, 65, and 75 dB sound pressure level (SPL) and aided stimuli at 55 dB SPL with three different gains in steps of 10 dB. CAEPs were recorded and their amplitudes analyzed. Stimulus SNRs and audibility were determined. No significant effect of stimulus level or hearing aid gain was found in normal hearers. Conversely, a significant effect was found in hearing-impaired individuals. Audibility of the signal, which in some cases is determined by the signal level relative to threshold and in other cases by the SNR, is the dominant factor explaining changes in CAEP amplitude. CAEPs can potentially be used to assess the effects of hearing aid gain in hearing-impaired users. PMID:27587919

  9. Early care in children with permanent hearing impairment.

    PubMed

    Giuntini, G; Forli, F; Nicastro, R; Ciabotti, A; Bruschini, L; Berrettini, S

    2016-02-01

    The implementation of regional protocols for newborn hearing screening and early audiologic diagnosis represent the first step of the entire diagnostic, rehabilitative and prosthetic programme for children with permanent hearing impairment. The maximum benefit of early diagnosis can indeed be obtained only by prompt rehabilitation aimed at fostering the child's communicative, linguistic and cognitive development. Within the framework of the CMM 2013 project of the Ministry of Health entitled "Preventing Communication Disorders: a Regional Program for Early Identification, Intervention and Care of Hearing Impaired Children", the problems concerning the promotion of the global development of children with PHI through an early rehabilitation project based on shared knowledge and scientific evidence. In this project, our specific aim was to define the features and modes of access to a precise and specialised rehabilitation project for the small hearing-impaired child within three months from audiologic diagnosis. Three main recommendations relative to assessment and rehabilitation aspects of early care emerged from the study.

  10. Peculiarities of hearing impairment depending on interaction with acoustic stimuli

    PubMed Central

    Myshchenko, Iryna; Nazarenko, Vasyl; Kolganov, Anatoliy; Tereshchenko, Pavlo

    2015-01-01

    Aims: The functional state of the auditory analyzer of several operators groups was study. The objective of this study was to determine some characteristics of hearing impairment in relation with features of acoustic stimuli and informative significance of noise. Materials and Methods: 236 employees (middle age 35.4 ± 0.74 years) were divided into four groups according to features of noise perception at the workplaces. The levels of permanent shifts of acoustic thresholds were estimated using audiometric method. Statistical Analysis Used: Common statistical methods were used in research. Mean quantity and mean absolute errors were calculated. Statistical significance between operators' groups was calculated with 0.05 confidential intervals. Results: The peculiarities of hearing impairment in observed groups were different. Operators differentiating acoustic signals had peak of hearing impairment in the field of language frequencies, while the employees who work with noise background at the workplaces had maximal hearing threshold on the 4000 Hz frequency (P ≤ 0.05). Conclusions: Hearing impairment depends both on energy and human interaction with acoustic irritant. The distinctions in hearing impairment may be related with the necessity of recognizing of acoustic signals and their frequency characteristics. PMID:26957812

  11. The DFNA2 locus for hearing impairment: two genes regulating K+ ion recycling in the inner ear.

    PubMed

    Van Hauwe, P; Coucke, P; Van Camp, G

    1999-10-01

    DFNA2 is a locus for autosomal dominant non-syndromal hearing impairment (ADNSHI) located on chromosome 1p34 and six linked families have been identified. An audiometric study of these families showed that despite small differences in the phenotype all families suffer from progressive hearing impairment starting in the high frequencies. A detailed genetic analysis revealed that this deafness locus contains more than one gene responsible for hearing impairment. Thus far, two genes on chromosome 1p34 have been implicated in ADNSHI. The first, connexin 31 (GJB3), is a member of the connexin gene family. Connexins form gap junctions. These are connections between neighbouring cells that allow transport of small molecules. GJB3 mutations were found in two small Chinese families with ADNSHI. The second is KCNQ4, a voltage-gated K+ channel. Mutations in KCNQ4 were first found in a small French family, later in five of the six linked DFNA2 families. No GJB3 or KCNQ4 mutations were detected in patients of an extended Indonesian DFNA2 family. Two pathways have been proposed for the recycling of K+ from the hair cells back to the endolymph. These pathways involve the use of gap junctions, K+ pumps and K+ channels. The expression of GJB3 and KCNQ4 in the inner ear and their functions suggest that both DFNA2 genes may play a role in K+ homeostasis.

  12. Screening for mutations in the GJB3 gene in Brazilian patients with nonsyndromic deafness.

    PubMed

    Alexandrino, Fabiana; Oliveira, Camila A; Reis, Fernanda C; Maciel-Guerra, Andréa T; Sartorato, Edi L

    2004-01-01

    Deafness is a complex disorder that is affected by a high number of genes and environmental factors. Recently, enormous progress has been made in nonsyndromic deafness research, with the identification of 90 loci and 33 nuclear and 2 mitochondrial genes involved (http://dnalab-www.uia.ac.be/dnalab/hhh/). Mutations in the GJB3 gene, encoding the gap junction protein connexin 31 (Cx31), have been pathogenically linked to erythrokeratodermia variabilis and nonsyndromic autosomal recessive or dominant hereditary hearing impairment. To determine the contribution of the GJB3 gene to sporadic deafness, we analysed the GJB3 gene in 67 families with nonsyndromic hearing impairment. A single coding exon of the GJB3 gene was amplified from genomic DNA and then sequenced. Here we report on three amino acid changes: Y177D (c.529T > G), 49delK (c.1227C > T), and R32W (c.144-146delGAA). The latter substitution has been previously described, but its involvement in hearing impairment remains uncertain. We hypothesize that mutations in the GJB3 gene are an infrequent cause of nonsyndromic deafness.

  13. Vowel Formant Values in Hearing and Hearing-Impaired Children: A Discriminant Analysis

    ERIC Educational Resources Information Center

    Ozbic, Martina; Kogovsek, Damjana

    2010-01-01

    Hearing-impaired speakers show changes in vowel production and formant pitch and variability, as well as more cases of overlapping between vowels and more restricted formant space, than hearing speakers; consequently their speech is less intelligible. The purposes of this paper were to determine the differences in vowel formant values between 32…

  14. Comparison of Human Figure Drawings by Hearing and Hearing-Impaired Children.

    ERIC Educational Resources Information Center

    Cates, Jim A.

    1991-01-01

    The study compared human figure drawings (using the Goodenough-Harris Drawing Test and Koppitz' Emotional Indicators) of 26 hearing-impaired and 26 normal-hearing children and adolescents. No significant differences were found between groups but the Emotional Indicators did not perform as predicted in determining emotional disturbance. (Author/DB)

  15. Broadband Auditory Stream Segregation by Hearing-Impaired and Normal-Hearing Listeners

    ERIC Educational Resources Information Center

    Valentine, Susie; Lentz, Jennifer J.

    2008-01-01

    Purpose: To investigate the effects of hearing loss on auditory stream segregation of broadband inharmonic sounds. Method: Auditory stream segregation by listeners with normal and impaired hearing was measured for 6-component inharmonic sounds ("A" and "B") using objective and subjective methods. Components in the A stimuli ranged between 1000 and…

  16. National Strategic Research Plan for Hearing and Hearing Impairment and Voice and Voice Disorders.

    ERIC Educational Resources Information Center

    National Inst. on Deafness and Other Communications Disorders, Bethesda, MD.

    This monograph presents an update to the strategic plan of the National Institute on Deafness and Other Communication Disorders (NIDCD), focusing on recent accomplishments, program goals, strategies, and priorities in research opportunities in the areas of hearing/hearing impairment and voice/voice disorders. Specifically considered for the…

  17. Spectrum and frequency of GJB2, GJB6 and SLC26A4 gene mutations among nonsyndromic hearing loss patients in eastern part of India.

    PubMed

    Adhikary, Bidisha; Ghosh, Sudakshina; Paul, Silpita; Bankura, Biswabandhu; Pattanayak, Arup Kumar; Biswas, Subhradev; Maity, Biswanath; Das, Madhusudan

    2015-12-01

    Genetically caused nonsyndromic hearing loss is highly heterogeneous. Inspite of this large heterogeneity, mutations in the genes GJB2, GJB6 and SLC26A4 are major contributors. The mutation spectrum of these genes varies among different ethnic groups. Only a handful of studies focused on the altered genetic signature of these genes in different demographic regions of India but never focused on the eastern part of the country. Our study for the first time aimed to characterize the mutation profile of these genes in hearing loss patients of West Bengal state, India. Mutations in GJB2, GJB6 and SLC26A4 genes were screened by bidirectional sequencing from 215 congenital nonsyndromic hearing loss patients. Radiological diagnosis was performed in patients with SLC26A4 mutations by temporal bone CT scan. The study revealed that 4.65% and 6.97% patients had monoallelic and biallelic GJB2 mutations respectively. Six mutations were identified, p.W24X being the most frequent one accounting for 71.05% of the mutated alleles. Mutations in GJB6 including the previously identified deletion mutation (GJB6-D13S1830) were not identified in our study. Further, no patients harbored biallelic mutations in the SLC26A4 gene or the common inner ear malformation Enlarged Vestibular Aqueduct (EVA). The mutation profile of GJB2 in our study is distinct from other parts of India, suggesting that the mutation spectrum of this gene varies with ethnicity and geographical origin. The absence of GJB6 mutations and low frequency of SLC26A4 mutations suggest that additional genetic factors may also contribute to this disease.

  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. [Rehabilitative measures in hearing-impaired children].

    PubMed

    von Wedel, H; von Wedel, U C; Zorowka, P

    1991-12-01

    On the basis of certain fundamental data on the maturation processes of the central auditory pathways in early childhood the importance of early intervention with hearing aids is discussed and emphasized. Pathological hearing, that is acoustical deprivation in early childhood will influence the maturation process. Very often speech development is delayed if diagnosis and therapy or rehabilitation are not early enough. Anamnesis, early diagnosis and clinical differential diagnosis are required before a hearing aid can be fitted. Selection criteria and adjustment parameters are discussed, showing that the hearing aid fitting procedure must be embedded in a complex matrix of requirements related to the development of speech as well as to the cognitive, emotional and social development of the child. As a rule, finding and preparing the "best" hearing aids (binaural fitting is obligatory) for a child is a long and often difficult process, which can only be performed by specialists who are pedo-audiologists. After the binaural fitting of hearing aids an intensive hearing and speech education in close cooperation between parents, pedo-audiologist and teacher must support the whole development of the child.

  20. Hearing impairment and contributing factors among fertilizer factory workers

    PubMed Central

    Saffree Jeffree, Mohammad; Ismail, Noorhassim; Awang Lukman, Khamisah

    2016-01-01

    Introduction: Hearing impairment remains the main occupational health problem in the manufacturing industry, and its contributing factors have not been well controlled. Methods: Unmatched case control and comparative studies were carried out among fertilizer factory workers in Sarawak with the aim of determining contributing factors for hearing impairment. Respondents consisted of 49 cases that were diagnosed from 2005 to 2008 with 98 controls from the same work places. Chi-square test and Mann-Whitney test were used in a univariate analysis to determine the association between hearing impairment and the contributing risks being studied. Results: The results of the univariate analysis showed that hearing impairment was significantly (p<0.05) associated with older age, lower education level, high smoking dose, high occupational daily noise dose, longer duration of service, infrequent used of hearing protection device (HPD), and low perception of sound on HPD usage. Multivariate logistic regression of hearing impairment after controlling for age found the following five variables: occupational daily noise dose ≥50% (OR 3.48, 95% CI 1.36-8.89), ≥15 years of services (OR 2.92, 95% CI 1.16-7.33), infrequent use of HPD (OR 2.79, 95% CI 1.15-6.77), low perception of sound on HPD (POR 2.77, 95% CI 1.09-6.97), and smoking more than 20 packs per year (OR 4.71, 95% CI 1.13-19.68). Discussion: In conclusion, high occupational noise exposure level, longer duration of service, low perception of sound on HPD, infrequent used of HPD, and smoking more than 20 packs per year were the contributing factors to hearing impairment, and appropriate intervention measures should be proposed and taken into considerations. PMID:27488035

  1. Environmental Education and Hearing-Impaired Pupils.

    ERIC Educational Resources Information Center

    Lock, Roger

    1998-01-01

    Reports on a way in which student teachers are given the opportunity to work with partially hearing pupils in the context of environmental education outside the science laboratory. Focuses on student investigation of moss on trees. (DDR)

  2. Recognition of sine wave modeled consonants by normal hearing and hearing-impaired individuals

    NASA Astrophysics Data System (ADS)

    Balachandran, Rupa

    Sine wave modeling is a parametric tool for representing the speech signal with a limited number of sine waves. It involves replacing the peaks of the speech spectrum with sine waves and discarding the rest of the lower amplitude components during synthesis. It has the potential to be used as a speech enhancement technique for hearing-impaired adults. The present study answers the following basic questions: (1) Are sine wave synthesized speech tokens more intelligible than natural speech tokens? (2) What is the effect of varying the number of sine waves on consonant recognition in quiet? (3) What is the effect of varying the number of sine waves on consonant recognition in noise? (4) How does sine wave modeling affect the transmission of speech feature in quiet and in noise? (5) Are there differences in recognition performance between normal hearing and hearing-impaired listeners? VCV syllables representing 20 consonants (/p/, /t/, /k/, /b/, /d/, /g/, /f/, /theta/, /s/, /∫/, /v/, /z/, /t∫/, /dy/, /j/, /w/, /r/, /l/, /m/, /n/) in three vowel contexts (/a/, /i/, /u/) were modeled with 4, 8, 12, and 16 sine waves. A consonant recognition task was performed in quiet, and in background noise (+10 dB and 0 dB SNR). Twenty hearing-impaired listeners and six normal hearing listeners were tested under headphones at their most comfortable listening level. The main findings were: (1) Recognition of unprocessed speech was better that of sine wave modeled speech. (2) Asymptotic performance was reached with 8 sine waves in quiet for both normal hearing and hearing-impaired listeners. (3) Consonant recognition performance in noise improved with increasing number of sine waves. (4) As the number of sine waves was decreased, place information was lost first, followed by manner, and finally voicing. (5) Hearing-impaired listeners made more errors then normal hearing listeners, but there were no differences in the error patterns made by both groups.

  3. Hearing Impairment Is Associated with Smaller Brain Volume in Aging

    PubMed Central

    Rigters, Stephanie C.; Bos, Daniel; Metselaar, Mick; Roshchupkin, Gennady V.; Baatenburg de Jong, Robert J.; Ikram, M. Arfan; Vernooij, Meike W.; Goedegebure, André

    2017-01-01

    Although recent studies show that age-related hearing impairment is associated with cerebral changes, data from a population perspective are still lacking. Therefore, we studied the relation between hearing impairment and brain volume in a large elderly cohort. From the population-based Rotterdam Study, 2,908 participants (mean age 65 years, 56% female) underwent a pure-tone audiogram to quantify hearing impairment. By performing MR imaging of the brain we quantified global and regional brain tissue volumes (total brain volume, gray matter volume, white matter (WM) volume, and lobe-specific volumes). We used multiple linear regression models, adjusting for age, sex, head size, time between hearing test and MR imaging, and relevant cognitive and cardiovascular covariates. Furthermore, we performed voxel-based morphometry to explore sub-regional differences. We found that a higher pure-tone threshold was associated with a smaller total brain volume [difference in standardized brain volume per decibel increase in hearing threshold in the age-sex adjusted model: -0.003 (95% confidence interval -0.004; -0.001)]. Specifically, WM volume was associated. Both associations were more pronounced in the lower frequencies. All associations were consistently present in all brain lobes in the lower frequencies and in most lobes in the higher frequencies, and were independent of cognitive function and cardiovascular risk factors. In voxel-based analyses we found associations of hearing impairment with smaller white volumes and some smaller and larger gray volumes, yet these were statistically non-significant. Our findings demonstrate that hearing impairment in elderly is related to smaller total brain volume, independent of cognition and cardiovascular risk factors. This mainly seems to be driven by smaller WM volume, throughout the brain. PMID:28163683

  4. Hearing-Impaired Children in Venezuela: 1985.

    ERIC Educational Resources Information Center

    Schildroth, Arthur; And Others

    1987-01-01

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

  5. Balance assessment in hearing-impaired children.

    PubMed

    Walicka-Cupryś, Katarzyna; Przygoda, Łukasz; Czenczek, Ewelina; Truszczyńska, Aleksandra; Drzał-Grabiec, Justyna; Zbigniew, Trzaskoma; Tarnowski, Adam

    2014-11-01

    According to the scientific reports the postural stability is inseparably associated with hearing organ's correct functioning. The aim of the study was to evaluate the degree of disorders occurring in balance reactions in this group of children with profound hearing loss compared to their healthy peers. The study worked with a total of 228 children, including 65 who are deaf (DCH) and 163 subjects without any hearing deficits (CON) in the control group. Stabilometric measurements were performed with the use of a force distribution platform. The results indicate statistically significant differences in terms of one parameter (the total path length) recorded in the test with the eyes open and a whole range of parameters recorded when the subjects had their eyes closed (the width, height, and area of the ellipse, the total path length, and the horizontal and vertical sway). The study results showed better values of the static balance parameters in deaf children as compared to their peers without hearing disorders and the differences were particularly evident in the test with the subject's eyes closed. The results suggest significantly better processing of sensory stimuli in postural reactions particularly from propioception, and to a lesser extent, from the vision system observed in the subjects as compared to their peers in the control group.

  6. An investigation of the career development of high school adolescents with hearing impairments in New Zealand.

    PubMed

    Furlonger, B

    1998-07-01

    Although the need for better-skilled workers has been signaled by the marketplace, people with hearing impairments generally are employed in unskilled or semiskilled jobs. They are, therefore, at heightened risk of unemployment and underemployment. Compounding this risk are the levels of vocational preparation and job awareness of adolescents with hearing impairments, which are generally considered inadequate. With a view to improving prevocational programs for hearing impaired adolescents, the researcher collected information on career awareness and vocational maturity. Significant differences were identified between hearing impaired and hearing adolescents on a range of career measures. In particular, adolescents with hearing impairments were identified as having less career awareness.

  7. Communication and special health care needs of a profoundly hearing impaired adolescent.

    PubMed

    Chacko, M R; Buttler, J T; Kirkland, R T

    1987-08-01

    This report describes the authors' experience in providing medical and contraceptive care to a profoundly hearing impaired adolescent in a hospital setting. Ways to communicate effectively with the hearing impaired are described.

  8. Achieving effective hearing aid fitting within one month after identification of childhood permanent hearing impairment.

    PubMed

    Bastanza, G; Gallus, R; De Carlini, M; Picciotti, P M; Muzzi, E; Ciciriello, E; Orzan, E; Conti, G

    2016-02-01

    Diagnosis of child permanent hearing impairment (PHI) can be made with extreme timeliness compared to the past thanks to improvements in PHI identification through newborn hearing screening programmes. It now becomes essential to provide an effective amplification as quickly as possible in order to restore auditory function and favour speech and language development. The early fitting of hearing aids and possible later cochlear implantation indeed prompts the development of central auditory pathways, connections with secondary sensory brain areas, as well as with motor and articulatory cortex. The aim of this paper is to report the results of a strategic analysis that involves identification of strengths, weaknesses, opportunities and threats regarding the process of achieving early amplification in all cases of significant childhood PHI. The analysis is focused on the Italian situation and is part of the Italian Ministry of Health project CCM 2013 "Preventing Communication Disorders: a Regional Program for Early Identification, Intervention and Care of Hearing Impaired Children".

  9. Prevalence of mitochondrial gene mutations among hearing impaired patients

    PubMed Central

    Usami, S.; Abe, S.; Akita, J.; Namba, A.; Shinkawa, H.; Ishii, M.; Iwasaki, S.; Hoshino, T.; Ito, J.; Doi, K.; Kubo, T.; Nakagawa, T.; Komiyama, S.; Tono, T.; Komune, S.

    2000-01-01

    The frequency of three mitochondrial point mutations, 1555A→G, 3243A→G, and 7445A→G, known to be associated with hearing impairment, was examined using restriction fragment length polymorphism (RFLP) analysis in two Japanese groups: (1) 319 unrelated SNHL outpatients (including 21 with aminoglycoside antibiotic injection history), and (2) 140 cochlear implantation patients (including 22 with aminoglycoside induced hearing loss). Approximately 3% of the outpatients and 10% of the cochlear implantation patients had the 1555A→G mutation. The frequency was higher in the patients with a history of aminoglycoside injection (outpatient group 33%, cochlear implantation group 59%). One outpatient (0.314%) had the 3243A→G mutation, but no outpatients had the 7445A→G mutation and neither were found in the cochlear implantation group. The significance of the 1555A→G mutation, the most prevalent mitochondrial mutation found in this study of a hearing impaired population in Japan, among subjects with specific backgrounds, such as aminoglycoside induced hearing loss, is evident.


Keywords: mitochondria; point mutation; hearing impairment; frequencies PMID:10633132

  10. Functional analysis of a nonsyndromic hearing loss-associated mutation in the transmembrane II domain of the GJC3 gene

    PubMed Central

    Wong, Swee-Hee; Wang, Wen-Hung; Chen, Pin-Hua; Li, Shuan-Yow; Yang, Jiann-Jou

    2017-01-01

    In a previous study, we identified a novel missense mutation, p.W77S, in the GJC3 gene encoding connexin30.2/connexin31.3 (CX30.2/CX31.3) from patients with hearing loss. The functional alteration of CX30.2/CX31.3 caused by the p.W77S mutant of GJC3 gene, however, remains unclear. In the current study, our result indicated that the p.W77 is localized at the second membrane-spanning segments (TM2) and near border of the E1 domain of the CX30.2/CX31.3 protein and highly conserved (Conseq score = 8~9) in all species. The p.W77S missense mutation proteins in the intracellular distribution are different CX30.2/CX31.3WT and an accumulation of the mutant protein in the endoplasmic reticulum (ER) of the HeLa cell. Furthermore, co-expression of WT and p.W77S mutant chimerae proteins showed that the heteromeric connexon accumulated in the cytoplasm, thereby impairing the WT proteins' expression in the cell membranes. In addition, we found that CX30.2/CX31.3W77S missense mutant proteins were degraded by lysosomes and proteosomes in the transfected HeLa cell. Based on these findings, we suggest that p.W77S mutant has a dominant negative effect on the formation and function of the gap junction. These results give a novel molecular elucidation for the mutation of GJC3 in the development of hearing loss. PMID:28367085

  11. Otitis Media and Children with Hearing Impairments: A Sequela.

    ERIC Educational Resources Information Center

    Beattie, R. G. John L.

    1991-01-01

    This review of research on otitis media in children with existing hearing impairments concludes that the incidence of otitis media is probably higher than in the general population. The possible reasons for higher incidence, effects of otitis media on children with known losses, and the role of the classroom teacher are discussed. (Author/DB)

  12. A Resource Guide for Teachers of Young Hearing Impaired Children.

    ERIC Educational Resources Information Center

    Capitol Region Education Council, West Hartford, CT.

    Directed to teachers of young hearing impaired children, the guide attempts to avoid a step-by-step approach to language acquisition and undue emphasis on grammatical form. Instead, the teacher is viewed as a guide who leads the children to more sources of information and understanding and encourages curiosity, spontaneity, and creativity. Content…

  13. Kindergarten Curriculum for Children with Hearing Impairments: Jordanian Teachers' Perspectives

    ERIC Educational Resources Information Center

    Al-Zboon, Eman

    2016-01-01

    This study describes a kindergarten curriculum for children with hearing impairments, from their teachers' perspectives. Qualitative research data from interviews with 20 teachers were analysed using content analysis methodology. The results pinpoint a collection of proposed curriculum components (i.e. a general framework and outcomes document;…

  14. A Hearing-Impaired Child's Acquisition of Schemata: Something's Missing.

    ERIC Educational Resources Information Center

    Yoshinaga-Itano, Christine; Downey, Doris M.

    1986-01-01

    The difficulties hearing impaired students experience in acquiring the conceptual information underlying narratives is discussed in terms of schemata development and the role of incidental learning. Principles for teaching concepts and labels, elaborating the schema, using questions to fill in conceptual gaps, and using imaginary play and…

  15. Auditory Threshold Variability with Severely Hearing-Impaired Preschool Children.

    ERIC Educational Resources Information Center

    Fulton, Robert T.

    1979-01-01

    Threshold variability across repeated measures (N=10) was observed at 250 and 1,000 Hz with five severely hearing-impaired preschool children. Results indicated that variability at 1,000 Hz was within a 10 dB range (except for one measure) across Ss, while variability at 250 Hz was substantially larger, even though false positive responses to…

  16. Hemispheric Specialization and Implications for Education of the Hearing Impaired.

    ERIC Educational Resources Information Center

    Levine, Shari

    The paper reviews research on cerebral hemispheric functioning and considers the implications for instruction of the deaf. The nature of right and left hemisphere function and communication is addressed, as are findings on cerebral asymmetry for aspects of language processing. Studies on hemispheric specialization of hearing impaired persons…

  17. Guidelines for Science Programs for Hearing Impaired Adolescents.

    ERIC Educational Resources Information Center

    Mertens, Donna M.

    This evaluation study examined the implementation of the Marine Science Young Scholars Program, which provided 32 gifted deaf and hearing-impaired adolescents with a 4-week summer enrichment program in 1988 and 1989. The instructional program: used a cognitively based curriculum; included labs, lectures, and field experiences; promoted one-to-one…

  18. Vocabulary Developing Strategies Applied to Individuals with Hearing Impairments

    ERIC Educational Resources Information Center

    Karasu, Guzin; Girgin, Umit; Uzuner, Yildiz; Kaya, Zehranur

    2016-01-01

    The general purpose of this research was to investigate the strategies utilized for vocabulary development of ten individuals (first year college students) in Graphic Art Department, School for The Handicapped, Anadolu University with hearing impairment. The reflective and cyclical data were consisted of videotape recordings of the actual lessons,…

  19. Parenting and the Hearing Impaired: Attachment and Coping.

    ERIC Educational Resources Information Center

    Leigh, Irene W.

    1987-01-01

    The author provides parents with information and suggestions about ways to alleviate strains placed on the natural attachment (bonding) process when their child has a hearing impairment. Strategies to foster the child's communicative and behavioral development are presented and the roles of parental responsivity, letting go, and fathers are…

  20. Phonological Space in the Speech of the Hearing Impaired.

    ERIC Educational Resources Information Center

    Shukla, R. S.

    1989-01-01

    First and second formant frequencies of the vowels /a/, /i/, and /u/ were measured to determine the phonological space in the speech of 30 Kannada-speaking hearing-impaired individuals in India. Compared to controls, subjects' phonological space was found to be reduced, primarily due to the lowering of the second formant of the vowel /i/.…

  1. E-Learning Environment for Hearing Impaired Students

    ERIC Educational Resources Information Center

    Hashim, Hisyamuddin; Tasir, Zaidatun; Mohamad, Siti Khadijah

    2013-01-01

    The usage of technology within the educational department has become more vital by each year passing. One of the most popular technological approaches used is the e-learning environment. The usage of e-learning environment in education involves a wide range of types of students, and this includes the hearing impaired ones. Some adjustment or…

  2. Hearing Impaired (HI) Support Services and Caseload Prioritisation

    ERIC Educational Resources Information Center

    Rodd, Cathy; Young, Alys

    2009-01-01

    This study considers the process of prioritisation undertaken by Hearing Impaired (HI) Support Services in England in a context of change driven by early screening, early intervention and reform in children's services. The aim of prioritisation is to identify the relative needs of deaf children and their families fairly, transparently and…

  3. Narrative Skills Following Early Confirmation of Permanent Childhood Hearing Impairment

    ERIC Educational Resources Information Center

    Worsfold, Sarah; Mahon, Merle; Yuen, Ho Ming; Kennedy, Colin

    2010-01-01

    Aim: The aim of this study was to compare spoken language production in children with permanent childhood hearing impairment (PCHI) whose PCHI was confirmed either early or late. Method: Audio-taped spoken narrative was assessed for syntax, phonology, morphology, and narrative in transcripts from a population-based sample of 89 children (49 males,…

  4. 38 CFR 4.85 - Evaluation of hearing impairment.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... determine a Roman numeral designation (I through XI) for hearing impairment based on a combination of the percent of speech discrimination (horizontal rows) and the puretone threshold average (vertical columns... entitled to special monthly compensation due either to deafness, or to deafness in combination with...

  5. Microelectronic Technology and the Hearing Impaired: The Future. Keynote Address.

    ERIC Educational Resources Information Center

    Thorkildsen, Ron

    1985-01-01

    The potential of microelectronic technology for alleviating communication problems of hearing-impaired persons is discussed from a futuristic point of view. The need for computer literacy training is related to changing career opportunities. Computer literacy, artificial intelligence, and videodisc technology are described and related to training…

  6. An Update of Classroom Acoustics for Children with Hearing Impairment.

    ERIC Educational Resources Information Center

    Crandell, Carl C.; Smaldino, Joseph J.

    1994-01-01

    This study examined ambient noise levels, reverberation times, and noise/reverberation reduction treatments in 32 classrooms utilized for students with hearing impairment. None of the classrooms met recommended acoustical criteria for ambient noise levels, and only nine rooms complied with recommended standards for reverberation. (Author/DB)

  7. Couples' attributions about behaviours modulated by hearing impairment: links with relationship satisfaction.

    PubMed

    Anderson, Donnah L; Noble, William

    2005-04-01

    The aim of the study was to examine the link between the nature of attributions made by partners about behaviours modulated by hearing loss, and overall relationship satisfaction. Forty-three hearing-impaired males and 23 hearing-impaired females together with their partners, each rated the hearing-impaired person's hearing ability, their own relationship satisfaction, and their attributions for hypothetical behaviours typically associated with impaired hearing. Attributions covered perceived causes of behaviour and perceived responsibility. The extent to which the behaviours typically associated with impaired hearing, were attributed to personal causes and responsibilities was inversely correlated with relationship satisfaction. Female partners (more than male partners, or females with impaired hearing) made attributions that accentuated the unpleasant implications of behaviour. Where attributions and ratings of degree of hearing loss were greater in partners than in the person with the loss, relationship satisfaction was lower. Implications for rehabilitation counselling are discussed.

  8. Laryngeal Aerodynamics in Children with Hearing Impairment versus Age and Height Matched Normal Hearing Peers.

    PubMed

    Das, Barshapriya; Chatterjee, Indranil; Kumar, Suman

    2013-01-01

    Lack of proper auditory feedback in hearing-impaired subjects results in functional voice disorder. It is directly related to discoordination of intrinsic and extrinsic laryngeal muscles and disturbed contraction and relaxation of antagonistic muscles. A total of twenty children in the age range of 5-10 years were considered for the study. They were divided into two groups: normal hearing children and hearing aid user children. Results showed a significant difference in the vital capacity, maximum sustained phonation, and fast adduction abduction rate having equal variance for normal and hearing aid user children, respectively, but no significant difference was found in the peak flow value with being statistically significant. A reduced vital capacity in hearing aid user children suggests a limited use of the lung volume for speech production. It may be inferred from the study that the hearing aid user children have poor vocal proficiency which is reflected in their voice. The use of voicing component in hearing impaired subjects is seen due to improper auditory feedback. It was found that there was a significant difference in the vital capacity, maximum sustained phonation (MSP), and fast adduction abduction rate and no significant difference in the peak flow.

  9. 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…

  10. The Learning Achievement in Thai Language for Hearing Impaired Students in Thailand

    ERIC Educational Resources Information Center

    Nuttaya, Iam-Khong; Surachai, Suksakulchai; Wacheerapan, Kaewprapan

    2011-01-01

    The development of language skills, reading and writing, is very important for hearing impaired students. However, there is no evident about the current language proficiency of Thai hearing impaired students. Therefore, the purposes of this research were to compare the language achievement of Thai hearing impaired students with the national…

  11. Anger Expression Styles of Hearing Impaired Individuals Doing Sport and Those Not Doing Sport

    ERIC Educational Resources Information Center

    Altin, Mehmet

    2015-01-01

    The aim of this research was to determine the anger expression styles between the sportive hearing impaired individuals and the sedentary hearing impaired individuals. In the sportive hearing impaired group, there were 170 participants: 62 females and 108 males doing basketball, volleyball and football teams as licensed sportsmen in various clubs…

  12. Hearing Impaired Preservice Teachers' Experiences: A Qualitative Study of Perceived Barriers.

    ERIC Educational Resources Information Center

    Otis-Wilborn, Amy K.; Paddock, Lisa

    1989-01-01

    This study is part of a longitudinal research endeavor to investigate hearing-impaired students' experience in teacher training. The first phase, reported here, examines qualitatively-assessed barriers three hearing-impaired students encountered during acclimation to the training program for teachers of the hearing impaired at one university. (PB)

  13. 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

  14. Mutation in the Hair Cell Specific Gene POU4F3 Is a Common Cause for Autosomal Dominant Nonsyndromic Hearing Loss in Chinese Hans

    PubMed Central

    He, Longxia; Pang, Xiuhong; Chen, Penghui

    2016-01-01

    Autosomal dominant nonsyndromic hearing loss (ADNSHL) is extremely heterogeneous. So far the genetic etiological contribution of the gene POU4F3 associated with ADNSHL has been rarely reported. In our previous study, a c.603_604delGG mutation in the hair cell specific gene POU4F3 has been identified as the pathogenic cause in one of the seven Chinese Han ADNSHL families. In the present study, we performed targeted next-generation sequencing of 144 known deafness genes in another nine Chinese Han ADNSHL families and identified two more novel mutations in POU4F3, p.Leu311Pro and c.120+1G>C, as the pathogenic cause. Clinical characterization of the affected individuals in these three families showed that the three POU4F3 mutations may lead to progressive hearing loss with variable ages of onset and degrees of severity. Our results suggested that mutations in POU4F3 are a relatively common cause (3/16) for ADNSHL in Chinese Hans, which should be routinely screened in such cases during genetic testing. PMID:28053790

  15. Spanish hearing impairment inventory for the elderly.

    PubMed

    López-Vázquez, Mónica; Orozco, José Antonio; Jiménez, Graciela; Berruecos, Pedro

    2002-06-01

    Self-assessment tools have proven to be useful in everyday practice in the audiology field, mostly in developed countries. There is a lack of such tests in Spanish. Our objective was to construct an inventory that could help us to identify elderly non-institutionalized patients who need an audiological assessment; we did not intend to identify or qualify emotional or social/situational reactions towards hearing handicap. As a prototype we initially translated, adapted and standardized the Hearing Handicap Inventory for the Elderly. This questionnaire was given to 60 elderly subjects. The performance on each question was compared with the audiometric results. After selecting useful and suitable questions, modifying some others and eliminating those with a poor performance, a new inventory in Spanish was developed. This final version was then tested in 63 elderly subjects. Very good scores were obtained for each question. An 'easy-to-use' rule is proposed in order to identify those patients who need to be audiologically tested. A brief meta-linguistic analysis is made on semantic and cultural factors that contributed to good translation and cultural adaptation.

  16. Hearing impairment and nightmares: a theoretical insight.

    PubMed

    Rego, Maria Francisca; Duarte, Ivone; Nunes, Rui

    2015-01-01

    The aim of this article is to address the issue of nightmares in the deaf population, given that there are no documented studies on this matter to the best of our knowledge. The study of nightmares in the deaf population is of high relevance given their specific characteristics (impossibility of verbalisation) and the lack of studies with this population. Nightmares are dreams of negative content that trigger an awakening associated with a rapid return to a full state of alert and a persistent feeling of anxiety and fear, which may cause significant distress. Various studies show that the deaf population has dreams with more negative imagery and emotions, are more exposed to interpersonal traumas and have higher rates of dissociation, than hearing people. These concepts seem to be connected given that, in the presence of traumatic events, dissociation may act as a defence mechanism and nightmares may operate as an adaptive coping strategy.

  17. 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

  18. Cochlear microphonic potential recorded by transtympanic electrocochleography in normally-hearing and hearing-impaired ears

    PubMed Central

    Santarelli, R; Scimemi, P; Dal Monte, E; Arslan, E

    2006-01-01

    Summary The cochlear microphonic is a receptor potential believed to be generated primarily by outer hair cells. Its detection in surface recordings has been considered a distinctive sign of outer hair cell integrity in patients with auditory neuropathy. This report focuses on the results of an analysis performed on cochlear microphonic recorded by transtympanic electrocochleography in response to clicks in 502 subjects with normal hearing threshold or various degrees of hearing impairment, and in 20 patients with auditory neuropathy. Cochlear microphonics recorded in normally-hearing and hearing-impaired ears showed amplitudes decreasing by the elevation of compound action potential Cochlear microphonic responses were clearly detected in ears with profound hearing loss. After separating recordings according to the presence or absence of central nervous system pathology (CNS+ and CNS-, respectively), cochlear microphonic amplitude was significantly higher in CNS+ than in CNS- subjects with normally-hearing ears and at 70 dB nHL compound action potential threshold. Cochlear microphonic responses were detected in all auditory neuropathy patients, with similar amplitudes and thresholds to those calculated for normally-hearing CNS- subjects. Cochlear microphonic duration was significantly higher in auditory neuropathy and normally-hearing CNS+ patients compared to CNS- subjects. Our results show that: 1. cochlear microphonic detection is not a distinctive feature of auditory neuropathy; 2. CNS+ subjects showed enhancement in cochlear microphonic amplitude and duration, possibly due to efferent system dysfunction; 3. long-lasting, high frequency cochlear microphonics with amplitudes comparable to those obtained from CNS- ears were found in auditory neuropathy patients. This could result from a variable combination of afferent compartment lesion, efferent system dysfacilitation and loss of outer hair cells. PMID:16886850

  19. The Relationship between Word and Stress Pattern Recognition Ability and Hearing Level in Hearing-Impaired Young Adults.

    ERIC Educational Resources Information Center

    Jackson, Pamela; Kelly-Ballweber, Denise

    1986-01-01

    The relationship between word and stress pattern recognition ability and hearing level was explored by administering the Children's Auditory Test to hearing-impaired young adults (N=27). For word recognition, subjects with average hearing loss between 85 and 100 decibels demonstrated a wide range of performance not predictable from their…

  20. Hearing impairment in Parkinson's disease: expanding the nonmotor phenotype.

    PubMed

    Vitale, Carmine; Marcelli, Vincenzo; Allocca, Roberto; Santangelo, Gabriella; Riccardi, Pasquale; Erro, Roberto; Amboni, Marianna; Pellecchia, Maria Teresa; Cozzolino, Autilia; Longo, Katia; Picillo, Marina; Moccia, Marcello; Agosti, Valeria; Sorrentino, G; Cavaliere, Michele; Marciano, Elio; Barone, Paolo

    2012-10-01

    The objective of this study was to evaluate hearing impairment in patients affected by Parkinson's disease compared with hearing scores observed in normal age- and sex-matched controls. One hundred eighteen consecutive patients with a clinical diagnosis of Parkinson's disease were screened. Severity of motor symptoms and staging were measured with the Unified Parkinson's Disease Rating Scale (section III) and the Hoehn and Yahr scale. Audiometric evaluation consisted of a comprehensive audiologic case history and questionnaire, visual otoscopic examination, acoustic immittance measures (tympanogram and acoustic reflexes), pure tone audiometry, and measurement of brain stem auditory-evoked potentials. Healthy age- and sex-matched subjects were selected as the control group. One hundred six of 118 patients were enrolled. Pure tone audiometry revealed age-dependent high-frequency hearing loss in patients with Parkinson's disease compared with both normative values and values for healthy age- and sex-matched controls (75/106 [71%], χ(2) = 5.959, P = .02; 92/106 [86.8%] vs 60/106 [56.6%], χ(2) = 23.804, P < .001, respectively). Pure tone audiometry scores correlated with Hoehn and Yahr scale scores (P < .05). Brain stem auditory-evoked potentials were normal in all patients. Our patients with Parkinson's disease showed age-dependent peripheral, unilateral, or bilateral hearing impairment. Whether these auditory deficits are intrinsic to Parkinson's disease or secondary to a more complex impaired processing of sensorial inputs occurring over the course of illness remains to be determined. Because α-synuclein is located predominately in the efferent neuronal system within the inner ear, it could affect susceptibility to noise-induced hearing loss or presbycusis. It is feasible that the natural aging process combined with neurodegenerative changes intrinsic to Parkinson's disease might interfere with cochlear transduction mechanisms, thus anticipating presbycusis.

  1. Acoustics and sociolinguistics: Patterns of communication in hearing impairing classrooms

    NASA Astrophysics Data System (ADS)

    McKellin, William; Shahin, Kimary; Jamieson, Janet; Hodgson, Murray; Pichora-Fuller, Kathleen

    2005-04-01

    In elementary school classes, noise during student led activities is often taken as evidence of successful interaction and learning. In this complex social environment of elementary school classrooms, acquisition of complex language and social skills-the focus of activities in early education-is expected to take place in hearing-hostile environments. Communication and language processing in these contexts requires interactive strategies, discourse forms, and syntactic structures different from the educationally desired forms used in acoustically advantageous environments. Recordings were made of the interaction of groups of students in grades 1-3, 5, and 7 during collaborative group work in their regular classrooms. Each student wore microphones at the ear level and head-mounted video cameras. Each group as a whole was also audio- and videotaped and noise level readings were recorded. Analysis of the acoustical and phonological properties of language heard by each student has demonstrated that the language variety used in these noisy and reverberant settings is similar to that of individuals with hearing impairments. This paper reports similarities between the syntactic structures and pragmatic strategies used by hearing impaired children and normally hearing children in noisy contexts. [Work supported by Peter Wall Institute for Advanced Studies, University of British Columbia.

