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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. SLC26A4 gene is frequently involved in nonsyndromic hearing impairment with enlarged vestibular aqueduct in Caucasian populations.

    PubMed

    Albert, Sébastien; Blons, Hélène; Jonard, Laurence; Feldmann, Delphine; Chauvin, Pierre; Loundon, Nathalie; Sergent-Allaoui, Annie; Houang, Muriel; Joannard, Alain; Schmerber, Sébastien; Delobel, Bruno; Leman, Jacques; Journel, Hubert; Catros, Hélène; Dollfus, Hélène; Eliot, Marie-Madeleine; David, Albert; Calais, Catherine; Drouin-Garraud, Valérie; Obstoy, Marie-Françoise; Tran Ba Huy, Patrice; Lacombe, Didier; Duriez, Françoise; Francannet, Christine; Bitoun, Pierre; Petit, Christine; Garabédian, Eréa-Noël; Couderc, Rémy; Marlin, Sandrine; Denoyelle, Françoise

    2006-06-01

    Sensorineural hearing loss is the most frequent sensory deficit of childhood and is of genetic origin in up to 75% of cases. It has been shown that mutations of the SLC26A4 (PDS) gene were involved in syndromic deafness characterized by congenital sensorineural hearing impairment and goitre (Pendred's syndrome), as well as in congenital isolated deafness (DFNB4). While the prevalence of SLC26A4 mutations in Pendred's syndrome is clearly established, it remains to be studied in large cohorts of patients with nonsyndromic deafness and detailed clinical informations. In this report, 109 patients from 100 unrelated families, aged from 1 to 32 years (median age: 10 years), with nonsyndromic deafness and enlarged vestibular aqueduct, were genotyped for SLC26A4 using DHPLC molecular screening and sequencing. In all, 91 allelic variants were observed in 100 unrelated families, of which 19 have never been reported. The prevalence of SLC26A4 mutations was 40% (40/100), with biallelic mutation in 24% (24/100), while six families were homozygous. All patients included in this series had documented deafness, associated with EVA and without any evidence of syndromic disease. Among patients with SLC26A4 biallelic mutations, deafness was more severe, fluctuated more than in patients with no mutation. In conclusion, the incidence of SLC26A4 mutations is high in patients with isolated deafness and enlarged vestibular aqueduct and could represent up to 4% of nonsyndromic hearing impairment. SLC26A4 could be the second most frequent gene implicated in nonsyndromic deafness after GJB2, in this Caucasian population.

  5. GJB2 mutation spectrum in 2063 Chinese patients with nonsyndromic hearing impairment

    PubMed Central

    Dai, Pu; Yu, Fei; Han, Bing; Liu, Xuezhong; Wang, Guojian; Li, Qi; Yuan, Yongyi; Liu, Xin; Huang, Deliang; Kang, Dongyang; Zhang, Xin; Yuan, Huijun; Yao, Kun; Hao, Jinsheng; He, Jia; He, Yong; Wang, Youqin; Ye, Qing; Yu, Youjun; Lin, Hongyan; Liu, Lijia; Deng, Wei; Zhu, Xiuhui; You, Yiwen; Cui, Jinghong; Hou, Nongsheng; Xu, Xuehai; Zhang, Jin; Tang, Liang; Song, Rendong; Lin, Yongjun; Sun, Shuanzhu; Zhang, Ruining; Wu, Hao; Ma, Yuebing; Zhu, Shanxiang; Wu, Bai-lin; Han, Dongyi; Wong, Lee-Jun C

    2009-01-01

    Background Mutations in GJB2 are the most common molecular defects responsible for autosomal recessive nonsyndromic hearing impairment (NSHI). The mutation spectra of this gene vary among different ethnic groups. Methods In order to understand the spectrum and frequency of GJB2 mutations in the Chinese population, the coding region of the GJB2 gene from 2063 unrelated patients with NSHI was PCR amplified and sequenced. Results A total of 23 pathogenic mutations were identified. Among them, five (p.W3X, c.99delT, c.155_c.158delTCTG, c.512_c.513insAACG, and p.Y152X) are novel. Three hundred and seven patients carry two confirmed pathogenic mutations, including 178 homozygotes and 129 compound heterozygotes. One hundred twenty five patients carry only one mutant allele. Thus, GJB2 mutations account for 17.9% of the mutant alleles in 2063 NSHI patients. Overall, 92.6% (684/739) of the pathogenic mutations are frame-shift truncation or nonsense mutations. The four prevalent mutations; c.235delC, c.299_c.300delAT, c.176_c.191del16, and c.35delG, account for 88.0% of all mutantalleles identified. The frequency of GJB2 mutations (alleles) varies from 4% to 30.4% among different regions of China. It also varies among different sub-ethnic groups. Conclusion In some regions of China, testing of the three most common mutations can identify at least one GJB2 mutant allele in all patients. In other regions such as Tibet, the three most common mutations account for only 16% the GJB2 mutant alleles. Thus, in this region, sequencing of GJB2 would be recommended. In addition, the etiology of more than 80% of the mutant alleles for NSHI in China remains to be identified. Analysis of other NSHI related genes will be necessary. PMID:19366456

  6. 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

  7. 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

  8. 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.

  9. 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.

  10. A Point Mutation in the Gene for Asparagine-Linked Glycosylation 10B (Alg10b) Causes Nonsyndromic Hearing Impairment in Mice (Mus musculus)

    PubMed Central

    Probst, Frank J.; Corrigan, Rebecca R.; del Gaudio, Daniela; Salinger, Andrew P.; Lorenzo, Isabel; Gao, Simon S.; Chiu, Ilene; Xia, Anping

    2013-01-01

    The study of mouse hearing impairment mutants has led to the identification of a number of human hearing impairment genes and has greatly furthered our understanding of the physiology of hearing. The novel mouse mutant neurological/sensory 5 (nse5) demonstrates a significantly reduced or absent startle response to sound and is therefore a potential murine model of human hearing impairment. Genetic analysis of 500 intercross progeny localized the mutant locus to a 524 kilobase (kb) interval on mouse chromosome 15. A missense mutation in a highly-conserved amino acid was found in the asparagine-linked glycosylation 10B gene (Alg10b), which is within the critical interval for the nse5 mutation. A 20.4 kb transgene containing a wildtype copy of the Alg10b gene rescued the mutant phenotype in nse5/nse5 homozygous animals, confirming that the mutation in Alg10b is responsible for the nse5/nse5 mutant phenotype. Homozygous nse5/nse5 mutants had abnormal auditory brainstem responses (ABRs), distortion product otoacoustic emissions (DPOAEs), and cochlear microphonics (CMs). Endocochlear potentials (EPs), on the other hand, were normal. ABRs and DPOAEs also confirmed the rescue of the mutant nse5/nse5 phenotype by the wildtype Alg10b transgene. These results suggested a defect in the outer hair cells of mutant animals, which was confirmed by histologic analysis. This is the first report of mutation in a gene involved in the asparagine (N)-linked glycosylation pathway causing nonsyndromic hearing impairment, and it suggests that the hearing apparatus, and the outer hair cells in particular, are exquisitely sensitive to perturbations of the N-linked glycosylation pathway. PMID:24303013

  11. A point mutation in the gene for asparagine-linked glycosylation 10B (Alg10b) causes nonsyndromic hearing impairment in mice (Mus musculus).

    PubMed

    Probst, Frank J; Corrigan, Rebecca R; Del Gaudio, Daniela; Salinger, Andrew P; Lorenzo, Isabel; Gao, Simon S; Chiu, Ilene; Xia, Anping; Oghalai, John S; Justice, Monica J

    2013-01-01

    The study of mouse hearing impairment mutants has led to the identification of a number of human hearing impairment genes and has greatly furthered our understanding of the physiology of hearing. The novel mouse mutant neurological/sensory 5 (nse5) demonstrates a significantly reduced or absent startle response to sound and is therefore a potential murine model of human hearing impairment. Genetic analysis of 500 intercross progeny localized the mutant locus to a 524 kilobase (kb) interval on mouse chromosome 15. A missense mutation in a highly-conserved amino acid was found in the asparagine-linked glycosylation 10B gene (Alg10b), which is within the critical interval for the nse5 mutation. A 20.4 kb transgene containing a wildtype copy of the Alg10b gene rescued the mutant phenotype in nse5/nse5 homozygous animals, confirming that the mutation in Alg10b is responsible for the nse5/nse5 mutant phenotype. Homozygous nse5/nse5 mutants had abnormal auditory brainstem responses (ABRs), distortion product otoacoustic emissions (DPOAEs), and cochlear microphonics (CMs). Endocochlear potentials (EPs), on the other hand, were normal. ABRs and DPOAEs also confirmed the rescue of the mutant nse5/nse5 phenotype by the wildtype Alg10b transgene. These results suggested a defect in the outer hair cells of mutant animals, which was confirmed by histologic analysis. This is the first report of mutation in a gene involved in the asparagine (N)-linked glycosylation pathway causing nonsyndromic hearing impairment, and it suggests that the hearing apparatus, and the outer hair cells in particular, are exquisitely sensitive to perturbations of the N-linked glycosylation pathway.

  12. 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.

  13. 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.

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

    PubMed Central

    Du, Jihong; Deng, Jianhua

    2016-01-01

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

  15. Hearing Impairment

    MedlinePlus

    ... may include inserting an object such as a cotton swab too far into the ear, a sudden explosion or other loud noise, a sudden change in air pressure, a head injury, or repeated ear infections. Sensorineural hearing impairment results from problems with or damage ...

  16. 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.

  17. 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 ...

  18. 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 ...

  19. 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

  20. 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

  1. 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.

  2. 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.

  3. Mainstreaming the Hearing Impaired Student.

    ERIC Educational Resources Information Center

    Szelazkiewicz, Sara

    This review of the literature on mainstreaming students with hearing impairments begins by defining "hearing impairment,""deafness," and "hard of hearing". The paper briefly considers causes of hearing impairments and types of hearing impairments. Suggestions for preparing a class for a student with hearing loss includes having a visible class…

  4. 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.

  5. 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. ...

  6. Chinese children with nonsyndromic cleft lip/palate: Factors associated with hearing disorder.

    PubMed

    Ma, Xiaoran; Li, Yue Wing; Ma, Lian; McPherson, Bradley

    2016-09-01

    This study examined the auditory status of Chinese children with nonsyndromic cleft lip/palate (NSCL/P), investigated factors associated with peripheral hearing loss and compared results with earlier studies in western countries. Case history profiles and audiological data from 148 Chinese children with NSCL/P, aged between 6 and 15 years, who attended the Cleft Lip and Palate Clinic Center in a major Chinese urban hospital from July 2012 to September 2013 were acquired. The audiological status of the participants was reviewed, based on the results of their pure tone audiometry, tympanometry and acoustic reflex thresholds assessments. Factors including age, gender, cleft type, residential locality and school achievement were examined in relation to auditory status. Findings revealed that 17% of the Chinese children with NSCL/P had hearing impairment at the time of assessment. Unilateral hearing loss was noted in 12% of children and in 5% of cases bilateral hearing loss was noted. In the majority of cases the hearing loss was slight and conductive in nature. Age, gender, residential locality and school achievement were found to have no relationship with severity of hearing loss. Children with cleft lip showed a lower degree of hearing impairment than children with cleft palate or cleft lip and palate. Similar to studies for western children, Chinese children with CL/P associated with no known syndrome are at risk of peripheral hearing loss, generally of conductive type. However, the prevalence of peripheral hearing loss appears to be less than in western children with NSCL/P. Ethnic/racial factors may be a major contributing factor accounting for the discrepancies between the current results and western studies. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  7. 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.

  8. 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.

  9. 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);…

  10. 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);…

  11. 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.

  12. 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.

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

    PubMed

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

    2017-06-01

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

  14. Molecular screening of patients with nonsyndromic hearing loss from Nanjing city of China☆

    PubMed Central

    Lu, Yajie; Dai, Dachun; Chen, Zhibin; Cao, Xin; Bu, Xingkuan; Wei, Qinjun; Xing, Guangqian

    2011-01-01

    Hearing loss is the most frequent sensory disorder involving a multitude of factors, and at least 50% of cases are due to genetic etiology. To further characterize the molecular etiology of hearing loss in the Chinese population, we recruited a total of 135 unrelated patients with nonsyndromic sensorineural hearing loss (NSHL) for mutational screening of GJB2, GJB3, GJB6, SLC26A4, SLC26A5 IVS2-2A>G and mitochondrial 12SrRNA, tRNASer(UCN) by PCR amplification and direct DNA sequencing. The carrier frequencies of deafness-causing mutations in these patients were 35.55% in GJB2, 3.70% in GJB6, 15.56% in SLC26A4 and 8.14% in mitochondrial 12SrRNA, respectively. The results indicate the necessity of genetic screening for mutations of these causative genes in Chinese population with nonsyndromic hearing loss. PMID:23554706

  15. Accessibility for the hearing impaired.

    PubMed

    Alberti, P W

    1999-10-05

    The social anthropology of mild hearing loss is gradually being accepted, as it affects both children and adults. With this comes the understanding that effective aural communication requires adequate sound sources and a good transmission medium as well as good hearing. If the first two conditions are met, much hearing disability might be avoided without resorting to a hearing aid. A plea is made for better accessibility for the hearing impaired by improving environmental conditions for acoustic signals, especially speech.

  16. 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

  17. Visual impairment in the hearing impaired students.

    PubMed

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

    2009-01-01

    Ocular problems are more common in children with hearing problems than in normal children. Neglected visual impairment could aggravate educational and social disability. To detect and treat visual impairment, if any, in hearing-impaired children. Observational, clinical case series of hearing-impaired children in schools providing special education. 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. Excel software was used for data entry and SSPS for analysis. 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. 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.

  18. 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…

  19. 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…

  20. Prevalence of mitochondrial DNA mutations in sporadic patients with nonsyndromic sensorineural hearing loss.

    PubMed

    Jiang, Hua; Chen, Jia; Li, Ying; Lin, Peng-Fang; He, Jian-Guo; Yang, Bei-Bei

    2016-01-01

    Several mitochondrial DNA mutations have been reported to be associated with nonsyndromic hearing loss in several families. However, little is known about the prevalence of these mutations in sporadic patients with nonsyndromic sensorineural hearing loss. The purpose of our study was to investigate the incidence of these mitochondrial DNA mutations in such population. A total of 178 sporadic patients with nonsyndromic sensorineural hearing loss were enrolled in this study. Genomic DNA was extracted from the peripheral blood sample. We employed the SNaPshot(®) sequencing method to detect five mitochondrial DNA mutations, including A1555G and A827G in 12S rRNA gene and A7445G, 7472insC, and T7511C in tRNA(Ser(UCN)) gene. Meanwhile, we used polymerase chain reaction and sequenced the products to screen GJB2 gene mutations in patients carrying mitochondrial DNA mutations. We failed to detect the presence of A1555G mutation in 12S rRNA gene, and of A7445G, 7472insC, T7511C mutations in tRNA(Ser(UCN)) gene in our population. However, we found that 6 patients (3.37%) were carriers of a homozygous A827G mutation and one of them also carried homozygous GJB2 235delC mutation. Our findings in the present study indicate that even in sporadic patients with nonsyndromic sensorineural hearing loss, mitochondrial DNA mutations might also contribute to the clinical phenotype. Copyright © 2015 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  1. 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

  2. Genetic Counseling in Hearing Impairment.

    ERIC Educational Resources Information Center

    Fraser, George R.

    1979-01-01

    The problem of counseling is dealt with mainly in the context of severe hearing impairment, since moderate forms are often due to illness or other nongenetic factors and do not constitute a grave handicap. (DLS)

  3. Association of TMTC2 With Human Nonsyndromic Sensorineural Hearing Loss

    PubMed Central

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

    2016-01-01

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

  4. A dominant variant in DMXL2 is linked to nonsyndromic hearing loss.

    PubMed

    Chen, Dong-Ye; Liu, Xing-Feng; Lin, Xiao-Jiang; Zhang, Dan; Chai, Yong-Chuan; Yu, De-Hong; Sun, Chang-Ling; Wang, Xue-Ling; Zhu, Wei-Dong; Chen, Ying; Sun, Lian-Hua; Wang, Xiao-Wen; Shi, Fu-Xin; Huang, Zhi-Wu; Yang, Tao; Wu, Hao

    2017-05-01

    To explore the genetic etiology of deafness in a dominant family with late-onset, progressive, nonsyndromic hearing loss. Genome-wide linkage analysis was performed for 21 family members. Candidate pathogenic variants were identified by whole-exome sequencing of selected family members and confirmed by Sanger sequencing of all family members. Cochlear expression of Dmxl2 was investigated by reverse-transcription polymerase chain reaction (RT-PCR) and immunostaining of the organ of Corti from mice. The causative gene was mapped to a 9.68-Mb candidate region on chromosome 15q21.2 (maximum logarithm of the odds score = 4.03) that contained no previously described deafness genes. Whole-exome sequencing identified heterozygous c.7250G>A (p.Arg2417His) in DMXL2 as the only candidate pathogenic variant segregating the hearing loss. In mouse cochlea, expression of DMXL2 was restricted to the hair cells and the spiral ganglion neurons. Our data indicated that the p.Arg2417His variant in DMXL2 is associated with dominant, nonsyndromic hearing loss and suggested an important role of DMXL2 in inner ear function.Genet Med advance online publication 22 September 2016.

  5. Noncoding Mutations of HGF Are Associated with Nonsyndromic Hearing Loss, DFNB39

    PubMed Central

    Schultz, Julie M.; Khan, Shaheen N.; Ahmed, Zubair M.; Riazuddin, Saima; Waryah, Ali M.; Chhatre, Dhananjay; Starost, Matthew F.; Ploplis, Barbara; Buckley, Stephanie; Velásquez, David; Kabra, Madhulika; Lee, Kwanghyuk; Hassan, Muhammad J.; Ali, Ghazanfar; Ansar, Muhammad; Ghosh, Manju; Wilcox, Edward R.; Ahmad, Wasim; Merlino, Glenn; Leal, Suzanne M.; Riazuddin, Sheikh; Friedman, Thomas B.; Morell, Robert J.

    2009-01-01

    A gene causing autosomal-recessive, nonsyndromic hearing loss, DFNB39, was previously mapped to an 18 Mb interval on chromosome 7q11.22-q21.12. We mapped an additional 40 consanguineous families segregating nonsyndromic hearing loss to the DFNB39 locus and refined the obligate interval to 1.2 Mb. The coding regions of all genes in this interval were sequenced, and no missense, nonsense, or frameshift mutations were found. We sequenced the noncoding sequences of genes, as well as noncoding genes, and found three mutations clustered in intron 4 and exon 5 in the hepatocyte growth factor gene (HGF). Two intron 4 deletions occur in a highly conserved sequence that is part of the 3′ untranslated region of a previously undescribed short isoform of HGF. The third mutation is a silent substitution, and we demonstrate that it affects splicing in vitro. HGF is involved in a wide variety of signaling pathways in many different tissues, yet these putative regulatory mutations cause a surprisingly specific phenotype, which is nonsydromic hearing loss. Two mouse models of Hgf dysregulation, one in which an Hgf transgene is ubiquitously overexpressed and the other a conditional knockout that deletes Hgf from a limited number of tissues, including the cochlea, result in deafness. Overexpression of HGF is associated with progressive degeneration of outer hair cells in the cochlea, whereas cochlear deletion of Hgf is associated with more general dysplasia. PMID:19576567

  6. 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.

  7. Teaching Students with Hearing Impairments.

    ERIC Educational Resources Information Center

    Kalivoda, Karen S.; And Others

    1997-01-01

    Reviews the needs of students with hearing impairments, focusing on Americans with Disabilities Act guidelines and the role of developmental education faculty in meeting these needs. Discusses the use of speech reading, assistive listening devices, and sign language interpreters to enhance oral communication; the use of written communication; and…

  8. 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.

  9. 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.

  10. 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

  11. 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

  12. 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

  13. Learning Aids for the Hearing Impaired Child.

    ERIC Educational Resources Information Center

    National Learning Resource Center of Pennsylvania, King of Prussia.

    Intended for parents, the booklet provides a practical guide to the types of learning aids that are helpful to the hearing impaired child. Sections cover the following: an explanation of residual hearing; types of hearing aids and hearing aid equipment; language development aids (brief descriptions are provided for materials in beginning language,…

  14. 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…

  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. Media Development Project for the Hearing Impaired.

    ERIC Educational Resources Information Center

    Stepp, Robert E.; And Others

    1978-01-01

    The Media Development Project for the Hearing Impaired is a project to serve the educational technology needs of the deaf and hard of hearing population, by identifying, adapting, developing, and evaluating the effectiveness of instructional media and materials. (BD)

  17. Association between mutations in the gap junction β4 gene and nonsyndromic hearing loss: genotype-phenotype correlation patterns.

    PubMed

    Li, Tung-Cheng; Wang, Wen-Hung; Li, Chuan; Yang, Jiann-Jou

    2015-01-01

    Numerous studies have confirmed that gap junctions, composed of connexin (Cx) protein, are essential for auditory function. However, few studies have investigated the correlation between variants in the gap junction β4 (GJB4) gene and phenotype in patients with nonsyndromic hearing loss. Our previous study identified 11 patients with GJB4 gene variants in 253 unrelated patients with nonsyndromic hearing loss. In the present study, the phenotype-genotype correlation was examined in the 11 deaf patients with the different variants of GJB4. Analytical results revealed that the majority of probands had congenital hearing loss, which was bilateral, stable and without associated dermatological manifestations or morphological changes of the inner ear. An audiometric profile, including the observed consistency with severe-profound and flat shape dominance, may enable screening for variants of GJB4. On the basis of the above results, it was hypothesized that GJB4 may be a genetic risk factor for the development of nonsyndromic hearing loss and the data from the present study can be used to direct the clinical evaluation and effectively manage the care of families of children with GJB4.

  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. Voice Cues in Hearing and Hearing Impaired Hebrew Speaking Children.

    ERIC Educational Resources Information Center

    Rosenhouse, Judith; Gelinas-Chebat, Claire

    The difficulties and differences of the Hebrew speech of nine children (ages 7 through 11) with severe hearing impairments were compared with the speech of seven children (ages 4-8) without hearing impairments. Each child was asked to name the objects in 20 colored pictures. From the 20 objects, words were selected which included the five main…

  20. [Correlation between degree of mitochondrial DNA 1555 mutation and clinical phenotype of nonsyndromic hearing loss].

    PubMed

    Cheng, Zu-jian; Zhang, Rong; Yang, Bin; Liu, Qi-cai; Jiang, Ling; Chen, Jing; Chen, Yong; Ou, Qi-shui

    2009-09-29

    To study the correlation between the number of mtDNA (mitochondrial DNA) copies containing mtDNA A1555G mutation site and phenotype and further elucidate the molecular genetic basis of phenotype diversity of nonsyndromic hearing loss. Real time-amplification refractory mutation system-quantitative PCR was employed to detect the number of mtDNA copies in mild type and mutant type of mtDNA 1555. In the sporadic group, there was no significant correlation between mtDNA A1555G homogeneity mutation copies and phenotype (R = 0.001, P = 0.997) while significant correlation existed between mtDNA A1555G heteroplasmic mutations and phenotype (R = 0.771, P = 0.003). In the familial group there was significant correlation mtDNA 1555 homogeneity mutation copies and phenotype (R = 0.341, P = 0.022) and significant correlation existed between mtDNA 1555 heterogenicity mutation copies and phenotype (R = 0.85, P = 0.015). There is significant correlation between the mtDNA A1555G mutation copies and the severity of hearing loss.

  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

    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.

  3. 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

  4. 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

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

    PubMed

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

    2015-10-10

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

  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. Apoptosis in acquired and genetic hearing impairment

    PubMed Central

    de Beeck, Ken Op; Schacht, Jochen; Van Camp, Guy

    2012-01-01

    Apoptosis is an important physiological process. Normally, a healthy cell maintains a delicate balance between pro- and anti-apoptotic factors, allowing it to live and proliferate. It is thus not surprising that disturbance of this delicate balance may result in disease. It is a well known fact that apoptosis also contributes to several acquired forms of hearing impairment. Noise-induced hearing loss is the result of prolonged exposure to excessive noise, triggering apoptosis in terminally differentiated sensory hair cells. Moreover, hearing loss caused by the use of therapeutic drugs such as aminoglycoside antibiotics and cisplatin potentially may result in the activation of apoptosis in sensory hair cells leading to hearing loss due to the “ototoxicity” of the drugs. Finally, apoptosis is a key contributor to the development of presbycusis, age-related hearing loss. Recently, several mutations in apoptosis genes were identified as the cause of monogenic hearing impairment. These genes are TJP2, DFNA5 and MSRB3. This implies that apoptosis not only contributes to the pathology of acquired forms of hearing impairment, but also to genetic hearing impairment as well. We believe that these genes constitute a new functional class within the hearing loss field. Here, the contribution of apoptosis in the pathology of both acquired and genetic hearing impairment is reviewed. PMID:21782914

  8. 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.

  9. [Diagnosing hearing impairment in children].

