Science.gov

Sample records for nonsyndromic hearing impairment

  1. Developing regional genetic counseling for southern Chinese with nonsyndromic hearing impairment: a unique mutational spectrum

    PubMed Central

    2014-01-01

    Background Racial and regional factors are important for the clinical diagnosis of non-syndromic hearing impairment. Comprehensive genetic analysis of deaf patients in different regions of China must be performed to provide effective genetic counseling. To evaluate the mutational spectrum of south Chinese families, we performed genetic analysis for non-syndromic hearing impairment in this population. Methods Complete clinical evaluations were performed on 701 unrelated patients with non-syndromic hearing impairment from six provinces in south China. Each subject was screened for common mutations, including SLC26A4 c.IVS7-2A > G, c.2168A > G; mitochondrial DNA m.1555A > G, m.1494C > T, m.7444G > A, m.7445A > G; GJB3 c.538C > T, c.547G > A; and WFS1 c.1901A > C, using pyrosequencing. GJB2 and SLC26A4 coding region mutation detection were performed using Sanger sequencing. Results Genetic analysis revealed that among the etiology of non-syndromic hearing impairment, GJB2, SLC26A4, and mitochondrial m.1555A > G mutations accounted for 18.0%, 13.1%, and 0.9%, respectively. Common mutations included GJB2 c.235delC, c.109G > A, SLC26A4 c.IVS7-2A > G, c.1229 T > C, and mitochondrial m.1555A > G. The total mutation rate was 45.1% in all patients examined in south China. Overall, the clear contribution of GJB2, SLC26A4, and mitochondrial m.1555A > G to the etiology of the non-syndromic deafness population in south China was 32.0%. Conclusions Our study is the first genetic analysis of non-syndromic hearing impairment in south China, and revealed that a clear genetic etiology accounted for 32.0% of non-syndromic hearing cases in patients from these regions. The mutational spectrum of non-syndromic hearing impairment in the south Chinese population provides useful and targeted information to aid in genetic counseling. PMID:24612839

  2. Nonsyndromic hereditary hearing loss.

    PubMed

    Alford, Raye L

    2011-01-01

    The etiology of hereditary hearing loss is extraordinarily complex. More than 400 genetic syndromes are associated with hearing loss and more than 140 genetic loci associated with nonsyndromic hearing loss have been mapped, with more than 60 genes identified to date. Hereditary hearing loss can be inherited as an autosomal dominant, autosomal recessive, X-linked or mitochondrial (maternally inherited) condition. The overlapping audiologic phenotypes associated with many genes and the variability and/or reduced, sometimes age-related, penetrance of some phenotypic features of syndromic hearing loss can complicate the distinction between various genetic causes of nonsyndromic hearing loss and between nonsyndromic and syndromic hearing loss, especially in childhood. Testing for individual genes associated with nonsyndromic hearing loss, beyond GJB2 which encodes Connexin 26, can become expensive and, without specific phenotypic features to guide selection of genes for testing (such as enlarged vestibular aqueducts, low frequency hearing loss or auditory neuropathy), it is not likely to yield an etiology. Advances in DNA sequencing and the rapid decline in the cost of sequencing presage the availability of testing that can identify the etiology in the majority of cases of genetic hearing loss. However, until comprehensive genetic testing of hearing loss is clinically available and cost-effective, thorough phenotypic and audiologic evaluation and careful documentation of risk factors, infectious exposures and patient and family medical history will continue to be important to efforts directed toward etiologic diagnosis. The complexities associated with interpretation of genetic test results, genetic counseling and genetic risk assessment make consultation with medical geneticists important for many patients.

  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. Common Molecular Etiologies Are Rare in Nonsyndromic Tibetan Chinese Patients with Hearing Impairment

    PubMed Central

    Huang, Shasha; Zuo, Lujie; Zhang, Guozheng; Song, Yueshuai; Wang, Guojian; Wang, Hongtian; Huang, Deliang; Han, Dongyi; Dai, Pu

    2012-01-01

    Background Thirty thousand infants are born every year with congenital hearing impairment in mainland China. Racial and regional factors are important in clinical diagnosis of genetic deafness. However, molecular etiology of hearing impairment in the Tibetan Chinese population living in the Tibetan Plateau has not been investigated. To provide appropriate genetic testing and counseling to Tibetan families, we investigated molecular etiology of nonsyndromic deafness in this population. Methods A total of 114 unrelated deaf Tibetan children from the Tibet Autonomous Region were enrolled. Five prominent deafness-related genes, GJB2, SLC26A4, GJB6, POU3F4, and mtDNA 12S rRNA, were analyzed. Inner ear development was evaluated by temporal CT. A total of 106 Tibetan hearing normal individuals were included as genetic controls. For radiological comparison, 120 patients, mainly of Han ethnicity, with sensorineural hearing loss were analyzed by temporal CT. Results None of the Tibetan patients carried diallelic GJB2 or SLC26A4 mutations. Two patients with a history of aminoglycoside usage carried homogeneous mtDNA 12S rRNA A1555G mutation. Two controls were homozygous for 12S rRNA A1555G. There were no mutations in GJB6 or POU3F4. A diagnosis of inner ear malformation was made in 20.18% of the Tibetan patients and 21.67% of the Han deaf group. Enlarged vestibular aqueduct, the most common inner ear deformity, was not found in theTibetan patients, but was seen in 18.33% of the Han patients. Common molecular etiologies, GJB2 and SLC26A4 mutations, were rare in the Tibetan Chinese deaf population. Conclusion The mutation spectrum of hearing loss differs significantly between Chinese Tibetan patients and Han patients. The incidence of inner ear malformation in Tibetans is almost as high as that in Han deaf patients, but the types of malformation vary greatly. Hypoxia and special environment in plateau may be one cause of developmental inner ear deformity in this population

  5. Genetic Epidemiology of Mitochondrial Pathogenic Variants Causing Nonsyndromic Hearing Loss in a Large Cohort of South Indian Hearing Impaired Individuals.

    PubMed

    Subathra, Mahalingam; Ramesh, Arabandi; Selvakumari, Mathiyalagan; Karthikeyen, N P; Srisailapathy, C R Srikumari

    2016-09-01

    Mitochondria play a critical role in the generation of metabolic energy in the form of ATP. Tissues and organs that are highly dependent on aerobic metabolism are involved in mitochondrial disorders including nonsyndromic hearing loss (NSHL). Seven pathogenic variants leading to NSHL have so far been reported on two mitochondrial genes: MT-RNR1 encoding 12SrRNA and MT-TS1 encoding tRNA for Ser((UCN)) . We screened 729 prelingual NSHL subjects to determine the prevalence of MT-RNR1 variants at position m.961, m.1555A>G and m.1494C>T, and MT-TS1 m.7445A>G, m.7472insC m.7510T>C and m.7511T>C variants. Mitochondrial pathogenic variants were found in eight probands (1.1%). Five of them were found to have the m.1555A>G variant, two others had m.7472insC and one proband had m.7444G>A. The extended relatives of these probands showed variable degrees of hearing loss and age at onset. This study shows that mitochondrial pathogenic alleles contribute to about 1% prelingual hearing loss. This study will henceforth provide the reference for the prevalence of mitochondrial pathogenic alleles in the South Indian population, which to date has not been estimated. The m.1555A>G variant is a primary predisposing genetic factor for the development of hearing loss. Our study strongly suggests that mitochondrial genotyping should be considered for all hearing impaired individuals and particularly in families where transmission is compatible with maternal inheritance, after ruling out the most common variants.

  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

    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. Hereditary Non-Syndromic Sensorineural Hearing Loss

    PubMed Central

    Schrijver, Iris

    2004-01-01

    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. PMID:15507665

  9. A new autosomal recessive nonsyndromic hearing impairment locus DFNB96 on chromosome 1p36.31-p36.13.

    PubMed

    Ansar, Muhammad; Lee, Kwanghyuk; Naqvi, Syed Kamran-Ul-Hassan; Andrade, Paula B; Basit, Sulman; Santos-Cortez, Regie Lyn P; Ahmad, Wasim; Leal, Suzanne M

    2011-12-01

    A novel locus for autosomal recessive nonsyndromic hearing impairment (ARNSHI), DFNB96, was mapped to the 1p36.31-p36.13 region. A whole-genome linkage scan was performed using DNA samples from a consanguineous family from Pakistan with ARNSHI. A maximum two-point logarithm of odds (LOD) score of 3.2 was obtained at marker rs8627 (chromosome 1: 8.34 Mb) at θ=0 and a significant maximum multipoint LOD score of 3.8 was achieved at 15 contiguous markers from rs630075 (9.3 Mb) to rs10927583 (15.13 Mb). The 3-unit support interval and the region of homozygosity were both delimited by markers rs3817914 (6.42 Mb) and rs477558 (18.09 Mb) and contained 11.67 Mb. Of the 125 genes within the DFNB96 interval, the previously identified ARNSHI gene for DFNB36, ESPN, and two genes that cause Bartter syndrome, CLCNKA and CLCNKB, were sequenced, but no potentially causal variants were identified.

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

    PubMed Central

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

    2010-01-01

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

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

    PubMed

    Du, Jihong; Deng, Jianhua

    2016-01-01

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

  12. 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

  13. 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.

  14. A novel missense mutation in the connexin30 causes nonsyndromic hearing loss.

    PubMed

    Wang, Wen-Hung; Liu, Yu-Fan; Su, Ching-Chyuan; Su, Mao-Chang; Li, Shuan-Yow; Yang, Jiann-Jou

    2011-01-01

    Dysfunctional gap junctions caused by GJB2 (CX26) and GJB6 (CX30) mutations are implicated in nearly half of nonsyndromic hearing loss cases. A recent study identified a heterozygous mutation, c.119C>T (p.A40V), in the GJB6 gene of patients with nonsyndromic hearing loss. However, the functional role of the mutation in hearing loss remains unclear. In this study, analyses of cell biology indicated that a p.A40V missense mutation of CX30 causes CX30 protein accumulation in the Golgi body rather than in the cytoplasmic membrane. The tet-on protein expression system was used for further study of mutant proteins in CX30 and CX30A40V co-expressions and in CX26 and CX30A40V co-expressions. The p.A40V missense mutation exerted a dominant negative effect on both normal CX30 and CX26, which impaired gap junction formation. Moreover, computer-assisted modeling suggested that this p.A40V mutation affects the intra molecular interaction in the hydrophobic core of Trp44, which significantly alters the efficiency of gap junction formation. These findings suggest that the p.A40V mutation in CX30 causes autosomal-dominant nonsyndromic hearing loss. These data provide a novel molecular explanation for the role of GJB6 in hearing loss.

  15. Hearing Impairment

    MedlinePlus

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

  16. 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

  17. TBC1D24 mutation causes autosomal-dominant nonsyndromic hearing loss.

    PubMed

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

    2014-07-01

    Hereditary hearing loss 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 nonsyndromic hearing loss (NSHL), we used OtoSCOPE® to exclude mutations in known deafness genes and then performed segregation mapping and whole-exome sequencing 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, syndromic hearing impairment associated with onychodystrophy, osteodystrophy, mental retardation, and seizures (DOORS syndrome), and a wide range of epileptic disorders. PMID:24729539

  18. 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. ...

  19. Hearing Impaired: Curriculum Guide.

    ERIC Educational Resources Information Center

    Alberta Dept. of Education, Edmonton.

    The curriculum guide is intended to assist families, school administrators, and teachers providing educational services to hearing impaired (HI) children in regular and special classes in Alberta, Canada. Explained in the introduction are such curriculum aspects as goals and purpose, population to be served, eligibility criteria, three…

  20. 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.

  1. Novel form of X-linked nonsyndromic hearing loss with cochlear malformation caused by a mutation in the type IV collagen gene COL4A6.

    PubMed

    Rost, Simone; Bach, Elisa; Neuner, Cordula; Nanda, Indrajit; Dysek, Sandra; Bittner, Reginald E; Keller, Alexander; Bartsch, Oliver; Mlynski, Robert; Haaf, Thomas; Müller, Clemens R; Kunstmann, Erdmute

    2014-02-01

    Hereditary hearing loss is the most common human sensorineural disorder. Genetic causes are highly heterogeneous, with mutations detected in >40 genes associated with nonsyndromic hearing loss, to date. Whereas autosomal recessive and autosomal dominant inheritance is prevalent, X-linked forms of nonsyndromic hearing impairment are extremely rare. Here, we present a Hungarian three-generation family with X-linked nonsyndromic congenital hearing loss and the underlying genetic defect. Next-generation sequencing and subsequent segregation analysis detected a missense mutation (c.1771G>A, p.Gly591Ser) in the type IV collagen gene COL4A6 in all affected family members. Bioinformatic analysis and expression studies support this substitution as being causative. COL4A6 encodes the alpha-6 chain of type IV collagen of basal membranes, which forms a heterotrimer with two alpha-5 chains encoded by COL4A5. Whereas mutations in COL4A5 and contiguous X-chromosomal deletions involving COL4A5 and COL4A6 are associated with X-linked Alport syndrome, a nephropathy associated with deafness and cataract, mutations in COL4A6 alone have not been related to any hereditary disease so far. Moreover, our index patient and other affected family members show normal renal and ocular function, which is not consistent with Alport syndrome, but with a nonsyndromic type of hearing loss. In situ hybridization and immunostaining demonstrated expression of the COL4A6 homologs in the otic vesicle of the zebrafish and in the murine inner ear, supporting its role in normal ear development and function. In conclusion, our results suggest COL4A6 as being the fourth gene associated with X-linked nonsyndromic hearing loss. PMID:23714752

  2. Novel form of X-linked nonsyndromic hearing loss with cochlear malformation caused by a mutation in the type IV collagen gene COL4A6.

    PubMed

    Rost, Simone; Bach, Elisa; Neuner, Cordula; Nanda, Indrajit; Dysek, Sandra; Bittner, Reginald E; Keller, Alexander; Bartsch, Oliver; Mlynski, Robert; Haaf, Thomas; Müller, Clemens R; Kunstmann, Erdmute

    2014-02-01

    Hereditary hearing loss is the most common human sensorineural disorder. Genetic causes are highly heterogeneous, with mutations detected in >40 genes associated with nonsyndromic hearing loss, to date. Whereas autosomal recessive and autosomal dominant inheritance is prevalent, X-linked forms of nonsyndromic hearing impairment are extremely rare. Here, we present a Hungarian three-generation family with X-linked nonsyndromic congenital hearing loss and the underlying genetic defect. Next-generation sequencing and subsequent segregation analysis detected a missense mutation (c.1771G>A, p.Gly591Ser) in the type IV collagen gene COL4A6 in all affected family members. Bioinformatic analysis and expression studies support this substitution as being causative. COL4A6 encodes the alpha-6 chain of type IV collagen of basal membranes, which forms a heterotrimer with two alpha-5 chains encoded by COL4A5. Whereas mutations in COL4A5 and contiguous X-chromosomal deletions involving COL4A5 and COL4A6 are associated with X-linked Alport syndrome, a nephropathy associated with deafness and cataract, mutations in COL4A6 alone have not been related to any hereditary disease so far. Moreover, our index patient and other affected family members show normal renal and ocular function, which is not consistent with Alport syndrome, but with a nonsyndromic type of hearing loss. In situ hybridization and immunostaining demonstrated expression of the COL4A6 homologs in the otic vesicle of the zebrafish and in the murine inner ear, supporting its role in normal ear development and function. In conclusion, our results suggest COL4A6 as being the fourth gene associated with X-linked nonsyndromic hearing loss.

  3. Novel form of X-linked nonsyndromic hearing loss with cochlear malformation caused by a mutation in the type IV collagen gene COL4A6

    PubMed Central

    Rost, Simone; Bach, Elisa; Neuner, Cordula; Nanda, Indrajit; Dysek, Sandra; Bittner, Reginald E; Keller, Alexander; Bartsch, Oliver; Mlynski, Robert; Haaf, Thomas; Müller, Clemens R; Kunstmann, Erdmute

    2014-01-01

    Hereditary hearing loss is the most common human sensorineural disorder. Genetic causes are highly heterogeneous, with mutations detected in >40 genes associated with nonsyndromic hearing loss, to date. Whereas autosomal recessive and autosomal dominant inheritance is prevalent, X-linked forms of nonsyndromic hearing impairment are extremely rare. Here, we present a Hungarian three-generation family with X-linked nonsyndromic congenital hearing loss and the underlying genetic defect. Next-generation sequencing and subsequent segregation analysis detected a missense mutation (c.1771G>A, p.Gly591Ser) in the type IV collagen gene COL4A6 in all affected family members. Bioinformatic analysis and expression studies support this substitution as being causative. COL4A6 encodes the alpha-6 chain of type IV collagen of basal membranes, which forms a heterotrimer with two alpha-5 chains encoded by COL4A5. Whereas mutations in COL4A5 and contiguous X-chromosomal deletions involving COL4A5 and COL4A6 are associated with X-linked Alport syndrome, a nephropathy associated with deafness and cataract, mutations in COL4A6 alone have not been related to any hereditary disease so far. Moreover, our index patient and other affected family members show normal renal and ocular function, which is not consistent with Alport syndrome, but with a nonsyndromic type of hearing loss. In situ hybridization and immunostaining demonstrated expression of the COL4A6 homologs in the otic vesicle of the zebrafish and in the murine inner ear, supporting its role in normal ear development and function. In conclusion, our results suggest COL4A6 as being the fourth gene associated with X-linked nonsyndromic hearing loss. PMID:23714752

  4. [Mapping of gene underlying autosomal dominant non-syndromic hearing loss(DFNA)].

    PubMed

    Sun, Han-Jun; Tao, Ran; Cheng, Jing; Yang, Shu-Zhi; Cao, Ju-Yang; Yu, Li-Ming; Hong, Meng-Di; Feng, Guo-Yin; Dai, Pu; Yuan, Hui-Jun; Han, Dong-Yi; He, Lin

    2006-12-01

    Hereditary non-syndromic sensorineural hearing loss is a genetically highly heterogeneous group of disorders. To date, at least 50 loci for autosomal dominant non-syndromic sensorineural hearing loss (DFNA) have been identified by linkage analysis. Here we report a huge family with late onset autosomal dominant hereditary non-syndromic hearing loss. In this family, 73 of 170 family members have been conducted physical examination, pure-tone audiometry, immittance testing and auditory brainstem response testing (ABR). The results indicated that 39 of 73 tested family members have sensorineural hearing loss in various degrees. No associated visible abnormalities in other systems were found in this family. After exclusion of the 14 known DFNA loci with markers from the Hereditary Hearing Loss Homepage (URL: http://dnalab-www.uia.ac.be/dnalab/hhh), a genome wide scan was carried out using 382 highly informative microsatellite markers at approximately 9.2 cM intervals throughout the genome. Linkage analysis was carried out under a fully penetrant autosomal dominant mode of inheritance with no phenocopies. A maximum two-point LOD score of 6.69 at theta=0 was obtained for marker D14S1040. Haplotype analysis placed the locus within a 7.6 cM genetic interval defined by marker D14S1021 and D14S70, overlapping with the DFNA9 locus. PMID:17138532

  5. 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 Poland are also…

  6. Narrative Schemata in Hearing-Impaired Readers.

    ERIC Educational Resources Information Center

    Knight, David L.

    The study involving 82 hearing and 78 hearing impaired undergraduates was undertaken to test the hypothesis that hearing impaired Ss would cluster prepositions into different sentence groups when operating from scrambled story presentations than would hearing Ss. It was also hypothesized that hearing impaired Ss would show different cluster…

  7. Deletion of the entire POU4F3 gene in a familial case of autosomal dominant non-syndromic hearing loss.

    PubMed

    Freitas, Érika L; Oiticica, Jeanne; Silva, Amanda G; Bittar, Roseli S M; Rosenberg, Carla; Mingroni-Netto, Regina C

    2014-03-01

    In 20% of cases, hereditary non-syndromic hearing loss has an autosomal dominant inheritance (ADNSHL). To date, more than 50 loci for ADNSHL have been mapped to different chromosomal regions. In order to verify whether genomic alterations contribute to the hearing loss etiology and to search for novel deafness candidate loci, we investigated probands from families with ADNSHL by oligonucleotide array-CGH. A deletion in the 5q32 region encompassing only one gene, POU4F3, which corresponds to DFNA15, was detected in one family. POU4F3 protein has an important role in the maturation, differentiation and survival of cochlear hair cells. Defects in these cells may therefore explain sensorineural hearing loss. Mutations in this gene have already been associated with autosomal dominant hearing loss but this is the first description of a germline POUF4F3 deletion associated with hearing impairment.

  8. [Hearing impairment and dementia].

    PubMed

    Kilimann, I; Óvari, A; Hermann, A; Witt, G; Pau, H W; Teipel, S

    2015-07-01

    The World Health Organization (WHO) burden of disease study identified dementia and hearing problems as leading causes of loss of quality of life in the industrial world. The prevalence of dementia and hearing problems increases in aging societies. Comorbidity of these two diseases causes increasing demands on healthcare systems. The similarity and possible interaction of symptoms renders diagnosis and therapy of dementia and hearing loss a challenge for neurologists, psychiatrists, ear, nose and throat (ENT) and hearing specialists. Knowledge of both diseases enables an early intervention and helps preserve participation in society and thereby reducing the risk of developing dementia. This paper focuses on the characteristics of the diagnosis and therapy of hearing problems and dementia.

  9. 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.

  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.

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

    PubMed

    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

  12. 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

  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. Evaluation of the pathogenicity of GJB3 and GJB6 variants associated with nonsyndromic hearing loss.

    PubMed

    Oh, Se-Kyung; Choi, Soo-Young; Yu, Song Hee; Lee, Kyu-Yup; Hong, Jeong Hwa; Hur, Sung Won; Kim, Sang Jeong; Jeon, Chang-Jin; Kim, Un-Kyung

    2013-01-01

    A number of genes responsible for hearing loss are related to ion recycling and homeostasis in the inner ear. Connexins (Cx26 encoded by GJB2, Cx31 encoded by GJB3 and Cx30 encoded by GJB6) are core components of gap junctions in the inner ear. Gap junctions are intercellular communication channels and important factors that are associated with hearing loss. To date, a molecular genetics study of GJB3 and GJB6 as a causative gene for hearing loss has not been performed in Korea. This study was therefore performed to elucidate the genetic characteristics of Korean patients with nonsyndromic sensorineural hearing loss and to determine the pathological mechanism of hearing loss by analyzing the intercellular communication function of Cx30 and Cx31 variants. Sequencing analysis of the GJB3 and GJB6 genes in our population revealed a total of nine variants, including four novel variants in the two genes. Three of the novel variants (Cx31-p.V27M, Cx31-p.V43M and Cx-30-p.I248V) and two previously reported variants (Cx31-p.V84I and Cx30-p.A40V) were selected for functional studies using a pathogenicity prediction program and assessed for whether the mutations were located in a conserved region of the protein. The results of biochemical and ionic coupling tests showed that both the Cx31-p.V27M and Cx31-p.V84I variants did not function normally when each was expressed as a heterozygote with the wild-type Cx31. This study demonstrated that two variants of Cx31 were pathogenic mutations with deleterious effect. This information will be valuable in understanding the pathogenic role of GJB3 and GJB6 mutations associated with hearing loss.

  15. 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

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

    PubMed

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

    2016-01-01

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

  17. 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.

  18. Mechanism of two novel human GJC3 missense mutations in causing non-syndromic hearing loss.

    PubMed

    Su, Ching-Chyuan; Li, Shuan-Yow; Yen, Yung-Chang; Nian, Jhih-Hao; Liang, Wei-Guang; Yang, Jiann-Jou

    2013-06-01

    Connexins (CXs), as a component of gap junction channel, are homologous four transmembrane-domain proteins, with numerous studies confirming their auditory functions. Among a cohort of patients having incurred non-syndromic hearing loss, we identified two novel missense mutations, p.R15G and p.L23H, in the GJC3 gene encoding CX30.2/CX31.3, as causally related to hearing loss in previous study. However, the functional alteration of CX30.2/CX31.3 caused by the mutant GJC3 gene remains unknown. In this study, we compared the intracellular distribution of mutant CX30.2/CX31.3 (p.R15G and p.L23H) with the wild-type (WT) protein in HeLa cells and the effect of the mutant protein had on those cells. Analytical results indicated that p.R15G and p.L23H mutant exhibited continuous staining along apposed cell membranes in the fluorescent localization assay, which is the same with the WT. Moreover, ATP release (hemichannel function) is less in HeLa cells carrying mutant GJC3 genes than those of WT expressing cells. We believe that although p.R15G and p.L23H mutants do not decrease the trafficking of CX proteins, mutations in GJC3 genes result in a loss of hemichannel function of CX30.2/CX31.3 protein, possibly causing hearing loss. Results of this study provide a novel molecular explanation for the role of GJC3 in hearing loss.

  19. 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

  20. 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.

  1. 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

  2. Genetic analysis of genes related to tight junction function in the Korean population with non-syndromic hearing loss.

    PubMed

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

    2014-01-01

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

  3. A novel frameshift mutation in KCNQ4 in a family with autosomal recessive non-syndromic hearing loss.

    PubMed

    Wasano, Koichiro; Mutai, Hideki; Obuchi, Chie; Masuda, Sawako; Matsunaga, Tatsuo

    2015-08-01

    Mutation of KCNQ4 has been reported to cause autosomal dominant non-syndromic hearing loss (DFNA2A) that usually presents as progressive hearing loss starting from mild to moderate hearing loss during childhood. Here, we identified a novel KCNQ4 mutation, c.1044_1051del8, in a family with autosomal recessive non-syndromic hearing loss. The proband was homozygous for the mutation and was born to consanguineous parents; she showed severe hearing loss that was either congenital or of early childhood onset. The proband had a sister who was heterozygous for the mutation but showed normal hearing. The mutation caused a frameshift that eliminated most of the cytoplasmic C-terminus, including the A-domain, which has an important role for protein tetramerization, and the B-segment, which is a binding site for calmodulin (CaM) that regulates channel function via Ca ions. The fact that the heterozygote had normal hearing indicates that sufficient tetramerization and CaM binding sites were present to preserve a normal phenotype even when only half the proteins contained an A-domain and B-segment. On the other hand, the severe hearing loss in the homozygote suggests that complete loss of the A-domain and B-segment in the protein caused loss of function due to the failure of tetramer formation and CaM binding. This family suggests that some KCNQ4 mutations can cause autosomal recessive hearing loss with more severe phenotype in addition to autosomal dominant hearing loss with milder phenotype. This genotype-phenotype correlation is analogous to that in KCNQ1 which causes autosomal dominant hereditary long QT syndrome 1 with milder phenotype and the autosomal recessive Jervell and Lange-Nielsen syndrome 1 with more severe phenotype due to deletion of the cytoplasmic C-terminus of the potassium channel.