  2. Correlates of syntactic abilities in hearing-impaired students.

    PubMed

    Clarke, B R; Rogers, W T

    1981-03-01

    Total scores on the recently developed Screening Test from the Test of Syntactic Abilities for 382 hearing-impaired subjects between eight and 19 years and in various educational programs were found to be significantly related to hearing threshold level, number of multiple handicaps, age, educational setting, method of communication, and hearing aid usage. Multivariate analysis of variance on the effect of age controlled for hearing loss showed no significant increase in scores after eleven years of age, thus lending support to the thesis that the capacity to acquire language may cease to function at about puberty. The results of stepwise multiple regression analyses showed that, when personal variables were first forced to enter the equation, degree of hearing loss, multiple handicaps, and age accounted for 14%, nine %, an four % of the explained variability, respectively. Over and above these contributions, two manipulable variables--educational setting (a surrogate for integration) and method of communication--added significantly a further 12% and three % to the explained variability in syntactic ability.

  3. The Relationship of Audibility and the Development of Canonical Babbling in Young Children with Hearing Impairment

    ERIC Educational Resources Information Center

    Bass-Ringdahl, Sandie M.

    2010-01-01

    This article investigated the relationship between age at onset of canonical babbling and audibility of amplified speech in children with hearing impairment. Thirteen children with severe-profound hearing impairment and two children with normal hearing participated in a longitudinal investigation of vocalization development. A nonconcurrent…

  4. 45 CFR 1308.11 - Eligibility criteria: Hearing impairment including deafness.

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... amplification, and learning is affected. A child is classified as hard of hearing who has a permanent or fluctuating hearing impairment which adversely affects learning; or (b) Meets the legal criteria for being... hearing loss can include impaired listening skills, delayed language development, and...

  5. Form and Meaning in the Written Language of Hearing-Impaired Children.

    ERIC Educational Resources Information Center

    Yoshinaga-Itano, Christine; Snyder, Lynn

    1985-01-01

    Semantic discourse features of written narratives of 49 hearing impaired children (10-15 years old) were examined in an analysis of the relationship between form and meaning in the writing of both hearing and hearing impaired Ss. Syntactic and semantic written language growth appear to be qualitatively and quantitatively different in the groups.…

  6. Implementation and Evaluation of Computer-Aided Mandarin Phonemes Training System for Hearing-Impaired Students

    ERIC Educational Resources Information Center

    Yang, Hui-Jen; Lay, Yun-Long

    2005-01-01

    A computer-aided Mandarin phonemes training (CAMPT) system was developed and evaluated for training hearing-impaired students in their pronunciation of Mandarin phonemes. Deaf or hearing-impaired people have difficulty hearing their own voice, hence most of them cannot learn how to speak. Phonemes are the basis for learning to read and speak in…

  7. 45 CFR 1308.11 - Eligibility criteria: Hearing impairment including deafness.

    Code of Federal Regulations, 2012 CFR

    2012-10-01

    ... fluctuating hearing loss caused by otitis media, allergies, or eardrum perforations and other outer or middle... hearing loss can include impaired listening skills, delayed language development, and articulation... 45 Public Welfare 4 2012-10-01 2012-10-01 false Eligibility criteria: Hearing impairment...

  8. Positive Experiences and Life Aspirations among Adolescents with and without Hearing Impairments.

    ERIC Educational Resources Information Center

    Magen, Zipora

    1990-01-01

    Comparison of 79 normally hearing and 42 hearing-impaired adolescents found no differences regarding the intensity of their remembered positive experiences. Hearing-impaired subjects reported more positive interpersonal experiences, rarely experienced positive experiences "with self," and showed less desire for transpersonal commitment,…

  9. Cigarette Smoking Causes Hearing Impairment among Bangladeshi Population

    PubMed Central

    Sumit, Ahmed Faisal; Das, Anindya; Sharmin, Zinat; Ahsan, Nazmul; Ohgami, Nobutaka; Kato, Masashi; Akhand, Anwarul Azim

    2015-01-01

    Lifestyle including smoking, noise exposure with MP3 player and drinking alcohol are considered as risk factors for affecting hearing synergistically. However, little is known about the association of cigarette smoking with hearing impairment among subjects who carry a lifestyle without using MP3 player and drinking alcohol. We showed here the influence of smoking on hearing among Bangladeshi subjects who maintain a lifestyle devoid of using MP3 player and drinking alcohol. A total of 184 subjects (smokers: 90; non-smokers: 94) were included considering their duration and frequency of smoking for conducting this study. The mean hearing thresholds of non-smoker subjects at 1, 4, 8 and 12 kHz frequencies were 5.63±2.10, 8.56±5.75, 21.06±11.06, 40.79±20.36 decibel (dB), respectively and that of the smokers were 7±3.8, 13.27±8.4, 30.66±12.50 and 56.88±21.58 dB, respectively. The hearing thresholds of the smokers at 4, 8 and 12 kHz frequencies were significantly (p<0.05) higher than those of the non-smokers, while no significant differences were observed at 1 kHz frequency. We also observed no significant difference in auditory thresholds among smoker subgroups based on smoking frequency. In contrast, subjects smoked for longer duration (>5 years) showed higher level of auditory threshold (62.16±19.87 dB) at 12 kHz frequency compared with that (41.52±19.21 dB) of the subjects smoked for 1-5 years and the difference in auditory thresholds was statistically significant (p<0.0002). In this study, the Brinkman Index (BI) of smokers was from 6 to 440 and the adjusted odds ratio showed a positive correlation between hearing loss and smoking when adjusted for age and body mass index (BMI). In addition, age, but not BMI, also played positive role on hearing impairment at all frequencies. Thus, these findings suggested that cigarette smoking affects hearing level at all the frequencies tested but most significantly at extra higher frequencies. PMID:25781179

  10. A Novel Frameshift Mutation of SLC26A4 in a Korean Family With Nonsyndromic Hearing Loss and Enlarged Vestibular Aqueduct

    PubMed Central

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

    2017-01-01

    Objectives We aimed to identify the causative mutation for siblings in a Korean family with nonsyndromic hearing loss (HL) and enlarged vestibular aqueduct (EVA). The siblings were a 19-year-old female with bilateral profound HL and an 11-year-old male with bilateral moderately severe HL. Methods We extracted genomic DNA from blood samples of the siblings with HL, their parents, and 100 controls. We performed mutation analysis for SLC26A4 using direct sequencing. Results The two siblings were compound heterozygotes with the novel mutation p.I713LfsX8 and the previously described mutation p.H723R. Their parents had heterozygous mono-allelic mutations. Father had p.I713LfsX8 mutation as heterozygous, and mother had p.H723R mutation as heterozygous. However, novel mutation p.I713LfsX8 was not detected in 100 unrelated controls. Conclusion Both mutations identified in this study were located in the sulfate transporter and anti-sigma factor antagonist domain, the core region for membrane targeting of SulP/SLC26 anion transporters, which strongly suggests that failure in membrane trafficking by SLC26A4 is a direct cause of HL in this family. Our study could therefore provide a foundation for further investigations elucidating the SLC26A4-related mechanisms of HL. PMID:27384033

  11. 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

  12. 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.

  13. The effect of symmetrical and asymmetrical hearing impairment on music quality perception.

    PubMed

    Cai, Yuexin; Zhao, Fei; Chen, Yuebo; Liang, Maojin; Chen, Ling; Yang, Haidi; Xiong, Hao; Zhang, Xueyuan; Zheng, Yiqing

    2016-09-01

    The purpose of this study was to investigate the effect of symmetrical, asymmetrical and unilateral hearing impairment on music quality perception. Six validated music pieces in the categories of classical music, folk music and pop music were used to assess music quality in terms of its 'pleasantness', 'naturalness', 'fullness', 'roughness' and 'sharpness'. 58 participants with sensorineural hearing loss [20 with unilateral hearing loss (UHL), 20 with bilateral symmetrical hearing loss (BSHL) and 18 with bilateral asymmetrical hearing loss (BAHL)] and 29 normal hearing (NH) subjects participated in the present study. Hearing impaired (HI) participants had greater difficulty in overall music quality perception than NH participants. Participants with BSHL rated music pleasantness and naturalness to be higher than participants with BAHL. Moreover, the hearing thresholds of the better ears from BSHL and BAHL participants as well as the hearing thresholds of the worse ears from BSHL participants were negatively correlated to the pleasantness and naturalness perception. HI participants rated the familiar music pieces higher than unfamiliar music pieces in the three music categories. Music quality perception in participants with hearing impairment appeared to be affected by symmetry of hearing loss, degree of hearing loss and music familiarity when they were assessed using the music quality rating test (MQRT). This indicates that binaural symmetrical hearing is important to achieve a high level of music quality perception in HI listeners. This emphasizes the importance of provision of bilateral hearing assistive devices for people with asymmetrical hearing impairment.

  14. Update of the spectrum of GJB2 mutations in 107 patients with nonsyndromic hearing loss in the Fujian population of China.

    PubMed

    Chen, Tianbin; Jiang, Ling; Liu, Can; Shan, Hongyan; Chen, Jing; Yang, Bin; Ou, Qishui

    2014-05-01

    Mutations in the GJB2 gene, encoding connexin26, which is expressed in the inner ear, have been shown to be responsible for the majority of nonsyndromic hearing loss (NSHL) cases. To update and evaluate the spectrum and prevalence of GJB2 mutations in the Fujian population, we screened exon 2 (coding), exon 1, and flanking introns of GJB2 in 107 NSHL probands and 61 individuals with normal hearing. Twelve different variants were identified, including three pathogenic mutations (c.235delC, c.299_300delAT, and c.508insAACG), one hypomorphic allele (p.V37I), three polymorphic variants (p.V27I, p.E114G, and p.I230T), and five rare variants (p.N62N, p.F115C, p.T123N, p.G21E, and p.F142I). The p.G21E and p.F142I variants were potentially pathogenic as predicted by PolyPhen-2, SIFT, and PROVEAN. The most common mutation was c.235delC with allele frequency 12.6% (27/214). The most common polymorphisms in the Fujian population were p.V27I and p.E114G, both detected at high frequency in probands and controls. The p.E114G variant was always in cis with p.V27I, and formed the haplotype, p.[V27I; E114G] in the Fujian population. Interestingly, only 17.76% (19/107) of NSHL probands had clearly defined pathogenic mutations in GJB2, indicating that the pathogenesis of NSHL in the Fujian population is heterogenous, and that further analysis of other NSHL genes is necessary.

  15. Preliminary evaluation of a light based Contact Hearing Device for the hearing impaired

    PubMed Central

    Fay, Jonathan P.; Perkins, Rodney; Levy, Suzanne Carr; Nilsson, Michael; Puria, Sunil

    2013-01-01

    Objective To assess the safety, stability, and performance of the broad spectrum, light based Contact Hearing Device (CHD) on listeners with hearing impairment. Study Design Feasibility study. Setting Single-Site Research and Development Facility. Subjects Thirteen subjects with symmetric mild to severe sensorineural hearing impairment had the CHD placed bilaterally. Intervention A custom-molded light activated Tympanic Contact Actuator (TCA) was placed into each ear by a physician, where it stayed in contact with the umbo and a portion of the medial wall of the ear canal for four months. Each CHD was calibrated and programmed to provide appropriate broad-spectrum amplification. Main Outcome Measures Safety was determined through routine otologic examinations. Aided and pre-TCA-insertion unaided audiometric thresholds, Reception Threshold for Sentences (RTS), and Abbreviated Profile of Hearing Aid Benefit (APHAB) measurements were made to characterize system performance as well as the benefits of amplification via the CHD. Results The TCAs remained on subjects’ ears for an average total of 122 days, without causing signs of inflammation or infection, and there were no serious device-related adverse events. Measured average maximum output of 90–110 dB SPL in the 0.25–10 kHz range, average maximum gain before feedback of 40 dB, and functional gain through 10 kHz show extended bandwidth broad spectrum output and gain. RTS results showed significant aided improvements of up to 2.8 dB, and APHAB results showed clinically significant aided benefits in 11/12 (92%) subjects. Conclusion The safety, stability, and performance demonstrated in this initial 4-month study suggest that the CHD may offer a feasible way of providing broad-spectrum amplification appropriate to treat listeners with mild to severe hearing impairment. PMID:23524632

  16. [Organization of medico-genetic counseling for hereditary hearing impairment].

    PubMed

    Markova, T G

    2009-01-01

    High frequency of hereditary pathologies resulting in congenital and prelingual auditory inefficiency necessitates organization of readily available medico-genetic counseling services based at specialized centres and an integrated dispensary system for the observation of families at risk of hereditary hearing impairment and identification of high-risk groups among the general population. Correct clinical diagnosis provides a basis for the strategy of molecular-genetic examination of a given patient and members of his (her) family. Results of the examination can be used for family planning and the choice of measures for the prevention of recurrent cases. The objective of the present work was to draw up the main lines of cooperative activities of deafness specialists and geneticists, propose indications for medico-genetic counseling, and distinguish groups at high risk of development of hereditary hearing impairment among the affected subjects and in the general population. It is shown that the efficiency of measures for the prevention of hereditary hearing loss is directly related to the availability of medico-genetic aid and the degree of involvement of risk groups in the examination.

  17. Multisensory dysfunction accompanies crossmodal plasticity following adult hearing impairment.

    PubMed

    Meredith, M A; Keniston, L P; Allman, B L

    2012-07-12

    Until now, cortical crossmodal plasticity has largely been regarded as the effect of early and complete sensory loss. Recently, massive crossmodal cortical reorganization was demonstrated to result from profound hearing loss in adult ferrets (Allman et al., 2009a). Moderate adult hearing loss, on the other hand, induced not just crossmodal reorganization, but also merged new crossmodal inputs with residual auditory function to generate multisensory neurons. Because multisensory convergence can lead to dramatic levels of response integration when stimuli from more than one modality are present (and thereby potentially interfere with residual auditory processing), the present investigation sought to evaluate the multisensory properties of auditory cortical neurons in partially deafened adult ferrets. When compared with hearing controls, partially-deaf animals revealed elevated spontaneous levels and a dramatic increase (∼2 times) in the proportion of multisensory cortical neurons, but few of which showed multisensory integration. Moreover, a large proportion (68%) of neurons with somatosensory and/or visual inputs was vigorously active in core auditory cortex in the absence of auditory stimulation. Collectively, these results not only demonstrate multisensory dysfunction in core auditory cortical neurons from hearing impaired adults but also reveal a potential cortical substrate for maladaptive perceptual effects such as tinnitus.

  18. Installation and impact of sound field systems on hearing and hearing impaired children and their teachers

    NASA Astrophysics Data System (ADS)

    Dockrell, Julie; Rigby, Kate; Shield, Bridget; Carey, Anne

    2005-04-01

    An evaluation of the installation and use of sound field systems in ten schools in England has been carried out. The evaluation included noise surveys of classrooms, questionnaire surveys of pupils and teachers and experimental testing of children with and without the use of SFS. The aim of this project was to investigate the impact of SFS on teaching and learning in elementary school classrooms, in particular, to ascertain whether the SFS differentially benefited children with hearing impairments. Barriers to teachers use of SFS were found in terms of equipment placement and maintenance, appropriate training, and teacher's knowledge. Nonetheless positive reports are recorded from both teachers and pupils. Teachers' and pupils' perceptions are compared with objective data evaluating change in performance when SFS are used for language and cognitive tasks. Data from children with hearing impairments and additional learning needs are analyzed for comparative purposes. The results are discussed in terms of effective practice for the use of SFS with elementary school pupils.

  19. An instructional guide for reducing the stress of hearing parents of hearing-impaired children.

    PubMed

    Adams, J W; Tidwell, R

    1989-12-01

    The impact of a self-instructional program to reduce stress levels of parents of hearing-impaired children is examined. Fifty parents with hearing-impaired children were randomly assigned to one of two groups: Participating Parents, who received a Self-Instructional Parent Program (SIPP); and Non-participating Parents, who received no such program. Findings indicate that the participating parents did not experience significant decreases in their stress levels or in the perceived incidence of misbehavior in their children in comparison with non-participating parents. Participating parents, however, reported a decrease in their children's misbehavior; non-participating parents noted an increase. Additionally, the findings also associated increasing stress on the part of parents with parental perceptions of burgeoning misbehavior on the part of the child.

  20. The Investigation of Physical Performance Status of Visually and Hearing Impaired Applying Judo Training Program

    ERIC Educational Resources Information Center

    Karakoc, Onder

    2016-01-01

    It was aimed to investigate the physical performances of visually and hearing impaired doing judo training in this study. 32 male athletes, who were doing judo training, volunteer and, visually and hearing impaired, participated in this study. The investigation was applied to visually impaired (N = 12, mean ± SD; age: 25.75 ± 3.55 years, height:…

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

    PubMed

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

    2015-08-01

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

  2. Evaluation of the sparse coding shrinkage noise reduction algorithm in normal hearing and hearing impaired listeners.

    PubMed

    Sang, Jinqiu; Hu, Hongmei; Zheng, Chengshi; Li, Guoping; Lutman, Mark E; Bleeck, Stefan

    2014-04-01

    Although there are numerous single-channel noise reduction strategies to improve speech perception in noise, most of them improve speech quality but do not improve speech intelligibility, in circumstances where the noise and speech have similar frequency spectra. Current exceptions that may improve speech intelligibility are those that require a priori knowledge of the speech or noise statistics, which limits practical application. Hearing impaired (HI) listeners suffer more in speech intelligibility than normal hearing listeners (NH) in the same noisy environment, so developing better single-channel noise reduction algorithms for HI listeners is justified. Our model-based "sparse coding shrinkage" (SCS) algorithm extracts key speech information in noisy speech. We evaluate it by comparison with a state-of-the-art Wiener filtering approach using speech intelligibility tests with NH and HI listeners. The model-based SCS algorithm relies only on statistical signal information without prior information. Results show that the SCS algorithm improves speech intelligibility in stationary noise and is comparable to the Wiener filtering algorithm. Both algorithms improve intelligibility for HI listeners but not for NH listeners. Improvement is less in fluctuating (babble) noise than in stationary noise. Both noise reduction algorithms perform better at higher input signal-to-noise ratios (SNR) where HI listeners can benefit but where NH listeners have already reached ceiling performance. The difference between NH and HI subjects in intelligibility gain depends fundamentally on the input SNR rather than the hearing loss level. We conclude that HI listeners need different signal processing algorithms from NH subjects and that the SCS algorithm offers a promising alternative to Wiener filtering. Performance of all noise reduction algorithms is likely to vary according to extent of hearing loss and algorithms that show little benefit for listeners with moderate hearing loss may

  3. 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.

  4. Whole Exome Sequencing Reveals Homozygous Mutations in RAI1, OTOF, and SLC26A4 Genes Associated with Nonsyndromic Hearing Loss in Altaian Families (South Siberia)

    PubMed Central

    Karafet, Tatiana M.; Morozov, Igor V.; Mikhalskaia, Valeriia Yu.; Zytsar, Marina V.; Bondar, Alexander A.

    2016-01-01

    Hearing loss (HL) is one of the most common sensorineural disorders and several dozen genes contribute to its pathogenesis. Establishing a genetic diagnosis of HL is of great importance for clinical evaluation of deaf patients and for estimating recurrence risks for their families. Efforts to identify genes responsible for HL have been challenged by high genetic heterogeneity and different ethnic-specific prevalence of inherited deafness. Here we present the utility of whole exome sequencing (WES) for identifying candidate causal variants for previously unexplained nonsyndromic HL of seven patients from four unrelated Altaian families (the Altai Republic, South Siberia). The WES analysis revealed homozygous missense mutations in three genes associated with HL. Mutation c.2168A>G (SLC26A4) was found in one family, a novel mutation c.1111G>C (OTOF) was revealed in another family, and mutation c.5254G>A (RAI1) was found in two families. Sanger sequencing was applied for screening of identified variants in an ethnically diverse cohort of other patients with HL (n = 116) and in Altaian controls (n = 120). Identified variants were found only in patients of Altaian ethnicity (n = 93). Several lines of evidences support the association of homozygosity for discovered variants c.5254G>A (RAI1), c.1111C>G (OTOF), and c.2168A>G (SLC26A4) with HL in Altaian patients. Local prevalence of identified variants implies possible founder effect in significant number of HL cases in indigenous population of the Altai region. Notably, this is the first reported instance of patients with RAI1 missense mutation whose HL is not accompanied by specific traits typical for Smith-Magenis syndrome. Presumed association of RAI1 gene variant c.5254G>A with isolated HL needs to be proved by further experimental studies. PMID:27082237

  5. Coordination of Gaze and Speech in Communication between Children with Hearing Impairment and Normal-Hearing Peers

    ERIC Educational Resources Information Center

    Sandgren, Olof; Andersson, Richard; van de Weijer, Joost; Hansson, Kristina; Sahlén, Birgitta

    2014-01-01

    Purpose: To investigate gaze behavior during communication between children with hearing impairment (HI) and normal-hearing (NH) peers. Method: Ten HI-NH and 10 NH-NH dyads performed a referential communication task requiring description of faces. During task performance, eye movements and speech were tracked. Using verbal event (questions,…

  6. Binaural speech discrimination under noise in hearing-impaired listeners

    NASA Technical Reports Server (NTRS)

    Kumar, K. V.; Rao, A. B.

    1988-01-01

    This paper presents the results of an assessment of speech discrimination by hearing-impaired listeners (sensori-neural, conductive, and mixed groups) under binaural free-field listening in the presence of background noise. Subjects with pure-tone thresholds greater than 20 dB in 0.5, 1.0 and 2.0 kHz were presented with a version of the W-22 list of phonetically balanced words under three conditions: (1) 'quiet', with the chamber noise below 28 dB and speech at 60 dB; (2) at a constant S/N ratio of +10 dB, and with a background white noise at 70 dB; and (3) same as condition (2), but with the background noise at 80 dB. The mean speech discrimination scores decreased significantly with noise in all groups. However, the decrease in binaural speech discrimination scores with an increase in hearing impairment was less for material presented under the noise conditions than for the material presented in quiet.

  7. Affective Properties of Mothers' Speech to Infants with Hearing Impairment and Cochlear Implants

    ERIC Educational Resources Information Center

    Kondaurova, Maria V.; Bergeson, Tonya R.; Xu, Huiping; Kitamura, Christine

    2015-01-01

    Purpose: The affective properties of infant-directed speech influence the attention of infants with normal hearing to speech sounds. This study explored the affective quality of maternal speech to infants with hearing impairment (HI) during the 1st year after cochlear implantation as compared to speech to infants with normal hearing. Method:…

  8. Mothers' Speech to Hearing-Impaired Infants and Children with Cochlear Implants

    ERIC Educational Resources Information Center

    Bergeson, Tonya R.; Miller, Rachel J.; McCune, Kasi

    2006-01-01

    This study investigated the effects of age, hearing loss, and cochlear implantation on mothers' speech to infants and children. We recorded normal-hearing (NH) mothers speaking to their children as they typically would do at home and speaking to an adult experimenter. Nine infants (10-37 months) were hearing-impaired and had used a cochlear…

  9. Members of Faculty with Hearing Impairments in Academia: What Are Their Needs?

    ERIC Educational Resources Information Center

    Roufs, Kathleen S.

    2011-01-01

    Seventeen percent of adults in the United States suffer from some degree of hearing loss, and this impairment can pose considerable personal, professional, social, and psychological challenges, often, to people reluctant to seek help (Hearing Loss Association, 2011). Post-secondary faculty members with hearing loss are among us, and most of them…

  10. 45 CFR 1308.11 - Eligibility criteria: Hearing impairment including deafness.

    Code of Federal Regulations, 2014 CFR

    2014-10-01

    ... fluctuating hearing loss caused by otitis media, allergies, or eardrum perforations and other outer or middle... hearing loss can include impaired listening skills, delayed language development, and articulation... frequently, and receive speech, language or hearing services as indicated by the IEPs. As soon as...

  11. 45 CFR 1308.11 - Eligibility criteria: Hearing impairment including deafness.

    Code of Federal Regulations, 2013 CFR

    2013-10-01

    ... fluctuating hearing loss caused by otitis media, allergies, or eardrum perforations and other outer or middle... hearing loss can include impaired listening skills, delayed language development, and articulation... frequently, and receive speech, language or hearing services as indicated by the IEPs. As soon as...

  12. 45 CFR 1308.11 - Eligibility criteria: Hearing impairment including deafness.

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... fluctuating hearing loss caused by otitis media, allergies, or eardrum perforations and other outer or middle... hearing loss can include impaired listening skills, delayed language development, and articulation... frequently, and receive speech, language or hearing services as indicated by the IEPs. As soon as...

  13. Screening of Cognitive Function and Hearing Impairment in Older Adults: A Preliminary Study

    PubMed Central

    Wong, Lena Lar Nar; Yu, Joannie Ka Yin; Chan, Shaina Shing; Tong, Michael Chi Fai

    2014-01-01

    Background. Previous research has found that hearing loss is associated with poorer cognitive function. The question is that when a hearing impairment is being compensated for by appropriately fitted monaural hearing aids, special precautions are still needed when screening cognitive function in older adults. Objective. This research examined cognitive function in elderly hearing aid users who used monaural hearing aids and whether the presence of a hearing impairment should be accounted for when screening cognitive function in these individuals. Methods. Auditory thresholds, sentence reception thresholds, and self-reported outcomes with hearing aids were measured in 34 older hearing aid users to ensure hearing aids were appropriately fitted. Mini-Mental State Examination (MMSE) results obtained in these participants were then compared to normative data obtained in a general older population exhibiting similar demographic characteristics. Stepwise multiple regression analyses were used to examine the effects of demographic and auditory variables on MMSE scores. Conclusions. Results showed that, even with appropriately fitted hearing aids, cognitive decline was significant. Besides the factors commonly measured in the literature, we believed that auditory deprivation was not being fully compensated for by hearing aids. Most importantly, screening of cognitive function should take into account the effects of hearing impairment, even when hearing devices have been appropriately fitted. PMID:25140321

  14. 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

  15. Homozygosity for the V37I GJB2 mutation in fifteen probands with mild to moderate sensorineural hearing impairment: further confirmation of pathogenicity and haplotype analysis in Asian populations.

    PubMed

    Gallant, Emily; Francey, Lauren; Tsai, Ellen A; Berman, Micah; Zhao, Yaru; Fetting, Heather; Kaur, Maninder; Deardorff, Matthew A; Wilkens, Alisha; Clark, Dinah; Hakonarson, Hakon; Rehm, Heidi L; Krantz, Ian D

    2013-09-01

    Hearing impairment affects 1 in 650 newborns, making it the most common congenital sensory impairment. Autosomal recessive nonsyndromic sensorineural hearing impairment (ARNSHI) comprises 80% of familial hearing impairment cases. Mutations in GJB2 account for a significant number of ARNSHI (and up to 50% of documented recessive (e.g., more than 1 affected sibling) hearing impairment in some populations). Mutations in the GJB2 gene are amongst the most common causes of hearing impairment in populations of various ethnic backgrounds. Two mutations of this gene, 35delG and 167delT, account for the majority of reported mutations in Caucasian populations, especially those of Mediterranean and Ashkenazi Jewish background. The 235delC mutation is most prevalent in East Asian populations. Some mutations are of less well-characterized significance. The V37I missense mutation, common in Asian populations, was initially described as a polymorphism and later as a potentially pathogenic mutation. We report here on 15 unrelated individuals with ARNSHI and homozygosity for the V37I GJB2 missense mutation. Nine individuals are of Chinese ancestry, two are of unspecified Asian descent, one is of Japanese descent, one individual is of Vietnamese ancestry, one of Philippine background and one of Italian and Cuban/Caucasian background. Homozygosity for the V37I GJB2 mutation may be a more common pathogenic missense mutation in Asian populations, resulting in mild to moderate sensorineural hearing impairment. We report a presumed haplotype block specific to East Asian individuals with the V37I mutation encompassing the GJB2 gene that may account for the high prevalence in East Asian populations.

  16. Contemporary Issues in Phoneme Production by Hearing-Impaired Persons: Physiological and Acoustic Aspects.

    ERIC Educational Resources Information Center

    McGarr, Nancy S.; Whitehead, Robert

    1992-01-01

    This paper on physiologic correlates of speech production in children and youth with hearing impairments focuses specifically on the production of phonemes and includes data on respiration for speech production, phonation, speech aerodynamics, articulation, and acoustic analyses of speech by hearing-impaired persons. (Author/DB)

  17. 38 CFR 21.152 - Interpreter service for the hearing impaired.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 2 2013-07-01 2013-07-01 false Interpreter service for... Employment Under 38 U.S.C. Chapter 31 Special Rehabilitation Services § 21.152 Interpreter service for the hearing impaired. (a) General. The main purpose of interpreter service for the hearing impaired is...

  18. 38 CFR 21.152 - Interpreter service for the hearing impaired.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 2 2012-07-01 2012-07-01 false Interpreter service for... Employment Under 38 U.S.C. Chapter 31 Special Rehabilitation Services § 21.152 Interpreter service for the hearing impaired. (a) General. The main purpose of interpreter service for the hearing impaired is...

  19. 38 CFR 21.152 - Interpreter service for the hearing impaired.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 2 2014-07-01 2014-07-01 false Interpreter service for... Employment Under 38 U.S.C. Chapter 31 Special Rehabilitation Services § 21.152 Interpreter service for the hearing impaired. (a) General. The main purpose of interpreter service for the hearing impaired is...

  20. 38 CFR 21.152 - Interpreter service for the hearing impaired.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 2 2011-07-01 2011-07-01 false Interpreter service for... Employment Under 38 U.S.C. Chapter 31 Special Rehabilitation Services § 21.152 Interpreter service for the hearing impaired. (a) General. The main purpose of interpreter service for the hearing impaired is...

  1. Teachers' Knowledge of the Relationship of Auditory Acuity and Hearing Impairment to Reading.

    ERIC Educational Resources Information Center

    Marshall, Evelyn Myrtle

    Teacher's knowledge of the relationship of auditory acuity and hearing impairment to reading was evaluated with 60 teachers and specialists (regular classroom primary and upper elementary teachers, reading teachers, teachers of the learning disabled, teachers of the hearing impaired, and speech and language pathologists). Ss were administered a…

  2. Opening the World of Television for the Hearing Impaired Through Closed Caption.

    ERIC Educational Resources Information Center

    Caldwell, Doris C.

    A producer of captioned films for the deaf describes the role of The Public Broadcasting System (PBS) in providing captioned television programming for the hearing impaired. It is explained that PBS has been working on a closed-caption production and transmission system for the hearing impaired. Recounted are Federal Communications Commission…

  3. A Review of Self-Esteem of the Hearing Impaired Football Players

    ERIC Educational Resources Information Center

    Açak, Mahmut; Kaya, Oktay

    2016-01-01

    The current study aimed at reviewing the level of self-esteem of the hearing impaired football players. The sample of the study was composed of 95 football players who played in the 1st hearing impaired football league. To gather the study-data; a Personal Information Form and Self-esteem Scale were used. The data obtained were analyzed through…

  4. The Influence of Speaking Rate on Nasality in the Speech of Hearing-Impaired Individuals

    ERIC Educational Resources Information Center

    Dwyer, Claire H.; Robb, Michael P.; O'Beirne, Greg A.; Gilbert, Harvey R.

    2009-01-01

    Purpose: The purpose of this study was to determine whether deliberate increases in speaking rate would serve to decrease the amount of nasality in the speech of severely hearing-impaired individuals. Method: The participants were 11 severely to profoundly hearing-impaired students, ranging in age from 12 to 19 years (M = 16 years). Each…

  5. Causes and Prevalence of Antisocial Behaviour among Students with Hearing Impairment in Ibadan, Nigeria

    ERIC Educational Resources Information Center

    Ojo, Isaiah Olugbenga

    2015-01-01

    This study examined the causes and prevalence of antisocial behaviour among secondary school students with hearing impairment in Ibadan, Nigeria. Descriptive survey research design was adopted to carry out the study. Purposive sampling technique was used to select 60 students with hearing impairment from Methodist Grammar School (Deaf Unit),…

  6. Attrition among Hearing-Impaired College Students in the U.S.

    ERIC Educational Resources Information Center

    Walter, Gerard G.; DeCaro, James J.

    The growth in enrollments of hearing-impaired students at colleges and universities indicates that the issue of accessibility is being addressed; however, it is not clear whether institutions have made adequate accommodations to meet the communicative and educational handicaps imposed by severe to profound hearing impairment. A survey was…

  7. Hearing Impaired Adolescents' Signed and Written Expression of Locative State Relations.

    ERIC Educational Resources Information Center

    Kempt, Donna; Maxwell, Madeline M.

    1989-01-01

    Analysis of hearing-impaired adolescents' signed and written sentences expressing simple locative state relations found noun reversal and pragmatic focus errors in 7 percent of signed and 15 percent of written responses. Most errors were produced by profoundly hearing-impaired signers attending public day school. (Author/CB)

  8. Analyzing the Subjective Consciousness of the Hearing-Impaired Students in Fine-Art Teaching

    ERIC Educational Resources Information Center

    Gao, Yang

    2009-01-01

    Initiative plays an important role in special fine art education. Teachers should take into full consideration the profile of the hearing-impaired students. For better teaching achievement, it is the teachers who shoulder the responsibility to activate the subjective role and consciousness of the hearing-impaired students by arousing their sense…

  9. A Practical Guide to Play, Piaget, and Language in Preprimary Hearing Impaired Programs.

    ERIC Educational Resources Information Center

    Smith, Dawn

    This detailed curriculum guide describes a model program (the Preschool Hearing Impaired Support Center) implemented in the Detroit Public Schools' preprimary classes for the hearing impaired. The auditory-oral approach is based on the developmental philosophy of Jean Piaget and on principles of regular early childhood education programs. Section…

  10. Use of the Kaufman-Assessment Battery for Children with the Hearing Impaired.

    ERIC Educational Resources Information Center

    Ulissi, Stephen Mark; And Others

    1989-01-01

    Fifty hearing-impaired elementary-school students completed the Kaufman-Assessment Battery for Children; scores correlated highly with scores from the Wechsler Intelligence Scale for Children-Revised and with school achievement. The Simultaneous Processing Scale and the Nonverbal Scale seemed appropriate for hearing-impaired children while the…

  11. Transition from School to Work for Youth with Hearing Impairments in Taiwan.

    ERIC Educational Resources Information Center

    Lin, Hung-Chih

    This paper examines job development and vocational training for persons with hearing impairments in Taiwan and reports on a survey of 26 individuals just graduated or about to graduate with hearing impairment. Discussion includes an historical overview and examination of the concept of transition in Taiwan and transition to employment for people…

  12. Development and Evaluation of Computer-Aided Music-Learning System for the Hearing Impaired

    ERIC Educational Resources Information Center

    Yang, H.-J.; Lay, Y.-L.; Liou, Y.-C.; Tsao, W.-Y.; Lin, C.-K.

    2007-01-01

    A computer-assisted music-learning system (CAMLS) has been developed to help the hearing impaired practice playing a musical melody. The music-learning performance is evaluated to test the usability of the system. This system can be a computer-supported learning tool for the hearing impaired to help them understand what pitch and tempo are, and…

  13. Perception of Quality of Life for Adults with Hearing Impairment in the LGBT Community

    ERIC Educational Resources Information Center

    Kelly-Campbell, Rebecca J.; Atcherson, Samuel R.

    2012-01-01

    The purpose of the this study was to examine the perception of both generic and disease-specific quality of life (QoL) in adults with hearing impairment who are members of the lesbian, gay, bisexual, and transgender (LGBT) community. Eighty-three adults who self-identified as having hearing impairment and as being members of the LGBT community and…

  14. Unique Approach to Dental Management of Children with Hearing Impairment

    PubMed Central

    Renahan, Navanith; Kumaran, Parvathy; Xavier, Arun M

    2017-01-01

    The number of deaf children has dramatically increased in the past few decades. These children present to the pediatric dentist a unique set of challenges mostly pertaining to the establishment of communication with them. There have been very few attempts in the past to break down these challenges and formulate a strategy on how to manage them effectively. This is a case report of a child who was successfully managed using two different modes of communication. Finally, the advantages and disadvantages are mentioned, and a common strategy incorporating the positives of both the methods has been devised. How to cite this article Renahan N, Varma RB, Kumaran P, Xavier AM. Unique Approach to Dental Management of Children with Hearing Impairment. Int J Clin Pediatr Dent 2017;10(1):107-110. PMID:28377666

  15. Professional Attributes in Teacher Preparation for Teaching Students with Hearing Impairment: Implications for Inclusive Education in Nigeria

    ERIC Educational Resources Information Center

    Onu, V. C.; Eskay, M. K.; Ugwuanyi, L.; Igbo, J. N.; Obiyo, N. O.

    2012-01-01

    This descriptive survey research study assessed the professional attributes of teacher preparation for teaching students with hearing impairment. It discussed the role of teaching competence in ensuring the smooth delivery of lessons to children with special needs, especially the hearing-impaired. THAQ (Teaching Hearing-impaired Assessment…

  16. Identifying the needs of elderly, hearing-impaired persons: the importance and utility of hearing aid attributes.

    PubMed

    Meister, Hartmut; Lausberg, Isabel; Kiessling, Juergen; von Wedel, Hasso; Walger, Martin

    2002-11-01

    Older patients represent the majority of hearing-aid users. The needs of elderly, hearing-impaired subjects are not entirely identified. The present study aims to determine the importance of fundamental hearing-aid attributes and to elicit the utility of associated hypothetical hearing aids for older patients. This was achieved using a questionnaire-based conjoint analysis--a decompositional approach to preference measurement offering a realistic study design. A random sample of 200 experienced hearing-aid users participated in the study. Though three out of the six examined attributes revealed age-related dependencies, the only significant effect was found for the attribute "handling", which was considerably more important for older than younger hearing-aid users. A trend of decreasing importance of speech intelligibility in noise and increasing significance of speech in quiet was observed for subjects older than 70 years. In general, the utility of various hypothetical hearing aids was similar for older and younger subjects. Apart from the attribute "handling", older and younger subjects have comparable needs regarding hearing-aid features. On the basis of the examined attributes, there is no requirement for hearing aids designed specifically for elderly hearing-aid users, provided that ergonomic features are considered and the benefits of modern technology are made fully available for older patients.

  17. Autosomal recessive nonsyndromic deafness genes: a review.

    PubMed

    Duman, Duygu; Tekin, Mustafa

    2012-06-01

    More than 50 Percent of prelingual hearing loss is genetic in origin, and of these up to 93 Percent are monogenic autosomal recessive traits. Some forms of genetic deafness can be recognized by their associated syndromic features, but in most cases, hearing loss is the only finding and is referred to as nonsyndromic deafness. To date, more than 700 different mutations have been identified in one of 42 genes in individuals with autosomal recessive nonsyndromic hearing loss (ARNSHL). Reported mutations in GJB2, encoding connexin 26, makes this gene the most common cause of hearing loss in many populations. Other relatively common deafness genes include SLC26A4, MYO15A, OTOF, TMC1, CDH23, and TMPRSS3. In this report we summarize genes and mutations reported in families with ARNSHL. Founder effects were demonstrated for some recurrent mutations but the most significant findings are the extreme locus and allelic heterogeneity and different spectrum of genes and mutations in each population.

  18. Decision strategies of hearing-impaired listeners in spectral shape discrimination

    NASA Astrophysics Data System (ADS)

    Lentz, Jennifer J.; Leek, Marjorie R.

    2002-03-01

    The ability to discriminate between sounds with different spectral shapes was evaluated for normal-hearing and hearing-impaired listeners. Listeners detected a 920-Hz tone added in phase to a single component of a standard consisting of the sum of five tones spaced equally on a logarithmic frequency scale ranging from 200 to 4200 Hz. An overall level randomization of 10 dB was either present or absent. In one subset of conditions, the no-perturbation conditions, the standard stimulus was the sum of equal-amplitude tones. In the perturbation conditions, the amplitudes of the components within a stimulus were randomly altered on every presentation. For both perturbation and no-perturbation conditions, thresholds for the detection of the 920-Hz tone were measured to compare sensitivity to changes in spectral shape between normal-hearing and hearing-impaired listeners. To assess whether hearing-impaired listeners relied on different regions of the spectrum to discriminate between sounds, spectral weights were estimated from the perturbed standards by correlating the listener's responses with the level differences per component across two intervals of a two-alternative forced-choice task. Results showed that hearing-impaired and normal-hearing listeners had similar sensitivity to changes in spectral shape. On average, across-frequency correlation functions also were similar for both groups of listeners, suggesting that as long as all components are audible and well separated in frequency, hearing-impaired listeners can use information across frequency as well as normal-hearing listeners. Analysis of the individual data revealed, however, that normal-hearing listeners may be better able to adopt optimal weighting schemes. This conclusion is only tentative, as differences in internal noise may need to be considered to interpret the results obtained from weighting studies between normal-hearing and hearing-impaired listeners.

  19. 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.

  20. 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.

  1. [The problems of hearing impairment in the flying staff of commercial aviation in Russia].