    PubMed

    Hempel, John-Martin

    2006-05-11

    Failure to recognize the presence of a hearing impairment in early childhood can severely harm the development of the child. A differentiation is made between sound conduction deafness and sensorineural.deafness. The former may be temporary or permanent. The latter may be inherited or acquired. Inherited sensorineural deafness occurs within the framework of syndromes, or may be monosymptomatic. Acquired sensorineural deafness may occur prenatally, perinatally or postnatally. A range of subjective and objective audiological tests is available, which, depending on the age and state of developmental of the child, can be used in combination. Cases of syndromal deafness are investigated on an interdisciplinary basis and involve the pediatrician, geneticist, laboratory physician and radiologist.

  10. 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.

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

    SciTech Connect

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

    2006-04-14

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

  12. Hearing outcome of infants with congenital cytomegalovirus and hearing impairment.

    PubMed

    Bilavsky, Efraim; Shahar-Nissan, Keren; Pardo, Joseph; Attias, Joseph; Amir, Jacob

    2016-05-01

    Congenital cytomegalovirus (cCMV) is the most common non-genetic cause of childhood sensorineural hearing loss. Antiviral treatment has been shown to prevent hearing deterioration in these infants. However, studies focused on infants with hearing impairment at birth and on the specific degree of impairment and further improvement or deterioration are lacking. To investigate the relationship between hearing status at birth and any change in hearing status at the end of a prolonged follow-up period, after receiving 12 months of antiviral treatment in children born with hearing impairment due to congenital cCMV. Clinical, laboratory, radiological and audiological data of all infants with cCMV infection followed in our centre between 2005 and 2013 were reviewed. Treatment with antiviral medication for hearing impairment found during the neonatal period was12 months of gan/valganciclovir. Hearing studies were performed only on infants who had been followed up for more than 1 year after treatment. Hearing impairment at birth was found in 54 (36.2%) of the 149 infants diagnosed with symptomatic cCMV, and found in 77 affected ears; unilateral in 31 (57.4%) and bilateral in 23 (42.6%). After 1 year of antiviral treatment and a long-term follow-up of the 77 affected ears at baseline, 50 (64.9%) had improved, 22 (28.6%) remained unchanged and 5 (6.5%) had deteriorated. Most improved ears (38/50=76%) returned to normal hearing. Improvement was most likely to occur in infants born with mild or moderate hearing loss and less in those with severe impairment. We found that infants born with cCMV and hearing impairment, receiving 12 months of antiviral treatment, showed significant improvement in hearing status. The probability of hearing improvement seems inversely related to the severity of the impairment at birth. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

  13. 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.

  14. 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

  15. 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)

  16. 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.…

  17. 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).

  18. 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.

  19. Hearing dummies: individualized computer models of hearing impairment.

    PubMed

    Panda, Manasa R; Lecluyse, Wendy; Tan, Christine M; Jürgens, Tim; Meddis, Ray

    2014-10-01

    Objective: Our aim was to explore the usage of individualized computer models to simulate hearing loss based on detailed psychophysical assessment and to offer hypothetical diagnoses of the underlying pathology. Individualized computer models of normal and impaired hearing were constructed and evaluated using the psychophysical data obtained from human listeners. Computer models of impaired hearing were generated to reflect the hypothesized underlying pathology (e.g. dead regions, outer hair cell dysfunction, or reductions in endocochlear potential). These models were evaluated in terms of their ability to replicate the original patient data. Auditory profiles were measured for two normal and five hearing-impaired listeners using a battery of three psychophysical tests (absolute thresholds, frequency selectivity, and compression). The individualized computer models were found to match the data. Useful fits to the impaired profiles could be obtained by changing only a single parameter in the model of normal hearing. Sometimes, however, it was necessary to include an additional dead region. The creation of individualized computer models of hearing loss can be used to simulate auditory profiles of impaired listeners and suggest hypotheses concerning the underlying peripheral pathology.

  20. 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

  1. Subclinical Congenital Cytomegalovirus Infection and Hearing Impairment

    ERIC Educational Resources Information Center

    Dahle, Arthur J.; And Others

    1974-01-01

    When the hearing sensitivity of children with subclinical congenital cytomegalovirus infection was evaluated and compared with that of a group of matched control subjects, nine of the 18 infected subjects were found to have some hearing loss, ranging from slight high-frequency impairments to a severe-to-profound unilateral loss. (MYS)

  2. 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…

  3. Hearing Impairment in Congenitally Hypothyroid Patients

    PubMed Central

    Hashemipour, Mahin; Hovsepian, Silva; Hashemi, Mostafa; Amini, Massoud; Kelishadi, Roya; Sadeghi, Somaye

    2012-01-01

    Objective Thyroid hormone is necessary for normal development of the auditory system. The aim of this study was to investigate the rate of hearing impairment in congenitally hypothyroid (CH) patients, and its relation with factors such as CH severity and age at starting treatment, during CH screening program in Isfahan. Methods Hearing acuity was assessed in two groups of children with (94 patients aged 4 months – 3 years) and without CH (450), between 2000-2006. Otoacostic emission (OAE) was performed by a two step method. After two tests without OAE signals bilaterally, they were referred for auditory brainstem response (ABR) test. Subjects with both OAE and ABR abnormal test results were considered to have hearing problem. Obtained data was compared in case and control group and also CH patients with and without hearing impairment. Findings Three (3.2%) of patients and 1 of control group (0.2%) were diagnosed with sensorineural hearing loss. The rate of hearing loss was not different significantly in two studied groups (P>0.05). There was no difference between age of starting treatment and first T4 and TSH level in CH patients with and without hearing loss (P>0.05). CH neonates with hearing impairment had thyroid dyshormonogenesis according to the follow up results. Conclusion The rate of hearing loss was low among our studied CH patients. It may be due to proper management of CH patients. In view of the fact that all CH neonates were dyshormonogentic and considering the relation between certain gene mutations and hearing impairment in CH patients, further studies with larger sample size, with regard to different etiologies of CH should be investigated to indicate the possible gene mutations related to hearing loss in CH. PMID:23056865

  4. 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…

  5. 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.

  6. 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.

  7. Single Nucleotide Polymorphisms of the GJB2 and GJB6 Genes Are Associated with Autosomal Recessive Nonsyndromic Hearing Loss

    PubMed Central

    Grillo, Ana Paula; de Oliveira, Flávia Marcorin; de Carvalho, Gabriela Queila; Medrano, Ruan Felipe Vieira; da Silva-Costa, Sueli Matilde; Sartorato, Edi Lúcia; de Oliveira, Camila Andréa

    2015-01-01

    Single nucleotide polymorphisms (SNPs) are important markers in many studies that link DNA sequence variations to phenotypic changes; such studies are expected to advance the understanding of human physiology and elucidate the molecular basis of diseases. The DFNB1 locus, which contains the GJB2 and GJB6 genes, plays a key role in nonsyndromic hearing loss. Previous studies have identified important mutations in this locus, but the contribution of SNPs in the genes has not yet been much investigated. The aim of this study was to investigate the association of nine polymorphisms located within the DFNB1 locus with the occurrence of autosomal recessive nonsyndromic hearing loss (ARNSHL). The SNPs rs3751385 (C/T), rs7994748 (C/T), rs7329857 (C/T), rs7987302 (G/A), rs7322538 (G/A), rs9315400 (C/T), rs877098 (C/T), rs945369 (A/C), and rs7333214 (T/G) were genotyped in 122 deaf patients and 132 healthy controls using allele-specific PCR. There were statistically significant differences between patients and controls, in terms of allelic frequencies in the SNPs rs3751385, rs7994748, rs7329857, rs7987302, rs945369, and rs7333214 (P < 0.05). No significant differences between the two groups were observed for rs7322538, rs9315400, and rs877098. Our results suggest that SNPs present in the GJB2 and GJB6 genes may have an influence on ARNSHL in humans. PMID:26075227

  8. 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.

  9. 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.

  10. Demographic Aspects of Hearing Impairment: Questions and Answers.

    ERIC Educational Resources Information Center

    Hotchkiss, David

    This pamphlet answers many questions about demographic aspects of hearing-impaired individuals, such as the number of hearing-impaired and deaf persons in the United States, the number with hearing problems that restrict communication, the number of hearing-impaired students and number of deaf college students, the demographic groups which have a…

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

    PubMed

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

    2008-12-01

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

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

    PubMed

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

    2015-01-01

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

  13. 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. Progressive Hearing Impairment in Children with Congenital Cytomegalovirus Infection.

    ERIC Educational Resources Information Center

    Dahle, Arthur J.; And Others

    1979-01-01

    Audiological assessment of 86 children (mean age 38 months at last evaluation time) with congenital cytomegalovirus infection revealed progressive hearing loss in four of 12 Ss with sensorineural hearing impairments. Case descriptions documented the progression of the hearing loss. (Author)

  15. 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,...

  16. 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

  17. 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.

  18. 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.…

  19. 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)

  20. 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…

  1. 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…

  2. 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.…

  3. 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.…

  4. 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…

  5. Music Instruction and the Hearing Impaired.

    ERIC Educational Resources Information Center

    Walczyk, Eugenia Bulawa

    1993-01-01

    Contends music education can help hearing-impaired students achieve both academic and social goals of Individuals with Disabilities Education Act (1990). Describes instructional content and methods of elementary program utilizing sign language, vocal music, and keyboard computer music systems. Concludes with description of school performance in…

  6. 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.…

  7. 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…

  8. Prelinguistic Vocalizations of Hearing-Impaired and Normally Hearing Subjects: A Comparison of Consonantal Inventories.

    ERIC Educational Resources Information Center

    Stoel-Gammon, Carol

    1988-01-01

    Babbling samples from 11 normally hearing infants and 14 hearing-impaired (HI) subjects, aged 4-39 months, were analyzed. Differences were observed between consonantal phone inventories of hearing-impaired subjects and hearing infants, between inventories of HI subjects with sensori-neural loss and those with conductive loss, and between younger…

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

    PubMed

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

    2005-09-01

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

  10. Hair phenotype in non-syndromic deafness.

    PubMed

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

    2013-08-01

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

  11. Linking hearing impairment, employment and education.

    PubMed

    Garramiola-Bilbao, I; Rodríguez-Álvarez, A

    2016-12-01

    To analyse the impact that hearing impairment and other relevant variables have on the education and employment situation of those affected by it in the Principality of Asturias, Spain. To achieve this objective, two discrete choice models (probit) are presented. The first one associates, among other variables, hearing impairment with the individual's employment status and in the second model, an ordered multinomial probit model is used to analyse, among other variables, how the impairment affects the individual's level of studies. Although the levels of statistical significance are low, the model's estimates appear to indicate that hearing impairment in Spain increases the probability of being unemployed by 18.4% (P = 0.09). Additionally, the people suffering from such a disability are, compared with the rest of the population, 10.2% (P = 0.05) more likely to have only completed elementary studies without pursuing any further education. If an individual is able to reach a level of secondary or higher education thus enabling a future incorporation to the work place, a benefit is obviously generated for both the individual as well as society (which has additionally incurred an investment in human capital). In this regard, encouraging the education of hearing-impaired students would profit both the individual (who receives an early integration as a child), which may contribute positively to family and social factors, as well as society who have incurred the investment. Therefore, our result could indicate that programmes created to support individuals with this type of disability represent an increase of welfare both individually and socially. Copyright © 2016 The Royal Society for Public Health. Published by Elsevier Ltd. All rights reserved.

  12. 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.

  13. 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

  14. 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.

  15. Intervention for Children With Hearing Impairment in General Education Settings.

    PubMed

    Brackett, Diane

    1997-10-01

    Students with hearing impairments who are educated in classes with normally hearings peers represent the majority of children with hearing impairment. With hearing losses ranging from mild to profound, they require services to optimize their use of residual hearing and reduce the secondary effects of hearing loss, such as communication deficits and academic delays. For most of these students, the speech-language pathologist will be the on-site specialist in hearing responsible for designing a collaborative rehabilitation/education plan that addresses all deficit areas and for assisting in its implementation in the classroom.

  16. 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.

  17. The Concept of Fractional Number among Hearing-Impaired Students.

    ERIC Educational Resources Information Center

    Titus, Janet C.

    This study investigated hearing-impaired students' understanding of the mathematical concept of fractional numbers, as measured by their ability to determine the order and equivalence of fractional numbers. Twenty-one students (ages 10-16) with hearing impairments were compared with 26 students with normal hearing. The study concluded that…

  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. 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…

  20. 38 CFR 4.85 - Evaluation of hearing impairment.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 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 percentage...

  1. 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...

  2. 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...

  3. Using Bilingual Instructional Models in Teaching Hearing-Impaired Students.

    ERIC Educational Resources Information Center

    Luetke-Stahlman, Barbara

    1983-01-01

    Four basic models used to classify bilingual programs for hearing students are explained briefly, and advantages and disadvantages for use of the models with hearing-impaired minority-language-speaking students are provided. (Author)

  4. 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.

  5. Relationships among Speech Perception, Production, Language, Hearing Loss, and Age in Children with Impaired Hearing.

    ERIC Educational Resources Information Center

    Blamey, Peter J.; Sarant, Julia Z.; Paatsch, Louise E.; Barry, Johanna G.; Bow, Catherine P.; Wales, Roger J.; Wright, Maree; Psarros, Colleen; Rattigan, Kylie; Tooher, Rebecca

    2001-01-01

    Forty seven children with severely impaired hearing, fitted with a cochlear implant, and 40 children with moderately impaired hearing, fitted with hearing aids, were evaluated using speech perception, production, and language measures over a 3-year period. Few differences were found between the groups, suggesting the implant improves hearing…

  6. Adenylate cyclase 1 (ADCY1) mutations cause recessive hearing impairment in humans and defects in hair cell function and hearing in zebrafish

    PubMed Central

    Santos-Cortez, Regie Lyn P.; Lee, Kwanghyuk; Giese, Arnaud P.; Ansar, Muhammad; Amin-Ud-Din, Muhammad; Rehn, Kira; Wang, Xin; Aziz, Abdul; Chiu, Ilene; Hussain Ali, Raja; Smith, Joshua D.; Shendure, Jay; Bamshad, Michael; Nickerson, Deborah A.; Ahmed, Zubair M.; Ahmad, Wasim; Riazuddin, Saima; Leal, Suzanne M.

    2014-01-01

    Cyclic AMP (cAMP) production, which is important for mechanotransduction within the inner ear, is catalyzed by adenylate cyclases (AC). However, knowledge of the role of ACs in hearing is limited. Previously, a novel autosomal recessive non-syndromic hearing impairment locus DFNB44 was mapped to chromosome 7p14.1-q11.22 in a consanguineous family from Pakistan. Through whole-exome sequencing of DNA samples from hearing-impaired family members, a nonsense mutation c.3112C>T (p.Arg1038*) within adenylate cyclase 1 (ADCY1) was identified. This stop-gained mutation segregated with hearing impairment within the family and was not identified in ethnically matched controls or within variant databases. This mutation is predicted to cause the loss of 82 amino acids from the carboxyl tail, including highly conserved residues within the catalytic domain, plus a calmodulin-stimulation defect, both of which are expected to decrease enzymatic efficiency. Individuals who are homozygous for this mutation had symmetric, mild-to-moderate mixed hearing impairment. Zebrafish adcy1b morphants had no FM1-43 dye uptake and lacked startle response, indicating hair cell dysfunction and gross hearing impairment. In the mouse, Adcy1 expression was observed throughout inner ear development and maturation. ADCY1 was localized to the cytoplasm of supporting cells and hair cells of the cochlea and vestibule and also to cochlear hair cell nuclei and stereocilia. Ex vivo studies in COS-7 cells suggest that the carboxyl tail of ADCY1 is essential for localization to actin-based microvilli. These results demonstrate that ADCY1 has an evolutionarily conserved role in hearing and that cAMP signaling is important to hair cell function within the inner ear. PMID:24482543

  7. 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.

  8. 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.

  9. 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

  10. 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.

  11. 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.

  12. 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.

  13. Auditory Compensation for Hearing Impaired Infants and Children in Japan.

    ERIC Educational Resources Information Center

    Sato, Masayuki D. S.; Onuma, Nakoi

    1996-01-01

    Gives an overview of the present school system in Japan, focusing on early intervention for hearing impairments, auditory compensation devices, and instruction in auditory training. Suggests that recent developments in hearing aid technology have resulted in substantial improvements in the identification and rehabilitation of hearing-impaired…

  14. 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.

  15. 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…

  16. 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.

  17. A nonsynonymous mutation in the WFS1 gene in a Finnish family with age-related hearing impairment.

    PubMed

    Kytövuori, Laura; Hannula, Samuli; Mäki-Torkko, Elina; Sorri, Martti; Majamaa, Kari

    2017-09-28

    Wolfram syndrome (WS) is caused by recessive mutations in the Wolfram syndrome 1 (WFS1) gene. Sensorineural hearing impairment (HI) is a frequent feature in WS and, furthermore, certain mutations in WFS1 cause nonsyndromic dominantly inherited low-frequency sensorineural HI. These two phenotypes are clinically distinct indicating that WFS1 is a reasonable candidate for genetic studies in patients with other phenotypes of HI. Here we have investigated, whether the variation in WFS1 has a pathogenic role in age-related hearing impairment (ARHI). WFS1 gene was investigated in a population sample of 518 Finnish adults born in 1938-1949 and representing variable hearing phenotypes. Identified variants were evaluated with respect to pathogenic potential. A rare mutation predicted to be pathogenic was found in a family with many members with impaired hearing. Twenty members were recruited to a segregation study and a detailed clinical examination. Heterozygous p.Tyr528His variant segregated completely with late-onset HI in which hearing deteriorated first at high frequencies and progressed to mid and low frequencies later in life. We report the first mutation in the WFS1 gene causing late-onset HI with audiogram configurations typical for ARHI. Monogenic forms of ARHI are rare and our results add WFS1 to the short list of such genes. Copyright © 2017 Elsevier B.V. All rights reserved.

  18. Problems of hearing impaired children and suggested solutions.

    PubMed

    Prasad, B

    1992-01-01

    Factors related to development of verbal skills in hearing-impaired children include early diagnosis, early age of obtaining hearing aids, and early intervention. The development of speech occurs in the first 2 years of one's life. In addition to the hearing aid the child should also get early stimulation and training by parents and professionals toward developing speech and language. The hearing-impaired child is likely to get the benefit of a hearing aid only if it amplifies sound in accordance with his or her hearing loss. Proper audiological examination assesses the level of hearing loss and helps select a suitable hearing aid by conducting an aided audiogram. Parents who accept their child's problem are in a better emotional state to assist their child. The relationship between the degree of hearing impairment and development of verbal skills is analyzed by performance on the verbal section of the Weschler Intelligence Scale for Children (WISC). Analysis of WISC results showed that in a group of 24 children with severe to profound hearing loss only 12% were able to answer questions on the verbal section of WISC. These children were attending normal schools and had developed almost normal speech and language with high parental involvement. In a group of 16 children with moderate and conductive hearing loss 80% were able to deal with the verbal section. Hearing-impaired children who attend school have better prospects of receiving structured stimulation for language development. There are only 2 special schools available for 3-5 year old children in Delhi. Services could be improved by spelling out in the educational policy of training programs for teachers of hearing-impaired children that 1) the method of teaching must be decided on the basis of the hearing-impaired child's capabilities and 2) teachers must conduct proper auditory stimulation programs.

  19. 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…

  20. 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…

  1. 38 CFR 3.385 - Disability due to impaired hearing.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2010-07-01 2010-07-01 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 the...

  2. 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…

  3. 38 CFR 4.86 - Exceptional patterns of hearing impairment.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2010-07-01 2010-07-01 false Exceptional patterns of hearing impairment. 4.86 Section 4.86 Pensions, Bonuses, and Veterans' Relief DEPARTMENT OF VETERANS... Exceptional patterns of hearing impairment. (a) When the puretone threshold at each of the four specified...

  4. An Optimistic Look at Literacy and Hearing Impairment.

    ERIC Educational Resources Information Center

    Luetke-Stahlman, Barbara

    1982-01-01

    Discusses learning to read by hearing impaired children and maintains that given a language base (in sign alone, oral and/or signed English), a total communication environment, and the opportunity to utilize various modes to decode written English, it appears likely that hearing impaired children can develop reading and writing skills in English.…

  5. Project LITERACY-HI: Hypermedia for Readers with Hearing Impairments.

    ERIC Educational Resources Information Center

    Horney, Mark; And Others

    This paper describes Project LITERACY-HI, an ongoing 3-year federally funded study of the benefits of electronically enhanced text for mainstreamed students with hearing impairments. Preliminary information documents the ongoing difficulties with reading experienced by most students with hearing impairments. The project is creating electronic…

  6. 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…

  7. 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…

  8. 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...

  9. 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...

  10. 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...

  11. 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...

  12. 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.

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

    PubMed Central

    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. PMID:25426086

  14. 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.

  15. Mitochondrial 12S rRNA variants in 1642 Han Chinese pediatric subjects with aminoglycoside-induced and nonsyndromic hearing loss

    PubMed Central

    Lu, Jianxin; Li, Zhiyuan; Zhu, Yi; Yang, Aifen; Li, Ronghua; Zheng, Jing; Cai, Qin; Peng, Guanghua; Zheng, Wuwei; Tang, Xiaowen; Chen, Bobei; Chen, Jianfu; Liao, Zhisu; Yang, Li; Li, Yongyan; You, Junyan; Ding, Yu; Yu, Hong; Wang, Jindan; Sun, Dongmei; Zhao, Jianyue; Xue, Ling; Wang, Jieying; Guan, Min-Xin

    2010-01-01

    In this report, we investigated the frequency and spectrum of mitochondrial 12S rRNA variants in a large cohort of 1642 Han Chinese pediatric subjects with aminoglycoside-induced and nonsyndromic hearing loss. Mutational analysis of 12S rRNA gene in these subjects identified 68 (54 known and 14 novel) variants. The frequencies of known 1555A>G and 1494C>T mutations were 3.96% and 0.18%, respectively, in this cohort with nonsyndromic and aminoglycoside-induced hearing loss. Prevalence of other putative deafness-associated mutation at positions 1095 and 961 were 0.61% and 1.7% in this cohort, respectively. Furthermore, the 745A>G, 792C>T, 801A>G, 839A>G, 856A>G, 1027A>G, 1192C>T, 1192C>A, 1310C>T, 1331A>G, 1374A>G and 1452T>C variants conferred increased sensitivity to ototoxic drugs or nonsyndromic deafness as they were absent in 449 Chinese controls and localized at highly conserved nucleotides of this rRNA. However, other variants appeared to be polymorphisms. Moreover, 65 Chinese subjects carrying the 1555A>G mutation exhibited bilateral and sensorineural hearing loss. A wide range of severity, age-of-onset and audiometric configuration was observed among these subjects. In particular, the sloping and flat shaped patterns were the common audiograms in individuals carrying the 1555A>G mutation. The phenotypic variability in subjects carrying these 12S rRNA mutations indicated the involvement of nuclear modifier genes, mitochondrial haplotypes, epigenetic and environmental factors in the phenotypic manifestation of these mutations. Therefore, our data demonstrated that mitochondrial 12S rRNA is the hot spot for mutations associated with aminoglycoside ototoxicity. PMID:20100600

  16. Development of cognitive screening test for the severely hearing impaired: Hearing-impaired MoCA.

    PubMed

    Lin, Vincent Y W; Chung, Janet; Callahan, Brandy L; Smith, Leah; Gritters, Nils; Chen, Joseph M; Black, Sandra E; Masellis, Mario

    2017-05-01

    To develop a version of the Montreal Cognitive Assessment (MoCA) to be administered to the severely hearing impaired (HI-MoCA), and to assess its performance in two groups of cognitively intact adults over the age of 60. Test development followed by prospective subject recruitment. The MoCA was converted into a timed PowerPoint (Microsoft Corp., Redmond, WA) presentation, and verbal instructions were converted into visual instructions. Two groups of subjects over the age of 60 were recruited. All subjects passed screening questionnaires to eliminate those with undiagnosed mild cognitive impairment. The first group had normal hearing (group 1). The second group was severely hearing impaired (group 2). Group 1 received either the MoCA or HI-MoCA test (T1). Six months later (T2), subjects were administered the test (MoCA or HI-MoCA) they had not received previously to determine equivalency. Group 2 received the HI-MoCA at T1 and again at T2 to determine test-retest reliability. One hundred and three subjects were recruited into group 1, with a score of 26.66 (HI-MoCA) versus 27.14 (MoCA). This was significant (P < 0.05), but scoring uses whole numerals and the 0.48 difference was found not clinically significant using post hoc sensitivity analyses. Forty-nine subjects were recruited into group 2. They scored 26.18 and 26.49 (HI-MoCA at T1 and T2). No significance was noted (P > 0.05), with a test-retest coefficient of 0.66. The HI-MoCA is easy to administer and reliable for screening cognitive impairment in the severely hearing impaired. No conversion factor is required in our prospectively tested cohort of cognitively intact subjects. 1b. Laryngoscope, 127:S4-S11, 2017. © 2017 The American Laryngological, Rhinological and Otological Society, Inc.

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

    PubMed

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

    2016-09-01

    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. 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. 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). 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.