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

    SciTech Connect

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

    2006-04-14

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

  5. 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

  6. 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…

  7. 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)

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

    PubMed Central

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

    2015-01-01

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

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

    PubMed

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

    2014-02-01

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

  10. 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.

  11. Applying Bilingual Models in Classrooms for the Hearing Impaired.

    ERIC Educational Resources Information Center

    Luetke-Stahlman, B.

    The author examines the use of oral bilingual models in programs for the hearing impaired, contending that the native language of a hearing impaired child of hearing parents is frequently sign language. It is suggested that classrooms for the hearing impaired could combine numerous languages (Signed English, Signing Exact English, and American…

  12. Parental suspicion and identification of hearing impairment.

    PubMed

    Watkin, P M; Baldwin, M; Laoide, S

    1990-08-01

    The contribution of parental suspicion in the original identification of a 16 year cohort of 171 children with varying degrees of hearing impairment who were screened and identified in childhood was studied. Only a quarter of the children with permanent hearing loss were identified as a result of parental concern. The presence of parental suspicion preceding the audiological diagnosis was also measured. Of the children with severe or profound deafness, the parents only suspected the presence of hearing loss in 44%. Parental suspicion was even lower for those with a mild or moderate permanent hearing loss, and for those with an otherwise symptomless conductive hearing loss caused by otitis media with effusion. Some parents did identify hearing impairment in their children, and parental suspicion should never be professionally disregarded. Most parents, however, experienced initial difficulty in recognising their children's hearing loss, even when the children were comparatively old. The study confirms the need to continue to identify deafness early by both parental vigilance and sensitive hearing screening programmes.

  13. 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)

  14. 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

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

    PubMed

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

    2015-08-01

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

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

    PubMed Central

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

    2015-01-01

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

  17. 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…

  18. 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…

  19. 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. Wierzchowska and R. Szymanska of Poland;…

  20. 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. Pronovost. Papers from the U.S.S.R.,…

  1. 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.

  2. 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…

  3. 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...

  4. 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...

  5. Diagnostic Evaluation and Educational Programing for Hearing Impaired Children.

    ERIC Educational Resources Information Center

    McCoy, George F.

    Various aspects of diagnostic evaluation and educational programing for hearing impaired children are described. Discussion of hearing disorders from a medical perspective covers anatomy of the ear, types and causes of hearing disorders, and medical and surgical treatment. A review of audiological assessment of hearing disorders focuses upon…

  6. 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)

  7. Intelligibility of digital speech masked by noise: Normal hearing and hearing impaired listeners

    NASA Astrophysics Data System (ADS)

    Nixon, Charles W.; Stephenson, Mark S.; McKinley, Richard L.; Fisher, Vernie G.; Jacobs, Michael J.

    1990-06-01

    The intelligibility in noise of normal and digital speech (ADPCM, CVSD, LPC-10 vocoders) was measured for normal hearing and hearing impaired listeners. The digitally coded speech was generally less intelligible than normal speech; however, the highest quality digital system provided speech that was similar in intelligibility to normal speech. The speech from some digital systems was more vulnerable to noise masking than from others. Hearing impaired persons with no prior experience listening to digital speech required more time to attain maximum listening performance than normal hearing listeners. The rank ordering of intelligibility of the three types of digital speech was the same for the hearing impaired as for the normal hearing listeners. Persons with moderate hearing loss will have greater difficulty than normal hearing listeners in understanding digital speech in noise. Personnel with hearing impairment using digital speech systems in operational noise environment may be contributing to voice communication problems attributed only to the digital speech.

  8. 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.

  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. Prevalence of Hearing Impairment in High Risk Infants.

    PubMed

    Vashistha, Ishika; Aseri, Yogesh; Singh, B K; Verma, P C

    2016-06-01

    Hearing impairment is prevalent in the general population, early intervention facilitates proper development. The aim of this study was to establish the prevalence of hearing impairment in high-risk infants born between 2013 and 2014. 100 newborns were evaluated using evoked otoacoustic emissions and distortion produce and auditory behavior. Tests were reported if the results were altered. If altered results persisted, the child was referred for impedance testing and when necessary for medical evaluation. Infants referred for BOA and OAE undergone Brainstem auditory evoked potential testing. Of 100 children, 85 children have hearing within normal limits. Hearing impairment was found in 15 out of which 7 had unilateral hearing loss and 8 had bilateral hearing loss. The high prevalence of hearing impairment in this population underlines the importance of early audiological testing.

  11. Prevalence of Hearing Impairment in High Risk Infants.

    PubMed

    Vashistha, Ishika; Aseri, Yogesh; Singh, B K; Verma, P C

    2016-06-01

    Hearing impairment is prevalent in the general population, early intervention facilitates proper development. The aim of this study was to establish the prevalence of hearing impairment in high-risk infants born between 2013 and 2014. 100 newborns were evaluated using evoked otoacoustic emissions and distortion produce and auditory behavior. Tests were reported if the results were altered. If altered results persisted, the child was referred for impedance testing and when necessary for medical evaluation. Infants referred for BOA and OAE undergone Brainstem auditory evoked potential testing. Of 100 children, 85 children have hearing within normal limits. Hearing impairment was found in 15 out of which 7 had unilateral hearing loss and 8 had bilateral hearing loss. The high prevalence of hearing impairment in this population underlines the importance of early audiological testing. PMID:27340640

  12. 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.

  13. Nonsyndromic Craniosynostosis

    PubMed Central

    Garza, Rebecca M.; Khosla, Rohit K.

    2012-01-01

    Nonsyndromic craniosynostosis is more commonly encountered than syndromic cases in pediatric craniofacial surgery. Affected children display characteristic phenotypes according to the suture or sutures involved. Restricted normal growth of the skull can lead to increased intracranial pressure and changes in brain morphology, which in turn may contribute to neurocognitive deficiency. Management has primarily focused on surgical correction of fused sutures prior to 12 months of age to optimize correction of the deformity and to ameliorate the effects of increased intracranial pressure. However, emphasis has recently shifted to better understanding the pathogenesis of neurocognitive impairment observed in these children, along with genetic mutations that contribute to premature suture fusion. Such understanding will provide opportunities for earlier and more specific neurocognitive interventions and for the development of targeted genetic therapy to prevent pathologic suture fusion. The authors review the common types of nonsyndromic craniosynostosis and the epidemiological, genetic, and neurodevelopmental details that are currently known from the literature. In addition, they present the rationale for surgical correction, offer suggestions for timing of intervention, and present some nuances of techniques that they find important in producing consistent results. PMID:23633932

  14. 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.

  15. Spectrum of GJB2 mutations in a cohort of nonsyndromic hearing loss cases from the Kingdom of Saudi Arabia.

    PubMed

    Al-Qahtani, Mohammed H; Baghlab, Ibtessam; Chaudhary, Adeel G; Abuzenadah, Adel M; Bamanie, Afaf; Daghistani, Kamal J; Safieh, Malek; Fida, Loai; Dallol, Ashraf

    2010-02-01

    Nonsyndromic hearing loss (NSHL) affects a substantial proportion of newborns in the world every year. This proportion increases proportionally with the degree of consanguineous marriages in any society. In the Kingdom of Saudi Arabia, consanguineous marriages are common practice and this is associated with a noticeably high frequency of inherited conditions affecting the resulting progeny, including NSHL. Until now there is no published data on the genetic causes of NSHL in Saudi Arabia, which greatly hindered the ability of local genetic counseling and family planning centers to distinguish between hereditary and nonhereditary forms of NSHL and subsequently could not give information on the possible inheritance of deafness. In addition, the lack of validated genetic tests for NSHL delayed the detection of deafness in affected individuals and may have lowered the efficiency of later medical interventions. Further, the population covered in this study is likely to have a multiethnic background caused by decades of religious and economic migration to this region. To address such problems, we undertook the task of unraveling the genetic causes of hearing loss in Saudi Arabia, starting with identifying the GJB2/DFNB1 mutation spectrum in a cohort of unrelated individuals suffering from mild to profound NSHL. A total of 12 reported GJB2 mutations were identified in 17 out of 109 (15.59%) NSHL cases. Biallelic GJB2 mutations were identified in 11 out of the 109 NSHL cases (10.09%), with c.35delG being the most common (7/11, 63.63%). The remaining six patients were found to have monoallelic GJB2 mutations. Interestingly, biallelic GJB2 mutations were not detected in patients of Arab tribal origins, reflecting the genetic heterogeneity of the western area of the Kingdom of Saudi Arabia. Therefore, ethnically targeted genetic screening for GJB2 mutations could be a useful tool toward the management of NSHL in this area. PMID:19929407

  16. 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

  17. Assessing the Semantic Language Development of Hearing Impaired Students.

    ERIC Educational Resources Information Center

    Luetke-Stahlman, B.

    1988-01-01

    Improvements in English-language skills made by hearing-impaired students can be assessed using the taxonomy of semantic development phases from L. Bloom and M. Lahey. Teachers are encouraged to transcribe, code, and analyze bimodal language samples and to write both form and content language objectives for hearing-impaired students. (JDD)

  18. 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.…

  19. Expanding Educational Services to the Hearing-Impaired.

    ERIC Educational Resources Information Center

    Boukouvalas, Costas S.; Gay, Melvin L.

    1979-01-01

    Reveals trends influencing the enrollment rate of hearing-impaired college students and discusses the elements of a comprehensive support service program. These include: planning; establishment of services, i.e., counseling, interpreting, note-taking, and tutorial services; and benefits to the institution, the hearing-impaired students, and…

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

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 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, whichever results in the higher numeral. That numeral will then be elevated to the next higher Roman numeral....

  1. 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, whichever results in the higher numeral. That numeral will then be elevated to the next higher Roman numeral....

  2. 38 CFR 4.86 - Exceptional patterns of hearing impairment.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 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, whichever results in the higher numeral. That numeral will then be elevated to the next higher Roman numeral....

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

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 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, whichever results in the higher numeral. That numeral will then be elevated to the next higher Roman numeral....

  4. 38 CFR 4.86 - Exceptional patterns of hearing impairment.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... the Roman numeral designation for hearing impairment from either Table VI or Table VIa, whichever... determine the Roman numeral designation for hearing impairment from either Table VI or Table VIa, whichever results in the higher numeral. That numeral will then be elevated to the next higher Roman numeral....

  5. Physical Education, Recreation and Sports for Individuals with Hearing Impairments.

    ERIC Educational Resources Information Center

    American Alliance for Health, Physical Education, and Recreation, Washington, DC.

    Reviewed in the third of seven related documents are resources and research on physical education, recreation, and sports for hearing impaired persons. An annotated list of resources is supplied for each of the following topics: integration of the hearing impaired into regular physical education and recreation programs, physical education and…

  6. 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…

  7. Evaluating Students with Hearing Impairment. Technical Assistance Document.

    ERIC Educational Resources Information Center

    New Jersey State Dept. of Education, Trenton. Div. of Special Education.

    This guide, written to assist child study teams in New Jersey, reviews legal and other requirements in evaluating students with hearing impairments. Implications of hearing impairment for the evaluation process include its impact on syntactic, semantic, and pragmatic aspects of language; environmental interaction; and social interaction.…

  8. Nonverbal Intelligence and Academic Achievement in the Hearing Impaired.

    ERIC Educational Resources Information Center

    Watson, Betty; And Others

    1986-01-01

    A study examining the relationship between nonverbal intelligence and academic achievement in 53 hearing impaired 7-18 year olds supported using the Hickey-Nebraska Test of Learning Aptitude (along with other measures) in interpreting individual differences in the academic achievement of hearing impaired students. (Author/CL)

  9. 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…

  10. 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.

  11. 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.

  12. 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.

  13. 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

  14. 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

  15. 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…

  16. Diversity of the causal genes in hearing impaired Algerian individuals identified by whole exome sequencing

    PubMed Central

    Ammar-Khodja, Fatima; Bonnet, Crystel; Dahmani, Malika; Ouhab, Sofiane; Lefèvre, Gaelle M; Ibrahim, Hassina; Hardelin, Jean-Pierre; Weil, Dominique; Louha, Malek; Petit, Christine

    2015-01-01

    The genetic heterogeneity of congenital hearing disorders makes molecular diagnosis expensive and time-consuming using conventional techniques such as Sanger sequencing of DNA. In order to design an appropriate strategy of molecular diagnosis in the Algerian population, we explored the diversity of the involved mutations by studying 65 families affected by autosomal recessive forms of nonsyndromic hearing impairment (DFNB forms), which are the most prevalent early onset forms. We first carried out a systematic screening for mutations in GJB2 and the recurrent p.(Arg34*) mutation in TMC1, which were found in 31 (47.7%) families and 1 (1.5%) family, respectively. We then performed whole exome sequencing in nine of the remaining families, and identified the causative mutations in all the patients analyzed, either in the homozygous state (eight families) or in the compound heterozygous state (one family): (c.709C>T: p.(Arg237*)) and (c.2122C>T: p.(Arg708*)) in OTOF, (c.1334T>G: p.(Leu445Trp)) in SLC26A4, (c.764T>A: p.(Met255Lys)) in GIPC3, (c.518T>A: p.(Cys173Ser)) in LHFPL5, (c.5336T>C: p.(Leu1779Pro)) in MYO15A, (c.1807G>T: p.(Val603Phe)) in OTOA, (c.6080dup: p.(Asn2027Lys*9)) in PTPRQ, and (c.6017del: p.(Gly2006Alafs*13); c.7188_7189ins14: p.(Val2397Leufs*2)) in GPR98. Notably, 7 of these 10 mutations affecting 8 different genes had not been reported previously. These results highlight for the first time the genetic heterogeneity of the early onset forms of nonsyndromic deafness in Algerian families. PMID:26029705

  17. A Comparison of Psychometric Functions for Detection in Normal-Hearing and Hearing-Impaired Listeners.

    ERIC Educational Resources Information Center

    Arehart, Kathryn Hoberg; And Others

    1990-01-01

    Psychometric functions (PFD) for the detection of pure tones were obtained from 10 adolescent and young adult listeners with normal hearing and 10 adult listeners with sensorineural impairments of presumed cochlear origin. The slopes of the PFDs were abnormally steep in some of the hearing-impaired listeners but were statistically significant only…

  18. Characteristics of Hearing Impaired Students by Hearing Status. United States: 1970-71.

    ERIC Educational Resources Information Center

    Rawlings, Brenda; And Others

    Presented were data from the Annual Survey of Hearing Impaired Children on selected characteristics of approximately 41,000 hearing impaired students who were enrolled in special educational programs during the 1970-1971 school year. Included was information on sex, age, additional handicapping conditions, ages of onset and of discovery of the…

  19. 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.

  20. 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

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

    PubMed

    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

    2016-02-01

    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

  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. [The new regulations for diagnostics of occupational hearing impairment].

    PubMed

    Pankova, V B

    2014-01-01

    The author presents information about the new regulations for diagnostics of occupational hearing impairment included in the technical documentation of the Russian Ministry of Health and Social Development and Ministry of Health governing provision of medical assistance to the subjects suffering from occupational hearing impairment and establishing the new rules for the compulsory prophylactic examination (preliminary and periodic) of the candidates for employment in the noisy environment. In addition, the newly-established criteria for the estimation of hearing impairment and occupational fitness of the subjects presenting with this condition are considered. The advantages of the new regulations are discussed along with their drawbacks that require further correction.

  4. [The new regulations for diagnostics of occupational hearing impairment].

    PubMed

    Pankova, V B

    2014-01-01

    The author presents information about the new regulations for diagnostics of occupational hearing impairment included in the technical documentation of the Russian Ministry of Health and Social Development and Ministry of Health governing provision of medical assistance to the subjects suffering from occupational hearing impairment and establishing the new rules for the compulsory prophylactic examination (preliminary and periodic) of the candidates for employment in the noisy environment. In addition, the newly-established criteria for the estimation of hearing impairment and occupational fitness of the subjects presenting with this condition are considered. The advantages of the new regulations are discussed along with their drawbacks that require further correction. PMID:24781171

  5. Phonemic restoration by hearing-impaired listeners with mild to moderate sensorineural hearing loss.

    PubMed

    Başkent, Deniz; Eiler, Cheryl L; Edwards, Brent

    2010-02-01

    The auditory system is capable of perceptually restoring inaudible portions of speech. This restoration may be compromised as a result of hearing impairment, particularly if it is combined with advanced age, because of degradations in the bottom-up and top-down processes. To test this hypothesis, phonemic restoration was quantitatively measured with hearing-impaired listeners of varying ages and degrees of hearing impairment, as well as with a normal hearing control group. The results showed that the restoration benefit was negatively correlated with both hearing impairment and age, supporting the original hypothesis. Group data showed that listeners with mild hearing loss were able to perceptually restore the missing speech segments as well as listeners with normal hearing. By contrast, the moderately-impaired listeners showed no evidence of perceptual restoration. Further analysis using the articulation index showed that listeners with mild hearing loss were able to increase phonemic restoration with audibility. Moderately-impaired listeners, on the other hand, were unable to do so, even when the articulation index was high. The overall findings suggest that, in addition to insufficient audibility, degradations in the bottom-up and/or top-down mechanisms as a result of hearing loss may limit or entirely prevent phonemic restoration. PMID:19922784

  6. [Therapeutic education for young hearing-impaired children].

    PubMed

    Tanaka, Y

    1994-03-01

    Modern therapeutic education of young hearing-impaired children is premised on the active use of residual hearing for helping the deaf child learn language and speech. In this case, measurement of degree of the child's hearing loss and proper fitting of hearing aids are indispensable. Today, accurate measurement of hearing can be made even in infancy by the combined use of behavioral audiometry and auditory brain stem response audiometry. Fitting hearing aids for young hearing-impaired children is possible, if there is an experienced clinician. During the period 1973-1991, I conducted a home training program to 1,613 young hearing-impaired children at the Department of Otolaryngology, Teikyo University Hospital. Out of the 1,613 children, 222 were under one year old, 481 one year old, 470 two years old, 246 three years old, 104 four years old, 69 five years old and 21 six years old. In 1983, we made a follow-up study of hearing-impaired children who had received my home training program and were receiving compulsory education at that time. According to the replies of 87 children to our questionnaire, approximately 70 per cent of the children were attending ordinary schools.

  7. 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. PMID:22387708

  8. 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

  9. 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)

  10. 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

  11. A Spondee Recognition Test for Young Hearing-Impaired Children

    ERIC Educational Resources Information Center

    Cramer, Kathryn D.; Erber, Norman P.

    1974-01-01

    An auditory test of 10 spondaic words recorded on Language Master cards was presented monaurally, through insert receivers to 58 hearing-impaired young children to evaluate their ability to recognize familiar speech material. (MYS)

  12. 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…

  13. 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…

  14. 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)

  15. Removing communication barriers for the hearing-impaired elderly.

    PubMed

    Lindblade, D D; McDonald, M

    1995-10-01

    Hearing impairment is a significant disability affecting many older adults. A number of technologies and services can provide the means to improve communication with these individuals. Nurses play an important role in identifying patients experiencing hearing loss and providing information and assistance that can reduce the consequences of this serious problem. PMID:7550512

  16. Correlates of Mental Health Disorders among Children with Hearing Impairments

    ERIC Educational Resources Information Center

    Fellinger, Johannes; Holzinger, Daniel; Sattel, Heribert; Laucht, Manfred; Goldberg, David

    2009-01-01

    Aim: The aim of this study was to elucidate factors related to the high rate of mental health disorders seen in those with impaired hearing, including social factors and audiological measures. Method: A representative sample of 95 pupils (47 females, 48 males; mean age 11y 1mo, range 6y 5mo to 16y, SD 2y 7mo) with hearing impairments of at least…

  17. 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…

  18. 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

  19. High-frequency audibility: benefits for hearing-impaired listeners.

    PubMed

    Hogan, C A; Turner, C W

    1998-07-01

    The present study was a systematic investigation of the benefit of providing hearing-impaired listeners with audible high-frequency speech information. Five normal-hearing and nine high-frequency hearing-impaired listeners identified nonsense syllables that were low-pass filtered at a number of cutoff frequencies. As a means of quantifying audibility for each condition, Articulation Index (AI) was calculated for each condition for each listener. Most hearing-impaired listeners demonstrated an improvement in speech recognition as additional audible high-frequency information was provided. In some cases for more severely impaired listeners, increasing the audibility of high-frequency speech information resulted in no further improvement in speech recognition, or even decreases in speech recognition. A new measure of how well hearing-impaired listeners used information within specific frequency bands called "efficiency" was devised. This measure compared the benefit of providing a given increase in speech audibility to a hearing-impaired listener to the benefit observed in normal-hearing listeners for the same increase in speech audibility. Efficiencies were calculated using the old AI method and the new AI method (which takes into account the effects of high speech presentation levels). There was a clear pattern in the results suggesting that as the degree of hearing loss at a given frequency increased beyond 55 dB HL, the efficacy of providing additional audibility to that frequency region was diminished, especially when this degree of hearing loss was present at frequencies of 4000 Hz and above. A comparison of analyses from the "old" and "new" AI procedures suggests that some, but not all, of the deficiencies of speech recognition in these listeners was due to high presentation levels.

  20. 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. PMID:15131355

  1. 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.

  2. 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

  3. Comparison of the Speech Syntactic Features between Hearing-Impaired and Normal Hearing Children

    PubMed Central

    PahlavanNezhad, Mohammad Reza; Tayarani Niknezhad, Hamid

    2014-01-01

    Introduction: The present study seeks to describe and analyze the syntactic features of children with severely hearing loss who had access to the hearing aids compared with children with normal hearing, assigning them to the same separate gender classes. Materials and Methods: In the present study, eight children with severe hearing impairment who used a hearing aid and eight hearing children matched for age and gender were selected using an available sampling method based on the principles of auditory-verbal approach. Hearing children had an average age of 5.45 ±1.9 years and subjects had a mean age of 5.43±2.17 years and their rehabilitation had begun before they were 18 months old. The assessment instrument of the study included the language development test, TOLDP-3. The syntactic skills of these children were analyzed and compared with the hearing children of the same age based on gender. Results: There was a significant difference between the syntactic scores of the hearing-impaired children and the scores of the hearing children of the same age in the “sentence imitation” (t=−2/90, P<0/05) and “grammatical completion” (t=−3/39, P<0/05) subtests, with no significant difference in the “grammatical understanding” subtest (t=1/67, P>0/05). Moreover, there was no significant difference between male and female children with hearing impairment in terms of syntactic skills development. Conclusion: With early diagnosis and timely rehabilitating intervention, children with hearing loss can perform in a similar way to children of their age with normal hearing in some syntactical areas. Furthermore, the gender factor in the present study had no effect on the development of syntactical skills of children with hearing loss. PMID:24744994

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

    PubMed

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

    2016-02-01

    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

  5. 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. PMID:27054391

  6. 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.

  7. 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.

  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. 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…

  10. 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…

  11. PECONPI: a novel software for uncovering pathogenic copy number variations in non-syndromic sensorineural hearing loss and other genetically heterogeneous disorders.

    PubMed

    Tsai, Ellen A; Berman, Micah A; Conlin, Laura K; Rehm, Heidi L; Francey, Lauren J; Deardorff, Matthew A; Holst, Jenelle; Kaur, Maninder; Gallant, Emily; Clark, Dinah M; Glessner, Joseph T; Jensen, Shane T; Grant, Struan F A; Gruber, Peter J; Hakonarson, Hakon; Spinner, Nancy B; Krantz, Ian D

    2013-09-01

    This report describes an algorithm developed to predict the pathogenicity of copy number variants (CNVs) in large sample cohorts. CNVs (genomic deletions and duplications) are found in healthy individuals and in individuals with genetic diagnoses, and differentiation of these two classes of CNVs can be challenging and usually requires extensive manual curation. We have developed PECONPI, an algorithm to assess the pathogenicity of CNVs based on gene content and CNV frequency. This software was applied to a large cohort of patients with genetically heterogeneous non-syndromic hearing loss to score and rank each CNV based on its relative pathogenicity. Of 636 individuals tested, we identified the likely underlying etiology of the hearing loss in 14 (2%) of the patients (1 with a homozygous deletion, 7 with a deletion of a known hearing loss gene and a point mutation on the trans allele and 6 with a deletion larger than 1 Mb). We also identified two probands with smaller deletions encompassing genes that may be functionally related to their hearing loss. The ability of PECONPI to determine the pathogenicity of CNVs was tested on a second genetically heterogeneous cohort with congenital heart defects (CHDs). It successfully identified a likely etiology in 6 of 355 individuals (2%). We believe this tool is useful for researchers with large genetically heterogeneous cohorts to help identify known pathogenic causes and novel disease genes.

  12. [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. PMID:1770955

  13. [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.

  14. Subclinical Atherosclerosis and Increased Risk of Hearing Impairment

    PubMed Central

    Fischer, Mary E; Schubert, Carla R; Nondahl, David M; Dalton, Dayna S; Huang, Guan-Hua; Keating, Brendan J; Klein, Barbara EK; Klein, Ronald; Tweed, Ted S; Cruickshanks, Karen J.

    2014-01-01

    Objective The study’s purpose was to test if subclinical atherosclerosis was associated with the risk of developing HI in a large cohort of middle-aged participants. Methods Study subjects were members of the Beaver Dam Offspring Study (BOSS), a longitudinal study of adult children of participants in the population-based Epidemiology of Hearing Loss Study (1993-present). BOSS examinations took place in 2005–2008 (baseline) and 2010–2013 (5-year follow-up). The 5-year incidence of hearing impairment was defined as a pure-tone average (PTA) of thresholds at 0.5, 1, 2 and 4 kHz > 25 decibels Hearing Level (dB HL) in either ear at follow-up among participants at risk (baseline PTA in both ears < = 25 dB HL; n=2,436, mean age = 47.7 years). Atherosclerosis was measured as the mean carotid intima-media thickness and the presence of carotid artery plaque. Results Among the 1,984 participants at-risk with a follow-up audiometric examination, the 5-year incidence of hearing impairment was 8.3% (95% Confidence Interval (C.I.) 7.1, 9.5). With multivariable adjustment, carotid intima-media thickness was positively associated with hearing impairment incidence (Odds Ratio (OR) = 1.18 per 0.1 mm, 95% C.I. 1.05, 1.32). The number of sites (0–6) with plaque was also positively associated with the incidence of impairment (OR = 1.19 per site, 95% C.I. 1.01, 1.41). Conclusion Atherosclerosis was associated with the 5-year incidence of hearing impairment in this predominantly middle-aged cohort. Interventions targeting atherosclerosis prevention may help to prevent or delay the onset of hearing impairment. PMID:25555266

  15. A bone-anchored hearing aid for patients with pure sensorineural hearing impairment: a pilot study.

    PubMed

    Stenfelt, S; Håkansson, B; Jönsson, R; Granström, G

    2000-01-01

    This pilot study assesses the potential benefits of an optimized bone-anchored hearing aid (BAHA) for patients with a mild to moderate pure sensorineural high frequency hearing impairment. The evaluation was conducted with eight first-time hearing aid users by means of psycho-acoustic sound field measurements and a questionnaire on subjective experience; all of the patients benefited from the BAHA. On average, the eight patients showed improvement in PTA threshold of 3.4 dB and in speech intelligibility in noise of 14%. Seven of the subjects, also fitted with present standard air conduction hearing aids (ACHA) found the ACHA thresholds to be improved more than the BAHA ones. In speech tests, the ACHA was only slightly better; these patients chose between their different hearing aids according to the sound environment. Although the BAHA was preferred for wearing and sound comfort, it cannot be used as the sole aid for patients with pure sensorineural impairment.