    PubMed

    Pankova, V B; Bushmanov, A Y U

    2014-01-01

    The authors discuss the problems pertaining to the growing incidence of hearing impairment in the members of the flying staff employed in commercial aviation of Russia and the main criteria used to elucidate the causes behind occupational diseases of the organs of hearing. Special attention is given to the principal normative documents regulating the methodological basis on which the acoustic factor in the aircraft cockpit is evaluated, peculiarities of occupational sensorineural hearing impairment and the methods for its detection. The main errors in the determination of the relationship between the working conditions and the diseases of the organs of hearing are discussed.

  2. Prevalence and Evolutionary Origins of the del(GJB6-D13S1830) Mutation in the DFNB1 Locus in Hearing-Impaired Subjects: a Multicenter Study

    PubMed Central

    del Castillo, Ignacio; Moreno-Pelayo, Miguel A.; del Castillo, Francisco J.; Brownstein, Zippora; Marlin, Sandrine; Adina, Quint; Cockburn, David J.; Pandya, Arti; Siemering, Kirby R.; Chamberlin, G. Parker; Ballana, Ester; Wuyts, Wim; Maciel-Guerra, Andréa Trevas; Álvarez, Araceli; Villamar, Manuela; Shohat, Mordechai; Abeliovich, Dvorah; Dahl, Hans-Henrik M.; Estivill, Xavier; Gasparini, Paolo; Hutchin, Tim; Nance, Walter E.; Sartorato, Edi L.; Smith, Richard J. H.; Van Camp, Guy; Avraham, Karen B.; Petit, Christine; Moreno, Felipe

    2003-01-01

    Mutations in GJB2, the gene encoding connexin-26 at the DFNB1 locus on 13q12, are found in as many as 50% of subjects with autosomal recessive, nonsyndromic prelingual hearing impairment. However, genetic diagnosis is complicated by the fact that 10%–50% of affected subjects with GJB2 mutations carry only one mutant allele. Recently, a deletion truncating the GJB6 gene (encoding connexin-30), near GJB2 on 13q12, was shown to be the accompanying mutation in ∼50% of these deaf GJB2 heterozygotes in a cohort of Spanish patients, thus becoming second only to 35delG at GJB2 as the most frequent mutation causing prelingual hearing impairment in Spain. Here, we present data from a multicenter study in nine countries that shows that the deletion is present in most of the screened populations, with higher frequencies in France, Spain, and Israel, where the percentages of unexplained GJB2 heterozygotes fell to 16.0%–20.9% after screening for the del(GJB6-D13S1830) mutation. Our results also suggest that additional mutations remain to be identified, either in DFNB1 or in other unlinked genes involved in epistatic interactions with GJB2. Analysis of haplotypes associated with the deletion revealed a founder effect in Ashkenazi Jews and also suggested a common founder for countries in Western Europe. These results have important implications for the diagnosis and counseling of families with DFNB1 deafness. PMID:14571368

  3. Phoneme recognition in modulated maskers by normal-hearing and aided hearing-impaired listeners.

    PubMed

    Phatak, Sandeep A; Grant, Ken W

    2012-09-01

    This study measured the influence of masker fluctuations on phoneme recognition. The first part of the study compared the benefit of masker modulations for consonant and vowel recognition in normal-hearing (NH) listeners. Recognition scores were measured in steady-state and sinusoidally amplitude-modulated noise maskers (100% modulation depth) at several modulation rates and signal-to-noise ratios. Masker modulation rates were 4, 8, 16, and 32 Hz for the consonant recognition task and 2, 4, 12, and 32 Hz for the vowel recognition task. Vowel recognition scores showed more modulation benefit and a more pronounced effect of masker modulation rate than consonant scores. The modulation benefit for word recognition from other studies was found to be more similar to the benefit for vowel recognition than that for consonant recognition. The second part of the study measured the effect of modulation rate on the benefit of masker modulations for vowel recognition in aided hearing-impaired (HI) listeners. HI listeners achieved as much modulation benefit as NH listeners for slower masker modulation rates (2, 4, and 12 Hz), but showed a reduced benefit for the fast masker modulation rate of 32 Hz.

  4. Hearing impairment and risk of Alzheimer's disease: a meta-analysis of prospective cohort studies.

    PubMed

    Zheng, Yuqiu; Fan, Shengnuo; Liao, Wang; Fang, Wenli; Xiao, Songhua; Liu, Jun

    2017-02-01

    Observational studies suggested an association between hearing impairment and cognitive disorders. However, whether hearing impairment is an independent risk factor or a harbinger of Alzheimer's disease remains controversial. Our goal was to assess the association between hearing impairment (HI) and the risk of Alzheimer's disease (AD) by conducting a meta-analysis of prospective cohort studies. We comprehensively searched the PubMed, Embase, Web of Science and Cochrane Library databases on January 19, 2016 to incorporate all the prospective cohort studies meeting the inclusion criteria to perform a systematic review and meta-analysis. Four prospective cohort studies with comparison between hearing impairment and normal hearing were incorporated, with 7461 participants. The outcomes of three studies were the incidence of Alzheimer's disease and the outcome of the fourth study was the incidence of mild cognitive impairment. The overall combined relative risk of people with hearing impairment to develop Alzheimer's disease was 4.87 (95% CI 0.90-26.35; p = 0.066), compared with the control group. Since both Alzheimer's disease and mild cognitive impairment are cognitive disorders, we incorporated all the four studies and the overall combined relative risk was 2.82 (95% CI 1.47-5.42; p = 0.002), indicating that the difference was significant. This meta-analysis suggests that hearing impairment significantly increases the risk of cognitive disorders and future well-designed prospective cohort studies are awaited to confirm the association between hearing impairment and risk of Alzheimer's disease.

  5. Assistive Hearing Technologies among Students with Hearing Impairment: Factors that Promote Satisfaction

    ERIC Educational Resources Information Center

    Rekkedal, Ann Mette

    2012-01-01

    Hearing technology can play an essential part in the education of deaf and hard-of-hearing children in inclusive schools. Few studies have examined these children's experiences with this technology. This article explores factors pertaining to children's use of and attitudes toward hearing technologies, such as hearing aids, cochlear implants,…

  6. The Prevalence and Characteristics of Tinnitus with Profound Sensori-Neural Hearing Impairment.

    ERIC Educational Resources Information Center

    Drukier, Gale S.

    1989-01-01

    Of 331 children (aged 6-18) with profound hearing impairment, 96 were found to have tinnitus. More females than males reported tinnitus. Most of the children with tinnitus were bothered to some degree by it and indicated that the noises adversely affected their ability to hear voices. (JDD)

  7. Characteristics of Successful Mainstreamed Hearing-Impaired Students: A Review of Selected Research.

    ERIC Educational Resources Information Center

    Saur, Rosemary E.; Stinson, Michael S.

    1986-01-01

    The literature review organizes research of the National Technical Institute for the Deaf concerning characteristics of successfully mainstreamed hearing-impaired students in terms of: family background; previous educational experience; cognitive skills; motivation; speech, hearing, and communication skills; previous course grades, lecture…

  8. Language and Literacy Development in Children Who Are Deaf or Hearing Impaired

    ERIC Educational Resources Information Center

    Briggle, Sandra J.

    2005-01-01

    Since the enactment of Public Law 94-142 in 1975, now referred to as the Individuals with Disabilities Education Act, more children who are deaf are attending public schools instead of residential schools for the deaf. Calculating how many children in public schools currently have a hearing loss is difficult because hearing impairment is not…

  9. Evaluation of Turkish Hearing Impaired Students' Reading Comprehension with the Miscue Analysis Inventory

    ERIC Educational Resources Information Center

    Girgin, Umit

    2006-01-01

    The purpose of the current study is to evaluate the reading comprehension of hearing impaired 8th graders who are being trained through an auditory-oral approach. The evaluation is conducted through miscue analysis using complex stories. To realize this aim, the following research questions are formulated. (1) To what extent do hearing impaired…

  10. An Investigation of the Career Development of High School Adolescents with Hearing Impairments in New Zealand.

    ERIC Educational Resources Information Center

    Furlonger, Brett

    1998-01-01

    A survey compared the career development and vocational maturity of 26 New Zealand adolescents with deafness and their hearing peers. Significant differences were identified between the two groups on career measures. In particular, adolescents with hearing impairments were identified as having less career awareness. Gender differences were also…

  11. School to Work: The Aberdeen Hearing-Impaired School-Leaver 1960-72

    ERIC Educational Resources Information Center

    Welsh, Jennifer

    1976-01-01

    A small scale survey of hearing-impaired graduates from Aberdeen schools was carried out in March 1973. It focused on the relation between types of schooling received, degree of hearing loss and levels of communication ability, employment satisfaction, training for work, promotion, and social attitudes. (Author/RK)

  12. Children's and Adolescents' Moral Emotion Attributions and Judgements about Exclusion of Peers with Hearing Impairments

    ERIC Educational Resources Information Center

    Chilver-Stainer, Jennifer; Gasser, Luciano; Perrig-Chiello, Pasqualina

    2014-01-01

    Children and adolescents with hearing impairments are at risk of being excluded from activities with hearing peers. Moral emotion attributions may represent important indicators for children's identification with the moral norm not to exclude peers based on disability. Against this background, we investigated how 10-, 12- and 15-year-olds…

  13. Wechsler Performance IQ Scores and Social Behaviors of Hearing-Impaired Students.

    ERIC Educational Resources Information Center

    Stewart, James H.

    1981-01-01

    Some significant relationships were established between certain observable behaviors and Wechsler performance test scores earned by 104 hearing-impaired persons, all students or former students at a state residential school for the deaf. (Author)

  14. Mother Goose Goes to School: Using Rhymes with ESL, Deaf and Hearing-Impaired Children.

    ERIC Educational Resources Information Center

    Dowd, Frances Smardo

    1991-01-01

    Because traditional Mother Goose rhymes accustom the ear and the tongue to the musical aspects of the English language, they are particularly valuable for English-as-a-Second-Language students, and for deaf and hearing-impaired children. (BB)

  15. Applying Microcomputer Technology to Investigations of Communication Skills in Severely Hearing Impaired Children.

    ERIC Educational Resources Information Center

    Fulton, Robert T.

    1982-01-01

    A computerized audiovisual communications system requires only a touch response by young difficult-to-test hearing impaired children. The system focuses on single word reading comprehension and sensory perception and interference. (CL)

  16. Study of reaction time to visual stimuli in athletes with and without a hearing impairment.

    PubMed

    Soto-Rey, Javier; Pérez-Tejero, Javier; Rojo-González, Jesús J; Reina, Raúl

    2014-08-01

    This study analyzes the differences in manual reaction time (RT) to visual stimuli in two samples of physically active persons: a group of athletes without hearing impairment (n = 79; M age = 22.6 yr., SD = 3.7) and a group of athletes with hearing impairment (n = 44, M age = 25.6 yr., SD = 5.0). Reaction time (RT) was measured and then differences between both groups were assessed by sex, type of sport (individual vs team sports), and competition level. RT to visual stimuli was significantly shorter for athletes with hearing impairment than for those without hearing impairment, with a significant sex difference (shorter RT for males), but no differences regarding type of sport or competition level. Suggestions for further research and sport applications are provided.

  17. Reported Hearing Impairment in Essential Tremor: A Population-Based Case-Control Study

    PubMed Central

    Benito-León, Julián; Louis, Elan D.; Bermejo-Pareja, Félix

    2008-01-01

    In a population-based sample, we determined whether a larger proportion of essential tremor (ET) cases reported hearing impairment compared with controls. Ninety-six (38.7%) of 248 ET cases versus 1,371 (29.4%) of 4,669 controls (p = 0.002) reported hearing impairment. In a logistic regression analysis adjusted for age, gender, educational level, depressive symptoms, and dementia, participants who reported hearing impairment were 30% more likely to suffer from ET than were controls (odds ratio 1.3; 95% confidence interval 1.01–1.7; p = 0.04). ET seemed to be associated with reported hearing impairment. The basis for this finding, which has been noted in several studies, deserves further exploration. PMID:18073494

  18. Mental Health of the People with Hearing Impairment in Korea: A Population-Based Cross-Sectional Study

    PubMed Central

    Shin, Hyun-Young

    2017-01-01

    Background The prevalence of hearing impairment is increasing and an association between hearing impairment and mental health has been reported. Our study aimed to determine the association between hearing impairment and mental health in Korea. Methods This was a cross-sectional study of data from the Korean National Health and Nutrition Examination Survey 2010–2013, with a sample size of 18,563 individuals (6,395 with hearing impairment and 12,168 without hearing impairment), aged ≥20 years. Results The female group with hearing impairment tended to have a higher rate of stress (odds ratio [OR], 1.32; 95% confidence interval [CI], 1.11 to 1.56). The association between hearing impairment and depressive symptoms was higher in elderly males (OR, 1.60; 95% CI, 1.10 to 2.32), while the association of hearing impairment with suicide ideation was higher in elderly females (OR, 1.32; 95% CI, 1.00 to 1.75). Conclusion Elderly individuals with hearing impairment are easily susceptible to poor mental health status. Early targeted intervention to address mental health problems is recommended for people with hearing impairment. PMID:28360980

  19. The Effect of Early Confirmation of Hearing Loss on the Behaviour in Middle Childhood of Children with Bilateral Hearing Impairment

    ERIC Educational Resources Information Center

    Stevenson, Jim; McCann, Donna C.; Law, Catherine M.; Mullee, Mark; Petrou, Stavros; Worsfold, Sarah; Yuen, Ho M.; Kennedy, Colin R.

    2011-01-01

    Aim: To determine if the benefit of early confirmation of permanent childhood hearing impairment (PCHI) on children's receptive language development is associated with fewer behavioural problems. Method: Follow-up of a total population cohort of 120 children with PCHI of moderate or greater severity (greater than or equal to 40 decibels relative…

  20. Motor control of speaking rate and oral diadochokinesis in hearing-impaired Farsi speakers.

    PubMed

    Seifpanahi, Sadegh; Dadkhah, Asghar; Dehqan, Ali; Bakhtiar, Mehdi; Salmalian, Tahmineh

    2008-01-01

    Although speech motor control has been studied intensively in normal-hearing and hearing-impaired (HI) speakers in America and Europe, essentially no research has been performed using Persian-speaking participants. A total of 46 prelingual hearing-impaired 15-18-year-old males and 15 normally hearing control participants from Iran participated in the study. Three speaking performance measures, oral diadochokinesis (DDK), speaking rate (words per minute), and intelligibility ratings, were obtained for the two groups and compared to previously published research for English-speaking participants. The DDK results in general showed that the normal-hearing group produced the fastest syllable rates, and the profoundly HI group produced the slowest. Similar results were obtained for speaking rates. Speech intelligibility was highest in the normal-hearing group and lowest in the profoundly HI group. Correlation analysis between DDK and speaking rates showed that for HI group only, a slow speaking rate corresponded to slow DDK rates. It is shown that generally there are significant differences in measures of speech motor control in normal-hearing and hearing-impaired participants. These results concord with those from other language groups.

  1. Perception of a Sung Vowel as a Function of Frequency-Modulation Rate and Excursion in Listeners with Normal Hearing and Hearing Impairment

    ERIC Educational Resources Information Center

    Vatti, Marianna; Santurette, Sébastien; Pontoppidan, Niels Henrik; Dau, Torsten

    2014-01-01

    Purpose: Frequency fluctuations in human voices can usually be described as coherent frequency modulation (FM). As listeners with hearing impairment (HI listeners) are typically less sensitive to FM than listeners with normal hearing (NH listeners), this study investigated whether hearing loss affects the perception of a sung vowel based on FM…

  2. Hearing impairment and language delay in infants: Diagnostics and genetics

    PubMed Central

    Lang-Roth, Ruth

    2014-01-01

    This overview study provides information on important phoniatric and audiological aspects of early childhood hearing and language development with the aim of presenting diagnostic and therapeutic approaches. The article first addresses the universal newborn hearing screening that has been implemented in Germany for all infants since January 2009. The process of newborn hearing screening from the maternity ward to confirmation diagnostics is presented in accordance with a decision by the Federal Joint Committee (G-BA). The second topic is pediatric audiology diagnostics. Following confirmation of a permanent early childhood hearing disorder, the search for the cause plays an important role. Hereditary hearing disorders and intrauterine cytomegalovirus (CMV) infection, probably the most common cause of an acquired hearing disorder, are discussed and compared with the most common temporary hearing disorder, otitis media with effusion, which in some cases is severe enough to be relevant for hearing and language development and therefore requires treatment. The third topic covered in this article is speech and language development in the first 3 years of life, which is known today to be crucial for later language development and learning to read and write. There is a short overview and introduction to modern terminology, followed by the abnormalities and diagnostics of early speech and language development. Only some aspects of early hearing and language development are addressed here. Important areas such as the indication for a cochlear implant in the first year of life or because of unilateral deafness are not included due to their complexity. PMID:25587365

  3. Hearing impairment and language delay in infants: Diagnostics and genetics.

    PubMed

    Lang-Roth, Ruth

    2014-01-01

    This overview study provides information on important phoniatric and audiological aspects of early childhood hearing and language development with the aim of presenting diagnostic and therapeutic approaches. The article first addresses the universal newborn hearing screening that has been implemented in Germany for all infants since January 2009. The process of newborn hearing screening from the maternity ward to confirmation diagnostics is presented in accordance with a decision by the Federal Joint Committee (G-BA). The second topic is pediatric audiology diagnostics. Following confirmation of a permanent early childhood hearing disorder, the search for the cause plays an important role. Hereditary hearing disorders and intrauterine cytomegalovirus (CMV) infection, probably the most common cause of an acquired hearing disorder, are discussed and compared with the most common temporary hearing disorder, otitis media with effusion, which in some cases is severe enough to be relevant for hearing and language development and therefore requires treatment. The third topic covered in this article is speech and language development in the first 3 years of life, which is known today to be crucial for later language development and learning to read and write. There is a short overview and introduction to modern terminology, followed by the abnormalities and diagnostics of early speech and language development. Only some aspects of early hearing and language development are addressed here. Important areas such as the indication for a cochlear implant in the first year of life or because of unilateral deafness are not included due to their complexity.

  4. A novel locus for non-syndromic sensorineural deafness (DFN6) maps to chromosome Xp22.

    PubMed

    del Castillo, I; Villamar, M; Sarduy, M; Romero, L; Herraiz, C; Hernández, F J; Rodríguez, M; Borrás, I; Montero, A; Bellón, J; Tapia, M C; Moreno, F

    1996-09-01

    Non-syndromic X-linked deafness is highly heterogeneous. At least five different clinical forms have been described, but only two loci have been mapped. Here we report a Spanish family affected by a previously undescribed X-linked form of hearing impairment. Deafness is non-syndromic, sensorineural, and progressive. In affected males, the auditory impairment is first detected at school age, affecting mainly the high frequencies. Later it evolves to become severe to profound, involving all frequencies for adulthood. Carrier females manifest a moderate hearing impairment in the high frequencies, with the onset delayed to the fourth decade of life. Deafness was assumed to be X-linked dominant, with incomplete penetrance and variable expressivity in carrier females. The family was genotyped for a set of microsatellite markers evenly spaced at intervals of about 10 cM. We found evidence of linkage to markers in the Xp22 region (maximum lod score of 5.30 at theta = 0.000 for DXS8036 and for DXS8022). The position of the novel deafness locus (DFN6) was refined by haplotype analysis. Mapping of the breakpoints in two critical recombinants allowed us to define an interval for DFN6, delimited by DXS7108 on the distal side and by DXS7105 on the proximal side, and spanning a genetic distance of about 15 cM.

  5. A Large Genome-Wide Association Study of Age-Related Hearing Impairment Using Electronic Health Records

    PubMed Central

    Hoffmann, Thomas J.; Keats, Bronya J.; Yoshikawa, Noriko; Risch, Neil

    2016-01-01

    Age-related hearing impairment (ARHI), one of the most common sensory disorders, can be mitigated, but not cured or eliminated. To identify genetic influences underlying ARHI, we conducted a genome-wide association study of ARHI in 6,527 cases and 45,882 controls among the non-Hispanic whites from the Genetic Epidemiology Research on Adult Health and Aging (GERA) cohort. We identified two novel genome-wide significant SNPs: rs4932196 (odds ratio = 1.185, p = 4.0x10-11), 52Kb 3’ of ISG20, which replicated in a meta-analysis of the other GERA race/ethnicity groups (1,025 cases, 12,388 controls, p = 0.00094) and in a UK Biobank case-control analysis (30,802 self-reported cases, 78,586 controls, p = 0.015); and rs58389158 (odds ratio = 1.132, p = 1.8x10-9), which replicated in the UK Biobank (p = 0.00021). The latter SNP lies just outside exon 8 and is highly correlated (r2 = 0.96) with the missense SNP rs5756795 in exon 7 of TRIOBP, a gene previously associated with prelingual nonsyndromic hearing loss. We further tested these SNPs in phenotypes from audiologist notes available on a subset of GERA (4,903 individuals), stratified by case/control status, to construct an independent replication test, and found a significant effect of rs58389158 on speech reception threshold (SRT; overall GERA meta-analysis p = 1.9x10-6). We also tested variants within exons of 132 other previously-identified hearing loss genes, and identified two common additional significant SNPs: rs2877561 (synonymous change in ILDR1, p = 6.2x10-5), which replicated in the UK Biobank (p = 0.00057), and had a significant GERA SRT (p = 0.00019) and speech discrimination score (SDS; p = 0.0019); and rs9493627 (missense change in EYA4, p = 0.00011) which replicated in the UK Biobank (p = 0.0095), other GERA groups (p = 0.0080), and had a consistent significant result for SRT (p = 0.041) and suggestive result for SDS (p = 0.081). Large cohorts with GWAS data and electronic health records may be a useful

  6. Prevalence and Social Risk Factors for Hearing Impairment in Chinese Children—A National Survey

    PubMed Central

    Yun, Chunfeng; Wang, Zhenjie; Gao, Jiamin; He, Ping; Guo, Chao; Chen, Gong; Zheng, Xiaoying

    2017-01-01

    Hearing impairment may affect children’s communication skills, social development, and educational achievement. Little is known about the prevalence of hearing impairment among Chinese children. Data were taken from the 2006 second China National Survey on Disability (CNSD). Hearing impairment was defined as moderate (41–60 dB HL), severe (61–80 dB HL), profound (81–90 dB HL), or complete (>91 dB HL). Logistic regression was used to estimate the odds ratio (OR) and 95% confidence intervals (CI). A weighted number of 567,915 hearing impairment children were identified, yielding a prevalence of 17.49 per 10,000 people (95% CI: 16.90–18.08), with prevention or treatment options possible for 64.6% of hearing impairment children. The main causes of hearing impairment were hereditary, tympanitis, and drug intoxication. Illiteracy in one or both parents (mother: OR = 1.388, 95% CI: 1.125–1.714, p < 0.0001; father: OR = 1.537, 95% CI: 1.152–2.049, p < 0.0001 relative to no school or primary school), annual family income lower than national average (OR = 1.323, 95% CI: 1.044–1.675, p = 0.0203, relative to higher than national average), household size larger than three people (OR = 1.432, 95% CI: 1.164–1.762, p = 0.0007, relative to smaller than three people) and single-mother family (OR = 2.056, 95% CI: 1.390–3.042, p = 0.0176, relative to intact family) were the independence risk factors for hearing impairment among Chinese children. Lower annual family income, male children, larger household size, single-mother family, and lower levels of maternal and paternal education were independent risk factors for hearing impairment for Chinese children. Further studies on hearing impairment prevention and the relationship between parental social factors and the risk of hearing impairment are needed. PMID:28106811

  7. 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.

  8. It's About Time Hearing Impairments Came Out in the Open!

    ERIC Educational Resources Information Center

    Davis, Kay

    Written for vocational educators as part of a project called "Modifying Regular Programs and Developing Curriculum Materials for the Vocational Education of the Handicapped," this guide contains discussion on 13 topics: (1) What Must It Be Like? (2) The Terminology Jungle (Degree of Hearing Loss, Age at Onset, Type of Hearing Loss), (3)…

  9. Parenting a Hearing-Impaired Child: A Model Program.

    ERIC Educational Resources Information Center

    Pearson, Helen R.

    1984-01-01

    The program at the Helen Beebe Speech and Hearing Center (Easton, Pennsylvania), a unisensory approach capitalizing on residual hearing, promotes parent participation through an intensive education program. The approach is intended to help the family support, accept, and encourage the child. (CL)

  10. Prediction of speech intelligibility in spatial noise and reverberation for normal-hearing and hearing-impaired listeners.

    PubMed

    Beutelmann, Rainer; Brand, Thomas

    2006-07-01

    Binaural speech intelligibility of individual listeners under realistic conditions was predicted using a model consisting of a gammatone filter bank, an independent equalization-cancellation (EC) process in each frequency band, a gammatone resynthesis, and the speech intelligibility index (SII). Hearing loss was simulated by adding uncorrelated masking noises (according to the pure-tone audiogram) to the ear channels. Speech intelligibility measurements were carried out with 8 normal-hearing and 15 hearing-impaired listeners, collecting speech reception threshold (SRT) data for three different room acoustic conditions (anechoic, office room, cafeteria hall) and eight directions of a single noise source (speech in front). Artificial EC processing errors derived from binaural masking level difference data using pure tones were incorporated into the model. Except for an adjustment of the SII-to-intelligibility mapping function, no model parameter was fitted to the SRT data of this study. The overall correlation coefficient between predicted and observed SRTs was 0.95. The dependence of the SRT of an individual listener on the noise direction and on room acoustics was predicted with a median correlation coefficient of 0.91. The effect of individual hearing impairment was predicted with a median correlation coefficient of 0.95. However, for mild hearing losses the release from masking was overestimated.

  11. Effect of Early Intervention on Language Development in Hearing-Impaired Children

    PubMed Central

    Shojaei, Elahe; Jafari, Zahra; Gholami, Maryam

    2016-01-01

    Introduction: Hearing loss from birth up to the age of 3 years has a negative effect on speech/language development and results in sensory, cognitive, emotional, and academic defects in adulthood by causing delayed development of communicative-linguistic abilities. The present study was performed in order to assess the effect of early intervention on language development in Persian children aged 6-7 years with severe sensorineural hearing loss. Materials and Methods: Thirty boys and girls aged 6-7 years participated in this study, all of them had severe congenital sensorineural hearing loss in both ears. All children were using bilateral behind-the-ear hearing aid, and had similar economic/socio-cultural backgrounds. Subjects were categorized into two groups based on the age of identification/intervention of hearing loss (3-6 and 12-15 months of age). The Persian TOLD-P3 test was used to evaluate language development in all subjects. Data collection was accomplished by observation, completion of questionnaires, and speech recording. Results: There was a significant difference in language development in 11 sub-tests and five lingual gains on the Persian TOLD-P3 test between early (3-6 months of age) and late identified/intervened (12-15 months of age) hearing-impaired children (P<0.05). Early identified/intervened hearing-impaired children had a notable preference in all assessed sub-tests and lingual gains. Conclusion: Early identification/intervention of hearing loss before the age of 6 months has a significant positive effect on a child’s language development in terms of picture/relational/oral vocabulary, grammatical comprehension, sentence combining, grammatical completion, phonologic analysis, word differentiation, word production, semantics, and syntax. Moreover, early identification/ intervention of hearing loss develops the hearing-impaired child’s lingual gains in visual vocabulary, grammatical completion, word differentiation, phonologic analysis, and

  12. How hearing impairment affects sentence comprehension: using eye fixations to investigate the duration of speech processing.

    PubMed

    Wendt, Dorothea; Kollmeier, Birger; Brand, Thomas

    2015-04-24

    The main objective of this study was to investigate the extent to which hearing impairment influences the duration of sentence processing. An eye-tracking paradigm is introduced that provides an online measure of how hearing impairment prolongs processing of linguistically complex sentences; this measure uses eye fixations recorded while the participant listens to a sentence. Eye fixations toward a target picture (which matches the aurally presented sentence) were measured in the presence of a competitor picture. Based on the recorded eye fixations, the single target detection amplitude, which reflects the tendency of the participant to fixate the target picture, was used as a metric to estimate the duration of sentence processing. The single target detection amplitude was calculated for sentence structures with different levels of linguistic complexity and for different listening conditions: in quiet and in two different noise conditions. Participants with hearing impairment spent more time processing sentences, even at high levels of speech intelligibility. In addition, the relationship between the proposed online measure and listener-specific factors, such as hearing aid use and cognitive abilities, was investigated. Longer processing durations were measured for participants with hearing impairment who were not accustomed to using a hearing aid. Moreover, significant correlations were found between sentence processing duration and individual cognitive abilities (such as working memory capacity or susceptibility to interference). These findings are discussed with respect to audiological applications.

  13. Modeling speech intelligibility in quiet and noise in listeners with normal and impaired hearing.

    PubMed

    Rhebergen, Koenraad S; Lyzenga, Johannes; Dreschler, Wouter A; Festen, Joost M

    2010-03-01

    The speech intelligibility index (SII) is an often used calculation method for estimating the proportion of audible speech in noise. For speech reception thresholds (SRTs), measured in normally hearing listeners using various types of stationary noise, this model predicts a fairly constant speech proportion of about 0.33, necessary for Dutch sentence intelligibility. However, when the SII model is applied for SRTs in quiet, the estimated speech proportions are often higher, and show a larger inter-subject variability, than found for speech in noise near normal speech levels [65 dB sound pressure level (SPL)]. The present model attempts to alleviate this problem by including cochlear compression. It is based on a loudness model for normally hearing and hearing-impaired listeners of Moore and Glasberg [(2004). Hear. Res. 188, 70-88]. It estimates internal excitation levels for speech and noise and then calculates the proportion of speech above noise and threshold using similar spectral weighting as used in the SII. The present model and the standard SII were used to predict SII values in quiet and in stationary noise for normally hearing and hearing-impaired listeners. The present model predicted SIIs for three listener types (normal hearing, noise-induced, and age-induced hearing loss) with markedly less variability than the standard SII.

  14. Multicenter audiometric results with the Vibrant Soundbridge, a semi-implantable hearing device for sensorineural hearing impairment.

    PubMed

    Snik, A F; Mylanus, E A; Cremers, C W; Dillier, N; Fisch, U; Gnadeberg, D; Lenarz, T; Mazolli, M; Babighian, G; Uziel, A S; Cooper, H R; O'Connor, A F; Fraysse, B; Charachon, R; Shehata-Dieler, W E

    2001-04-01

    The Vibrant Soundbridge, a semi-implantable hearing device for subjects with moderate to severe sensorineural hearing impairment was introduced commercially. First audiologic results are presented on 63 patients from 10 European implant centers. Hearing loss was at 0.5, 1, 2, and 4 kHz varying between 43 and 81 dB HL. The patients used the analogue audio processor, type 302. Measured sound-field gain was compared with NAL-R target values. For most patients an acceptable agreement was found. There was a subgroup of patients, however, with relatively low gain. The results suggest that this was related to the suboptimal positioning and fixation of the transducer to the incus.

  15. Education, occupation, noise exposure history and the 10-yr cumulative incidence of hearing impairment in older adults.

    PubMed

    Cruickshanks, Karen J; Nondahl, David M; Tweed, Ted S; Wiley, Terry L; Klein, Barbara E K; Klein, Ronald; Chappell, Rick; Dalton, Dayna S; Nash, Scott D

    2010-06-01

    The purpose of this study was to determine the 10-yr cumulative incidence of hearing impairment and associations of education, occupation and noise exposure history with the incidence of hearing impairment in a population-based cohort study of 3753 adults ages 48-92 yr at the baseline examinations during 1993-1995 in Beaver Dam, WI. Hearing thresholds were measured at baseline, 2.5 yr-, 5 yr-, and 10-yr follow-up examinations. Hearing impairment was defined as a pure-tone average (PTA)>25 dB HL at 500, 1000, 2000, and 4000 Hz. Demographic characteristics and occupational histories were obtained by questionnaire. The 10-yr cumulative incidence of hearing impairment was 37.2%. Age (5 yr; Hazard Ratio (HR)=1.81), sex (M vs W; HR=2.29), occupation based on longest held job (production/operations/farming vs others; HR=1.34), marital status (unmarried vs married; HR=1.29) and education (<16 vs 16+yr; HR=1.40) were associated with the 10 yr incidence. History of noisy jobs was not associated with the 10-yr incidence of hearing impairment. The risk of hearing impairment was high, with women experiencing a slightly later onset. Markers of socioeconomic status were associated with hearing impairment, suggesting that hearing impairment in older adults may be associated with modifiable lifestyle and environmental factors, and therefore, at least partially preventable.

  16. Medical aspects of ageing in a population with intellectual disability: II. Hearing impairment.

    PubMed

    Evenhuis, H M

    1995-02-01

    Hearing function of an institutionalized population with intellectual disability, consisting of 70 subjects with a mean age of 70.1 (range 60-92) years at initial evaluation, was assessed during a 10-year longitudinal study. One subject had Down's syndrome and could not be assessed as a result of dementia. The total prevalence of mild to severe hearing loss (33.3% in the 60-70 age group and 70.4% in those over age 70) was comparable to reported data from an ageing population without intellectual disability in the United Kingdom (37%, respectively 60%). However, the proportion of moderate to severe losses might be higher (16.7% vs. 7% in the 60-70 age group and 33.3% vs. 18% in the older age group). Excess impairment was caused by severe congenital and childhood hearing impairment on one hand, and by conductive losses, probably caused by unrecognized chronic middle ear infections, superposed upon presbyacusis, on the other. Impacted ear wax was also a major problem. The incidence of new cases with hearing loss during follow-up was 50%. After individual habituation training hearing aids were used without difficulties by 20 out of 24 subjects. The importance of active screening and treatment of middle ear infections and hearing impairment from a young age onwards, and regular cleaning of the external ear canals is stressed.

  17. Discrimination and identification of vowels by young, hearing-impaired adults

    NASA Astrophysics Data System (ADS)

    Richie, Carolyn; Kewley-Port, Diane; Coughlin, Maureen

    2003-11-01

    This study examined the effects of mild-to-moderate sensorineural hearing loss on vowel perception abilities of young, hearing-impaired (YHI) adults. Stimuli were presented at a low conversational level with a flat frequency response (approximately 60 dB SPL), and in two gain conditions: (a) high level gain with a flat frequency response (95 dB SPL), and (b) frequency-specific gain shaped according to each listener's hearing loss (designed to simulate the frequency response provided by a linear hearing aid to an input signal of 60 dB SPL). Listeners discriminated changes in the vowels /smcapi ee eh invv æ/ when F1 or F2 varied, and later categorized the vowels. YHI listeners performed better in the two gain conditions than in the conversational level condition. Performances in the two gain conditions were similar, suggesting that upward spread of masking was not seen at these signal levels for these tasks. Results were compared with those from a group of elderly, hearing-impaired (EHI) listeners, reported in Coughlin, Kewley-Port, and Humes [J. Acoust. Soc. Am. 104, 3597-3607 (1998)]. Comparisons revealed no significant differences between the EHI and YHI groups, suggesting that hearing impairment, not age, is the primary contributor to decreased vowel perception in these listeners.

  18. Assistive video or assistive courseware: What hearing-impaired learners say?

    NASA Astrophysics Data System (ADS)

    Mutalib, Ariffin Abdul; Salam, Sobihatun Nur Abdul; Ahmad, Mazida; Mahmuddin, Massudi; Yahaya, Sharifah Nadiya Syed

    2016-08-01

    This paper explains about a comparison between assistive video for hearing-impaired learners (AV4HI) and assistive courseware for hearing-impaired learners (AC4HI). The rationale is that previous statement was made after learners experience normal courseware, not the courseware specifically designed for them. Hence, perhaps if the courseware is designed special for them, the hearing-impaired learners would experience differently, and behave more positive upon the AC4HI. Hence, this paper aims at describing the execution of the comparison. It involves a 3-stage process: designing the AC4HI, developing the AC4HI, and user experience. In the end, it was found that users are happy with both, but in terms of content acquisition, they prefer to have notes in the learning material.

  19. Workplace discrimination, deafness and hearing impairment: the national EEOC ADA research project.

    PubMed

    Bowe, Frank G; McMahon, Brian T; Chang, Tai; Louvi, Ioanna

    2005-01-01

    Data compiled by the US Equal Employment Opportunity Commission (EEOC), in its Integrated Mission System, provide documentation regarding the employment discrimination experience of Americans who are deaf or hard of hearing. This paper presents an analysis of 8,936 allegations filed by persons with hearing impairment and closed by EEOC between July 26, 1992 and September 30, 2003, as compared to 165,674 allegations filed by individuals with other physical or sensory disabilities. The investigators compare and contrast demographic characteristics of Charging Parties, characteristics of Respondents, the nature of allegations, and the outcomes of the allegations in order to illustrate how these variables differ between the two groups, herein referred to as HEARING (deaf, hard of hearing, or other hearing impairment) and GENDIS (general disability). Most allegations derived from both groups were filed against larger Respondents (those with 500+ workers). The most common allegation issues in the HEARING group involved matters of discharge, reasonable accommodation, and hiring. Outcomes derived from HEARING allegations were more likely to result in merit resolutions when compared to GENDIS, by a 25% to 21% margin.

  20. Demographics of Meningitis-Induced Hearing Impairment: Implications for Immunization of Children Against Hemophilus Influenzae Type B.

    ERIC Educational Resources Information Center

    Wolff, Anthony B.; Brown, Scott Campbell

    1987-01-01

    The incidence of hearing impairments caused by meningitis (8.7% of which 63% have profound hearing loss) suggests that universal vaccination at age 18 months could prevent at least 115 cases of hearing loss anuually in the United States. Reported prevalence rates were higher for blacks and for males. (Author/DB)

  1. Cognitive Compensation of Speech Perception With Hearing Impairment, Cochlear Implants, and Aging

    PubMed Central

    Clarke, Jeanne; Pals, Carina; Benard, Michel R.; Bhargava, Pranesh; Saija, Jefta; Sarampalis, Anastasios; Wagner, Anita; Gaudrain, Etienne

    2016-01-01

    External degradations in incoming speech reduce understanding, and hearing impairment further compounds the problem. While cognitive mechanisms alleviate some of the difficulties, their effectiveness may change with age. In our research, reviewed here, we investigated cognitive compensation with hearing impairment, cochlear implants, and aging, via (a) phonemic restoration as a measure of top-down filling of missing speech, (b) listening effort and response times as a measure of increased cognitive processing, and (c) visual world paradigm and eye gazing as a measure of the use of context and its time course. Our results indicate that between speech degradations and their cognitive compensation, there is a fine balance that seems to vary greatly across individuals. Hearing impairment or inadequate hearing device settings may limit compensation benefits. Cochlear implants seem to allow the effective use of sentential context, but likely at the cost of delayed processing. Linguistic and lexical knowledge, which play an important role in compensation, may be successfully employed in advanced age, as some compensatory mechanisms seem to be preserved. These findings indicate that cognitive compensation in hearing impairment can be highly complicated—not always absent, but also not easily predicted by speech intelligibility tests only.

  2. A novel missense mutation p.L76P in the GJB2 gene causing nonsyndromic recessive deafness in a Brazilian family.

    PubMed

    Batissoco, A C; Auricchio, M T B M; Kimura, L; Tabith-Junior, A; Mingroni-Netto, R C

    2009-02-01

    Mutations in the GJB2 gene, encoding connexin 26 (Cx26), are a major cause of nonsyndromic recessive hearing loss in many countries. We report here on a novel point mutation in GJB2, p.L76P (c.227C>T), in compound heterozygosity with a c.35delG mutation, in two Brazilian sibs, one presenting mild and the other profound nonsyndromic neurosensorial hearing impairment. Their father, who carried a wild-type allele and a p.L76P mutation, had normal hearing. The mutation leads to the substitution of leucine (L) by proline (P) at residue 76, an evolutionarily conserved position in Cx26 as well as in other connexins. This mutation is predicted to affect the first extracellular domain (EC1) or the second transmembrane domain (TM2). EC1 is important for connexon-connexon interaction and for the control of channel voltage gating. The segregation of the c.227C>T (p.L76P) mutation together with c.35delG in this family indicates a recessive mode of inheritance. The association between the p.L76P mutation and hearing impairment is further supported by its absence in a normal hearing control group of 100 individuals, 50 European-Brazilians and 50 African-Brazilians.