  18. 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

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

    ERIC Educational Resources Information Center

    Gentile, Augustine; Rambin, J. Bentley

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

  20. 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

  1. 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.

  2. 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.

  3. 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.

  4. Summarization strategies of hearing-impaired and normally hearing college students.

    PubMed

    Peterson, L N; French, L

    1988-09-01

    The purpose of this study was to compare the summary writing skills of hearing-impaired and normally hearing college students. Summarization was defined in terms of the following measures: deletion of trivial text information, inclusion of most important ideas, selection of topic sentences, creation of topic statements, and integration of information within and among several paragraphs. Inclusion of opinionated, incorrect, and uninterpretable information was measured also. Thirty hearing-impaired and 30 normally hearing students read and summarized two expository science passages that were controlled for the number of topic (main idea) sentences and that had been rated previously for the importance of "idea units." Students' factual comprehension also was assessed. Hearing-impaired and normally hearing students exhibited a similar pattern of use among several measured summarization strategies, except for the inclusion of opinions or comments in their summaries. Hearing-impaired students were not as sensitive as normally hearing students to importance of ideas and used the following summarization strategies significantly less often: inclusion of important ideas, selection of topic sentences, creation of topic statements, and integration of ideas within and among paragraphs. The results indicated that hearing-impaired college students have basic summarization skills but do not apply summarization strategies as effectively as normally hearing students.

  5. Societal effects of hearing aid fitting among the moderately hearing impaired.

    PubMed

    Joore, Manuela A; Brunenberg, Danielle E M; Chenault, Michelene N; Anteunis, Lucien J C

    2003-04-01

    The objective of this study was to evaluate hearing aid fitting from a societal viewpoint, rather than the more traditional patient perspective. The effects of hearing aid fitting on generic quality of life (EuroQol), social functioning (SF36), auditory disability, productivity at paid and unpaid labour, and medical consumption, were assessed in a prospective study among 80 moderately hearing-impaired first-time hearing aid applicants. The study showed that hearing aid fitting solved problems with paid employment, but did not seem to affect unpaid work. Use of medical services remained relatively stable after first-time hearing aid fitting. The Amsterdam Inventory proved to be a useful questionnaire to assess the effects on hearing disability. No effects of hearing aid fitting on generic quality of life could be determined with the EuroQol, while hearing aid fitting did lead to an improvement in one aspect of generic quality of life; namely social functioning.

  6. Story retelling skills in Persian speaking hearing-impaired children.

    PubMed

    Jarollahi, Farnoush; Mohamadi, Reyhane; Modarresi, Yahya; Agharasouli, Zahra; Rahimzadeh, Shadi; Ahmadi, Tayebeh; Keyhani, Mohammad-Reza

    2017-05-01

    Since the pragmatic skills of hearing-impaired Persian-speaking children have not yet been investigated particularly through story retelling, this study aimed to evaluate some pragmatic abilities of normal-hearing and hearing-impaired children using a story retelling test. 15 normal-hearing and 15 profound hearing-impaired 7-year-old children were evaluated using the story retelling test with the content validity of 89%, construct validity of 85%, and reliability of 83%. Three macro structure criteria including topic maintenance, event sequencing, explicitness, and four macro structure criteria including referencing, conjunctive cohesion, syntax complexity, and utterance length were assessed. The test was performed with live voice in a quiet room where children were then asked to retell the story. The tasks of the children were recorded on a tape, transcribed, scored and analyzed. In the macro structure criteria, utterances of hearing-impaired students were less consistent, enough information was not given to listeners to have a full understanding of the subject, and the story events were less frequently expressed in a rational order than those of normal-hearing group (P < 0.0001). Regarding the macro structure criteria of the test, unlike the normal-hearing students who obtained high scores, hearing-impaired students failed to gain any scores on the items of this section. These results suggest that Hearing-impaired children were not able to use language as effectively as their hearing peers, and they utilized quite different pragmatic functions. Copyright © 2017 Elsevier B.V. All rights reserved.

  7. 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

  8. Validity of hearing impairment calculation methods for prediction of self-reported hearing handicap.

    PubMed

    John, Andrew B; Kreisman, Brian M; Pallett, Stephen

    2012-01-01

    Worker's compensation for hearing loss caused by occupational noise exposure is calculated by varying methods, from state to state within the United States (US), with many employing arithmetic formulas based on the pure-tone audiogram, to quantify hearing loss. Several assumptions unsupported or weakly supported by empirical data underlie these formulas. The present study evaluated the ability of various arithmetic hearing impairment calculations to predict a self-reported hearing handicap in a sample of presenting with sensorineural hearing loss. 204 adults (127 male, 77 female) ranging in age from 18 to 94 served as participants. The sample was selected to exclude patients who had been referred for hearing testing for a medicolegal examination or a hearing conservation appointment. A hearing handicap was measured by the Hearing Handicap Inventory for Adults/for the Elderly (HHIA/E). The covariance analysis of linear structural equations was used to assess the relative strength of correlation with the HHIA/E score among the six formulas and various forms of pure-tone average. The results revealed that all the hearing impairment calculations examined were significantly, but weakly, correlated with the self-reported hearing impairment scores. No significant differences among the predictive abilities of the impairment calculations were evident; however, the average binaural impairment assigned differed significantly among the six calculations examined. Individuals who demonstrated 0% impairment had significantly lower (i.e., better) HHIA/E scores compared to those with non-zero impairment for each formula. These results supported the idea that audiometric data provided an insufficient explanation for real-world hearing difficulties.

  9. 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

  10. General dental practitioners and hearing impairment.

    PubMed

    Messano, Giuseppe Alessio; Petti, Stefano

    2012-10-01

    Hearing impairment (HI) remains a problem among dentists Hearing loss at speech frequencies was recently reported among dentists and dental hygienists. This study aimed to investigate prevalence and factors associated with perceived HI among dentists. In 2009-2010, 100 general dental practitioners (GDPs) and 115 general (medical) practitioners (GPs) (mean ages, 43.7 and 44.4 years) from Rome (Italy), who commenced practice ≥ 10 years ago, were interviewed on a series of occupation- and recreation-related HI risk factors and on HI-associated symptoms (tinnitus, sensation of fullness, hypoacusis). Prevalence of presumptive HI (≥ 1 symptom perceived during workdays and weekends) was assessed and factors associated with presumptive HI were investigated. Prevalence was 30.0% (95% confidence interval, 21.0-39.0%) and 14.8% (95% confidence interval, 8.3-21.3%) among GDPs and GPs, respectively. Occupation (GDP vs. GP), family history of hypoacusis, hypertension, ear diseases and smoking were significantly associated with presumptive HI. Within GDPs alone, significant associations were found for frequent use of ultrasonic scalers, use of dental turbines aged≥1 year and prosthodontics as prevalent specialty. GDPs experienced HI risk than GPs. Such a risk was not generalized to all dentists, but was specific for those who frequently used noisy equipment (aged turbines, ultrasonic scalers) during their daily practice. GDPs with 10 or more years of practice who routinely use potentially noisy equipment, could be at risk of HI. In order to prevent such condition, daily maintenance and periodical replacement of dental instruments is recommended. Copyright © 2012 Elsevier Ltd. All rights reserved.

  11. 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

  12. 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.

  13. A novel KCNQ4 mutation and a private IMMP2L-DOCK4 duplication segregating with nonsyndromic hearing loss in a Brazilian family.

    PubMed

    Uehara, Daniela T; Freitas, Érika L; Alves, Leandro U; Mazzeu, Juliana F; Auricchio, Maria Tbm; Tabith, Alfredo; Monteiro, Mário Lr; Rosenberg, Carla; Mingroni-Netto, Regina C

    2015-01-01

    Here we describe a novel missense variant in the KCNQ4 gene and a private duplication at 7q31.1 partially involving two genes (IMMP2L and DOCK4). Both mutations segregated with nonsyndromic hearing loss in a family with three affected individuals. Initially, we identified the duplication in a screening of 132 unrelated cases of hearing loss with a multiplex ligation-dependent probe amplification panel of genes that are candidates to have a role in hearing, including IMMP2L. Mapping of the duplication by array-CGH revealed that the duplication also encompassed the 3'-end of DOCK4. Subsequently, whole-exome sequencing identified the breakpoint of the rearrangement, thereby confirming the existence of a fusion IMMP2L-DOCK4 gene. Transcription products of the fusion gene were identified, indicating that they escaped nonsense-mediated messenger RNA decay. A missense substitution (c.701A>T) in KCNQ4 (a gene at the DFNA2A locus) was also identified by whole-exome sequencing. Because the substitution is predicted to be probably damaging and KCNQ4 has been implicated in hearing loss, this mutation might explain the deafness in the affected individuals, although a hypothetical effect of the product of the fusion gene on hearing cannot be completely ruled out.

  14. A novel KCNQ4 mutation and a private IMMP2L-DOCK4 duplication segregating with nonsyndromic hearing loss in a Brazilian family

    PubMed Central

    Uehara, Daniela T; Freitas, Érika L; Alves, Leandro U; Mazzeu, Juliana F; Auricchio, Maria TBM; Tabith, Alfredo; Monteiro, Mário LR; Rosenberg, Carla; Mingroni-Netto, Regina C

    2015-01-01

    Here we describe a novel missense variant in the KCNQ4 gene and a private duplication at 7q31.1 partially involving two genes (IMMP2L and DOCK4). Both mutations segregated with nonsyndromic hearing loss in a family with three affected individuals. Initially, we identified the duplication in a screening of 132 unrelated cases of hearing loss with a multiplex ligation-dependent probe amplification panel of genes that are candidates to have a role in hearing, including IMMP2L. Mapping of the duplication by array-CGH revealed that the duplication also encompassed the 3′-end of DOCK4. Subsequently, whole-exome sequencing identified the breakpoint of the rearrangement, thereby confirming the existence of a fusion IMMP2L-DOCK4 gene. Transcription products of the fusion gene were identified, indicating that they escaped nonsense-mediated messenger RNA decay. A missense substitution (c.701A>T) in KCNQ4 (a gene at the DFNA2A locus) was also identified by whole-exome sequencing. Because the substitution is predicted to be probably damaging and KCNQ4 has been implicated in hearing loss, this mutation might explain the deafness in the affected individuals, although a hypothetical effect of the product of the fusion gene on hearing cannot be completely ruled out. PMID:27081546

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

    PubMed

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

    2015-01-01

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

  16. 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

  17. 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)

  18. Evaluation of hearing impairment in leprosy patients taking multidrug therapy.

    PubMed

    Rawlani, S; Patil, C Y; Bhowte, R; Degwekar, S; Rawlani, S; Chandak, R; Rawlani, S

    2013-01-01

    Present descriptive study was carried out for the assessment of hearing capability in leprosy patients. After getting approval from Institutional ethical committee, the present descriptive study was carried out on 60 subjects. All the patients were indoor-patients at the Leprosy Rehabilitation Center Maharogi Sewa Samiti Anandvan Warora, and were on multidrug therapy described by World Health Organization from an average period of 6 months. Study Group I consisted of 30 diagnosed Leprosy patients taking multidrug therapy from an average period of 6 months. Group II (Control group) consisted of 30 normal healthy individuals of same age. Patients suffering from acute or chronic ear discharge, Presence of wax in external auditory canal, diabetes mellitus, hypertension, impaired renal function and patients having history of trauma were excluded from the study. All the subjects underwent Pure tone audiometry, Tuning Fork test to check the level of hearing loss and type of hearing loss and detailed clinical examination for cranial nerve function was done in all the patients of study group and control group. Audiometry findings in study group patients showed that 23 patients (76.66%; 45 ears) of the leprosy patients had sensory neural hearing impairment and 7 patients (23.33%) showed normal hearing. Out of these affected patients, 10 patients (43.47%; 19 ears) had mild sensory neural hearing impairment, 10 patients (43.47%; 20 ears) had moderate sensory neural hearing impairment. 2 patients (8.69%; 04 ears) had moderate to severe hearing impairment, 1 patient (4.34%; 02 ears) showed severe sensory neural hearing impairment. In the absence of any local or systemic disease or drugs likely to have side effects on the cochleovestibular function, leprosy affects the cochleovestibular system, and effect on cochlear function is seen more often than effect on the vestibular system. Thus hearing loss which is seen in patients suffering from Hansen's disease is of cochlear origin.

  19. Auditory handicap of hearing impairment and the limited benefit of hearing aids.

    PubMed

    Plomp, R

    1978-02-01

    The aim of this article is to promote a better understanding of hearing impairment as a communicative handicap, primarily in noisy environments, and to explain by means of a quantitative model the essentially limited applicability of hearing aids. After data on the prevalence of hearing impairment and of auditory handicap have been reviewed, it is explained that every hearing loss for speech can be interpreted as the sum of a loss class A (attenuation), characterized by a reduction of the levels of both speech signal and noise, and a loss D (distortion), comparable with a decrease in speech-to-noise ratio. On the average, the hearing loss of class D (hearing loss in noise) appears to be about one-third (in decibels) of the total hearing loss (A + D, hearing loss in quiet). A hearing aid can compensate for class-A-hearing losses, giving difficulties primarily in quiet, but not for class-D hearing losses, giving difficulties primarily in noise. The latter class represents the first stage of auditory handicap, beginning at an average hearing loss of about 24 dB.

  20. Hearing Loss Severity: Impaired Processing of Formant Transition Duration

    ERIC Educational Resources Information Center

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

    2010-01-01

    Normal hearing listeners exploit the formant transition (FT) detection to identify place of articulation for stop consonants. Neuro-imaging studies revealed that short FT induced less cortical activation than long FT. To determine the ability of hearing impaired listeners to distinguish short and long formant transitions (FT) from vowels of the…

  1. 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…

  2. 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)

  3. 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…

  4. 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)

  5. Assessment of Auditory Speech Perception in Hearing-Impaired Infants.

    ERIC Educational Resources Information Center

    Salisbury, Jean E. T.

    The paper describes the use of an operant procedure to assess the speech perception of 11 young (7 to 35 months old) hearing impaired children and 11 normally hearing subjects. Subjects were presented with a repeating background stimulus and conditioned to turn their head on presentation of a contrasting syllable. The head-turning responses were…

  6. Overview of Psychological and Demographic Research with Hearing Impaired People.

    ERIC Educational Resources Information Center

    Trybus, Raymond J.

    A researcher involved with the study of deafness covers such topics as prevalence of deafness, educational placement options, and statistics on special education programs for the deaf and hard of hearing. Among findings of the Annual Survey of Hearing Impaired Children and Youth are that only 10% of special programs report genetic counseling for…

  7. 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)

  8. 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…

  9. 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)

  10. 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…

  11. 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…

  12. 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…

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

    PubMed

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

    2015-01-01

    To examine the association of cognitive function, age, and hearing loss with clinically assessed hearing aid benefit in older hearing-impaired persons. Hearing aid benefit was assessed using objective measures regarding speech recognition in quiet and noisy environments as well as a subjective measure reflecting everyday situations captured using a standardized questionnaire. A broad range of general cognitive functions such as attention, memory, and intelligence were determined using different neuropsychological tests. Linear regression analyses were conducted with the outcome of the neuropsychological tests as well as age and hearing loss as independent variables and the benefit measures as dependent variables. Thirty experienced older hearing aid users with typical age-related hearing impairment participated. Most of the benefit measures revealed that the participants obtained significant improvement with their hearing aids. Regression models showed a significant relationship between a fluid intelligence measure and objective hearing aid benefit. When individual hearing thresholds were considered as an additional independent variable, hearing loss was the only significant contributor to the benefit models. Lower cognitive capacity - as determined by the fluid intelligence measure - was significantly associated with greater hearing loss. Subjective benefit could not be predicted by any of the variables considered. The present study does not give evidence that hearing aid benefit is critically associated with cognitive function in experienced hearing aid users. However, it was found that lower fluid intelligence scores were related to higher hearing thresholds. Since greater hearing loss was associated with a greater objective benefit, these results strongly support the advice of using hearing aids regardless of age and cognitive function to counter hearing loss and the adverse effects of age-related hearing impairment. Still, individual cognitive capacity might

  14. 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

  15. 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.

  16. 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

  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. 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

  19. Prevalence and causes of hearing impairment in Africa.

    PubMed

    Mulwafu, W; Kuper, H; Ensink, R J H

    2016-02-01

    To systematically assess the data on the prevalence and causes of hearing impairment in Africa. Systematic review on the prevalence and causes of hearing loss in Africa. We undertook a literature search of seven electronic databases (EMBASE, PubMed, Medline, Global Health, Web of Knowledge, Academic Search Complete and Africa Wide Information) and manually searched bibliographies of included articles. The search was restricted to population-based studies on hearing impairment in Africa. Data were extracted using a standard protocol. We identified 232 articles and included 28 articles in the final analysis. The most common cut-offs used for hearing impairment were 25 and 30 dB HL, but this ranged between 15 and 40 dB HL. For a cut-off of 25 dB, the median was 7.7% for the children- or school-based studies and 17% for population-based studies. For a cut-off of 30 dB HL, the median was 6.6% for the children or school-based studies and 31% for population-based studies. In schools for the deaf, the most common cause of hearing impairment was cryptogenic deafness (50%) followed by infectious causes (43%). In mainstream schools and general population, the most common cause of hearing impairment was middle ear disease (36%), followed by undetermined causes (35%) and cerumen impaction (24%). There are very few population-based studies available to estimate the prevalence of hearing impairment in Africa. Those studies that are available use different cut-offs, making comparison difficult. However, the evidence suggests that the prevalence of hearing impairment is high and that much of it is avoidable or treatable. © 2015 John Wiley & Sons Ltd.

  20. Exploration of a physiologically-inspired hearing-aid algorithm using a computer model mimicking impaired hearing.

    PubMed

    Jürgens, Tim; Clark, Nicholas R; Lecluyse, Wendy; Meddis, Ray

    2016-01-01

    To use a computer model of impaired hearing to explore the effects of a physiologically-inspired hearing-aid algorithm on a range of psychoacoustic measures. A computer model of a hypothetical impaired listener's hearing was constructed by adjusting parameters of a computer model of normal hearing. Absolute thresholds, estimates of compression, and frequency selectivity (summarized to a hearing profile) were assessed using this model with and without pre-processing the stimuli by a hearing-aid algorithm. The influence of different settings of the algorithm on the impaired profile was investigated. To validate the model predictions, the effect of the algorithm on hearing profiles of human impaired listeners was measured. A computer model simulating impaired hearing (total absence of basilar membrane compression) was used, and three hearing-impaired listeners participated. The hearing profiles of the model and the listeners showed substantial changes when the test stimuli were pre-processed by the hearing-aid algorithm. These changes consisted of lower absolute thresholds, steeper temporal masking curves, and sharper psychophysical tuning curves. The hearing-aid algorithm affected the impaired hearing profile of the model to approximate a normal hearing profile. Qualitatively similar results were found with the impaired listeners' hearing profiles.

  1. 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.

  2. 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.

  3. 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

  4. 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…

  5. 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…

  6. 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…

  7. 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…

  8. 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…

  9. 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)

  10. Metaphor Performance in Children with Hearing Impairment.

    ERIC Educational Resources Information Center

    Wolgemuth, Keith S.; Kamhi, Alan G.; Lee, Rene F.

    1998-01-01

    Comparison of 13 children with mild-to-moderate sensorineural hearing loss but normal language skills and 12 children with normal hearing and development found no significant group differences on three verbal metaphor tasks (comprehension, preference, and completion), and one visual metaphor task. (Author/DB)

  11. Binaural Loudness Summation in the Hearing Impaired.

    ERIC Educational Resources Information Center

    Hawkins, David B.; And Others

    1987-01-01

    Binaural loudness summation was measured using three different paradigms with 10 normally hearing and 20 bilaterally symmetrical high-frequency sensorineural hearing loss subjects. Binaural summation increased with presentation level using the loudness matching procedure, with values in the 6-10 dB range. Summation decreased with level using the…

  12. Hearing impairment and vowel production. A comparison between normally hearing, hearing-aided and cochlear implanted Dutch children.

    PubMed

    Verhoeven, Jo; Hide, Oydis; De Maeyer, Sven; Gillis, San; Gillis, Steven

    2016-01-01

    This study investigated the acoustic characteristics of the Belgian Standard Dutch vowels in children with hearing impairment and in children with normal hearing. In a balanced experimental design, the 12 vowels of Belgian Standard Dutch were recorded in three groups of children: a group of children with normal hearing, a group with a conventional hearing aid and a group with a cochlear implant. The formants, the surface area of the vowel space and the acoustic differentiation between the vowels were determined. The analyses revealed that many of the vowels in hearing-impaired children showed a reduction of the formant values. This reduction was particularly significant with respect to F2. The size of the vowel space was significantly smaller in the hearing-impaired children. Finally, a smaller acoustic differentiation between the vowels was observed in children with hearing impairment. The results show that even after 5 years of device use, the acoustic characteristics of the vowels in hearing-assisted children remain significantly different as compared to their NH peers. Copyright © 2015 Elsevier Inc. All rights reserved.

  13. Strong founder effect of p.P240L in CDH23 in Koreans and its significant contribution to severe-to-profound nonsyndromic hearing loss in a Korean pediatric population.

    PubMed

    Kim, So Young; Kim, Ah Reum; Kim, Nayoung K D; Kim, Min Young; Jeon, Eun-Hee; Kim, Bong Jik; Han, Young Eun; Chang, Mun Young; Park, Woong-Yang; Choi, Byung Yoon

    2015-08-13

    Despite the prevalence of CDH23 mutations in East Asians, its large size hinders investigation. The pathologic mutation p.P240L in CDH23 is common in East Asians. However, whether this mutation represents a common founder or a mutational hot spot is unclear. The prevalence of CDH23 mutations with prelingual severe-to-profound sporadic or autosomal recessive sensorineural hearing loss (arSNHL) is unknown in Koreans. From September 2010 to October 2014, children with severe-to-profound sporadic or arSNHL without phenotypic markers, and their families, were tested for mutations in connexins GJB2, GJB6 and GJB3. Sanger sequencing of CDH23 p.P240L was performed on connexin-negative samples without enlarged vestibular aqueducts (EVA), followed by targeted resequencing of 129 deafness genes, including CDH23, unless p.P240L homozygotes were detected in the first screening. Four p.P240L-allele-linked STR markers were genotyped in 40 normal-hearing control subjects, and the p.P240L carriers in the hearing-impaired cohort, to identify the haplotypes. Four (3.1 %) of 128 children carried two CDH23 mutant alleles, and SLC26A4 and GJB2 accounted for 18.0 and 17.2 %, respectively. All four children showed profound nonsyndromic SNHL with minimal residual hearing. Interestingly, all had at least one p.P240L mutant allele. Analysis of p.P240L-linked STR markers in these children and other postlingual hearing-impaired adults carrying p.P240L revealed that p.P240L was mainly carried on a single haplotype. p.P240L contributed significantly to Korean pediatric severe arSNHL with a strong founder effect, with implications for future phylogenetic studies. Screening for p.P240L as a first step in GJB2-negative arSNHL Koreans without EVA is recommended.

  14. [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.

  15. [The assessment of hearing impairment in patients over 60 years of age using hearing aids].

    PubMed

    Skrzypek, Aleksandra; Sekula, Alicja; Deryło, Maria Bratumiła; Kuśmierczyk, Joanna; Talar, Marcin

    2014-01-01

    To assess the hearing impairment in people over 60 years old using hearing aids. This was a single-center study, but it is planned to extend it further to the whole country. The study was focused on patients with hearing aids. During the assessment 57 people were included in the observation in order to control the status of their hearing loss and benefit from traditional hearing aids as well as the possibility to apply the auditory implants in case of a little benefit from hearing aids. The otoscopy and pure tone audiometry were performed as well as the questionnaires on demographic and epidemiological data of patients were collected as well as the quality of their life with hearing aids was subjectively assessed. The results show that 91% of patients have sensorineural hearing loss (SHL), the remaining 9%--severe mixed hearing loss. Severe SHL was found in 22 patients, the moderate hearing loss was observed in 37%, and the profound SHL was the case in 5 patients. Minimal SHL was observed in 7% of patients (n=4). More than 73% of the study subjects were male (n=38). The average age of the patients who completed the survey was 74 years old. Thirty-five patients used their hearing aid over 3 years and less than 70% of them used it every day all day. Hearing aid was not actively used by 10 patients. Over the last year 51.92% of the patients underwent a hearing examination. The bone anchored hearing aid was suggested to 2% of subjects and the cochlear implant was offered to 10 patients. The data analysis shows the need to educate and inform the elderly about alternative methods of hearing loss treatment. Copyright © 2013 Polish Otorhinolaryngology - Head and Neck Surgery Society. Published by Elsevier Urban & Partner Sp. z.o.o. All rights reserved.