  16. Behavioural observation audiometry in testing young hearing-impaired children.

    PubMed

    Karikoski, J O; Marttila, T I; Jauhiainen, T

    1998-01-01

    The aim was to examine the accuracy of unconditioned behavioural observation audiometry (BOA) in predicting hearing acuity in children and the validity of test results at various frequencies. The study was designed to longitudinally compare each child's best BOA response level (at the age >12 months) with the conclusive pure-tone threshold of the better ear. The subjects were 119 children derived from a material of 353 children fitted with a hearing aid at Helsinki University Central Hospital. BOA was carried out on 119 children, 19 of whom did not respond to frequency-specific stimuli. The predictive power of BOA depended on the severity of hearing loss. At the hearing level of 30-39 dB, BOA registered 10-15 dB poorer levels than the pure-tone audiometry. The pure-tone averages (0.5, 1, 2 kHz) of 50-69 dB agreed best with the BOA responses. In severe impairments (more than 70 dB HL), the BOA registered too good hearing. Correlation of the results from the two modes to measure hearing level was highly significant (r = 0.71, p = 0.000), and the pure-tone hearing level agreed with that of BOA at the frequencies 0.5 to 4 kHz. Our results show that BOA averages < or =30 dB rarely indicate hearing loss demanding fitting of a hearing aid.

  17. 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.

  18. Association Between Hearing Impairment and Albuminuria in the Korean Adults

    PubMed Central

    Hong, Jae Won; Ku, Cheol Ryong; Noh, Jung Hyun; Kim, Dong-Jun

    2015-01-01

    Abstract Although the associations between albuminuria and renal and cardiovascular diseases, including diabetes and hypertension, have been extensively studied, few studies have investigated the association between albuminuria and hearing impairment. In this study, we assessed the relationship between albuminuria and hearing impairment in 9786 adult Korean subjects, using data from the Korea National Health and Nutrition Examination Survey (KNHANES) performed in 2011–2012. The range of urinary albumin-to-creatinine ratio (UACR) was divided into 4 grades: grade 1 (first tertile of low-grade albuminuria [LGA]), 0.00 to 1.99 mg/g Cr; grade 2 (second tertile of LGA), 2.00 to 5.49 mg/g Cr; grade 3 (third tertile of LGA), 5.50 to 29.99 mg/g Cr; grade 4 (albuminuria), ≥30.00 mg/g Cr. The age- and sex-adjusted weighted UACR was higher in subjects with hearing impairment compared with those without hearing impairment (26.2 ± 4.7 mg/g Cr vs 14.1 ± 1.5 mg/g Cr, P = 0.020). The age- and sex-adjusted weighted prevalence of albuminuria was also higher in subjects with hearing impairment compared with subjects without hearing impairment. (8.3 ± 0.9% vs 5.8 ± 0.4%, P = 0.013) The age- and sex-adjusted weighted percentage of hearing impairment increased as UACR increased (18.0% ± 0.6%, 20.0% ± 0.8%, 22.2% ± 0.9%, 25.3% ± 2.0%, respectively; P < 0.001). Logistic regression analyses were performed for hearing impairment by albuminuria, with age, sex, tobacco use, heavy alcohol use, educational background, occupational noise exposure, obesity, hypertension, diabetes, total serum cholesterol, and estimated glomerular filtration rate (eGFR) <60 mL/min/1.73 m2 as covariates. Using grade 1 of UACR as the control, grade 3 (odds ratio [OR] 1.28, 95% confidence interval [CI] 1.05–1.53, P = 0.005) and grade 4 (OR 1.41, 95% CI 1.04–1.90, P = 0.026) of UACR were correlated with hearing impairment, respectively

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

    PubMed

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

    2016-05-01

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

  20. 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. PMID:27054392

  1. 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.

  2. 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)

  3. Spectral shape discrimination by hearing-impaired and normal-hearing listeners

    NASA Astrophysics Data System (ADS)

    Lentz, Jennifer J.; Leek, Marjorie R.

    2003-03-01

    The ability to discriminate between sounds with different spectral shapes was evaluated for normal-hearing and hearing-impaired listeners. Listeners discriminated between a standard stimulus and a signal stimulus in which half of the standard components were decreased in level and half were increased in level. In one condition, the standard stimulus was the sum of six equal-amplitude tones (equal-SPL), and in another the standard stimulus was the sum of six tones at equal sensation levels re: audiometric thresholds for individual subjects (equal-SL). Spectral weights were estimated in conditions where the amplitudes of the individual tones were perturbed slightly on every presentation. Sensitivity was similar in all conditions for normal-hearing and hearing-impaired listeners. The presence of perturbation and equal-SL components increased thresholds for both groups, but only small differences in weighting strategy were measured between the groups depending on whether the equal-SPL or equal-SL condition was tested. The average data suggest that normal-hearing listeners may rely more on the central components of the spectrum whereas hearing-impaired listeners may have been more likely to use the edges. However, individual weighting functions were quite variable, especially for the HI listeners, perhaps reflecting difficulty in processing changes in spectral shape due to hearing loss. Differences in weighting strategy without changes in sensitivity suggest that factors other than spectral weights, such as internal noise or difficulty encoding a reference stimulus, also may dominate performance.

  4. Hearing-Impaired Children in Venezuela: 1985.

    ERIC Educational Resources Information Center

    Schildroth, Arthur; And Others

    1987-01-01

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

  5. 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. PMID:9680734

  6. The Parent Teaching Home: An Early Intervention Program for Hearing-Impaired Children

    ERIC Educational Resources Information Center

    McConnell, Freeman

    1974-01-01

    The Bill Wilkerson Hearing and Speech Center Program for hearing-impaired children emphasizes the value of detecting hearing impairment in infancy, followed by immediate intervention in the form of an intensive parent teaching program which stresses the maximization of residual hearing to enhance natural language acquisition. (Author/JA)

  7. 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". PMID:27054389

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

    PubMed

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

    2016-02-01

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

  9. Does Computer Software Help Hearing-Impaired Students Learn?

    ERIC Educational Resources Information Center

    Luetke-Stahlman, Barbara

    1988-01-01

    Single-subject studies conducted with 7 hearing-impaired students (aged 6-19) provided empirical support for determining the effectiveness of computerized versus noncomputerized instructional methods and the effectiveness of certain types of software. Drill and practice software helped students learn spelling and vocabulary, but a game-simulation…

  10. Building a Language Base in Hearing-Impaired Students.

    ERIC Educational Resources Information Center

    Luetke-Stahlman, Barbara

    1986-01-01

    This article discusses the importance of fostering the development of a "first language" as demonstrated by each hearing impaired student. Nine factors are presented for consideration by educators working with children who do not have a strong first language base on which to build second language (i.e., English) skills. (Author/CB)

  11. Hearing-Impaired Preschoolers in Integrated Child Care.

    ERIC Educational Resources Information Center

    Luetke-Stahlman, Barbara

    1991-01-01

    The article offers 10 questions to aid in integrating hearing-impaired preschoolers into a child care program. Questions address such concerns as staff and children learning to communicate with the deaf child, interpreting, social integration, group size, and helping the deaf child achieve status. (DB)

  12. 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…

  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. 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)

  15. Basic Education Needs of Adults Who Are Hearing Impaired.

    ERIC Educational Resources Information Center

    Leigh, Gregory R.; And Others

    This study examined the extent to which people in Australia with impaired hearing were receiving some form of literacy assistance and whether requests for service were fulfilled by providers of Adult Literacy and Basic Education (ALBE) services. A questionnaire was sent to a sample of 71 providers. In addition, telephone interviews were conducted…

  16. 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…

  17. Selected Research, Development and Organizational Needs of the Hearing Impaired.

    ERIC Educational Resources Information Center

    Garrett, Charles W., Ed.

    Identified are research, development, and organizational needs regarding sensory aids for the hearing impaired. Discussion of the present status of sensory aids focuses on acoustic and nonacoustic aids and points out that practical long-term utility has been extremely limited. Described are organizational and planning needs such as demographic…

  18. Damage Control: Closing Problematic Sequences in Hearing-Impaired Interaction

    ERIC Educational Resources Information Center

    Skelt, Louise

    2007-01-01

    When a problem of understanding arises for a hearing-impaired recipient in the course of a conversation, and is detected, repairing that problem is only one of several possible courses of action for participants. Another possibility is the collaborative closing of the part of the conversation which has proved problematic for understanding, to…

  19. The Classroom Behavior of Emotionally Disturbed Hearing Impaired Children.

    ERIC Educational Resources Information Center

    Goulder, T. Jordon; Trybus, Raymond J.

    The classroom behavior of 148 hearing impaired children (7-13 years old) was rated by their teachers on the School Behavior Check List. Behavioral descriptions were analyzed in terms of whether or not the student was reproted to have an emotional/behavioral problem or other educationally significant handicapping conditions, and the student's sex…

  20. 38 CFR 4.85 - Evaluation of hearing impairment.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... determine a Roman numeral designation (I through XI) for hearing impairment based on a combination of the...). The Roman numeral designation is located at the point where the percentage of speech discrimination... Based Only on Puretone Threshold Average,” is used to determine a Roman numeral designation (I...

  1. 38 CFR 4.85 - Evaluation of hearing impairment.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... determine a Roman numeral designation (I through XI) for hearing impairment based on a combination of the...). The Roman numeral designation is located at the point where the percentage of speech discrimination... Based Only on Puretone Threshold Average,” is used to determine a Roman numeral designation (I...

  2. 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...). The Roman numeral designation is located at the point where the percentage of speech discrimination... Based Only on Puretone Threshold Average,” is used to determine a Roman numeral designation (I...

  3. 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"…

  4. 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…

  5. 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…

  6. The Effectiveness of CAI Designed for the Hearing-Impaired.

    ERIC Educational Resources Information Center

    Fogel, Nancy S.

    Two pilot studies probed effectiveness of linguistically controlled, highly visual computer-assisted instruction (CAI) for English grammar instruction with hearing-impaired high school students (N=29 in the first study and N=71 in the second). Results from the first study suggested that state-of-the-art CAI designed specifically for use with this…

  7. 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…

  8. Soybean β-Conglycinin Prevents Age-Related Hearing Impairment.

    PubMed

    Tanigawa, Tohru; Shibata, Rei; Kondo, Kazuhisa; Katahira, Nobuyuki; Kambara, Takahiro; Inoue, Yoko; Nonoyama, Hiroshi; Horibe, Yuichiro; Ueda, Hiromi; Murohara, Toyoaki

    2015-01-01

    Obesity-related complications are associated with the development of age-related hearing impairment. β-Conglycinin (β-CG), one of the main storage proteins in soy, offers multiple health benefits, including anti-obesity and anti-atherosclerotic effects. Here, to elucidate the potential therapeutic application of β-CG, we investigated the effect of β-CG on age-related hearing impairment. Male wild-type mice (age 6 months) were randomly divided into β-CG-fed and control groups. Six months later, the body weight was significantly lower in β-CG-fed mice than in the controls. Consumption of β-CG rescued the hearing impairment observed in control mice. Cochlear blood flow also increased in β-CG-fed mice, as did the expression of eNOS in the stria vascularis (SV), which protects vasculature. β-CG consumption also ameliorated oxidative status as assessed by 4-HNE staining. In the SV, lipofuscin granules of marginal cells and vacuolar degeneration of microvascular pericytes were decreased in β-CG-fed mice, as shown by transmission electron microscopy. β-CG consumption prevented loss of spiral ganglion cells and reduced the frequencies of lipofuscin granules, nuclear invaginations, and myelin vacuolation. Our observations indicate that β-CG ameliorates age-related hearing impairment by preserving cochlear blood flow and suppressing oxidative stress.

  9. [The provision of hearing aids to young children with congenital hearing impairment].

    PubMed

    Snik, A F; Admiraal, R J; van den Broek, P

    1992-07-18

    Early screening for hearing impairment in children is generally advised to prevent sensory deprivation and speech, language and learning problems. In the present study, the age at which 176 probably congenitally hearing impaired infants were referred to the Audiological Centre in Nijmegen between 1983 and 1990 was examined. The mean age of fitting the hearing aids was 35 months. This was about 2 years after the auditory screening test (Ewing test) as performed in most baby health care centres. In general, if a screening test is positive, the child will be referred to the general practitioner, who can refer the child to either a general otolaryngologist or a ENT clinic with a (paedo-) audiological centre. Hearing aids can only be provided by an audiological centre. On average, hearing-impaired children who were directly referred to our clinic/audiological centre were provided with hearing aids 13 months earlier than children initially referred to a general otolaryngologist. It is concluded that young children who fail an auditory screening test should be thoroughly tested audiometrically after an otoscopic examination. PMID:1641048

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

    ERIC Educational Resources Information Center

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

    2011-01-01

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

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

    ERIC Educational Resources Information Center

    Altin, Mehmet

    2015-01-01

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

  12. Additional Handicapping Conditions Among Hearing Impaired Students. United States: 1971-72.

    ERIC Educational Resources Information Center

    Gentile, Augustine; McCarthy Barbara

    The Annual Survey of Hearing Impaired Children and Youth (1971-72) obtained information on 42,513 students enrolled in 636 preschool, elementary, and secondary educational programs for the hearing impaired. Data were gathered on the number of hearing impaired students with additional handicapping conditions, the types of conditions reported, the…

  13. 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.

  14. 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…

  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. Identifying congenital hearing impairment. Personal experience based on selective hearing screening.

    PubMed

    Molini, E; Ricci, G; Baroni, S; Ciorba, A; Bellocci, A; Simoncelli, C

    2004-06-01

    If all degrees of permanent uni- or bilateral hypoacusis are taken into consideration, hearing impairment is the most common congenital disease. Early detection of permanent infantile hearing impairment has become extremely important in preventive medicine, since steps can be taken with hearing aids and rehabilitation to ensure better development of language and higher cognitive functions. Aim of this study is to provide a critical review of the time of diagnosis of hypoacusis at our audiology laboratory, where two methods were used to screen hearing of children with/without risk indicators. Results of approximately 10 years' work were re-examined during which time outpatient screening was conducted on children referred by colleagues in neonatology and paediatrics. All were carriers of congenital risk indicators associated with sensorineural and/or conductive hearing loss, based on the Joint Committee on Infant Hearing findings, or were suspected of being hypoacusic even if they had no known congenital risk factors. Hearing screening was conducted in hospital on newborns with no risk factors, within the first few days of birth. Results of the present study showed that when selective hearing screening was performed, the mean age of high-risk patients diagnosed with hypoacusis was slightly higher than that in international guidelines. Moreover, these patients represent approximately half the hypoacusic population identified in the study period. The other half of congenital hypoacusic subjects identified had no risk indicators and there was a significant delay in diagnosis due to later manifestation of symptoms indicating hypoacusis, and thus, in turn, delayed referral for hearing tests. In contrast, subjects without risk indicators who underwent in-hospital hearing screening and proved to be hypoacusic, were diagnosed early. In our experience, however, universal screening has considerable disadvantages, such as difficulty in covering the entire population, difficulty

  17. Better-ear glimpsing in hearing-impaired listeners

    PubMed Central

    Best, Virginia; Mason, Christine R.; Kidd, Gerald; Iyer, Nandini; Brungart, Douglas S.

    2015-01-01

    When competing speech sounds are spatially separated, listeners can make use of the ear with the better target-to-masker ratio. Recent studies showed that listeners with normal hearing are able to efficiently make use of this “better-ear,” even when it alternates between left and right ears at different times in different frequency bands, which may contribute to the ability to listen in spatialized speech mixtures. In the present study, better-ear glimpsing in listeners with bilateral sensorineural hearing impairment, who perform poorly in spatialized speech mixtures, was investigated. The results suggest that this deficit is not related to better-ear glimpsing. PMID:25698053

  18. The impact of hearing impairment, perceptions and attitudes about hearing loss, and noise exposure risk patterns on hearing handicap among farm family members.

    PubMed

    Carruth, Ann; Robert, Ashly E; Hurley, Annette; Currie, Paula S

    2007-06-01

    Farm noise exposure is the major cause of noise-induced hearing loss among farmers. Hearing impairment associated with hearing loss, however, may not adequately represent communication handicap and the impact on quality of life. This descriptive correlational study examines the impact of hearing impairment, perceptions and attitudes about hearing loss, and noise exposure risk patterns on hearing handicap among farm family members. A convenience sample of 56 farmers and family members recruited from community-based agricultural events was studied. A hearing impairment was noted in the majority of the participants (80.4%). Fewer than 10 reported regularly wearing protection during work or recreational activities. High-frequency hearing loss in the left ear (F= 10.30, p < .002), the attitude that wearing hearing protection prevents others from getting one's attention (F= 11.90, p < .001), and the self-reported description of hearing (F= 6.66, p < .013) significantly predicted hearing handicap using multiple regression analysis. Farmers and family members do not use hearing protection because they are concerned they will not hear others who are trying to communicate with them. The findings of this study confirm that this attitude, along with hearing loss in the left ear, is associated with a communication handicap in work settings. Although being able to hear others on a day-to-day basis is important, this attitude may contribute to behaviors leading to hearing loss and decreased communication over time. PMID:17601063

  19. 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

  20. 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.

  1. 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.

  2. 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…

  3. Communication Partners' Journey through Their Partner's Hearing Impairment

    PubMed Central

    Manchaiah, Vinaya K. C.; Stephens, Dafydd; Lunner, Thomas

    2013-01-01

    The objective of this study was to further develop the Ida Institute model on communication partners' (CPs) journey through experiences of person with hearing impairment (PHI), based on the perspectives of CPs. Nine CPs of hearing aid users participated in this study, recruited through the Swansea hearing impaired support group. Semi-structured interviews were conducted, the data were analysed using qualitative thematic analysis and presented with the use of process mapping approach. Seven main phases were identified in the CP journey which includes: (1) contemplation, (2) awareness, (3) persuasion, (4) validation, (5) rehabilitation, (6) adaptation, and (7) resolution. The Ida Institute model (based on professionals' perspective) was compared with the new template developed (based on CPs' perspectives). The results suggest some commonalities and differences between the views of professionals and CPs. A new phase, adaptation, was identified from CPs reported experiences, which was not identified by professionals in the Ida Institute model. The CP's journey model could be a useful tool during audiological enablement/rehabilitation sessions to promote discussion between the PHI and the CP. In addition, it can be used in the training of hearing healthcare professionals. PMID:23533422

  4. 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.

  5. Use of Adaptive Digital Signal Processing to Improve Speech Communication for Normally Hearing aand Hearing-Impaired Subjects.

    ERIC Educational Resources Information Center

    Harris, Richard W.; And Others

    1988-01-01

    A two-microphone adaptive digital noise cancellation technique improved word-recognition ability for 20 normal and 12 hearing-impaired adults by reducing multitalker speech babble and speech spectrum noise 18-22 dB. Word recognition improvements averaged 37-50 percent for normal and 27-40 percent for hearing-impaired subjects. Improvement was best…

  6. Adiponectin deficiency exacerbates age-related hearing impairment.

    PubMed

    Tanigawa, T; Shibata, R; Ouchi, N; Kondo, K; Ishii, M; Katahira, N; Kambara, T; Inoue, Y; Takahashi, R; Ikeda, N; Kihara, S; Ueda, H; Murohara, T

    2014-04-24

    Obesity-related disorders are closely associated with the development of age-related hearing impairment (ARHI). Adiponectin (APN) exerts protective effects against obesity-related conditions including endothelial dysfunction and atherosclerosis. Here, we investigated the impact of APN on ARHI. APN-knockout (APN-KO) mice developed exacerbation of hearing impairment, particularly in the high frequency range, compared with wild-type (WT) mice. Supplementation with APN prevented the hearing impairment in APN-KO mice. At 2 months of age, the cochlear blood flow and capillary density of the stria vascularis (SV) were significantly reduced in APN-KO mice as compared with WT mice. APN-KO mice also showed a significant increase in terminal deoxynucleotidyl transferase dUTP nick end labeling (TUNEL)-positive apoptotic cells in the organ of Corti in the cochlea at 2 months of age. At the age of 6 months, hair cells were lost at the organ of Corti in APN-KO mice. In cultured auditory HEI-OC1 cells, APN reduced apoptotic activity under hypoxic conditions. Clinically, plasma APN levels were significantly lower in humans with ARHI. Multiple logistic regression analysis identified APN as a significant and independent predictor of ARHI. Our observations indicate that APN has an important role in preventing ARHI.

  7. Better-ear glimpsing at low frequencies in normal-hearing and hearing-impaired listeners.

    PubMed

    Rana, Baljeet; Buchholz, Jörg M

    2016-08-01

    Better-ear glimpsing is an auditory process that takes advantage of short-term interaural level differences (ILDs) to improve the understanding of speech in spatial fluctuating noise. Since ILDs are mainly present at high frequencies, where most hearing-impaired (HI) listeners have the strongest hearing loss, HI individuals cannot fully utilize ILDs for better-ear glimpsing, which may lead to poorer understanding of speech in noise. This problem may be alleviated by hearing aids that artificially generate ILDs at low frequencies where hearing is typically less impaired. The present study therefore investigated the spatial benefit in speech intelligibility that is provided by better-ear glimpsing with low-frequency extended ILDs in a symmetric two-distractor speech background. Speech reception thresholds were measured in a spatially co-located and separated condition as a function of frequency region in ten normal-hearing (NH) and ten mild-to-moderate sensorineural HI subjects. In both groups the extended ILDs provided a substantial spatial advantage on top of the advantage already provided by natural ILDs. Moreover, the spatial advantage was largely independent of frequency region, suggesting that both NH and HI subjects can utilize low-frequency ILDs for improving speech understanding in noise. Overall performance as well as spatial advantage was reduced in the HI group. PMID:27586748

  8. Audiological Examinations of Hearing Impaired Students, United States: 1969-70.

    ERIC Educational Resources Information Center

    Gallaudet Coll., Washington, DC. Office of Demographic Studies.

    Presented are data on audiological examinations collected by the Annual Survey of Hearing Impaired Children and Youth for over 35,000 students enrolled in special educational programs for the hearing impaired during the 1969-70 school year. Statistics reported include age, sex, and hearing threshold levels (better ear averages) of the students;…

  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. An Acoustic and Perceptual Study of Initial Stops Produced by Profoundly Hearing Impaired Adolescents

    ERIC Educational Resources Information Center

    Khouw, Edward; Ciocca, Valter

    2007-01-01

    This study investigated the role of Voice Onset Time (VOT) as perceptual cue to the aspiration contrast of Cantonese initial stops produced by adolescent profoundly hearing impaired speakers. Speakers with normal hearing signalled the aspiration contrast through VOT differences. Hearing impaired speakers produced initial stops with no significant…

  11. 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...

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

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... fluctuating hearing loss caused by otitis media, allergies, or eardrum perforations and other outer or middle... hearing loss can include impaired listening skills, delayed language development, and articulation... 45 Public Welfare 4 2011-10-01 2011-10-01 false Eligibility criteria: Hearing impairment...

  13. 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... 45 Public Welfare 4 2014-10-01 2014-10-01 false Eligibility criteria: Hearing impairment...

  14. 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... 45 Public Welfare 4 2013-10-01 2013-10-01 false Eligibility criteria: Hearing impairment...

  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. 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, even with…

  17. 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…

  18. Optimization of speech in noise with three signal processing algorithms for normal-hearing and hearing-impaired subjects

    NASA Astrophysics Data System (ADS)

    Franck, Bas A. M.; Dreschler, Wouter A.; Lyzenga, Johannes

    2002-05-01

    In this study a three-dimensional Simplex procedure was applied to optimize speech in noise by a combination of signal processing algorithms for different acoustic conditions and hearing losses. The algorithms used to span the three dimensions are noise reduction, spectral tilting, and spectral enhancement, respectively. Additionally, we studied the algorithms for their main effects and interaction effects within the optimization process. The subjects were asked to evaluate two consecutive, differently processed sentences on listening comfort. Three different noise types and two signal-to-noise ratios (S/N) were used. Three groups of subjects participated: normal hearing, normal hearing with simulated impaired auditory filtering (by spectral smearing), and sensorineurally hearing-impaired subjects. For the normal-hearing group we applied S/N=0 dB. For the hearing-impaired and the simulated hearing-impaired subjects we applied S/N=5 dB. We will discuss the similarities and differences in the response patterns of the three groups. Also, the individual preferences will be related to the hearing capacity, and to the type of interfering noise. Finally, we will discuss differences in the perceptual features that are used to judge listening comfort of the fragments by normal-hearing and hearing-impaired subjects.

  19. Exome sequencing identifies a novel frameshift mutation of MYO6 as the cause of autosomal dominant nonsyndromic hearing loss in a Chinese family.

    PubMed

    Cheng, Jing; Zhou, Xueya; Lu, Yu; Chen, Jing; Han, Bing; Zhu, Yuhua; Liu, Liyang; Choy, Kwong-Wai; Han, Dongyi; Sham, Pak C; Zhang, Michael Q; Zhang, Xuegong; Yuan, Huijun

    2014-11-01

    Autosomal dominant types of nonsyndromic hearing loss (ADNSHL) are typically postlingual in onset and progressive. High genetic heterogeneity, late onset age, and possible confounding due to nongenetic factors hinder the timely molecular diagnoses for most patients. In this study, exome sequencing was applied to investigate a large Chinese family segregating ADNSHL in which we initially failed to find strong evidence of linkage to any locus by whole-genome linkage analysis. Two affected family members were selected for sequencing. We identified two novel mutations disrupting known ADNSHL genes and shared by the sequenced samples: c.328C>A in COCH (DFNA9) resulting in a p.Q110K substitution and a deletion c. 2814_2815delAA in MYO6 (DFNA22) causing a frameshift alteration p.R939Tfs*2. The pathogenicity of novel coding variants in ADNSHL genes was carefully evaluated by analysis of co-segregation with phenotype in the pedigree and in light of established genotype-phenotype correlations. The frameshift deletion in MYO6 was confirmed as the causative variant for this pedigree, whereas the missense mutation in COCH had no clinical significance. The results allowed us to retrospectively identify the phenocopy in one patient that contributed to the negative finding in the linkage scan. Our clinical data also supported the emerging genotype-phenotype correlation for DFNA22.