  3. Hearing impairment risk and interaction of folate metabolism related gene polymorphisms in an aging study

    PubMed Central

    2011-01-01

    Background Recent investigations demonstrated many genetic contributions to the development of human age-related hearing impairment (ARHI), however, reports of factors associated with a reduction in the ARHI risk are rare. Folate metabolism is essential for cellular functioning. Despite the extensive investigations regarding the roles of folate metabolism related gene polymorphisms in the pathophysiology of complex diseases, such as cancer, cardio-cerebrovascular disease, and atherosclerosis, little is known about the association with ARHI. The aim of this study is to investigate the effects of the methionine synthase (MTR) A2756G and methylenetetrahydrofolate reductase (MTHFR) C677T gene polymorphisms on the risk of hearing impairment in middle-aged and elderly Japanese. Methods Data were collected from community-dwelling Japanese adults aged 40-84 years who participated in the Longitudinal Study of Aging biennially between 1997 and 2008. We analyzed cumulative data (5,167 samples in accumulated total) using generalized estimating equations. Results The MTHFR 677T allele was significantly associated with a reduced risk of hearing impairment only when the subjects were wild-type homozygotes for MTR A2756G. The per-T allele odds ratio of MTHFR for the risk of developing hearing impairment was 0.7609 (95% CI: 0.6178-0.9372) in the MTR AA genotype. In addition, a subgroup analysis demonstrated that the favorable effect of the MTHFR 677T allele on the risk of developing hearing impairment was independent of folate and homocysteine level, whereas plasma total homocysteine level was independently associated with an increased risk of developing hearing impairment. The interactive effect of gene polymorphisms associated with folate metabolism may modify the risk of developing hearing impairment after middle age. These results contribute to the elucidation of the causes of ARHI. Conclusions The present study has found that the MTHFR 677T allele has a favorable effect on a

  4. Effect of recreational noise exposure on hearing impairment among teenage students.

    PubMed

    Tung, Chen-Yin; Chao, Keh-Ping

    2013-01-01

    Several studies have focused on the potential impact of children's hearing loss on learning and development. Recently, numerous teenage students have been found to be fond of listening to music on personal devices and participating in recreational music activities. The objective of this study was to investigate teenage students' hearing impairment, their experience with recreational noise exposure, and their self-reported hearing. The participants were 1878 first-year students at a university in Taiwan. The result of the pure tone audiometry test showed that 11.9% of the participants had one or two ears with a hearing threshold over 25 dB. Over the past year, approximately 80.9% of the participants had taken part in at least one loud-noise recreational activity, and 90.9% of the participants were in the habit of using earphones. Among the participants, 190 students with a high level of recreational noise exposure were assigned to the exposure group, and 191 students with a low level of recreational noise exposure constituted the control group. The exposure group had more hearing problems than the control group, but no significant difference existed between the two groups in the pure tone audiometry test (p=0.857). It is suggested that the schools should reinforce hearing health education and proactively provide intervention measures, such as hearing tests, evaluation of noise exposure, and hearing protection.

  5. Project SKI*HI Outreach Programming for Hearing Impaired Infants and Families: Recertification Statement, Questions, Responses, and Approval.

    ERIC Educational Resources Information Center

    Strong, Carol J.; Clark, Thomas C.

    This program evaluation report presents information on Project SKI*HI, a home-based program for infants and young children with hearing impairments and their families. The program's major goals are early identification of hearing-impaired infants and a home program to facilitate child development. A parent advisor makes weekly home visits to each…

  6. A Neurodevelopmental Profile of the Rural Hearing-Impaired Child in the QwaQwa Region, South Africa

    ERIC Educational Resources Information Center

    Bothma, Jó-Marié v. d. M.; Dunn, Munita; Kokot, Shirley

    2015-01-01

    Being hearing impaired does not only affect a child's academic performance, but can also influence a child's overall development and ability to succeed academically. Children with hearing impairment often experience delays in other areas of their development and an understanding of the inter-relatedness of these delays are important in order to…

  7. Outdoor-Adventure Education as an Ancillary Component in Rehabilitation Programs for the Hearing Impaired: A Pilot Study.

    ERIC Educational Resources Information Center

    Luckner, John L.

    This study investigated the effectiveness of using an outdoor-adventure education program as a method of intervention for enhancing the self-concept of a group of hearing impaired individuals receiving services from the Department of Vocational Rehabilitation. Nine adults (6 male, 3 female) with hearing impairments participated in a 4-day…

  8. iPads Enhance Social Interaction Skills among Hearing-Impaired Children of Low Income Families in Saudi Arabia

    ERIC Educational Resources Information Center

    Bahatheg, Raja Omar

    2015-01-01

    This research tries to investigate the technical contribution on improving the social interaction of hearing-impaired children from low income families in Saudi Arabia. It compares the social interaction skills of hearing-impaired children who do and do not have access to iPads. To achieve the goals of the study; seventeen children aged five years…

  9. 14 CFR 382.119 - What information must carriers give individuals with vision or hearing impairment on aircraft?

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... 14 Aeronautics and Space 4 2013-01-01 2013-01-01 false What information must carriers give individuals with vision or hearing impairment on aircraft? 382.119 Section 382.119 Aeronautics and Space... carriers give individuals with vision or hearing impairment on aircraft? (a) As a carrier, you must...

  10. 14 CFR 382.53 - What information must carriers give individuals with a vision or hearing impairment at airports?

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 14 Aeronautics and Space 4 2011-01-01 2011-01-01 false What information must carriers give individuals with a vision or hearing impairment at airports? 382.53 Section 382.53 Aeronautics and Space... information must carriers give individuals with a vision or hearing impairment at airports? (a)(1) As a...

  11. 14 CFR 382.53 - What information must carriers give individuals with a vision or hearing impairment at airports?

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... 14 Aeronautics and Space 4 2012-01-01 2012-01-01 false What information must carriers give individuals with a vision or hearing impairment at airports? 382.53 Section 382.53 Aeronautics and Space... information must carriers give individuals with a vision or hearing impairment at airports? (a)(1) As a...

  12. 14 CFR 382.53 - What information must carriers give individuals with a vision or hearing impairment at airports?

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... 14 Aeronautics and Space 4 2013-01-01 2013-01-01 false What information must carriers give individuals with a vision or hearing impairment at airports? 382.53 Section 382.53 Aeronautics and Space... information must carriers give individuals with a vision or hearing impairment at airports? (a)(1) As a...

  13. 14 CFR 382.119 - What information must carriers give individuals with vision or hearing impairment on aircraft?

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... 14 Aeronautics and Space 4 2014-01-01 2014-01-01 false What information must carriers give individuals with vision or hearing impairment on aircraft? 382.119 Section 382.119 Aeronautics and Space... carriers give individuals with vision or hearing impairment on aircraft? (a) As a carrier, you must...

  14. 14 CFR 382.53 - What information must carriers give individuals with a vision or hearing impairment at airports?

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... 14 Aeronautics and Space 4 2014-01-01 2014-01-01 false What information must carriers give individuals with a vision or hearing impairment at airports? 382.53 Section 382.53 Aeronautics and Space... information must carriers give individuals with a vision or hearing impairment at airports? (a)(1) As a...

  15. 14 CFR 382.119 - What information must carriers give individuals with vision or hearing impairment on aircraft?

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 14 Aeronautics and Space 4 2011-01-01 2011-01-01 false What information must carriers give individuals with vision or hearing impairment on aircraft? 382.119 Section 382.119 Aeronautics and Space... carriers give individuals with vision or hearing impairment on aircraft? (a) As a carrier, you must...

  16. 14 CFR 382.119 - What information must carriers give individuals with vision or hearing impairment on aircraft?

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... 14 Aeronautics and Space 4 2012-01-01 2012-01-01 false What information must carriers give individuals with vision or hearing impairment on aircraft? 382.119 Section 382.119 Aeronautics and Space... carriers give individuals with vision or hearing impairment on aircraft? (a) As a carrier, you must...

  17. Providing Hearing-Impaired Students with Learning Care after Classes through Smart Phones and the GPRS Network

    ERIC Educational Resources Information Center

    Liu, Chen-Chung; Hong, Yi-Ching

    2007-01-01

    Although computers and network technology have been widely utilised to assist students learn, few technical supports have been developed to help hearing-impaired students learn in Taiwan. A significant challenge for teachers is to provide after-class learning care and assistance to hearing-impaired students that sustain their motivation to…

  18. Vaccine May Reduce Incidence of Meningitis-Related Hearing Impairment.

    ERIC Educational Resources Information Center

    Smith, Dorothy

    1988-01-01

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

  19. Perception of Spectral Contrast by Hearing-Impaired Listeners

    ERIC Educational Resources Information Center

    Dreisbach, Laura E.; Leek, Marjorie R.; Lentz, Jennifer J.

    2005-01-01

    The ability to discriminate the spectral shapes of complex sounds is critical to accurate speech perception. Part of the difficulty experienced by listeners with hearing loss in understanding speech sounds in noise may be related to a smearing of the internal representation of the spectral peaks and valleys because of the loss of sensitivity and…

  20. Intensive Training for Teacher Aides Working with Hearing Impaired Children.

    ERIC Educational Resources Information Center

    Garrett, Ann; Hastings, Chester R.

    In summer 1975, McLennan Community College selected 64 counselor-trainees to participate in two 2-week summer camp sessions, each of which involved 50 deaf or hard of hearing children, ranging from 7-12 years of age. Trainees had minimal manual communication skills, and were selected from applicants who were either enrolled in training programs…

  1. Human nonsyndromic hereditary deafness DFNA17 is due to a mutation in nonmuscle myosin MYH9.

    PubMed

    Lalwani, A K; Goldstein, J A; Kelley, M J; Luxford, W; Castelein, C M; Mhatre, A N

    2000-11-01

    The authors had previously mapped a new locus-DFNA17, for nonsyndromic hereditary hearing impairment-to chromosome 22q12.2-q13. 3. DFNA17 spans a 17- to 23-cM region, and MYH9, a nonmuscle-myosin heavy-chain gene, is located within the linked region. Because of the importance of myosins in hearing, MYH9 was tested as a candidate gene for DFNA17. Expression of MYH9 in the rat cochlea was confirmed using reverse transcriptase-PCR and immunohistochemistry. MYH9 was immunolocalized in the organ of Corti, the subcentral region of the spiral ligament, and the Reissner membrane. Sequence analysis of MYH9 in a family with DFNA17 identified, at nucleotide 2114, a G-->A transposition that cosegregated with the inherited autosomal dominant hearing impairment. This missense mutation changes codon 705 from an invariant arginine (R) to histidine (H), R705H, within a highly conserved SH1 linker region. Previous studies have shown that modification of amino acid residues within the SH1 helix causes dysfunction of the ATPase activity of the motor domain in myosin II. Both the precise role of MYH9 in the cochlea and the mechanism by which the R705H mutation leads to the DFNA17 phenotype (progressive hearing impairment and cochleosaccular degeneration) remain to be elucidated.

  2. An Overview of the Major Phenomena of the Localization of Sound Sources by Normal-Hearing, Hearing-Impaired, and Aided Listeners

    PubMed Central

    2014-01-01

    Localizing a sound source requires the auditory system to determine its direction and its distance. In general, hearing-impaired listeners do less well in experiments measuring localization performance than normal-hearing listeners, and hearing aids often exacerbate matters. This article summarizes the major experimental effects in direction (and its underlying cues of interaural time differences and interaural level differences) and distance for normal-hearing, hearing-impaired, and aided listeners. Front/back errors and the importance of self-motion are noted. The influence of vision on the localization of real-world sounds is emphasized, such as through the ventriloquist effect or the intriguing link between spatial hearing and visual attention. PMID:25492094

  3. Proposed iso standard determination of occupational noise exposure and estimation of noise-induced hearing impairment

    SciTech Connect

    Von Gierke, H.E.

    1986-01-01

    Research on the relationship between noise exposure and noise-induced hearing loss has been very intense over the last 30 years, and steady progress has been made in spite of many remaining questions and unresolved problems regarding the mechanisms. For the time being, avoidance of excessive noise exposure is the only way to prevent noise-induced hearing loss; this is the reason why governments, industry, workers and their representatives have been looking for scientific exposure criteria and guidelines to prevent hazardous noise exposure as part of comprehensive hearing conservation programs. Although it was clear from the beginning that noise-induced hearing loss in a population with exactly defined noise exposure would exhibit a statistical distribution due to differences in biological susceptibility, the epidemiological statistical data were not available to describe quantitatively the difference between the percentage of people with impaired hearing in a noise-exposed group and the percentage of people in a non-noise-exposed group, i.e., the risk of noise-induced hearing impairment.

  4. Glomerular Filtration Rate and Urine Albumin to Creatinine Ratio Associated With Hearing Impairment Among Korean Adults With Diabetes

    PubMed Central

    Cho, Yunji; Kim, Do Hoon; Choi, June; Lee, Joo Kyung; Roh, Yong-Kyun; Nam, Hyo-Yun; Nam, Ga-Eun; Kim, Dong-Won; Lee, Seung-Hyun; Lee, Chung-Woo; Han, Kyungdo; Park, Yong-Gyu

    2016-01-01

    Abstract The objective of this study was to examine the association of estimated glomerular filtration rate (eGFR) and urine albumin to creatinine ratio (ACR) with hearing impairment among diabetic adults in Korea. The study was based on data from Korea National Health and Nutrition Examination Survey 2011 to 2012. Participants were 1206 diabetic adults, aged over 19 years, who completed audiometric testing supervised by nationally certified clinicians. Hearing impairment was defined in three grades: no hearing impairment (pure-tone average 0–25 dB), slight hearing impairment (26–40 dB), and disabling hearing impairment (>40 dB) in the better ear at frequencies 0.5, 1, 2, 3, 4 and 6 kHz. Using logistic regression, risk of hearing impairment was assessed after having controlled for confounding factors. Higher levels of ACR and lower levels of eGFR correlated with an increase in percentage of disabling hearing impairment both unilaterally and bilaterally (P < 0.001). Controlling for possible confounding covariates, odds ratios for hearing impairment showed tendency to increase in higher ACR groups (P for trend = 0.029). Similar pattern was examined between eGFR and hearing impairment (P for trend = 0.006). Odds ratios were 1.981 (1.146, 3.424) for ACR Q4 and 2.773 (1.286, 5.983) for eGFR < 60 mL/min. Fall in eGFR and rise in ACR correlated with severity of hearing impairment. The association existed independently of age, sex, body mass index (BMI), smoking, drinking, exercise, new onset of diabetes, education, income, mental stress, noise exposure, and metabolic syndrome. PMID:27124027

  5. Mediterranean spotted fever and hearing impairment: a rare complication.

    PubMed

    Rossio, Raffaella; Conalbi, Valeria; Castagna, Valentina; Recalcati, Sebastiano; Torri, Adriana; Coen, Massimo; Cassulini, Lucia Restano; Peyvandi, Flora

    2015-06-01

    Mediterranean spotted fever (MSF) is caused by Rickettsia conorii and transmitted by the brown dog tick Rhipicephalus sanguineus. It is prevalent in southern Europe, Africa and central Asia. The disease usually has a benign course and is characterized by fever, myalgia and a characteristic papular rash with an inoculation eschar ('tache noir') at the site of the tick bite. Severe forms of disease can have cardiac, neurologic or renal involvement. Nervous system complications are unusual and may develop in the early phase of disease or as a delayed complication. Neurological symptoms include headache and alterations of the level of consciousness, and some cases of meningoenchefalitis and Guillain-Barrè syndrome have been also reported. Peripheral nerve involvement is reported only in a limited number of case reports. We describe a case of Rickettsia conorii that was complicated with hearing loss and did not respond to specific treatment. Hearing loss is a rare event, but clinicians should be aware of this complication.

  6. Quality of life in adolescents with hearing deficiencies and visual impairments

    PubMed Central

    Marques Freire Torres, Vanthauze; Lidianne Alencar Marinho, Christielle; Gabriela Gomes de Oliveira, Carolina; Conceição Maria Vieira, Sandra

    2013-01-01

    Summary Introduction: The term quality of life (in Portuguese, Qualidade de Vida; QV) has been expanded and modified over the years and has come to signify social development in terms of education, health, and leisure as well as economic issues. Objective: To analyze the perception of QV in adolescents with hearing and visual impairments and the effects of socio-demographic characteristics on the domains of QV. Method: This descriptive series study comprised 42 adolescents aged 10 to 19 years who were students at Recife's state schools. The World Health Organization Quality of Life-Abbreviated questionnaire was used to evaluate QV. The data were analyzed using descriptive statistics and the Mann-Whitney and Kruskal-Wallis tests with a significance level of p < 0.05. Results: The global perception of QV was higher among adolescents with visual impairments than among those with hearing impairments. Among the individual components of QV, the environment domain garnered the lowest scores independent of the type of impairment. The subjects with visual impairments reported higher scores for social relationships, while the psychological domain scored higher among those with hearing impairments. The students integrated into normal classrooms perceived better QV in the psychological and social relationships domains than did those who sat in special classrooms. Conclusion: The environmental domain was the worst component of the QV of handicapped adolescents, suggesting a need for greater investments in policies to improve the QV of this population. PMID:26029272

  7. Detection threshold for sound distortion resulting from noise reduction in normal-hearing and hearing-impaired listeners.

    PubMed

    Brons, Inge; Dreschler, Wouter A; Houben, Rolph

    2014-09-01

    Hearing-aid noise reduction should reduce background noise, but not disturb the target speech. This objective is difficult because noise reduction suffers from a trade-off between the amount of noise removed and signal distortion. It is unknown if this important trade-off differs between normal-hearing (NH) and hearing-impaired (HI) listeners. This study separated the negative effect of noise reduction (distortion) from the positive effect (reduction of noise) to allow the measurement of the detection threshold for noise-reduction (NR) distortion. Twelve NH subjects and 12 subjects with mild to moderate sensorineural hearing loss participated in this study. The detection thresholds for distortion were determined using an adaptive procedure with a three-interval, two-alternative forced-choice paradigm. Different levels of distortion were obtained by changing the maximum amount of noise reduction. Participants were also asked to indicate their preferred NR strength. The detection threshold for overall distortion was higher for HI subjects than for NH subjects, suggesting that stronger noise reduction can be applied for HI listeners without affecting the perceived sound quality. However, the preferred NR strength of HI listeners was closer to their individual detection threshold for distortion than in NH listeners. This implies that HI listeners tolerate fewer audible distortions than NH listeners.

  8. Ira as a pioneer in audiology: His contributions to the clinical measurement of hearing and hearing impairment

    NASA Astrophysics Data System (ADS)

    Formby, C.; Gagne, J. P.

    2002-05-01

    Ira Hirsh's contributions to clinical science and research are diverse and significant. In fact, approximately one-third of the 100+ publications that Ira lists in his curriculum vitae (CV) are clinical in nature, dealing with various aspects of audiology, deafness, hearing aids, aural rehabilitation, and speech and language pathology. The majority of these citations, fully one-quarter of his publication list, addresses problems specific to the clinical measurement of hearing and hearing impairment. Undoubtedly, the most influential of these published works appears in his CV under the citation ``The Measurement of Hearing.'' The forward for this publication, his only textbook, was penned in June, 1952 (now precisely half a century past at the time of this session). The aims of this presentation are to (1) provide perspective on the fundamental importance of his virtually timeless text in shaping the fledgling discipline of audiology, and (2) celebrate Ira's many contributions to the profession and practice of audiology. [Preparation for this presentation was supported, in part, by a K24 career development award from NIDCD.

  9. [The level of the musical loud sound and noise induced hearing impairment].

    PubMed

    Ono, H; Deguchi, T; Ino, T; Okamoto, K; Takyu, H

    1986-03-20

    Recently, there has been an increasing number of reports concerning hearing impairment which musical loud sound is thought to be one of the causes. We are getting more of this musical loud sound as cassette tape recorders with head phones such as Walkman and so forth get popular as well as occasions to attending rock concerts and going to discotheques increase. This hearing impairment is generally called discotheque deafness and the following three types are considered; 1) deafness which have fixed by accumulation of loud sound over a long period of time as seen in people involved in musical performance such as rock musicians and mixing engineers; 2) abrupt noise induced hearing impairment triggered by loud sound and 3) state of deafness which is a progressing stage towards recovery of noise induced temporary threshold shift (NITTS), which occurs temporarily by a loud sound stimulus, and hearing ability recovers afterward. However, it is considered that these musical loud sounds not only changes every moment according to method of performing or type of music, but the volume of the sound actually reaching the auditory sense differs largely by locations and direction of the ear and speakers. So it becomes necessary to measure the accumulation of the noise which each individual is exposed under over a long period of time and at the same time carry out the regular medical checkups including hearing test to check the initiation and advancement of the noise induced hearing impairment. Then we can examine the relationship between loudness of the environmental noise and initiation and advancement of the hearing impairment. However, there has not been a device which is compact and measures noise exposure individually over a long period of time. So we have experimentally produced ultra compact noise dosimeter which we named Noise Badge, and with it we actually measured individual noise exposure over a long time in rock music, noise in discotheque and noisy factory. Then we

  10. Development of a low cost assistive listening system for hearing-impaired student classroom.

    PubMed

    Pan-ngum, Setha; Soonrach, Tharapong; Seesutas, Sangvorn; Noymai, Anukool; Israsena, Pasin

    2013-01-01

    This paper describes the design, development, and tests of a low cost ALS. It was designed for hearing-impaired student classrooms. It utilised digital wireless technology and was aimed to be an alternative to a popular FM ALS. Key specifications include transmitting in 2.4 GHz ISM band with eight selectable transmission channels, battery operated and chargeable, pocket size, and ranged up to thirty metres. Audio characteristics and user tests show that it is comparable to a commercial system, currently employed in our partner school. The results also show that wearing an ALS clearly improves hearing of hearing-impaired students. Long-term usage by school children will be monitored to evaluate the system robustness and durability.

  11. Effects of assistance dogs on persons with mobility or hearing impairments: a pilot study.

    PubMed

    Rintala, Diana H; Matamoros, Rebeca; Seitz, Laura L

    2008-01-01

    Service dogs help persons with mobility impairments by retrieving items and performing other tasks. Hearing dogs alert persons with hearing impairments to environmental sounds. We conducted a pre-post, wait list-controlled pilot study to assess the impact of the dogs on the lives of recipients. Participants were recruited through two assistance dog training organizations and completed an initial questionnaire packet. The Experimental group completed another packet 6 months after receiving a dog. The Control group completed a second packet 6 months after the initial data collection. On average, dog recipients were very satisfied with their assistance dogs. Both service and hearing dog recipients reduced their dependence on other persons. Service dog recipients reduced hours of paid assistance. No other significant change occurred in various standardized outcome measures. Assistance dogs had a major positive impact on the lives of recipients. More appropriate measurement instruments are needed to capture the impact of these dogs.

  12. Telephone speech enhancement for hearing-impaired listeners using multi-channel compression

    NASA Astrophysics Data System (ADS)

    Natarajan, Harikrishna P.; Krishnamurthy, Ashok K.; Feth, Lawrence L.

    2002-05-01

    Elderly listeners with sensorineural hearing impairment usually have difficulty with telephone communication if they do not use a hearing aid or telephone amplifier. In many cases, these devices may not be readily available or maybe uncomfortable to use. We describe here an alternative strategy to enhance the speech by pre-processing the signal before sending it over the telephone line. The pre-processing is based on a multi-channel compression algorithm that modifies the critical band spectrum of the speech signal to bring it within the dynamic hearing range of the listener, while also compensating for the effects of the telephone line. We describe the algorithm and the results of a simulation study in which the Articulation Index (AI) is used to measure the intelligibility enhancement, and the Glasberg, Moore and Stone (1999) model is used to simulate the effects of the hearing loss.

  13. The function of sentence accents and given/new information in speech processing: different strategies for normal-hearing and hearing-impaired listeners?

    PubMed

    van Donselaar, W; Lentz, J

    1994-01-01

    Two experiments were carried out to investigate how the correspondence between sentence accentuation and distribution of information is used in human word processing. A forced-choice task with target words embedded in sentences was employed for this purpose. Target words provided either 'given' or 'new' information, and were either accented or unaccented. The subjects had to choose between two words that differed in the last consonant by one phonetic feature (e.g., mouth/mouse). The first experiment involved both normal-hearing and hearing-impaired listeners. A fixed amount of noise was used to reduce the quality of speech for normal-hearing listeners, in order to enable a comparison between the two listener groups. The results of the first experiment showed different processing patterns for normal-hearing and hearing-impaired listeners. The hearing-impaired listeners were more accurate with words that were properly accented for their information value, whereas the normal-hearing subjects were more accurate with accented than unaccented words regardless of their information value. A new group of normal-hearing subjects was tested in a second experiment with speech of more severely reduced quality. The results indicated that, under these circumstances, the normal-hearing listeners changed their strategy and also showed an interaction between information value and accent. It seems that, as speech becomes less intelligible, listeners depend increasingly on linguistic expectations stemming from the correlation between information value and accentuation.

  14. Evaluation of Computer-Assisted Instruction in Elementary Mathematics for Hearing-Impaired Students.

    ERIC Educational Resources Information Center

    Suppes, Patrick; And Others

    A sample of approximately 385 hearing-impaired elementary and secondary school students whose mathematical achievement ranged from second to sixth grades were examined to determine the effectiveness of a computer assisted elementary mathematics program on the acquisition of computational skills. The students selected were from a population of over…

  15. Pretend Play and Maternal Scaffolding: Comparisons of Toddlers with Advanced Development, Typical Development, and Hearing Impairment.

    ERIC Educational Resources Information Center

    Morelock, Martha J.; Brown, P. Margaret; Morrissey, Anne-Marie

    2003-01-01

    A study involving three children with impaired hearing, three typical children, and three showing intellectual advancement, found children scoring above 130 IQ at age four demonstrated significantly advanced pretend play as toddlers. Mothers of the high IQ children engaged in scaffolding behaviors involving higher stages of pretend transformation,…

  16. Auditory, Visual, and Auditory-Visual Perception of Vowels by Hearing-Impaired Children.

    ERIC Educational Resources Information Center

    Hack, Zarita Caplan; Erber, Norman P.

    1982-01-01

    Vowels were presented through auditory, visual, and auditory-visual modalities to 18 hearing impaired children (12 to 15 years old) having good, intermediate, and poor auditory word recognition skills. All the groups had difficulty with acoustic information and visual information alone. The first two groups had only moderate difficulty identifying…

  17. Model Programs of Early Education for Hearing-Impaired Children and Their Families.

    ERIC Educational Resources Information Center

    Gatty, Janice C.

    This paper describes five programs of early intervention for children with hearing impairments and their families. Programs are described according to their mission, services, and unique contribution to the field of early intervention. First, essential components of all programs are identified. These are evaluation, audiological management, parent…

  18. Acoustic vs. Magnetic Coupling for Telephone Listening of Hearing-Impaired Subjects.

    ERIC Educational Resources Information Center

    Holmes, Alice E.

    1985-01-01

    The study investigated the effects of six telephone conditions on discrimination in 19 hearing impaired adults. No significant differences were found among the unaided, acoustically coupled, and magnetically coupled listening modes. Ss understood words significantly better using an amplifier handset, regardless of listening mode. (Author/CL)

  19. The Classroom Notetaker: How To Organize a Program Serving Students with Hearing Impairments.

    ERIC Educational Resources Information Center

    Wilson, Jimmie Joan

    This guide describes how to establish a notetaking program to benefit students with hearing impairments in mainstream settings. Chapter 1 discusses the need for notetakers and includes subjects such as providing equal access, high-tech and low-tech notetaking, how the notes can be used, and who can use the notes. Chapter 2 provides information on…

  20. Guidelines for Evaluating Auditory-Oral Programs for Children Who Are Hearing Impaired.

    ERIC Educational Resources Information Center

    Alexander Graham Bell Association for the Deaf, Inc., Washington, DC.

    These guidelines are intended to assist parents in evaluating educational programs for children who are hearing impaired, where a program's stated intention is promoting the child's optimal use of spoken language as a mode of everyday communication and learning. The guidelines are applicable to programs where spoken language is the sole mode or…

  1. Students Hearing Impaired from the 1963-1965 Rubella Epidemic Begin to Enter College.

    ERIC Educational Resources Information Center

    Stuckless, E. Ross; Walter, Gerard G.

    1983-01-01

    Analysis of the postsecondary qualifications among 8,000 or more children born hearing impaired as a result of the 1963-65 rubella epidemic revealed a favorable prognosis for postsecondary education for many rubella students graduating from secondary programs in 1983, l984, and l985. (Author/CL)

  2. Correcting the Enuresis of a Hearing-Impaired, Developmentally Disabled Adolescent Using an Auditory Enuresis Alarm.

    ERIC Educational Resources Information Center

    Hanson, Ronald H.

    1983-01-01

    The enuresis of a hearing-impaired, developmentally disabled adolescent was corrected through the use of an auditory alarm and specific training procedures. The young man progressed from wetting the bed every night to being consistently dry after five weeks of treatment. He has remaind dry for over two years. (Author/CL)

  3. Interpreting Television for Hearing Impaired Viewers: A Guidebook for Producers and Interpreters.

    ERIC Educational Resources Information Center

    National Technical Inst. for the Deaf, Rochester, NY.

    This manual presents production guidelines and suggestions for use by commercial, public, and educational broadcast facilities that seek to provide television interpreting for the hearing impaired. Topics covered include a brief overview of the need for television services for this audience and the use of closed captioning and sign language to…

  4. Implementation of Biofeedback Techniques To Reduce Stress Involving Communication Skills with Elementary School Hearing Impaired Students.

    ERIC Educational Resources Information Center

    Litus, Tonyia J.

    Two sixth-grade, hearing-impaired students were studied to determine the effectiveness of stress management techniques using biofeedback instruments to monitor their nervous and cardiovascular systems. The male student had behavior problems, exhibiting explosive behavior without warning. The female student experienced excessive audible inhalations…

  5. Assessing Special Needs of Students with Hearing Impairment in Jordan and Its Relation to Some Variables

    ERIC Educational Resources Information Center

    El-Zraigat, Ibrahim A.

    2013-01-01

    The main purpose of the present study was to assess special needs for students with hearing impairment. The sample of the study consisted of 231 students enrolled at schools for deaf children in Jordan, 113 males and 118 females. A special needs scale was developed and used in assessing special needs for students. The reliability and validity of…

  6. Beyond the Sentence Level: What's in a Hearing-Impaired Child's Story?

    ERIC Educational Resources Information Center

    Yoshinaga-Itano, Christine

    1986-01-01

    A model for studying written language is described and illustrated in a written language sample of a 12-year-old with profound hearing impairment. Approaches are noted for analyzing semantic components, coherence, and causality. Implications for remediation are offered. (CL)

  7. Learning to Communicate: Babies with Hearing Impairments Make Their Needs Known.

    ERIC Educational Resources Information Center

    Yoshinaga-Itano, Christine; Stredler-Brown, Arlene

    1992-01-01

    This study of 82 hearing-impaired children (ages 6 to 36 months) sought to provide normative data concerning their communication efforts and to document the course of development of these earliest communication efforts. The study found that certain cognitive abilities and strategies are prerequisite to the development of certain language…

  8. Psycho-Social Aspects of Deafness Course as a Counseling Tool for the Hearing Impaired.

    ERIC Educational Resources Information Center

    Skyer, Solange C.

    1982-01-01

    A course, PsychoSocial Aspects of Deafness, is offered to hearing impaired students at the undergraduate level at the National Technical Institute for the Deaf, and is designed to enhance their understanding of the impact of deafness on their educational, psychological, emotional, and social development. Emphasis is placed on the affective as well…

  9. Locus of Control: Review and Implications for Instruction of the Hearing-Impaired.

    ERIC Educational Resources Information Center

    Dowaliby, Fred J.; Pagano, Jo Anne

    The authors review research on locus of control (LOC), particularly as it pertains to the instruction of hearing impaired students. According to social learning theory, the LOC construct is explained to theorize that two distinctly different types of persons may be identified--"internals" who assume responsibility for their activities and…

  10. 1970-1975 Follow-Up of Hearing Impaired Graduates of Pennsylvania.

    ERIC Educational Resources Information Center

    Powers, Gerry; Lewis, Jim

    A followup study of 167 hearing impaired graduates from Pennsylvania educational institutions from 1970-1975 was conducted. Four instruments--a student questionnaire, an educational history form, a parent followup survey, and an employer survey were used to gather data. Among results were that the jobs held by deaf Ss did not require the use of…

  11. Breaking the Sound Barrier: Working with Hearing-Impaired Adults in an Educational Setting.

    ERIC Educational Resources Information Center

    West, Lois

    College staff must understand the nature of hearing impairment, its effect on communication, and teaching methods that accommodate functional limitations in order to provide for the special needs of deaf students. Since the extent of a student's ability to communicate is determined by whether the student became deaf before or after he/she…

  12. Affective Films for the Hearing Impaired Child: A Test of Captioned Inside/Out Programs.

    ERIC Educational Resources Information Center

    Middleton, John

    Two captioned affective films from the AIT series Inside/Out were tested with 14 intermediate (ages 13 to 15) and 39 elementary (ages 11 and 12) hearing impaired children at the Hawaii School for the Deaf and Blind. Measures of attention, post-film discussion, student comprehension, and student and teacher opinions were taken to determine the…

  13. The Development, Dissemination, and Evaluation of Science Activities for Hearing-Impaired Children. Final Report.

    ERIC Educational Resources Information Center

    Barman, Charles R.; Shedd, Jill D.

    The main focus of this project was to prepare a handbook of science activities and to disseminate this handbook to K-8 teachers of the hearing-impaired in the State of Indiana. The handbook, titled "Doing Science Using the Learning Cycle," was developed by a select group of teachers and several consultants with expertise in curriculum…

  14. Test of Auditory Comprehension (TAC) for Hearing Impaired Pupils--Reliability and Validity Study.

    ERIC Educational Resources Information Center

    Hoversten, Gloria H.

    The Test of Auditory Comprehension (TAC), one component of the Auditory Skills Instructional Planning System, is a comprehensive test instrument designed for individual use with hearing impaired pupils, four through twelve years of age, for assessing receptive auditory functioning. Included in the TAC are ten discrete subtests measuring auditory…

  15. The Needs of the Deaf and Hearing Impaired. Legislative Document No. 99.

    ERIC Educational Resources Information Center

    New York State Temporary Commission to Study and Investigate the Problems of the Deaf, Albany.

    The Temporary State Commission to Study and Investigate the Problems of the Deaf presents a third annual report on the needs, services, and programs to aid the deaf and hearing impaired in New York State. The commission engaged in research activities to provide data necessary to substantiate legislation and to pursue the implementation of previous…

  16. Computer-Assisted Learning for the Hearing Impaired: An Interactive Written Language Enviroment.

    ERIC Educational Resources Information Center

    Ward, R. D.; Rostron, A. B.

    1983-01-01

    To help hearing-impaired children develop their linguistic competence, a computer system that can process sentences and give feedback about their acceptability was developed. Suggestions are made of ways to use the system as an environment for interactive written communication. (Author/CL)

  17. Progress and Prospects in Human Genetic Research into Age-Related Hearing Impairment

    PubMed Central

    Sugiura, Saiko; Ueda, Hiromi; Nakashima, Tsutomu

    2014-01-01

    Age-related hearing impairment (ARHI) is a complex, multifactorial disorder that is attributable to confounding intrinsic and extrinsic factors. The degree of impairment shows substantial variation between individuals, as is also observed in the senescence of other functions. This individual variation would seem to refute the stereotypical view that hearing deterioration with age is inevitable and may indicate that there is ample scope for preventive intervention. Genetic predisposition could account for a sizable proportion of interindividual variation. Over the past decade or so, tremendous progress has been made through research into the genetics of various forms of hearing impairment, including ARHI and our knowledge of the complex mechanisms of auditory function has increased substantially. Here, we give an overview of recent investigations aimed at identifying the genetic risk factors involved in ARHI and of what we currently know about its pathophysiology. This review is divided into the following sections: (i) genes causing monogenic hearing impairment with phenotypic similarities to ARHI; (ii) genes involved in oxidative stress, biologic stress responses, and mitochondrial dysfunction; and (iii) candidate genes for senescence, other geriatric diseases, and neurodegeneration. Progress and prospects in genetic research are discussed. PMID:25140308

  18. Identifying Current and Relevant Curricular Sequences for Multiply Involved Hearing-Impaired Learners.

    ERIC Educational Resources Information Center

    Schloss, Patrick J.

    1984-01-01

    The article provides guidelines for developing curriculum objectives for multiply involved hearing-impaired students. Emphasis is placed on procedures for establishing instructional priorities, assessing the learner against these priorities, selecting and using prosthetics to overcome learning and behavioral deficits, providing educational…

  19. Maternal Communicative Style with Children with Hearing Impairment--An Old Question Revisited

    ERIC Educational Resources Information Center

    Bhat, Rashmi J.; Nagaraja, M. N.

    2012-01-01

    Infants from birth itself are in constant interaction with their mothers and the environment. Thus the maternal styles of communication would play an important role in developing communicative competency in children. This process happens so naturally and incidentally that no one pays special attention to it. However, when a hearing impairment is…

  20. The Relationship of Cognitive Style Variables to Optimal Test Performance in Hearing-Impaired Children.

    ERIC Educational Resources Information Center

    Dillon, Ronna F.

    The relationship of cognitive style variables and conditions of test administration was investigated in cognitive assessments of hearing-impaired children, aged six through eleven. One hundred-twenty children were given the Raven Coloured Progressive Matrices (CPM) and a Piagetian battery under one of six conditions of testing: (1) standard; (2)…

  1. Investigation of the Home Reading Patterns of Families with Children with Hearing Impairment: ICEM Case

    ERIC Educational Resources Information Center

    Girgin, Umit

    2012-01-01

    Parents play a critical role in the literacy development of their children. The current case study explores the reading patterns that parents have with their children with hearing impairment. First, the home reading patterns of parents with their children were investigated. Then, the home reading patterns preferred by children were addressed along…

  2. Mother's Perspective toward Al-Quran Education for Hearing Impaired Children in Malaysia

    ERIC Educational Resources Information Center

    Ghadim, Nafiseh Alaghehband; Jomhari, Nazean; Alias, Norlidah; Rashid, Syar Meeze Mohd; Yusoff, Mohd Yakub Zulkifli Bin Mohd

    2013-01-01

    An interview with parents of children with hearing impairment was carried out in the initial study since the coordinated effort of parents and children is essential in the education of children. Considering that this interview was appropriate for collecting qualitative-oriented data, it has been chosen as the knowledge elicitation method. In most…

  3. Science Education for Hearing-Impaired Students in the Eighties: Priorities and Projections.

    ERIC Educational Resources Information Center

    Lang, Harry G.; And Others

    1983-01-01

    Needs and projections in science education for hearing impaired students fall into eight areas, including needs for quality teacher education, exemplary materials, infusion of career education into science curriculum offerings, increased involvement of professional organizations, and development of fundamental mathematics skills within the context…

  4. An Across-Frequency Processing Deficit in Listeners with Hearing Impairment Is Supported by Acoustic Correlation

    ERIC Educational Resources Information Center

    Healy, Eric W.; Kannabiran, Anand; Bacon, Sid P.

    2005-01-01

    It has been recently suggested that listeners having a sensorineural hearing impairment (HI) may possess a deficit in their ability to integrate speech information across different frequencies. When presented with a task that required across-frequency integration of speech patterns, listeners with HI performed more poorly than their normal-hearing…

  5. The Factors that Motivate and Hinder the Students with Hearing Impairment to Use Mobile Technology

    ERIC Educational Resources Information Center

    Kuzu, Abdullah

    2011-01-01

    This research which aims to find out the factors that motivate students with hearing impairment to use PDA (Personal Digital Assistant), a product of mobile technology, in instructional activities, interactions with their peers and instructors, and in their daily lives, and the factors that hinder these individuals from using PDA was designed as…

  6. The Ability of Hearing Impaired Children to Comprehend the Semantics of English Prepositions.

    ERIC Educational Resources Information Center

    Kluwin, Thomas N.

    Hearing impaired adolescents were administered one of four tests of syntax in preposition usage. Results indicated a clear developmental sequence: generally prepositions are first acquired in locative phrases, then as temporals, and then as manner relationships. The acquisition process is detailed. The author concludes that the complexity and…

  7. Physical therapy management for balance deficits in children with hearing impairments: A systematic review.

    PubMed

    Fernandes, Romita; Hariprasad, Shalini; Kumar, Vijaya K

    2015-08-01

    Childhood hearing impairment is a significant problem, associated with long-term academic, communicative and physical impairments. Studies have shown that children with hearing loss also present with balance and/or vestibular deficits along with complain of frequent falls. Various interventions have been studied to improvise postural control and balance in these children on different outcome measures. This review will provide the existing evidence on interventions to improve vestibular and/or balance functions. Three trials met our study criteria with PEDro score ≥6, and data were extracted, entered by two independent review authors. Though there was variability with regard to the focus and intensity of the intervention, subject characteristics and in outcome measures, vestibular rehabilitation has a positive influence on functional independence. Heterogeneity in the studies limits the comparisons of intervention programmes. We conclude that there was considerable evidence for a positive effect on balance outcomes among the hearing-impaired population with vestibular deficits. Further investigations of high-quality studies are needed to determine to compare interventions for improving vestibular deficits in hearing-impaired children.

  8. Conditions for Positive Psychological and Educational Development of Hearing Impaired Persons.

    ERIC Educational Resources Information Center

    Luckner, John L.