  16. 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

  17. 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)

  18. Speech Intelligibility in Persian Hearing Impaired Children with Cochlear Implants and Hearing Aids.

    PubMed

    Rezaei, Mohammad; Emadi, Maryam; Zamani, Peyman; Farahani, Farhad; Lotfi, Gohar

    2017-04-01

    The aim of present study is to evaluate and compare speech intelligibility in hearing impaired children with cochlear implants (CI) and hearing aid (HA) users and children with normal hearing (NH). The sample consisted of 45 Persian-speaking children aged 3 to 5-years-old. They were divided into three groups, and each group had 15, children, children with CI and children using hearing aids in Hamadan. Participants was evaluated by the test of speech intelligibility level. Results of ANOVA on speech intelligibility test showed that NH children had significantly better reading performance than hearing impaired children with CI and HA. Post-hoc analysis, using Scheffe test, indicated that the mean score of speech intelligibility of normal children was higher than the HA and CI groups; but the difference was not significant between mean of speech intelligibility in children with hearing loss that use cochlear implant and those using HA. It is clear that even with remarkabkle advances in HA technology, many hearing impaired children continue to find speech production a challenging problem. Given that speech intelligibility is a key element in proper communication and social interaction, consequently, educational and rehabilitation programs are essential to improve speech intelligibility of children with hearing loss.

  19. Perceptions Toward Internet-Based Delivery of Hearing Aids among Older Hearing-Impaired Adults.

    PubMed

    Chandra, Navshika; Searchfield, Grant D

    2016-06-01

    Despite evidence that hearing aids can improve the social and psychological functioning of older hearing-impaired adults, hearing aid uptake is low. High cost of hearing aids and poor access to audiology services in rural areas are potential barriers to hearing aid acquisition. Methods of hearing aid delivery deviating from the traditional clinician-based model have been available to consumers for many years. One such method is Internet hearing aid sales. However, research exploring Internet-based hearing aid delivery, as a method to improve hearing aid uptake in this population, is limited. The purpose of this study was to explore the perceptions of older hearing aid users (aged ≥65 yr) toward Internet-based hearing aid delivery. A qualitative approach was adopted to investigate older adults' perceptions of buying hearing aids online. The sample consisted of 18 participants aged between 64 and 81 yr. Fourteen men and four women participated in this study. Participants were all experienced hearing aid users. Face-to-face semistructured interviews were conducted. An interview schedule guided the interview. Interviews were recorded with a voice recorder and transcribed verbatim. Thematic analysis of the data was carried out. Seven main themes emerged from the data. A general lack of awareness, but willingness to learn more about Internet hearing aid sales, was found. Two perceived benefits of Internet-based hearing aid delivery were identified: lower cost of hearing aids and greater convenience or physical accessibility. Numerous concerns and limitations were communicated. Concerns regarding the availability of clinical procedures, such as hearing tests, obtaining the correct-sized earmolds, and fine-tuning of hearing aids, were expressed. Participants conveyed distrust in online retailers. However, trust in and a preference for audiologists' expertise, which was not perceived to be available online, was found. Participants further conveyed a preference for face

  20. SCALES AND PROCEDURES FOR ASSESSING SOCIAL-PSYCHOLOGICAL CHARACTERISTICS OF VISUALLY IMPAIRED AND HEARING IMPAIRED STUDENTS.

    ERIC Educational Resources Information Center

    ERICKSON, EDSEL L.; JOINER, LEE M.

    THIS IS A METHODOLOGICAL STUDY TO DETERMINE IF RELIABLE AND VALIDLY COMPARABLE DATA CAN BE OBTAINED FROM SCALES DESIGNED FOR USE WITH HEARING IMPAIRED, VISUALLY IMPAIRED, AND NON-IMPAIRED HIGH SCHOOL STUDENTS. THE MAJOR INSTRUMENTS ASSESSED SELF CONCEPT OF ACADEMIC ABILITY. IT WAS CONCLUDED ON THE BASIS OF CROSS-VALIDATION AND OTHER ANALYTICAL…

  1. 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.

  2. Children in educational programs for the hearing impaired whose impairment was caused by mumps.

    PubMed

    Jensema, C

    1975-05-01

    Mumps was reported to be a cause of hearing loss in 259 persons from a sample of 43,946 students enrolled in special educational programs for the hearing impaired. Males and young children appear to be more prone to deafening by mumps, and there may be a relationship between age of onset and degree of hearing loss. Overall, those deafened by mumps appear to have characteristics quite different from those of children deafened by other causes.

  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. High-Level Psychophysical Tuning Curves: Forward Masking in Normal-Hearing and Hearing-Impaired Listeners.

    ERIC Educational Resources Information Center

    Nelson, David A.

    1991-01-01

    Forward-masked psychophysical tuning curves were obtained at multiple probe levels from 26 normal-hearing listeners and 24 ears of 21 hearing-impaired listeners with cochlear hearing loss. Results indicated that some cochlear hearing losses influence the sharp tuning capabilities usually associated with outer hair cell function. (Author/JDD)

  5. 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

  6. Follow-up of permanent hearing impairment in childhood.

    PubMed

    Della Volpe, A; De Lucia, A; Pastore, V; Bracci Laudiero, L; Buonissimo, I; Ricci, G

    2016-02-01

    Programmes for early childhood childhood hearing impairment identification allows to quickly start the appropriate hearing aid fitting and rehabilitation process; nevertheless, a large number of patients do not join the treatment program. The goal of this article is to present the results of a strategic review of the strengths, weaknesses, opportunities and threats connected with the audiologic/prosthetic/language follow-up process of children with bilateral permanent hearing impairment. Involving small children, the follow-up includes the involvement of specialised professionals of a multidisciplinary team and a complex and prolonged multi-faced management. Within the framework 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", the purpose of this analysis was to propose recommendations that can harmonise criteria for outcome evaluation and provide guidance on the most appropriate assessment methods to be used in the follow-up course of children with permanent hearing impairment. © Copyright by Società Italiana di Otorinolaringologia e Chirurgia Cervico-Facciale.

  7. Psychopathology among a sample of hearing impaired adolescents.

    PubMed

    Mosaku, Kolawole; Akinpelu, Victoria; Ogunniyi, Grace

    2015-12-01

    Hearing impairment is a recognized cause of emotional and psychological disturbances worldwide, however little is known about this condition in Nigeria. The aim of this study is to compare the prevalence of psychopathology between hearing impaired adolescents and healthy adolescents. Students attending two special schools for the hearing impaired were assessed for psychopathology with the help of a trained signer and their teacher, using the International Classification of Diseases Diagnostic Criteria (ICD 10). Fifty two hearing impaired students and 52 age and sex matched controls from the same school were also interviewed using the same instrument. The mean age of the hearing impaired students was 16 (sd=3.8), while for the controls the mean age was 16 (sd=2.5). Psychopathology was present in 10 (19%) of the hearing impaired adolescents compared to 2 (4%) among the control group, this difference was statistically significant (χ(2)=4.62 p=0.03). The most common diagnosis was generalized anxiety disorder 4 (8%), followed by depression 2 (4%). Years spent in school (t=4.81, p=0.001), primary guardian (χ(2)=18.3, p=0.001) and mean income of guardian (t=7.10, p=0.001) were all significantly different between the two groups. Psychopathology is relatively common in this population. Proper assessment and treatment should be made available for this population group. A limitation to this study is communication difficulty which made only a third party assessment possible; this may affect the generalizability of the findings. Copyright © 2015 Elsevier B.V. All rights reserved.

  8. Perceptions of adults with hearing impairment regarding the promotion of trust in hearing healthcare service delivery.

    PubMed

    Preminger, Jill E; Oxenbøll, Maria; Barnett, Margaret B; Jensen, Lisbeth D; Laplante-Lévesque, Ariane

    2015-01-01

    This paper describes how trust is promoted in adults with hearing impairment within the context of hearing healthcare (HHC) service delivery. Data were analysed from a previously published descriptive qualitative study that explored perspectives of adults with hearing impairment on hearing help-seeking and rehabilitation. Interview transcripts from 29 adults from four countries with different levels of hearing impairment and different experience with the HHC system were analysed thematically. Patients enter into the HHC system with service expectations resulting in a preconceived level of trust that can vary from low to high. Relational competence, technical competence, commercialized approach, and clinical environment (relevant to both the clinician and the clinic) influence a patient's resulting level of trust. Trust is evolving rather than static in HHC: Both clinicians and clinics can promote trust. The characteristics of HHC that engender trust are: practicing good communication, supporting shared decision making, displaying technical competence, offering comprehensive hearing rehabilitation, promoting self-management, avoiding a focus on hearing-aid sales, and offering a professional clinic setting.

  9. [Sensorineural hearing impairment in combination with mycoplasma infection].

    PubMed

    Gurov, A V; Levina, Yu V; Rudenko, V V

    2015-01-01

    The objective of the present study was to elucidate the incidence of mycoplasma infection concomitant with sensorineural hearing impairment and its clinical manifestations with special reference to the methods for its diagnostics and treatment. The main method for the detection of mycoplasma infection is PCR in real time and the auxiliary one is the immunoenzymatic assay. The study revealed mycoplasma infection in 15 (13.9%) of the examined patients. The results of our investigations give evidence of the necessity to further study the clinical symptoms of mycoplasma infection associated with sensorineural hearing impairment and to search for the methods of the management of this condition.

  10. 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.

  11. 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)

  12. 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)

  13. 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.

  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. 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

  16. 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.

  17. 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

  18. Binaural pitch perception in normal-hearing and hearing-impaired listeners.

    PubMed

    Santurette, Sébastien; Dau, Torsten

    2007-01-01

    The effects of hearing impairment on the perception of binaural-pitch stimuli were investigated. Several experiments were performed with normal-hearing and hearing-impaired listeners, including detection and discrimination of binaural pitch, and melody recognition using different types of binaural pitches. For the normal-hearing listeners, all types of binaural pitches could be perceived immediately and were musical. The hearing-impaired listeners could be divided into three groups based on their results: (a) some perceived all types of binaural pitches, but with decreased salience or musicality compared to normal-hearing listeners; (b) some could only perceive the strongest pitch types; (c) some were unable to perceive any binaural pitch at all. The performance of the listeners was not correlated with audibility. Additional experiments investigated the correlation between performance in binaural-pitch perception and performance in measures of spectral and temporal resolution. Reduced frequency discrimination appeared to be linked to poorer melody recognition skills. Reduced frequency selectivity was also found to impede the perception of binaural-pitch stimuli. Overall, binaural-pitch stimuli might be very useful tools within clinical diagnostics for detecting specific deficiencies in the auditory system.

  19. 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.

  20. 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…

  1. 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.

  2. Alternative Summer Educational Programs for Urban Hearing-Impaired Children.

    ERIC Educational Resources Information Center

    Goldfarb, Mark; Israelson, Jo

    The paper describes the summer programs offered by Kendall Demonstration Elementary School, a year-round day school for elementary level hearing impaired students in Washington, D.C. The programs are explained to be based on a three-point philosophy: (1) summer programs should be different from those of the rest of the year; (2) summer programs…

  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. Hearing-Impaired Children's Comprehension of Verb-Particle Combinations.

    ERIC Educational Resources Information Center

    Payne, John-Allen; Quigley, Stephen

    1987-01-01

    A study of 45 hearing-impaired students (ages 10 to 19) investigated their understanding of English verb-particle combinations of varying syntactic and semantic difficulty. Results suggest an order of difficulty in learning. Idioms were found to be more difficult than literal meanings with little improvement over the age range of the subjects.…

  5. 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/.…

  6. The Benefits of Art for Mainstreamed Hearing-Impaired Children.

    ERIC Educational Resources Information Center

    James, Philip; James, Carol

    1980-01-01

    Art experiences can promote feelings of confidence in mainstreamed hearing impaired children. Because of the emphasis on the production of visual/tactile images and objects and the lower demands for verbal communication within the art class, children with low language comprehension and limited verbal facility have a greater opportunity for success…

  7. 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…

  8. 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...

  9. Issues in the Intellectual Assessment of Hearing Impaired Children

    ERIC Educational Resources Information Center

    Hughes, Deana; Sapp, Gary L.; Kohler, Maxie P.

    2006-01-01

    The assessment of hearing impaired children is fraught with a number of problems. These include lack of valid assessment measures, faulty theoretical assumptions, lack of knowledge regarding the functioning of cognitive processes of these children, and biases against these children. This article briefly considers these issues and describes a study…

  10. 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…

  11. 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,…

  12. 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…

  13. Diagnosing a Learning Disability in a Hearing-Impaired Child.

    ERIC Educational Resources Information Center

    Plapinger, Donald; Sikora, Darryn

    1990-01-01

    This paper presents a case study that used an interdisciplinary diagnostic approach to determine whether a hearing-impaired nine-year-old child had a learning disability and to determine the type of disability. The assessment included medical examination, gross and fine motor skills, psychological functioning, mental processing and achievement,…

  14. 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…

  15. 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…

  16. A Computer-Controlled Vowel Corrector for the Hearing Impaired.

    ERIC Educational Resources Information Center

    Povel, Dirk-Jan; Wansink, Marty

    1986-01-01

    Properties of the Vowel Corrector, a visual aid used in speech training of the hearing impaired, are examined. Implications for differentiating utterances of male, female, and child speakers are studied. Results show that the device has promising features and is especially useful for exploring the vowel space and learning a global differentiation…

  17. 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;…

  18. Hearing-Impaired Students: Options for Far Visual Acuity Screening.

    ERIC Educational Resources Information Center

    Johnson, Donald D.; Caccamise, Frank

    1983-01-01

    The comparison of Orthorater vision tester and Snellen chart far visual acuity results for 261 National Technical Institute for the Deaf (NTID) students. Results indicated that a rear illuminated Snellen chart is an acceptable alternative to the Orthorater for screening postsecondary, hearing impaired students' far visual acuity. (Author)

  19. 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)

  20. Auditory Learning and Teaching of Hearing-Impaired Infants.

    ERIC Educational Resources Information Center

    Mischook, Muriel; Cole, Elizabeth

    1986-01-01

    The chapter examines audition and early intervention with hearing impaired infants, the normal development of audition, a model of auditory learning and teaching (involving discrimination, identification, comprehension, and detection), progression along the developmental sequence, natural interactions which aid learning, and parent role. (DB)

  1. Association of Hearing Impairment and Emotional Vitality in Older Adults

    PubMed Central

    Contrera, Kevin J.; Betz, Josh; Deal, Jennifer A.; Choi, Janet S.; Ayonayon, Hilsa N.; Harris, Tamara; Helzner, Elizabeth; Martin, Kathryn R.; Mehta, Kala; Pratt, Sheila; Rubin, Susan M.; Satterfield, Suzanne; Yaffe, Kristine; Garcia, Melissa; Simonsick, Eleanor M.

    2016-01-01

    Objectives: To better understand the potential impact of hearing impairment (HI) and hearing aid use on emotional vitality and mental health in older adults. Method: We investigated the cross-sectional association of HI with emotional vitality in 1,903 adults aged 76–85 years in the Health ABC study adjusted for demographic and cardiovascular risk factors. Hearing was defined by the speech frequency pure tone average (no impairment < 25 dB, mild impairment 25–40 dB, and moderate or greater impairment > 40 dB). Emotional vitality was defined as having a high sense of personal mastery, happiness, low depressive symptomatology, and low anxiety. Results: Compared with individuals with no HI, participants with moderate or greater HI had a 23% lower odds of emotional vitality (odds ratio [OR] = 0.77; 95% confidence interval [CI]: 0.59–0.99). Hearing aid use was not associated with better emotional vitality (OR = 0.98; 95% CI: 0.81–1.20). Discussion: HI is associated with lower odds of emotional vitality in older adults. Further studies are needed to examine the longitudinal impact of HI on mental health and well-being. PMID:26883806

  2. Association of Hearing Impairment and Emotional Vitality in Older Adults.

    PubMed

    Contrera, Kevin J; Betz, Josh; Deal, Jennifer A; Choi, Janet S; Ayonayon, Hilsa N; Harris, Tamara; Helzner, Elizabeth; Martin, Kathryn R; Mehta, Kala; Pratt, Sheila; Rubin, Susan M; Satterfield, Suzanne; Yaffe, Kristine; Garcia, Melissa; Simonsick, Eleanor M; Lin, Frank R

    2016-05-01

    To better understand the potential impact of hearing impairment (HI) and hearing aid use on emotional vitality and mental health in older adults. We investigated the cross-sectional association of HI with emotional vitality in 1,903 adults aged 76-85 years in the Health ABC study adjusted for demographic and cardiovascular risk factors. Hearing was defined by the speech frequency pure tone average (no impairment < 25 dB, mild impairment 25-40 dB, and moderate or greater impairment > 40 dB). Emotional vitality was defined as having a high sense of personal mastery, happiness, low depressive symptomatology, and low anxiety. Compared with individuals with no HI, participants with moderate or greater HI had a 23% lower odds of emotional vitality (odds ratio [OR] = 0.77; 95% confidence interval [CI]: 0.59-0.99). Hearing aid use was not associated with better emotional vitality (OR = 0.98; 95% CI: 0.81-1.20). HI is associated with lower odds of emotional vitality in older adults. Further studies are needed to examine the longitudinal impact of HI on mental health and well-being. © The Author 2016. Published by Oxford University Press on behalf of The Gerontological Society of America. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  3. 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;…

  4. 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…

  5. 38 CFR 4.85 - Evaluation of hearing impairment.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... audiologist and must include a controlled speech discrimination test (Maryland CNC) and a puretone audiometry... Designation of Hearing Impairment Based on Puretone Threshold Average and Speech Discrimination,” is used to... percent of speech discrimination (horizontal rows) and the puretone threshold average (vertical columns...

  6. Your Computerized Classroom: Using Computers with Hearing Impaired Students.

    ERIC Educational Resources Information Center

    Abrams, Mary, Ed.

    Thirteen papers submitted to a proposed conference (1986) at Gallaudet College on using computers to teach hearing impaired students are collected in this book. Many papers emphasize the use of word processing software to build language skills. Papers have the following titles and authors: "The Business of Writing: The Special Kids Card Company"…

  7. 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)

  8. Educational Ramifications of Various Instructional Inputs for Hearing Impaired Students.

    ERIC Educational Resources Information Center

    Luetke-Stahlman, Barbara

    1988-01-01

    The study compared scores of 2 groups of hearing-impaired students ages 5 to 12 years on a literacy battery. Subjects (n=73) were receiving instruction which either completely encoded spoken English or incompletely encoded spoken English. Those receiving completely encoded English instruction tended to score higher on achievement tests especially…

  9. Teaching Sign Language to Hearing Impaired-Mentally Retarded Students.

    ERIC Educational Resources Information Center

    Stanovich, Paula J.; Stanovich, Keith E.

    Students with the dual handicaps of hearing impairment and mental retardation display special problems in language acquisition. These problems do not appear to have been addressed by curricula that have been designed for either of the single handicap groups. Since specially designed curricula for this population are virtually nonexistent, a…

  10. 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…

  11. 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…

  12. 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…

  13. Syntactic Movement in Orally Trained Children with Hearing Impairment

    ERIC Educational Resources Information Center

    Friedmann, Naama; Szterman, Ronit

    2006-01-01

    This study explored the comprehension and production of sentences derived by syntactic movement, in orally trained school-age Hebrew-speaking children with moderate to profound hearing impairment, aged 7;8?9;9 years. Experiments 1 and 2 tested the comprehension of relative clauses and topicalization sentences (with word orders of OVS [object,…

  14. 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,…

  15. Hearing-Impaired Children in Black and Hispanic Families.

    ERIC Educational Resources Information Center

    Fischgrund, Joseph E.; And Others

    1987-01-01

    The article presents sociocultural information about Black and Hispanic families and the implications for intervention programs for parents of hearing-impaired minority children. Discussion addresses the importance of the extended family and community in providing care, differing belief systems about handicaps, and anxieties in adjusting to a new…

  16. 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…

  17. 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)

  18. 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…

  19. Teaching Vowels to Profoundly Hearing-Impaired Speakers Using Glossometry.

    ERIC Educational Resources Information Center

    Fletcher, Samuel G.; And Others

    1991-01-01

    Glossometry, a method of providing visual feedback of tongue positions, was used to teach four vowel sounds to six profoundly hearing-impaired children. After 15 to 20 50-minute training sessions, all subjects showed greater diversification of tongue postures for the vowels. Listener identifications were also generally better after therapy.…

  20. A Family Involvement Model for Hearing-Impaired Infants.

    ERIC Educational Resources Information Center

    Fitzgerald, Mary Trabue; Fischer, Rebecca M.

    1987-01-01

    The Mama Lere Parent-Infant Training Program (Nashville, Tennessee) which serves hearing- and speech-impaired children focuses on family involvement and an intervention plan which includes four service delivery components: facilitation of child communicative competence; educational advocacy and team decision making; information exchange; and…

  1. 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)

  2. 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…

  3. Teaching Hearing-Impaired Children in Regular Classrooms. Language in Education: Theory and Practice, No. 54.

    ERIC Educational Resources Information Center

    Blackwell, Peter M.

    This guide is designed to assist regular classroom teachers in helping the hearing impaired child to master the English language. It is noted that the hearing impaired population is not homogenous. Different factors involved in the decision to place a hearing impaired child in a regular classroom are considered. The types of language problems…

  4. 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)

  5. 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…

  6. Solar-powered hearing aids for children with impaired hearing in Vietnam: a pilot study.

    PubMed

    Vo, Quang Thanh; Pham, Dung; Choi, Kevin J; Nguyen, Uyen T T; Le, Lan; Shanewise, Trudy; Tran, Lien; Nguyen, Nga; Lee, Walter T

    2017-01-25

    Hearing loss is a barrier to speech and social and cognitive development. This can be especially pronounced in children living in low- and middle-income countries with limited resources. To determine the feasibility, durability and social impact of ComCare GLW solar-powered hearing aids provided for Vietnamese children with hearing impairment. A retrospective review of data from an international, multi-discipline humanitarian visit was performed. Hearing aids were given to 28 children enrolled at the Khoai Chau Functional Rehabilitation School, Hung Yen Province, Vietnam. Device inspection and observational assessments were performed by teachers using a modified Parents' Evaluation of Aural/Oral Performance of Children and an Infant Hearing Program Amplification Benefit Questionnaire. Qualitative interviews were undertaken to assess the study aims. Hearing aids were well tolerated for use during regular school hours. All units remained functional during the study period (12 months). Teachers noted increased student awareness and responsiveness to surrounding sounds, but the degree of response to amplification varied between children. There was no significant improvement in speech development as all subjects had prelingual deafness. Teachers felt confident in troubleshooting any potential device malfunction. A solar-powered hearing aid may be a viable option for children in low- and middle-income countries. This study demonstrates that device distribution, maintenance and function can be established in countries with limited resources, while providing feasibility data to support future studies investigating how similar devices may improve the quality of life of those with hearing loss.

  7. Temporal modulation transfer functions obtained using sinusoidal carriers with normally hearing and hearing-impaired listeners.

    PubMed

    Moore, B C; Glasberg, B R

    2001-08-01

    Temporal modulation transfer functions were obtained using sinusoidal carriers for four normally hearing subjects and three subjects with mild to moderate cochlear hearing loss. Carrier frequencies were 1000, 2000 and 5000 Hz, and modulation frequencies ranged from 10 to 640 Hz in one-octave steps. The normally hearing subjects were tested using levels of 30 and 80 dB SPL. For the higher level, modulation detection thresholds varied only slightly with modulation frequency for frequencies up to 80 Hz, but decreased for high modulation frequencies. The decrease can be attributed to the detection of spectral sidebands. For the lower level, thresholds varied little with modulation frequency for all three carrier frequencies. The absence of a decrease in the threshold for large modulation frequencies can be explained by the low sensation level of the spectral sidebands. The hearing-impaired subjects were tested at 80 dB SPL, except for two cases where the absolute threshold at the carrier frequency was greater than 70 dB SPL; in these cases a level of 90 dB was used. The results were consistent with the idea that spectral sidebands were less detectable for the hearing-impaired than for the normally hearing subjects. For the two lower carrier frequencies, there were no large decreases in threshold with increasing modulation frequency, and where decreases did occur, this happened only between 320 and 640 Hz. For the 5000-Hz carrier, thresholds were roughly constant for modulation frequencies from 10 to 80 or 160 Hz, and then increased monotonically, becoming unmeasurable at 640 Hz. The results for this carrier may reflect "pure" effects of temporal resolution, without any influence from the detection of spectral sidebands. The results suggest that temporal resolution for deterministic stimuli is similar for normally hearing and hearing-impaired listeners.

  8. 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.

  9. Health-related quality of life in adults with hearing impairment before and after hearing-aid rehabilitation in Finland.

    PubMed

    Niemensivu, Riina; Manchaiah, Vinaya; Roine, Risto P; Kentala, Erna; Sintonen, Harri

    2015-01-01

    The aim of this study was to evaluate health-related quality of life (HRQoL) in adults with hearing impairment in Finland before and after hearing rehabilitation. The study was prospective with hearing-aid rehabilitation as the intervention. The data was collected, using the 15D instrument, before and six months after hearing-aid rehabilitation. The data was analysed using t-tests and multiple linear regression methods. The study sample included 949 adults with hearing impairment, and the control group included a sample of age- and gender-standardized general population. The study population had significantly poorer HRQoL on most dimensions of the 15D when compared to the control group both before and after hearing-aid rehabilitation. Hearing-aid rehabilitation resulted in improved mean scores on the dimensions of hearing and in the overall 15D score that were statistically significant, although the mean improvement in the overall score was marginal. Self-reported hearing ability can better predict the change in HRQoL, as a result of a hearing aid, when compared with measured hearing sensitivity. The study supports the hypothesis that on average, use of a unilateral hearing aid results in improved subjective hearing and marginal improvement in HRQoL in adults with hearing impairment.