  20. 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.

  1. A novel mutation within the MIR96 gene causes non-syndromic inherited hearing loss in an Italian family by altering pre-miRNA processing

    PubMed Central

    Soldà, Giulia; Robusto, Michela; Primignani, Paola; Castorina, Pierangela; Benzoni, Elena; Cesarani, Antonio; Ambrosetti, Umberto; Asselta, Rosanna; Duga, Stefano

    2012-01-01

    The miR-96, miR-182 and miR-183 microRNA (miRNA) family is essential for differentiation and function of the vertebrate inner ear. Recently, point mutations within the seed region of miR-96 were reported in two Spanish families with autosomal dominant non-syndromic sensorineural hearing loss (NSHL) and in a mouse model of NSHL. We screened 882 NSHL patients and 836 normal-hearing Italian controls and identified one putative novel mutation within the miR-96 gene in a family with autosomal dominant NSHL. Although located outside the mature miR-96 sequence, the detected variant replaces a highly conserved nucleotide within the companion miR-96*, and is predicted to reduce the stability of the pre-miRNA hairpin. To evaluate the effect of the detected mutation on miR-96/mir-96* biogenesis, we investigated the maturation of miR-96 by transient expression in mammalian cells, followed by real-time reverse-transcription polymerase chain reaction (PCR). We found that both miR-96 and miR-96* levels were significantly reduced in the mutant, whereas the precursor levels were unaffected. Moreover, miR-96 and miR-96* expression levels could be restored by a compensatory mutation that reconstitutes the secondary structure of the pre-miR-96 hairpin, demonstrating that the mutation hinders precursor processing, probably interfering with Dicer cleavage. Finally, even though the mature miR-96 sequence is not altered, we demonstrated that the identified mutation significantly impacts on miR-96 regulation of selected targets. In conclusion, we provide further evidence of the involvement of miR-96 mutations in human deafness and demonstrate that a quantitative defect of this miRNA may contribute to NSHL. PMID:22038834

  2. 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. PMID:26611684

  3. 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:…

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

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 45 Public Welfare 4 2010-10-01 2010-10-01 false Eligibility criteria: Hearing impairment including deafness. 1308.11 Section 1308.11 Public Welfare Regulations Relating to Public Welfare (Continued) OFFICE... impairment including deafness. (a) A child is classified as deaf if a hearing impairment exists which is...

  5. 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

  6. 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.

  7. The impact of acquired hearing impairment on intimate relationships: implications for rehabilitation.

    PubMed

    Hétu, R; Jones, L; Getty, L

    1993-01-01

    Hearing disabilities, due to their interactive nature, strongly affect intimate relationships. This paper reviews the literature on this dimension of the experience of hearing impairment. The effects of progressive hearing loss and of the coping process within the couple are examined borrowing concepts from symbolic interaction theory. A summary of the interactive dimensions of the effects of hearing impairment on the couple is drawn from the relevant studies. Specific needs have been identified for each partner in terms of information, support and communication facilitation. Implications are drawn for the audiological rehabilitation of hearing-impaired adults. PMID:8267527

  8. [Youth Health Care Services guideline 'Early screening for hearing impairment, 0-19 years'].

    PubMed

    van Leerdam, F J

    2000-03-25

    The Youth Health Care guideline 'Early screening for hearing impairment, ages 0-19 years' draws the attention of all parties involved to the case finding of, notably, perceptive hearing loss; it helps workers in the Youth Health Care argue their referrals and improve the next steps in the process. Referrals always aim at ruling out perceptive hearing impairment. It is essential to diagnose and subsequently treat children with perceptive hearing impairment. It is important to keep track of the process after the referral. Screening methods are the Ewing test, the 'Compacte Amsterdamse paedo-audiometrische screener' (CAPAS) test and audiograms. Each age category has groups at risk for hearing impairment. Problems in neonates are a low specificity of the Ewing/CAPAS tests, treatment of middle ear disease without ruling out perceptive hearing impairment and absence of interdisciplinary agreements regarding the steps to be taken and the necessary feedback. Children with perceptive hearing loss should be diagnosed before 18 months of age. In the near future neonatal screening may detect congenital perceptive hearing impairment, but hearing impairment may still develop after the screening. Alertness with respect to hearing impairment will remain necessary in every consultation of a child.

  9. [Youth Health Care Services guideline 'Early screening for hearing impairment, 0-19 years'].

    PubMed

    van Leerdam, F J

    2000-03-25

    The Youth Health Care guideline 'Early screening for hearing impairment, ages 0-19 years' draws the attention of all parties involved to the case finding of, notably, perceptive hearing loss; it helps workers in the Youth Health Care argue their referrals and improve the next steps in the process. Referrals always aim at ruling out perceptive hearing impairment. It is essential to diagnose and subsequently treat children with perceptive hearing impairment. It is important to keep track of the process after the referral. Screening methods are the Ewing test, the 'Compacte Amsterdamse paedo-audiometrische screener' (CAPAS) test and audiograms. Each age category has groups at risk for hearing impairment. Problems in neonates are a low specificity of the Ewing/CAPAS tests, treatment of middle ear disease without ruling out perceptive hearing impairment and absence of interdisciplinary agreements regarding the steps to be taken and the necessary feedback. Children with perceptive hearing loss should be diagnosed before 18 months of age. In the near future neonatal screening may detect congenital perceptive hearing impairment, but hearing impairment may still develop after the screening. Alertness with respect to hearing impairment will remain necessary in every consultation of a child. PMID:10761546

  10. [Decline of speech understanding in the hearing impaired elderly listeners who visited our hearing aid clinic].

    PubMed

    Yasue, Minori; Sugiura, Saiko; Uchida, Yasue; Nakashima, Tsutomu

    2014-08-01

    The aim of this study was to investigate distinctive change in the hearing impaired elderly listeners, especially about speech recognition. Subjects were 525 patients (235 males, 290 females), from 60 to 98 years of age who had visited the Hearing Aid Clinic of Otorhinolaryngology, National Center for Geriatrics and Gerontology Hospital, between June 2001 and December 2012. Pure-tone air conduction threshold determination was administered to each subject. The speech audiometry materials used to define speech discrimination ability were Japanese monosyllabic word lists, 67S word lists. Sex- and age-specific trends in maximum discrimination score (MDS) and rollover index (RI) were shown in this study. RI was computed by the formula (PBmax-PBmin)/PBmax. PBmax has almost the same meaning as MDS in Japan. PBmin represented the lowest PB score above the test intensity level of PBmax. Mean MDSs were 80.8% in their sixties, 75.3% in their seventies, 60.7% in their eighties, and 45.5% in their nineties. The rate of decrease in mean MDS per decade accelerated in the older generation. Mean RIs were 0.18 in patients in their sixties, 0.24 in their seventies, 0.30 in their eighties, and 0.30 in their nineties. It increased until the age of eighties. In the model 1, multiple logistic analyses were performed to examine the effect of age (in 10-year increments), sex and mean hearing levels in pure tone average of values at 500, 1000, 2000, 4000 Hz (in 10 dB increments) on the MDS < 60%. Significant associations were observed between MDS < 60% and age (odds ratio, 3.03; 95% confidence interval, 2.38 to 3.85), and mean hearing levels in pure tone average of values at 500, 1000, 2000, 4000 Hz (2.33; 2.03 to 2.68). Sex was not associated with MDS < 60%. In the model 2, multiple logistic analyses adjusted for age and sex were performed to examine the effect of hearing level at test frequencies of 125, 250, 500, 1000, 2000, 4000, and 8000 Hz (in 10 dB increments)on the MDS < 60

  11. HI-SIMv1.0--towards the virtual reality of hearing impairments.

    PubMed

    Körkkö, P; Huttunen, K; Sorri, M

    2001-01-01

    The virtual reality of hearing impairments has obvious practical applications in areas such as audiology, speech therapy and hearing aid technology and serves as an informational tool for the family members of the hearing impaired. To simulate hearing impairment, a CD-ROM with filtered speech material accessible through a graphical user-interface was produced; the user-interface was created with standard multimedia tools. The CD-ROM, HI-SIMv1.0, intended as an interactive educational tool, offers a virtual experience of the effects of a selection of common types of hearing impairment. The options available in this simulation include grade of hearing impairment, audiometric configuration and the type and level of background noise. Word recognition scores can be computed for standard Finnish audiometric material.

  12. [New recurrent extended deletion, including GJB2 and GJB6 genes, results in isolated sensorineural hearing impairment with autosomal recessive type of inheritance].

    PubMed

    Bliznets, E A; Makienko, O N; Okuneva, E G; Markova, T G; Poliakov, A V

    2014-04-01

    Hereditary hearing loss with the autosomal recessive type of inheritance of the DFNB 1 genetic type, caused by mutations in the GJB2 gene, is the main reason of innate non-syndromal hearing impairment in most developed countries of the world (including Russia). Intragenic point mutations prevail among the GJB2 gene defectors; however, extended deletions in the DFNB1 locus are also found with considerable frequency in some populations (for example, Spain, Great Britain, France, United States, and Brazil). Among the four known extended deletions, only one deletion affects directly the GJB2 gene sequence and was described in a single family. A new extended deletion in the GJB2 and GJB6 gene sequences (approximately 101 kb in size; NC_000013.10:g.20,757,021_20,858,394del), detected in three unrelated Russian patients, was described and characterized. Ingush origin of this mutation is assumed. If the new deletion is frequent, its detection is very important for the genetic consulting of families with hereditary hearing impairment. PMID:25715449

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

    PubMed

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

    2016-06-01

    Deafness is one of the most common genetic diseases in humans and is subject to important genetic heterogeneity. The most common cause of non syndromic hearing loss (NSHL) is mutations in the GJB2 gene. This study aims to update and evaluate the spectrum of GJB2 allele variants in 152 Moroccan multiplex families with non syndromic hearing loss. Seven different mutations were detected: c.35delG, p.V37I, p.E47X, p.G200R, p.Del120E, p.R75Q, the last three mutations were described for the first time in Moroccan deaf patients, in addition to a novel nonsense mutation, the c.385G>T which is not referenced in any database. Sixty six families (43.42%) have mutations in the coding region of GJB2, while the homozygous c.35delG mutation still to date the most represented 51/152 (33.55%). The analysis of the geographical distribution of mutations located in GJB2 gene showed more allelic heterogeneity in the north and center compared to the south of Morocco. Our results showed that the GJB2 gene is a major contributor to non syndromic hearing loss in Morocco. Thus, this report of the GJB2 mutations spectrum all over Morocco has an important implication for establishing a suitable molecular diagnosis. PMID:27169813

  14. 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

  15. 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

  16. Hearing Impairment Affects Dementia Incidence. An Analysis Based on Longitudinal Health Claims Data in Germany

    PubMed Central

    Teipel, Stefan; Óvári, Attila; Kilimann, Ingo; Witt, Gabriele; Doblhammer, Gabriele

    2016-01-01

    Recent research has revealed an association between hearing impairment and dementia. The objective of this study is to determine the effect of hearing impairment on dementia incidence in a longitudinal study, and whether ear, nose, and throat (ENT) specialist care, care level, institutionalization, or depression mediates or moderates this pathway. The present study used a longitudinal sample of 154,783 persons aged 65 and older from claims data of the largest German health insurer; containing 14,602 incident dementia diagnoses between 2006 and 2010. Dementia and hearing impairment diagnoses were defined according to International Classification of Diseases, Tenth Revision, codes. We used a Kaplan Meier estimator and performed Cox proportional hazard models to explore the effect of hearing impairment on dementia incidence, controlling for ENT specialist care, care level, institutionalization, and depression. Gender, age, and comorbidities were controlled for as potential confounders. Patients with bilateral (HR = 1.43, p<0.001) and side-unspecified (HR = 1.20, p<0.001) hearing impairment had higher risks of dementia incidence than patients without hearing impairment. We found no significant effect for unilateral hearing impairment and other diseases of the ear. The effect of hearing impairment was only partly mediated through ENT specialist utilization. Significant interaction between hearing impairment and specialist care, care level, and institutionalization, respectively, indicated moderating effects. We discuss possible explanations for these effects. This study underlines the importance of the association between hearing impairment and dementia. Preserving hearing ability may maintain social participation and may reduce the burden associated with dementia. The particular impact of hearing aid use should be the subject of further investigations, as it offers potential intervention on the pathway to dementia. PMID:27391486

  17. MTHFR 677T Is a Strong Determinant of the Degree of Hearing Loss Among Polish Males with Postlingual Sensorineural Hearing Impairment

    PubMed Central

    Pollak, Agnieszka; Mueller-Malesinska, Malgorzata; Lechowicz, Urszula; Skorka, Agata; Korniszewski, Lech; Sobczyk-Kopciol, Agnieszka; Waskiewicz, Anna; Broda, Grazyna; Iwanicka-Pronicka, Katarzyna; Oldak, Monika; Skarzynski, Henryk

    2012-01-01

    Hearing impairment (HI) is the most common sensory handicap. Congenital HI often has a genetic basis, whereas the etiology of nonsyndromic postlingual HI (npHI) usually remains unidentified. Our purpose was to test whether the MTHFR C677T (rs1801133) polymorphism affecting folate metabolism is associated with the occurrence or severity of npHI. We studied rs1801133 genotypes in 647 npHI patients (age <40, sudden sensorineural loss excluded, HI characterized as mean of better ear hearing thresholds for 0.5–8 kHz) and 3273 adult controls from the background population. Genotype distribution among patients and controls was similar, but among male cases (n=302) we found a dose-dependent correlation of MTHFR 677T with the degree of HI (mean thresholds in dB: 38.8, 44.9, and 53.3, for CC, CT, and TT genotypes, respectively; p=0.0013, pcor.=0.017). Among male patients rs1801133 TT significantly increased the risk of severe/profound HI (odds ratio=4.88, p=0.001). Among controls the known effect of MTHFR 677T on plasma total homocysteine was more pronounced in men than in women (p<0.00004 for genotype-sex interaction) suggesting that in Poland folate deficiency is more prevalent in males. In conclusion, we report a novel strong effect of MTHFR 677T among males with npHI. The functional significance of rs1801133 suggests that these patients may benefit from folate supplementation—an intervention which is simple, cheap, and devoid of side effects. PMID:22424391

  18. 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.

  19. 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,…

  20. 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…

  1. 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:…

  2. 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…

  3. Vowel Identification by Listeners with Hearing Impairment in Response to Variation in Formant Frequencies

    ERIC Educational Resources Information Center

    Molis, Michelle R.; Leek, Marjorie R.

    2011-01-01

    Purpose: This study examined the influence of presentation level and mild-to-moderate hearing loss on the identification of a set of vowel tokens systematically varying in the frequency locations of their second and third formants. Method: Five listeners with normal hearing (NH listeners) and five listeners with hearing impairment (HI listeners)…

  4. 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…

  5. 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…

  6. 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

  7. Speech pattern hearing aids for the profoundly hearing impaired: speech perception and auditory abilities.

    PubMed

    Faulkner, A; Ball, V; Rosen, S; Moore, B C; Fourcin, A

    1992-04-01

    A family of prototype speech pattern hearing aids for the profoundly hearing impaired has been compared to amplification. These aids are designed to extract acoustic speech patterns that convey essential phonetic contrasts, and to match this information to residual receptive abilities. In the first study, the presentation of voice fundamental frequency information from a wearable SiVo (sinusoidal voice) aid was compared to amplification in 11 profoundly deafened adults. Intonation reception was often better, and never worse, with fundamental frequency information. Four subjects scored more highly in audio-visual consonant identification with fundamental frequency information, five performed better with amplified speech, and two performed similarly under these two conditions. Five of the 11 subjects continued use of the SiVo aid after the tests were complete. A second study examined a laboratory prototype compound speech pattern aid, which encoded voice fundamental frequency, amplitude envelope, and the presence of voiceless excitation. In five profoundly deafened adults, performance was better in consonant identification when additional speech patterns were present than with fundamental frequency alone; the main advantage was derived from amplitude information. In both consonant identification and connected discourse tracking, performance with appropriately matched compound speech pattern signals was better than with amplified speech in three subjects, and similar to performance with amplified speech in the other two. In nine subjects, frequency discrimination, gap detection, and frequency selectivity were measured, and were compared to speech receptive abilities with both amplification and fundamental frequency presentation. The subjects who showed the greatest advantage from fundamental frequency presentation showed the greatest average hearing losses, and the least degree of frequency selectivity. Compound speech pattern aids appear to be more effective for some

  8. Dialogue enabling speech-to-text user assistive agent system for hearing-impaired person.

    PubMed

    Lee, Seongjae; Kang, Sunmee; Han, David K; Ko, Hanseok

    2016-06-01

    A novel approach for assisting bidirectional communication between people of normal hearing and hearing-impaired is presented. While the existing hearing-impaired assistive devices such as hearing aids and cochlear implants are vulnerable in extreme noise conditions or post-surgery side effects, the proposed concept is an alternative approach wherein spoken dialogue is achieved by means of employing a robust speech recognition technique which takes into consideration of noisy environmental factors without any attachment into human body. The proposed system is a portable device with an acoustic beamformer for directional noise reduction and capable of performing speech-to-text transcription function, which adopts a keyword spotting method. It is also equipped with an optimized user interface for hearing-impaired people, rendering intuitive and natural device usage with diverse domain contexts. The relevant experimental results confirm that the proposed interface design is feasible for realizing an effective and efficient intelligent agent for hearing-impaired.

  9. Classifier Recognition by Hearing-Impaired Children in Residential and Public Schools.

    ERIC Educational Resources Information Center

    Luetke-Stahlman, Barbara

    1984-01-01

    Study indicates that hearing impaired residential students are more proficient users of American Sign Language than are hearing impaired children enrolled in local, public school programs, and older such residential students are more proficient in the language than are younger students. (SL)

  10. 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…

  11. Parent-Infant Program for the Hearing Impaired: A Resource Guide.

    ERIC Educational Resources Information Center

    Elwood, Patricia C.; And Others

    Intended as a guide for the design and implementation of parent-infant programs for the hearing impaired, the volume provides suggestions and sample materials from the program in Prince George's County, Maryland. Included in the guidelines for assessment of local need and identification of hearing impaired infants in the community are sample…

  12. An Acoustic and Perceptual Study of Final Stops Produced by Profoundly Hearing Impaired Adolescents

    ERIC Educational Resources Information Center

    Khouw, Edward; Ciocca, Valter

    2006-01-01

    Purpose: This study investigated formant frequencies for their role as acoustic and perceptual correlates to the place of articulation of Cantonese final stops produced by profoundly hearing impaired speakers. Method: Speakers were 10 Cantonese adolescents (mean age = 13;5 [years;months]) who were profoundly hearing impaired (HI). Control speakers…

  13. 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…

  14. 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…

  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 Curriculum for Mainstreamed Preschool Children Who Are Hearing Impaired developed by Project CHIME.

    ERIC Educational Resources Information Center

    Pucciarelli, Catherine S., Ed.

    Project CHIME (Children with Hearing Impairments in Mainstreamed Environments) was developed to create a curriculum for use by professionals who are providing mainstreaming opportunities for hearing-impaired preschoolers. The CHIME curriculum is divided into five activity areas that are part of the growth and development process for all…

  17. 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…

  18. 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…

  19. 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…

  20. 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…

  1. Language Assessment of Hearing-Impaired Children and Youth: Patterns of Test Use.

    ERIC Educational Resources Information Center

    Abraham, Suzanne; Stoker, Richard

    1988-01-01

    A survey of 182 educational programs for hearing-impaired children and youth identified those test instruments most widely used to assess language at infant, preschool, primary, and secondary levels. Also analyzed were communication modes and manual systems used in testing, difficulties encountered in assessing hearing-impaired children, and…

  2. 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…

  3. 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),…

  4. 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)

  5. A Picture-Identification Test for Hearing-Impaired Children. Final Report.

    ERIC Educational Resources Information Center

    Ross, Mark; Lerman, Jay

    The Word Intelligibility by Picture Identification Test (WIPI) was developed to measure speech discrimination ability in hearing impaired children. In the first phase of development, the word stimuli were evaluated to determine whether they were within the recognition vocabulary of 15 hearing impaired children (aged 6 to 12) and whether the…

  6. Hearing impairment and its predictors in childhood bacterial meningitis in Angola.

    PubMed

    Roine, Irmeli; Pelkonen, Tuula; Cruzeiro, Manuel Leite; Kataja, Matti; Peltola, Heikki; Pitkäranta, Anne

    2013-05-01

    Hearing was evaluated in 244 ears of 124 children in Angola by auditory brainstem response audiometry 3 months after bacterial meningitis. Of all ears, 81% recovered without impairment. Of all children, 74% recovered without impairment, 5% had unilateral and 11% bilateral impairment. Seizures before or during hospital stay and disease severity were the best predictors of ≥ 80 dB impairment.

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

    PubMed

    Trevino, Andrea; Allen, Jont B

    2013-07-01

    The consonant recognition of 17 ears with sensorineural hearing loss is evaluated for 14 consonants /p, t, k, f, s, ∫, b, d, g, v, z, 3, m, n/+/a/, 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

  8. 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…

  9. [Decline of speech understanding in the hearing impaired elderly listeners who visited our hearing aid clinic].

    PubMed

    Yasue, Minori; Sugiura, Saiko; Uchida, Yasue; Nakashima, Tsutomu

    2014-08-01

    The aim of this study was to investigate distinctive change in the hearing impaired elderly listeners, especially about speech recognition. Subjects were 525 patients (235 males, 290 females), from 60 to 98 years of age who had visited the Hearing Aid Clinic of Otorhinolaryngology, National Center for Geriatrics and Gerontology Hospital, between June 2001 and December 2012. Pure-tone air conduction threshold determination was administered to each subject. The speech audiometry materials used to define speech discrimination ability were Japanese monosyllabic word lists, 67S word lists. Sex- and age-specific trends in maximum discrimination score (MDS) and rollover index (RI) were shown in this study. RI was computed by the formula (PBmax-PBmin)/PBmax. PBmax has almost the same meaning as MDS in Japan. PBmin represented the lowest PB score above the test intensity level of PBmax. Mean MDSs were 80.8% in their sixties, 75.3% in their seventies, 60.7% in their eighties, and 45.5% in their nineties. The rate of decrease in mean MDS per decade accelerated in the older generation. Mean RIs were 0.18 in patients in their sixties, 0.24 in their seventies, 0.30 in their eighties, and 0.30 in their nineties. It increased until the age of eighties. In the model 1, multiple logistic analyses were performed to examine the effect of age (in 10-year increments), sex and mean hearing levels in pure tone average of values at 500, 1000, 2000, 4000 Hz (in 10 dB increments) on the MDS < 60%. Significant associations were observed between MDS < 60% and age (odds ratio, 3.03; 95% confidence interval, 2.38 to 3.85), and mean hearing levels in pure tone average of values at 500, 1000, 2000, 4000 Hz (2.33; 2.03 to 2.68). Sex was not associated with MDS < 60%. In the model 2, multiple logistic analyses adjusted for age and sex were performed to examine the effect of hearing level at test frequencies of 125, 250, 500, 1000, 2000, 4000, and 8000 Hz (in 10 dB increments)on the MDS < 60

  10. Cantonese tone production performance of mainstream school children with hearing impairment.

    PubMed

    Cheung, Karen K L; Lau, Ada H Y; Lam, Joffee H S; Lee, Kathy Y S

    2014-12-01

    This study investigated the Cantonese tone production ability of children with hearing impairment studying in mainstream schools. The participants were 87 Cantonese-speaking children with mild-to-profound degrees of hearing loss aged 5.92-13.58 in Hong Kong. Most of the children were fitted with hearing aids (n = 65); 17 of them had profound hearing impairment, one who had severe hearing loss had cochlear implantation, and four who had mild hearing loss were without any hearing device. The Hong Kong Cantonese Articulation Test was administered, and the tones produced were rated by two of the authors and a speech-language pathologist. Group effects of tones, hearing loss level, and also an interaction of the two were found to be significant. The children with profound hearing impairment performed significantly worse than most of the other children. Tone 1 was produced most accurately, whereas tone 6 productions were the poorest. No relationship was found between the number of years of mainstreaming and tone production ability. Tone production error pattern revealed that confusion patterns in tone perception coincided with those in production. Tones having a similar fundamental frequency (F0) at the onset also posed difficulty in tone production for children with hearing impairment.

  11. 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.

  12. Impairment of SLC17A8 Encoding Vesicular Glutamate Transporter-3, VGLUT3, Underlies Nonsyndromic Deafness DFNA25 and Inner Hair Cell Dysfunction in Null Mice

    PubMed Central

    Ruel, Jérôme; Emery, Sarah; Nouvian, Régis; Bersot, Tiphaine; Amilhon, Bénédicte; Van Rybroek, Jana M.; Rebillard, Guy; Lenoir, Marc; Eybalin, Michel; Delprat, Benjamin; Sivakumaran, Theru A.; Giros, Bruno; El Mestikawy, Salah; Moser, Tobias; Smith, Richard J.H.; Lesperance, Marci M.; Puel, Jean-Luc

    2008-01-01

    Autosomal-dominant sensorineural hearing loss is genetically heterogeneous, with a phenotype closely resembling presbycusis, the most common sensory defect associated with aging in humans. We have identified SLC17A8, which encodes the vesicular glutamate transporter-3 (VGLUT3), as the gene responsible for DFNA25, an autosomal-dominant form of progressive, high-frequency nonsyndromic deafness. In two unrelated families, a heterozygous missense mutation, c.632C→T (p.A211V), was found to segregate with DFNA25 deafness and was not present in 267 controls. Linkage-disequilibrium analysis suggested that the families have a distant common ancestor. The A211 residue is conserved in VGLUT3 across species and in all human VGLUT subtypes (VGLUT1-3), suggesting an important functional role. In the cochlea, VGLUT3 accumulates glutamate in the synaptic vesicles of the sensory inner hair cells (IHCs) before releasing it onto receptors of auditory-nerve terminals. Null mice with a targeted deletion of Slc17a8 exon 2 lacked auditory-nerve responses to acoustic stimuli, although auditory brainstem responses could be elicited by electrical stimuli, and robust otoacoustic emissions were recorded. Ca2+-triggered synaptic-vesicle turnover was normal in IHCs of Slc17a8 null mice when probed by membrane capacitance measurements at 2 weeks of age. Later, the number of afferent synapses, spiral ganglion neurons, and lateral efferent endings below sensory IHCs declined. Ribbon synapses remaining by 3 months of age had a normal ultrastructural appearance. We conclude that deafness in Slc17a8-deficient mice is due to a specific defect of vesicular glutamate uptake and release and that VGLUT3 is essential for auditory coding at the IHC synapse. PMID:18674745

  13. [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. PMID:25734302

  14. [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.