    1989-01-01

    Gathered information from deaf individuals (N=14) about positive psychological and educational development of hearing-impaired persons. Identified six common variables: supportive family climate; frequent and diverse experiences; educational curricula promoting problem-solving, independence, and self-reliance; supplementary opportunities for…

  9. Medications Used by Students with Visual and Hearing Impairments: Implications for Teachers.

    ERIC Educational Resources Information Center

    Kelley, Pat; And Others

    This document presents summary information in chart form on medications used by students with visual and hearing impairments. First, a checklist identifies educational considerations for students who are medicated. Next, common antipsychotic, anticonvulsant, antiasthmatic and other drugs are listed in chart form with drug name, indications, peak…

  10. Hearing Impairment and Undiagnosed Disease: The Potential Role of Clinical Recommendations

    ERIC Educational Resources Information Center

    Marlow, Nicole M.; Malaty, John; Jo, Ara; Tanner, Rebecca J.; Beau de Rochars, Valery M.; Carek, Peter J.; Mainous, Arch G., III

    2017-01-01

    Purpose: The objective of this study was to use cross-sectional, nationally representative data to examine the relationship between self-reported hearing impairment and undetected diabetes, hypertension, hypercholesterolemia, and chronic kidney disease. Method: We analyzed the National Health and Nutrition Examination Survey for the years…

  11. A Multisensory Language Approach to the Introduction of the Alphabet to Hearing Impaired Preschoolers.

    ERIC Educational Resources Information Center

    Jaworski, Anne Porter; Schroder, Ann

    The project was designed to develop a multisensory, language-oriented curriculum to introduce the letters of the alphabet to six hearing impaired preschoolers. Every week a new letter is introduced via such tasks as art and cooking activities, snacks, beginning sound picture cards, yarn and lacing letters, sandpaper letters, alphabet string beads,…

  12. Phonetics for the Hearing-Impaired University Student: An Alternate Strategy.

    ERIC Educational Resources Information Center

    Beaupre, Walter

    1985-01-01

    A hearing impaired undergraduate participated in all class lectures, classroom drills, and examinations involving oral dictation of transcription in a mainstreamed summer session. Success was ascribed to the university's use of cued speech, a phonemic system compatible with phonetic analysis and transcription. (Author/CL)

  13. The Spoken Language of Teachers and Pupils in the Education of Hearing-Impaired Children.

    ERIC Educational Resources Information Center

    Huntington, Alan; Watton, Faval

    1986-01-01

    Spoken language of 24 teachers and 131 hearing impaired students (6, 10, and 14-year levels) were analyzed for sentence length and complexity. Results revealed that the oral-alone (OA) teachers in OA institutions created richer language environments and helped children display relatively enhanced oral linguistic growth compared to laissez faire…

  14. Social Studies: A Resource Guide for Hearing-Impaired High School Students.

    ERIC Educational Resources Information Center

    Jaggers, Robert A.; Jaggers, Barbara A.

    The guide was written to give secondary level hearing impaired students exposure to real life problems from a social studies perspective. Units are outlined for the three levels of the high school years and provide an overview, information on vocabulary, target competencies, suggested activities, sources, and a list of objectives. The units are…

  15. Remediation of Phonation Problems in Hearing-Impaired Children: Speech Training and Sensory Aids.

    ERIC Educational Resources Information Center

    McGarr, Nancy S.; And Others

    1989-01-01

    Fifty hearing-impaired students, aged 8-12 and 15-19, who used an auditory, visual, or tactile aid in speech training made more progress than controls who received speech training without sensory aids. A visual display was more effective for conveying appropriate average pitch, while the "Portapitch" was useful for conveying dynamic…

  16. An Outline for Working with the Hearing Impaired in an Inpatient Substance Abuse Treatment Program.

    ERIC Educational Resources Information Center

    Wentzer, Carol; Dhir, Annie

    1986-01-01

    Guidelines for working with the hearing impaired in an inpatient substance abuse treatment program include recognition of the deaf culture, use of a qualified sign language interpreter, fluency in American Sign Language and deaf culture by the primary substance abuse counselor, and patient contact with recovering deaf persons. (DB)

  17. Family Education Program for Families of Preschool Hearing Impaired Children in Rural Alaska.

    ERIC Educational Resources Information Center

    Berglund, Jean B.; And Others

    Presented are the guidelines of an Alaskan educational program for native and nonnative rural families of preschool hearing impaired children. The program is described as involving initial identification of the handicapped child, residence for 1 week by the family (at no cost) in a demonstration home in Anchorage at which time parents are…

  18. Individual Literacy Activities with Hearing-Impaired Children in the Preschool Period

    ERIC Educational Resources Information Center

    Karasu, H. Pelin

    2014-01-01

    The ability to recognize sight words, phonological awareness, syntax, semantics, and pragmatic skills begins to develop during the preschool period, and is important for formal reading education. The purpose of this study was to define individualized studies that support the development of literacy skills among hearing-impaired preschool children.…

  19. The Effect of iPad on School Preparedness among Preschool Children with Hearing-Impairments

    ERIC Educational Resources Information Center

    Turkestani, Maryam Hafez

    2015-01-01

    With modern technological developments and with the fast expansion of mobile technical equipment, conducting a field study to find out how technology influences various developmental aspects of normal and special needs children at the preschool stage was deemed appropriate and timely hearing impairment. In this study aimed at finding out the…

  20. Deficiency of sphingomyelin synthase-1 but not sphingomyelin synthase-2 causes hearing impairments in mice.

    PubMed

    Lu, Mei-Hong; Takemoto, Makoto; Watanabe, Ken; Luo, Huan; Nishimura, Masataka; Yano, Masato; Tomimoto, Hidekazu; Okazaki, Toshiro; Oike, Yuichi; Song, Wen-Jie

    2012-08-15

    Sphingomyelin (SM) is a sphingolipid reported to function as a structural component of plasma membranes and to participate in signal transduction. The role of SM metabolism in the process of hearing remains controversial. Here, we examined the role of SM synthase (SMS), which is subcategorized into the family members SMS1 and SMS2, in auditory function. Measurements of auditory brainstem response (ABR) revealed hearing impairment in SMS1−/− mice in a low frequency range (4–16 kHz). As a possible mechanism of this impairment, we found that the stria vascularis (SV) in these mice exhibited atrophy and disorganized marginal cells. Consequently, SMS1−/− mice exhibited significantly smaller endocochlear potentials (EPs). As a possible mechanism for EP reduction, we found altered expression patterns and a reduced level of KCNQ1 channel protein in the SV of SMS1−/− mice. These mice also exhibited reduced levels of distortion product otoacoustic emissions. Quantitative comparison of the SV atrophy, KCNQ1 expression, and outer hair cell density at the cochlear apical and basal turns revealed no location dependence, but more macrophage invasion into the SV was observed in the apical region than the basal region, suggesting a role of cochlear location-dependent oxidative stress in producing the frequency dependence of hearing loss in SMS1−/− mice. Elevated ABR thresholds, decreased EPs, and abnormal KCNQ1 expression patterns in SMS1−/− mice were all found to be progressive with age. Mice lacking SMS2, however, exhibited neither detectable hearing loss nor changes in their EPs. Taken together, our results suggest that hearing impairments occur in SMS1−/− but not SMS2−/− mice. Defects in the SV with subsequent reductions in EPs together with hair cell dysfunction may account, at least partially, for hearing impairments in SMS1−/− mice.

  1. Screening of hearing impairment in the newborn using the auditory response cradle.

    PubMed Central

    Tucker, S M; Bhattacharya, J

    1992-01-01

    The Auditory Response Cradle (ARC) is a fully automated microprocessor controlled machine that was designed for the hearing screening of full term neonates. In order to evaluate the ARC, 6000 babies were screened at a district maternity hospital over a period of three years. Every infant subsequently entered a three year follow up programme. One hundred and two babies (1.7%) failed the ARC screen (that is, they failed two ARC tests) and 20 of these were found to have some hearing impairment: in 10 it was severe (80-90 dBHL), in seven moderate (45-60 dBHL), and in three it was mild to moderate (less than 45 dBHL). In addition, of the 20 babies who failed a first test and were discharged before a second could be performed, two were confirmed to have a severe hearing loss; 79 infants failing the screen were cleared on further testing, giving the ARC a false positive rate of 1.3%. On following up all 6000 infants for three years, seven children who passed the neonatal screen were subsequently found to have a hearing loss. For two babies the aetiology was unknown but for five the hearing impairment was either due to a hereditary progressive loss or definite postnatal factors. Progressive and acquired hearing losses cannot be detected at a neonatal screen and this emphasises the need for follow up screens at other stages in the child's life. In this long term study the ARC has been found to have a high detection rate for severe hearing loss and confirms the practical possibility of using a behavioural technique for the universal screening of hearing in neonates. Images Figure 1 Figure 2 PMID:1519957

  2. Association between idiopathic hearing loss and mitochondrial DNA mutations: A study on 169 hearing-impaired subjects

    PubMed Central

    GUARAN, VALERIA; ASTOLFI, LAURA; CASTIGLIONE, ALESSANDRO; SIMONI, EDI; OLIVETTO, ELENA; GALASSO, MARCO; TREVISI, PATRIZIA; BUSI, MICOL; VOLINIA, STEFANO; MARTINI, ALESSANDRO

    2013-01-01

    Mutations in mitochondrial DNA (mtDNA) have been shown to be an important cause of sensorineural hearing loss (SNHL). In this study, we performed a clinical and genetic analysis of 169 hearing-impaired patients and some of their relatives suffering from idiopathic SNHL, both familial and sporadic. The analysis of four fragments of their mtDNA identified several polymorphisms, the well known pathogenic mutation, A1555G, and some novel mutations in different genes, implying changes in the aminoacidic sequence. A novel sporadic mutation in 12S rRNA (MT-RNR1), not previously reported in the literature, was found in a case of possible aminoglycoside-induced progressive deafness. PMID:23969527

  3. Temporal Resolution of the Normal Ear in Listeners with Unilateral Hearing Impairment.

    PubMed

    Mishra, Srikanta K; Dey, Ratul; Davessar, Jai Lal

    2015-12-01

    Unilateral hearing loss (UHL) leads to an imbalanced input to the brain and results in cortical reorganization. In listeners with unilateral impairments, while the perceptual deficits associated with the impaired ear are well documented, less is known regarding the auditory processing in the unimpaired, clinically normal ear. It is commonly accepted that perceptual consequences are unlikely to occur in the normal ear for listeners with UHL. This study investigated whether the temporal resolution in the normal-hearing (NH) ear of listeners with long-standing UHL is similar to those in listeners with NH. Temporal resolution was assayed via measuring gap detection thresholds (GDTs) in within- and between-channel paradigms. GDTs were assessed in the normal ear of adults with long-standing, severe-to-profound UHL (N = 13) and age-matched, NH listeners (N = 22) at two presentation levels (30 and 55 dB sensation level). Analysis indicated that within-channel GDTs for listeners with UHL were not significantly different than those for the NH subject group, but the between-channel GDTs for listeners with UHL were poorer (by greater than a factor of 2) than those for the listeners with NH. The hearing thresholds in the normal or impaired ears were not associated with the elevated between-channel GDTs for listeners with UHL. Contrary to the common assumption that auditory processing capabilities are preserved for the normal ear in listeners with UHL, the current study demonstrated that a long-standing unilateral hearing impairment may adversely affect auditory perception--temporal resolution--in the clinically normal ear. From a translational perspective, these findings imply that the temporal processing deficits in the unimpaired ear of listeners with unilateral hearing impairments may contribute to their overall auditory perceptual difficulties.

  4. Effect of Cigarette Smoking and Passive Smoking on Hearing Impairment: Data from a Population–Based Study

    PubMed Central

    Chang, Jiwon; Ryou, Namhyung; Jun, Hyung Jin; Hwang, Soon Young; Song, Jae-Jun; Chae, Sung Won

    2016-01-01

    Objectives In the present study, we aimed to determine the effect of both active and passive smoking on the prevalence of the hearing impairment and the hearing thresholds in different age groups through the analysis of data collected from the Korea National Health and Nutrition Examination Survey (KNHANES). Study Design Cross-sectional epidemiological study. Methods The KNHANES is an ongoing population study that started in 1998. We included a total of 12,935 participants aged ≥19 years in the KNHANES, from 2010 to 2012, in the present study. Pure-tone audiometric (PTA) testing was conducted and the frequencies tested were 0.5, 1, 2, 3, 4, and 6 kHz. Smoking status was categorized into three groups; current smoking group, passive smoking group and non-smoking group. Results In the current smoking group, the prevalence of speech-frequency bilateral hearing impairment was increased in ages of 40−69, and the rate of high frequency bilateral hearing impairment was elevated in ages of 30−79. When we investigated the impact of smoking on hearing thresholds, we found that the current smoking group had significantly increased hearing thresholds compared to the passive smoking group and non-smoking groups, across all ages in both speech-relevant and high frequencies. The passive smoking group did not have an elevated prevalence of either speech-frequency bilateral hearing impairment or high frequency bilateral hearing impairment, except in ages of 40s. However, the passive smoking group had higher hearing thresholds than the non-smoking group in the 30s and 40s age groups. Conclusion Current smoking was associated with hearing impairment in both speech-relevant frequency and high frequency across all ages. However, except in the ages of 40s, passive smoking was not related to hearing impairment in either speech-relevant or high frequencies. PMID:26756932

  5. Unilateral versus bilateral amplification for adults with impaired hearing.

    PubMed

    Walden, Therese C; Walden, Brian E

    2005-09-01

    This study compared unilateral and bilateral aided speech recognition in background noise in 28 patients being fitted with amplification. Aided QuickSIN (Quick Speech-in-Noise test) scores were obtained for bilateral amplification and for unilateral amplification in each ear. In addition, right-ear directed and left-ear directed recall on the Dichotic Digits Test (DDT) was obtained from each participant. Results revealed that the vast majority of patients obtained better speech recognition in background noise on the QuickSIN from unilateral amplification than from bilateral amplification. There was a greater tendency for bilateral amplification to have a deleterious effect among older patients. Most frequently, better aided QuickSIN performance was obtained in the right ear of participants, despite similar hearing thresholds in both ears. Finally, patients tended to perform better on the DDT in the ear that provided less SNR loss on the QuickSIN. Results suggest that bilateral amplification may not always be beneficial in every daily listening environment when background noise is present, and it may be advisable for patients wearing bilateral amplification to remove one hearing aid when difficulty is encountered understanding speech in background noise.

  6. Management of hearing aid assembly by urban-dwelling hearing-impaired adults in a developed country: implications for a self-fitting hearing aid.

    PubMed

    Convery, Elizabeth; Keidser, Gitte; Hartley, Lisa; Caposecco, Andrea; Hickson, Louise; Meyer, Carly

    2011-12-01

    A self-fitting hearing aid, designed to be assembled and programmed without audiological or computer support, could bring amplification to millions of people in developing countries, who remain unaided due to the lack of a local, professional, audiological infrastructure. The ability to assemble and insert a hearing aid is fundamental to the successful use of a self-fitting device. In this study, the management of such tasks was investigated. Eighty older, urban-dwelling, hearing-impaired adults in a developed country were asked to follow a set of written, illustrated instructions to assemble two slim-fit behind-the-ear hearing aids. Participants were allowed to access assistance with the task from an accompanying partner. A range of personal and audiometric variables was measured through the use of structured questionnaires and standardized tests of health literacy, cognitive function, and manual dexterity. The results showed that 99% of participants were able to complete the hearing aid assembly task, either on their own or with assistance. Health literacy, or the ability to read and understand health-related text, and gender most strongly influenced participants' ability to complete the assembly task independently and accurately. Higher levels of health literacy were associated with an increased likelihood of independent and successful task completion. Male participants were more likely to complete the task on their own, while female participants were more likely to assemble the device without errors. The results of this study will inform future work regarding development of educational material for the self-fitting hearing aid as well as candidacy for such a device.

  7. Spatial release from masking in normally hearing and hearing-impaired listeners as a function of the temporal overlap of competing talkersa

    PubMed Central

    Best, Virginia; Mason, Christine R.; Kidd, Gerald

    2011-01-01

    Listeners with sensorineural hearing loss are poorer than listeners with normal hearing at understanding one talker in the presence of another. This deficit is more pronounced when competing talkers are spatially separated, implying a reduced “spatial benefit” in hearing-impaired listeners. This study tested the hypothesis that this deficit is due to increased masking specifically during the simultaneous portions of competing speech signals. Monosyllabic words were compressed to a uniform duration and concatenated to create target and masker sentences with three levels of temporal overlap: 0% (non-overlapping in time), 50% (partially overlapping), or 100% (completely overlapping). Listeners with hearing loss performed particularly poorly in the 100% overlap condition, consistent with the idea that simultaneous speech sounds are most problematic for these listeners. However, spatial release from masking was reduced in all overlap conditions, suggesting that increased masking during periods of temporal overlap is only one factor limiting spatial unmasking in hearing-impaired listeners. PMID:21428524

  8. Parasympathetic Nervous System Dysfunction, as Identified by Pupil Light Reflex, and Its Possible Connection to Hearing Impairment

    PubMed Central

    Zekveld, Adriana A.; Naylor, Graham; Ohlenforst, Barbara; Jansma, Elise P.; Lorens, Artur; Lunner, Thomas; Kramer, Sophia E.

    2016-01-01

    Context Although the pupil light reflex has been widely used as a clinical diagnostic tool for autonomic nervous system dysfunction, there is no systematic review available to summarize the evidence that the pupil light reflex is a sensitive method to detect parasympathetic dysfunction. Meanwhile, the relationship between parasympathetic functioning and hearing impairment is relatively unknown. Objectives To 1) review the evidence for the pupil light reflex being a sensitive method to evaluate parasympathetic dysfunction, 2) review the evidence relating hearing impairment and parasympathetic activity and 3) seek evidence of possible connections between hearing impairment and the pupil light reflex. Methods Literature searches were performed in five electronic databases. All selected articles were categorized into three sections: pupil light reflex and parasympathetic dysfunction, hearing impairment and parasympathetic activity, pupil light reflex and hearing impairment. Results Thirty-eight articles were included in this review. Among them, 36 articles addressed the pupil light reflex and parasympathetic dysfunction. We summarized the information in these data according to different types of parasympathetic-related diseases. Most of the studies showed a difference on at least one pupil light reflex parameter between patients and healthy controls. Two articles discussed the relationship between hearing impairment and parasympathetic activity. Both studies reported a reduced parasympathetic activity in the hearing impaired groups. The searches identified no results for pupil light reflex and hearing impairment. Discussion and Conclusions As the first systematic review of the evidence, our findings suggest that the pupil light reflex is a sensitive tool to assess the presence of parasympathetic dysfunction. Maximum constriction velocity and relative constriction amplitude appear to be the most sensitive parameters. There are only two studies investigating the

  9. Storms in Space: Bringing NASA Earth-Sun Science Educational Resources to Hearing- Impaired Students.

    NASA Astrophysics Data System (ADS)

    Lowry, K.; Sindt, M.; Jahn, J.

    2007-12-01

    Using assistive technology, children with hearing loss can actively participate in the hearing world. However, to develop the necessary skills, hearing-impaired students need to be immersed in a language-rich environment which compensates for the lack of "incidental" learning that typifies the language acquisition of their peers with typical hearing. For any subject matter taught in class, this means that the conceptual and language framework of the topic has to be provided in addition to regular class materials. In a collaboration between the Sunshine Cottage School for Deaf Children and the Southwest Research Institute, we are exploring how NASA-developed educational resources covering Space Science topics can be incorporated successfully in blended classrooms containing children with hearing loss and those with typical hearing in grades 3-5. Utilizing the extensive routine language monitoring performed at Sunshine Cottage, student progress is directly monitored during the year as well as from year to year. This allow us to evaluate the effectiveness of the resources used. Since all instruction at Sunshine Cottage is auditory-oral, our experiences in using those materials can be fed back directly into mainstream classrooms of the same grade levels.

  10. Affective Properties of Mothers' Speech to Infants With Hearing Impairment and Cochlear Implants

    PubMed Central

    Bergeson, Tonya R.; Xu, Huiping; Kitamura, Christine

    2015-01-01

    Purpose The affective properties of infant-directed speech influence the attention of infants with normal hearing to speech sounds. This study explored the affective quality of maternal speech to infants with hearing impairment (HI) during the 1st year after cochlear implantation as compared to speech to infants with normal hearing. Method Mothers of infants with HI and mothers of infants with normal hearing matched by age (NH-AM) or hearing experience (NH-EM) were recorded playing with their infants during 3 sessions over a 12-month period. Speech samples of 25 s were low-pass filtered, leaving intonation but not speech information intact. Sixty adults rated the stimuli along 5 scales: positive/negative affect and intention to express affection, to encourage attention, to comfort/soothe, and to direct behavior. Results Low-pass filtered speech to HI and NH-EM groups was rated as more positive, affective, and comforting compared with the such speech to the NH-AM group. Speech to infants with HI and with NH-AM was rated as more directive than speech to the NH-EM group. Mothers decreased affective qualities in speech to all infants but increased directive qualities in speech to infants with NH-EM over time. Conclusions Mothers fine-tune communicative intent in speech to their infant's developmental stage. They adjust affective qualities to infants' hearing experience rather than to chronological age but adjust directive qualities of speech to the chronological age of their infants. PMID:25679195

  11. [The prevalence of hearing impairment in transport workers and peculiarities of management of occupational loss of hearing (as exemplified by the situation in the air and railway transport)].

    PubMed

    Pankova, V B; Skryabina, L Yu; Kas'kov, Yu N

    2016-01-01

    This article presents data on the prevalence of hearing impairment among the workers engaged in the main means of transportation(air and railway transport). They show that the relative frequency of occupational loss of hearing in the cockpit members of commercial aviation amounts to one third of all cases of analogous diseases in this country. The main professional groups of transport works suffering from hearing impairment are constituted by the representatives of the so-called elite specialities, such as flying crew personnel, locomotive engineers, and their assistants. This fact constitutes an important aspect (not only of medical but also of socio-economic significance) of the problem under consideration. The high prevalence of professional hearing impairment among the transport workers is attributable to the high noise level in the cabins of locomotives and aircraft cockpits as well as to the inadequate expert and diagnostic work or imperfection of the regulatory documentation.

  12. Gated auditory speech perception in elderly hearing aid users and elderly normal-hearing individuals: effects of hearing impairment and cognitive capacity.

    PubMed

    Moradi, Shahram; Lidestam, Björn; Hällgren, Mathias; Rönnberg, Jerker

    2014-07-31

    This study compared elderly hearing aid (EHA) users and elderly normal-hearing (ENH) individuals on identification of auditory speech stimuli (consonants, words, and final word in sentences) that were different when considering their linguistic properties. We measured the accuracy with which the target speech stimuli were identified, as well as the isolation points (IPs: the shortest duration, from onset, required to correctly identify the speech target). The relationships between working memory capacity, the IPs, and speech accuracy were also measured. Twenty-four EHA users (with mild to moderate hearing impairment) and 24 ENH individuals participated in the present study. Despite the use of their regular hearing aids, the EHA users had delayed IPs and were less accurate in identifying consonants and words compared with the ENH individuals. The EHA users also had delayed IPs for final word identification in sentences with lower predictability; however, no significant between-group difference in accuracy was observed. Finally, there were no significant between-group differences in terms of IPs or accuracy for final word identification in highly predictable sentences. Our results also showed that, among EHA users, greater working memory capacity was associated with earlier IPs and improved accuracy in consonant and word identification. Together, our findings demonstrate that the gated speech perception ability of EHA users was not at the level of ENH individuals, in terms of IPs and accuracy. In addition, gated speech perception was more cognitively demanding for EHA users than for ENH individuals in the absence of semantic context.

  13. Masked speech perception across the adult lifespan: Impact of age and hearing impairment.

    PubMed

    Goossens, Tine; Vercammen, Charlotte; Wouters, Jan; van Wieringen, Astrid

    2017-02-01

    As people grow older, speech perception difficulties become highly prevalent, especially in noisy listening situations. Moreover, it is assumed that speech intelligibility is more affected in the event of background noises that induce a higher cognitive load, i.e., noises that result in informational versus energetic masking. There is ample evidence showing that speech perception problems in aging persons are partly due to hearing impairment and partly due to age-related declines in cognition and suprathreshold auditory processing. In order to develop effective rehabilitation strategies, it is indispensable to know how these different degrading factors act upon speech perception. This implies disentangling effects of hearing impairment versus age and examining the interplay between both factors in different background noises of everyday settings. To that end, we investigated open-set sentence identification in six participant groups: a young (20-30 years), middle-aged (50-60 years), and older cohort (70-80 years), each including persons who had normal audiometric thresholds up to at least 4 kHz, on the one hand, and persons who were diagnosed with elevated audiometric thresholds, on the other hand. All participants were screened for (mild) cognitive impairment. We applied stationary and amplitude modulated speech-weighted noise, which are two types of energetic maskers, and unintelligible speech, which causes informational masking in addition to energetic masking. By means of these different background noises, we could look into speech perception performance in listening situations with a low and high cognitive load, respectively. Our results indicate that, even when audiometric thresholds are within normal limits up to 4 kHz, irrespective of threshold elevations at higher frequencies, and there is no indication of even mild cognitive impairment, masked speech perception declines by middle age and decreases further on to older age. The impact of hearing

  14. Audio reproduction for personal ambient home assistance: concepts and evaluations for normal-hearing and hearing-impaired persons.

    PubMed

    Huber, Rainer; Meis, Markus; Klink, Karin; Bartsch, Christian; Bitzer, Joerg

    2014-01-01

    Within the Lower Saxony Research Network Design of Environments for Ageing (GAL), a personal activity and household assistant (PAHA), an ambient reminder system, has been developed. One of its central output modality to interact with the user is sound. The study presented here evaluated three different system technologies for sound reproduction using up to five loudspeakers, including the "phantom source" concept. Moreover, a technology for hearing loss compensation for the mostly older users of the PAHA was implemented and evaluated. Evaluation experiments with 21 normal hearing and hearing impaired test subjects were carried out. The results show that after direct comparison of the sound presentation concepts, the presentation by the single TV speaker was most preferred, whereas the phantom source concept got the highest acceptance ratings as far as the general concept is concerned. The localization accuracy of the phantom source concept was good as long as the exact listening position was known to the algorithm and speech stimuli were used. Most subjects preferred the original signals over the pre-processed, dynamic-compressed signals, although processed speech was often described as being clearer.

  15. Measuring the apparent width of auditory sources in normal and impaired hearing.

    PubMed

    Whitmer, William M; Seeber, Bernhard U; Akeroyd, Michael A

    2013-01-01

    It is often assumed that single sources of sound are perceived as being punctate, but this cannot be guaranteed, especially for hearing-impaired listeners. Any impairment that gives a reduction at the periphery in the accuracy of coding fine-scale temporal information must give a slight interaural jitter in the temporal information passed to higher centres, and so would be expected to lead to an effective reduction in the interaural coherence (IC) of any stimulus. This would lead to deficits in locating sounds, but deficits of imprecision, not inaccuracy. In turn, this implies that older hearing-impaired individuals should have a diminished perception of auditory space, affecting their abilities to perceive clear, concise, punctate spatial impressions or to separate sounds by location. The current work tested this hypothesis by using two separate visual-analogy methods to measure auditory source width for broadband sounds. In one method, the listener sketched the auditory image, a visual-description task, and for the other, the listener selected the closest one of a set of pre-drawn visual sketches (note that the first is an open-set experiment, whereas the second is a closed-set experiment). We found that older hearing-impaired listeners had increased difficulty in judging changes in interaural coherence, showing a corresponding insensitivity to auditory source width in the visual-analogy tasks.

  16. The global burden of disabling hearing impairment: a call to action

    PubMed Central

    Neumann, Katrin J; Saunders, James E

    2014-01-01

    Abstract At any age, disabling hearing impairment has a profound impact on interpersonal communication, psychosocial well-being, quality of life and economic independence. According to the World Health Organization’s estimates, the number of people with such impairment increased from 42 million in 1985 to about 360 million in 2011. This last figure includes 7.5 million children less than 5 years of age. In 1995, a “roadmap” for curtailing the burden posed by disabling hearing impairment was outlined in a resolution of the World Health Assembly. While the underlying principle of this roadmap remains valid and relevant, some updating is required to reflect the prevailing epidemiologic transition. We examine the traditional concept and grades of disabling hearing impairment – within the context of the International Classification of Functioning, Disability and Health – as well as the modifications to grading that have recently been proposed by a panel of international experts. The opportunity offered by the emerging global and high-level interest in promoting disability-inclusive post-2015 development goals and disability-free child survival is also discussed. Since the costs of rehabilitative services are so high as to be prohibitive in low- and middle-income countries, the critical role of primary prevention is emphasized. If the goals outlined in the World Health Assembly’s 1995 resolution on the prevention of hearing impairment are to be reached by Member States, several effective country-level initiatives – including the development of public–private partnerships, strong leadership and measurable time-bound targets – will have to be implemented without further delay. PMID:24839326

  17. The global burden of disabling hearing impairment: a call to action.

    PubMed

    Olusanya, Bolajoko O; Neumann, Katrin J; Saunders, James E

    2014-05-01

    At any age, disabling hearing impairment has a profound impact on interpersonal communication, psychosocial well-being, quality of life and economic independence. According to the World Health Organization's estimates, the number of people with such impairment increased from 42 million in 1985 to about 360 million in 2011. This last figure includes 7.5 million children less than 5 years of age. In 1995, a "roadmap" for curtailing the burden posed by disabling hearing impairment was outlined in a resolution of the World Health Assembly. While the underlying principle of this roadmap remains valid and relevant, some updating is required to reflect the prevailing epidemiologic transition. We examine the traditional concept and grades of disabling hearing impairment - within the context of the International Classification of Functioning, Disability and Health - as well as the modifications to grading that have recently been proposed by a panel of international experts. The opportunity offered by the emerging global and high-level interest in promoting disability-inclusive post-2015 development goals and disability-free child survival is also discussed. Since the costs of rehabilitative services are so high as to be prohibitive in low- and middle-income countries, the critical role of primary prevention is emphasized. If the goals outlined in the World Health Assembly's 1995 resolution on the prevention of hearing impairment are to be reached by Member States, several effective country-level initiatives - including the development of public-private partnerships, strong leadership and measurable time-bound targets - will have to be implemented without further delay.

  18. 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

  19. 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

  20. Informational Masking in Normal-Hearing and Hearing-Impaired Listeners Measured in a Nonspeech Pattern Identification Task.

    PubMed

    Roverud, Elin; Best, Virginia; Mason, Christine R; Swaminathan, Jayaganesh; Kidd, Gerald

    2016-04-08

    Individuals with sensorineural hearing loss (SNHL) often experience more difficulty with listening in multisource environments than do normal-hearing (NH) listeners. While the peripheral effects of sensorineural hearing loss certainly contribute to this difficulty, differences in central processing of auditory information may also contribute. To explore this issue, it is important to account for peripheral differences between NH and these hearing-impaired (HI) listeners so that central effects in multisource listening can be examined. In the present study, NH and HI listeners performed a tonal pattern identification task at two distant center frequencies (CFs), 850 and 3500 Hz. In an attempt to control for differences in the peripheral representations of the stimuli, the patterns were presented at the same sensation level (15 dB SL), and the frequency deviation of the tones comprising the patterns was adjusted to obtain equal quiet pattern identification performance across all listeners at both CFs. Tonal sequences were then presented at both CFs simultaneously (informational masking conditions), and listeners were asked either to selectively attend to a source (CF) or to divide attention between CFs and identify the pattern at a CF designated after each trial. There were large differences between groups in the frequency deviations necessary to perform the pattern identification task. After compensating for these differences, there were small differences between NH and HI listeners in the informational masking conditions. HI listeners showed slightly greater performance asymmetry between the low and high CFs than did NH listeners, possibly due to central differences in frequency weighting between groups.

  1. Informational Masking in Normal-Hearing and Hearing-Impaired Listeners Measured in a Nonspeech Pattern Identification Task

    PubMed Central

    Best, Virginia; Mason, Christine R.; Swaminathan, Jayaganesh; Kidd, Gerald

    2016-01-01

    Individuals with sensorineural hearing loss (SNHL) often experience more difficulty with listening in multisource environments than do normal-hearing (NH) listeners. While the peripheral effects of sensorineural hearing loss certainly contribute to this difficulty, differences in central processing of auditory information may also contribute. To explore this issue, it is important to account for peripheral differences between NH and these hearing-impaired (HI) listeners so that central effects in multisource listening can be examined. In the present study, NH and HI listeners performed a tonal pattern identification task at two distant center frequencies (CFs), 850 and 3500 Hz. In an attempt to control for differences in the peripheral representations of the stimuli, the patterns were presented at the same sensation level (15 dB SL), and the frequency deviation of the tones comprising the patterns was adjusted to obtain equal quiet pattern identification performance across all listeners at both CFs. Tonal sequences were then presented at both CFs simultaneously (informational masking conditions), and listeners were asked either to selectively attend to a source (CF) or to divide attention between CFs and identify the pattern at a CF designated after each trial. There were large differences between groups in the frequency deviations necessary to perform the pattern identification task. After compensating for these differences, there were small differences between NH and HI listeners in the informational masking conditions. HI listeners showed slightly greater performance asymmetry between the low and high CFs than did NH listeners, possibly due to central differences in frequency weighting between groups. PMID:27059627

  2. Temporal Fine-Structure Coding and Lateralized Speech Perception in Normal-Hearing and Hearing-Impaired Listeners

    PubMed Central

    Pedersen, Julie H.; Laugesen, Søren; Santurette, Sébastien; Dau, Torsten; MacDonald, Ewen N.

    2016-01-01

    This study investigated the relationship between speech perception performance in spatially complex, lateralized listening scenarios and temporal fine-structure (TFS) coding at low frequencies. Young normal-hearing (NH) and two groups of elderly hearing-impaired (HI) listeners with mild or moderate hearing loss above 1.5 kHz participated in the study. Speech reception thresholds (SRTs) were estimated in the presence of either speech-shaped noise, two-, four-, or eight-talker babble played reversed, or a nonreversed two-talker masker. Target audibility was ensured by applying individualized linear gains to the stimuli, which were presented over headphones. The target and masker streams were lateralized to the same or to opposite sides of the head by introducing 0.7-ms interaural time differences between the ears. TFS coding was assessed by measuring frequency discrimination thresholds and interaural phase difference thresholds at 250 Hz. NH listeners had clearly better SRTs than the HI listeners. However, when maskers were spatially separated from the target, the amount of SRT benefit due to binaural unmasking differed only slightly between the groups. Neither the frequency discrimination threshold nor the interaural phase difference threshold tasks showed a correlation with the SRTs or with the amount of masking release due to binaural unmasking, respectively. The results suggest that, although HI listeners with normal hearing thresholds below 1.5 kHz experienced difficulties with speech understanding in spatially complex environments, these limitations were unrelated to TFS coding abilities and were only weakly associated with a reduction in binaural-unmasking benefit for spatially separated competing sources. PMID:27601071

  3. Interactive Auroral Science for Hearing-Impaired Students

    NASA Astrophysics Data System (ADS)

    Samara, M.; Michell, R. G.; Jahn, J.; Pfeifer, M.; Ibarra, S.; Hampton, D. L.; Powell, D.

    2012-12-01

    Under a NASA E/PO grant, we have partnered with San Antonio's Sunshine Cottage School for Deaf Children to develop a science class experience where students directly interact with scientists and participate in a research-grade space science measurement campaign. The unique aspect of partnering with Sunshine Cottage lies in Sunshine's approach of auditory-verbal communication. Aided by technology (hearing aids, cochlear implants), a diverse student body with students of all levels of hearing loss (moderate through profound) is taught in an entirely auditory-verbal environment at Sunshine Cottage. Bringing these students into early contact with research work can lay the foundation for future careers in the STEM field that normally they might not consider as indicated by the first year of this collaboration where the student response was distinctly positive. Here we report on the first year of those activities, as they related to a ground based imaging approach to exploring the northern lights and from the point of view of the scientists that participated. The major components of that activity included a site visit to SwRI by the students and their teachers, a semester long lab at school utilizing current research tools and a real-time campaign night. The students used a number of diagnostics to first predict and then verify auroral activity. One of the tools used was the MOOSE observatory which is a community resource state of the art observatory comprised of 5 EMCCD imagers in Alaska, established through an NSF MRI grant. We will discuss the approach and lessons learned during the first year of the project and the directions that we will likely take in the second year. Lessons learned from teaching these students space science related topic can be flowed right back into mainstream classroom settings. One other significant and unexpected aspect of this first year was that we were able to connect two groups of students through skype (in the 4th to 5th grades) that

  4. The effects of hearing impairment, age, and hearing aids on the use of self motion for determining front/back location

    PubMed Central

    Owen Brimijoin, W.; Akeroyd, Michael A.

    2016-01-01

    Background There are two cues that listeners use to disambiguate the front/back location of a sound source: high-frequency spectral cues associated with the head and pinnae, and self-motion-related binaural cues. The use of these cues can be compromised in listeners with hearing impairment and users of hearing aids. Purpose To determine how age, hearing impairment, and the use of hearing aids affects a listener’s ability to determine front from back based on both self motion and spectral cues. Research Design We utilized a previously published front/back illusion: signals whose physical source location is rotated around the head at twice the angular rate of the listener’s head movements are perceptually located in the opposite hemifield from where they physically are. In normal hearing listeners the strength of this illusion decreases as a function of low-pass filter cutoff frequency; this is the result of a conflict between spectral cues and dynamic binaural cues for sound source location. The illusion was used as an assay of self-motion processing in listeners with hearing impairment and users of hearing aids. Study Sample We recruited 40 hearing impaired subjects, with an average age of 62 years. The data for three listeners were discarded because they did not move their heads enough during the experiment. Data Collection and Analysis Listeners sat at the center of a ring of 24 loudspeakers, turned their heads back and forth, and used a wireless keypad to report the front/back location of statically presented signals and of dynamically moving signals with illusory locations. Front/back accuracy for static signals, the strength of front/back illusions, and minimum audible movement angle was measured for each listener in each condition. All measurements were made in each listener both aided and unaided. Results Hearing impaired listeners were less accurate at front/back discrimination for both static and illusory conditions. Neither static nor illusory

  5. Associations between Visual, Hearing, and Dual Sensory Impairments and History of Motor Vehicle Collision Involvement by Older Drivers

    PubMed Central

    Green, Kimberly A.; McGwin, Gerald; Owsley, Cynthia

    2012-01-01

    Objectives To examine the association between visual and hearing impairment and motor vehicle collision (MVC) involvement in older drivers. Design Retrospective cohort study. Setting North central Alabama Participants Population-based sample of 2,000 licensed-drivers, age 70 and older. Measurements Visual acuity was measured using the Electronic Visual Acuity test. Contrast sensitivity was measured using the Pelli-Robson chart. Presence of subjective hearing loss and other health conditions were determined using a general health questionnaire. Information regarding MVCs for all participants spanning the five years prior to study enrollment was obtained from the Alabama Department of Public Safety. Results Following adjustment for age, race, gender, number of miles driven, number of medical conditions, general cognitive status, and visual processing speed, older drivers having both visual acuity and hearing impairment (rate ratio RR 1.52, 95% confidence interval CI 1.01–2.30), contrast sensitivity impairment alone (RR 1.42, 95% CI 1.00–2.02), and both contrast sensitivity and hearing impairment (RR 2.41, 95% CI 1.62–3.57) had elevated MVC rates, compared to drivers with no visual or hearing impairments. Drivers with visual acuity loss alone or hearing loss alone did not have significantly different MVC rates when compared to the no impairment group after adjustment for multiple variables. Conclusion Older drivers with dual sensory impairment are at greater MVC risk than those with only a visual acuity or a hearing deficit alone. A combined screening approach of screening for both hearing impairment and visual impairment may be a useful tool to identify older drivers at risk for MVC involvement. PMID:23350867

  6. Assessment of the genetic causes of recessive childhood non-syndromic deafness in the UK - implications for genetic testing.

    PubMed

    Hutchin, T; Coy, N N; Conlon, H; Telford, E; Bromelow, K; Blaydon, D; Taylor, G; Coghill, E; Brown, S; Trembath, R; Liu, X Z; Bitner-Glindzicz, M; Mueller, R

    2005-12-01

    Approximately one in 2000 children is born with a genetic hearing impairment, mostly inherited as a non-syndromic, autosomal recessive trait, for which more than 30 different genes have been identified. Previous studies have shown that one of these genes, connexin 26 (GJB2), accounts for 30-60% of such deafness, but the relative contribution of the many other genes is not known, especially in the outbred UK population. This lack of knowledge hampers the development of diagnostic genetic services for deafness. In an effort to determine the molecular aetiology of deafness in the population, 142 sib pairs with early-onset, non-syndromic hearing impairment were recruited. Those in whom deafness could not be attributed to GJB2 mutations were investigated further for other mapped genes. The genetic basis of 55 cases (38.7%) was established, 33.1% being due to mutations in the GJB2 gene and 3.5% due to mutations in SLC26A4. None of the remaining 26 loci investigated made a significant contribution to deafness in a Caucasian population. We suggest that screening the GJB2 and SLC26A4 genes should form the basis of any genetic testing programme for childhood deafness and highlight a number of important issues for consideration and future work.

  7. [Effect of the rate of speech flow on speech intelligibility in normal and hearing-impaired subjects].

    PubMed

    Hosoi, H; Murata, K; Ohta, F; Imaizumi, S

    1992-04-01

    The relationship between the rate of speech flow and speech intelligibility was investigated in normal and hearing-impaired subjects. It is usually observed that a slowly and clearly delivered speech is easier for hearing-impaired patients to understand. The purpose of paper is to analyze this phenomenon clinically and to present useful data for developing new hearing aids. Four or 5 syllabic words lists were prepared for this experiment and speech stimuli were chosen from these lists. The subjects consisted of 15 normal subjects and 79 hearing-impaired patients (57 with inner ear hearing loss and 22 with retrocochlear hearing loss). Hearing tests were performed using a tape recorder with various speech control systems in a soundproof room. Speech samples were presented at three speaking rates, that is, a conversational speech rate, a rate one and a half times as fast as the conversational speech rate and a rate twice as fast as that rate. The results obtained in the normal subjects confirmed that the faster the speaking rate was, the more the word intelligibility was reduced. According to the results in the hearing-impaired subjects, both the correlation coefficient and regression parameter between the word intelligibility in this experiment and speech discrimination scores measured by 57S-monosyllabic lists were low at the conversational speaking rate, but the higher the speaking rate was, the closer the relation between the both factors was. It was estimated by analyzing the data of inner ear hearing loss and retrocochler hearing loss separately that the subjects with retrocochler hearing loss had more difficulty in speech-mediated communication than the subjects with inner ear hearing loss.