  10. Mathematics achievement of hearing impaired adolescents in different placements.

    PubMed

    Kluwin, T N; Moores, D F

    1989-01-01

    This study, involving 215 students and 63 teachers, addressed three concerns related to mainstreaming for hearing imparied students: the selection process, the difference between a mainstream placement with an interpreter and a self-contained placement, and the quality of the educational experience. Almost half of the variance in achievement between the two settings is described. Three conclusions can be drawn. First, student background factors are a primary determinant of achievement. Second, mainstreaming with an interpreter has no specific effect on achievement for hearing impaired students. Third, the quality of instruction is the prime determinant of achievement, regardless of placement.

  11. 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.

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

    PubMed Central

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

    2015-01-01

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

  13. Oral health conditions and behaviors among hearing impaired and normal hearing college students at Ratchasuda College, Nakhon Pathom, Thailand.

    PubMed

    Vichayanrat, Tippanart; Kositpumivate, Waritorn

    2014-09-01

    This study aimed to explore oral health and oral health related behaviors among hearing impaired and normal hearing students at Rachasuda College. The association between socioeconomic factors, hearing status, oral health behaviors, oral hygiene levels and dental caries status were also examined. The students filled out a self-administered questionnaire with assistance of a sign language video to obtain personal and behavior information. A total of 180 students, 83 normal hearing and 97 hearing impaired students completed the questionnaire and underwent an oral examination. The prevalences of caries were 53.6% and 50.6% among students with hearing impairment and normal hearing, respectively (p=0.354). After age stratification, the hearing impaired students aged 18-21 years had significantly less filled teeth (p=0.012), and those older than 21 years had less missing teeth due to caries than normal-hearing students (p=0.023). Poor oral hygiene was found in 51.8% and 42.2% of normal and hearing-impaired students, respectively (p=0.365). Caries status was significantly associated with maternal education level (OR 3.56; 95% CI: 1.52-8.32) and oral hygiene (OR 3.26; 95% CI: 1.64-6.45). The high prevalence of dental caries and poor oral hygiene among college students is alarming. Hearing impairment did not appear to affect the prevalences of these conditions compared to those with normal hearing. Oral health education tools need to be developed and utilized for both normal hearing and hearing impaired college students in Thailand.

  14. 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

  15. 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…

  16. 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.

  17. 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

  18. GJB2 and GJB6 mutations are an infrequent cause of autosomal-recessive nonsyndromic hearing loss in residents of Mexico.

    PubMed

    Hernández-Juárez, Aideé Alejandra; Lugo-Trampe, José de Jesús; Campos-Acevedo, Luis Daniel; Lugo-Trampe, Angel; Treviño-González, José Luis; de-la-Cruz-Ávila, Israel; Martínez-de-Villarreal, Laura Elia

    2014-12-01

    Mutations in the DFNB1 locus are the most common cause of autosomal-recessive nonsyndromic hearing loss (ARNSHL) worldwide. The aim of this study was to identify the most frequent mutations in patients with ARNSHL who reside in Northeastern Mexico. We determined the nucleotide sequence the coding region of GJB2 of 78 patients with ARNSHL. Polymerase chain reaction assays were used to detect the GJB2 IVS1+1G>A mutation and deletions within GJB6. GJB2 mutations were detected in 9.6% of the alleles, and c.35delG was the most frequent. Six other less-frequent mutations were detected, including an extremely rare variant (c.645_648delTAGA), a novel mutation (c.35G>A), and one of possible Mexican origin (c.34G>T). GJB6 deletions and GJB2 IVS1+1G>A were not detected. These data suggest that mutations in the DFNB1 locus are a rare cause of ARNSHL among the population of Northeastern Mexico. This confirms the genetic heterogeneity of this condition and indicates that further research is required to determine the other mechanisms of pathogenesis of ARNSHL in Mexicans. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

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

    PubMed

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

    2017-08-08

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

  20. 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

  1. 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.

  2. 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.

  3. 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.

  4. Hearing Characteristics of Stroke Patients: Prevalence and Characteristics of Hearing Impairment and Auditory Processing Disorders in Stroke Patients.

    PubMed

    Koohi, Nehzat; Vickers, Deborah A; Lakshmanan, Rahul; Chandrashekar, Hoskote; Werring, David J; Warren, Jason D; Bamiou, Doris-Eva

    2017-06-01

    Stroke survivors may suffer from a range of hearing impairments that may restrict their participation in postacute rehabilitation programs. Hearing impairment may have a significant impact on listening, linguistic skills, and overall communication of the affected stroke patient. However, no studies sought to systematically characterize auditory function of stroke patients in detail, to establish the different types of hearing impairments in this cohort of patients. Such information would be clinically useful in understanding and addressing the hearing needs of stroke survivors. The present study aimed to characterize and classify the hearing impairments, using a detailed audiological assessment test battery, in order to determine the level of clinical need and inform appropriate rehabilitation for this patient population. A case-control study. Forty-two recruited stroke patients who were discharged from a stroke unit and 40 control participants matched for age. All participants underwent pure-tone audiometry and immittance measurements including acoustic reflex threshold, transient-evoked otoacoustic emissions, auditory-evoked brainstem response, and a central auditory processing assessment battery, performed in a single session. Hearing impairments were classified as peripheral hearing loss (cochlear and neural type), central auditory processing disorder (CAPD), and as a combination of CAPD and peripheral hearing loss. Overall mean hearing thresholds were not significantly different between the control and stroke groups. The most common type of hearing impairment in stroke patients was the combination type, "peripheral and CAPD," in the 61- to 80-yr-old subgroup (in 55%), and auditory processing deficits in 18- to 60-yr-olds (in 40%), which were both significantly higher than in controls. This is the first study to examine hearing function in detail in stroke patients. Given the importance of hearing for the efficiency of communication, it is essential to identify

  5. Effects of Hearing Impairment and Hearing Aid Amplification on Listening Effort: A Systematic Review

    PubMed Central

    Ohlenforst, Barbara; Jansma, Elise P.; Wang, Yang; Naylor, Graham; Lorens, Artur; Lunner, Thomas; Kramer, Sophia E.

    2017-01-01

    Objectives: To undertake a systematic review of available evidence on the effect of hearing impairment and hearing aid amplification on listening effort. Two research questions were addressed: Q1) does hearing impairment affect listening effort? and Q2) can hearing aid amplification affect listening effort during speech comprehension? Design: English language articles were identified through systematic searches in PubMed, EMBASE, Cinahl, the Cochrane Library, and PsycINFO from inception to August 2014. References of eligible studies were checked. The Population, Intervention, Control, Outcomes, and Study design strategy was used to create inclusion criteria for relevance. It was not feasible to apply a meta-analysis of the results from comparable studies. For the articles identified as relevant, a quality rating, based on the 2011 Grading of Recommendations Assessment, Development, and Evaluation Working Group guidelines, was carried out to judge the reliability and confidence of the estimated effects. Results: The primary search produced 7017 unique hits using the keywords: hearing aids OR hearing impairment AND listening effort OR perceptual effort OR ease of listening. Of these, 41 articles fulfilled the Population, Intervention, Control, Outcomes, and Study design selection criteria of: experimental work on hearing impairment OR hearing aid technologies AND listening effort OR fatigue during speech perception. The methods applied in those articles were categorized into subjective, behavioral, and physiological assessment of listening effort. For each study, the statistical analysis addressing research question Q1 and/or Q2 was extracted. In seven articles more than one measure of listening effort was provided. Evidence relating to Q1 was provided by 21 articles that reported 41 relevant findings. Evidence relating to Q2 was provided by 27 articles that reported 56 relevant findings. The quality of evidence on both research questions (Q1 and Q2) was very low

  6. Effects of Hearing Impairment and Hearing Aid Amplification on Listening Effort: A Systematic Review.

    PubMed

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

    To undertake a systematic review of available evidence on the effect of hearing impairment and hearing aid amplification on listening effort. Two research questions were addressed: Q1) does hearing impairment affect listening effort? and Q2) can hearing aid amplification affect listening effort during speech comprehension? English language articles were identified through systematic searches in PubMed, EMBASE, Cinahl, the Cochrane Library, and PsycINFO from inception to August 2014. References of eligible studies were checked. The Population, Intervention, Control, Outcomes, and Study design strategy was used to create inclusion criteria for relevance. It was not feasible to apply a meta-analysis of the results from comparable studies. For the articles identified as relevant, a quality rating, based on the 2011 Grading of Recommendations Assessment, Development, and Evaluation Working Group guidelines, was carried out to judge the reliability and confidence of the estimated effects. The primary search produced 7017 unique hits using the keywords: hearing aids OR hearing impairment AND listening effort OR perceptual effort OR ease of listening. Of these, 41 articles fulfilled the Population, Intervention, Control, Outcomes, and Study design selection criteria of: experimental work on hearing impairment OR hearing aid technologies AND listening effort OR fatigue during speech perception. The methods applied in those articles were categorized into subjective, behavioral, and physiological assessment of listening effort. For each study, the statistical analysis addressing research question Q1 and/or Q2 was extracted. In seven articles more than one measure of listening effort was provided. Evidence relating to Q1 was provided by 21 articles that reported 41 relevant findings. Evidence relating to Q2 was provided by 27 articles that reported 56 relevant findings. The quality of evidence on both research questions (Q1 and Q2) was very low, according to the Grading of

  7. 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.

  8. Real world issues in classroom acoustics for hearing impaired students

    NASA Astrophysics Data System (ADS)

    Kleinschmidt, Klaus

    2005-04-01

    Experience is being gained from evaluating and modifying the acoustical properties and background noise levels of existing classrooms for use by hearing impaired students. Projects include more than 25 schools in public school systems that are mainstreaming handicapped children. Various practical and economic restrictions have led to recommendations for modifications that do not necessarily comply with ANSI S12.60 2002. Examples of real world conditions and practical solutions will be described.

  9. 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…

  10. Development of a System for Collecting Data on Hearing Impaired School Children. Final Report.

    ERIC Educational Resources Information Center

    Gentile, Augustine

    A method for collecting and disseminating information on hearing handicapped children was developed, and the availability of recorded information on both the incidence and development of hearing impaired children was determined. Detailed information was collected on 4,300 of the estimated 6,755 hearing impaired students enrolled in schools,…

  11. 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...

  12. 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,…

  13. 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…

  14. 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…

  15. Correlation of SPINE Test Scores to Judges' Ratings of Speech Intelligibility in Hearing-Impaired Children.

    ERIC Educational Resources Information Center

    Kelly, Colleen; And Others

    1986-01-01

    The SPINE test (SPeech INtelligibility Evaluation), designed to measure speech intelligibility of severely to profoundly hearing-impaired children was administered to 30 hearing-impaired children (12-16 years old) to examine its validity. Results suggested that the SPINE test is a valid measure of speech intelligibility with hearing-impaired…

  16. 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 including...

  17. 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,…

  18. 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.…

  19. 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…

  20. 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.

  1. 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

  2. Cigarette smoking causes hearing impairment among Bangladeshi population.

    PubMed

    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.

  3. Hearing handicap, rather than measured hearing impairment, predicts poorer quality of life over 10 years in older adults.

    PubMed

    Gopinath, Bamini; Schneider, Julie; Hickson, Louise; McMahon, Catherine M; Burlutsky, George; Leeder, Stephen R; Mitchell, Paul

    2012-06-01

    We aimed to determine the prospective association between measured hearing impairment, self-reported hearing handicap and hearing aid use with quality of life. 829 Blue Mountains Hearing Study participants (≥ 55 years) were examined between 1997-1999 and 2007-2009. The shortened version of the hearing handicap inventory was administered. Hearing levels were measured using pure-tone audiometry. Quality of life was assessed using the 36-Item Short-Form Survey (SF-36); higher scores reflect better quality of life. Hearing impairment at baseline compared with no impairment was associated with lower mean SF-36 mental composite score 10 years later (multivariable-adjusted p=0.03). Physical composite score and mean scores for seven of the eight SF-36 domains after 10-year follow-up were significantly lower among participants who self-reported hearing handicap at baseline. Differences in the adjusted means between participants with and without hearing handicap ranged from 2.7 (physical composite score) to 10.4 units ('role limitations due to physical problems' domain). Individuals who developed incident hearing impairment compared to those who did not, had adjusted mean scores 9.5- and 7.7-units lower in the 'role limitation due to physical problems', and 'bodily pain' domains, respectively, at the 10-year follow-up. Hearing aid users versus non-users at baseline showed a 1.82-point (p=0.03) and 3.32-point (p=0.01) increase in SF-36 mental composite score and mental health domain over the 10-year follow-up, respectively. Older adults with self-perceived hearing handicap constitute a potential risk group for overall deterioration in quality of life, while hearing aid use could help improve the well-being of hearing impaired adults. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

  4. Perception of Temporally Processed Speech by Listeners with Hearing Impairment

    PubMed Central

    Calandruccio, Lauren; Doherty, Karen A.; Carney, Laurel H.; Kikkeri, Harshavardhana N.

    2008-01-01

    Objectives The purpose of this study was to assess a new signal-processing strategy, Spatiotemporal Pattern Correction (SPC), for a group of listeners with varying degrees of sensorineural hearing loss. SPC is based on a physiological model of the level-dependent temporal response properties of auditory nerve (AN) fibers in normal and impaired ears. SPC attempts to “correct” AN response patterns by introducing time-varying group delays that differ across frequency channels. Listeners’ speech intelligibility and preference judgments were obtained at different strengths of SPC processing. Design Fifteen listeners, 12 with bilateral sensorineural hearing loss and 3 with normal hearing, participated in this study. Listeners with hearing impairment were separated into 3 groups (mild, moderate, and moderate-to-severe), based on their pure-tone averages at 0.5, 1, 2, and 4 kHz. All preference judgments were made using sentences from the Hearing-In-Noise-Test (HINT). The sentences were processed at five SPC strengths (1.0, 1.1, 1.2, 1.3, and 1.4). At an SPC strength of 1.0, the speech was processed through the SPC signal processor, becoming bandlimited, but no dynamic group delays were added to the signal. On each trial, listeners were presented a single sentence at two different SPC strengths, 1.0 (uncorrected) and a randomly selected SPC strength. Listeners were instructed to identify which of the two sentences they preferred. Sixteen vowel-consonant syllables, a subset of the Nonsense Syllable Test (NST), were used to assess speech intelligibility at three SPC strengths (1.0, 1.1, and 1.2). Listeners were asked to push one of 16 buttons on a response box that corresponded to the vowel-consonant syllable they heard. The moderate-to-severe hearing loss group was also tested using the low-probability sentences of the Speech-Perception-In-Noise (SPIN) test. Results Listeners with normal hearing and listeners with mild hearing loss preferred the quality of uncorrected

  5. 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.

  6. Mainstreaming hearing-impaired students: the effect of effort in communicating on cooperation and interpersonal attraction.

    PubMed

    Johnson, D W; Johnson, R T

    1985-01-01

    Cooperative and individualistic learning experiences were compared in terms of their effects on interaction and relationships between hearing and hearing-impaired students. Two contradictory hypotheses were tested--one stating that the effort required for hearing and hearing-impaired students to communicate would lead to frustration, withdrawal, exclusion, and rejection; the other stating that cooperative learning experiences would lead to cross-handicap interpersonal attraction regardless of communication difficulties. Thirty 3rd-grade students (20 hearing and 10 hearing impaired) were assigned to conditions on a stratified, random basis controlling for handicap, sex, and ability level. They participated in the study for 55 min a day for 15 instructional days. The results indicate that subjects involved in cooperative learning experiences performed higher on measures of interaction and interpersonal attraction between hearing and hearing-impaired students than did subjects involved in individualistic learning experiences.

  7. Assessing the Social Participation and Cognitive Play Abilities of Hearing-Impaired Preschoolers.

    ERIC Educational Resources Information Center

    Higginbotham, D. Jeffery; And Others

    1980-01-01

    Since several investigators have found language and play development to be interrelated, free play classifications were constructed for the assessment of social participation, cognitive play, and nonplayful activities for both normally hearing and hearing impaired preschoolers. (Author)

  8. Factors associated with third-party disability in spouses of older people with hearing impairment.

    PubMed

    Scarinci, Nerina; Worrall, Linda; Hickson, Louise

    2012-01-01

    This study had two aims: (1) to describe the extent of third-party disability in a sample of spouses of older people with hearing impairment, and (2) to investigate factors associated with third-party hearing disability. Third-party disability is defined as the disability and functioning of family members as a result of the health condition of their significant other. One hundred older spouses who had partners with hearing impairment participated. All assessments were self-reported, and included a set of measures (the 36-item Significant Other Scale for Hearing Disability, SOS-HEAR; the Relationship Assessment Scale; and the Significant Other Assessment of Communication) administered to the target population of normally hearing older spouses, and those administered to the hearing impaired partners of the spouses in the study (the Self Assessment of Communication). To address the first aim, descriptive statistics were used to describe the extent of third-party hearing disability in the spouses. To address the second aim, binary logistic regression analysis was used to examine factors associated with third-party hearing disability. Factors investigated were focused on the spouse (e.g., spouse perception of relationship satisfaction) and on the partners with hearing impairment (e.g., partners' level of hearing impairment). Most spouses (98%) reported some degree of third-party hearing disability on at least one item of the SOS-HEAR, with the majority reporting a mild disability. Three risk factors were found to be significantly associated with severe or complete third-party disability: lower relationship satisfaction as reported by the spouse (Relationship Assessment Scale), spousal age difference, and spouse perception of their partner's hearing disability (Significant Other Assessment of Communication). Partners' level of hearing impairment, self-reported hearing disability (Self Assessment of Communication), use of hearing aids, and spouse satisfaction that their

  9. 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

  10. 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". © Copyright by Società Italiana di Otorinolaringologia e Chirurgia Cervico-Facciale.

  11. 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.

  12. 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.

  13. 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.

  14. [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.

  15. 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:…

  16. 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.

  17. Adaptive psychological structure in childhood hearing impairment: audiological correlations.

    PubMed

    Serra, A; Spinato, G; Cocuzza, S; Licciardello, L; Pavone, P; Maiolino, L

    2017-06-01

    . On the contrary, in normal hearing children, the emotion 'fear' is the most difficult to identify. Deaf children seem to be more susceptible to recognition of visual emotions. Furthermore, deaf children present significant problem-solving skills and emotional recognition skills, possibly as a result of their hearing impairment. © Copyright by Società Italiana di Otorinolaringologia e Chirurgia Cervico-Facciale, Rome, Italy.

  18. Evidence-based practice in audiology: rehabilitation options for adults with hearing impairment.

    PubMed

    Hickson, Louise; Laplante-Lévesque, Ariane; Wong, Lena

    2013-12-01

    The authors address 3 questions: (a) What is evidence-based practice (EBP), and why is it important for adults with hearing impairment? (b) What is the evidence about intervention options for adults who fail a hearing screening and are identified with hearing impairment? (c) What intervention options do adults choose when identified with hearing impairment for the first time? The 5 steps of the EBP process are discussed in relation to a clinical question about whether hearing aids and communication programs reduce activity limitations and participation restrictions compared with no treatment for adults who fail a hearing screening and are identified with hearing impairment. Systematic reviews of the evidence indicate that both hearing aids and communication programs reduce activity limitations and participation restrictions for this population and are therefore appropriate options. A study is then described in which these options were presented to 153 clients identified with hearing impairment for the first time: 43% chose hearing aids, 18% chose communication programs, and the remaining 39% chose not to take any action. EBP supports the offer of intervention options to adults who fail a hearing screening and are identified with hearing impairment.

  19. The effect of hearing impairment in older people on the spouse: development and psychometric testing of the significant other scale for hearing disability (SOS-HEAR).

    PubMed

    Scarinci, Nerina; Worrall, Linda; Hickson, Louise

    2009-01-01

    The effects of hearing impairment on the person with the impairment and on their significant others are pervasive and affect the quality of life for all involved. The effect of hearing impairment on significant others is known as a third-party disability. This study aimed to develop and psychometrically test a scale to measure the third-party disability experienced by spouses of older people with hearing impairment. The Significant Other Scale for Hearing Disability (SOS-HEAR) was based on results of a previous qualitative study investigating the effect of hearing impairment on a spouse's everyday life. Psychometric testing with 100 spouses was conducted using item analysis, Cronbach's alpha, factor analysis, and test-retest reliability. Principal components analysis identified six key underlying factors. A combined set of 27 items was found to be reliable (alpha = 0.94), with weighted kappa for items ranging from fair to very good. The SOS-HEAR is a brief, easy to administer instrument that has evidence of reliability and validity. The SOS-HEAR could serve as a means of identifying spouses of older people with hearing impairment in need of intervention, directed towards either the couple or the spouse alone.

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

    PubMed

    Сhurbanov, Alexander Y; Karafet, Tatiana M; Morozov, Igor V; Mikhalskaia, Valeriia Yu; Zytsar, Marina V; Bondar, Alexander A; Posukh, Olga L

    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.

  1. 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

  2. Sentence intelligibility in noise for listeners with normal hearing and hearing impairment: influence of measurement procedure and masking parameters.

    PubMed

    Wagener, Kirsten Carola; Brand, Thomas

    2005-03-01

    Speech intelligibility measurements strongly depend on several procedural parameters. In order to obtain comparable results from different test procedures, these parameters must be investigated as to which should be standardized and which could be set freely. This study investigates the influence of noise level, noise type, and presentation mode on speech reception thresholds (SRTs), and intelligibility function slopes in noise for normal-hearing and hearing-impaired subjects. The noise presentation level had no significant influence on either SRTs or slope values, provided that the presentation level exceeded hearing threshold. Two stationary, speech-shaped noises produced identical results. Speech-simulating fluctuating noise yielded about 14 dB lower SRTs for normal-hearing subjects and about 10 dB lower SRTs for 20% of the heating-impaired subjects. Of the hearing-impaired subjects, 30% did not benefit from the modulations and showed similar SRTs as for stationary noise. Using continuous noise yielded lower SRTs compared to gated noise. However, the difference between the results in continuous and gated noise was not significant for the hearing-impaired subjects. A presentation level of 65 dB SPL (normal-hearing subjects) or 80 dB SPL (hearing-impaired subjects) and an interfering noise with a spectrum similar to the mean long-term average speech spectrum (LTASS) is suggested for comparable adaptive measurement procedures. A fluctuating, speech-shaped noise is recommended to differentiate between subjects.

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

    PubMed Central

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

    2016-01-01

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

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

    PubMed Central

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

    2013-01-01

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

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

    PubMed

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

    2013-01-01

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

  6. 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

  7. [Hearing impairment : successful communication as the main source of positive experiences].

    PubMed

    Eisenwort, B; Schlanitz, F; Niederkrotenthaler, T

    2010-05-01

    In recent years some studies could show that besides the negative effects of stressful encounters resulting from disorders and handicaps, positive experiences can also be made. The aim of this study was to determine which strategies can help persons with hearing impairments to gain positive experiences. A questionnaire in the German language consisting of the Trier Personality Inventory and a German translation of a questionnaire designed to measure the positive experiences in life with acquired hearing impairment was distributed to persons with acquired hearing impairment, who were recruited from hearing aid audiologists and ear nose and throat physicians. Persons with hearing impairments showed no differences to the general population with respect to their personality profile. Positive experiences are significantly more common by successful communication behavior than by other strategies. Successful communication helps persons with hearing impairments to gain positive experiences.

  8. On the PC Interface for Hearing-Impaired

    NASA Astrophysics Data System (ADS)

    Kitamura, Mitsuhiro; Akiyama, Kouichi; Hama, Hiromitsu

    2002-12-01

    A human being takes in the outer world information by using the five senses, and lives. So he is forced very inconvenient life even when one sense is missing. Among others, it is said that the sense of hearing has importance next to the sight, but it is one of the organs which surely become weak with aging, and "hearing defect" is particularly the problem which all people face someday. But, technically and socially, the system which the person whose physical function is poor can entry into and contribute to the society is very important. In this research, the way of making up for a lost function by the medium change to the sense of touch information from the auditory information is examined, as a help that hearing-impaired gets "safety", "independence", "the tranquility of the heart" from the technical side. In this paper, as a concrete system, it paid attention to the mouse of the PC interface, and a vibration mouse was used under the environment which a PC was being used for, and thought about building of the system which can acquire the sound information of the life environment in real time.

  9. 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,…

  10. 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,…

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

    PubMed

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

    2017-10-01

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

  12. Comparing participation in out of school activities between children with visual impairments, children with hearing impairments and typical peers.