  15. 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.

  16. Genetic and Environmental Factors in Age-Related Hearing Impairment.

    PubMed

    Momi, Sukhleen K; Wolber, Lisa E; Fabiane, Stella Maris; MacGregor, Alex J; Williams, Frances M K

    2015-08-01

    Age-related hearing impairment (ARHI) is a common condition with complex etiology but a recognized genetic component. Heritability estimates for pure tone audiogram-determined hearing ability lie in the range 26-75%. The speech-in-noise (SIN) auditory test, however, may be better at encapsulating ARHI symptoms, particularly the diminished ability to segregate environmental sounds into comprehendible auditory streams. As heritability of SIN has not previously been reported, we explored the genetic and environmental contributions to ARHI determined by SIN in 2,076 twins (87.8% female) aged 18-87 (mean age 54.4). SIN was found to be significantly heritable (A, unadjusted for age=40%; 95% confidence intervals, CI=32%-47%). With age adjustment, heritability fell (A=25%; 95% CI=16-33%), and a relatively strong influence of environmental exposure unshared within twin siblings was identified (E=75%). To explore the environmental aspects further, we assessed the influence of diet (through the Food Frequency Questionnaire, FFQ), smoking (through self-report and cotinine metabolite levels) and alcohol intake (through the FFQ). A negative influence of high cholesterol diet was observed after adjustment (p=.037). A protective effect of raised serum high-density lipoprotein (HDL) cholesterol levels was observed after adjustment (p=.004). This study is the first assessment of the genetic and environmental influence on SIN perception. The findings suggest SIN is less heritable than pure tone audiogram (PTA) ability and highly influenced by the environment unique to each twin. Furthermore, a possible role of dietary fat in the etiology of ARHI is highlighted.

  17. Early ERP Signature of Hearing Impairment in Visual Rhyme Judgment

    PubMed Central

    Classon, Elisabet; Rudner, Mary; Johansson, Mikael; Rönnberg, Jerker

    2013-01-01

    Postlingually acquired hearing impairment (HI) is associated with changes in the representation of sound in semantic long-term memory. An indication of this is the lower performance on visual rhyme judgment tasks in conditions where phonological and orthographic cues mismatch, requiring high reliance on phonological representations. In this study, event-related potentials (ERPs) were used for the first time to investigate the neural correlates of phonological processing in visual rhyme judgments in participants with acquired HI and normal hearing (NH). Rhyme task word pairs rhymed or not and had matching or mismatching orthography. In addition, the inter-stimulus interval (ISI) was manipulated to be either long (800 ms) or short (50 ms). Long ISIs allow for engagement of explicit, top-down processes, while short ISIs limit the involvement of such mechanisms. We hypothesized lower behavioral performance and N400 and N2 deviations in HI in the mismatching rhyme judgment conditions, particularly in short ISI. However, the results showed a different pattern. As expected, behavioral performance in the mismatch conditions was lower in HI than in NH in short ISI, but ERPs did not differ across groups. In contrast, HI performed on a par with NH in long ISI. Further, HI, but not NH, showed an amplified N2-like response in the non-rhyming, orthographically mismatching condition in long ISI. This was also the rhyme condition in which participants in both groups benefited the most from the possibility to engage top-down processes afforded with the longer ISI. Taken together, these results indicate an early ERP signature of HI in this challenging phonological task, likely reflecting use of a compensatory strategy. This strategy is suggested to involve increased reliance on explicit mechanisms such as articulatory recoding and grapheme-to-phoneme conversion. PMID:23653613

  18. Hypertension and hearing impairment in workers of iron and steel industry.

    PubMed

    Narlawar, Uday W; Surjuse, Bhooshan G; Thakre, Sushama S

    2006-01-01

    The objectives of the present study were to study the (i) prevalence of hypertension and hearing impairment in iron and steel industry workers, (ii) association between hypertension and hearing impairment, (iii) association between hypertension and hearing impairment, with duration of exposure, and (iv) correlation between levels of sound and noise induced health problems viz. hypertension and hearing impairment in a cross sectional study involving Workers working in iron and steel industry at Nagpur. Of a total of 804 workers, 770 workers participated in the study. There were five sections in the factory; Steel melting section (SMS), rolling mill section (RMS), quality control department (QCD), maintenance department (MD) and administration department (AD). Workers working in SMS, RMS, & QCD formed a continuously exposed group (CEG); workers of MD and AD formed intermittently exposed group (IEG). Workers were interviewed, examined and information was collected in a pre-tested pro-forma. Blood pressure was measured, and tuning fork tests were done to assess hearing ability. Prevalence of hypertension among CEG (25.51%) was significantly higher than IEG (14.05%). (X2-14.28, df-1, P<0.001). There was positive association between duration of exposure and prevalence of hypertension. The prevalence of hearing impairment was significantly more in CEG (20.5%) as compared to IEG (8.91%). (X2-11.69, df-1, P<0.001). Occurrence of hearing impairment was also directly proportional to the duration of exposure. The correlation between level of sound exposure and hearing impairment was found to be significant (r=0.98; P<0.05), the correlation between level of sound and hypertension was found to be statistically insignificant (r=0.84; P>0.05). The results of the present study indicate that hypertension and hearing impairment are commoner in workers continuously exposed to high levels of occupational noise.

  19. A Philosophy for Assessing the Language Proficiency of Hearing-Impaired Students to Promote English Literacy.

    ERIC Educational Resources Information Center

    Luetke-Stahlman, Barbara

    1982-01-01

    Research is reviewed on bilingual education and its application to the education of hearing-impaired students. Assessment procedures for obtaining valid samples of language behavior, distinguishing between competence and performance, and considering language variation are emphasized. (CL)

  20. The Microcomputer as an Administrative/Educational Tool in Education of the Hearing Impaired.

    ERIC Educational Resources Information Center

    Graham, Richard

    1982-01-01

    Administrative and instructional uses of microcomputers with hearing impaired students (infants to junior high level) are described. Uses include data storage and retrieval, maintenance of student history files, storage of test data, and vocabulary reinforcement for students. (CL)

  1. Syllable Reception by Hearing-Impaired Children Trained from Infancy in Auditory-Oral Programs.

    ERIC Educational Resources Information Center

    Ling, Daniel; And Others

    1981-01-01

    Under both the audition alone and combined audition and lipreading conditions, Ss' performance was superior to that previously reported for profoundly hearing impaired children trained in schools in which a "multisensory" approach is typically used. (Author)

  2. 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)

  3. 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

  4. 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)

  5. 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)

  6. Hearing-Impaired/Developmentally Disabled Persons in the United States: Definitions, Causes, Effects, and Prevalence Estimates

    ERIC Educational Resources Information Center

    Stewart, Larry G.

    1978-01-01

    Information is presented on the definition, causes, effects and population estimates for developmental disabilities; and a tentative hypothesis regarding the hearing impaired/developmentally disabled is set forth. (CL)

  7. 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,…

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

    PubMed

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

    2016-01-01

    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. PMID:27101435

  9. Effect of Auditory Training on Reading Comprehension of Children with Hearing Impairment in Enugu State

    ERIC Educational Resources Information Center

    Ugwuanyi, L. T.; Adaka, T. A.

    2015-01-01

    The paper focused on the effect of auditory training on reading comprehension of children with hearing impairment in Enugu State. A total of 33 children with conductive, sensory neural and mixed hearing loss were sampled for the study in the two schools for the Deaf in Enugu State. The design employed for the study was a quasi experiment (pre-test…

  10. Evaluation of Programs for the Hearing Impaired: Bergen County Special Services School District.

    ERIC Educational Resources Information Center

    Lederer, James B.; And Others

    The report describes evaluation of the elementary and secondary programs which serve 158 Bergen County (NJ) severely and profoundly hard of hearing and deaf students in preschool through Grade 12. An initial section focuses on the Elementary Hearing Impaired Program (HIP). Among the program components addressed are the language development…

  11. Audiological assessment, rehabilitation, and spatial hearing considerations associated with visual impairment in adults: an overview.

    PubMed

    Blumsack, Judith T

    2003-12-01

    The purpose of this paper is to increase awareness and interest among audiologists regarding the needs of adults who have both hearing loss and visual impairment, particularly people who are blind and travel independently. Case history, audiometric testing, and rehabilitation issues are considered, and extensive discussion of spatial hearing research as it relates to orientation and mobility is provided.

  12. 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…

  13. 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)

  14. An Evaluation of Methods Used to Teach Speech to the Hearing Impaired Using a Simulation Technique.

    ERIC Educational Resources Information Center

    Abraham, Suzanne; Stoker, Richard G.

    1984-01-01

    The effectiveness of syllable practice and word methods in teaching speech to severely hearing-impaired children was investigated with normal-hearing listeners. The effect of Cued Speech was evaluated for each teaching method. Results indicated that a syllable practice approach showed significantly higher average gains in acquisition of novel…

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

    PubMed

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

    2016-01-01

    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.

  16. Intelligibility of Telephone Speech for the Hearing Impaired When Various Microphones Are Used for Acoustic Coupling.

    ERIC Educational Resources Information Center

    Janota, Claus P.; Janota, Jeanette Olach

    1991-01-01

    Various candidate microphones were evaluated for acoustic coupling of hearing aids to a telephone receiver. Results from testing by 9 hearing-impaired adults found comparable listening performance with a pressure gradient microphone at a 10 decibel higher level of interfering noise than with a normal pressure-sensitive microphone. (Author/PB)

  17. Encoding voice pitch for profoundly hearing-impaired listeners.

    PubMed

    Grant, K W

    1987-08-01

    The ability of five profoundly hearing-impaired subjects to "track" connected speech and to make judgments about the intonation and stress in spoken sentences was evaluated under a variety of auditory-visual conditions. These included speechreading alone, speechreading plus speech (low-pass filtered at 4 kHz), and speechreading plus a tone whose frequency, intensity, and temporal characteristics were matched to the speaker's fundamental frequency (F0). In addition, several frequency transfer functions were applied to the normal F0 range resulting in new ranges that were both transposed and expanded with respect to the original F0 range. Three of the five subjects were able to use several of the tonal representations of F0 nearly as well as speech to improve their speechreading rates and to make appropriate judgments concerning sentence intonation and stress. The remaining two subjects greatly improved their identification performance for intonation and stress patterns when expanded F0 signals were presented alone (i.e., without speechreading), but had difficulty integrating visual and auditory information at the connected discourse level, despite intensive training in the connected discourse tracking procedure lasting from 27.8-33.8 h.

  18. Phenotypic variability in a seven-generation Swedish family segregating autosomal dominant hearing impairment due to a novel EYA4 frameshift mutation.

    PubMed

    Frykholm, Carina; Klar, Joakim; Arnesson, Hanna; Rehnman, Anna-Carin; Lodahl, Marianne; Wedén, Ulla; Dahl, Niklas; Tranebjærg, Lisbeth; Rendtorff, Nanna D

    2015-05-25

    Linkage to an interval overlapping the DFNA10 locus on chromosome 6q22-23 was found through genome wide linkage analysis in a seven-generation Swedish family segregating postlingual, autosomal dominant nonsyndromic sensorineural hearing impairment. A novel heterozygous frame-shift mutation (c.579_580insTACC, p.(Asp194Tyrfs*52)) in EYA4 was identified that truncates the so-called variable region of the protein. The mutation is predicted to result in haploinsufficiency of the EYA4 product. No evidence for dilated cardiomyopathy was found in the family, contrasting to a previous family with a deletion resulting in a similar truncation in the variable region. A highly variable age of onset was seen in the mutation carriers. For assessment of the aetiology of this variability, clinical and audiometric data analyses were performed. The affected family members all had similar cross-sectional and longitudinal deterioration of pure tone average (PTA) once the process of hearing deterioration had started, and no gender, parent-of-origin or family branch differences on PTA could be found. Age at onset varied between the family branches. In summary, this is the ninth published genetically verified DFNA10 family. The results imply that unidentified factors, genetic or environmental, other than the EYA4 mutation, are of importance for the age at onset of DFNA10, and that mutation early in the variable region of the EYA4 protein can occur in the absence of dilated cardiomyopathy.

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

    ERIC Educational Resources Information Center

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

    2011-01-01

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

  20. Modulation rate detection and discrimination by normal-hearing and hearing-impaired listeners.

    PubMed

    Grant, K W; Summers, V; Leek, M R

    1998-08-01

    Modulation detection and modulation rate discrimination thresholds were obtained at three different modulation rates (fm = 80, 160, and 320 Hz) and for three different ranges of modulation depths (m): full (100%), mid (70%-80%), and low (40%-60%) with both normal-hearing (NH) and hearing-impaired (HI) subjects. The results showed that modulation detection thresholds increased with modulation rate, but significantly more so for HI than for NH subjects. Similarly, rate discrimination thresholds (delta r) increased with increases in fm and decreases in modulation depth. When compared to NH subjects, rate discrimination thresholds for HI subjects were significantly worse for all rates and for all depths. At the fastest modulation rate with less than 100% modulation depth, most HI subjects could not discriminate any change in rate. When valid thresholds for rate discrimination were obtained for HI subjects, they ranged from 2.5 semitones (delta r = 12.7 Hz, fm = 80 Hz, m = 100%) to 8.7 semitones (delta r = 214.5 Hz, fm = 320 Hz, m = 100%). In contrast, average rate discrimination thresholds for NH subjects ranged from 0.9 semitones (delta r = 4.2 Hz, fm = 80 Hz, m = 100%) to 4.7 semitones (delta r = 103.5 Hz, fm = 320 Hz, m = 60%). Some of the differences in temporal processing between NH and HI subjects, especially those related to modulation detection, may be accounted for by differences in signal audibility, especially for high-frequency portions of the modulated noise. However, in many cases, HI subjects encountered great difficulty discriminating a change in modulation rate even though the modulation components of the standard and test stimuli were detectable.

  1. 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

  2. A Novel de novo Mutation in CEACAM16 Associated with Postlingual Hearing Impairment

    PubMed Central

    Hofrichter, Michaela A.H.; Nanda, Indrajit; Gräf, Jens; Schröder, Jörg; Shehata-Dieler, Wafaa; Vona, Barbara; Haaf, Thomas

    2015-01-01

    Mutations in CEACAM16 cause autosomal dominant nonsyndromic hearing loss (DFNA4B). So far, 2 families have been reported with segregating missense mutations, both in the immunoglobulin constant domain A of the CEACAM16 protein. In this study, we used the TruSight One panel to investigate a parent-child trio without familial history of hearing loss and one affected child. When filtering for recessive inheritance and de novo events, we discovered a de novo CEACAM16 mutation (c.1094T>G, p.Leu365Arg) as the sole likely pathogenic variant. The de novo mutation was confirmed by Sanger sequencing and STR analysis. The proband's hearing loss closely matches the described onset and severity for DFNA4B. We present the third CEACAM16 variant and the first de novo mutation in CEACAM16. This de novo mutation is robustly described as a pathogenic mutation according to in silico mutation prediction tools and affects a highly conserved amino acid in the most strongly conserved CEACAM16 N2 domain. Our strategy of screening family trios enhances de novo mutation discovery and the exclusion of other variants of potential interest through pedigree filtering. PMID:26648831

  3. 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

  4. 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…

  5. Auditory cortical activation in severe-to-profound hearing-impaired patients monitored by SPET.

    PubMed

    Di Nardo, W; Di Giuda, D; Scarano, E; Picciotti, P M; Galla, S; De Rossi, G

    2006-08-01

    Single photon emission tomography was used to map blood flow increase in temporal and parietal cortex after auditory stimulation in 25 subjects: 10 normal-hearing, 10 severe-profound hearing-impaired and 5 totally deaf. After a 500 Hz pure tone stimulation, a marked perfusion increase was observed, particularly at the level of the contralateral auditory temporal cortex. Blood flow increase in temporal and parietal cortical areas of normal subjects was significantly higher than that observed in severe-to-profound hearing-impaired patients. In all cases, following 500 Hz pure tone acoustic stimulation, the most lateral sagittal slice tomograms (48.75 and 56.25 mm) showed the highest blood flow increase. Statistically significant differences were also observed between normal subjects and hearing-impaired patients in the 48.75 mm sagittal tomogram. In 2 hearing-impaired patients, the single photon emission tomography pattern showed activation of the intermediate sagittal tomogram, suggesting a possible new tonotopic cortical arrangement. No significant activation was present in totally deaf patients. In conclusion, Single Photon Emission Tomography appears to be a useful tool in the evaluation of auditory cortical activation and cortical plasticity, in severe-to-profound hearing-impaired patients. Moreover, it could be a useful test for the study of auditory central pathways.

  6. Factors Associated With Age-related Hearing Impairment

    PubMed Central

    Moon, Il Joon; Byun, Hayoung; Woo, Sook-young; Gwak, Geum-Youn; Hong, Sung Hwa; Chung, Won-Ho; Cho, Yang-Sun

    2015-01-01

    Abstract Age-related hearing impairment (ARHI) is a complex degenerative disease in the elderly. As multiple factors interact during the development of ARHI, it is important to elucidate the major influencing factors to understand and prevent ARHI. We aimed to identify risk factors associated with the development of ARHI with a retrospective cohort from 2001 to 2010. The records of the adult subjects over 40 years of age who consecutively underwent a comprehensive health checkup including pure-tone audiometry at the Health Promotion Center were reviewed. During this period, 1560 subjects who underwent pure-tone audiometry more than twice, had no other otologic diseases, and were followed-up more than 2 years were included. A pure-tone average (PTA: 0.5, 1, 2, 4 kHz) was calculated. Development of ARHI was defined as a PTA at follow-up more than 10 dB greater than the baseline PTA. Times to the first development of ARHI were investigated. Overall, 12.7% of subjects developed ARHI within the first 4 years. High blood ionized calcium (hazard ratio [HR] 0.084), albumin (HR 0.239), systolic blood pressure (HR 0.577), thyroid hormone (T3) (HR 0.593), and alpha fetoprotein levels (HR 0.883) were associated with decreased hazard for the development of ARHI. In contrast, high blood high-density lipoprotein (HR 2.105), uric acid (HR 1.684), total protein (HR 1.423), and total bilirubin levels (HR 1.220) were potential risk factors for the development of ARHI. Development of ARHI is common among the aged population, and a variety of factors may interact during this process. The results of this study can be used for counseling of adults at high-risk of developing ARHI with regard to regular audiological check-up. PMID:26512592

  7. 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.…

  8. 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)

  9. 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

  10. Effects of noise and distortion on speech quality judgments in normal-hearing and hearing-impaired listeners.

    PubMed

    Arehart, Kathryn H; Kates, James M; Anderson, Melinda C; Harvey, Lewis O

    2007-08-01

    Noise and distortion reduce speech intelligibility and quality in audio devices such as hearing aids. This study investigates the perception and prediction of sound quality by both normal-hearing and hearing-impaired subjects for conditions of noise and distortion related to those found in hearing aids. Stimuli were sentences subjected to three kinds of distortion (additive noise, peak clipping, and center clipping), with eight levels of degradation for each distortion type. The subjects performed paired comparisons for all possible pairs of 24 conditions. A one-dimensional coherence-based metric was used to analyze the quality judgments. This metric was an extension of a speech intelligibility metric presented in Kates and Arehart (2005) [J. Acoust. Soc. Am. 117, 2224-2237] and is based on dividing the speech signal into three amplitude regions, computing the coherence for each region, and then combining the three coherence values across frequency in a calculation based on the speech intelligibility index. The one-dimensional metric accurately predicted the quality judgments of normal-hearing listeners and listeners with mild-to-moderate hearing loss, although some systematic errors were present. A multidimensional analysis indicates that several dimensions are needed to describe the factors used by subjects to judge the effects of the three distortion types. PMID:17672661

  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. Practical screening priorities for hearing impairment among children in developing countries.

    PubMed Central

    Gell, F. M.; White, E. M.; Newell, K.; Mackenzie, I.; Smith, A.; Thompson, S.; Hatcher, J.

    1992-01-01

    Routine screening for hearing impairment in childhood is now widespread in industrial countries, although there is considerable controversy over the most efficient techniques and procedures. In most developing countries, however, routine screening programmes for hearing impairment do not currently exist. The problems involved in implementing screening programmes in developing and industrial countries are very different, and in selecting screening procedures for a particular population the following factors have to be taken into consideration: the environmental test conditions; the availability of resources for equipment and the training of testers; the local attitudes towards disability; the level of hearing impairment that may cause handicaps; and the major types of pathology causing hearing impairment. We suggest that in developing countries children should be screened at school entry using a simple field audiometer and that the external ear be inspected for the presence of a discharge. There is an urgent need to develop reliable and simple screening procedures for infants and young children; where possible, all children should be screened for severe or significant hearing impairment before the age of 2 years. No screening should, however, be implemented until appropriate follow-up services are available. Images Fig. 1 Fig. 2 PMID:1464152

  14. Effects of the Simultaneous Application of Nonlinear Frequency Compression and Dichotic Hearing on the Speech Recognition of Severely Hearing-Impaired Subjects: Simulation Test

    PubMed Central

    Hwang, Jong Ho; Nam, Kyoung Won; Yoon, Sung Hoon; Kim, Jinryoul; Yook, Sunhyun; Hong, Sung Hwa; Jang, Dong Pyo

    2015-01-01

    Objectives The clinical effects of the simultaneous application of nonlinear frequency compression and dichotic hearing on people with hearing impairments have not been evaluated previously. In this study, the clinical effects of the simultaneous application of these two techniques on the recognition of consonant-vowel-consonant (CVC) words with fricatives were evaluated using normal-hearing subjects and a hearing loss simulator operated in the severe hearing loss setting. Methods A total of 21 normal-hearing volunteers whose native language was English were recruited for this study, and two different hearing loss simulators, which were configured for severe hearing loss in the high-frequency range, were utilized. The subjects heard 82 English CVC words, and the word recognition score and response time were measured. Results The experimental results demonstrated that the simultaneous application of these two techniques showed almost even performance compared to the sole application of nonlinear frequency compression in a severe hearing loss setting. Conclusion Though it is generally accepted that dichotic hearing can decrease the spectral masking thresholds of an hearing-impaired person, simultaneous application of the nonlinear frequency compression and dichotic hearing techniques did not significantly improve the recognition of words with fricatives compared to the sole application of nonlinear frequency compression in a severe hearing loss setting. PMID:26045907

  15. Working memory and referential communication-multimodal aspects of interaction between children with sensorineural hearing impairment and normal hearing peers.

    PubMed

    Sandgren, Olof; Hansson, Kristina; Sahlén, Birgitta

    2015-01-01

    Whereas the language development of children with sensorineural hearing impairment (SNHI) has repeatedly been shown to differ from that of peers with normal hearing (NH), few studies have used an experimental approach to investigate the consequences on everyday communicative interaction. This mini review gives an overview of a range of studies on children with SNHI and NH exploring intra- and inter-individual cognitive and linguistic systems during communication. Over the last decade, our research group has studied the conversational strategies of Swedish speaking children and adolescents with SNHI and NH using referential communication, an experimental analog to problem-solving in the classroom. We have established verbal and non-verbal control and validation mechanisms, related to working memory capacity and phonological short term memory. We present main findings and future directions relevant for the field of cognitive hearing science and for the clinical and school-based management of children and adolescents with SNHI. PMID:25806012

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

    PubMed

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

    2010-06-01

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

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

    PubMed

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

    2009-02-01

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

  18. The Effects of Sensorineural Hearing Impairment on Asynchronous Glimpsing of Speech

    PubMed Central

    Buss, Emily; Hall, Joseph W.

    2016-01-01

    In a previous study with normal-hearing listeners, we evaluated consonant identification masked by two or more spectrally contiguous bands of noise, with asynchronous square-wave modulation applied to neighboring bands. Speech recognition thresholds were 5.1–8.5 dB better when neighboring bands were presented to different ears (dichotic) than when all bands were presented to one ear (monaural), depending on the spectral width of the frequency bands. This dichotic advantage was interpreted as reflecting masking release from peripheral spread of masking from neighboring frequency bands. The present study evaluated this effect in listeners with sensorineural hearing loss, a population more susceptible to spread of masking. Speech perception (vowel-consonant-vowel stimuli, as in /aBa/) was measured in the presence of fluctuating noise that was either modulated synchronously across frequency or asynchronously. Hearing-impaired listeners (n = 9) and normal-hearing controls were tested at either the same intensity (n = 7) or same sensation level (n = 8). Hearing-impaired listeners had mild-to-moderate hearing loss and symmetrical, flat audiometric thresholds. While all groups of listeners performed better in the dichotic than monaural condition, this effect was smaller for the hearing-impaired (3.5 dB) and equivalent-sensation-level controls (3.3 dB) than controls tested at the same intensity (11.0 dB). The present study is consistent with the idea that dichotic presentation can improve speech-in-noise listening for hearing-impaired listeners, and may be enhanced when combined with amplification. PMID:27144601

  19. [The clinical aspects of occupational sensorineural impairment of hearing of the acoustic origin].

    PubMed

    Zinkin, V N; Sheshegov, P M; Chistov, S D

    2015-01-01

    The objective of the present study was to elucidate the specific clinical features of occupational sensorineural impairment of hearing (OSNHI) depending on the origin of this pathology, viz. noise, infrasound and/or their combination. The review of research concerning this problem made it possible to systematize variants of ODNHI based on the clinical signs taking into consideration the influence of these physical factors. Pathophysiological features of the exposure of the organs of hearing to infrasound are described. PMID:27006983

  20. Childhood hearing impairment: auditory and linguistic interactions during multidimensional speech processing.

    PubMed

    Jerger, S; Martin, R; Pearson, D A; Dinh, T

    1995-08-01

    Children with mild-severe sensorineural hearing losses often use hearing aids and aural/oral language as their primary mode of communication, yet we know little about how speech is processed by these children. The purpose of this research was to investigate how the multidimensional information underlying accurate speech perception is processed by children with mild-severe hearing impairments. The processing of the auditory and linguistic dimensions of speech was assessed with a speeded selective-attention task (Garner, 1974a). Listeners were required to attend selectively to an auditory dimension (gender of the talker) and ignore a linguistic dimension (word) and vice versa. The hypothesis underlying the task is that performance for the target dimension will be unaffected by what is happening on the nontarget dimension if the dimensions are processed independently. On the other hand, if the dimensions are not processed independently, listeners will not be able to attend selectively and performance for the relevant dimension will be affected by what is happening on the irrelevant dimension (termed "Garner" interference). Both children with normal hearing (N = 90) and children with hearing impairment (N = 40) showed some degree of Garner interference, implying that the dimensions of speech are not processed independently by these children. However, relative to the children with normal hearing, the children with hearing impairment showed normal Garner interference when attending selectively to the word dimension (normally effective at ignoring talker-gender input) and reduced Garner interference when attending selectively to the talker-gender dimension (more effective at ignoring word input). This pattern of results implies that the auditory dimension has a normal strength-of-processing level that makes it normally distracting and that the linguistic dimension has an underdeveloped strength-of-processing level that makes it easier to ignore in children with hearing

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

    NASA Astrophysics Data System (ADS)

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

    2016-08-01

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

  2. Contribution of consonant versus vowel information to sentence intelligibility for young normal-hearing and elderly hearing-impaired listeners.