  8. Summation bandwidths at threshold in normal and hearing-impaired listeners.

    PubMed

    Higgins, M B; Turner, C W

    1990-12-01

    The bandwidths for summation at threshold were measured for subjects with normal hearing and subjects with sensorineural hearing loss. Thresholds in quiet and in the presence of a masking noise were measured for complex stimuli consisting of 1 to 40 pure-tone components spaced 20 Hz apart. The single component condition consisted of a single pure tone at 1100 Hz; additional components were added below this frequency, in a replication of the Gässler [Acustica 4, 408-414 (1954)] procedure. For the normal subjects, thresholds increased approximately 3 dB per doubling of bandwidth for signal bandwidths exceeding the critical bandwidth. This slope was less for the hearing-impaired subjects. Summation bandwidths, as estimated from two-line fits, were wider for the hearing-impaired than for the normal subjects. These findings provide evidence that hearing-impaired subjects integrate sound energy over a wider-than-normal frequency range for the detection of complex signals. A second experiment used stimuli similar to those of Spiegel [J. Acoust. Soc. Am. 66, 1356-1363 (1979)], and added components both above and below the frequency of the initial component. Using these stimuli, the slope of the threshold increase beyond the critical bandwidth was approximately 1.5 dB per doubling of bandwidth, thus replicating the Spiegel (1979) experiment. It is concluded that the differences between the Gässler (1954) and Spiegel (1979) studies were due to the different frequency content of the stimuli used in each study. Based upon the present results, it would appear that the slope of threshold increase is dependent upon the direction of signal expansion, and the size of the critical bands into which the signal is expanded.

  9. The influence of informational masking on speech perception and pupil response in adults with hearing impairment.

    PubMed

    Koelewijn, Thomas; Zekveld, Adriana A; Festen, Joost M; Kramer, Sophia E

    2014-03-01

    A recent pupillometry study on adults with normal hearing indicates that the pupil response during speech perception (cognitive processing load) is strongly affected by the type of speech masker. The current study extends these results by recording the pupil response in 32 participants with hearing impairment (mean age 59 yr) while they were listening to sentences masked by fluctuating noise or a single-talker. Efforts were made to improve audibility of all sounds by means of spectral shaping. Additionally, participants performed tests measuring verbal working memory capacity, inhibition of interfering information in working memory, and linguistic closure. The results showed worse speech reception thresholds for speech masked by single-talker speech compared to fluctuating noise. In line with previous results for participants with normal hearing, the pupil response was larger when listening to speech masked by a single-talker compared to fluctuating noise. Regression analysis revealed that larger working memory capacity and better inhibition of interfering information related to better speech reception thresholds, but these variables did not account for inter-individual differences in the pupil response. In conclusion, people with hearing impairment show more cognitive load during speech processing when there is interfering speech compared to fluctuating noise.

  10. The who, when, where, what of daydreams among a young adult hearing-impaired population.

    PubMed

    Anthony, S

    1990-07-01

    A sample of 251 hearing-impaired college students responded to a daydreaming questionnaire. Only those responses from subjects who daydreamed 14-plus hours per week, or who daydreamed one and a half hours or less per week were used. This resulted in 40 high-frequency and 40 low-frequency daydreamers. Comparisons were made between male and female hearing impaired, high- and low-frequency daydreamers. The three significant differences were (a) when daydreaming occurred for these subjects; (b) time orientation of the daydreams; and (c) having an imaginary companion as a child. Although daydreaming did not differ significantly by frequency or sex in the students, other characteristics emerged. Subjects use actual, or desired, romantic partners as their central fantasy figures; change both themes and characters over a several month period; discuss their fantasies with a friend or family member; daydream most frequently while driving, while studying, or in class about the themes of love, sex, friendship, and owning things.

  11. Comparing two causal models of career maturity for hearing-impaired adolescents.

    PubMed

    King, S

    1990-01-01

    Conte (1983) suggested that existing theories of career development are inadequate for disabled populations because they fail to take into consideration the special life events and characteristics of people with a disability. The purpose of this study was to determine if Conte's reservations about contemporary theories could be supported by data. To this end, two causal models of career development were developed: one with five variables unique to the experience of the hearing impaired and the other without. Using data collected from 71 hearing-impaired adolescents, path analyses were conducted and the two models were compared for their ability to explain variance in career maturity. The results suggest that, although the second model may be more descriptive of the career development process for the deaf, it is no more powerful than the first in explaining variance in career maturity.

  12. Children's literature for the primary inclusive classroom: increasing understanding of children with hearing impairments.

    PubMed

    Turner, N D; Traxler, M

    1997-12-01

    As a result of recent federal legislation and positive research findings of educators, children with disabilities, including hearing impairment, are being included in general education classrooms. This development has many implications at the state, district, and classroom levels. While helping the child with disabilities assimilate into the general education classroom may appear to be the teacher's primary challenge, increasing awareness and understanding among those students without disabilities is a growing concern of educators. The use of children's literature featuring children with disabilities is one way to promote this understanding. This article includes an annotated bibliography of children's literature featuring children with hearing impairment, along with related classroom activities to enhance experiences with the books.

  13. Deaf Awareness: A Program To Increase Student Awareness of What It Is Like To Have a Hearing Impairment.

    ERIC Educational Resources Information Center

    Lambert, Tania

    Learning activities are presented to promote awareness of hearing impairments and to help children understand and accept people with disabilities. Through games, stories, information, and personal dialogue, students learn about hearing loss and communication methods as well as the broader issue of differences and the experience of being disabled.…

  14. The SKI*HI Model: Programming for Hearing Impaired Infants through Home Intervention, Home Visit Curriculum. Fourth Edition.

    ERIC Educational Resources Information Center

    Clark, Thomas C.; Watkins, Susan

    The manual describes the SKI*HI Model, a comprehensive approach to identification and home intervention treatment of hearing impaired children and their families. The model features home programing in four basic areas: the home hearing aid program (nine lessons which facilitate the proper fit and acceptance of amplification by the child), home…

  15. The Hearing Impaired Mentally/Retarded: A Survey of State Institutions for the Retarded. Monograph No. 4.

    ERIC Educational Resources Information Center

    Brannan, A. Clark; And Others

    Reported is a survey of state institutions for the mentally handicapped in which information about hearing imparied mentally handicapped HI/MH persons was solicited. Existing data on hearing impairment and mental retardation, its diagnosis and related programing are reviewed briefly. It is explained that 158 of 212 surveyed institutions (75…

  16. Improving word recognition in noise among hearing-impaired subjects with a single-channel cochlear noise-reduction algorithm.

    PubMed

    Fink, Nir; Furst, Miriam; Muchnik, Chava

    2012-09-01

    A common complaint of the hearing impaired is the inability to understand speech in noisy environments even with their hearing assistive devices. Only a few single-channel algorithms have significantly improved speech intelligibility in noise for hearing-impaired listeners. The current study introduces a cochlear noise reduction algorithm. It is based on a cochlear representation of acoustic signals and real-time derivation of a binary speech mask. The contribution of the algorithm for enhancing word recognition in noise was evaluated on a group of 42 normal-hearing subjects, 35 hearing-aid users, 8 cochlear implant recipients, and 14 participants with bimodal devices. Recognition scores of Hebrew monosyllabic words embedded in Gaussian noise at several signal-to-noise ratios (SNRs) were obtained with processed and unprocessed signals. The algorithm was not effective among the normal-hearing participants. However, it yielded a significant improvement in some of the hearing-impaired subjects under different listening conditions. Its most impressive benefit appeared among cochlear implant recipients. More than 20% improvement in recognition score of noisy words was obtained by 12, 16, and 26 hearing-impaired at SNR of 30, 24, and 18 dB, respectively. The algorithm has a potential to improve speech intelligibility in background noise, yet further research is required to improve its performances.

  17. Developmental hearing loss impairs signal detection in noise: putative central mechanisms

    PubMed Central

    Gay, Jennifer D.; Voytenko, Sergiy V.; Galazyuk, Alexander V.; Rosen, Merri J.

    2014-01-01

    Listeners with hearing loss have difficulty processing sounds in noisy environments. This is most noticeable for speech perception, but is reflected in a basic auditory processing task: detecting a tonal signal in a noise background, i.e., simultaneous masking. It is unresolved whether the mechanisms underlying simultaneous masking arise from the auditory periphery or from the central auditory system. Poor detection in listeners with sensorineural hearing loss (SNHL) is attributed to cochlear hair cell damage. However, hearing loss alters neural processing in the central auditory system. Additionally, both psychophysical and neurophysiological data from normally hearing and impaired listeners suggest that there are additional contributions to simultaneous masking that arise centrally. With SNHL, it is difficult to separate peripheral from central contributions to signal detection deficits. We have thus excluded peripheral contributions by using an animal model of early conductive hearing loss (CHL) that provides auditory deprivation but does not induce cochlear damage. When tested as adults, animals raised with CHL had increased thresholds for detecting tones in simultaneous noise. Furthermore, intracellular in vivo recordings in control animals revealed a cortical correlate of simultaneous masking: local cortical processing reduced tone-evoked responses in the presence of noise. This raises the possibility that altered cortical responses which occur with early CHL can influence even simple signal detection in noise. PMID:25249949

  18. Noise induced hearing loss impairs spatial learning/memory and hippocampal neurogenesis in mice

    PubMed Central

    Liu, Lijie; Shen, Pei; He, Tingting; Chang, Ying; Shi, Lijuan; Tao, Shan; Li, Xiaowei; Xun, Qingying; Guo, Xiaojing; Yu, Zhiping; Wang, Jian

    2016-01-01

    Hearing loss has been associated with cognitive decline in the elderly and is considered to be an independent risk factor for dementia. One of the most common causes for acquired sensorineural hearing loss is exposure to excessive noise, which has been found to impair learning ability and cognitive performance in human subjects and animal models. Noise exposure has also been found to depress neurogenesis in the hippocampus. However, the effect is mainly attributed to the oxidant stress of noise on the cognitive brain. In the present study, young adult CBA/CAJ mice (between 1.5 and 2 months of age) were briefly exposed a high sound level to produce moderate-to-severe hearing loss. In both the blood and hippocampus, only transient oxidative stress was observed after noise exposure. However, a deficit in spatial learning/memory was revealed 3 months after noise exposure. Moreover, the deficit was correlated with the degree of hearing loss and was associated with a decrease in neurogenesis in the hippocampus. We believe that the observed effects were likely due to hearing loss rather than the initial oxidant stress, which only lasted for a short period of time. PMID:26842803

  19. Dynamic Range Across Music Genres and the Perception of Dynamic Compression in Hearing-Impaired Listeners.

    PubMed

    Kirchberger, Martin; Russo, Frank A

    2016-02-10

    Dynamic range compression serves different purposes in the music and hearing-aid industries. In the music industry, it is used to make music louder and more attractive to normal-hearing listeners. In the hearing-aid industry, it is used to map the variable dynamic range of acoustic signals to the reduced dynamic range of hearing-impaired listeners. Hence, hearing-aided listeners will typically receive a dual dose of compression when listening to recorded music. The present study involved an acoustic analysis of dynamic range across a cross section of recorded music as well as a perceptual study comparing the efficacy of different compression schemes. The acoustic analysis revealed that the dynamic range of samples from popular genres, such as rock or rap, was generally smaller than the dynamic range of samples from classical genres, such as opera and orchestra. By comparison, the dynamic range of speech, based on recordings of monologues in quiet, was larger than the dynamic range of all music genres tested. The perceptual study compared the effect of the prescription rule NAL-NL2 with a semicompressive and a linear scheme. Music subjected to linear processing had the highest ratings for dynamics and quality, followed by the semicompressive and the NAL-NL2 setting. These findings advise against NAL-NL2 as a prescription rule for recorded music and recommend linear settings.

  20. Dynamic Range Across Music Genres and the Perception of Dynamic Compression in Hearing-Impaired Listeners

    PubMed Central

    Kirchberger, Martin

    2016-01-01

    Dynamic range compression serves different purposes in the music and hearing-aid industries. In the music industry, it is used to make music louder and more attractive to normal-hearing listeners. In the hearing-aid industry, it is used to map the variable dynamic range of acoustic signals to the reduced dynamic range of hearing-impaired listeners. Hence, hearing-aided listeners will typically receive a dual dose of compression when listening to recorded music. The present study involved an acoustic analysis of dynamic range across a cross section of recorded music as well as a perceptual study comparing the efficacy of different compression schemes. The acoustic analysis revealed that the dynamic range of samples from popular genres, such as rock or rap, was generally smaller than the dynamic range of samples from classical genres, such as opera and orchestra. By comparison, the dynamic range of speech, based on recordings of monologues in quiet, was larger than the dynamic range of all music genres tested. The perceptual study compared the effect of the prescription rule NAL-NL2 with a semicompressive and a linear scheme. Music subjected to linear processing had the highest ratings for dynamics and quality, followed by the semicompressive and the NAL-NL2 setting. These findings advise against NAL-NL2 as a prescription rule for recorded music and recommend linear settings. PMID:26868955

  1. 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

  2. Communication Variables Associated with Hearing-Impaired/Vision-Impaired Persons--A Pilot-Study.

    ERIC Educational Resources Information Center

    Hicks, Wanda M.

    1979-01-01

    A study involving eight youths and adults with retinitis pigmentosa (and only 20 degree visual field and hearing loss of at least 20 decibels) determined variance in the ability to perceive and comprehend visual stimuli presented by way of the manual modality when modifications were made in configuration, movement speed, movement size, and…

  3. Breaking the sound barrier: oral health education for children with hearing impairment.

    PubMed

    Shetty, Vabitha; Kumar, Jithendra; Hegde, Amitha

    2014-01-01

    In our study, a visual oral health education (OHE) program was specially designed for children with hearing impairment. Its efficacy in improving their oral health status was evaluated after periods of reinforcement and nonreinforcement. One hundred and ten institutionalized children with moderate-to-severe hearing impairment aged 6-14 years were selected for the study. Oral health status was evaluated at the start of the study (pre-OHE level) using the Modified Gingival Index (MGI) and the Turesky-Gilmore-Glickman modification of the Quigley Hein Plaque Index (MQPI). Salivary Streptococcus mutans levels of the children were also evaluated. Brushing skills were assessed using the Simmons index at the start of the study. Significant decreases were observed in the mean values of both the MGI and MQPI from the baseline up to the values obtained at the end of both periods of reinforcement and nonreinforcement. Significant reduction in S. mutans counts was observed, from Pre-OHE levels up to the levels at the end of the period of nonreinforcement. Brushing skills of children improved significantly at the end of study, notably in areas where brushing was previously deemed unsatisfactory. The OHE program specially formulated for the hearing impaired children was effective in improving their oral health status significantly.

  4. Adaptation of the Fresenius PD+ Cycler for a hearing-impaired patient.

    PubMed

    Kushner, A

    2000-01-01

    Continuous cycling peritoneal dialysis (CCPD) uses a cycler to perform dialysis exchanges and requires the patient to respond to an audible alarm signifying an interruption in the therapy. Consequently, an unassisted hearing-impaired patient could not use the system. By converting the standard alarm to a vibrating signal, the cycler was successfully adapted to accommodate the special needs of our hearing-impaired patient. The items required for the modification were the Sonic Alert Wake Up Alarm (Model SA-WA300: Sonic Alert, Troy, MI, U.S.A.) and the Sonic Alert Super Shaker Bed Vibrator (Model SA-SS120V: Sonic Alert). The patient can place the vibrator under either the pillow or the mattress. When the cycler alarm is activated, vibration wakens the patient. The equipment was purchased from Harris Communications (Eden Prairie, MN, U.S.A.) through a referral by the Easter Seal Society. Three days were needed to complete training compared to an average of one or two days for patients previously trained for continuous ambulatory peritoneal dialysis (CAPD). The patient remained on cycler therapy for approximately four months when the unrelated development of an abdominal hernia required termination of peritoneal dialysis and subsequent transfer to hemodialysis. In conclusion, a modified cycler can provide a safe and efficient renal replacement therapy option for a hearing-impaired patient.

  5. [Changes in the connexin 26 (GJB2) gene in Russian patients with hearing disorders: results of long-term molecular diagnostics of hereditary nonsyndromic deafness].

    PubMed

    Bliznets, E A; Galkina, V A; Matiushchenko, G N; Kisina, A G; Markova, T G; Poliakov, A V

    2012-01-01

    Search for mutations in the connexin 26 gene (GJB2) is a routine molecular-genetic analysis ofthe hereditary deafness worldwide. However, till now there is no assessment of the diagnostic significance of this analysis for Russian patients, and there are difficulties in interpretation of the results of DNA diagnostics. In the present study, a sample of 705 patients with nonsyndromic autosomal recessive deafness from different regions of Russian Federation was investigated. A portion of deafness like DFNB1 caused by mutations in the GJB2 gene among the sample was 46%. The frequency of deafness of such genetic type was 1:1000, that is, the frequency of isolated autosomal recessive deafness was 1:500 in the population. It was found that each sixteenth individual in Russia is a heterozygous carrier of the mutation in the GJB2gene. Totally, 20 pathological GJB2 alleles were detected; among them, a c.35delG mutation with the allelic frequency 81% prevails. Six most frequent mutations (c.35delG, c.313_326de114, c.-23+1G>A (IVS1+1G>A), c.235delC, c.167delT, and p.Glul20del), which account for 95% of pathological GJB2 alleles, were detected. Mutations previously not described in the GJB2 gene (c.129delG, p.Gly200Arg, and c[Arg127His, Gly160Ser]) were found. An optimal algorithm of molecular investigation of Russian patients which detects up to 100% of mutations in the GJB2 gene was suggested. Data concerning a clinical significance of p.Met34Thr and p.Va137Ile mutations are confirmed in the study. Eight polymorphic substitutions in the GJB2gene which do not have clinical significance (p.Va127Ile, c.*3C>A, p.Va115311e, p.Gly160Ser, c.Arg127His, p.Glull4Gly (c.341A>G), c.-45C>A, and p.Ala149Thr) were also detected.

  6. Novel Mutations and Mutation Combinations of TMPRSS3 Cause Various Phenotypes in One Chinese Family with Autosomal Recessive Hearing Impairment

    PubMed Central

    Wang, Guo-Jian; Xu, Jin-Cao; Su, Yu

    2017-01-01

    Autosomal recessive hearing impairment with postlingual onset is rare. Exceptions are caused by mutations in the TMPRSS3 gene, which can lead to prelingual (DFNB10) as well as postlingual deafness (DFNB8). TMPRSS3 mutations can be classified as mild or severe, and the phenotype is dependent on the combination of TMPRSS3 mutations. The combination of two severe mutations leads to profound hearing impairment with a prelingual onset, whereas severe mutations in combination with milder TMPRSS3 mutations lead to a milder phenotype with postlingual onset. We characterized a Chinese family (number FH1523) with not only prelingual but also postlingual hearing impairment. Three mutations in TMPRSS3, one novel mutation c.36delC [p.(Phe13Serfs⁎12)], and two previously reported pathogenic mutations, c.916G>A (p.Ala306Thr) and c.316C>T (p.Arg106Cys), were identified. Compound heterozygous mutations of p.(Phe13Serfs⁎12) and p.Ala306Thr manifest as prelingual, profound hearing impairment in the patient (IV: 1), whereas the combination of p.Arg106Cys and p.Ala306Thr manifests as postlingual, milder hearing impairment in the patient (II: 2, II: 3, II: 5), suggesting that p.Arg106Cys mutation has a milder effect than p.(Phe13Serfs⁎12). We concluded that different combinations of TMPRSS3 mutations led to different hearing impairment phenotypes (DFNB8/DFNB10) in this family. PMID:28246597

  7. Investigation of in-vehicle speech intelligibility metrics for normal hearing and hearing impaired listeners

    NASA Astrophysics Data System (ADS)

    Samardzic, Nikolina

    The effectiveness of in-vehicle speech communication can be a good indicator of the perception of the overall vehicle quality and customer satisfaction. Currently available speech intelligibility metrics do not account in their procedures for essential parameters needed for a complete and accurate evaluation of in-vehicle speech intelligibility. These include the directivity and the distance of the talker with respect to the listener, binaural listening, hearing profile of the listener, vocal effort, and multisensory hearing. In the first part of this research the effectiveness of in-vehicle application of these metrics is investigated in a series of studies to reveal their shortcomings, including a wide range of scores resulting from each of the metrics for a given measurement configuration and vehicle operating condition. In addition, the nature of a possible correlation between the scores obtained from each metric is unknown. The metrics and the subjective perception of speech intelligibility using, for example, the same speech material have not been compared in literature. As a result, in the second part of this research, an alternative method for speech intelligibility evaluation is proposed for use in the automotive industry by utilizing a virtual reality driving environment for ultimately setting targets, including the associated statistical variability, for future in-vehicle speech intelligibility evaluation. The Speech Intelligibility Index (SII) was evaluated at the sentence Speech Receptions Threshold (sSRT) for various listening situations and hearing profiles using acoustic perception jury testing and a variety of talker and listener configurations and background noise. In addition, the effect of individual sources and transfer paths of sound in an operating vehicle to the vehicle interior sound, specifically their effect on speech intelligibility was quantified, in the framework of the newly developed speech intelligibility evaluation method. Lastly

  8. Getting in and Getting On? The Experiences of Young People with Visual Impairments and Hearing Impairments in Third-Level Education

    ERIC Educational Resources Information Center

    Byrne, Bronagh

    2014-01-01

    Young disabled people continue to be under-represented throughout further and higher education settings. Drawing on Pierre Bourdieu's social theory of habitus, capital and field, this paper explores the practices of domination and oppression that have made it difficult for young people with visual impairments and hearing impairments to participate…

  9. Maternal Interactions with a Hearing and Hearing-Impaired Twin: Similarities and Differences in Speech Input, Interaction Quality, and Word Production

    ERIC Educational Resources Information Center

    Lam, Christa; Kitamura, Christine

    2010-01-01

    Purpose: This study examined a mother's speech style and interactive behaviors with her twin sons: 1 with bilateral hearing impairment (HI) and the other with normal hearing (NH). Method: The mother was video-recorded interacting with her twin sons when the boys were 12.5 and 22 months of age. Mean F0, F0 range, duration, and F1/F2 vowel space of…

  10. Association of Bone Mineral Density With Hearing Impairment in Postmenopausal Women in Korea

    PubMed Central

    Jung, Da Jung; Cho, Hyun Ho; Lee, Kyu-Yup

    2016-01-01

    Objectives Previous studies examining the association between osteoporosis (OP) and hearing loss (HL) have shown conflicting results. The objective of the present study was to examine the association between hearing impairment and OP in postmenopausal women, using appropriate statistical analyses. Methods Total 1,009 participants were included in the current study. The propensity score matched (PSM) cohort was defined as the cohort including participants diagnosed with OP and participants without OP. Three statistical models were developed where model 1 was unadjusted, model 2 included age, and model 3 included age, body mass index, alcohol intake, smoking habit, diabetes mellitus, hypertension, high density lipoprotein cholesterol, triglyceride level, vitamin D, and alkaline phosphatase. Results There were 776 and 233 participants in the groups diagnosed without and with OP, respectively. For propensity score matching, 233 pairs were selected from the 776 participants without OP. In the total cohort, using statistical models 2 and 3, no significant difference in the four hearing thresholds was identified between the 2 groups. Logistic regression indicated that, in model 3, participants with OP had a 1.128 (P=0.323) increased risk HL. A significant HL risk was not observed in participants with OP. Using statistical model 3, there were no significant associations among lumbar spine or femoral neck T-scores and changes in the hearing thresholds. In the PSM cohort, statistical models also showed similar results. Conclusion The current study did not demonstrate and association between bone mineral density and hearing impairment in the study population of postmenopausal Korean women. PMID:27136368

  11. Speech quality evaluation of a sparse coding shrinkage noise reduction algorithm with normal hearing and hearing impaired listeners.

    PubMed

    Sang, Jinqiu; Hu, Hongmei; Zheng, Chengshi; Li, Guoping; Lutman, Mark E; Bleeck, Stefan

    2015-09-01

    Although there are numerous papers describing single-channel noise reduction strategies to improve speech perception in a noisy environment, few studies have comprehensively evaluated the effects of noise reduction algorithms on speech quality for hearing impaired (HI). A model-based sparse coding shrinkage (SCS) algorithm has been developed, and has shown previously (Sang et al., 2014) that it is as competitive as a state-of-the-art Wiener filter approach in speech intelligibility. Here, the analysis is extended to include subjective quality ratings and a method called Interpolated Paired Comparison Rating (IPCR) is adopted to quantitatively link the benefit of speech intelligibility and speech quality. The subjective quality tests are performed through IPCR to efficiently quantify noise reduction effects on speech quality. Objective measures including frequency-weighted segmental signal-to-noise ratio (fwsegSNR), perceptual evaluation of speech quality (PESQ) and hearing aid speech quality index (HASQI) are adopted to predict the noise reduction effects. Results show little difference in speech quality between the SCS and the Wiener filter algorithm but a difference in quality rating between the HI and NH listeners. HI listeners generally gave better quality ratings of noise reduction algorithms than NH listeners. However, SCS reduced the noise more efficiently at the cost of higher distortions that were detected by NH but not by the HI. SCS is a promising candidate for noise reduction algorithms for HI. In general, care needs to be taken when adopting algorithms that were originally developed for NH participants into hearing aid applications. An algorithm that is evaluated negatively with NH might still bring benefits for HI participants.

  12. Comparing Binaural Pre-processing Strategies III: Speech Intelligibility of Normal-Hearing and Hearing-Impaired Listeners.

    PubMed

    Völker, Christoph; Warzybok, Anna; Ernst, Stephan M A

    2015-12-30

    A comprehensive evaluation of eight signal pre-processing strategies, including directional microphones, coherence filters, single-channel noise reduction, binaural beamformers, and their combinations, was undertaken with normal-hearing (NH) and hearing-impaired (HI) listeners. Speech reception thresholds (SRTs) were measured in three noise scenarios (multitalker babble, cafeteria noise, and single competing talker). Predictions of three common instrumental measures were compared with the general perceptual benefit caused by the algorithms. The individual SRTs measured without pre-processing and individual benefits were objectively estimated using the binaural speech intelligibility model. Ten listeners with NH and 12 HI listeners participated. The participants varied in age and pure-tone threshold levels. Although HI listeners required a better signal-to-noise ratio to obtain 50% intelligibility than listeners with NH, no differences in SRT benefit from the different algorithms were found between the two groups. With the exception of single-channel noise reduction, all algorithms showed an improvement in SRT of between 2.1 dB (in cafeteria noise) and 4.8 dB (in single competing talker condition). Model predictions with binaural speech intelligibility model explained 83% of the measured variance of the individual SRTs in the no pre-processing condition. Regarding the benefit from the algorithms, the instrumental measures were not able to predict the perceptual data in all tested noise conditions. The comparable benefit observed for both groups suggests a possible application of noise reduction schemes for listeners with different hearing status. Although the model can predict the individual SRTs without pre-processing, further development is necessary to predict the benefits obtained from the algorithms at an individual level.

  13. Hearing Impairment in Relation to Severity of Diabetes in a Veteran Cohort

    PubMed Central

    Konrad-Martin, Dawn; Reavis, Kelly M.; Austin, Donald; Reed, Nicholas; Gordon, Jane; McDermott, Dan; Dille, Marilyn F.

    2015-01-01

    Objective Type 2 diabetes is epidemic among veterans, approaching three times the prevalence of the general population. Diabetes leads to devastating complications of vascular and neurologic malfunction and appears to impair auditory function. Hearing loss prevention is a major health-related initiative in the Veterans Health Administration. Thus, this research sought to identify, and quantify with effect sizes, differences in hearing, speech recognition, and hearing-related quality of life (QOL) measures associated with diabetes and to determine whether well-controlled diabetes diminishes the differences. Design The authors examined selected cross-sectional data from the baseline (initial) visit of a longitudinal study of Veterans with and without type 2 diabetes designed to assess the possible differences in age-related trajectories of peripheral and central auditory function between the two groups. In addition, the diabetes group was divided into subgroups on the basis of medical diagnosis of diabetes and current glycated hemoglobin (HbA1c) as a metric of disease severity and control. Outcome measures were pure-tone thresholds, word recognition using sentences presented in noise or time-compressed, and an inventory assessing the self-perceived impact of hearing loss on QOL. Data were analyzed from 130 Veterans ages 24 to 73 (mean 48) years with well-controlled (controlled) diabetes, poorly controlled (uncontrolled) diabetes, prediabetes, and no diabetes. Regression was used to identify any group differences in age, noise exposure history, and other sociodemographic factors, and multiple regression was used to model each outcome variable, adjusting for potential confounders. Results were evaluated in relation to diabetes duration, use of insulin (yes, no), and presence of selected diabetes complications (neuropathy and retinopathy). Results Compared with nondiabetics, Veterans with uncontrolled diabetes had significant differences in hearing at speech frequencies

  14. Opinions of hearing parents about the causes of hearing impairment of their children with biallelic GJB2 mutations.

    PubMed

    Solovyev, Aisen V; Dzhemileva, Lilya U; Posukh, Olga L; Barashkov, Nikolay A; Bady-Khoo, Marita S; Lobov, Semen L; Popova, Natalya Yu; Romanov, Georgii P; Sazonov, Nikolay N; Bondar, Alexander A; Morozov, Igor V; Tomsky, Mikhail I; Fedorova, Sardana A; Khusnutdinova, Elza K

    2017-03-21

    Hereditary hearing impairment (HI) caused by recessive GJB2 mutations is a frequent sensory disorder. The results of the molecular-based studies of HI are widely used in various genetic test systems. However, the ethical aspects are less described than the genetic aspects. The concerns expressed by individuals from groups with genetic risks must be included in the counseling of patients and their families. For evaluation of subjective opinions of hearing parents about the presumed causes of HI of their children, we analyze the cohort of parents having children with confirmed hereditary HI caused by biallelic recessive GJB2 mutations (in a homozygous or a compound heterozygous state). This study included 70 deaf children with HI due to mutations in the GJB2 gene and 91 questionnaires about the presumed causes of their deafness filled by their parents. Most of the parents at 78% (CI 68.4-85.4%) attributed their children's HI to "non-hereditary" causes and 22% (CI 14.7-31.6%) to "hereditary" causes (p < 0.05). Therefore, the prior opinions of the parents did not correspond to positive GJB2 genetic testing results. The subjective opinions of parents are probably partly based on family history, since respondents with deaf relatives in their pedigree more likely supposed hereditary causes for HI in their children than the respondents without deaf relatives (p < 0.001).

  15. The sensitivity of hearing-impaired adults to acoustic attributes in simulated rooms

    PubMed Central

    Whitmer, William M.; McShefferty, David; Akeroyd, Michael A.

    2016-01-01

    In previous studies we have shown that older hearing-impaired individuals are relatively insensitive to changes in the apparent width of broadband noises when those width changes were based on differences in interaural coherence [W. Whitmer, B. Seeber and M. Akeroyd, J. Acoust. Soc. Am. 132, 369-379 (2012)]. This insensitivity has been linked to senescent difficulties in resolving binaural fine-structure differences. It is therefore possible that interaural coherence, despite its widespread use, may not be the best acoustic surrogate of spatial perception for the aged and impaired. To test this, we simulated the room impulse responses for various acoustic scenarios with differing coherence and lateral (energy) fraction attributes using room modelling software (ODEON). Bilaterally impaired adult participants were asked to sketch the perceived size of speech tokens and musical excerpts that were convolved with these impulse responses and presented to them in a sound-dampened enclosure through a 24-loudspeaker array. Participants’ binaural acuity was also measured using an interaural phase discrimination task. Corroborating our previous findings, the results showed less sensitivity to interaural coherence in the auditory source width judgments of older hearing-impaired individuals, indicating that alternate acoustic measurements in the design of spaces for the elderly may be necessary. PMID:27213028

  16. Effects of noise reduction on AM perception for hearing-impaired listeners.

    PubMed

    Ives, D Timothy; Kalluri, Sridhar; Strelcyk, Olaf; Sheft, Stanley; Miermont, Franck; Coez, Arnaud; Bizaguet, Eric; Lorenzi, Christian

    2014-10-01

    Noise reduction (NR) systems are commonplace in modern digital hearing aids. Though not improving speech intelligibility, NR helps the hearing-aid user in terms of lowering noise annoyance, reducing cognitive load and improving ease of listening. Previous psychophysical work has shown that NR does in fact improve the ability of normal-hearing (NH) listeners to discriminate the slow amplitude-modulation (AM) cues representative of those found in speech. The goal of this study was to assess whether this improvement of AM discrimination with NR can also be observed for hearing-impaired (HI) listeners. AM discrimination was measured at two audio frequencies of 500 Hz and 2 kHz in a background noise with a signal-to-noise ratio of 12 dB. Discrimination was measured for ten HI and ten NH listeners with and without NR processing. The HI listeners had a moderate sensorineural hearing loss of about 50 dB HL at 2 kHz and normal hearing (≤ 20 dB HL) at 500 Hz. The results showed that most of the HI listeners tended to benefit from NR at 500 Hz but not at 2 kHz. However, statistical analyses showed that HI listeners did not benefit significantly from NR at any frequency region. In comparison, the NH listeners showed a significant benefit from NR at both frequencies. For each condition, the fidelity of AM transmission was quantified by a computational model of early auditory processing. The parameters of the model were adjusted separately for the two groups (NH and HI) of listeners. The AM discrimination performance of the HI group (with and without NR) was best captured by a model simulating the loss of the fast-acting amplitude compression applied by the normal cochlea. This suggests that the lack of benefit from NR for HI listeners results from loudness recruitment.

  17. Auditory-nerve responses predict pitch attributes related to musical consonance-dissonance for normal and impaired hearing.

    PubMed

    Bidelman, Gavin M; Heinz, Michael G

    2011-09-01

    Human listeners prefer consonant over dissonant musical intervals and the perceived contrast between these classes is reduced with cochlear hearing loss. Population-level activity of normal and impaired model auditory-nerve (AN) fibers was examined to determine (1) if peripheral auditory neurons exhibit correlates of consonance and dissonance and (2) if the reduced perceptual difference between these qualities observed for hearing-impaired listeners can be explained by impaired AN responses. In addition, acoustical correlates of consonance-dissonance were also explored including periodicity and roughness. Among the chromatic pitch combinations of music, consonant intervals/chords yielded more robust neural pitch-salience magnitudes (determined by harmonicity/periodicity) than dissonant intervals/chords. In addition, AN pitch-salience magnitudes correctly predicted the ordering of hierarchical pitch and chordal sonorities described by Western music theory. Cochlear hearing impairment compressed pitch salience estimates between consonant and dissonant pitch relationships. The reduction in contrast of neural responses following cochlear hearing loss may explain the inability of hearing-impaired listeners to distinguish musical qualia as clearly as normal-hearing individuals. Of the neural and acoustic correlates explored, AN pitch salience was the best predictor of behavioral data. Results ultimately show that basic pitch relationships governing music are already present in initial stages of neural processing at the AN level.

  18. Association of Age Related Macular Degeneration and Age Related Hearing Impairment

    PubMed Central

    Ghasemi, Hassan; Pourakbari, Malihe Shahidi; Entezari, Morteza; Yarmohammadi, Mohammad Ebrahim

    2016-01-01

    Purpose: To evaluate the association between age-related macular degeneration (ARMD) and sensory neural hearing impairment (SHI). Methods: In this case-control study, hearing status of 46 consecutive patients with ARMD were compared with 46 age-matched cases without clinical ARMD as a control group. In all patients, retinal involvements were confirmed by clinical examination, fluorescein angiography (FA) and optical coherence tomography (OCT). All participants were examined with an otoscope and underwent audiological tests including pure tone audiometry (PTA), speech reception threshold (SRT), speech discrimination score (SDS), tympanometry, reflex tests and auditory brainstem response (ABR). Results: A significant (P = 0.009) association was present between ARMD, especially with exudative and choroidal neovascularization (CNV) components, and age-related hearing impairment primarily involving high frequencies. Patients had higher SRT and lower SDS against anticipated presbycusis than control subjects. Similar results were detected in exudative, CNV and scar patterns supporting an association between late ARMD with SRT and SDS abnormalities. ABR showed significantly prolonged wave I and IV latency times in ARMD (P = 0.034 and 0.022, respectively). Average latency periods for wave I in geographic atrophy (GA) and CNV, and that for wave IV in drusen patterns of ARMD were significantly higher than controls (P = 0.030, 0.007 and 0.050, respectively). Conclusion: The association between ARMD and age-related SHI may be attributed to common anatomical components such as melanin in these two sensory organs. PMID:27195086

  19. MEHIDA: an intelligent multimedia tutoring system for the hearing-impaired

    NASA Astrophysics Data System (ADS)

    Alonso, Fernando; de Antonio, Angelica; Fuertes, Jose L.; Montes, Cesar

    1995-03-01

    MEHIDA is a multimedia system offering hearing-impaired children an easy and attractive method to communicate with their hearing and deaf peers. It is a TOTAL COMMUNICATION method whose objective is the acquisition of various forms of communication available to the hearing impaired simultaneously: gesture, speech, dactylology, formal signing, lip reading, reading and writing. Didactic activities and games are used to teach the different means of communication. The approach gives the child the chance to practice the different types of communication. A character has been created in the shape of a pear to assist and guide the child. The pupil identifies with the character at all times, as it explains what the child is being asked to do during each activity. The MEHIDA learning process is divided into six stages: basic learning, prereading and prewriting, syllable, word, simple and complex sentence reading and writing. Each phase establishes a hierarchy of didactic objectives which are the expression of the skills and knowledge to be acquired by the child during the learning process (e.g., learning concepts of similarity) broken down into a series of lower level operational objectives (e.g., select figures of the same shape, size and color).

  20. Comparative multivariate analyses of transient otoacoustic emissions and distorsion products in normal and impaired hearing

    PubMed Central

    STAMATE, MIRELA CRISTINA; TODOR, NICOLAE; COSGAREA, MARCEL

    2015-01-01

    Background and aim The clinical utility of otoacoustic emissions as a noninvasive objective test of cochlear function has been long studied. Both transient otoacoustic emissions and distorsion products can be used to identify hearing loss, but to what extent they can be used as predictors for hearing loss is still debated. Most studies agree that multivariate analyses have better test performances than univariate analyses. The aim of the study was to determine transient otoacoustic emissions and distorsion products performance in identifying normal and impaired hearing loss, using the pure tone audiogram as a gold standard procedure and different multivariate statistical approaches. Methods The study included 105 adult subjects with normal hearing and hearing loss who underwent the same test battery: pure-tone audiometry, tympanometry, otoacoustic emission tests. We chose to use the logistic regression as a multivariate statistical technique. Three logistic regression models were developed to characterize the relations between different risk factors (age, sex, tinnitus, demographic features, cochlear status defined by otoacoustic emissions) and hearing status defined by pure-tone audiometry. The multivariate analyses allow the calculation of the logistic score, which is a combination of the inputs, weighted by coefficients, calculated within the analyses. The accuracy of each model was assessed using receiver operating characteristics curve analysis. We used the logistic score to generate receivers operating curves and to estimate the areas under the curves in order to compare different multivariate analyses. Results We compared the performance of each otoacoustic emission (transient, distorsion product) using three different multivariate analyses for each ear, when multi-frequency gold standards were used. We demonstrated that all multivariate analyses provided high values of the area under the curve proving the performance of the otoacoustic emissions. Each

  1. Phonological development in 4-6-year-old moderately hearing impaired children.

    PubMed

    Huttunen, K H

    2001-01-01

    The acquisition of clear phonological rules is of utmost importance for a child when he/she is learning to decode and convey meanings using spoken language. Fifteen Finnish-speaking children, five normally hearing (NH) 3-year-olds and 10 moderately hearing impaired (HId) 4-6-year-olds were studied here. Speech samples consisting of 62 words were collected from each child during a picture naming task. Frequent phonetic (articulation) errors together with normal (but delayed) and deviant phonological processes of HId children were found in this study. The frequency and quality of the processes seemed to be related more to the grade of HI than, for example, the age of the child. Implications for speech therapy are briefly discussed.

  2. The perception of apparent auditory source width in hearing-impaired adults

    PubMed Central

    Whitmer, William M.; Seeber, Bernhard U.; Akeroyd, Michael A.