    PubMed

    Engel-Yeger, Batya; Hamed-Daher, Shaima

    2013-10-01

    Hearing or visual impairments may negatively affect child's development and participation. Yet the literature about participation of children with hearing or visual impairments is insufficient. The present study aimed to compare participation patterns of children with visual impairments to those of children with hearing impairments and to typical peers and to examine the correlations between participation and socio-demographic parameters in each group. Participants were 70 children between the ages of 6-11: 25 with hearing impairments, 20 with visual impairments and 25 typical peers. All children filled the Children's Assessment of Participation and Enjoyment (CAPE). This self-report refers to participation in daily out of school activities. Children with hearing or visual impairments showed significant limited participation compared to typical peers, expressed in lower number of activities, lower participation intensity; more activities performed at home and with someone else. The limited participation was more emphasized among children with visual impairments. Socio-demographic variables (age, mother's education and socio-economic level) correlated with participation dimensions in both study groups. In conclusion, children with hearing or visual impairments may have restricted participation in out of school activities. Socio-demographic parameters may play a role in encouraging child's participation. Participation among these populations should be further studied in order to assist service providers to create intervention programs together with the child, for enhancing his/her inclusion in the community. Copyright © 2013. Published by Elsevier Ltd.

  13. Spoken and Written Narratives in Swedish Children and Adolescents with Hearing Impairment

    ERIC Educational Resources Information Center

    Asker-Arnason, Lena; Akerlund, Viktoria; Skoglund, Cecilia; Ek-Lagergren, Ingela; Wengelin, Asa; Sahlen, Birgitta

    2012-01-01

    Twenty 10- to 18-year-old children and adolescents with varying degrees of hearing impairment (HI) and hearing aids (HA), ranging from mild-moderate to severe, produced picture-elicited narratives in a spoken and written version. Their performance was compared to that of 63 normally hearing (NH) peers within the same age span. The participants…

  14. 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…

  15. 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:…

  16. Opening Ears to the Performing Arts: A Guide to Serving the Hearing Impaired.

    ERIC Educational Resources Information Center

    Spero, Ruth L.; And Others

    Intended for coordinators of performing arts organizations who want to make their programs accessible to hearing impaired persons, the booklet presents a general overview of Project HEAR (Hearing Electronics Arts Research) and step-by-step instructions for establishing an access program. Information is provided in question-and-answer format. An…

  17. 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…

  18. 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:…

  19. Spectral Tilt Change in Stop Consonant Perception by Listeners with Hearing Impairment

    ERIC Educational Resources Information Center

    Alexander, Joshua M.; Kluender, Keith R.

    2009-01-01

    Purpose: To evaluate how perceptual importance of spectral tilt is altered when formant information is degraded by sensorineural hearing loss. Method: Eighteen listeners with mild to moderate hearing impairment (HI listeners) and 20-23 listeners with normal hearing (NH listeners) identified synthesized stimuli that varied in second formant…

  20. 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…

  1. 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...

  2. 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...

  3. 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...

  4. 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.

  5. 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.

  6. Does extracorporeal shock wave lithotripsy cause hearing impairment in children?

    PubMed

    Tuncer, Murat; Sahin, Cahit; Yazici, Ozgur; Kafkasli, Alper; Turk, Akif; Erdogan, Banu A; Faydaci, Gokhan; Sarica, Kemal

    2015-03-01

    We evaluated the possible effects of noise created by high energy shock waves on the hearing function of children treated with extracorporeal shock wave lithotripsy. A total of 65 children with normal hearing function were included in the study. Patients were divided into 3 groups, ie those becoming stone-free after 1 session of shock wave lithotripsy (group 1, 22 children), those requiring 3 sessions to achieve stone-free status (group 2, 21) and healthy children/controls (group 3, 22). Extracorporeal shock wave lithotripsy was applied with patients in the supine position with a 90-minute frequency and a total of 2,000 shock waves in each session (Compact Sigma, Dornier MedTech, Wessling, Germany). Second energy level was used with a maximum energy value of 58 joules per session in all patients. Hearing function and possible cochlear impairment were evaluated by transient evoked otoacoustic emissions test at 1.0, 1.4, 2.0, 2.8 and 4.0 kHz frequencies before the procedure, 2 hours later, and 1 month after completion of the first shock wave lithotripsy session in groups 1 and 2. In controls the same evaluation procedures were performed at the beginning of the study and 7 weeks later. Regarding transient evoked otoacoustic emissions data, in groups 1 and 2 there was no significant alteration in values obtained after shock wave lithotripsy compared to values obtained at the beginning of the study, similar to controls. A well planned shock wave lithotripsy procedure is a safe and effective treatment in children with urinary stones and causes no detectable harmful effect on hearing function. Copyright © 2015 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

  7. 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

  8. Low prevalence of hearing impairment among very low birthweight infants as detected by universal neonatal hearing screening

    PubMed Central

    Roth, D Ari‐Even; Hildesheimer, M; Maayan‐Metzger, A; Muchnik, C; Hamburger, A; Mazkeret, R; Kuint, J

    2006-01-01

    Objectives To (a) study the prevalence of hearing impairment in a cohort of very low birthweight (VLBW) infants and (b) evaluate the effectiveness of transient evoked otoacoustic emissions (TEOAE) as a first stage in‐hospital hearing screening tool in this population. Study design The study group was a cohort of 346 VLBW infants born in 1998–2000 at The Sheba Medical Center. The prevalence of hearing impairment in the study group was compared with that of all other newborn infants participating in a universal newborn hearing screening programme during the same period. To evaluate the effectiveness of TEOAE, a control group of 1205 healthy newborns who had no known risk factors for hearing impairment was selected. The results and follow up of hearing screening for these infants were examined retrospectively. Results Only one VLBW infant (0.3%) was diagnosed with bilateral sensory‐neural hearing loss. In addition, nine infants (2.7%) were diagnosed with conductive hearing loss. Bronchopulmonary dysplasia and low Apgar score were the most significant factors for predicting the occurrence of conductive hearing loss. The percentage of VLBW infants who successfully passed the in‐hospital TEOAE screening was 87.2, compared with 92.2% in the full term control group. No false negative cases were detected on follow up. Conclusions The study shows a low incidence of sensory‐neural hearing loss in a cohort of VLBW infants and a relatively high incidence of conductive hearing loss. TEOAE screening was found to be an effective first stage in‐hospital hearing screening tool in this population. PMID:16531449

  9. 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.

  10. [Articulation deficits in children with hearing impairment and children with specific language impairment - a matched-pairs-study].

    PubMed

    Keilmann, A; Klüsener, P; Freude, C

    2008-10-01

    Children with hearing impairment commonly present with articulation deficits. This is considered one main characteristic. 24 pairs of children (5;00 - 6;11 yrs) with articulation deficits in the context of a language disorder were matched in the severity of the language disorder, age, gender and nonverbal intelligence. Of each matched pair one child had a hearing impairment, the other a specific language impairment. Both groups were compared for their articulation. In children with hearing impairment the influence of the severity of the hearing loss was evaluated. In both groups the same type of articulation deficit for consonants was found. Both groups of children had most frequently problems with sibilants. Children with specific language impairment had slightly more severe articulation deficits. In unvoiced plosives children with specific language impairment demonstrated more often substitutions. The severity of the language disorder, but not the severity of the articulation deficit, was correlated with the degree of hearing loss. Articulation deficits in children with hearing impairment are slightly less than in children with specific language impairment, but follow the same pattern.

  11. 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.

  12. 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…

  13. 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…

  14. 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…

  15. 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…

  16. 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…

  17. 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…

  18. 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…

  19. 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)

  20. How Students with Hearing Impairments: Can Learn and Flourish in Your Music Classroom

    ERIC Educational Resources Information Center

    Butler, Maureen

    2004-01-01

    Activities in music class allow students, especially students with hearing impairments, to explore new means of expression and to enhance existing ones. Additional benefits may include increases in auditory awareness, cognitive ability, attention span, memory recall, and vocabulary. Students with hearing impairments can learn and flourish in music…

  1. 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)

  2. 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)

  3. 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...

  4. 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...

  5. 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...

  6. 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...

  7. The Future Is with Us: Servicing the Hearing Impaired Child and His Family in Rural Areas.

    ERIC Educational Resources Information Center

    Reese, E. Rosalie

    Hearing impaired children from birth to age 5 in rural areas throughout the state of Utah are served by a home-based program which trains parents to communicate effectively with their hearing impaired child. The program is based on the following premises: (1) the preschool years are critical for developing communication skills; (2) the home is the…

  8. 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…

  9. 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…

  10. 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…

  11. 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…

  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. Auditory/Vibratory Perception of Syllabic Structure in Words by Profoundly Hearing-Impaired Children.

    ERIC Educational Resources Information Center

    Zeiser, M. Lynn; Erber, Norman P.

    1977-01-01

    Sixty monosyllabic, disyllabic, and trisyllabic words were recorded and presented at different times through earphones and vibrators to 20 normal adults and 20 profoundly hearing impaired children (ages 8 to 15 years) to evaluate perception of number of syllables. Vibratory perception by profoundly hearing impaired and normal Ss and auditory…

  14. 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),…

  15. 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…

  16. Parents of Children with Hearing Impairment Accessing Counseling Services in Zimbabwe

    ERIC Educational Resources Information Center

    Charema, John; Eloff, Irma

    2011-01-01

    This paper explores how parents of children with hearing impairment access counseling services in Zimbabwe. A survey design was used in which a sample of 300 parents of children with hearing impairment completed a multi-item questionnaire. Interviews were then conducted with the 300 parent-participants in order to cross-check questionnaire…

  17. Learning Not to Listen: The Experiences of Musicians with Hearing Impairments

    ERIC Educational Resources Information Center

    Fulford, Robert; Ginsborg, Jane; Goldbart, Juliet

    2011-01-01

    The journey from playful musical exploration in childhood to an adult identity as a skilled musician is likely to be problematic for people with hearing impairments. Although a number of subjective accounts have been published, there is a lack of empirical research in the area. In this study, twelve musicians with hearing impairments were…

  18. Effects of signal level and spectral contrast on vowel formant discrimination for normal-hearing and hearing-impaired listeners.

    PubMed

    Woodall, Ashley; Liu, Chang

    2013-06-01

    The aim of this study was to determine whether increasing the overall speech level or the individual spectral contrasts of vowel sounds can improve vowel formant discrimination for listeners both with and without normal hearing. Thresholds of vowel formant discrimination were examined for the F2 frequencies of 3 American English vowels for listeners with and without normal hearing. Spectral contrasts of the F2 were enhanced by 3, 6, and 9 dB. Vowel stimuli were presented at 70 and 90 dB SPL. The thresholds of listeners with hearing impairment were reduced significantly after spectral enhancement was implemented, especially at 90 dB SPL, whereas normal-hearing listeners did not benefit from spectral enhancement. These results indicate that a combination of spectral enhancement of F2 and high speech level is most beneficial to improve vowel formant discrimination for listeners with hearing impairment.

  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. 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.

  1. Lexical tone perception ability of profoundly hearing-impaired children: performance of cochlear implant and hearing aid users.

    PubMed

    Lee, Kathy Y S; van Hasselt, Charles Andrew; Tong, Michael C F

    2010-09-01

    In tone languages such as Cantonese, a change in tone denotes a change in lexical meaning. The present study investigates the functional benefit of hearing devices in assisting tone perception among children with profound hearing impairment. Fifty-two children with profound hearing loss were categorized into two groups based on their primary type of hearing device - a hearing aid group and cochlear implant group. A 75-item tone identification test covering all 15 Cantonese tone contrast pairs was administered to each subject under two conditions - unaided (hearing devices turned off) and aided (devices turned on). The proportion of correct responses was computed as the total score for all items and subtotal contrast scores for each of the 15 tone contrasts. The results indicated no significant differences between the children wearing hearing aids and those with cochlear implants under the unaided or the aided condition (z = -0.91, p = 0.36; z = -0.40, p = 0.69, respectively). Regardless of the type of device used, the total scores under the aided condition were higher than those under the unaided condition (z = -3.55 for the hearing aid group; z = -4.87 for the cochlear implant group, both ps < 0.01). Children wearing hearing devices generally have a satisfactory functional gain in tone perception. No major observable difference was noted between children using cochlear implants and those using hearing aids. Tone contrast pairs with dissimilar fundamental frequency at onset and dissimilar tone contour patterns were more easily identified.

  2. Within-consonant perceptual differences in the hearing impaired ear

    PubMed Central

    Trevino, Andrea; Allen, Jont B.

    2013-01-01

    The consonant recognition of 17 ears with sensorineural hearing loss is evaluated for 14 consonants /p, t, k, f, s, ʃ , b, d, g, v, z, ʒ, m, n/+/ɑ/, under four speech-weighted noise conditions (0, 6, 12 dB SNR, quiet). One male and one female talker were chosen for each consonant, resulting in 28 total consonant-vowel test tokens. For a given consonant, tokens by different talkers were observed to systematically differ, in both the robustness to noise and/or the resulting confusion groups. Such within-consonant token differences were observed for over 60% of the tested consonants and all HI ears. Only when HI responses are examined on an individual token basis does one find that the error may be limited to a small subset of tokens with confusion groups that are restricted to fewer than three confusions on average. Averaging different tokens of the same consonant can raise the entropy of a listener's responses (i.e., the size of the confusion group), causing the listener to appear to behave in a less systematic way. Quantifying these token differences provides insight into HI perception of speech under noisy conditions and characterizes each listener's hearing impairment. PMID:23862835

  3. A computer-controlled vowel corrector for the hearing impaired.

    PubMed

    Povel, D J; Wansink, M

    1986-03-01

    The Vowel Corrector is a visual aid to be used in speech training of the hearing impaired. The display provides information about the identity of vowels spoken in isolation or in monosyllables. Vowels appear on a screen as differently located areas in a space, the coordinates of which are obtained from a dimensional analysis of vowel spectra. Resolving properties of the device, indexed by a measure of area overlap, are examined in detail. In particular, implications for differentiating utterances of male, female, and child speakers are studied. Results show that the device has promising features and is especially useful for exploring the vowel space as well as for learning a global differentiation of the vowels. The discrimination of spectrally similar vowels is limited, however. Ideas for future improvements are discussed.

  4. 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

  5. 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…

  6. Neuromagnetic evaluation of a communication support system for hearing-impaired patients.

    PubMed

    Nakagawa, Kei; Nakaishi, Shinichiro; Imura, Takeshi; Kawahara, Yumi; Hashizume, Akira; Kurisu, Kaoru; Yuge, Louis

    2017-08-16

    Hearing-impaired patients often encounter obstacles in communication. Not all of them wear hearing aids, citing issues with usage difficulty and discomfort in wearing. To overcome these difficulties, a new endeavor was started to improve sound intelligibility from the speaker's side. The present study objectively evaluated an intelligible-hearing (IH) loudspeaker by means of magnetoencephalography. Magnetic counterparts of mismatch negativity (MMNm) to pronunciation ('mi' and 'ni') were recorded and compared when they were transmitted from the IH loudspeaker and from a normal-hearing loudspeaker. On using the IH loudspeaker, the peak latency was found to be significantly shortened. In the case of hearing-impaired participants, marked MMNm responses were observed only when the IH loudspeaker was used. These findings suggest that improving sound intelligibility may be a supportive and rehabilitative approach for hearing-impaired patients.

  7. 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.

  8. Case studies of pronoun development in two hearing-impaired children: normal, delayed or deviant?

    PubMed

    Cole, E B; Oshima-Takane, Y; Yaremko, R L

    1994-01-01

    This paper is a study of first and second person pronoun development in the spoken language of two young hearing-impaired children. Pronoun development was examined over a period of 11 months, starting at the age of 29 and 28 months, to determine whether the children's acquisition of these pronouns would reflect normal, delayed or deviant patterns of development. Comparison of data from these children with data regarding normally developing children shows the hearing-impaired children's acquisition to be within normal expectations for hearing age and overall linguistic level, and only slightly delayed in terms of chronological age. These results lend support to the view that differences in the hearing-impaired child's language ability are probably the result of a relative lack of auditory and linguistic experience, rather than reorganisation of the hearing-impaired child's psychological and cognitive processing abilities.

  9. Auditory Function and Hearing Loss in Children and Adults with Williams Syndrome: Cochlear Impairment in Individuals with Otherwise Normal Hearing

    PubMed Central

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

    2010-01-01

    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 to 59.50 years. 63% 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 4000-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. PMID:20425785

  10. 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.

  11. What makes adults with hearing impairment take up hearing AIDS or communication programs and achieve successful outcomes?

    PubMed

    Laplante-Lévesque, Ariane; Hickson, Louise; Worrall, Linda

    2012-01-01

    Client involvement in health decision making, or shared decision making, is increasingly being advocated. For example, rehabilitation interventions such as hearing aids and communication programs can be presented as options to adults with hearing impairment seeking help for the first time. Our previous research focused on the predictors of intervention decisions when options were presented with a decision aid. However, not all participants took up the intervention they initially decided upon. Although it is interesting to understand what informs adults with hearing impairment's intervention decisions, it is their intervention uptake and outcomes which best represent the ultimate end result of the rehabilitation process. This prospective study investigated the predictors of uptake and of successful outcomes of hearing aids and communication programs in middle-aged and older adults with hearing impairment seeking help for the first time. Using shared decision making, 153 participants with hearing impairment (average of air conduction thresholds at 0.5, 1, 2, and 4 kHz greater than 25 dB HL in at least one ear) aged 50 yr and older were presented with intervention options: hearing aids, communication programs (group or individual), and no intervention. Each participant received a decision aid and had at least 1 wk to consider intervention options before the intervention decision was made. Outcome measures for both hearing aids and communication programs at 3 mo after intervention completion were benefit (measured with the Client-Oriented Scale of Improvement), composite outcomes (measured with the International Outcome Inventory), and reduction in self-reported hearing disability (measured with the Hearing Handicap Questionnaire). Multivariate analysis (logistic and linear regression) identified predictors of intervention uptake and of successful outcomes when all other variables were held constant. Almost a quarter of the 153 participants (24%) did not take up the

  12. [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.

  13. Sources of variability of speech, spatial, and qualities of hearing scale (SSQ) scores in normal-hearing and hearing-impaired populations.

    PubMed

    Moulin, Annie; Richard, Celine

    2016-01-01

    To identify and quantify sources of variability in scores on the speech, spatial, and qualities of hearing scale (SSQ) and its short forms among normal-hearing and hearing-impaired subjects using a French-language version of the SSQ. Multi-regression analyses of SSQ scores were performed using age, gender, years of education, hearing loss, and hearing-loss asymmetry as predictors. Similar analyses were performed for each subscale (Speech, Spatial, and Qualities), for several SSQ short forms, and for differences in subscale scores. One hundred normal-hearing subjects (NHS) and 230 hearing-impaired subjects (HIS). Hearing loss in the better ear and hearing-loss asymmetry were the two main predictors of scores on the overall SSQ, the three main subscales, and the SSQ short forms. The greatest difference between the NHS and HIS was observed for the Speech subscale, and the NHS showed scores well below the maximum of 10. An age effect was observed mostly on the Speech subscale items, and the number of years of education had a significant influence on several Spatial and Qualities subscale items. Strong similarities between SSQ scores obtained across different populations and languages, and between SSQ and short forms, underline their potential international use.

  14. 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

  15. Psychological impact of a genetic diagnosis on hearing impairment-An exploratory study.

    PubMed

    Oonk, A M M; Ariens, S; Kunst, H P M; Admiraal, R J C; Kremer, H; Pennings, R J E

    2017-05-29

    Genetic testing for hereditary hearing impairment has become more routinely available as a diagnostic tool in the outpatient clinic. However, little is known about the psychological impact of a genetic diagnosis. To evaluate this impact, an exploratory study was conducted. Prospectively, 48 individuals who underwent genetic testing for hereditary hearing impairment were included in this study. Study participants were asked to fill out the following questionnaires: Hospital Anxiety Depression Scale, Impact of Event Scale, Self-Efficacy 24, Illness Cognition Questionnaire and the Inventory for Social Reliance. Questionnaires were filled out on three occasions: before genetic testing, directly after counselling on either positive or negative test results, and six weeks thereafter. No significant differences were found between the group that received a genetic diagnosis for their hearing impairment and the group that did not. This study did not demonstrate differences between receiving a genetic diagnosis or not; however, special attention to psychological well-being should be offered to hearing-impaired patients who seek a genetic diagnosis for their hearing impairment. Additionally, the psychological impact of sensorineural hearing impairment might be greater than the impact of a genetic diagnosis itself. Based on the current exploratory study, there are no psychological reasons in favour of or against genetic testing for hereditary hearing impairment. © 2017 John Wiley & Sons Ltd.

  16. Audiovisual asynchrony detection and speech intelligibility in noise with moderate to severe sensorineural hearing impairment.

    PubMed

    Başkent, Deniz; Bazo, Danny

    2011-01-01

    The objective of this study is to explore the sensitivity to intermodal asynchrony in audiovisual speech with moderate to severe sensorineural hearing loss. Based on previous studies, two opposing expectations were an increase in sensitivity, as hearing-impaired listeners heavily rely on lipreading in daily life, and a reduction in sensitivity, as hearing-impaired listeners tend to be elderly and advanced age could potentially impair audiovisual integration. Adults with normal (N = 11, ages between 23 and 50 yrs) and impaired hearing (N = 11, ages between 54 and 81 yrs, the pure-tone average between 42 and 67 dB HL) participated in two experiments. In the first experiment, the synchrony judgments were recorded for varying intermodal time differences in audiovisual sentence recordings. In the second experiment, the intelligibility of audiovisual and audio-only speech was measured in speech-shaped noise, and correlations were explored between the synchrony window and intelligibility scores for individual listeners. Similar to previous studies, a sensitivity window on the order of a few hundred milliseconds was observed with all listeners. The average window shapes did not differ between normal-hearing and hearing-impaired groups; however, there was large individual variability. Individual windows were quantified by Gaussian curve fitting. Point of subjective simultaneity, a measure of window peak shift from the actual synchrony point, and full-width at half-maximum, a measure of window duration, were not correlated with participant's age or the degree of hearing loss. Points of subjective simultaneity were also not correlated with speech intelligibility scores. A moderate negative correlation that was significant at most conditions was observed between the full-width at half-maximum values and intelligibility scores. Contrary to either expectation per se, there was no indication of an effect of hearing impairment or age on the sensitivity to intermodal asynchrony in

  17. Motor Skills in Hearing Impaired Children with or without Cochlear Implant--A Systematic Review.

    PubMed

    Vidranski, Tihomir; Farkaš, Daria

    2015-07-01

    Hearing impairment is a major limitation in communication, and it can obstruct psychological development, development of social skills and motor development. Hearing impairment is the third most common contemporary chronic health condition, and it has become a public health problem. The effectiveness of problem solving in everyday life and in emergency situations depends greatly on the amount and quality of the motor programs. Therefore, it is evident that the normal motor development in persons with hearing impairment is essential for everyday life. The aim of this research is to analyze the available information pertaining to motor skills of hearing impaired children both with and without a cochlear implant (CI) and to analyze possibilities of influencing their motor skills. The relevant studies on motor skills of hearing impaired children both with and without CI were obtained by an extensive computer search of various databases using special keywords and extraction with respect to certain criteria, resulting in 22 studies. The overall results of this systematic review indicate that the children with hearing impairment exhibit suboptimal levels of motor skills especially balance. Very few studies compared children with hearing impairment with CI units and without CI units and the results of those studies are quite contradictory. Numerous studies have confirmed that the regular and appropriate physical exercise can improve motor skills of children with hearing impairment, especially balance. The fact that the development of motor skills is crucial for the child's interaction with the outside world, action, perception and acquisition of academic skills and other skills necessary for life shows the importance of motor skills development for children with hearing impairment.

  18. The effect of spatial separation on informational masking of speech in normal-hearing and hearing-impaired listeners

    NASA Astrophysics Data System (ADS)

    Arbogast, Tanya L.; Mason, Christine R.; Kidd, Gerald

    2005-04-01

    The ability to understand speech in a multi-source environment containing informational masking may depend on the perceptual arrangement of signal and masker objects in space. In normal-hearing listeners, Arbogast et al. [J. Acoust. Soc. Am. 112, 2086-2098 (2002)] found an 18-dB spatial release from a primarily informational masker, compared to 7 dB for a primarily energetic masker. This article extends the earlier work to include the study of listeners with sensorineural hearing loss. Listeners performed closed-set speech recognition in two spatial conditions: 0° and 90° separation between signal and masker. Three maskers were tested: (1) the different-band sentence masker was designed to be primarily informational; (2) the different-band noise masker was a control for the different-band sentence; and (3) the same-band noise masker was designed to be primarily energetic. The spatial release from the different-band sentence was larger than for the other maskers, but was smaller (10 dB) for the hearing-impaired group than for the normal-hearing group (15 dB). The smaller benefit for the hearing-impaired listeners can be partially explained by masker sensation level. However, the results suggest that hearing-impaired listeners can use the perceptual effect of spatial separation to improve speech recognition in the presence of a primarily informational masker. .

  19. Neuropsychological assessment of persons with physical disability, visual impairment or blindness, and hearing impairment or deafness.