    PubMed

    Kewley-Port, Diane; Burkle, T Zachary; Lee, Jae Hee

    2007-10-01

    The purpose of this study was to examine the contribution of information provided by vowels versus consonants to sentence intelligibility in young normal-hearing (YNH) and typical elderly hearing-impaired (EHI) listeners. Sentences were presented in three conditions, unaltered or with either the vowels or the consonants replaced with speech shaped noise. Sentences from male and female talkers in the TIMIT database were selected. Baseline performance was established at a 70 dB SPL level using YNH listeners. Subsequently EHI and YNH participants listened at 95 dB SPL. Participants listened to each sentence twice and were asked to repeat the entire sentence after each presentation. Words were scored correct if identified exactly. Average performance for unaltered sentences was greater than 94%. Overall, EHI listeners performed more poorly than YNH listeners. However, vowel-only sentences were always significantly more intelligible than consonant-only sentences, usually by a ratio of 2:1 across groups. In contrast to written English or words spoken in isolation, these results demonstrated that for spoken sentences, vowels carry more information about sentence intelligibility than consonants for both young normal-hearing and elderly hearing-impaired listeners.

  3. Phonological Abilities of Hearing-Impaired Cantonese-Speaking Children with Cochlear Implants or Hearing Aids

    ERIC Educational Resources Information Center

    Law, Zoe W. Y.; So, Lydia K. H.

    2006-01-01

    Purpose: This article examined the phonological skills of 2 groups of Cantonese-speaking children with prelingual, profound bilateral hearing loss. The phonological abilities of 7 children fitted with hearing aids were compared with the abilities of 7 children who wore cochlear implants. Method: Participants in each group ranged in age from 5;1…

  4. Effects of inherent envelope fluctuations in forward maskers for listeners with normal and impaired hearing.

    PubMed

    Svec, Adam; Dubno, Judy R; Nelson, Peggy B

    2015-03-01

    Gaussian noise simultaneous maskers yield higher masked thresholds for pure tones than low-fluctuation noise simultaneous maskers for listeners with normal hearing. This increased masking effectiveness is thought to be due to inherent fluctuations in the temporal envelope of Gaussian noise, but effects of fluctuating forward maskers are unknown. Because differences in forward masking due to age and hearing loss are known, the current study assessed effects of masker envelope fluctuations for forward maskers in younger and older adults with normal hearing and older adults with hearing loss. Detection thresholds were measured in these three participant groups for a pure-tone probe in quiet and in Gaussian and low-fluctuation noise forward maskers with either 1 or 1/3 equivalent rectangular bandwidths. Higher masked thresholds were obtained for forward maskers with greater inherent envelope fluctuations for younger adults with normal hearing. This increased effectiveness of highly fluctuating forward maskers was similar for older adults with normal and impaired hearing. Because differences in recovery from forward masking between listeners with normal and impaired hearing may relate to differences in cochlear nonlinearities, these results suggest that mechanisms other than cochlear nonlinearities may be responsible for recovery from rapid masker envelope fluctuations. PMID:25786946

  5. SOX10 mutations mimic isolated hearing loss.

    PubMed

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

    2015-10-01

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

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

    PubMed Central

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

    2016-01-01

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

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

    ERIC Educational Resources Information Center

    Wolff, Anthony B.; Brown, Scott Campbell

    1987-01-01

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

  8. Development of a voice database to aid children with hearing impairments

    NASA Astrophysics Data System (ADS)

    Kuzman, M. G.; Agüero, P. D.; Tulli, J. C.; Gonzalez, E. L.; Uriz, A. J.; Cervellini, M. P.

    2011-12-01

    In the development of software for voice analysis or training, for people with hearing impairments, a database having sounds of properly pronounced words is of paramount importance. This paper shows the advantage that will be obtained from getting an own voice database, rather than using those coming from other countries, even having the same language, in the development of speech training software aimed to people with hearing impairments. This database will be used by software developers at the School of Engineering of Mar del Plata National University.

  9. Developmental dysgraphia with profound hearing impairment: intervention by auditory methods enabled by cochlear implant.

    PubMed

    Fukushima, Kunihiro; Kawasaki, Akihiro; Nagayasu, Rie; Kunisue, Kazuya; Maeda, Yukihide; Kariya, Shin; Kataoka, Yuko; Nishizaki, Kazunori

    2008-06-01

    Learning disability combined with hearing impairment (LDHI) is a poor prognostic factor for the language development of hearing impaired children after educational intervention. A typical example of a child with LDHI and effective interventions provided by cochlear implants are presented in this report. A case of congenital cytomegaloviral infection that showed dysgraphia as well as profound deafness was reported and an underlying visual processing problem diagnosed in the present case caused the patient's dysgraphia. The dysgraphia could be circumvented by the use of auditory memory fairly established by a cochlear implant. PMID:18082987

  10. Newborn Genetic Screening for Hearing Impairment: A Preliminary Study at a Tertiary Center

    PubMed Central

    Wu, Chen-Chi; Hung, Chia-Cheng; Lin, Shin-Yu; Hsieh, Wu-Shiun; Tsao, Po-Nien; Lee, Chien-Nan; Su, Yi-Ning; Hsu, Chuan-Jen

    2011-01-01

    Universal newborn hearing screening (UNHS) is of paramount importance for early identification and management of hearing impairment in children. However, infants with slight/mild, progressive, or late-onset hearing impairment might be missed in conventional UNHS. To investigate whether genetic screening for common deafness-associated mutations could assist in identifying these infants, 1017 consecutive newborns in a tertiary hospital were subjected to both newborn hearing screening using a two-step distortion-product otoacoustic emissions (DPOAE) screening and newborn genetic screening (NGS) for deafness. The NGS targeted 4 deafness-associated mutations commonly found in the Taiwanese population, including p.V37I (c.109G>A) and c.235delC of the GJB2 gene, c.919-2A>G of the SLC26A4 gene, and mitochondrial m.1555A>G of the 12S rRNA gene. The results of the NGS were then correlated to the results of the NHS. Of the 1017 newborns, 16 (1.6%) had unilateral DPOAE screening failure, and 22 (2.2%) had bilateral DPOAE screening failure. A total of 199 (19.6%) babies were found to have at least 1 mutated allele on the NGS for deafness, 11 (1.1%) of whom were homozygous for GJB2 p.V37I, 6 (0.6%) compound heterozygous for GJB2 p.V37I and c.235delC, and 1 (0.1%) homoplasmic for m.1555A>G, who may potentially have hearing loss. Among them, 3 babies, 5 babies, and 1 baby, respectively, passed the NHS at birth. Comprehensive audiological assessments in the 9 babies at 3 months identified 1 with slight hearing loss and 2 with mild hearing loss. NGS for common deafness-associated mutations may identify infants with slight/mild or potentially progressive hearing impairment, thus compensating for the inherent limitations of the conventional UNHS. PMID:21811586

  11. Newborn genetic screening for hearing impairment: a preliminary study at a tertiary center.

    PubMed

    Wu, Chen-Chi; Hung, Chia-Cheng; Lin, Shin-Yu; Hsieh, Wu-Shiun; Tsao, Po-Nien; Lee, Chien-Nan; Su, Yi-Ning; Hsu, Chuan-Jen

    2011-01-01

    Universal newborn hearing screening (UNHS) is of paramount importance for early identification and management of hearing impairment in children. However, infants with slight/mild, progressive, or late-onset hearing impairment might be missed in conventional UNHS. To investigate whether genetic screening for common deafness-associated mutations could assist in identifying these infants, 1017 consecutive newborns in a tertiary hospital were subjected to both newborn hearing screening using a two-step distortion-product otoacoustic emissions (DPOAE) screening and newborn genetic screening (NGS) for deafness. The NGS targeted 4 deafness-associated mutations commonly found in the Taiwanese population, including p.V37I (c.109G>A) and c.235delC of the GJB2 gene, c.919-2A>G of the SLC26A4 gene, and mitochondrial m.1555A>G of the 12S rRNA gene. The results of the NGS were then correlated to the results of the NHS. Of the 1017 newborns, 16 (1.6%) had unilateral DPOAE screening failure, and 22 (2.2%) had bilateral DPOAE screening failure. A total of 199 (19.6%) babies were found to have at least 1 mutated allele on the NGS for deafness, 11 (1.1%) of whom were homozygous for GJB2 p.V37I, 6 (0.6%) compound heterozygous for GJB2 p.V37I and c.235delC, and 1 (0.1%) homoplasmic for m.1555A>G, who may potentially have hearing loss. Among them, 3 babies, 5 babies, and 1 baby, respectively, passed the NHS at birth. Comprehensive audiological assessments in the 9 babies at 3 months identified 1 with slight hearing loss and 2 with mild hearing loss. NGS for common deafness-associated mutations may identify infants with slight/mild or potentially progressive hearing impairment, thus compensating for the inherent limitations of the conventional UNHS.

  12. 14 CFR 382.119 - What information must carriers give individuals with vision or hearing impairment on aircraft?

    Code of Federal Regulations, 2012 CFR

    2012-01-01

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

  13. 14 CFR 382.53 - What information must carriers give individuals with a vision or hearing impairment at airports?

    Code of Federal Regulations, 2011 CFR

    2011-01-01

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

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

    Code of Federal Regulations, 2011 CFR

    2011-01-01

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

  15. 14 CFR 382.53 - What information must carriers give individuals with a vision or hearing impairment at airports?

    Code of Federal Regulations, 2013 CFR

    2013-01-01

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

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

    Code of Federal Regulations, 2014 CFR

    2014-01-01

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

  17. 14 CFR 382.53 - What information must carriers give individuals with a vision or hearing impairment at airports?

    Code of Federal Regulations, 2014 CFR

    2014-01-01

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

  18. 14 CFR 382.119 - What information must carriers give individuals with vision or hearing impairment on aircraft?

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 14 Aeronautics and Space 4 2010-01-01 2010-01-01 false What information must carriers give individuals with vision or hearing impairment on aircraft? 382.119 Section 382.119 Aeronautics and Space... carriers give individuals with vision or hearing impairment on aircraft? (a) As a carrier, you must...

  19. 14 CFR 382.53 - What information must carriers give individuals with a vision or hearing impairment at airports?

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 14 Aeronautics and Space 4 2010-01-01 2010-01-01 false What information must carriers give individuals with a vision or hearing impairment at airports? 382.53 Section 382.53 Aeronautics and Space... information must carriers give individuals with a vision or hearing impairment at airports? (a)(1) As a...

  20. 14 CFR 382.119 - What information must carriers give individuals with vision or hearing impairment on aircraft?

    Code of Federal Regulations, 2013 CFR

    2013-01-01

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

  1. 14 CFR 382.53 - What information must carriers give individuals with a vision or hearing impairment at airports?

    Code of Federal Regulations, 2012 CFR

    2012-01-01

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

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

    ERIC Educational Resources Information Center

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

    2015-01-01

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

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

    ERIC Educational Resources Information Center

    Liu, Chen-Chung; Hong, Yi-Ching

    2007-01-01

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

  4. A Study of the Development of Eight Hearing-Impaired Preschool Children from Spanish-Speaking Homes.

    ERIC Educational Resources Information Center

    Grant, June M.

    Case studies of eight hearing impaired preschool children from Spanish speaking homes were presented in this dissertation. The children and their parents were enrolled in the Trinity University Parent/Infant Program for hearing impaired infants from Spanish speaking homes. The case studies consisted of family and medical histories, audiological…

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

    ERIC Educational Resources Information Center

    Bahatheg, Raja Omar

    2015-01-01

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

  6. The Turkish Standardization of the Meadow-Kendall Social-Emotional Assessment Inventory for Deaf and Hearing-Impaired Students

    ERIC Educational Resources Information Center

    Polat, Filiz

    2006-01-01

    The article present results of standardization of the Meadow-Kendall Social-Emotional Assessment Inventory for Deaf and Hearing-Impaired Students (Meadow, 1983), school-age version, for use in Turkey. The SEAI is a 59-item measure for assessing socioemotional adjustment of school-age deaf and hearing impaired students. A sample of 1,097 deaf…

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

    ERIC Educational Resources Information Center

    Strong, Carol J.; Clark, Thomas C.

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

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

    ERIC Educational Resources Information Center

    Luckner, John L.

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

  9. Perception of Spectral Contrast by Hearing-Impaired Listeners

    ERIC Educational Resources Information Center

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

    2005-01-01

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

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

    ERIC Educational Resources Information Center

    Garrett, Ann; Hastings, Chester R.

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

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

    ERIC Educational Resources Information Center

    Smith, Dorothy

    1988-01-01

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

  12. Preventive Audiology: Screening for Hearing Impairment in Children Having Recurrent URTI.

    PubMed

    Sharma, K; Pannu, M S; Arora, A; Sharma, V

    2016-06-01

    A late detection of hearing impairment in children can affect speech and language development. Otitis media with effusion (OME) associated with risk factors like recurrent upper respiratory tract infections (URTI) is considered the most common cause of silent hearing impairment among children. So this study was carried out to screen such at risk children for hearing impairment. The study was conducted on 1000 children in the age group of 2-12 years who presented to the OPD of ENT and Pediatrics Department, Govt Medical College with the complaint of recurrent URTI. The children were screened by history taking, general physical examination and local ENT examination. This was followed by impedance audiometry and pure tone audiometry (PTA, wherever indicated). Children with discharging ears and perforated tympanic membrane (TM) were excluded from the study. The most common presenting complaint was nasal discharge (67.9 %), followed by mouth breathing (66.3 %) and snoring (65 %). Only 16.2 % cases actually complained of some hearing impairment on exploratory history indicating the silent nature of OME. On examination 52.65 % cases had a normal looking TM whereas 41.2 % had a dull retracted TM. Results of impedance audiometry showed Type A graph in 56.75 %, Type B in 33.4 % indicating OME and Type C in 9.85 % indicating Eustachian tube dysfunction. PTA showed a mild conductive hearing loss in 26.7 % cases. OME is quite prevalent in high risk children and incidence of OME resulting in silent hearing impairment is quite high especially in young children having recurrent URTI. Impedance audiometry has been proved to be an objective screening tool for the same with a diagnostic accuracy of 87 % and thus under preventive audiology, it has a definite role in young children having recurrent URTI. PMID:27340630

  13. Risk of Sudden Sensorineural Hearing Loss in Patients with Common Preexisting Sensorineural Hearing Impairment: A Population-Based Study in Taiwan

    PubMed Central

    Koo, Malcolm; Hwang, Juen-Haur

    2015-01-01

    Objective The role of preexisting sensorineural hearing impairment on the risk for sudden sensorineural hearing loss (SSHL) is still unclear. In this study, we aimed to assess the risk of SSHL in patients with common preexisting sensorineural hearing impairment using population-based data. Methods A population-based case-control study design was used to analyze claims data between January 2001 and December 2011 obtained from the Taiwan National Health Insurance Research Database. The cases consisted of 514 patients with SSHL and the controls were frequency matched to 2,570 cases by sex, 10-year age group, and year of index date. Common sensorineural hearing impairments were retrospectively assessed in the cases and controls. Associations between sensorineural hearing impairment and risk of SSHL were evaluated using unconditional univariate and multivariate logistic regression analyses. Results The mean age for the 3,084 study subjects was 53.1 years (standard deviation, S.D. = 15.6). Of the 514 cases, 49 (9.5%) had sensorineural hearing impairment while only 44 (1.7%) of the 2,570 controls had the same condition. Univariate logistic regression analyses indicated that preexisting sensorineural hearing impairment was significantly associated with SSHL (odds ratio, OR = 6.05, p < 0.001). Other comorbidities including hypertension, diabetes mellitus, and hyperlipidemia also showed significant associations with SSHL. Similar results were obtained when the association between SSHL and sensorineural hearing impairment was adjusted with either all the covariates (adjusted OR = 6.22, p < 0.001) or with only those selected using a backward elimination procedure (adjusted OR = 6.20, p < 0.001). Conclusions Results from this population-based case-control study revealed that common sensorineural hearing impairment might be a novel risk factor for SSHL. PMID:25815507

  14. The impact of hearing impairment on television viewing in the UK.

    PubMed

    Sancho-Aldridge, J; Davis, A

    1993-06-01

    Just under one in 10 of a nationally representative sample of UK television viewers said that they experienced difficulty with their hearing. These hard-of-hearing viewers were found to report much greater difficulty watching programmes (mean difficulty rating = 32%) than elderly viewers with no reported hearing difficulty (mean difficulty rating = 10%), or those viewers generally who said they had no hearing problems (mean difficulty rating = 3%). Using a similarly constructed rating for reported enjoyment of different television programmes, hard-of-hearing viewers were found to exhibit a small reduction in enjoyment across the majority of programmes types. While it might have been anticipated that a greater proportion of those with impaired hearing owned a teletext television set giving them access to subtitling, this was not found to be the case. Across the sample as a whole, teletext ownership was shown to be 45%, but was lower than this (38.5%) amongst the hard-of-hearing. The findings corroborate what has been shown in another study, namely that teletext ownership is lower among older viewers. Hearing impairment, if it is not congenital or of early childhood origin, is a condition associated with increasing age. Thus, those whose viewing and appreciation of programmes might be enhanced by subtitles, in the main, do not have access to them. Among hard-of-hearing viewers who did have access to the teletext subtitle service, two thirds of those aged 51 years and over felt that subtitles assisted their understanding of television programmes. As one might expect, of those owning teletext, hard-of-hearing viewers reported greatest use of subtitles. Thirteen per cent of those with hearing difficulty and aged over 51 years said they used subtitles for all programmes watched and a further 26% of the over fifties with hearing difficulty reported regularly using subtitles for selected programmes. These data advocate that there are many hard-of-hearing viewers whose

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

    SciTech Connect

    Von Gierke, H.E.

    1986-01-01

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

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

    PubMed Central

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

    2016-01-01

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

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

    PubMed Central

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

    2013-01-01

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

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

    PubMed

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

    1986-03-20

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

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

    PubMed

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

    2013-01-01

    This paper describes the design, development, and tests of a low cost ALS. It was designed for hearing-impaired student classrooms. It utilised digital wireless technology and was aimed to be an alternative to a popular FM ALS. Key specifications include transmitting in 2.4 GHz ISM band with eight selectable transmission channels, battery operated and chargeable, pocket size, and ranged up to thirty metres. Audio characteristics and user tests show that it is comparable to a commercial system, currently employed in our partner school. The results also show that wearing an ALS clearly improves hearing of hearing-impaired students. Long-term usage by school children will be monitored to evaluate the system robustness and durability. PMID:23766709

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

    PubMed

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

    2013-01-01

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

  1. Development of a Low Cost Assistive Listening System for Hearing-Impaired Student Classroom

    PubMed Central

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

    2013-01-01

    This paper describes the design, development, and tests of a low cost ALS. It was designed for hearing-impaired student classrooms. It utilised digital wireless technology and was aimed to be an alternative to a popular FM ALS. Key specifications include transmitting in 2.4 GHz ISM band with eight selectable transmission channels, battery operated and chargeable, pocket size, and ranged up to thirty metres. Audio characteristics and user tests show that it is comparable to a commercial system, currently employed in our partner school. The results also show that wearing an ALS clearly improves hearing of hearing-impaired students. Long-term usage by school children will be monitored to evaluate the system robustness and durability. PMID:23766709

  2. [Intrauterine infections as a risk factor of the development of sensorineural impairment of hearing ].

    PubMed

    Boboshko, M Yu; Vikhnin, S M; Savenko, I V

    2016-01-01

    Intrauterine infections are a crucial pathogenic factor exerting an appreciable influence on the development of the fetus. They can provoke intrauterine death, cause multiple lesions in the organs and tissues as well as long-term complications that manifest themselves at the later stages of the growth and development of the child. One of such complications is the sensorineural loss of hearing. The importance of hearing impairment arises from the high prevalence of tis condition and frequent incapacitation it causes in the patients. The present publication is focused on various mechanisms underlying the development of hearing impairment depending on the primary infection. Special attention is given to the methods of diagnostics and treatment of intrauterine infections. PMID:27351041

  3. Postsecondary Education Facilities for Hearing Impaired Albertans: Cross-Canada Implications.

    ERIC Educational Resources Information Center

    Schein, Jerome D.; Mallory, Barbara L.

    1990-01-01

    A survey of 54 public and private postsecondary institutions in Alberta (Canada) found that 20 provided special services to hearing-impaired students in the past 5 years. Interviews with administrators at the 20 institutions examined enrollment, identification, services, and social life. Students receiving special educational assistance totaled…

  4. Selected Applications of Computer-Assisted Video Instruction in the Education of Hearing-Impaired Students.

    ERIC Educational Resources Information Center

    Dillingham, Larry M.; And Others

    1982-01-01

    The paper illustrates two applications of computer assisted video instruction (CAVI) techniques for educating deaf and hearing impaired students in vocational education. CAVI involves the interactive qualities of microcomputers and the visual impact of videotape. This partnership lends itself naturally to two basic approaches: linear instruction…

  5. Documenting Syntactically and Semantically Incomplete Bimodal Input to Hearing-Impaired Subjects.

    ERIC Educational Resources Information Center

    Luetke-Stahlman, Barbara

    1988-01-01

    The study evaluated characteristics of instructional bimodal communication in classrooms for the hearing impaired using Signing Exact English or Signed English. Findings indicated some teachers accurately and proficiently encoded semantic information in their instruction. A requirement of 80% or better voice-to-sign ratio ability is suggested for…

  6. Recruiting Black Teacher-Trainees into Programs for the Hearing Impaired.

    ERIC Educational Resources Information Center

    Luetke-Stahlman, B.

    1983-01-01

    To examine ways to increase recruitment of minority students into college deaf education training programs a questionnaire was randomly sent to programs for the hearing impaired employing more than 100 staff. Data from a variety of Black respondents (57 percent) resulted in suggestions for encouraging minority recruitment. (Author/CL)

  7. Gaining Methodological Insight through the Use of Single Subject Designs in Hearing-Impaired Classrooms.

    ERIC Educational Resources Information Center

    Luetke-Stahlman, Barbara

    1986-01-01

    Teachers should familiarize themselves with the practical advantages of single-subject methodology in attempting to meet the various learning needs of hearing-impaired students. Two examples are provided to illustrate how these designs can act as tools for deciding which of several teaching methods are most beneficial to particular students.…

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

    ERIC Educational Resources Information Center

    Holmes, Alice E.

    1985-01-01

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

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

    ERIC Educational Resources Information Center

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

    1983-01-01

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

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

    ERIC Educational Resources Information Center

    Wentzer, Carol; Dhir, Annie

    1986-01-01

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

  11. Cognitive Enhancement of Hearing-Impaired Post-Secondary Students. (Revised).

    ERIC Educational Resources Information Center

    Martin, David S.; Jonas, Bruce S.

    A study focusing on the serious need for active programs in improving the cognitive skills of hearing-impaired students is examined. Six hypotheses are presented as potential outcomes of using the Instrumental Enrichment (IE) program (classroom intervention for adolescents and adults using content-free paper and pencil exercises to correct…

  12. Teaching Music as an Aid for Speech Training of Hearing Impaired Students.

    ERIC Educational Resources Information Center

    Shaw, Jeanne

    1989-01-01

    The article reviews existing theories and programs for teaching music to hearing-impaired students. Recent empirical evidence indicates that an auditory-based music program can increase speech intelligibility through improvement of the suprasegmental aspects of speech. (Author/DB)

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

    ERIC Educational Resources Information Center

    Stuckless, E. Ross; Walter, Gerard G.

    1983-01-01

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

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

    ERIC Educational Resources Information Center

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

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

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

    ERIC Educational Resources Information Center

    Litus, Tonyia J.

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

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

    ERIC Educational Resources Information Center

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

    2013-01-01

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

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

    ERIC Educational Resources Information Center

    Hanson, Ronald H.

    1983-01-01

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

  18. The Effects of Arts and Crafts Education on Hearing-Impaired Children.

    ERIC Educational Resources Information Center

    Yuan-shih, Pan

    1984-01-01

    Arts and crafts education opens up the creative ambitions of hearing-impaired children, cultivates their ability to express themselves, strengthens their organizational ability, helps them develop visual and tactile sensitivity, and increases their interest in learning. Self-respect and self-confidence are the result. (RM)

  19. Aspects of Spatio-Temporal Variability during Consonant Production by Greek Speakers with Hearing Impairment

    ERIC Educational Resources Information Center

    Nicolaidis, Katerina

    2007-01-01

    This paper investigates spatio-temporal variability during the production of the lingual consonants /t, k, s, x, n, l, "r"/ by four Greek speakers with profound hearing impairment and with differences in the intelligibility of their speech. It examines important factors that have been documented to influence intelligibility, i.e. durational…

  20. Articulatory Variability during Consonant Production by Greek Speakers with Hearing Impairment: An Electropalatographic Study

    ERIC Educational Resources Information Center

    Nicolaidis, Katerina

    2004-01-01

    This study uses the technique of electropalatography to investigate lingualpalatal contact patterns during the production of the consonants /t, k, s, x, n, l, / by four Greek speakers with profound hearing impairment and with differences in the intelligibility of their speech. The study provides a detailed description of their tongue-palate…

  1. The Ohio Achievement Assessment and Deaf/Hearing Impaired Students: Have They Been Left Behind?

    ERIC Educational Resources Information Center

    Davis, Tanya S. Williams

    2013-01-01

    The purposes of this study were 1) to examine Ohio Achievement Assessment data in the content areas of Reading, Science and Social Studies between the years of 2004-2010) to determine whether an achievement gap exists within the disability category of Deaf/Hearing Impaired; 2) to determine whether the trends present in the data of normal hearing…

  2. Competence in Communication for Hearing Impaired Children: A Conversation, Activity, Experience Approach.

    ERIC Educational Resources Information Center

    Clarke, B. R.