    2014-01-01

    In a previous study [W. Whitmer, B. Seeber and M. Akeroyd, J. Acoust. Soc. Am. 132, 369-379 (2012)], it was demonstrated that older hearing-impaired (HI) listeners produced visual sketches of headphone-presented noises that were insensitive to changes in interaural coherence. The current study further explores this insensitivity by comparing (a) binaural temporal fine-stucture (TFS) resolution and (b) sound localization precision to (c) auditory source width judgments. Thirty-five participants aged 26-81 years with normal to moderately impaired hearing (a) discriminated interaurally phase-shifted tones from diotic tones presented over headphones, (b) located 500-ms speech-spectrum filtered click trains presented over loudspeakers between ±30° in quiet, and (c) sketched the perceived width of low-pass, high-pass and speech-spectrum noise stimuli presented over loudspeakers from 0° and simultaneously from ±45° at attenuations of 0-20 dB to generate partially coherent stimuli. The results showed a decreasing sensitivity to width with age and impairment which was related to binaural TFS threshold: the worse one’s threshold – which was correlated with age – the less the perceived width increased with decreasing interaural coherence. These results suggest that senescent changes to the auditory system do not necessarily lead to perceptions of broader, more diffuse sound images based on interaural coherence. PMID:24907818

  3. Noise Exposure and Hearing Impairment among Chinese Restaurant Workers and Entertainment Employees in Hong Kong

    PubMed Central

    Lao, Xiang Qian; Yu, Ignatius Tak Sun; Au, Dennis Kin Kwok; Chiu, Yuk Lan; Wong, Claudie Chiu Yi; Wong, Tze Wai

    2013-01-01

    Background Noise-induced hearing loss (NIHL) is a major concern in the non-manufacturing industries. This study aimed to investigate the occupational noise exposure and the NIHL among Chinese restaurant workers and entertainment employees working in the service industry in Hong Kong. Methods This cross-sectional survey involved a total of 1,670 participants. Among them, 937 were randomly selected from the workers of Chinese restaurants and 733 were selected from workers in three entertainment sectors: radio and television stations; cultural performance halls or auditoria of the Leisure and Cultural Services Department (LCSD); and karaoke bars. Noise exposure levels were measured in the sampled restaurants and entertainment sectors. Each participant received an audiometric screening test. Those who were found to have abnormalities were required to take another diagnostic test in the health center. The “Klockhoff digit” method was used to classify NIHL in the present study. Results The main source of noise inside restaurants was the stoves. The mean hearing thresholds showed a typical dip at 3 to 6 KHz and a substantial proportion (23.7%) of the workers fulfilled the criteria for presumptive NIHL. For entertainment sectors, employees in radio and television stations generally had higher exposure levels than those in the halls or auditoria of the LCSD and karaoke bars. The mean hearing thresholds showed a typical dip at 6 KHz and a substantial proportion of the employees fulfilled the criteria for presumptive NIHL (38.6%, 95%CI: 35.1–42.1%). Being male, older, and having longer service and daily alcohol consumption were associated with noise-induced hearing impairment both in restaurant workers and entertainment employees. Conclusion Excessive noise exposure is common in the Chinese restaurant and entertainment industries and a substantial proportion of restaurant workers and entertainment employees suffer from NIHL. Comprehensive hearing conservation programs

  4. Smoking, Central Adiposity, and Poor Glycemic Control Increase Risk of Hearing Impairment

    PubMed Central

    Cruickshanks, Karen J.; Nondahl, David M.; Dalton, Dayna S.; Fischer, Mary E.; Klein, Barbara E.K.; Klein, Ronald; Nieto, F. Javier; Schubert, Carla R.; Tweed, Ted S.

    2015-01-01

    Objectives To determine associations between smoking, adiposity, diabetes, and other cardiovascular disease (CVD) risk factors and the 15-yr incidence of hearing impairment (HI). Design The Epidemiology of Hearing Loss Study (EHLS) is a longitudinal population-based cohort study (1993–95 to 2009–2010). Setting Beaver Dam, WI. Participants Participants in the Beaver Dam Eye Study (1988–90; residents of Beaver Dam, WI ages 43–84 years in 1987–88) were eligible for the EHLS. There were 1925 participants with normal hearing at baseline. Measurements 15-year cumulative incidence of HI (pure-tone average (PTA) of hearing thresholds at 0.5, 1, 2 and 4 kHz > 25 decibels Hearing Level (dB HL) in either ear). Cigarette smoking, exercise, and other factors were ascertained by questionnaire. Blood pressure, waist circumference, body mass index and glycosylated hemoglobin were measured. Results Follow-up examinations (≥1) were obtained from 87.2% (n=1678; mean baseline age 61 years). The 15-year cumulative incidence of HI was 56.8%. Adjusting for age and sex, current smoking (Hazard Ratio (HR) =1.31, p=0.048), education (<16 yrs; HR=1.35, p=0.01), waist circumference (HR=1.08 per 10 cm, p=0.017), and poorly controlled diabetes (HR=2.03, p=0.048) were associated with increased risk of HI. Former smokers and people with better controlled diabetes were not at increased risk. Conclusion Smoking, central adiposity and poorly controlled diabetes predicted incident HI. These well-known CVD risk factors, suggest vascular changes may contribute to HI in aging. Interventions targeting reductions in smoking and adiposity, and improved glycemic control in people with diabetes, may help to prevent or delay the onset of HI. PMID:25953199

  5. Does Hamulotomy during Palatoplasty Have Any Effect on Hearing Ability in Nonsyndromic Cleft Palate Patients? A Prospective, Single Blind, Comparative Study

    PubMed Central

    Jain, Anuj; Nimonkar, Pranali; Bhola, Nitin; Borle, Rajiv; Sharma, Shishir; Oswal, Shrenik

    2016-01-01

    The primary goal of palatoplasty is to achieve a tension-free palatal closure ensuring no postoperative complications. Many surgeons fracture the pterygoid hamulus to minimize tension during palatoplasty. However, this maneuver gained criticism by some authors on the grounds that it may lead to Eustachian Tube dysfunction. Our study intended to figure out the relationship of hamulus fracture with the postoperative state of middle ear in cleft palate children. Fifty consecutive cleft palate patients with an age range of 10 months to 5 years were recruited. All the patients were assigned to either hamulotomy or nonhamulotomy group preoperatively. The patients were subjected to otoscopic examination and auditory function evaluation by brainstem evoked response audiometry (BERA) preoperatively and 1 month and 6 months postoperatively. Otoscopy revealed that the difference in the improvement of middle ear status in both groups was statistically insignificant. Moreover, there was no significant difference in the BERA outcomes of the fracture and nonfracture populations. Complication rate in both groups was also statistically not significant. It can be concluded that hamulotomy does not have any effect on the hearing ability in cleft palate population, so hamulotomy can be performed for tension-free closure during palatoplasty. PMID:27006862

  6. [The nursing process in helping a family with foreign mother and hearing impaired child].

    PubMed

    Wu, Meei-Lian; Tang, Jing-Shia

    2004-12-01

    This case report aims to present a nursing experience involving a child with severe hearing impairment and delayed language development. The patient was discovered during a home visit. At the time she was two and a half years old, but still had not developed any language behavior. She only used eye contact, physical touch, and body language to communicate with her family. She also did not respond to sound stimulation. The results of a Denver Developmental Screening Test (DDST) showed delayed development, especially of language. The child's mother is from Vietnam. The culture, education, language, and environment of Vietnam are totally different from Taiwan. In addition, the mother did not know how to raise her child. So the author tried to follow up on the case. Data were collected by home visits, phone calls, interviews, and communication with members of a professional health care team during the nursing care period (about six months). Data were recorded and it was written a processing analyzed. They revealed five health problems, as follows: (1) hearing impairment causing delayed language development; (2) poor family recognition deviation understanding of delayed development; (3) insufficient community resources; (4) low self-protection, limited capacity for caused by hearing impairment; (5) foreign mother's sense of helplessness about raising the child. The author provided supportive care to the patient and her family, counseled them, and transferred the child quickly to a treatment center. She also coordinated resources and the professional care team in assisting the parents in facing and adapting to the child's developmental delay. As a result, the parents gained knowledge and the ability to make judgments about developmental delay. This fostered a positive attitude on their part and acceptance of the child's admission to the treatment center. The child and family could deal with their problems appropriately because the nurse intervened at the appropriate time

  7. Cognitive-behavioral therapy for insomnia co-morbid with hearing impairment: a randomized controlled trial.

    PubMed

    Jansson-Fröjmark, Markus; Linton, Steven J; Flink, Ida K; Granberg, Sarah; Danermark, Berth; Norell-Clarke, Annika

    2012-06-01

    The purpose of the current study was to examine the effects of cognitive behavior therapy (CBT-I) for insomnia on patients with insomnia co-morbid with hearing impairment. A randomized controlled design was used with a 3-month follow-up. Thirty-two patients with insomnia co-morbid with hearing impairment were randomized to either CBT-I or a waitlist condition (WLC). The primary outcome was insomnia severity. Secondary outcomes were sleep diary parameters, dysfunction, anxiety, and depression. Compared to WLC, CBT-I resulted in lower insomnia severity at post-treatment and at follow-up (d = 1.18-1.56). Relative to WLC, CBT-I also led, at both assessment points, to reduced total wake time (d = 1.39) and increased sleep restoration (d = 1.03-1.07) and sleep quality (d = 0.91-1.16). Both groups increased their total sleep time, but no significant group difference emerged. Compared to WLC, CBT-I resulted in higher function (d = 0.81-0.96) and lower anxiety (d = 1.29-1.30) at both assessment points. Neither CBT-I nor WLC led to improvement on depression. Based on the Insomnia Severity Index, more CBT-I (53-77%) than WLC participants (0-7%) were treatment responders. Also, more CBT-I (24%) than WLC participants (0%) remitted. In patients with insomnia co-morbid with hearing impairment, CBT-I was effective in decreasing insomnia severity, subjective sleep parameters, dysfunction, and anxiety. These findings are in line with previous results on the effects of CBT-I in other medical conditions.

  8. "They Say I'm Stupid, but I Just Don't HEAR": Hearing-Impaired Adults' View of Finnish Society. Research Report 142.

    ERIC Educational Resources Information Center

    Takala, Marjatta

    Experiences of Finnish adults with hearing impairments (n=186) and their views of Finnish society were studied, and methods of collecting data were compared. Study questions focused on knowledge about and satisfaction with Finnish society, participation in activities, and values and attitudes. Respondents who were deaf (n=77) and those who were…

  9. Auditory and auditory-visual intelligibility of speech in fluctuating maskers for normal-hearing and hearing-impaired listeners.

    PubMed

    Bernstein, Joshua G W; Grant, Ken W

    2009-05-01

    Speech intelligibility for audio-alone and audiovisual (AV) sentences was estimated as a function of signal-to-noise ratio (SNR) for a female target talker presented in a stationary noise, an interfering male talker, or a speech-modulated noise background, for eight hearing-impaired (HI) and five normal-hearing (NH) listeners. At the 50% keywords-correct performance level, HI listeners showed 7-12 dB less fluctuating-masker benefit (FMB) than NH listeners, consistent with previous results. Both groups showed significantly more FMB under AV than audio-alone conditions. When compared at the same stationary-noise SNR, FMB differences between listener groups and modalities were substantially smaller, suggesting that most of the FMB differences at the 50% performance level may reflect a SNR dependence of the FMB. Still, 1-5 dB of the FMB difference between listener groups remained, indicating a possible role for reduced audibility, limited spectral or temporal resolution, or an inability to use auditory source-segregation cues, in directly limiting the ability to listen in the dips of a fluctuating masker. A modified version of the extended speech-intelligibility index that predicts a larger FMB at less favorable SNRs accounted for most of the FMB differences between listener groups and modalities. Overall, these data suggest that HI listeners retain more of an ability to listen in the dips of a fluctuating masker than previously thought. Instead, the fluctuating-masker difficulties exhibited by HI listeners may derive from the reduced FMB associated with the more favorable SNRs they require to identify a reasonable proportion of the target speech.

  10. [The algorithm for the medical maintenance of the aircraft personnel suffering from chronic sensorineural impairment of hearing].

    PubMed

    Pankova, V B; Skryabina, L Yu; Barkhatova, O A

    2016-01-01

    The present study was designed to systematize the causes underlying the development of chronic sensorineural impairment of hearing in the aircraft personnel engaged in commercial aviation of the Russian Federation. A detailed clinical and audiological picture of chronic sensorineural loss of hearing in the aircraft personnel is presented with special reference to the criteria accepted in the civil aviationfor the evaluation of professional suitability and occupational selection in terms of hearing conditions. The study has demonstrated the paramount importance of the aviation medical expertise for the flight safety control in civil aviation. We analyzed the results of the audiological examination of the aircraft personnel suffering from chronic sensorineural impairment of hearing and proposed the algorithm for the rehabilitation of such subjects taking into consideration the stage of the chronic process.

  11. The genetic bases for syndromic and nonsyndromic deafness among Jews.

    PubMed

    Ben-Yosef, Tamar; Friedman, Thomas B

    2003-11-01

    There are hundreds of different mutated genes associated with hearing loss. However, recent findings indicate that a large proportion of both syndromic and nonsyndromic forms of deafness in some Jewish populations is caused by a small number of founder mutations. This review is focused on genetic disorders such as nonsyndromic deafness, Usher syndrome and Alport syndrome, in which hearing loss is a major part of the phenotype and in which the underlying prevalent founder mutations have been recently identified in different Jewish populations. These and other examples of common mutations within a distinct population allow for sensitive and specific use of genetic testing for carrier screening and diagnosis, and are an impetus for development of therapeutic strategies.

  12. High prevalence of vaterite in sagittal otoliths causes hearing impairment in farmed fish

    PubMed Central

    Reimer, T.; Dempster, T.; Warren-Myers, F.; Jensen, A. J.; Swearer, S. E.

    2016-01-01

    The rapid growth of aquaculture raises questions about the welfare status of mass-produced species. Sagittal otoliths are primary hearing structures in the inner ear of all teleost (bony) fishes and are normally composed of aragonite, though abnormal vaterite replacement is sometimes seen in the wild. We provide the first widespread evaluation of the prevalence of vaterite in otoliths, showing that farmed fish have levels of vaterite replacement over 10 times higher than wild fish, regardless of species. We confirm this observation with extensive sampling of wild and farmed Atlantic salmon in Norway, the world’s largest producer, and verify that vateritic otoliths are common in farmed salmon worldwide. Using a mechanistic model of otolith oscillation in response to sound, we demonstrate that average levels of vaterite replacement result in a 28–50% loss of otolith functionality across most of a salmonid’s known hearing range and throughout its life cycle. The underlying cause(s) of vaterite formation remain unknown, but the prevalence of hearing impairment in farmed fish has important implications for animal welfare, the survival of escapees and their effects on wild populations, and the efficacy of restocking programs based on captive-bred fish. PMID:27121086

  13. Unraveling of Enigmatic Hearing-Impaired GJB2 Single Heterozygotes by Massive Parallel Sequencing: DFNB1 or Not?

    PubMed

    Kim, So Young; Kim, Ah Reum; Kim, Nayoung K D; Lee, Chung; Kim, Min Young; Jeon, Eun-Hee; Park, Woong-Yang; Choi, Byung Yoon

    2016-04-01

    The molecular etiology of nonsyndromic sensorineural hearing loss (SNHL) in subjects with only one detectable autosomal recessive GJB2 mutation is unclear. Here, we report GJB2 single heterozygotes with various final genetic diagnoses and suggest appropriate diagnostic strategies. A total of 160 subjects with SNHL without phenotypic markers were screened for GJB2 mutations. Single-nucleotide variants or structural variations within the DFNB1 locus or in other deafness genes were examined by Sanger sequencing, breakpoint PCR, and targeted exome sequencing (TES) of 129 deafness genes. We identified 27 subjects with two mutations and 10 subjects with only one detectable mutation in GJB2. The detection rate of the single GJB2 mutation among the 160 SNHL subjects in the present study (6.25%) was higher than 2.58% in normal hearing controls in Korean. The DFNB1 was clearly excluded as a molecular etiology in four (40%) subjects: other recessive deafness genes (N = 3) accounted for SNHL and the causative gene for the other non-DFNB1 subject (N = 1) was not identified. The etiology of additional two subjects was potentially explained by digenic etiology (N = 2) of GJB2 with MITF and GJB3, respectively. The contribution of the single GJB2 mutation in the four remaining subjects is unclear. Comprehensive diagnostic testing including TES is prerequisite for understanding GJB2 single heterozygotes.

  14. Unraveling of Enigmatic Hearing-Impaired GJB2 Single Heterozygotes by Massive Parallel Sequencing: DFNB1 or Not?

    PubMed Central

    Kim, So Young; Kim, Ah Reum; Kim, Nayoung K. D.; Lee, Chung; Kim, Min Young; Jeon, Eun-Hee; Park, Woong-Yang; Choi, Byung Yoon

    2016-01-01

    Abstract The molecular etiology of nonsyndromic sensorineural hearing loss (SNHL) in subjects with only one detectable autosomal recessive GJB2 mutation is unclear. Here, we report GJB2 single heterozygotes with various final genetic diagnoses and suggest appropriate diagnostic strategies. A total of 160 subjects with SNHL without phenotypic markers were screened for GJB2 mutations. Single-nucleotide variants or structural variations within the DFNB1 locus or in other deafness genes were examined by Sanger sequencing, breakpoint PCR, and targeted exome sequencing (TES) of 129 deafness genes. We identified 27 subjects with two mutations and 10 subjects with only one detectable mutation in GJB2. The detection rate of the single GJB2 mutation among the 160 SNHL subjects in the present study (6.25%) was higher than 2.58% in normal hearing controls in Korean. The DFNB1 was clearly excluded as a molecular etiology in four (40%) subjects: other recessive deafness genes (N = 3) accounted for SNHL and the causative gene for the other non-DFNB1 subject (N = 1) was not identified. The etiology of additional two subjects was potentially explained by digenic etiology (N = 2) of GJB2 with MITF and GJB3, respectively. The contribution of the single GJB2 mutation in the four remaining subjects is unclear. Comprehensive diagnostic testing including TES is prerequisite for understanding GJB2 single heterozygotes. PMID:27057829

  15. Effect of enhancement of spectral changes on speech intelligibility and clarity preferences for the hearing impaired.

    PubMed

    Chen, Jing; Baer, Thomas; Moore, Brian C J

    2012-04-01

    Most information in speech is carried in spectral changes over time, rather than in static spectral shape per se. A form of signal processing aimed at enhancing spectral changes over time was developed and evaluated using hearing-impaired listeners. The signal processing was based on the overlap-add method, and the degree and type of enhancement could be manipulated via four parameters. Two experiments were conducted to assess speech intelligibility and clarity preferences. Three sets of parameter values (one corresponding to a control condition), two types of masker (steady speech-spectrum noise and two-talker speech) and two signal-to-masker ratios (SMRs) were used for each masker type. Generally, the effects of the processing were small, although intelligibility was improved by about 8 percentage points relative to the control condition for one set of parameter values using the steady noise masker at -6 dB SMR. The processed signals were not preferred over those for the control condition, except for the steady noise masker at -6 dB SMR. Further work is needed to determine whether tailoring the processing to the characteristics of the individual hearing-impaired listener is beneficial.

  16. Auditory inspired machine learning techniques can improve speech intelligibility and quality for hearing-impaired listeners.

    PubMed

    Monaghan, Jessica J M; Goehring, Tobias; Yang, Xin; Bolner, Federico; Wang, Shangqiguo; Wright, Matthew C M; Bleeck, Stefan

    2017-03-01

    Machine-learning based approaches to speech enhancement have recently shown great promise for improving speech intelligibility for hearing-impaired listeners. Here, the performance of three machine-learning algorithms and one classical algorithm, Wiener filtering, was compared. Two algorithms based on neural networks were examined, one using a previously reported feature set and one using a feature set derived from an auditory model. The third machine-learning approach was a dictionary-based sparse-coding algorithm. Speech intelligibility and quality scores were obtained for participants with mild-to-moderate hearing impairments listening to sentences in speech-shaped noise and multi-talker babble following processing with the algorithms. Intelligibility and quality scores were significantly improved by each of the three machine-learning approaches, but not by the classical approach. The largest improvements for both speech intelligibility and quality were found by implementing a neural network using the feature set based on auditory modeling. Furthermore, neural network based techniques appeared more promising than dictionary-based, sparse coding in terms of performance and ease of implementation.

  17. Sentence Recognition Prediction for Hearing-impaired Listeners in Stationary and Fluctuation Noise With FADE

    PubMed Central

    Schädler, Marc René; Warzybok, Anna; Meyer, Bernd T.; Brand, Thomas

    2016-01-01

    To characterize the individual patient’s hearing impairment as obtained with the matrix sentence recognition test, a simulation Framework for Auditory Discrimination Experiments (FADE) is extended here using the Attenuation and Distortion (A+D) approach by Plomp as a blueprint for setting the individual processing parameters. FADE has been shown to predict the outcome of both speech recognition tests and psychoacoustic experiments based on simulations using an automatic speech recognition system requiring only few assumptions. It builds on the closed-set matrix sentence recognition test which is advantageous for testing individual speech recognition in a way comparable across languages. Individual predictions of speech recognition thresholds in stationary and in fluctuating noise were derived using the audiogram and an estimate of the internal level uncertainty for modeling the individual Plomp curves fitted to the data with the Attenuation (A-) and Distortion (D-) parameters of the Plomp approach. The “typical” audiogram shapes from Bisgaard et al with or without a “typical” level uncertainty and the individual data were used for individual predictions. As a result, the individualization of the level uncertainty was found to be more important than the exact shape of the individual audiogram to accurately model the outcome of the German Matrix test in stationary or fluctuating noise for listeners with hearing impairment. The prediction accuracy of the individualized approach also outperforms the (modified) Speech Intelligibility Index approach which is based on the individual threshold data only. PMID:27604782

  18. Empowering the family during the first months after identification of permanent hearing impairment in children.

    PubMed

    Ciciriello, E; Bolzonello, P; Marchi, R; Falzone, C; Muzzi, E; Orzan, E

    2016-02-01

    The latest international guidelines highlight the importance of involving the family in the diagnostic and rehabilitation process of children affected by permanent hearing impairment. This emphasises how meaningful this approach is for the development of the deaf child. So far, there is very little evidence about this approach in Italy, and there are still some barriers to its practical management. The aim of this paper is to report the results of a strategic analysis, which identifies the strengths, weaknesses, opportunities and threats of the family empowerment process during early auditory diagnosis and rehabilitation. The audiology programme should have the goal to offer information and support to families in order to achieve a conscious decision about the use and type of auditory prosthesis and rehabilitation choice within three months after audiologic diagnosis. Within the framework of the Ministry of Health project CCM 2013 "Preventing Communication Disorders: a Regional Program for Early Identification, Intervention and Care of Hearing Impaired Children", a group of professionals identified three main recommendations that can be useful to foster the natural communicative development of the child by strengthening the therapeutic alliance and empowerment of the family. The recommendations obtained with this analysis can help to develop new Italian guidelines with the aim to foster natural communicative development of the child by strengthening the therapeutic alliance and empowerment of the family.

  19. Genome-wide association analysis demonstrates the highly polygenic character of age-related hearing impairment

    PubMed Central

    Fransen, Erik; Bonneux, Sarah; Corneveaux, Jason J; Schrauwen, Isabelle; Di Berardino, Federica; White, Cory H; Ohmen, Jeffrey D; Van de Heyning, Paul; Ambrosetti, Umberto; Huentelman, Matthew J; Van Camp, Guy; Friedman, Rick A

    2015-01-01

    We performed a genome-wide association study (GWAS) to identify the genes responsible for age-related hearing impairment (ARHI), the most common form of hearing impairment in the elderly. Analysis of common variants, with and without adjustment for stratification and environmental covariates, rare variants and interactions, as well as gene-set enrichment analysis, showed no variants with genome-wide significance. No evidence for replication of any previously reported genes was found. A study of the genetic architecture indicates for the first time that ARHI is highly polygenic in nature, with probably no major genes involved. The phenotype depends on the aggregated effect of a large number of SNPs, of which the individual effects are undetectable in a modestly powered GWAS. We estimated that 22% of the variance in our data set can be explained by the collective effect of all genotyped SNPs. A score analysis showed a modest enrichment in causative SNPs among the SNPs with a P-value below 0.01. PMID:24939585

  20. Hearing impairment in patients with rheumatoid arthritis: association with anti-citrullinated protein antibodies.

    PubMed

    Lobo, Fabrício Silva; Dossi, Mario Orlando; Batista, Lígia; Shinzato, Márcia Midori

    2016-09-01

    It has been suggested that hearing impairment (HI) is one of the extra-articular features of rheumatoid arthritis (RA). Nevertheless, the prevalence and nature of HI in RA is still uncertain. The objectives were to study hearing function in patients with RA using audiometric tests and to examine whether HI correlates with autoantibodies. Hearing functions were investigated in 43 consecutive RA patients and 23 control subjects (less than 60 years old). Their sera were evaluated for the presence of rheumatoid factor (RF), anti-cyclic citrullinated peptide (anti-CCP), and anti-mutated citrullinated vimentin (anti-MCV) antibodies. HI was observed in 46.5 % of RA patients and in 30.4 % of control subjects, p = 0.32. HI was characterized as sensorineural in 80 and 85.7 % of RA patients and control subjects with HI, respectively, p = 1.00. RA patients had a worse hearing threshold for air conduction at 6 kHz in the right ear (p = 0.019) and had a decreased amplitude of otoacoustic emissions (OAEs) at 2 kHz bilaterally (p = 0.04) compared with control subjects. In the RA group, patients with and without HI were 80 and 34.78 % anti-CCP positive, respectively, p = 0.008. RA patients with and without HI were 85 and 43.48 % anti-MCV positive, respectively, p = 0.013. HI in RA patients was mainly sensorineural and was associated with anti-CCP and anti-MCV antibodies.

  1. Temporal Masking Contributions of Inherent Envelope Fluctuations for Listeners with Normal and Impaired Hearing

    NASA Astrophysics Data System (ADS)

    Svec, Adam

    Gaussian noise (GN) simultaneous maskers yield higher masked thresholds for pure tones than low-fluctuation noise (LFN) simultaneous maskers for listeners with normal hearing. This increased residual masking is thought to be due to inherent fluctuations in the temporal envelope of Gaussian noise, but these masking effects using forward maskers have been previously unexamined. Because differences in forward masking due to age and hearing loss are known, the first study measured forward-masked detection thresholds for younger and older adults with normal hearing (NH) and older adults with hearing loss (HI) for a 4000 Hz pure-tone probe at a single masker-probe delay in narrowband noises with maximal (GN) or minimal (LFN) inherent envelope fluctuations. As predicted, results suggested that no effect of age was observed. Surprisingly, forward-masked threshold differences between GN and LFN, an estimate of the magnitude of the effect of inherent masker envelope fluctuations, were not significantly different for older HI listeners compared to younger or older NH listeners. Due to the surprising similarities between listeners with normal and impaired hearing, the second study was designed to assess effects of hearing loss on the slopes and magnitudes of recovery from forward maskers that varied in inherent envelope fluctuations for masker-probe delays of 25, 50, and 75 ms. In addition to measuring these effects centered at 4000 Hz, forward-masked thresholds were also measured at 2000 Hz, a region of better hearing for the HI listeners. As hypothesized, regardless of masker fluctuations, slopes of recovery from forward masking were shallower for HI than NH listeners in all conditions. At 4000 Hz, additional residual masking was greater in HI than NH listeners at the longest masker-probe delays; whereas, no differences in additional residual masking between HI and NH listeners were observed for 2000 Hz. These results suggest that the masking effects from inherent envelope

  2. Detection of frequency modulation by hearing-impaired listeners: Effects of carrier frequency, modulation rate, and added amplitude modulation

    NASA Astrophysics Data System (ADS)

    Moore, Brian C. J.; Skrodzka, Ewa

    2002-01-01

    It has been proposed that the detection of frequency modulation (FM) of sinusoidal carriers can be mediated by two mechanisms: a place mechanism based on FM-induced amplitude modulation (AM) in the excitation pattern, and a temporal mechanism based on phase-locking in the auditory nerve. The temporal mechanism appears to be ``sluggish'' and does not play a role for FM rates above about 10 Hz. It also does not play a role for high carrier frequencies (above about 5 kHz). This experiment examined FM detection in three young subjects with normal hearing and four elderly subjects with cochlear hearing loss. Carrier frequencies were 0.25, 0.5, 1, 2, 4, and 6 kHz and modulation rates were 2, 5, 10, and 20 Hz. FM detection thresholds were measured both in the absence of AM, and with AM of a fixed depth (m=0.33) added in both intervals of a forced-choice trial. The added AM was intended to disrupt cues based on FM-induced AM in the excitation pattern. Generally, the hearing-impaired subjects performed markedly more poorly than the normal-hearing subjects. For the normal-hearing subjects, the disruptive effect of the AM tended to increase with increasing modulation rate, for carrier frequencies below 6 kHz, as found previously by Moore and Sek [J. Acoust. Soc. Am. 100, 2320-2331 (1996)]. For the hearing-impaired subjects, the disruptive effective of the AM was generally larger than for the normal-hearing subjects, and the magnitude of the disruption did not consistently increase with increasing modulation rate. The results suggest that cochlear hearing impairment adversely affects both temporal and excitation pattern mechanisms of FM detection.

  3. Consensus Development Conference on Early Identification of Hearing Impairment in Infants and Young Children (Bethesda, Maryland, March 1-3, 1993).

    ERIC Educational Resources Information Center

    National Inst. of Child Health and Human Development (NIH), Bethesda, MD.

    This document compiles abstracts of papers that were presented at a 3-day conference of experts which developed a consensus statement on early identification of hearing impairment in infants and young children. Papers addressed taxonomy; epidemiology; developmental consequences of early hearing impairment; methodology, instrumentation, and…

  4. Sleep Apnea Is Associated with Hearing Impairment: The Hispanic Community Health Study/Study of Latinos

    PubMed Central

    Chopra, Amit; Jung, Molly; Kaplan, Robert C.; Appel, David W.; Dinces, Elizabeth A.; Dhar, Sumitrajit; Zee, Phyllis C.; Gonzalez, Franklyn; Lee, David J.; Ramos, Alberto R.; Hoffman, Howard J.; Redline, Susan; Cruickshanks, Karen J.; Shah, Neomi A.

    2016-01-01

    Study Objective: Sleep apnea (SA) may promote hearing impairment (HI) through ischemia and inflammation of the cochlea. Our objective was to assess an independent association between SA and HI in the Hispanic Community Health Study/Study of Latinos (HCHS/SOL) participants. Methods: We used data from the HCHS/SOL, a multicenter population-based study of self- identifying Hispanic/Latinos 18- to 74-y-old adults from four US urban communities. We performed home SA testing and in-clinic audiometry testing in all participants. SA was defined as an apnea-hypopnea index (AHI) ≥ 15 events/h. HI was defined as a mean hearing threshold > 25 dB hearing level in either ear at the frequencies: 3,000 to 8,000 Hz for high-frequency HI (HF-HI) and 500 to 2,000 Hz for low-frequency HI (LF-HI). Combined-frequency HI (CF-HI) was defined as both conditions present, and Any-HI was considered as HI in either low or high frequencies. Results: Of 13,967 participants, 9.9% had SA and 32.3% had Any-HI. Adjusted for risk factors for HI, those with SA had a 30% higher odds of Any-HI (95% confidence interval [CI] = 8% to 57%), 26% higher odds of HF-HI (CI = 3% to 55%), 127% higher odds of LF-HI (CI = 21% to 326%), and 29% higher odds of CF-HI (CI = 0% to 65%). A dose-response association was observed between AHI severity and Any-HI (versus no SA, OR for AHI ≥ 15 and < 30 = 1.22, CI = 0.96 to 1.54, and OR for AHI ≥ 30 = 1.46, CI = 1.11 to 1.91, p = 0.002). Conclusion: SA is associated with HF-HI and LF-HI, independent of snoring and other confounders. Commentary: A commentary on this article appears in this issue on page 641. Citation: Chopra A, Jung M, Kaplan RC, Appel DW, Dinces EA, Dhar S, Zee PC, Gonzalez F, Lee DJ, Ramos AR, Hoffman HJ, Redline S, Cruickshanks KJ, Shah NA. Sleep apnea is associated with hearing impairment: the hispanic community health study/study of latinos. J Clin Sleep Med 2016;12(5):719–726. PMID:26951413

  5. Lexical Influences on Spoken Spondaic Word Recognition in Hearing-Impaired Patients

    PubMed Central

    Moulin, Annie; Richard, Céline

    2015-01-01

    Top-down contextual influences play a major part in speech understanding, especially in hearing-impaired patients with deteriorated auditory input. Those influences are most obvious in difficult listening situations, such as listening to sentences in noise but can also be observed at the word level under more favorable conditions, as in one of the most commonly used tasks in audiology, i.e., repeating isolated words in silence. This study aimed to explore the role of top-down contextual influences and their dependence on lexical factors and patient-specific factors using standard clinical linguistic material. Spondaic word perception was tested in 160 hearing-impaired patients aged 23–88 years with a four-frequency average pure-tone threshold ranging from 21 to 88 dB HL. Sixty spondaic words were randomly presented at a level adjusted to correspond to a speech perception score ranging between 40 and 70% of the performance intensity function obtained using monosyllabic words. Phoneme and whole-word recognition scores were used to calculate two context-influence indices (the j factor and the ratio of word scores to phonemic scores) and were correlated with linguistic factors, such as the phonological neighborhood density and several indices of word occurrence frequencies. Contextual influence was greater for spondaic words than in similar studies using monosyllabic words, with an overall j factor of 2.07 (SD = 0.5). For both indices, context use decreased with increasing hearing loss once the average hearing loss exceeded 55 dB HL. In right-handed patients, significantly greater context influence was observed for words presented in the right ears than for words presented in the left, especially in patients with many years of education. The correlations between raw word scores (and context influence indices) and word occurrence frequencies showed a significant age-dependent effect, with a stronger correlation between perception scores and word occurrence frequencies

  6. Identification of a second HOXA2 nonsense mutation in a family with autosomal dominant non-syndromic microtia and distinctive ear morphology.

    PubMed

    Piceci, F; Morlino, S; Castori, M; Buffone, E; De Luca, A; Grammatico, P; Guida, V

    2016-08-09

    Microtia is a congenital defect affecting external ears, which appear smaller and sometimes malformed. Here we describe a five-generation family with isolated bilateral microtia segregating as an autosomal dominant trait. Similar features have been previously observed in an autosomal dominant family with non-syndromic microtia and hearing loss segregating with a HOXA2 nonsense variant. HOXA2 biallelic mutations were also described in an inbreed family with autosomal recessive microtia, hearing impairment and incomplete cleft palate. In our family, sequence analysis detected a heterozygous protein truncating nonsense variant [c.670G>T, p.(Glu224*)] segregating in all affected individuals and absent in public databases. This study confirms the role of HOXA2 gene in dominant isolated microtia and contribute to further define the dysmorphogenetic effect of this gene on ear development.

  7. Oversight on Programs for the Deaf and Hearing Impaired, 1980. Hearing Before the Subcommittee on the Handicapped of the Committee on Labor and Human Resources, United States Senate, Ninety-Sixth Congress, Second Session on to Examine Current Problems and Programs of the Deaf and Hearing Impaired, and to Explore Future Technological Developments Designed to Handle Their Problems.

    ERIC Educational Resources Information Center

    Congress of the U.S., Washington, DC. Senate Committee on Labor and Human Resources.

    The 1980 Senate hearing focuses on federal programs for deaf and hearing impaired students. Thirteen prepared statements are presented from representatives of federal agencies (National Institute of Handicapped Research, Department of Education, and National Institute of Health); private associations (the Convention of American Instructors of the…

  8. Hearing

    ERIC Educational Resources Information Center

    Koehlinger, Keegan M.; Van Horne, Amanda J. Owen; Moeller, Mary Pat

    2013-01-01

    Purpose: Spoken language skills of 3- and 6-year-old children who are hard of hearing (HH) were compared with those of children with normal hearing (NH). Method: Language skills were measured via mean length of utterance in words (MLUw) and percent correct use of finite verb morphology in obligatory contexts based on spontaneous conversational…

  9. Qualitative analysis of dermatoglyphics of the digito-palmar complex in children with severe recessive perceptively impaired hearing.

    PubMed

    Sikić, Nada; Milicić, Jasna; Vrca, Andelko; Dulcić, Adinda; Runjić, Nada

    2009-06-01

    The possible hereditary indicated differences in the ascending family tree was investigated in children with severe recessive perceptively impaired hearing, their parents, and healthy controls, using qualitative analysis of dermatoglyphics of the digito-palmar complex. The chi2 test was performed and biological distance was investigated by means according to Hiernaux Ag methods, and presented using Ward's method for the examined groups. The results show that the healthy control group differs from the groups of boys and girls with impaired hearing and also from their fathers mostly in palmar variables. The mothers were biologically more distant from the examined groups of patients, and more similar to the control group of randomly selected healthy female controls. The results indicate polygenic inheritance of sporadic sensoneurial hearing loss.

  10. Separating Contributions of Hearing, Lexical Knowledge, and Speech Production to Speech-Perception Scores in Children with Hearing Impairments.

    ERIC Educational Resources Information Center

    Paatsch, Louise E.; Blamey, Peter J.; Sarant, Julia Z.; Martin, Lois F.A.; Bow, Catherine P.

    2004-01-01

    Open-set word and sentence speech-perception test scores are commonly used as a measure of hearing abilities in children and adults using cochlear implants and/or hearing aids. These tests ore usually presented auditorily with a verbal response. In the case of children, scores are typically lower and more variable than for adults with hearing…

  11. Hearing impairment in the P23H-1 retinal degeneration rat model

    PubMed Central

    Sotoca, Jorge V.; Alvarado, Juan C.; Fuentes-Santamaría, Verónica; Martinez-Galan, Juan R.; Caminos, Elena

    2014-01-01

    The transgenic P23H line 1 (P23H-1) rat expresses a variant of rhodopsin with a mutation that leads to loss of visual function. This rat strain is an experimental model usually employed to study photoreceptor degeneration. Although the mutated protein should not interfere with other sensory functions, observing severe loss of auditory reflexes in response to natural sounds led us to study auditory brain response (ABR) recording. Animals were separated into different hearing levels following the response to natural stimuli (hand clapping and kissing sounds). Of all the analyzed animals, 25.9% presented auditory loss before 50 days of age (P50) and 45% were totally deaf by P200. ABR recordings showed that all the rats had a higher hearing threshold than the control Sprague-Dawley (SD) rats, which was also higher than any other rat strains. The integrity of the central and peripheral auditory pathway was analyzed by histology and immunocytochemistry. In the cochlear nucleus (CN), statistical differences were found between SD and P23H-1 rats in VGluT1 distribution, but none were found when labeling all the CN synapses with anti-Syntaxin. This finding suggests anatomical and/or molecular abnormalities in the auditory downstream pathway. The inner ear of the hypoacusic P23H-1 rats showed several anatomical defects, including loss and disruption of hair cells and spiral ganglion neurons. All these results can explain, at least in part, how hearing impairment can occur in a high percentage of P23H-1 rats. P23H-1 rats may be considered an experimental model with visual and auditory dysfunctions in future research. PMID:25278831

  12. The Effectiveness and Usability of the Educational Software on Concept Education for Young Children with Impaired Hearing

    ERIC Educational Resources Information Center

    Goker, Hanife; Ozaydin, Latife; Tekedere, Hakan

    2016-01-01

    Early intervention and early education have a special place in educating the children with Impaired Hearing (IH). The advancements in information and communication technologies have led to adopting the view that such technologies could be applied in the educational process of the children with IH. Besides, the positive results acquired in the…

  13. Validating the Thinking Styles Inventory-Revised II among Chinese University Students with Hearing Impairment through Test Accommodations

    ERIC Educational Resources Information Center

    Cheng, Sanyin; Zhang, Li-Fang

    2014-01-01

    The present study pioneered in adopting test accommodations to validate the Thinking Styles Inventory-Revised II (TSI-R2; Sternberg, Wagner, & Zhang, 2007) among Chinese university students with hearing impairment. A series of three studies were conducted that drew their samples from the same two universities, in which accommodating test…

  14. The language of gestures. searching for effective communication between the dentist and the hearing-impaired patient.

    PubMed

    Wieczkowska, Ilona; Lisiecka, Krystyna

    2006-01-01

    The aim of this article is to draw attention to the sign language as an alternative form of communication to oral language. The need to discuss this topic stems from contacts of the practicing dentist with hearing-impaired patients with whom communication should be more effective.

  15. Directory of Services for the Multiply Handicapped Deaf and/or Hearing Impaired. Resources for the Rubella Deaf Child.

    ERIC Educational Resources Information Center

    Gallaudet Coll., Washington, DC.

    The directory contains information on centers, facilities, and schools which provide some services or programs suitable to the needs of the deaf or hearing impaired who have additonal handicaps (adults as well as children). A brief description of the facility, the clients served, and the services offered accompanies the listing of each facility's…

  16. Philosophic Considerations in Mainstreaming the Hearing Impaired in Occupational Education Programs: An Empirically Based Discussion of Goals and Objectives.