    PubMed

    Hill-Briggs, Felicia; Dial, Jack G; Morere, Donna A; Joyce, Arthur

    2007-03-01

    Conducting assessment with individuals with physical disability, visual impairment or blindness, and hearing impairment or deafness poses significant challenges for the neuropsychologist. Although standards for psychological testing have been devised to address assessment of persons with disabilities, little research has been conducted to validate neuropsychological test accommodation and modification practices that deviate from standard test administration or to develop test parameters and interpretive guidelines specifically for persons with different physical or sensory disabilities. This paper reviews issues pertaining to neuropsychological test selection and administration, common accommodation and modification practices, test development and validation, and disability-related factors that influence interpretation of test results. Systematic research is needed to develop methodological parameters for testing and to ensure reliable and valid neuropsychological assessment practices for individuals with physical or sensory disabilities.

  20. 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.

  1. Phoneme recognition in vocoded maskers by normal-hearing and aided hearing-impaired listeners.

    PubMed

    Phatak, Sandeep A; Grant, Ken W

    2014-08-01

    This study compared modulation benefit for phoneme recognition obtained by normal-hearing (NH) and aided hearing-impaired (HI) listeners. Consonant and vowel recognition scores were measured using nonsense syllables in the presence of a steady-state noise and four vocoded speech maskers. Vocoded maskers were generated by modulating the steady-state noise, in either one or six frequency channels, with the speech envelope extracted from the speech of either a single talker or a four-talker babble. Aided HI listeners obtained lower consonant recognition scores than NH listeners in all masker conditions. Vowel recognition scores for aided HI listeners were comparable to NH scores, except in the six-channel vocoded masker conditions where they were relatively lower. Analysis using the extended speech intelligibility index developed by Rhebergen, Versfeld, and Dreschler [(2006). J. Acoust. Soc. Am. 120(6), 3988-3997] suggested that the signal-to-noise ratio deficit observed in aided HI listeners was largely due to uncompensated audibility loss. There was no significant difference between modulation masking release obtained by NH and aided HI listeners for both consonant and vowel recognition.

  2. Spectral peak resolution and speech recognition in quiet: Normal hearing, hearing impaired, and cochlear implant listeners

    NASA Astrophysics Data System (ADS)

    Henry, Belinda A.; Turner, Christopher W.; Behrens, Amy

    2005-08-01

    Spectral peak resolution was investigated in normal hearing (NH), hearing impaired (HI), and cochlear implant (CI) listeners. The task involved discriminating between two rippled noise stimuli in which the frequency positions of the log-spaced peaks and valleys were interchanged. The ripple spacing was varied adaptively from 0.13 to 11.31 ripples/octave, and the minimum ripple spacing at which a reversal in peak and trough positions could be detected was determined as the spectral peak resolution threshold for each listener. Spectral peak resolution was best, on average, in NH listeners, poorest in CI listeners, and intermediate for HI listeners. There was a significant relationship between spectral peak resolution and both vowel and consonant recognition in quiet across the three listener groups. The results indicate that the degree of spectral peak resolution required for accurate vowel and consonant recognition in quiet backgrounds is around 4 ripples/octave, and that spectral peak resolution poorer than around 1-2 ripples/octave may result in highly degraded speech recognition. These results suggest that efforts to improve spectral peak resolution for HI and CI users may lead to improved speech recognition.

  3. 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,…

  4. 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,…

  5. A comparison of vowel productions in prelingually deaf children using cochlear implants, severe hearing-impaired children using conventional hearing aids and normal-hearing children.

    PubMed

    Baudonck, Nele; Van Lierde, K; Dhooge, I; Corthals, P

    2011-01-01

    The purpose of this study was to compare vowel productions by deaf cochlear implant (CI) children, hearing-impaired hearing aid (HA) children and normal-hearing (NH) children. 73 children [mean age: 9;14 years (years;months)] participated: 40 deaf CI children, 34 moderately to profoundly hearing-impaired HA children and 42 NH children. For the 3 corner vowels [a], [i] and [u], F(1), F(2) and the intrasubject SD were measured using the Praat software. Spectral separation between these vowel formants and vowel space were calculated. The significant effects in the CI group all pertain to a higher intrasubject variability in formant values, whereas the significant effects in the HA group all pertain to lower formant values. Both hearing-impaired subgroups showed a tendency toward greater intervowel distances and vowel space. Several subtle deviations in the vowel production of deaf CI children and hearing-impaired HA children could be established, using a well-defined acoustic analysis. CI children as well as HA children in this study tended to overarticulate, which hypothetically can be explained by a lack of auditory feedback and an attempt to compensate it by proprioceptive feedback during articulatory maneuvers. Copyright © 2010 S. Karger AG, Basel.

  6. Provision, perception and use of trainable hearing aids in Australia: a survey of clinicians and hearing impaired adults.

    PubMed

    Walravens, Els; Keidser, Gitte; Hickson, Louise

    2016-12-01

    This study set out to obtain information on the impact of trainable hearing aids among clinicians and hearing aid users and candidates. Two online adaptive surveys were developed to evaluate provision, uptake and experience or expectation of trainable hearing aids. Responses from 259 clinicians, 81 hearing aid users and 23 candidates for hearing aids were included. Over half of the clinicians surveyed activated trainable features in hearing aids. Most of these clinicians activated trainable features for selected users and reported positive findings. Most commonly trainable features were not activated because the hearing aid controls had already been disabled for management or client preference. One-third reported that they had no access to trainable aids or they were unsure about the presence or activation of trainable features. The remaining clinicians never activated trainable features. One in five users reported having used trainable aids and 93% would train again. Over 85% of the remaining hearing-impaired adults were interested in trainable aids. Positive reports from most providers and users who had experience with the trainable feature support the provision of trainable aids to selected clients, pending more evidence-based data to support the clinical management of such devices.

  7. 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.

  8. Hearing Aid Use and Mild Hearing Impairment: Learnings from Big Data.

    PubMed

    Timmer, Barbra H B; Hickson, Louise; Launer, Stefan

    2017-09-01

    Previous research, mostly reliant on self-reports, has indicated that hearing aid (HA) use is related to the degree of hearing impairment (HI). No large-scale investigation of the relationship between data-logged HA use and HI has been conducted to date. This study aimed to investigate if objective measures of overall daily HA use and HA use in various listening environments are different for adults with mild HI compared to adults with moderate HI. This retrospective study used data extracted from a database of fitting appointments from an international group of HA providers. Only data from the participants' most recent fitting appointment were included in the final dataset. A total of 8,489 bilateral HA fittings of adults over the age of 18 yr, conducted between January 2013 and June 2014, were included. Participants were subsequently allocated to HI groups, based on British Society of Audiology and American Speech-Language-Hearing Association audiometric descriptors. Fitting data from participating HA providers were regularly transferred to a central server. The data, with all personal information except age and gender removed, contained participants' four-frequency average (at 500, 1000, 2000, and 4000 Hz) as well as information on HA characteristics and usage. Following data cleaning, bivariate and post hoc statistical analyses were conducted. The total sample of adults' average daily HA use was 8.52 hr (interquartile range [IQR] = 5.49-11.77) in the left ear and 8.51 hr (IQR = 5.49-11.72) in the right ear. With a few exceptions, there were no statistical differences between hours of HA use for participants with mild HI compared to those with moderate impairment. Across all mild and moderate HI groups, the most common overall HA usage was between 8 and 12 hr per day. Other factors such as age, gender, and HA style also showed no relationship to hours of use. HAs were used, on average, for 7 hr (IQR = 4.27-9.96) per day in quiet and 1 hr (IQR = 0.33-1.41) per

  9. Students with hearing impairment at a South African university: Self-identity and disclosure.

    PubMed

    Bell, Diane; Carl, Arend; Swart, Estelle

    2016-01-01

    A growing number of students with hearing loss are being granted access to higher education in South Africa due to the adoption of inclusive educational policies. However, available statistics indicate that participation by students with hearing impairments in higher education remains low and research suggests that support provisioning for those who do gain access is inadequate. This article aims to illustrate that the assumed self-identity of students with hearing impairment influences their choice to disclose their disability. The choice not to disclose their hearing loss prevents them from accessing the necessary reasonable accommodations and this in turn may affect their eventual educational success. Reported here is a qualitative descriptive case study at a South African university. Purposive sampling methods were employed. Data were gathered from in-depth interviews with seven students with hearing impairment ranging from moderate to profound, using spoken language. Constructivist grounded theory was used as an approach to the process of generating and transforming the data, as well as the construction of theory. All the student participants identified as having a hearing rather than a D/deaf identity cultural paradigm and viewed themselves as 'normal'. Linked to this was their unwillingness to disclose their hearing impairment and thus access support. It is crucially important for academic, support and administrative staff to be aware of both the assumed 'hearing' identity and therefore subsequent non-disclosure practices of students with a hearing impairment using the oral method of communication. Universities need to put measures in place to encourage students to voluntarily disclose their hearing impairment in order to provide more targeted teaching and learning support. This could lead to improved educational outcomes for students.

  10. Predictors of Rehabilitation Intervention Decisions in Adults with Acquired Hearing Impairment

    ERIC Educational Resources Information Center

    Laplante-Levesque, Ariane; Hickson, Louise; Worrall, Linda

    2011-01-01

    Purpose: This study investigated the predictors of rehabilitation intervention decisions in middle-age and older adults with acquired hearing impairment seeking help for the first time. Method: Using shared decision making, 139 participants were offered intervention options: hearing aids, communication programs (group or individual), and no…

  11. 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…

  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. Hearing Impairment Among Noise-Exposed Workers - United States, 2003-2012.

    PubMed

    Masterson, Elizabeth A; Bushnell, P Timothy; Themann, Christa L; Morata, Thais C

    2016-04-22

    Hearing loss is the third most common chronic physical condition in the United States, and is more prevalent than diabetes or cancer (1). Occupational hearing loss, primarily caused by high noise exposure, is the most common U.S. work-related illness (2). Approximately 22 million U.S. workers are exposed to hazardous occupational noise (3). CDC compared the prevalence of hearing impairment within nine U.S. industry sectors using 1,413,789 noise-exposed worker audiograms from CDC's National Institute for Occupational Safety and Health (NIOSH) Occupational Hearing Loss Surveillance Project (4). CDC estimated the prevalence at six hearing impairment levels, measured in the better ear, and the impact on quality of life expressed as annual disability-adjusted life years (DALYs), as defined by the 2013 Global Burden of Disease (GBD) Study (5). The mining sector had the highest prevalence of workers with any hearing impairment, and with moderate or worse impairment, followed by the construction and manufacturing sectors. Hearing loss prevention, and early detection and intervention to avoid additional hearing loss, are critical to preserve worker quality of life.

  14. 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…

  15. Present Audiological Issues in the Education of Hearing-Impaired Children in Japan.

    ERIC Educational Resources Information Center

    Onuma, Nakoi

    1996-01-01

    Presents an overview of hearing aid fitting procedures and auditory rehabilitation for hearing-impaired children in Japan. Reviews issues of audiological management in Japanese schools and discusses two kinds of simplified auditory tests. Finally, highlights several controversial issues surrounding different perspectives and methods of education…

  16. 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…

  17. 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…

  18. 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)

  19. 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)

  20. Preparing the Hearing-Impaired Social Studies Teacher: Special Challenge to a Special Group.

    ERIC Educational Resources Information Center

    Martin, David S.

    The paper focuses on the special adaptations made at Gallaudet College (Washington, DC) to train hearing impaired students to teach social studies to hearing students at the elementary and secondary levels. It is pointed out that all instruction at Gallaudet employs the simultaneous communication method of voice and signed English while using…

  1. 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…

  2. 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…

  3. 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)

  4. 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.

  5. 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)

  6. 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

  7. 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)

  8. 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)

  9. 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)

  10. An Alternative Strategy for Teaching Reading to Hearing-Impaired High School Students.

    ERIC Educational Resources Information Center

    Gamble, Harold W., III

    1983-01-01

    Discusses a teaching technique that requires students to retell stories in reading classes for hearing impaired high school students as a way to provide maximum student involvement in classes, while developing reading, organization, vocabulary, and comprehension skills. (EKN)

  11. Classification of the hearing impaired for independent living using the Vineland Adaptive Behavior Scale.

    PubMed

    Dunlap, W R; Sands, D I

    1990-12-01

    Training hearing-impaired persons in independent living skills has become a focus of education and rehabilitation programs for the hearing impaired. Yet, few programs and assessment instruments are designed to evaluate a person's potential for acquiring independent living skills. In this study, the Vineland Adaptive Behavior Scale was used to classify 118 hearing-impaired persons in groups based on their ability to be trained in independent living skills. Cluster analysis was used to group the subjects according to four domains: communication, daily living, socialization, and maladaptive behavior. The results indicate that the behavior scale can be used to classify hearing-impaired persons according to their ability to acquire independent living skills. The cluster analysis resulted in three groups. The persons in the lowest group did not have the most severe hearing losses, but they were more likely to have additional handicaps. This suggests that additional handicaps may be more important than degree of hearing loss in determining whether hearing-impaired persons can acquire independent living skills.

  12. The effect of cochlear implantation on nasalance of speech in postlingually hearing-impaired adults.

    PubMed

    Hassan, Sabah M; Malki, Khalid H; Mesallam, Tamer A; Farahat, Mohamad; Bukhari, Manal; Murry, Thomas

    2012-09-01

    Hypernasality is considered a prevalent speech abnormality that could significantly contribute to the unintelligibility of the hearing-impaired speakers. The aim of this study was to evaluate the effect of cochlear implantation and the duration of hearing loss on nasalance of speech of a postlingually impaired group of Saudi adult patients. Retrospective study. This study included 25 postlingually hearing-impaired patients who underwent cochlear implantation and 25 age-matched control subjects. Patients were divided into three groups according to the duration of hearing loss. The nasometric data of the hearing-impaired group were compared with the control group. Also, the preoperative values were compared with the postoperative values 6, 12, and 24 months after surgery. Significant differences were demonstrated between the preimplantation nasalance scores of the three subgroups and between the patients and control groups. There were statistically significant differences demonstrated between the pre- and the postimplantation nasalance values for the three groups of patients. Cochlear implantation appears to have significant effects on improving the nasalance of the speech of postlingually hearing-impaired adult patients. However, the degree of improvement might vary according to the duration of hearing loss the patients had preimplantation. Copyright © 2012 The Voice Foundation. Published by Mosby, Inc. All rights reserved.

  13. Sunrise alarm clock for the hearing impaired - biomed 2011.

    PubMed

    Follum, James D; Catchpole, Jennifer M

    2011-01-01

    The Sunrise Alarm Clock is a device designed to be more effective than standard alarm clocks and more pleasant than specialty devices in waking the hearing impaired. This is accomplished with the inclusion of visual, physical, and audio alarms. The visual alarm stimulus is created by manipulating the light output of a bedside lamp to mimic the sunrise. This is achieved by varying the duty cycle of a pulse width modulated signal supplied through a standard three-prong receptacle located on the side of the alarm clock. Physical alarms are in the form of wristbands containing vibrating motors. Finally, audio alarms are provided with both volume and pitch control to match the user’s specific needs. The entire system is designed with two users in mind by providing two independently controlled receptacles, wristbands, and audio systems. At the conclusion of development, a nearly fully functional prototype has been produced. The prototype’s audio and physical alarm system along with one visual alarm are fully functional. Shortcomings include poor timekeeping accuracy and problems clearly displaying the time. Even so, this development in sleep technology is capable of performing its task and waking its user with all three alarm systems.

  14. 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.

  15. Delivery of Services to Hispanic Families with Young Hearing-Impaired Children: One Model. Part I [and] Part 2.

    ERIC Educational Resources Information Center

    Goring, Mary Beth; Martindale, Maura E.

    The number of Hispanic school-age children with hearing impairments in the United States is rising. Hispanic parents who discover that their baby has a hearing impairment lack basic information about deafness and experience guilt feelings about the cause of their child's hearing loss. They often do not know how to obtain information or medical…

  16. Masker phase effects in normal-hearing and hearing-impaired listeners: Evidence for peripheral compression at low signal frequencies

    NASA Astrophysics Data System (ADS)

    Oxenham, Andrew J.; Dau, Torsten

    2004-10-01

    The presence of cochlear-based compression at low frequencies was investigated by measuring phase effects in harmonic maskers. In normal-hearing listeners, the amount of masking produced depends strongly on the phase relationships between the individual masker components. This effect is thought to be determined primarily by properties of the cochlea, including the phase dispersion and compressive input-output function of the basilar membrane. Thresholds for signals of 250 and 1000 Hz were measured in harmonic maskers with fundamental frequencies of 12.5 and 100 Hz as a function of the masker phase curvature. Results from 12 listeners with sensorineural hearing loss showed reduced masker phase effects, when compared with data from normal-hearing listeners, at both 250- and 1000-Hz signal frequencies. The effects of hearing impairment on phase-related masking differences were not well simulated in normal-hearing listeners by an additive white noise, suggesting that the effects of hearing impairment are not simply due to reduced sensation level. Maximum differences in masked threshold were correlated with auditory filter bandwidths at the respective frequencies, suggesting that both measures are affected by a common underlying mechanism, presumably related to cochlear outer hair cell function. The results also suggest that normal peripheral compression remains strong even at 250 Hz. .

  17. 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.

  18. Road Traffic Noise Exposure and Hearing Impairment Among Traffic Policemen in Surat, Western India

    NASA Astrophysics Data System (ADS)

    Tandel, B. N.; Macwan, J. E. M.

    2017-06-01

    Among all of the sources responsible for noise pollution, traffic related sources are of great environmental concern and increasing level of discomfort in urban areas with increasing traffic concentration. It will be a larger and serious problem in the future, if effective precautions are not taken addressed and attended to. Hence, a study was undertaken to measure the levels of road traffic noise at major intersections of Surat city, located in Western India and assess the hearing impairment among the exposed group of population i.e. the traffic policemen. Manual noise measurements and vehicle counts were performed at three of the busiest traffic intersections in the city for a period of 12 h. Audiometric screening was done on 46 policemen working at these intersections to determine the occurrence of hearing impairment. At all the three intersections, Leq measured was well above the permissible levels of 65 dBA for daytime. The Lmax recorded for three intersections were 84.6 dBA (07:36 pm), 87.3 dBA (06:32 pm) and 85.6 dBA (11:41 am). Audiometric screening showed that only 10 policemen (22%) had normal hearing. Slight, moderate and severe hearing impairment was recorded among 13 (28%), 18 (39%) and 5 (11%) policemen respectively. Of the total 46 policemen, none had profound impairment (deafness, hearing loss >81 dB). Of the 36 policemen having hearing impairment, 27 had bilateral impairment.

  19. [Hearing impairment and psychopathological disorders in children and adolescents. Review of the recent literature].

    PubMed

    Bailly, D; Dechoulydelenclave, M-B; Lauwerier, L

    2003-01-01

    Hearing impairment is a multifaceted condition with medical and social aspects. If the neuropsychiatric impact of deafness on children has been investigated by researchers from a variety of fields and backgrounds, their conclusion is that children with hearing impairment follow many different developmental pathways. The aim of this paper is to examine the relationships between hearing impairment and mental health and the effect of impaired communication on family development. From a review of the literature, the authors examine the relationships between hearing impairment and mental disorders in children and adolescents in terms of prevalence, clinical features and etiological factors. The fami-ly dynamics and the parents-child interactions were also explored. The assessment of psychiatric disorders in hearing-impaired children sets some methodological pro-blems. Accurate evaluation is hampered by the immature language exhi-bited by many hearing-impaired children and by the difficulties that may be encountered in establishing rapport if the child does not understand the examiner's verbal exchanges. Several authors point out the lack of communication skills and experiences with hearing-impaired children on the part of many examiners. In addition, delays have been observed for the development of social maturity in hearing-impaired children and the parents' descriptions may reflect their own worries, rather than the emotional-behavioral functioning of the child. The measurement of psychiatric symptoms is then compromised insofar as many of the assessment procedures are highly verbal and were standardized for normal-hearing children. These difficulties may explain that the pre-valence rates of mental disorders in hearing-impaired children and adolescents found in the literature vary from 15% to 60%. If autism and deafness may both confound each others' dia-gnosis, several studies also point out the high comorbidity observed between these 2 conditions. The significance

  20. Prevalence and independent risk factors for hearing impairment among very low birth weight infants.

    PubMed

    Wang, Chien-Ho; Yang, Chang-Yo; Lien, Reyin; Chu, Shih-Ming; Hsu, Jen-Fu; Fu, Ren-Huei; Chiang, Ming-Chou

    2017-02-01

    Although we've made big strides in perinatal and neonatal care, auditory handicap remains a serious complication in those who were born very premature. The aim was to determine the prevalence and analyze possible risk factors of hearing impairment in very-low-birth-weight (VLBW) infants. This was a retrospective study by reviewing medical records of all VLBW infants (BW ≤ 1500 g) admitted to NICU of Chang Gung Children's Hospital over 2 years period from Jan. 2010 to 2011. Brainstem auditory evoked potentials (BAEP) hearing screening was performed at 3 months postnatal corrective age and repeated if failed the 1st time, then refer to ENT doctor if BAEP confirmed abnormal. All VLBW infants examined for hearing impairment were included and data were retrieved retrospectively and analyzed for neonatal risk factors using logistic regression. Over the period, 309 VLBW infants were screened. Prevalence of uni- or bilateral hearing impairment was 3.9% (12/309; 95% CI 2.6-4.1). The mean corrective age on diagnosed of hearing impairment was 2.9 ± 1.1 (range 1-5) months. Mean gestational age was 27.9 weeks (SD 1.4) and mean birth weight was 1028 g (SD 180). By univariant analysis for hearing impairment, severe birth asphyxia, craniofacial anomalies, ventilator dependence, patent ductus arteriosus ligation, and use of postnatal ototoxins yielded good prediction of hearing impairment in this population. However, using multivariate analysis revealed that the only independent risk factors for hearing impairment were ototoxins (OR: 3.62; CI: 1.67-7.82), PDA ligation (OR: 4.96; CI: 2.34-10.52), craniofacial anomalies (OR: 3.42; CI: 1.70-6.88)and assisted prolonged use of oxygen at gestational age of >36 weeks (OR: 5.94; CI: 2.61-13.54). The incidence of hearing impairment among VLBW infants was 3.9%. Prolonged supplemental oxygen use is a marker for predicting hearing impairment; this requires detailed analysis of the pathophysiologic features, to reduce the prevalence of

  1. Students with hearing impairment at a South African university: Self-identity and disclosure

    PubMed Central

    2016-01-01

    Background A growing number of students with hearing loss are being granted access to higher education in South Africa due to the adoption of inclusive educational policies. However, available statistics indicate that participation by students with hearing impairments in higher education remains low and research suggests that support provisioning for those who do gain access is inadequate. Objectives This article aims to illustrate that the assumed self-identity of students with hearing impairment influences their choice to disclose their disability. The choice not to disclose their hearing loss prevents them from accessing the necessary reasonable accommodations and this in turn may affect their eventual educational success. Method Reported here is a qualitative descriptive case study at a South African university. Purposive sampling methods were employed. Data were gathered from in-depth interviews with seven students with hearing impairment ranging from moderate to profound, using spoken language. Constructivist grounded theory was used as an approach to the process of generating and transforming the data, as well as the construction of theory. Findings All the student participants identified as having a hearing rather than a D/deaf identity cultural paradigm and viewed themselves as ‘normal’. Linked to this was their unwillingness to disclose their hearing impairment and thus access support. Conclusion It is crucially important for academic, support and administrative staff to be aware of both the assumed ‘hearing’ identity and therefore subsequent non-disclosure practices of students with a hearing impairment using the oral method of communication. Universities need to put measures in place to encourage students to voluntarily disclose their hearing impairment in order to provide more targeted teaching and learning support. This could lead to improved educational outcomes for students. PMID:28730053

  2. Assistive hearing technologies among students with hearing impairment: factors that promote satisfaction.

    PubMed

    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, teacher-worn microphones, and student-worn microphones. The study included 153 deaf and hard-of-hearing students. All students communicated orally and were in inclusive schools from grades 5-10. The results suggest that males view hearing technology more positively than do females. Having severe hearing loss also promoted positive attitudes toward hearing aids and cochlear implants, but not toward microphones. The students with positive self-descriptions tended to be more satisfied with hearing aids or cochlear implants than the students with negative self-descriptions. The main factors promoting the use of hearing aids were severe hearing loss, positive attitudes toward hearing aids, and the sound quality of hearing aids.

  3. 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

  4. 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…

  5. 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…

  6. 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…

  7. 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…

  8. The implantable hearing device for sensorineural hearing impairment. The Hough Ear Institute experience.

    PubMed

    Baker, R S; Wood, M W; Hough, J V

    1995-02-01

    This article describes one group's experience in developing and implanting rare earth magnets for conductive hearing loss and also for sensorineural hearing loss. Success and failures from over a decade of work are highlighted.

  9. 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.

  10. 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

  11. 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.