    1983-01-01

    A historical review of methods of language instruction for hearing impaired children is followed by a description of an approach which emphasizes conversation (via oral language, sign languge, or a combination) in natural settings. Activities to facilitate conversation as well as to directly teach language skills are incorporated in the approach.…

  3. Science for the Hearing Impaired. Teachers Guide. Introduction and Levels 3-7.

    ERIC Educational Resources Information Center

    Sunal, Dennis W.; Sunal, Cynthia Szymanski

    Intended for use with hearing impaired students, ages 9 to 13 years, the curriculum guide is an adaptation of an existing commercially available school science program with the addition of new materials. Adaptation included reorganization of some materials; paraphrasing to aid the student with textual material; isolation of key words and phrases;…

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

    ERIC Educational Resources Information Center

    Karasu, H. Pelin

    2014-01-01

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

  5. The Oralingua Social Studies Curriculum in a Functional Approach to Language for Preschool Hearing Impaired Children.

    ERIC Educational Resources Information Center

    Ritter-Brinton, Kathryn

    1990-01-01

    This paper discusses the positive effects on young deaf children's and teachers' communicative attitudes of a functional approach to language intervention (developed in the Preschool Program for the Hearing Impaired at the Glenrose Rehabilitation Hospital in Edmonton, Alberta, Canada) used in combination with the Oralingua preschool social studies…

  6. Level variations in speech: Effect on masking release in hearing-impaired listeners.

    PubMed

    Reed, Charlotte M; Desloge, Joseph G; Braida, Louis D; Perez, Zachary D; Léger, Agnès C

    2016-07-01

    Acoustic speech is marked by time-varying changes in the amplitude envelope that may pose difficulties for hearing-impaired listeners. Removal of these variations (e.g., by the Hilbert transform) could improve speech reception for such listeners, particularly in fluctuating interference. Léger, Reed, Desloge, Swaminathan, and Braida [(2015b). J. Acoust. Soc. Am. 138, 389-403] observed that a normalized measure of masking release obtained for hearing-impaired listeners using speech processed to preserve temporal fine-structure (TFS) cues was larger than that for unprocessed or envelope-based speech. This study measured masking release for two other speech signals in which level variations were minimal: peak clipping and TFS processing of an envelope signal. Consonant identification was measured for hearing-impaired listeners in backgrounds of continuous and fluctuating speech-shaped noise. The normalized masking release obtained using speech with normal variations in overall level was substantially less than that observed using speech processed to achieve highly restricted level variations. These results suggest that the performance of hearing-impaired listeners in fluctuating noise may be improved by signal processing that leads to a decrease in stimulus level variations. PMID:27475136

  7. Developing Effective Learning Material to Students with Hearing Impairment (HI) through ODL in Zimbabwe

    ERIC Educational Resources Information Center

    Mpofu, John; Chimhenga, Sylod; Mafa, Onias

    2013-01-01

    Students with Hearing Impairment (HI) are experiencing learning problems in most institutions of Higher Learning in Zimbabwe. Access to colleges and universities is limited and where they are accepted, there are no facilities to cater for their needs, hence there is need to develop learning materials that enable these students to learn effectively…

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

    ERIC Educational Resources Information Center

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

    2012-01-01

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

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

    ERIC Educational Resources Information Center

    Schloss, Patrick J.

    1984-01-01

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

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

    ERIC Educational Resources Information Center

    El-Zraigat, Ibrahim A.

    2013-01-01

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

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

    ERIC Educational Resources Information Center

    Wilson, Jimmie Joan

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

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

    ERIC Educational Resources Information Center

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

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

  13. Progress and prospects in human genetic research into age-related hearing impairment.

    PubMed

    Uchida, Yasue; Sugiura, Saiko; Sone, Michihiko; Ueda, Hiromi; Nakashima, Tsutomu

    2014-01-01

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

  14. Metalinguistic Abilities of Young Hearing-Impaired Children: Performance on a Judgment of Synonomy Task.

    ERIC Educational Resources Information Center

    Borman, Deborah L.; And Others

    1988-01-01

    Metalinguistic abilities of 20 hearing-impaired children, aged 4-10, were assessed by asking them to judge synonymy of sentence pairs presented in Signed English, Pidgin Sign English, and American Sign Language. None of the children had developed metalinguistic abilities in any of the sign language systems. (Author/JDD)

  15. Relationship between Imaginative Play and Language Development in Hearing-Impaired Children.

    ERIC Educational Resources Information Center

    Schirmer, Barbara R.

    1989-01-01

    Twenty severely/profoundly hearing-impaired children, aged three-six, interacted with the investigator while playing with toys. A significant relationship was found between imaginative play and language development. No relationship was found between imaginative play and chronological age. Correspondences were found between language development and…

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

    ERIC Educational Resources Information Center

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

    2003-01-01

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

  17. Intellectual Estimates of Hearing-Impaired Children: A Comparison of Three Measures

    ERIC Educational Resources Information Center

    Ritter, David R.

    1976-01-01

    The Arthur Adaptation of the Leiter International Performance Scale, Raven's Coloured Progressive Matrices, and Wechsler Intelligence Scale for Children-Performance Section were administered to 31 children with mild to moderate hearing impairments. A comparison of test results indicated moderate convergent validity among the measures. (Author)

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

    ERIC Educational Resources Information Center

    Jaggers, Robert A.; Jaggers, Barbara A.

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

  19. Sexual vulnerability and HIV seroprevalence among the deaf and hearing impaired in Cameroon

    PubMed Central

    2010-01-01

    Background This quantitative cross-sectional study examines sexual behaviour of a target group of hearing-impaired persons in Yaounde, the capital city of the Republic of Cameroon. It measures their HIV prevalence to enable assessment of their sexual vulnerability and to help reduce the gap in existing HIV serology data among people with disabilities in general and the deaf in particular. Methods The snowball sampling procedure was adopted as an adequate approach to meet this hard-to-reach group. A total of 118 deaf participants were interviewed for the behavioural component, using sign language as a means of data collection, while 101 participants underwent HIV serology testing. Descriptive analyses were done for behavioural data with Epi info software, while sera were tested by health personnel, using rapid and confirmation test reagents. Results From the results, it was clear that the hearing impaired were highly involved in risky sexual practices, as observed through major sexual indicators, such as: age at first sexual intercourse; condom use; and knowledge of sexually transmitted infections and AIDS. Furthermore, it was noted that the HIV prevalence rate of the hearing impaired in the capital of Cameroon was 4%, close to the prevalence in the city's general population (4.7%). Conclusions Such results suggest that there is a need for in-depth behavioural research and serological studies in this domain to better understand the determinants of risky sexual behaviour among the hearing impaired, and to propose operational prevention approaches for this group. PMID:20181091

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

    ERIC Educational Resources Information Center

    Turkestani, Maryam Hafez

    2015-01-01

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

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

    ERIC Educational Resources Information Center

    Suppes, Patrick; And Others

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

  2. So You Have a Hearing Impaired Student in Your Vocational Education Classroom. (A Prototypic Resource Unit).

    ERIC Educational Resources Information Center

    Thompson, Jeanne A.

    This resource unit is a prototype for others that can be developed as a team effort by vocational and special educators concerned with mainstreaming hearing impaired youth into regular vocational education programs. Although focusing on a specific vocational area (food service), the unit's step-by-step design can be applied to other vocational…

  3. Classification of the Hearing Impaired for Independent Living Using the Vineland Adaptive Behavior Scale.

    ERIC Educational Resources Information Center

    Dunlap, William R.; Sands, Deanna Iceman

    1990-01-01

    The Vineland Adaptive Behavior Scale was used to classify 118 hearing-impaired persons (88 percent were ages 16-21) into groups based on their ability to be trained in independent living skills. Using cluster analysis, the subjects were placed into three groups according to four domains: communication, daily living, socialization, and maladaptive…

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

    ERIC Educational Resources Information Center

    Jaworski, Anne Porter; Schroder, Ann

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

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

    ERIC Educational Resources Information Center

    Kuzu, Abdullah

    2011-01-01

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

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

    ERIC Educational Resources Information Center

    Hack, Zarita Caplan; Erber, Norman P.

    1982-01-01

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

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

    ERIC Educational Resources Information Center

    Gatty, Janice C.

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

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

    ERIC Educational Resources Information Center

    West, Lois

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

  9. Language and Learning Skills of Hearing-Impaired Students: ASHA Monographs Number 23.

    ERIC Educational Resources Information Center

    Osberger, Mary Joe, Ed.

    The monograph consists of 13 author contributed chapters arising out of a Nebraska study which attempted to quantify the performance of 150 profoundly hearing impaired students (4-20 years old) in the areas of language (receptive and expressive), academics (reading, spelling, math), and related learning (visual perception and short-term memory)…

  10. Literacy and the Hearing Impaired Conference (Washington, D.C., May 18-20, 1989). [Proceedings.

    ERIC Educational Resources Information Center

    Center for Systems and Program Development, Inc., Washington, DC.

    This document presents conference materials and five conference papers on literacy and the hearing impaired. Preceding the papers, a section of general information provides an introduction, conference format information, and a commentary by Michael Rodda. Recommendations from the work groups and an executive summary are then presented.…

  11. Modality Aspects of Mediation in Children with Normal and with Impaired Hearing Ability. Final Report.

    ERIC Educational Resources Information Center

    Allen, Doris V.

    Three experiments tested whether qualitative differences in processing of verbal materials result from congenital hearing impairment. Subjects were children with reading levels equivalent to grades 4 to 6. Experiment 1 used repeated measurements with two modes of response and two kinds of cues; experiment 2 used acoustic similarity to produce…

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

    PubMed

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

    2014-09-01

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

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

    NASA Astrophysics Data System (ADS)

    Formby, C.; Gagne, J. P.

    2002-05-01

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

  14. Recommendations of the NIDCD Working Group on Early Identification of Hearing Impairment on Acceptable Protocols for Use in State-Wide Universal Newborn Hearing Screening Programs.

    ERIC Educational Resources Information Center

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

    This document presents the consensus statement of a 2-day conference which addressed issues concerned with the early identification of hearing impairment. The conference resulted in the following consensus conclusions: (1) all infants admitted to the neonatal intensive care unit should be screened for hearing loss prior to discharge; (2) universal…

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

    PubMed Central

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

    2013-01-01

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

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

    PubMed

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

    2015-12-01

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

  17. Genetic Basis of Nonsyndromic Sensorineural Hearing Loss in the Sub-Saharan African Island Population of São Tomé and Príncipe: The Role of the DFNB1 Locus?

    PubMed

    Caroça, Cristina; de Matos, Tiago Morim; Ribeiro, Diogo; Lourenço, Vera; Martins, Tânia; Campelo, Paula; Fialho, Graça; Silva, Susana Nunes; Paço, João; Caria, Helena

    2016-08-01

    Hearing loss (HL) is a common condition with both genetic and environmental causes, and it greatly impacts global health. The prevalence of HL is reportedly higher in developing countries such as the Sub-Saharan African island of São Tomé and Príncipe, where the deaf community is estimated to be less than 1% of the population. We investigated the role of the DFNB1 locus (GJB2 and GJB6 genes) in the etiology of nonsyndromic sensorineural hearing loss (NSSHL) in São Tomé and Príncipe. A sample of 316 individuals, comprising 136 NSSHL patients (92 bilateral, 44 unilateral) and 180 controls, underwent a clinical and audiological examination. Sequencing of the GJB2 coding region and testing for the (GJB6-D13S1830) and del(GJB6-D13S1854) GJB6 deletions were performed. A total of 311 out of 316 individuals were successfully analyzed regarding the GJB2 and GJB6 genetic variations, respectively. The frequency of the GJB2 coding mutations in patients and controls was low. Some of those coding mutations are the most commonly found in Eurasian and Mediterranean populations and have also been identified in Portugal. None of the GJB6 deletions was present. The presence of certain coding variants in São Tomé and Príncipe suggests a non-Sub-Saharan genetic influx and supports the previously reported genetic influx from European (mainly Portuguese) ancestors. In summary, DFNB1 locus does not appear to be a major contributor to NSSHL in São Tomé and Príncipe. However, the presence of both pathogenic and likely pathogenic mutations in GJB2 suggests that GJB2-related NSSHL might still occur in this population, warranting further research on GJB2 testing in NSSHL cases. PMID:27501294

  18. Hearing impairment and incident disability and all-cause mortality in older British community-dwelling men

    PubMed Central

    Liljas, Ann E. M.; Wannamethee, S. Goya; Whincup, Peter H.; Papacosta, Olia; Walters, Kate; Iliffe, Steve; Lennon, Lucy T.; Carvalho, Livia A.; Ramsay, Sheena E.

    2016-01-01

    Background and objective: hearing impairment is common in older adults and has been implicated in the risk of disability and mortality. We examined the association between hearing impairment and risk of incident disability and all-cause mortality. Design and setting: prospective cohort of community-dwelling older men aged 63–85 followed up for disability over 2 years and for all-cause mortality for 10 years in the British Regional Heart Study. Methods: data were collected on self-reported hearing impairment including hearing aid use, and disability assessed as mobility limitations (problems walking/taking stairs), difficulties with activities of daily living (ADL) and instrumental ADL (IADL). Mortality data were obtained from the National Health Service register. Results: among 3,981 men, 1,074 (27%) reported hearing impairment. Compared with men with no hearing impairment, men who could hear and used a hearing aid, and men who could not hear despite a hearing aid had increased risks of IADL difficulties (age-adjusted OR 1.86, 95% CI 1.29–2.70; OR 2.74, 95% CI 1.53–4.93, respectively). The associations remained after further adjustment for covariates including social class, lifestyle factors, co-morbidities and social engagement. Associations of hearing impairment with incident mobility limitations, incident ADL difficulties and all-cause mortality were attenuated on adjustment for covariates. Conclusion: this study suggests that hearing problems in later life could increase the risk of having difficulties performing IADLs, which include more complex everyday tasks such as shopping and light housework. However, further studies are needed to determine the associations observed including the underlying pathways. PMID:27146303

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

    PubMed Central

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

    2016-01-01

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

  20. Auditory Spatial Discrimination and the Mismatch Negativity Response in Hearing-Impaired Individuals

    PubMed Central

    Cai, Yuexin; Zheng, Yiqing; Liang, Maojin; Zhao, Fei; Yu, Guangzheng; Liu, Yu; Chen, Yuebo; Chen, Guisheng

    2015-01-01

    The aims of the present study were to investigate the ability of hearing-impaired (HI) individuals with different binaural hearing conditions to discriminate spatial auditory-sources at the midline and lateral positions, and to explore the possible central processing mechanisms by measuring the minimal audible angle (MAA) and mismatch negativity (MMN) response. To measure MAA at the left/right 0°, 45° and 90° positions, 12 normal-hearing (NH) participants and 36 patients with sensorineural hearing loss, which included 12 patients with symmetrical hearing loss (SHL) and 24 patients with asymmetrical hearing loss (AHL) [12 with unilateral hearing loss on the left (UHLL) and 12 with unilateral hearing loss on the right (UHLR)] were recruited. In addition, 128-electrode electroencephalography was used to record the MMN response in a separate group of 60 patients (20 UHLL, 20 UHLR and 20 SHL patients) and 20 NH participants. The results showed MAA thresholds of the NH participants to be significantly lower than the HI participants. Also, a significantly smaller MAA threshold was obtained at the midline position than at the lateral position in both NH and SHL groups. However, in the AHL group, MAA threshold for the 90° position on the affected side was significantly smaller than the MMA thresholds obtained at other positions. Significantly reduced amplitudes and prolonged latencies of the MMN were found in the HI groups compared to the NH group. In addition, contralateral activation was found in the UHL group for sounds emanating from the 90° position on the affected side and in the NH group. These findings suggest that the abilities of spatial discrimination at the midline and lateral positions vary significantly in different hearing conditions. A reduced MMN amplitude and prolonged latency together with bilaterally symmetrical cortical activations over the auditory hemispheres indicate possible cortical compensatory changes associated with poor behavioral spatial

  1. Comparison of Various Anthropometric Indices as Risk Factors for Hearing Impairment in Asian Women

    PubMed Central

    Lee, Kyu Yup; Choi, Eun Woo; Do, Jun Young

    2015-01-01

    Background The objective of the present study was to examine the associations between various anthropometric measures and metabolic syndrome and hearing impairment in Asian women. Methods We identified 11,755 women who underwent voluntary routine health checkups at Yeungnam University Hospital between June 2008 and April 2014. Among these patients, 2,485 participants were <40 years old, and 1,072 participants lacked information regarding their laboratory findings or hearing and were therefore excluded. In total 8,198 participants were recruited into our study. Results The AUROC value for metabolic syndrome was 0.790 for the waist to hip ratio (WHR). The cutoff value was 0.939. The sensitivity and specificity for predicting metabolic syndrome were 72.7% and 71.7%, respectively. The AUROC value for hearing loss was 0.758 for WHR. The cutoff value was 0.932. The sensitivity and specificity for predicting hearing loss were 65.8% and 73.4%, respectively. The WHR had the highest AUC and was the best predictor of metabolic syndrome and hearing loss. Univariate and multivariate linear regression analyses showed that WHR levels were positively associated with four hearing thresholds including averaged hearing threshold and low, middle, and high frequency thresholds. In addition, multivariate logistic analysis revealed that those with a high WHR had a 1.347–fold increased risk of hearing loss compared with the participants with a low WHR. Conclusion Our results demonstrated that WHR may be a surrogate marker for predicting the risk of hearing loss resulting from metabolic syndrome. PMID:26575369

  2. Oral communication in individuals with hearing impairment-considerations regarding attentional, cognitive and social resources.

    PubMed

    Lemke, Ulrike; Scherpiet, Sigrid

    2015-01-01

    Traditionally, audiology research has focused primarily on hearing and related disorders. In recent years, however, growing interest and insight has developed into the interaction of hearing and cognition. This applies to a person's listening and speech comprehension ability and the neural realization thereof. The present perspective extends this view to oral communication, when two or more people interact in social context. Specifically, the impact of hearing impairment and cognitive changes with age is discussed. In focus are executive functions, a group of top-down processes that guide attention, thought and action according to goals and intentions. The strategic allocation of the limited cognitive processing capacity among concurrent tasks is often effortful, especially under adverse communication conditions and in old age. Working memory, a sub-function extensively discussed in cognitive hearing science, is here put into the context of other executive and cognitive functions required for oral communication and speech comprehension. Finally, taking an ecological view on hearing impairment, activity limitations and participation restrictions are discussed regarding their psycho-social impact and third-party disability. PMID:26236268

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

    PubMed Central

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

    2016-01-01

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

  4. Excitatory, inhibitory and facilitatory frequency response areas in the inferior colliculus of hearing impaired mice.

    PubMed

    Felix, Richard A; Portfors, Christine V

    2007-06-01

    Individuals with age-related hearing loss often have difficulty understanding complex sounds such as basic speech. The C57BL/6 mouse suffers from progressive sensorineural hearing loss and thus is an effective tool for dissecting the neural mechanisms underlying changes in complex sound processing observed in humans. Neural mechanisms important for processing complex sounds include multiple tuning and combination sensitivity, and these responses are common in the inferior colliculus (IC) of normal hearing mice. We examined neural responses in the IC of C57Bl/6 mice to single and combinations of tones to examine the extent of spectral integration in the IC after age-related high frequency hearing loss. Ten percent of the neurons were tuned to multiple frequency bands and an additional 10% displayed non-linear facilitation to the combination of two different tones (combination sensitivity). No combination-sensitive inhibition was observed. By comparing these findings to spectral integration properties in the IC of normal hearing CBA/CaJ mice, we suggest that high frequency hearing loss affects some of the neural mechanisms in the IC that underlie the processing of complex sounds. The loss of spectral integration properties in the IC during aging likely impairs the central auditory system's ability to process complex sounds such as speech. PMID:17412539

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

    PubMed Central

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

    2015-01-01

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

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

    NASA Astrophysics Data System (ADS)

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

    2007-12-01

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

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

    PubMed

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

    2016-01-01

    This article presents data on the prevalence of hearing impairment among the workers engaged in the main means of transportation(air and railway transport). They show that the relative frequency of occupational loss of hearing in the cockpit members of commercial aviation amounts to one third of all cases of analogous diseases in this country. The main professional groups of transport works suffering from hearing impairment are constituted by the representatives of the so-called elite specialities, such as flying crew personnel, locomotive engineers, and their assistants. This fact constitutes an important aspect (not only of medical but also of socio-economic significance) of the problem under consideration. The high prevalence of professional hearing impairment among the transport workers is attributable to the high noise level in the cabins of locomotives and aircraft cockpits as well as to the inadequate expert and diagnostic work or imperfection of the regulatory documentation. PMID:26977561

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

    PubMed

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

    2016-01-01

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

  9. The effects of hearing impairment on the ability to glimpse speech in a spectro-temporally complex noise

    NASA Astrophysics Data System (ADS)

    Ozmeral, Erol James

    The aim of this project was to investigate the effects of hearing impairment on speech perception in spectro-temporally complex noise. The specific objective of the project was to psychophysically and computationally assess speech reception in the presence of a masker that fluctuates both in time and frequency. The experiments were designed to compare hearing-impaired and normal-hearing listeners on a task which has been shown to highlight the effect of spread of masking. Through dichotic stimulation, a previous study had shown a sizeable benefit when compared to monaural stimulation. Experiment 1 tested normal-hearing and hearing-impaired listeners on consonant recognition in the presence of an asynchronously modulated noise. We tested the primary hypotheses that spread of masking reduces available glimpsing opportunities for hearing-impaired listeners, and that removing spread of masking enhances performance relative to normal-hearing listeners. Results showed greater masking release in normal-hearing listeners compared to hearing-impaired listeners, but all listeners achieved some benefit of reducing the effects of spread of masking. Experiment 2 tested consonant recognition in similar masking conditions as Experiment 1, testing normal-hearing listeners with simulated reduced audibility and reduced frequency resolution. We tested the primary hypothesis that reduced audibility is not the only limiting factor for hearing-impaired listeners to glimpse speech, but rather, that reduced frequency resolution also plays an important role in the ability to glimpse speech in spectro-temporally complex noise. Results showed that while reduced audibility was a key factor, reduced frequency resolution also contributes to deficits seen in Experiment 1. Experiment 3 tested a computational glimpsing model. We tested the hypotheses that spectral resolution plays a key role in glimpsing for both normal-hearing and hearing-impaired listeners; by analyzing dichotically presented

  10. Gated Auditory Speech Perception in Elderly Hearing Aid Users and Elderly Normal-Hearing Individuals: Effects of Hearing Impairment and Cognitive Capacity

    PubMed Central

    Lidestam, Björn; Hällgren, Mathias; Rönnberg, Jerker

    2014-01-01

    This study compared elderly hearing aid (EHA) users and elderly normal-hearing (ENH) individuals on identification of auditory speech stimuli (consonants, words, and final word in sentences) that were different when considering their linguistic properties. We measured the accuracy with which the target speech stimuli were identified, as well as the isolation points (IPs: the shortest duration, from onset, required to correctly identify the speech target). The relationships between working memory capacity, the IPs, and speech accuracy were also measured. Twenty-four EHA users (with mild to moderate hearing impairment) and 24 ENH individuals participated in the present study. Despite the use of their regular hearing aids, the EHA users had delayed IPs and were less accurate in identifying consonants and words compared with the ENH individuals. The EHA users also had delayed IPs for final word identification in sentences with lower predictability; however, no significant between-group difference in accuracy was observed. Finally, there were no significant between-group differences in terms of IPs or accuracy for final word identification in highly predictable sentences. Our results also showed that, among EHA users, greater working memory capacity was associated with earlier IPs and improved accuracy in consonant and word identification. Together, our findings demonstrate that the gated speech perception ability of EHA users was not at the level of ENH individuals, in terms of IPs and accuracy. In addition, gated speech perception was more cognitively demanding for EHA users than for ENH individuals in the absence of semantic context. PMID:25085610

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

    PubMed

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

    2014-01-01

    This study compared elderly hearing aid (EHA) users and elderly normal-hearing (ENH) individuals on identification of auditory speech stimuli (consonants, words, and final word in sentences) that were different when considering their linguistic properties. We measured the accuracy with which the target speech stimuli were identified, as well as the isolation points (IPs: the shortest duration, from onset, required to correctly identify the speech target). The relationships between working memory capacity, the IPs, and speech accuracy were also measured. Twenty-four EHA users (with mild to moderate hearing impairment) and 24 ENH individuals participated in the present study. Despite the use of their regular hearing aids, the EHA users had delayed IPs and were less accurate in identifying consonants and words compared with the ENH individuals. The EHA users also had delayed IPs for final word identification in sentences with lower predictability; however, no significant between-group difference in accuracy was observed. Finally, there were no significant between-group differences in terms of IPs or accuracy for final word identification in highly predictable sentences. Our results also showed that, among EHA users, greater working memory capacity was associated with earlier IPs and improved accuracy in consonant and word identification. Together, our findings demonstrate that the gated speech perception ability of EHA users was not at the level of ENH individuals, in terms of IPs and accuracy. In addition, gated speech perception was more cognitively demanding for EHA users than for ENH individuals in the absence of semantic context. PMID:25085610

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

    PubMed

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

    2014-05-01

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

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

    PubMed

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

    2014-05-01

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

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

    PubMed Central

    Neumann, Katrin J; Saunders, James E

    2014-01-01

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

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

    PubMed Central

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

    2014-01-01

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

  16. Interactive Auroral Science for Hearing-Impaired Students

    NASA Astrophysics Data System (ADS)

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

    2012-12-01

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

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

    PubMed

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

    2016-01-01

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

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

    PubMed Central

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

    2016-01-01

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

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

    PubMed Central

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

    2016-01-01

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

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

    PubMed

    King, S

    1990-01-01

    Conte (1983) suggested that existing theories of career development are inadequate for disabled populations because they fail to take into consideration the special life events and characteristics of people with a disability. The purpose of this study was to determine if Conte's reservations about contemporary theories could be supported by data. To this end, two causal models of career development were developed: one with five variables unique to the experience of the hearing impaired and the other without. Using data collected from 71 hearing-impaired adolescents, path analyses were conducted and the two models were compared for their ability to explain variance in career maturity. The results suggest that, although the second model may be more descriptive of the career development process for the deaf, it is no more powerful than the first in explaining variance in career maturity. PMID:2346105

  1. Audio-visual perception of compressed speech by profoundly hearing-impaired subjects.

    PubMed

    Drullman, R; Smoorenburg, G F

    1997-01-01

    For many people with profound hearing loss conventional hearing aids give only little support in speechreading. This study aims at optimizing the presentation of speech signals in the severely reduced dynamic range of the profoundly hearing impaired by means of multichannel compression and multichannel amplification. The speech signal in each of six 1-octave channels (125-4000 Hz) was compressed instantaneously, using compression ratios of 1, 2, 3, or 5, and a compression threshold of 35 dB below peak level. A total of eight conditions were composed in which the compression ratio varied per channel. Sentences were presented audio-visually to 16 profoundly hearing-impaired subjects and syllable intelligibility was measured. Results show that all auditory signals are valuable supplements to speechreading. No clear overall preference is found for any of the compression conditions, but relatively high compression ratios (> 3-5) have a significantly detrimental effect. Inspection of the individual results reveals that compression may be beneficial for one subject.