    ERIC Educational Resources Information Center

    Miller, John K.; And Others

    Described is a study of the philosophical basis of mainstreamed occupational center programs for hearing impaired secondary students in which 307 educators, parents of handicapped students, and handicapped students were interviewed. Among findings discussed for three issues (the general goals of education, the goals of occupational education, and…

  17. A Comparison Study of Gross Motor Development Skills of Normal, Hearing-Impaired and Down Syndrome Children.

    ERIC Educational Resources Information Center

    Bilir, Sule; And Others

    This study, conducted in Ankara, Turkey, compared motor development in 48 normal children (ages 3 to 6), 12 children (ages 5 to 7) with Down syndrome, and 33 children (ages 3 to 7) with hearing impairments. The Motor Development Section of the Portage Early Childhood Educational Program checklist was administered to all the children. Results…

  18. The Word Decoding Strategies of Hebrew Readers with and without Hearing Impairments: Some Insight from an Associative Learning Task

    ERIC Educational Resources Information Center

    Miller, Paul

    2004-01-01

    The aim of this study was to elucidate the word decoding strategies of Hebrew readers with pre-lingually-acquired, severe hearing impairments as opposed to regular Hebrew readers, and to determine their efficiency. The research paradigm used to clarify these issues asked participants to perform a sequence of three learning tasks, calling for the…

  19. Development of an Exercise Program to Improve the Static and Dynamic Balance of Profoundly Hearing-Impaired Children.

    ERIC Educational Resources Information Center

    Lewis, Sandra; And Others

    1985-01-01

    Sixteen hearing-impaired children, aged 6 to 10 years, participated in a six-week posture and body awareness activity program. Pre- and posttests showed that the balance of the experimental group Ss improved, whereas the balance of the control group Ss did not improve. (Author/CL)

  20. The Colorado Individual Performance Profile for Hearing-Impaired Students: A Data-Driven Approach to Decision Making.

    ERIC Educational Resources Information Center

    Yoshinaga-Itano, Christine; Ruberry, Joan

    1992-01-01

    The Colorado Individual Performance Profile for Hearing-Impaired Students is presented as a tool for providing objective criteria for determining appropriate levels of educational services. The profile rates each student's audiological acuity, communication, English language skills, social-emotional development, life skills, cognition, and other…

  1. When a Story Is Not a Story: A Process Analysis of the Written Language of Hearing-Impaired Children.

    ERIC Educational Resources Information Center

    Yoshinaga-Itano, Christine; Downey, Doris M.

    1992-01-01

    The Colorado Process Analysis of the Written Language of Hearing-Impaired Children assesses the semantic characteristics of expressive written language narratives prepared by beginning writers. The validity of the instrument was examined with 284 children (ages 7-21), indicating that the model does identify characteristics that are critical to the…

  2. The Use of Microcomputer Technology in Assessing and Training Communication Skills of Young Hearing-Impaired Children.

    ERIC Educational Resources Information Center

    Fulton, Robert T.; And Others

    1983-01-01

    An interactive computer controlled audiovisual system has been used to investigate the acquisition of simple reading tasks and the assessment of bimodal (auditory-speech reading) interactions/interferences with severely hearing impaired preschoolers. It is noted that the system's capabilities exceed the parameters of current investigations.…

  3. Preventing Sexual Abuse of Persons with Disabilities: A Curriculum for Hearing Impaired, Physically Disabled, Blind and Mentally Retarded Students.

    ERIC Educational Resources Information Center

    O'Day, Bonnie; And Others

    The curriculum on sexual abuse is intended for professionals working with hearing impaired, physically disabled, blind, and mentally retarded students. Introductory material addresses the vulnerability of disabled adolescents to sexual abuse; presents background information on such topics as victims, offenders, reporting abuse, and Minnesota laws…

  4. The Role of Music in Speech Intelligibility of Learners with Post Lingual Hearing Impairment in Selected Units in Lusaka District

    ERIC Educational Resources Information Center

    Katongo, Emily Mwamba; Ndhlovu, Daniel

    2015-01-01

    This study sought to establish the role of music in speech intelligibility of learners with Post Lingual Hearing Impairment (PLHI) and strategies teachers used to enhance speech intelligibility in learners with PLHI in selected special units for the deaf in Lusaka district. The study used a descriptive research design. Qualitative and quantitative…

  5. Designing Instructional Formats: The Effects of Verbal and Pictorial Components on Hearing-Impaired Students' Comprehension of Science Concepts.

    ERIC Educational Resources Information Center

    Diebold, Thomas J.; Waldron, Manjula B.

    1988-01-01

    Sixty hearing-impaired students, aged 12-22, studied one of four instructional formats describing basic science concepts. Comprehension gains were measured using a pretest-posttest procedure. Formats which featured highly pictorial content and simplified English produced significantly higher mean gain scores than formats with less pictorial…

  6. Efficacy of Information and Communication Technology in Enhancing Learning Outcomes of Students with Hearing Impairment in Ibadan

    ERIC Educational Resources Information Center

    Egaga, Patrick I.; Aderibigbe, S. Akinwumi

    2015-01-01

    The study aimed at examining the efficacy of Information and Communication Technology (ICT) in enhancing learning outcomes of students with hearing impairment in Ibadan. The study adopted a pretest, post-test, control group quasi-experimental research design. Purposive sampling techniques was used for the selection of thirty participants…

  7. An Approach to Teach Science to Students with Limited Language Proficiency: In the Case of Students with Hearing Impairment

    ERIC Educational Resources Information Center

    Im, Sungmin; Kim, Ok-Ja

    2014-01-01

    For over two decades, there has been increasing concern regarding the science learning of disadvantaged students such as indigenous or disabled students. The academic achievement of students with hearing impairment has been seen as relatively low. This low achievement has been mainly due to students' poor literal ability and not because of…

  8. Guidelines for the Administration of Educational Programs for Students Who Are Deaf/Hard of Hearing, Visually Impaired, or Deafblind

    ERIC Educational Resources Information Center

    Bruce, Susan; Ferrell, Kay; Luckner, John L.

    2016-01-01

    This paper presents the essential programming components resulting from a systematic review of research studies, legislation, and policy documents on the topic of administration issues in educational programming for students who are deaf/hard of hearing, visually impaired, or deafblind. It is recommended that educational teams should include a…

  9. General and Specific Characteristics of a University-School Partnership: Promoting Learning Opportunities for Students with Deafness or Hearing Impairments

    ERIC Educational Resources Information Center

    Kim, Dong-Joong; Park, Yong Joon; Cho, Jeong-IL; Kim, Daesang

    2013-01-01

    The purpose of this article is to identify six key features involved in the development and enhancement of a university-school partnership and to share insights on how these features can be used to promote richer experiential learning opportunities of both university students and young children with deafness or hearing impairments, eventually…

  10. Enhancing Literacy Skills of Students with Congenital and Profound Hearing Impairment in Nigeria Using Babudoh's Comprehension Therapy

    ERIC Educational Resources Information Center

    Babudoh, Gladys B.

    2014-01-01

    This study reports the effect of a treatment tool called "Babudoh's comprehension therapy" in enhancing the comprehension and writing skills of 10 junior secondary school students with congenital and profound hearing impairment in Plateau State, Nigeria. The study adopted the single group pretest-posttest quasi-experimental research…

  11. A Model for Teaching Parents of Young Hearing-Impaired Children Total Communication in the Home--Project RITCH.

    ERIC Educational Resources Information Center

    Watkins, Susan; Clark, Thomas C.

    1988-01-01

    Project RITCH (Research In Total Communication in the Home) developed a model videotape program to teach total communication to families of young hearing-impaired children in their homes. Discussed are the process of designing the videotape prototype, field testing, characteristics of a model home program, and videotape distribution through local…

  12. Factor Structure and Reliability of the Dutch Version of Seven Scales of the Communication Profile for the Hearing Impaired (CPHI)

    ERIC Educational Resources Information Center

    Mokkink, Lidwine B.; Knol, Dirk L.; Zekveld, Adriana A.; Goverts, S. Theo; Kramer, Sophia E.

    2009-01-01

    Purpose: Seven scales of the Communication Profile for the Hearing Impaired (CPHI; M. E. Demorest & S. A. Erdman, 1987) were translated into Dutch: Maladaptive Behavior, Verbal Strategies, and Nonverbal Strategies (within the area of Communication Strategies) and Self-Acceptance, Acceptance of Loss, Stress, and Withdrawal (within the area of…

  13. A National Survey of Current and Anticipated Media Equipment in Residential and Day Programs for the Hearing Impaired.

    ERIC Educational Resources Information Center

    Grant, William D.; Clark, Norma

    Five investigations sponsored by the Office of Research and Evaluation (ORE) of the Model Secondary School for the Deaf (MSSD) are reported. Presented first are results of a national survey (April 1974) of media equipment in 123 residential and day programs for the hearing impaired, in which the number of cassette video recorders and color video…

  14. 14 CFR 382.119 - What information must carriers give individuals with vision or hearing impairment on aircraft?

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 14 Aeronautics and Space 4 2010-01-01 2010-01-01 false What information must carriers give individuals with vision or hearing impairment on aircraft? 382.119 Section 382.119 Aeronautics and Space... NONDISCRIMINATION ON THE BASIS OF DISABILITY IN AIR TRAVEL Services on Aircraft § 382.119 What information...

  15. 14 CFR 382.53 - What information must carriers give individuals with a vision or hearing impairment at airports?

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 14 Aeronautics and Space 4 2010-01-01 2010-01-01 false What information must carriers give individuals with a vision or hearing impairment at airports? 382.53 Section 382.53 Aeronautics and Space... NONDISCRIMINATION ON THE BASIS OF DISABILITY IN AIR TRAVEL Accessibility of Airport Facilities § 382.53...

  16. Symptoms of Autism in Males with Fragile X Syndrome: A Comparison to Nonsyndromic ASD using Current ADI-R Scores

    PubMed Central

    McDuffie, Andrea; Thurman, Angela John; Hagerman, Randi J.; Abbeduto, Leonard

    2014-01-01

    Symptoms of autism are frequent in males with fragile X syndrome (FXS), but it is not clear whether symptom profiles differ from those of nonsyndromic ASD. Using individual item scores from the Autism Diagnostic Inventory-Revised (ADI-R), we examined which current symptoms of autism differed in boys with FXS relative to same-aged boys diagnosed with nonsyndromic ASD. In addition, different subsamples of participants were matched on autism diagnostic status and severity of autism symptoms. Between-group comparisons revealed that boys with FXS showed significantly less impairment in Social Smiling than did age-, diagnostic, and severity-matched boys with nonsyndromic ASD. Severity-matched boys with FXS showed more impairment in Complex Mannerisms than did boys with nonsyndromic ASD. Behavioral differences between FXS and nonsyndromic ASD may be of theoretical importance in understanding the causes and correlates of ASD in FXS and in developing and implementing appropriate treatments. PMID:24414079

  17. Prevalence and audiological profiles of GJB2 mutations in a large collective of hearing impaired patients.

    PubMed

    Burke, W F; Warnecke, A; Schöner-Heinisch, A; Lesinski-Schiedat, A; Maier, H; Lenarz, T

    2016-03-01

    Mutations in the GJB2 gene are known to represent the commonest cause of hereditary and congenital hearing loss. In this study, a complete sequencing of the GJB2 gene in a cohort of 506 patients from a single, large cochlear implant program in Europe was performed. Audiological testing for those patients who could actively participate was performed using pure tone audiometry (PTA). Those unable to undergo PTA were measured using click-auditory brainstem response (ABR). Data analysis was performed to determine genotype-phenotype correlations of the mutational status vs. audiological profiles and vs. age at the time of presentation. An overall prevalence of biallelic mutations of 13.4% was found for the total collective. When subsets of younger patients were examined, the prevalence increased to 27% of those up to age 18 and 35% of those up to age 5 at the time of testing, respectively. This increase was found to be highly significant (p < 0.001). Analysis of the mean PTA thresholds revealed a strong correlation between allele combination status and mean PTA (p = 0.021). The prevalence of simple heterozygotes was found to be approximately 10.1%, which is around 3.3 times the value expected in the general population. As GJB2 follows a recessive pattern of inheritance, the question arises as to why such a large fraction of simple heterozygotes was observed among the hearing impaired patients included in this study.

  18. Preparing for communication interactions: the value of anticipatory strategies for adults with hearing impairment.

    PubMed

    Tye-Murray, N

    1992-04-01

    Some people with hearing impairment may use anticipatory strategies to prepare for an upcoming communication interaction, such as a doctor's appointment. They may consider vocabulary and statements that might occur, and they may practice speechreading a partner saying the items. Experiment 1 evaluated the effectiveness of two types of anticipatory strategies: workbook activities and situation-specific lipreading practice. Two groups of normal-hearing subjects were asked to prepare for a communication interaction in a bank setting where they would be required to recognize speech using only the visual signal. Each group was assigned to one type of anticipatory strategy. A third group served as a control group. Experiment 2 evaluated whether multifaceted anticipatory practice improved cochlear implant users' ability to recognize statements and words audiovisually that might occur in a doctor's office, bank, movie theater, and gas station. One group of implanted subjects received 4 days of training, 1 day for each setting, and a second group served as a control group. In both experiments, subjects who used anticipatory strategies did not improve their performance on situation-specific sentence tests more than the control subjects.

  19. The Effect of Peripheral Compression on Syllable Perception Measured with a Hearing Impairment Simulator.

    PubMed

    Matsui, Toshie; Irino, Toshio; Nagae, Misaki; Kawahara, Hideki; Patterson, Roy D

    2016-01-01

    Hearing impaired (HI) people often have difficulty understanding speech in multi-speaker or noisy environments. With HI listeners, however, it is often difficult to specify which stage, or stages, of auditory processing are responsible for the deficit. There might also be cognitive problems associated with age. In this paper, a HI simulator, based on the dynamic, compressive gammachirp (dcGC) filterbank, was used to measure the effect of a loss of compression on syllable recognition. The HI simulator can counteract the cochlear compression in normal hearing (NH) listeners and, thereby, isolate the deficit associated with a loss of compression in speech perception. Listeners were required to identify the second syllable in a three-syllable "nonsense word", and between trials, the relative level of the second syllable was varied, or the level of the entire sequence was varied. The difference between the Speech Reception Threshold (SRT) in these two conditions reveals the effect of compression on speech perception. The HI simulator adjusted a NH listener's compression to that of the "average 80-year old" with either normal compression or complete loss of compression. A reference condition was included where the HI simulator applied a simple 30-dB reduction in stimulus level. The results show that the loss of compression has its largest effect on recognition when the second syllable is attenuated relative to the first and third syllables. This is probably because the internal level of the second syllable is attenuated proportionately more when there is a loss of compression.

  20. Consonant enhancement effects on speech recognition of hearing-impaired children

    NASA Astrophysics Data System (ADS)

    Smith, Lynn Z.

    This dissertation examined the effects of consonant enhancement on speech recognition by hearing-impaired children. The goal of this research was to determine if there is an optimal consonant amplitude in relation to a neighboring vowel whereby consonant recognition is maximized. The differences in gain required (enhancement, in dB) to optimize the consonant vowel (CV) ratio in nonsense syllables was determined for stops and fricatives, both voiced and voiceless. Twelve children with congenital moderate to severe sensorineural hearing loss acted as subjects. One group of 6 children ranged in age from 5 to 6 years, a second group of 6 children were 8 to 9 years of age. The test stimuli consisted of VC nonsense syllables with various levels of enhancement. The enhancement levels ranged from 0 dB (for the unprocessed stimulus) to 30 dB of gain, in steps of 3 dB. The gain was applied to only the consonant portion of each nonsense syllable. The arc sine transformation was used to stabilize the error variance of the test scores. Curves were fitted to the transformed scores as a function of enhancement level. The maximum value of this curve identified the CV ratio at which consonant recognition was maximized for experimental condition and consonant type. The results showed that significant improvements in consonant recognition can be obtained with individualized adjustment of consonant amplitude for children as young as 5 year of age.

  1. Programming generalization of social skills in preschool children with hearing impairments.

    PubMed

    Ducharme, D E; Holborn, S W

    1997-01-01

    The efficacy of a social skills training package in producing stimulus generalization, both with and without the systematic application of generalization programming techniques, was evaluated with 5 preschool children with hearing impairments. The evaluation was conducted within a multiple baseline design. Generalization probes were conducted daily. The social skills training package was implemented in a training setting and produced high, stable rates of social interaction in that setting. However, generalization of the social skills to new teachers, peers, and play activities did not occur until generalization programming strategies were applied in the original training setting. Using sufficient stimulus exemplars and contacting natural consequences appeared to be the key strategies for promoting generalization of social interaction. In addition, the use of supplementary procedures (e.g., a fluency criterion and treatment integrity checks) may have contributed to stimulus generalization.

  2. Acquisition of adjectives and adverbs in sentences written by hearing impaired and aphasic children.

    PubMed

    Heward, W L; Eachus, H T

    1979-01-01

    The effect of an instructional package, which included modeling, reinforcement, and remedial feedback on the rate, accuracy, and topography of sentences composed by four hearing impaired and aphasic children, was examined. In a specially designed classroom, students wrote sentences describing a stimulus picture on acetate sheets placed on the stage of an overhead projector which was built into each student's desk. This arrangement provided the teacher and other students immediate and continuous visual access to each student's sentences. In a multiple baseline design across behaviors, model sentences were projected and token reinforcment and remedial feedback were made contingent upon writing correct sentences containing prenominal adjectives only, then adverbs only, then prenomial adjectives plus adverbs. During baseline all student displayed poor written language skills and seldom wrote sentences containing modifiers. When the instructional package was implemented, all students demonstrated significant increases in response rate, accuracy, and percentage of correct sentences including prenominal adjectives and adverbs.

  3. Novel and recurrent CIB2 variants, associated with nonsyndromic deafness, do not affect calcium buffering and localization in hair cells.

    PubMed

    Seco, Celia Zazo; Giese, Arnaud P; Shafique, Sobia; Schraders, Margit; Oonk, Anne M M; Grossheim, Mike; Oostrik, Jaap; Strom, Tim; Hegde, Rashmi; van Wijk, Erwin; Frolenkov, Gregory I; Azam, Maleeha; Yntema, Helger G; Free, Rolien H; Riazuddin, Saima; Verheij, Joke B G M; Admiraal, Ronald J; Qamar, Raheel; Ahmed, Zubair M; Kremer, Hannie

    2016-04-01

    Variants in CIB2 can underlie either Usher syndrome type I (USH1J) or nonsyndromic hearing impairment (NSHI) (DFNB48). Here, a novel homozygous missense variant c.196C>T and compound heterozygous variants, c.[97C>T];[196C>T], were found, respectively, in two unrelated families of Dutch origin. Besides, the previously reported c.272 T>C functional missense variant in CIB2 was identified in two families of Pakistani origin. The missense variants are demonstrated not to affect subcellular localization of CIB2 in vestibular hair cells in ex vivo expression experiments. Furthermore, these variants do not affect the ATP-induced calcium responses in COS-7 cells. However, based on the residues affected, the variants are suggested to alter αIIβ integrin binding. HI was nonsyndromic in all four families. However, deafness segregating with the c.272T>C variant in one Pakistani family is remarkably less severe than that in all other families with this mutation. Our results contribute to the insight in genotype-phenotype correlations of CIB2 mutations.

  4. 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.

  5. Vibrotactile Presentation of Musical Notes to the Glabrous Skin for Adults with Normal Hearing or a Hearing Impairment: Thresholds, Dynamic Range and High-Frequency Perception

    PubMed Central

    Maté-Cid, Saúl; Fulford, Robert; Seiffert, Gary; Ginsborg, Jane

    2016-01-01

    Presentation of music as vibration to the skin has the potential to facilitate interaction between musicians with hearing impairments and other musicians during group performance. Vibrotactile thresholds have been determined to assess the potential for vibrotactile presentation of music to the glabrous skin of the fingertip, forefoot and heel. No significant differences were found between the thresholds for sinusoids representing notes between C1 and C6 when presented to the fingertip of participants with normal hearing and with a severe or profound hearing loss. For participants with normal hearing, thresholds for notes between C1 and C6 showed the characteristic U-shape curve for the fingertip, but not for the forefoot and heel. Compared to the fingertip, the forefoot had lower thresholds between C1 and C3, and the heel had lower thresholds between C1 and G2; this is attributed to spatial summation from the Pacinian receptors over the larger contactor area used for the forefoot and heel. Participants with normal hearing assessed the perception of high-frequency vibration using 1s sinusoids presented to the fingertip and were found to be more aware of transient vibration at the beginning and/or end of notes between G4 and C6 when stimuli were presented 10dB above threshold, rather than at threshold. An average of 94% of these participants reported feeling continuous vibration between G4 and G5 with stimuli presented 10dB above threshold. Based on the experimental findings and consideration of health effects relating to vibration exposure, a suitable range of notes for vibrotactile presentation of music is identified as being from C1 to G5. This is more limited than for human hearing but the fundamental frequencies of the human voice, and the notes played by many instruments, lie within it. However, the dynamic range might require compression to avoid the negative effects of amplitude on pitch perception. PMID:27191400

  6. Folic acid deficiency induces premature hearing loss through mechanisms involving cochlear oxidative stress and impairment of homocysteine metabolism.

    PubMed

    Martínez-Vega, Raquel; Garrido, Francisco; Partearroyo, Teresa; Cediel, Rafael; Zeisel, Steven H; Martínez-Álvarez, Concepción; Varela-Moreiras, Gregorio; Varela-Nieto, Isabel; Pajares, María A

    2015-02-01

    Nutritional imbalance is emerging as a causative factor of hearing loss. Epidemiologic studies have linked hearing loss to elevated plasma total homocysteine (tHcy) and folate deficiency, and have shown that folate supplementation lowers tHcy levels potentially ameliorating age-related hearing loss. The purpose of this study was to address the impact of folate deficiency on hearing loss and to examine the underlying mechanisms. For this purpose, 2-mo-old C57BL/6J mice (Animalia Chordata Mus musculus) were randomly divided into 2 groups (n = 65 each) that were fed folate-deficient (FD) or standard diets for 8 wk. HPLC analysis demonstrated a 7-fold decline in serum folate and a 3-fold increase in tHcy levels. FD mice exhibited severe hearing loss measured by auditory brainstem recordings and TUNEL-positive-apoptotic cochlear cells. RT-quantitative PCR and Western blotting showed reduced levels of enzymes catalyzing homocysteine (Hcy) production and recycling, together with a 30% increase in protein homocysteinylation. Redox stress was demonstrated by decreased expression of catalase, glutathione peroxidase 4, and glutathione synthetase genes, increased levels of manganese superoxide dismutase, and NADPH oxidase-complex adaptor cytochrome b-245, α-polypeptide (p22phox) proteins, and elevated concentrations of glutathione species. Altogether, our findings demonstrate, for the first time, that the relationship between hyperhomocysteinemia induced by folate deficiency and premature hearing loss involves impairment of cochlear Hcy metabolism and associated oxidative stress.

  7. Selective Inner Hair Cell Dysfunction in Chinchillas Impairs Hearing-in-Noise in the Absence of Outer Hair Cell Loss.

    PubMed

    Lobarinas, Edward; Salvi, Richard; Ding, Dalian

    2016-04-01

    Poorer hearing in the presence of background noise is a significant problem for the hearing impaired. Ototoxic drugs, ageing, and noise exposure can damage the sensory hair cells of the inner ear that are essential for normal hearing sensitivity. The relationship between outer hair cell (OHC) loss and progressively poorer hearing sensitivity in quiet or in competing background noise is supported by a number of human and animal studies. In contrast, the effect of moderate inner hair cell (IHC) loss or dysfunction shows almost no impact on behavioral measures of hearing sensitivity in quiet, when OHCs remain intact, but the relationship between selective IHC loss and hearing in noise remains relatively unknown. Here, a moderately high dose of carboplatin (75 mg/kg) that produced IHC loss in chinchillas ranging from 40 to 80 % had little effect on thresholds in quiet. However, when tested in the presence of competing broadband (BBN) or narrowband noise (NBN), thresholds increased significantly. IHC loss >60 % increased signal-to-noise ratios (SNRs) for tones (500-11,300 Hz) in competing BBN by 5-10 dB and broadened the masking function under NBN. These data suggest that IHC loss or dysfunction may play a significant role in listening in noise independent of OHC integrity and that these deficits may be present even when thresholds in quiet are within normal limits.

  8. CENTRAL AUDTIORY DEVELOPMENT IN CHILDREN WITH HEARING LOSS: CLINICAL RELEVANCE OF THE P1 CAEP BIOMARKER IN HEARING-IMPAIRED CHILDREN WITH MULTIPLE DISABILITIES*

    PubMed Central

    Sharma, Anu; Glick, Hannah; Campbell, Julia; Biever, Allison

    2013-01-01

    Objective First, we review the development and plasticity of the central auditory pathways in infants and children with hearing loss who are fitted with cochlear implants (CIs). Second, we describe case studies demonstrating the clinical utility of the P1 central auditory evoked potential (CAEP) for evaluating cortical auditory maturation in the rapidly increasing number of cochlear-implanted children who have multiple disabilities. Study Design Children who receive CIs provide a platform to examine the trajectories of deprivation-induced and experience-dependent plasticity in the central auditory system. We review the evidence for, and time limits of sensitive periods for cortical auditory maturation framing an optimal period for cochlear implantation. Finally, we evaluate the use of the P1 biomarker as an objective assessment tool in the special case of children with multiple disabilities. Results The P1 response was useful in assessing central auditory maturation in patients with CHARGE association, ANSD, and Pallister-Killian Syndrome concomitant with hearing loss. Conclusion The presence of co-existing disabilities in addition to hearing loss poses unique challenges regarding both pre-intervention evaluation and post-intervention rehabilitation for children with multiple disabilities. When combined with a standard audiological test battery, the P1 CAEP biomarker has a useful role in objectively evaluating the maturation of central auditory pathways to determine the effectiveness of various intervention strategies in hearing-impaired children with multiple disabilities. PMID:24273704

  9. Mutation Spectrum of Common Deafness-Causing Genes in Patients with Non-Syndromic Deafness in the Xiamen Area, China

    PubMed Central

    Wu, Lihua; Chen, Juan; Kang, Dongyang; Xu, Xiufeng; Li, Ruiyu; Han, Dongyi; Dai, Pu

    2015-01-01

    In China, approximately 30,000 babies are born with hearing impairment each year. However, the molecular factors causing congenital hearing impairment in the Xiamen area of Fujian province have not been evaluated. To provide accurate genetic testing and counseling in the Xiamen area, we investigated the molecular etiology of non-syndromic deafness in a deaf population from Xiamen. Unrelated students with hearing impairment (n = 155) who attended Xiamen Special Education School in Fujian Province were recruited for this study. Three common deafness-related genes, GJB2, SLC26A4, and mtDNA12SrRNA, were analyzed using all-exon sequencing. GJB2 mutations were detected in 27.1% (42/155) of the entire cohort. The non-syndromic hearing loss (NSHL) hotspot mutations c.109G>A (p.V37I) and c.235delC were found in this population, whereas the Caucasian hotspot mutation c.35delG was not. The allelic frequency of the c.109G>A mutation was 9.03% (28/310), slightly higher than that of c.235delC (8.39%, 26/310), which is the most common GJB2 mutation in most areas of China. The allelic frequency of the c.109G>A mutation was significantly higher in this Xiamen’s deaf population than that in previously reported cohorts (P = 0.00). The SLC26A4 mutations were found in 16.77% (26/155) of this cohort. The most common pathogenic allele was c.IVS7-2A>G (6.13%, 19/310), and the second most common was the c.1079C>T (p.A360V) mutation (1.94%, 6/310) which has rarely been reported as a hotspot mutation in other studies. The mutation rate of mtDNA12SrRNA in this group was 3.87% (6/155), all being the m.A1555G mutation. These findings show the specificity of the common deaf gene-mutation spectrum in this area. According to this study, there were specific hotspot mutations in Xiamen deaf patients. Comprehensive sequencing analysis of the three common deaf genes can help portray the mutation spectrum and develop optimal testing strategies for deaf patients in this area. PMID:26252218

  10. In search of correlation between hand preference and laterality of hearing impairment in patients with otosclerosis.

    PubMed

    Migirov, Lela; Wolf, Michael

    2014-10-01

    The aim of this study was to investigate a possible correlation between handedness and laterality of hearing impairment due to otosclerosis. All patients operated for otosclerosis between August 2008 and February 2014 were queried about their handedness. The study group consisted of 218 right-handed and 21 left-handed (8.9%) patients [139 female (58.2%) and 100 male] with an age range of 18-75 years (mean 46.1 years). One-hundred and fifty-seven patients had a bilateral otosclerosis (BO) and 82 (34.3%) had a unilateral otosclerosis (UO). There were 11 left-handed male and 10 left-handed female (11% vs. 7.2 %, p = 0.305). In patients with UO, the left ear (LE) was affected in 6/11 (58.3%) left handed ones, and the right ear (RE) in 41/71 (57.7%) right handed ones (p = 0.842). In patients with BO, the LE was more affected in the left-handed ones, and the RE in the right-handed ones (7/10, 70% and 87/147, 59.2 %, respectively, p = 0.5). Overall, 13/21 (61.9%) left-handed patients presented with only/mostly left-sided otosclerosis, while 128/218 (58.7%) right-handed patients presented with only/mostly right-sided otosclerosis (p = 0.584). Clinical relevance of presented findings is unclear yet nevertheless current study may contribute one more element in the multifactorial process of otosclerosis-related hearing loss.

  11. A genome-wide association study for age-related hearing impairment in the Saami.

    PubMed

    Van Laer, Lut; Huyghe, Jeroen R; Hannula, Samuli; Van Eyken, Els; Stephan, Dietrich A; Mäki-Torkko, Elina; Aikio, Pekka; Fransen, Erik; Lysholm-Bernacchi, Alana; Sorri, Martti; Huentelman, Matthew J; Van Camp, Guy

    2010-06-01

    This study aimed at contributing to the elucidation of the genetic basis of age-related hearing impairment (ARHI), a common multifactorial disease with an important genetic contribution as demonstrated by heritability studies. We conducted a genome-wide association study (GWAS) in the Finnish Saami, a small, ancient, genetically isolated population without evidence of demographic expansion. The choice of this study population was motivated by its anticipated higher extent of LD, potentially offering a substantial power advantage for association mapping. DNA samples and audiometric measurements were collected from 352 Finnish Saami individuals, aged between 50 and 75 years. To reduce the burden of multiple testing, we applied principal component (PC) analysis to the multivariate audiometric phenotype. The first three PCs captured 80% of the variation in hearing thresholds, while maintaining biologically important audiometric features. All subjects were genotyped with the Affymetrix 100 K chip. To account for multiple levels of relatedness among subjects, as well as for population stratification, association testing was performed using a mixed model. We summarised the top-ranking association signals for the three traits under study. The top-ranked SNP, rs457717 (P-value 3.55 x 10(-7)), was associated with PC3 and was localised in an intron of the IQ motif-containing GTPase-activating-like protein (IQGAP2). Intriguingly, the SNP rs161927 (P-value 0.000149), seventh-ranked for PC1, was positioned immediately downstream from the metabotropic glutamate receptor-7 gene (GRM7). As a previous GWAS of a European and Finnish sample set already suggested a role for GRM7 in ARHI, this study provides further evidence for the involvement of this gene.

  12. A genome-wide association study for age-related hearing impairment in the Saami

    PubMed Central

    Van Laer, Lut; Huyghe, Jeroen R; Hannula, Samuli; Van Eyken, Els; Stephan, Dietrich A; Mäki-Torkko, Elina; Aikio, Pekka; Fransen, Erik; Lysholm-Bernacchi, Alana; Sorri, Martti; Huentelman, Matthew J; Van Camp, Guy

    2010-01-01

    This study aimed at contributing to the elucidation of the genetic basis of age-related hearing impairment (ARHI), a common multifactorial disease with an important genetic contribution as demonstrated by heritability studies. We conducted a genome-wide association study (GWAS) in the Finnish Saami, a small, ancient, genetically isolated population without evidence of demographic expansion. The choice of this study population was motivated by its anticipated higher extent of LD, potentially offering a substantial power advantage for association mapping. DNA samples and audiometric measurements were collected from 352 Finnish Saami individuals, aged between 50 and 75 years. To reduce the burden of multiple testing, we applied principal component (PC) analysis to the multivariate audiometric phenotype. The first three PCs captured 80% of the variation in hearing thresholds, while maintaining biologically important audiometric features. All subjects were genotyped with the Affymetrix 100 K chip. To account for multiple levels of relatedness among subjects, as well as for population stratification, association testing was performed using a mixed model. We summarised the top-ranking association signals for the three traits under study. The top-ranked SNP, rs457717 (P-value 3.55 × 10−7), was associated with PC3 and was localised in an intron of the IQ motif-containing GTPase-activating-like protein (IQGAP2). Intriguingly, the SNP rs161927 (P-value 0.000149), seventh-ranked for PC1, was positioned immediately downstream from the metabotropic glutamate receptor-7 gene (GRM7). As a previous GWAS of a European and Finnish sample set already suggested a role for GRM7 in ARHI, this study provides further evidence for the involvement of this gene. PMID:20068591

  13. Self-Esteem in Hearing-Impaired Children: The Influence of Communication, Education, and Audiological Characteristics

    PubMed Central

    Theunissen, Stephanie C. P. M.; Rieffe, Carolien; Netten, Anouk P.; Briaire, Jeroen J.; Soede, Wim; Kouwenberg, Maartje; Frijns, Johan H. M.

    2014-01-01

    Objective Sufficient self-esteem is extremely important for psychosocial functioning. It is hypothesized that hearing-impaired (HI) children have lower levels of self-esteem, because, among other things, they frequently experience lower language and communication skills. Therefore, the aim of this study was to compare HI children's self-esteem across different domains with those of normal hearing (NH) children and to investigate the influence of communication, type of education, and audiological characteristics. Methods This large (N = 252) retrospective, multicenter study consisted of two age- and gender-matched groups: 123 HI children and 129 NH controls (mean age  = 11.8 years). Self-reports were used to measure self-esteem across four domains: perceived social acceptance by peers, perceived parental attention, perceived physical appearance, and global self-esteem. Results HI children experienced lower levels of self-esteem regarding peers and parents than NH controls. Particularly HI children who attended special education for the deaf were at risk, even after correcting for their language development and intelligence. Yet, levels of global self-esteem and self-esteem involving physical appearance in HI children equalled those of NH controls. Furthermore, younger age at implantation and longer duration of having cochlear implants (CIs) were related to higher levels of self-esteem. Conclusion HI children experience lower levels of self-esteem in the social domains. Yet, due to the heterogeneity of the HI population, there is high variability in levels of self-esteem. Discussion Clinicians must always be aware of the risk and protective factors related to self-esteem in order to help individual patients reach their full potential. PMID:24722329

  14. 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.

  15. 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.

  16. Postural control, motor skills, and health-related quality of life in children with hearing impairment: a systematic review.

    PubMed

    Rajendran, Venkadesan; Roy, Finita Glory; Jeevanantham, Deepa

    2012-04-01

    Children with hearing impairment have balance and motor deficits primarily due to concomitant damage to the vestibular structures. Psycho-intellectual and social developmental disorders, as well as elimination of social activities and participation may diminish health-related quality of life in these children. Despite the documentation, assessment of balance, motor deficits, and health-related quality of life of these children are not included in the educational program, unless obvious neurological or orthopedic disorders are diagnosed. The objective of this review was to systematically analyze the available information in the literatures regarding the postural control, motor skills, and health-related quality of life in children with hearing impairment. Searches of data sources PubMed, MEDLINE, CINHAL, EMBASE, SCOPUS, ISI of web science, Cochrane Library, and AMED database were performed from the earliest to 7 February 2011. Study eligibility criteria included non-interventional studies that addressed postural control, motor skills, and health-related quality of life in children with hearing impairment. For each eligible article, data were extracted using custom-designed forms by a single investigator. Collected data included study demographics, study design, study population, sample size, outcome measures, and results. A total of 11,872 articles were retrieved, and 17 articles were found to be eligible for inclusion. Of the 17 articles included, five articles analyzed health-related quality of life alone, two articles analyzed balance alone, two articles analyzed motor performance alone, two articles analyzed vestibular dysfunction alone, two articles included both vestibular dysfunction and balance, two articles included both motor performance and balance, and two articles investigated vestibular, balance as well as motor impairments. Heterogeneity of the studies prevented us from performing methodological quality assessment and meta-analysis. The results of

  17. [The mutation spectrum of the GJB2 gene in Belarussian patients with hearing loss. Results of pilot genetic screening of hearing impairment in newborns].

    PubMed

    Bliznets, E A; Marcul', D N; Khorov, O G; Markova, T G; Poliakov, A V

    2014-02-01

    A total of 111 unrelated probands and their 8 sibs from Grodno oblast (Belarus) with bilateral isolated sensorineural hearing impairment were studied for the presence of mutations in the connexin 26--GJB2gene. Mutations were detected in 51 probands (46% of the sample). A significantly higher frequency of the GJB2gene mutations was observed in familial cases of the disease with the autosomal recessive type of inheritance (in 78% of families). Detected peculiarities of the GJB2 gene mutation spectrum demonstrated that use of the algorithm, which was developed for Russian patients, is optimal for the molecular study of patients from Be- larus. In the sample of patients with hearing loss, the highest (among other similar samples studied in the world) allele frequency of c.313_326de114 mutation (7% out of all pathological GJB2 alleles) was registered; Polish origin of this deletion was suggested. It was demonstrated that detection of the GJB2 gene mutation on only one patient's chromosome is insufficient to confirm a molecular genetic diagnosis of hearing loss of the DFNB1 genetic type (autosomal recessive hearing loss caused by the GJB2 gene mutations). Pilot screening in the presence of GJB2 gene mutations in newborns from Grodno oblast was conducted. The material from 235 children was studied during the screening; nine heterozygous carriers of the mutation were found. The c.35delG mutation was detected in a homozygous state in a single newborn (hearing loss of moderate severity was subsequently audiologically confirmed in this child).

  18. Behavioral measures of cochlear compression and temporal resolution as predictors of speech masking release in hearing-impaired listeners.

    PubMed

    Gregan, Melanie J; Nelson, Peggy B; Oxenham, Andrew J

    2013-10-01

    Hearing-impaired (HI) listeners often show less masking release (MR) than normal-hearing listeners when temporal fluctuations are imposed on a steady-state masker, even when accounting for overall audibility differences. This difference may be related to a loss of cochlear compression in HI listeners. Behavioral estimates of compression, using temporal masking curves (TMCs), were compared with MR for band-limited (500-4000 Hz) speech and pure tones in HI listeners and age-matched, noise-masked normal-hearing (NMNH) listeners. Compression and pure-tone MR estimates were made at 500, 1500, and 4000 Hz. The amount of MR was defined as the difference in performance between steady-state and 10-Hz square-wave-gated speech-shaped noise. In addition, temporal resolution was estimated from the slope of the off-frequency TMC. No significant relationship was found between estimated cochlear compression and MR for either speech or pure tones. NMNH listeners had significantly steeper off-frequency temporal masking recovery slopes than did HI listeners, and a small but significant correlation was observed between poorer temporal resolution and reduced MR for speech. The results suggest either that the effects of hearing impairment on MR are not determined primarily by changes in peripheral compression, or that the TMC does not provide a sufficiently reliable measure of cochlear compression.

  19. [The expertise of health status dynamics in the members of locomotive crews presenting with occupational sensorineural impairment of hearing].

    PubMed

    Pankova, V B; Bulatskaia, T V; Merkulova, E P

    2013-01-01

    The objective of the present study was to estimate the diagnostic value of the characteristics of somatic pathology included in the algorithm for early diagnostics of occupational sensorineural impairment of hearing (OSNIH). The relationship between the development of the main nosological forms of somatic pathology as possible markers of internal ear diseases and professional sensorineural impairment of hearing was elucidated among the members of locomotive crews (n=224). The parameters of interest were evaluated 4 years before, within 1 year, and 4 years after the establishment of diagnosis of OSNIH. It was shown that the members of locomotive crews most frequently suffer atherosclerosis (20.0%), gastrointestinal disorders (34.8%), arterial hypertension (25.0%), osteochondrosis (largely vertebrogenic lumbodynia (21.3%)), and microcirculatory disturbances in the form of vascular spasm in the fundus of the eye (32.9%). 97.6% of the subjects above 35 years of age presented with hypercholesterolemia. Comparative analysis of the occurrence of these nosological forms among the members of locomotive crews with hearing problems on the one hand and the normally functioning organ of hearing on the other hand has demonstrated that none of these pathologies can be regarded as a marker for early diagnosis of OSNIH. At the same time, overweight even in the subjects under 35 years of age should be taken into account when planning and implementing preventive measures.

  20. Oral hygiene status in relation to sociodemographic factors of children and adults who are hearing impaired, attending a special school.

    PubMed

    Kumar, Santhosh; Dagli, Rushabh Jayesh; Mathur, Anmol; Jain, Manish; Duraiswamy, Prabu; Kulkarni, Suhas

    2008-01-01

    This study examined the oral hygiene levels and periodontal status in a group of children and adults with hearing impairment attending a special school in Udaipur, India. Oral hygiene status was assessed by the Simplified Oral Hygiene Index (OHI-S) of Greene and Vermillion and periodontal status by the Community Periodontal Index. An analysis using a bivariate analysis revealed that all the oral hygiene variables varied significantly with age, economic status, and education of the parents. A multiple regression analysis showed that the education of the mother was the single best predictor for oral hygiene status and explained 92% of the variance. These findings show that children with hearing impairment have poor oral hygiene and high levels of periodontal disease. This may be due to a lack of communication; hence, appropriate oral health education should be tailored to the needs of these students with the support of their teachers and their parents.