  12. 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

  13. French-Canadian translation and validation of four questionnaires assessing hearing impairment and handicap.

    PubMed

    Vincent, Claude; Gagné, Jean-Pierre; Leroux, Tony; Clothier, Audrey; Larivière, Marianne; Dumont, Frédéric S; Gendron, Martine

    2017-04-01

    Questionnaires evaluating hearing impairment are available in English but there is a need for French standardised questionnaires for researchers as well as for audiologists and other clinicians. The objective of this study is to describe the translation and validation of four questionnaires that assess different aspects of hearing impairment and handicap among elders with hearing loss, by comparing the main score and psychometric evaluation of original and French-Canadian (FC) versions of the World Health Organization Disability Assessment Scale II (WHO-DAS II), the Screening Test for Hearing Problems (STHP), the Abbreviated Profile of Hearing Aid Benefit (APHAB) and the Measure of Audiologic Rehabilitation Self-Efficacy for Hearing Aids (MARS-HA). Vallerand method: translation and back-translation by two translators, revision by a committee of experts and pre-tested with five bilingual older participants. Participants (n = 29) were 65 years of age or older including 21 with hearing aids. The psychometric properties (internal consistency, temporal stability after four weeks) indicate good reliability for most of the translated questionnaires and their subscales, especially the WHO-DAS II. The translations in FC of two hearing loss and two hearing aid questionnaires were validated. It is recommended to pursue the demonstration for temporal stability for the STHP.

  14. An overview of hearing impairment in older adults: perspectives for rehabilitation with hearing aids.

    PubMed

    Natalizia, A; Casale, M; Guglielmelli, E; Rinaldi, V; Bressi, F; Salvinelli, F

    2010-03-01

    Hearing loss is a common problem in modern society due to the combined effects of noise, aging, disease, and heredity. According to 2005 estimates by the World Health Organization (WHO), 278 million people worldwide have moderate to profound hearing loss in both ears. Incidence increases with age. Approximately 31.4% of people over age 65 have hearing loss and 40% to 50% of people 75 and older have a hearing loss. Only 1 out of 5 people who could benefit from a hearing aids actually wears one. To review literature for articles that focus on hearing aids. Hearing aids have continuously evolved over the past 50 years, in term of styles and technology. Technological advances in hearing aids and HATS (Hearing Assistive Technologies, and Rehabilitation Services) have expanded the range of options available to improve the success of a device use. Today's hearing aids differ significantly from their analog predecessors because the application of digital signal processing has permitted many adaptive and/or automatic features. Included in the benefits of digital hearing aids are improved sound quality, multiple listening programs for different listening environments, advanced noise reduction strategies, acoustic feedback reduction, compatibility with remote control options, and flexibility in manipulation of the frequency, compression, and gain. The hearing aids continue to be developed to enhance the characteristics in terms of rehabilitation and acceptability.

  15. 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

  16. Collaborative consultation: a method for improving educational services for mainstreamed students who are hearing impaired.

    PubMed

    Luckner, J L; Rude, H; Sileo, T W

    1989-12-01

    As a result of well-known recent federal mandates for appropriate special education to all disabled students, the majority of children who are hearing impaired currently are educated in public school settings, along with hearing students. Consequently, teachers of students who are hearing impaired have expanded their role to include the provision of ongoing support to regular classroom teachers, speech-language pathologists, paraprofessionals and administrators. One model for delivery of these services is that of collaborative consultation. The purpose of this paper is to provide an overview of collaborative consultation, and to delineate some principles that merit consideration by regular and special educators who are involved in the education of students who are hearing impaired.

  17. The factors associated with a self-perceived hearing handicap in elderly people with hearing impairment--results from a community-based study.

    PubMed

    Chang, Hsin-Pin; Ho, Chin-Yu; Chou, Pesus

    2009-10-01

    Elderly persons with a physiologic hearing deficit (hearing impairment) are not necessarily socially or emotionally disturbed by the deficit in everyday life (hearing handicap). The self-perception of a hearing handicap in elderly people is a key element in seeking consultation for a hearing impairment or using hearing aids. Thus, it is important to determine the factors associated with the self-perception of a hearing handicap. The aims of the present study were to report the relation between a hearing impairment and the self-perception of a hearing handicap, and the factors associated with a self-perceived hearing handicap among a group of randomly recruited, community-dwelling elderly persons, aged 65 yr and older, in Taipei, Taiwan. A cross-sectional survey of community-dwelling elderly persons aged 65 yr and older (N = 1220) participating in an annual general purpose geriatric health examination in 2005 in Taipei. Pure-tone audiometry and a questionnaire including the Hearing Handicap Inventory for the Elderly-Screening Version (HHIE-S) were administered, after obtaining the subject's consent to participate in the study. Demographic information, lifestyle, self-report health status, and biochemical data were also collected. There was a moderate association (gamma(s) = 0.52) between hearing impairment and self-perceived handicap. Only 21.4% of the study subjects with moderate to profound hearing impairment (M4 >or=41 dB HL, N = 555) perceived themselves as hearing-handicapped (HHIE-S total score >or=10). Besides hearing level, marital status (widowed) and self-perceived general health (bad or neutral) were factors that are significantly associated with a self-perceived hearing handicap among elderly subjects with moderate to profound hearing impairment. For study subjects with moderate to profound hearing impairment (M4 >or=41 dB HL), 5.0% of those with HHIE-S <10 and 45.4% of those with HHIE-S >or=10 used or felt that they required hearing aids (chi2 test, p

  18. Perception of quality of life for adults with hearing impairment in the LGBT community.

    PubMed

    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 80 adults with hearing impairment who do not self-identify as being members of the LGBT community (comparison group) participated in this study. The mean age of the participants in the LGBT group was 64.65 years, and the mean age for the comparison group was 63.26 years. Demographic information about the participants included: self-identification in the LGBT community, age, current and previous gender, relationship status, and hearing aid use. Participants received an audiometric evaluation and completed a disease-specific QoL instrument (Hearing Handicap Inventory for the Elderly/Adults) and a generic QoL instrument (Short-Form 36 Health Survey). The results from this study indicate that factors such as gender, relationship status, and hearing aid use are related to the perception of QoL for individuals with hearing impairment who are members of the LGBT community. Differences were found between the participants in the LGBT group and the comparison group. After reading this article, readers should be able to: (1) describe the disease-specific QoL affects of hearing loss on members of the LGBT community compared with a group of adults with hearing impairment who are not members of the LGBT community, (2) describe the gender diffferences in perception of QoL for members of the LGBT community and participants not in the LGBT community, and (3) discuss clinical implications of the findings. Copyright © 2012 Elsevier Inc. All rights reserved.

  19. 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)…

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

    ERIC Educational Resources Information Center

    Kahn, Gerri; Goldman, Ellen

    1991-01-01

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

  1. 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)

  2. High Prevalence of Hearing Impairment in HIV-Infected Peruvian Children

    PubMed Central

    Chao, Christina K.; Czechowicz, Josephine A.; Messner, Anna H.; Alarcón, Jorge; Roca, Lenka Kolevic; Rodriguez, Marsi M. Larragán; Villafuerte, César Gutiérrez; Montano, Silvia M.; Zunt, Joseph R.

    2012-01-01

    Objectives To measure the prevalence and to identify risk factors of hearing impairment in human immunodeficiency virus-infected children living in Peru. Study design Cross-sectional observational study. Setting Two public hospitals and 1 nonprofit center in Lima, Peru, between August 2009 and April 2010. Subjects A total of 139 HIV-infected children, ages 4 to 19 years. Methods Hearing impairment and otologic health were assessed with pure tone audiometry, tympanometry, and otoscopy. The primary outcome was hearing loss, defined as average threshold >25dB for 0.5, 1, 2, and 4 kHz, in one or both ears. Historical and socioeconomic information was obtained through parental survey and medical chart review. Statistical analysis included univariate analysis and multivariate logistic regression. Results Fifty-four (38.8%) of 139 children had hearing impairment. On multivariate analysis, risk factors included: tympanic membrane perforation (odds ratio [OR] 7.08; 95% confidence interval [CI], 1.65-30.5; P = .01), abnormal tympanometry (OR 2.71; 95% CI, 1.09-6.75; P = .03), cerebral infection (OR 11.6; 95% CI, 1.06-126; P = .05), seizures (OR 5.20; 95% CI, 1.21-22.4; P = .03), and CD4 cell count <500 cells/mm3 (OR 3.53; 95% CI, 1.18-10.5; P = .02). Conclusions The prevalence of hearing impairment in HIV-infected children in Lima, Peru was 38.8%. Middle ear disease, prior cerebral infection, and low CD4 cell count were significantly associated with hearing impairment. The high prevalence of hearing impairment emphasizes the need for periodic hearing assessment in the routine clinical care of HIV-infected children. PMID:22128111

  3. High prevalence of hearing impairment in HIV-infected Peruvian children.

    PubMed

    Chao, Christina K; Czechowicz, Josephine A; Messner, Anna H; Alarcón, Jorge; Kolevic Roca, Lenka; Larragán Rodriguez, Marsi M; Gutiérrez Villafuerte, César; Montano, Silvia M; Zunt, Joseph R

    2012-02-01

    To measure the prevalence and to identify risk factors of hearing impairment in human immunodeficiency virus-infected children living in Peru. Cross-sectional observational study. Two public hospitals and 1 nonprofit center in Lima, Peru, between August 2009 and April 2010. A total of 139 HIV-infected children, ages 4 to 19 years. Hearing impairment and otologic health were assessed with pure tone audiometry, tympanometry, and otoscopy. The primary outcome was hearing loss, defined as average threshold >25dB for 0.5, 1, 2, and 4 kHz, in one or both ears. Historical and socioeconomic information was obtained through parental survey and medical chart review. Statistical analysis included univariate analysis and multivariate logistic regression. Fifty-four (38.8%) of 139 children had hearing impairment. On multivariate analysis, risk factors included: tympanic membrane perforation (odds ratio [OR] 7.08; 95% confidence interval [CI], 1.65-30.5; P = .01), abnormal tympanometry (OR 2.71; 95% CI, 1.09-6.75; P = .03), cerebral infection (OR 11.6; 95% CI, 1.06-126; P = .05), seizures (OR 5.20; 95% CI, 1.21-22.4; P = .03), and CD4 cell count <500 cells/mm(3) (OR 3.53; 95% CI, 1.18-10.5; P = .02). The prevalence of hearing impairment in HIV-infected children in Lima, Peru was 38.8%. Middle ear disease, prior cerebral infection, and low CD4 cell count were significantly associated with hearing impairment. The high prevalence of hearing impairment emphasizes the need for periodic hearing assessment in the routine clinical care of HIV-infected children.

  4. 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.

  5. Protein energy malnutrition associates with different types of hearing impairments in toddlers: Anemia increases cochlear dysfunction.

    PubMed

    Kamel, Terez Boshra; Deraz, Tharwat Ezzat; Elkabarity, Rasha H; Ahmed, Rasha K

    2016-06-01

    This work aimed to highlight a challenging asymptomatic problem which is early detection of hearing impairment in toddlers with protein energy malnutrition (PEM) as a neuro-cognitive effect of PEM on developing brain in relation to hemoglobin level. 100 toddlers, aged 6-24 months, fifty with moderate/severe PEM and fifty healthy children, were included in study. Both TEOAEs and ABR testing were used to assess auditory function. Study reported an association between malnutrition and hearing impairment, 26% of cases had conductive deafness secondary to otitis media with effusion using tympanometry; 84.6% showed type B and 15.4% type C which may suggest developing or resolving otitis media. Their ABR showed 46% mild and 53% moderate impairment. 32% of PEM cases had sensory neural hearing loss and with type (A) tympanometry. Those were assessed using ABR; 58% had mild, 34% moderate and 8% profound impairment. 10% of PEM cases had mixed hearing loss with 50% type B and 50% type C tympanometry and their ABR showed moderate to profound impairment. TEOAEs latencies at different frequencies correlate negatively with hemoglobin level. Toddlers with moderate/severe PEM had hearing impairments of different types and degrees. Neuro-physiological methods could be early and safe detectors of auditory disorders especially in high-risk toddlers. Anemia increases risk for auditory dysfunction. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  6. [Lexical tone perception in sensorineural hearing-impaired and auditory neuropathy spectrum disorder].

    PubMed

    Wang, Shuo; Dong, Ruijuan; Wang, Yuan; Liu, Dongxin; Chen, Jing; Wu, Yanjun

    2015-09-01

    This study was aimed at investigating the ability of lexical tone perception in listeners with sensorineural hearing impairment and auditory. neuropathy spectrum disorder. Three groups of subjects were recruited in this study, including 11 subjects with normal hearing, 14 subjects with sensorineural hearing impairment, and 25 subjects with auditory neuropathy spectrum disorder. Ten monosyllabic syllables were selected, and combined with four lexical tones which were made up of 40 tone tokens as the original test materials. Then, these original words were recorded using one adult male and one adult female native Beijing Mandarin speaker. The speakers were asked to record these 40 monosyllabic words multiple times, and the 80 tokens in which the durations of four tones in each monosyllabic word were within 5ms precision were chosen as the test tone tokens. The subjects were asked to perform a four-alternative forced-choice study and select which tone they had heard. The mean and standard deviation of the tone perception correct scores for normal-hearing subjects, subjects with sensorineural hearing impairment, and subjects with auditory neuropathy spectrum disorder were (97.3 ± 2.8)%, (88.0 ± 9.9)%, and (65.7 ± 17.1)%, respectively. Significant differences in tone perception scores were found to be between subjects with normal hearing and subjects with sensorineural hearing impairment (P < 0.01) and between subjects with auditory neuropathy spectrum disorder (P < 0.01). In addition, a significant difference was found to be between subjects with sensorineural hearing impairment and auditory neuropathy spectrum disorder (P < 0.05). A significantly negative correlation was observed between tone perception score and pure tone hearing thresholds for both subjects with sensorineural hearing loss (r = -0.756, P < 0.01) and subjects with auditory neuropathy spectrum disorder (r = -0.546, P < 0.01). As the hearing loss became more severe, the ability to perceive lexical

  7. Perception of a Self-Fitting Hearing Aid Among Urban-Dwelling Hearing-Impaired Adults in a Developed Country

    PubMed Central

    Keidser, Gitte; Hartley, Lisa

    2011-01-01

    A self-fitting hearing aid is a personal amplification device that is designed to be assembled, programmed, and fine-tuned by the user, without the need for additional equipment or professional support. A written description of the device was presented to 80 older adults with a hearing impairment, all of whom were residents of an urban area in a developed country. In response to a structured questionnaire, the majority of participants reported that the self-fitting hearing aid concept was a good idea (83%), would be of personal benefit (60%), and could be managed independently by the user (90%). Overall, half of the participant group agreed with all three statements. Two were uncertain about the concept, but none of the participants rejected it outright. There were no significant differences between the opinions of participants with previous hearing aid experience and those without. Participant responses to open-ended questions revealed that the main benefits of a self-fitting hearing aid were thought to be the ability to self-adjust the device’s settings (reported by 33% of participants) and increased convenience (20% of participants). The main drawback, mentioned by 25% of participants, was a preference for professional guidance through the fitting process. These results suggest that the self-fitting hearing aid may present as an alternative product in developed countries for those users who prefer to be in control of the fitting process. PMID:22079900

  8. How internet telephony could improve communication for hearing-impaired individuals.

    PubMed

    Mantokoudis, Georgios; Kompis, Martin; Dubach, Patrick; Caversaccio, Marco; Senn, Pascal

    2010-09-01

    To test, whether modern Internet telephony with a broadband transmission (0.1-8 kHz) of speech improves speech perception in comparison to conventional telephony (0.3-3.5 kHz) in hearing-impaired and normal-hearing adults. Experimental clinical study. Audiologic laboratory in a tertiary referral center in Bern, Switzerland. Twenty-one adult hearing-impaired patients consisting of 11 users of cochlear implants and 10 users of hearing aids were selected from the institution's database based on pure tone audiograms, speech perception scores, and device settings. Ten normal-hearing adults served as controls. Superiority trial of speech perception assessed with Internet versus conventional telephone quality and noninferiority trial of Internet telephone versus frequency restricted, uncompressed audio CD quality. A modern broadband codec was chosen to simulate Internet telephone quality, and ideal network conditions without packet loss were assumed. Speech perception scores of a monosyllabic word test in quiet and a sentence test in background noise assessed in different audio qualities. : Test scores were, on average, 15% (4-33) higher in the monosyllabic word and 25% (8-51) higher in the sentence test using Internet versus conventional telephone quality across all subject groups. Speech perception was not significantly different when Internet telephone quality was compared with high-cut CD quality. Internet telephony offers significantly improved speech perception to hearing-impaired and normal-hearing adults under ideal laboratory conditions through doubling the frequency range and through conserving audio quality during digital sound processing.

  9. Gender difference in the relationships between vision and hearing impairments and negative well-being.

    PubMed

    Harada, Sei; Nishiwaki, Yuji; Michikawa, Takehiro; Kikuchi, Yuriko; Iwasawa, Satoko; Nakano, Makiko; Ishigami, Ai; Saito, Hideyuki; Takebayashi, Toru

    2008-10-01

    To evaluate the association of hearing impairment, vision impairment and their combination (dual sensory impairment) with negative well-being such as depression, subjective poor health and the reduced functional ability in community-dwelling older adults, and to determine whether any association varies by gender. Between 2005 and 2006, we objectively examined vision and hearing impairment (using best-corrected visual acuity and pure-tone audiometric test) in 843 people aged 65 years and older (351 males, 492 females) in a rural Japanese town. Through a home visit interview survey using a structured questionnaire, we also collected information on depression (the five-item Geriatric Depression Scale), subjective poor health, and reduced functional activity (the Tokyo Metropolitan Institute of Gerontology's Index of Competence). We observed gender differences in the association between sensory impairment and depression. Multiple logistic regression analysis revealed that hearing impairment in males (adjusted odds ratio: 2.22, 95% confidence interval; 1.07-4.61) and vision impairment in females (1.91, 1.14-3.21) were related to depression. Vision impairment and dual sensory impairment were also associated with subjective poor health and reduced functional activity in both sexes. Sensory impairment is significantly associated with negative well-being in older persons, and its association with depression may differ between males and females.

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

    PubMed

    Shojaei, Elahe; Jafari, Zahra; Gholami, Maryam

    2016-01-01

    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. 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. 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[Formula: see text]0.05). Early identified/intervened hearing-impaired children had a notable preference in all assessed sub-tests and lingual gains. 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 word production.

  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. 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.

  13. 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. © The Author(s) 2015.

  14. How Hearing Impairment Affects Sentence Comprehension: Using Eye Fixations to Investigate the Duration of Speech Processing

    PubMed Central

    Kollmeier, Birger; Brand, Thomas

    2015-01-01

    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. PMID:25910503

  15. Association between hearing impairment and lower levels of physical activity in older adults.

    PubMed

    Gispen, Fiona E; Chen, David S; Genther, Dane J; Lin, Frank R

    2014-08-01

    To determine whether hearing impairment, highly prevalent in older adults, is associated with activity levels. Cross-sectional. National Health and Nutritional Examination Survey (2005-06). Individuals aged 70 and older who completed audiometric testing and whose physical activity was assessed subjectively using questionnaires and objectively using body-worn accelerometers (N=706). Hearing impairment was defined according to the speech-frequency (0.5-4 kHz) pure-tone average in the better-hearing ear (normal <25.0 dB, mild 25.0-39.9 dB, moderate or greater ≥40 dB). Main outcome measures were self-reported leisure time physical activity and accelerometer-measured physical activity. Both were quantified using minutes of moderate-intensity physical activity and categorized as inactive, insufficiently active, or sufficiently active. Ordinal logistic regression analyses were conducted and adjusted for demographic and cardiovascular risk factors. Individuals with moderate or greater hearing impairment had greater odds than those with normal hearing of being in a lower category of physical activity as measured according to self-report (OR=1.59, 95% CI=1.11-2.28) and accelerometry (OR=1.70, 95% CI=0.99-2.91). Mild hearing impairment was not associated with level of physical activity. Moderate or greater hearing impairment in older adults is associated with lower levels of physical activity independent of demographic and cardiovascular risk factors. Future research is needed to investigate the basis of this association and whether hearing rehabilitative interventions could affect physical activity in older adults. © 2014, Copyright the Authors Journal compilation © 2014, The American Geriatrics Society.

  16. Hearing impairment after childhood bacterial meningitis dependent on etiology in Luanda, Angola.

    PubMed

    Karppinen, Mariia; Pelkonen, Tuula; Roine, Irmeli; Cruzeiro, Manuel Leite; Peltola, Heikki; Pitkäranta, Anne

    2015-11-01

    Childhood bacterial meningitis (BM) damages hearing, but the potential of different agents to cause impairment in developing countries is poorly understood. We compared the extent of hearing impairment in BM caused by Haemophilus influenzae type b (Hib), Streptococcus pneumoniae or Neisseria meningitidis among children aged 2 months to 13 years in Luanda, Angola. Hearing of 685 ears of 351 (78%) survivors among 723 enrolled patients was tested by brainstem-evoked response audiometry on day 7 of hospitalization. The causative agent was sought by cerebrospinal fluid culture, PCR or the latex-agglutination test. Altogether, 45 (12%) of the survivors were deaf (threshold >80 dB), and 20 (6%) had a threshold of 80 dB. The incidence of any kind of hearing loss, with ≥60 dB, was 34% with Hib, 30% with S. pneumoniae, 19% with N. meningitidis and 33% with other bacteria. Examining all ears combined and using the ≥60 dB threshold, the agents showed dissimilar harm (P=0.005), Hib being the most frequent and N. meningitidis the most infrequent cause. Compared to other agents, S. pneumoniae more often caused deafness (P=0.025) and hearing impairment at ≥60 dB (P=0.017) in infants, whereas this level of hearing loss in older survivors was most commonly caused by Hib (P=0.031). BM among children in Angola is often followed by hearing impairment, but the risk depends on the agent. S. pneumoniae is a major problem among infants, whereas Hib is mainly a risk beyond 12 months. N. meningitidis impairs hearing less frequently. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  17. Trends in Audiologic Services to Hearing-Impaired Children.

    ERIC Educational Resources Information Center

    Spitzer, Jaclyn B.

    1981-01-01

    The responses to a questionnaire received from 80 speech and hearing centers revealed changes in the number of children seen and in audiologic services in two age groups, birth to 3 years old and 3 to 6 years old. (Author)

  18. Auditory and visual orienting responses in listeners with and without hearing-impairment

    PubMed Central

    Brimijoin, W. Owen; McShefferty, David; Akeroyd, Michael A.

    2015-01-01

    Head movements are intimately involved in sound localization and may provide information that could aid an impaired auditory system. Using an infrared camera system, head position and orientation was measured for 17 normal-hearing and 14 hearing-impaired listeners seated at the center of a ring of loudspeakers. Listeners were asked to orient their heads as quickly as was comfortable toward a sequence of visual targets, or were blindfolded and asked to orient toward a sequence of loudspeakers playing a short sentence. To attempt to elicit natural orienting responses, listeners were not asked to reorient their heads to the 0° loudspeaker between trials. The results demonstrate that hearing-impairment is associated with several changes in orienting responses. Hearing-impaired listeners showed a larger difference in auditory versus visual fixation position and a substantial increase in initial and fixation latency for auditory targets. Peak velocity reached roughly 140 degrees per second in both groups, corresponding to a rate of change of approximately 1 microsecond of interaural time difference per millisecond of time. Most notably, hearing-impairment was associated with a large change in the complexity of the movement, changing from smooth sigmoidal trajectories to ones characterized by abruptly-changing velocities, directional reversals, and frequent fixation angle corrections. PMID:20550266

  19. 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.

  20. ELECTROPHYSIOLOGY AND PERCEPTION OF SPEECH IN NOISE IN OLDER LISTENERS: EFFECTS OF HEARING IMPAIRMENT & AGE

    PubMed Central

    Billings, Curtis J.; Penman, Tina M.; McMillan, Garnett P.; Ellis, Emily

    2015-01-01

    Objectives Speech perception in background noise is difficult for many individuals and there is considerable performance variability across listeners. The combination of physiological and behavioral measures may help to understand sources of this variability for individuals and groups and prove useful clinically with hard-to-test populations. The purpose of this study was threefold: (1) determine the effect of signal-to-noise ratio (SNR) and signal level on cortical auditory evoked potentials (CAEPs) and sentence-level perception in older normal-hearing (ONH) and older hearing-impaired (OHI) individuals, (2) determine the effects of hearing impairment and age on CAEPs and perception; and (3) explore how well CAEPs correlate with and predict speech perception in noise. Design Two groups of older participants (15 ONH and 15 OHI) were tested using speech-in-noise stimuli to measure CAEPs and sentence-level perception of speech. The syllable /ba/, used to evoke CAEPs, and sentences were presented in speech-spectrum background noise at four signal levels (50, 60, 70, and 80 dB SPL) and up to seven SNRs (−10, −5, 0, 5, 15, 25, 35 dB). These data were compared between groups to reveal the hearing impairment effect and then combined with previously published data for 15 young normal-hearing individuals (Billings et al., 2013) to determine the aging effect. Results Robust effects of SNR were found for perception and CAEPs. Small but significant effects of signal level were found for perception, primarily at poor SNRs and high signal levels, and in some limited instances for CAEPs. Significant effects of age were seen for both CAEPs and perception, while hearing impairment effects were only found with perception measures. CAEPs correlate well with perception and can predict SNR50s to within 2 dB for ONH. However, prediction error is much larger for OHI and varies widely (from 6 to 12 dB) depending on the model that was used for prediction. Conclusions When background