  2. Communication Variables Associated with Hearing-Impaired/Vision-Impaired Persons--A Pilot-Study.

    ERIC Educational Resources Information Center

    Hicks, Wanda M.

    1979-01-01

    A study involving eight youths and adults with retinitis pigmentosa (and only 20 degree visual field and hearing loss of at least 20 decibels) determined variance in the ability to perceive and comprehend visual stimuli presented by way of the manual modality when modifications were made in configuration, movement speed, movement size, and…

  3. Improving word recognition in noise among hearing-impaired subjects with a single-channel cochlear noise-reduction algorithm.

    PubMed

    Fink, Nir; Furst, Miriam; Muchnik, Chava

    2012-09-01

    A common complaint of the hearing impaired is the inability to understand speech in noisy environments even with their hearing assistive devices. Only a few single-channel algorithms have significantly improved speech intelligibility in noise for hearing-impaired listeners. The current study introduces a cochlear noise reduction algorithm. It is based on a cochlear representation of acoustic signals and real-time derivation of a binary speech mask. The contribution of the algorithm for enhancing word recognition in noise was evaluated on a group of 42 normal-hearing subjects, 35 hearing-aid users, 8 cochlear implant recipients, and 14 participants with bimodal devices. Recognition scores of Hebrew monosyllabic words embedded in Gaussian noise at several signal-to-noise ratios (SNRs) were obtained with processed and unprocessed signals. The algorithm was not effective among the normal-hearing participants. However, it yielded a significant improvement in some of the hearing-impaired subjects under different listening conditions. Its most impressive benefit appeared among cochlear implant recipients. More than 20% improvement in recognition score of noisy words was obtained by 12, 16, and 26 hearing-impaired at SNR of 30, 24, and 18 dB, respectively. The algorithm has a potential to improve speech intelligibility in background noise, yet further research is required to improve its performances.

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

    ERIC Educational Resources Information Center

    Lambert, Tania

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

  5. 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…

  6. Early Identification of Hearing Impairment in Infants and Young Children: NIH Consensus Statement [and] Summary of the NIH Consensus.

    ERIC Educational Resources Information Center

    NIH Consensus Statement, 1993

    1993-01-01

    This consensus statement on early identification of hearing impairment in infants and young children was developed by a nonadvocate, non-Federal panel of 58 experts during a 3-day meeting in 1993. The panel concluded that: (1) all infants admitted to the neonatal intensive care unit should be screened for hearing loss prior to discharge; (2)…

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

    ERIC Educational Resources Information Center

    Clark, Thomas C.; Watkins, Susan

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

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

    PubMed Central

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

    2014-01-01

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

  9. Noise induced hearing loss impairs spatial learning/memory and hippocampal neurogenesis in mice

    PubMed Central

    Liu, Lijie; Shen, Pei; He, Tingting; Chang, Ying; Shi, Lijuan; Tao, Shan; Li, Xiaowei; Xun, Qingying; Guo, Xiaojing; Yu, Zhiping; Wang, Jian

    2016-01-01

    Hearing loss has been associated with cognitive decline in the elderly and is considered to be an independent risk factor for dementia. One of the most common causes for acquired sensorineural hearing loss is exposure to excessive noise, which has been found to impair learning ability and cognitive performance in human subjects and animal models. Noise exposure has also been found to depress neurogenesis in the hippocampus. However, the effect is mainly attributed to the oxidant stress of noise on the cognitive brain. In the present study, young adult CBA/CAJ mice (between 1.5 and 2 months of age) were briefly exposed a high sound level to produce moderate-to-severe hearing loss. In both the blood and hippocampus, only transient oxidative stress was observed after noise exposure. However, a deficit in spatial learning/memory was revealed 3 months after noise exposure. Moreover, the deficit was correlated with the degree of hearing loss and was associated with a decrease in neurogenesis in the hippocampus. We believe that the observed effects were likely due to hearing loss rather than the initial oxidant stress, which only lasted for a short period of time. PMID:26842803

  10. Dynamic Range Across Music Genres and the Perception of Dynamic Compression in Hearing-Impaired Listeners

    PubMed Central

    Kirchberger, Martin

    2016-01-01

    Dynamic range compression serves different purposes in the music and hearing-aid industries. In the music industry, it is used to make music louder and more attractive to normal-hearing listeners. In the hearing-aid industry, it is used to map the variable dynamic range of acoustic signals to the reduced dynamic range of hearing-impaired listeners. Hence, hearing-aided listeners will typically receive a dual dose of compression when listening to recorded music. The present study involved an acoustic analysis of dynamic range across a cross section of recorded music as well as a perceptual study comparing the efficacy of different compression schemes. The acoustic analysis revealed that the dynamic range of samples from popular genres, such as rock or rap, was generally smaller than the dynamic range of samples from classical genres, such as opera and orchestra. By comparison, the dynamic range of speech, based on recordings of monologues in quiet, was larger than the dynamic range of all music genres tested. The perceptual study compared the effect of the prescription rule NAL-NL2 with a semicompressive and a linear scheme. Music subjected to linear processing had the highest ratings for dynamics and quality, followed by the semicompressive and the NAL-NL2 setting. These findings advise against NAL-NL2 as a prescription rule for recorded music and recommend linear settings. PMID:26868955

  11. Dynamic Range Across Music Genres and the Perception of Dynamic Compression in Hearing-Impaired Listeners.

    PubMed

    Kirchberger, Martin; Russo, Frank A

    2016-02-10

    Dynamic range compression serves different purposes in the music and hearing-aid industries. In the music industry, it is used to make music louder and more attractive to normal-hearing listeners. In the hearing-aid industry, it is used to map the variable dynamic range of acoustic signals to the reduced dynamic range of hearing-impaired listeners. Hence, hearing-aided listeners will typically receive a dual dose of compression when listening to recorded music. The present study involved an acoustic analysis of dynamic range across a cross section of recorded music as well as a perceptual study comparing the efficacy of different compression schemes. The acoustic analysis revealed that the dynamic range of samples from popular genres, such as rock or rap, was generally smaller than the dynamic range of samples from classical genres, such as opera and orchestra. By comparison, the dynamic range of speech, based on recordings of monologues in quiet, was larger than the dynamic range of all music genres tested. The perceptual study compared the effect of the prescription rule NAL-NL2 with a semicompressive and a linear scheme. Music subjected to linear processing had the highest ratings for dynamics and quality, followed by the semicompressive and the NAL-NL2 setting. These findings advise against NAL-NL2 as a prescription rule for recorded music and recommend linear settings.

  12. Recovery from on- and off-frequency forward masking in listeners with normal and impaired hearing.

    PubMed

    Wojtczak, Magdalena; Oxenham, Andrew J

    2010-07-01

    The aim of this study was to investigate the possible mechanisms underlying an effect reported earlier [Wojtczak, M., and Oxenham, A. J. (2009). J. Acoust. Soc. Am. 125, 270-281] in normal-hearing listeners, whereby recovery from forward masking can be slower for off-frequency tonal maskers than for on-frequency tonal maskers that produce the same amount of masking at a 0-ms masker-signal delay. To rule out potential effects of confusion between the tonal signal and tonal masker, one condition used a noise-band forward masker. To test whether the effect involved temporal build-up, another condition used a short-duration (30-ms) forward masker. To test whether the effect is dependent on normal cochlear function, conditions were tested in five listeners with sensorineural hearing loss. For the 150-ms noise maskers, the data from normal-hearing listeners replicated the findings from the previous study that used tonal maskers. In contrast, no significant difference in recovery from on- and off-frequency masking was observed for the 30-ms tonal maskers in normal-hearing listeners, or for the 150-ms tonal maskers in hearing-impaired listeners. Overall, the results are consistent with a mechanism based on efferent feedback that affects the recovery from forward masking in the normal auditory system. PMID:20649220

  13. Developmental hearing loss impairs signal detection in noise: putative central mechanisms

    PubMed Central

    Gay, Jennifer D.; Voytenko, Sergiy V.; Galazyuk, Alexander V.; Rosen, Merri J.

    2014-01-01

    Listeners with hearing loss have difficulty processing sounds in noisy environments. This is most noticeable for speech perception, but is reflected in a basic auditory processing task: detecting a tonal signal in a noise background, i.e., simultaneous masking. It is unresolved whether the mechanisms underlying simultaneous masking arise from the auditory periphery or from the central auditory system. Poor detection in listeners with sensorineural hearing loss (SNHL) is attributed to cochlear hair cell damage. However, hearing loss alters neural processing in the central auditory system. Additionally, both psychophysical and neurophysiological data from normally hearing and impaired listeners suggest that there are additional contributions to simultaneous masking that arise centrally. With SNHL, it is difficult to separate peripheral from central contributions to signal detection deficits. We have thus excluded peripheral contributions by using an animal model of early conductive hearing loss (CHL) that provides auditory deprivation but does not induce cochlear damage. When tested as adults, animals raised with CHL had increased thresholds for detecting tones in simultaneous noise. Furthermore, intracellular in vivo recordings in control animals revealed a cortical correlate of simultaneous masking: local cortical processing reduced tone-evoked responses in the presence of noise. This raises the possibility that altered cortical responses which occur with early CHL can influence even simple signal detection in noise. PMID:25249949

  14. Signal-processing strategy for restoration of cross-channel suppression in hearing-impaired listeners.

    PubMed

    Rasetshwane, Daniel M; Gorga, Michael P; Neely, Stephen T

    2014-01-01

    Because frequency components interact nonlinearly with each other inside the cochlea, the loudness growth of tones is relatively simple in comparison to the loudness growth of complex sounds. The term suppression refers to a reduction in the response growth of one tone in the presence of a second tone. Suppression is a salient feature of normal cochlear processing and contributes to psychophysical masking. Suppression is evident in many measurements of cochlear function in subjects with normal hearing, including distortion-product otoacoustic emissions (DPOAEs). Suppression is also evident, to a lesser extent, in subjects with mild-to-moderate hearing loss. This paper describes a hearing-aid signal-processing strategy that aims to restore both loudness growth and two-tone suppression in hearing-impaired listeners. The prescription of gain for this strategy is based on measurements of loudness by a method known as categorical loudness scaling. The proposed signal-processing strategy reproduces measured DPOAE suppression tuning curves and generalizes to any number of frequency components. The restoration of both normal suppression and normal loudness has the potential to improve hearing-aid performance and user satisfaction.

  15. Signal-Processing Strategy for Restoration of Cross-Channel Suppression in Hearing-Impaired Listeners

    PubMed Central

    Rasetshwane, Daniel M.; Gorga, Michael P.; Neely, Stephen T.

    2013-01-01

    Because frequency components interact nonlinearly with each other inside the cochlea, the loudness growth of tones is relatively simple in comparison to the loudness growth of complex sounds. The term suppression refers to a reduction in the response growth of one tone in the presence of a second tone. Suppression is a salient feature of normal cochlear processing and contributes to psychophysical masking. Suppression is evident in many measurements of cochlear function in subjects with normal hearing, including distortion-product otoacoustic emissions (DPOAEs). Suppression is also evident, to a lesser extent, in subjects with mild-to-moderate hearing loss. This paper describes a hearing-aid signal-processing strategy that aims to restore both loudness growth and two-tone suppression in hearing-impaired listeners. The prescription of gain for this strategy is based on measurements of loudness by a method known as categorical loudness scaling. The proposed signal-processing strategy reproduces measured DPOAE suppression tuning curves and generalizes to any number of frequency components. The restoration of both normal suppression and normal loudness has the potential to improve hearing-aid performance and user satisfaction. PMID:23925364

  16. Dynamic Range Across Music Genres and the Perception of Dynamic Compression in Hearing-Impaired Listeners.

    PubMed

    Kirchberger, Martin; Russo, Frank A

    2016-01-01

    Dynamic range compression serves different purposes in the music and hearing-aid industries. In the music industry, it is used to make music louder and more attractive to normal-hearing listeners. In the hearing-aid industry, it is used to map the variable dynamic range of acoustic signals to the reduced dynamic range of hearing-impaired listeners. Hence, hearing-aided listeners will typically receive a dual dose of compression when listening to recorded music. The present study involved an acoustic analysis of dynamic range across a cross section of recorded music as well as a perceptual study comparing the efficacy of different compression schemes. The acoustic analysis revealed that the dynamic range of samples from popular genres, such as rock or rap, was generally smaller than the dynamic range of samples from classical genres, such as opera and orchestra. By comparison, the dynamic range of speech, based on recordings of monologues in quiet, was larger than the dynamic range of all music genres tested. The perceptual study compared the effect of the prescription rule NAL-NL2 with a semicompressive and a linear scheme. Music subjected to linear processing had the highest ratings for dynamics and quality, followed by the semicompressive and the NAL-NL2 setting. These findings advise against NAL-NL2 as a prescription rule for recorded music and recommend linear settings. PMID:26868955

  17. A cognitive therapy program for hearing-impaired employees suffering from mental distress

    PubMed Central

    Falkum, Erik; Martinsen, Egil Wilhelm

    2015-01-01

    Objective: To develop a cognitive therapy program to reduce mental distress among hearing-impaired employees. Design: In a pilot study we measured the development of mental distress and avoidant coping among hearing-impaired employees. Levels of mental distress were assessed using the hospital anxiety and depression scale (HAD), and the extent of avoidance with conversation tactics checklist CONV(AVOID). The findings were compared with the development in a treatment as usual (TAU) sample. Study sample: Fifteen participants with an equal distribution of male and female participants (M = 49.2 years) took part. The majority had mild to moderate hearing impairment. Results: The program appeared to be feasible and the adherence was good. The mean depression score was identical at pre- and post-intervention in the intervention group, and increased from 2.9 (SD 2.1) to 3.1 (SD 2.0) in the TAU group. Symptoms of anxiety (p < 0.01, 95 % CI (.82, 3.98)) and avoidant communication (p < 0.05, 95% CI (.5, 4.61)) decreased significantly in the intervention group, while an opposite pattern was observed during the TAU program. Conclusions: The program showed promising results. However, the preliminary results should be further investigated in a randomized controlled trial using a larger sample. PMID:25328031

  18. Adaptation of the Fresenius PD+ Cycler for a hearing-impaired patient.

    PubMed

    Kushner, A

    2000-01-01

    Continuous cycling peritoneal dialysis (CCPD) uses a cycler to perform dialysis exchanges and requires the patient to respond to an audible alarm signifying an interruption in the therapy. Consequently, an unassisted hearing-impaired patient could not use the system. By converting the standard alarm to a vibrating signal, the cycler was successfully adapted to accommodate the special needs of our hearing-impaired patient. The items required for the modification were the Sonic Alert Wake Up Alarm (Model SA-WA300: Sonic Alert, Troy, MI, U.S.A.) and the Sonic Alert Super Shaker Bed Vibrator (Model SA-SS120V: Sonic Alert). The patient can place the vibrator under either the pillow or the mattress. When the cycler alarm is activated, vibration wakens the patient. The equipment was purchased from Harris Communications (Eden Prairie, MN, U.S.A.) through a referral by the Easter Seal Society. Three days were needed to complete training compared to an average of one or two days for patients previously trained for continuous ambulatory peritoneal dialysis (CAPD). The patient remained on cycler therapy for approximately four months when the unrelated development of an abdominal hernia required termination of peritoneal dialysis and subsequent transfer to hemodialysis. In conclusion, a modified cycler can provide a safe and efficient renal replacement therapy option for a hearing-impaired patient.

  19. Getting in and Getting On? The Experiences of Young People with Visual Impairments and Hearing Impairments in Third-Level Education

    ERIC Educational Resources Information Center

    Byrne, Bronagh

    2014-01-01

    Young disabled people continue to be under-represented throughout further and higher education settings. Drawing on Pierre Bourdieu's social theory of habitus, capital and field, this paper explores the practices of domination and oppression that have made it difficult for young people with visual impairments and hearing impairments to…

  20. Investigation of in-vehicle speech intelligibility metrics for normal hearing and hearing impaired listeners

    NASA Astrophysics Data System (ADS)

    Samardzic, Nikolina

    The effectiveness of in-vehicle speech communication can be a good indicator of the perception of the overall vehicle quality and customer satisfaction. Currently available speech intelligibility metrics do not account in their procedures for essential parameters needed for a complete and accurate evaluation of in-vehicle speech intelligibility. These include the directivity and the distance of the talker with respect to the listener, binaural listening, hearing profile of the listener, vocal effort, and multisensory hearing. In the first part of this research the effectiveness of in-vehicle application of these metrics is investigated in a series of studies to reveal their shortcomings, including a wide range of scores resulting from each of the metrics for a given measurement configuration and vehicle operating condition. In addition, the nature of a possible correlation between the scores obtained from each metric is unknown. The metrics and the subjective perception of speech intelligibility using, for example, the same speech material have not been compared in literature. As a result, in the second part of this research, an alternative method for speech intelligibility evaluation is proposed for use in the automotive industry by utilizing a virtual reality driving environment for ultimately setting targets, including the associated statistical variability, for future in-vehicle speech intelligibility evaluation. The Speech Intelligibility Index (SII) was evaluated at the sentence Speech Receptions Threshold (sSRT) for various listening situations and hearing profiles using acoustic perception jury testing and a variety of talker and listener configurations and background noise. In addition, the effect of individual sources and transfer paths of sound in an operating vehicle to the vehicle interior sound, specifically their effect on speech intelligibility was quantified, in the framework of the newly developed speech intelligibility evaluation method. Lastly

  1. Maternal Interactions with a Hearing and Hearing-Impaired Twin: Similarities and Differences in Speech Input, Interaction Quality, and Word Production

    ERIC Educational Resources Information Center

    Lam, Christa; Kitamura, Christine

    2010-01-01

    Purpose: This study examined a mother's speech style and interactive behaviors with her twin sons: 1 with bilateral hearing impairment (HI) and the other with normal hearing (NH). Method: The mother was video-recorded interacting with her twin sons when the boys were 12.5 and 22 months of age. Mean F0, F0 range, duration, and F1/F2 vowel space of…

  2. Speech quality evaluation of a sparse coding shrinkage noise reduction algorithm with normal hearing and hearing impaired listeners.

    PubMed

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

    2015-09-01

    Although there are numerous papers describing single-channel noise reduction strategies to improve speech perception in a noisy environment, few studies have comprehensively evaluated the effects of noise reduction algorithms on speech quality for hearing impaired (HI). A model-based sparse coding shrinkage (SCS) algorithm has been developed, and has shown previously (Sang et al., 2014) that it is as competitive as a state-of-the-art Wiener filter approach in speech intelligibility. Here, the analysis is extended to include subjective quality ratings and a method called Interpolated Paired Comparison Rating (IPCR) is adopted to quantitatively link the benefit of speech intelligibility and speech quality. The subjective quality tests are performed through IPCR to efficiently quantify noise reduction effects on speech quality. Objective measures including frequency-weighted segmental signal-to-noise ratio (fwsegSNR), perceptual evaluation of speech quality (PESQ) and hearing aid speech quality index (HASQI) are adopted to predict the noise reduction effects. Results show little difference in speech quality between the SCS and the Wiener filter algorithm but a difference in quality rating between the HI and NH listeners. HI listeners generally gave better quality ratings of noise reduction algorithms than NH listeners. However, SCS reduced the noise more efficiently at the cost of higher distortions that were detected by NH but not by the HI. SCS is a promising candidate for noise reduction algorithms for HI. In general, care needs to be taken when adopting algorithms that were originally developed for NH participants into hearing aid applications. An algorithm that is evaluated negatively with NH might still bring benefits for HI participants.

  3. Comparing Binaural Pre-processing Strategies III: Speech Intelligibility of Normal-Hearing and Hearing-Impaired Listeners.

    PubMed

    Völker, Christoph; Warzybok, Anna; Ernst, Stephan M A

    2015-01-01

    A comprehensive evaluation of eight signal pre-processing strategies, including directional microphones, coherence filters, single-channel noise reduction, binaural beamformers, and their combinations, was undertaken with normal-hearing (NH) and hearing-impaired (HI) listeners. Speech reception thresholds (SRTs) were measured in three noise scenarios (multitalker babble, cafeteria noise, and single competing talker). Predictions of three common instrumental measures were compared with the general perceptual benefit caused by the algorithms. The individual SRTs measured without pre-processing and individual benefits were objectively estimated using the binaural speech intelligibility model. Ten listeners with NH and 12 HI listeners participated. The participants varied in age and pure-tone threshold levels. Although HI listeners required a better signal-to-noise ratio to obtain 50% intelligibility than listeners with NH, no differences in SRT benefit from the different algorithms were found between the two groups. With the exception of single-channel noise reduction, all algorithms showed an improvement in SRT of between 2.1 dB (in cafeteria noise) and 4.8 dB (in single competing talker condition). Model predictions with binaural speech intelligibility model explained 83% of the measured variance of the individual SRTs in the no pre-processing condition. Regarding the benefit from the algorithms, the instrumental measures were not able to predict the perceptual data in all tested noise conditions. The comparable benefit observed for both groups suggests a possible application of noise reduction schemes for listeners with different hearing status. Although the model can predict the individual SRTs without pre-processing, further development is necessary to predict the benefits obtained from the algorithms at an individual level.

  4. Speech quality evaluation of a sparse coding shrinkage noise reduction algorithm with normal hearing and hearing impaired listeners.

    PubMed

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

    2015-09-01

    Although there are numerous papers describing single-channel noise reduction strategies to improve speech perception in a noisy environment, few studies have comprehensively evaluated the effects of noise reduction algorithms on speech quality for hearing impaired (HI). A model-based sparse coding shrinkage (SCS) algorithm has been developed, and has shown previously (Sang et al., 2014) that it is as competitive as a state-of-the-art Wiener filter approach in speech intelligibility. Here, the analysis is extended to include subjective quality ratings and a method called Interpolated Paired Comparison Rating (IPCR) is adopted to quantitatively link the benefit of speech intelligibility and speech quality. The subjective quality tests are performed through IPCR to efficiently quantify noise reduction effects on speech quality. Objective measures including frequency-weighted segmental signal-to-noise ratio (fwsegSNR), perceptual evaluation of speech quality (PESQ) and hearing aid speech quality index (HASQI) are adopted to predict the noise reduction effects. Results show little difference in speech quality between the SCS and the Wiener filter algorithm but a difference in quality rating between the HI and NH listeners. HI listeners generally gave better quality ratings of noise reduction algorithms than NH listeners. However, SCS reduced the noise more efficiently at the cost of higher distortions that were detected by NH but not by the HI. SCS is a promising candidate for noise reduction algorithms for HI. In general, care needs to be taken when adopting algorithms that were originally developed for NH participants into hearing aid applications. An algorithm that is evaluated negatively with NH might still bring benefits for HI participants. PMID:26232529

  5. The sensitivity of hearing-impaired adults to acoustic attributes in simulated rooms

    PubMed Central

    Whitmer, William M.; McShefferty, David; Akeroyd, Michael A.

    2016-01-01

    In previous studies we have shown that older hearing-impaired individuals are relatively insensitive to changes in the apparent width of broadband noises when those width changes were based on differences in interaural coherence [W. Whitmer, B. Seeber and M. Akeroyd, J. Acoust. Soc. Am. 132, 369-379 (2012)]. This insensitivity has been linked to senescent difficulties in resolving binaural fine-structure differences. It is therefore possible that interaural coherence, despite its widespread use, may not be the best acoustic surrogate of spatial perception for the aged and impaired. To test this, we simulated the room impulse responses for various acoustic scenarios with differing coherence and lateral (energy) fraction attributes using room modelling software (ODEON). Bilaterally impaired adult participants were asked to sketch the perceived size of speech tokens and musical excerpts that were convolved with these impulse responses and presented to them in a sound-dampened enclosure through a 24-loudspeaker array. Participants’ binaural acuity was also measured using an interaural phase discrimination task. Corroborating our previous findings, the results showed less sensitivity to interaural coherence in the auditory source width judgments of older hearing-impaired individuals, indicating that alternate acoustic measurements in the design of spaces for the elderly may be necessary. PMID:27213028

  6. Hearing Impairment in Relation to Severity of Diabetes in a Veteran Cohort

    PubMed Central

    Konrad-Martin, Dawn; Reavis, Kelly M.; Austin, Donald; Reed, Nicholas; Gordon, Jane; McDermott, Dan; Dille, Marilyn F.

    2015-01-01

    Objective Type 2 diabetes is epidemic among veterans, approaching three times the prevalence of the general population. Diabetes leads to devastating complications of vascular and neurologic malfunction and appears to impair auditory function. Hearing loss prevention is a major health-related initiative in the Veterans Health Administration. Thus, this research sought to identify, and quantify with effect sizes, differences in hearing, speech recognition, and hearing-related quality of life (QOL) measures associated with diabetes and to determine whether well-controlled diabetes diminishes the differences. Design The authors examined selected cross-sectional data from the baseline (initial) visit of a longitudinal study of Veterans with and without type 2 diabetes designed to assess the possible differences in age-related trajectories of peripheral and central auditory function between the two groups. In addition, the diabetes group was divided into subgroups on the basis of medical diagnosis of diabetes and current glycated hemoglobin (HbA1c) as a metric of disease severity and control. Outcome measures were pure-tone thresholds, word recognition using sentences presented in noise or time-compressed, and an inventory assessing the self-perceived impact of hearing loss on QOL. Data were analyzed from 130 Veterans ages 24 to 73 (mean 48) years with well-controlled (controlled) diabetes, poorly controlled (uncontrolled) diabetes, prediabetes, and no diabetes. Regression was used to identify any group differences in age, noise exposure history, and other sociodemographic factors, and multiple regression was used to model each outcome variable, adjusting for potential confounders. Results were evaluated in relation to diabetes duration, use of insulin (yes, no), and presence of selected diabetes complications (neuropathy and retinopathy). Results Compared with nondiabetics, Veterans with uncontrolled diabetes had significant differences in hearing at speech frequencies

  7. Effects of noise reduction on AM perception for hearing-impaired listeners.

    PubMed

    Ives, D Timothy; Kalluri, Sridhar; Strelcyk, Olaf; Sheft, Stanley; Miermont, Franck; Coez, Arnaud; Bizaguet, Eric; Lorenzi, Christian

    2014-10-01

    Noise reduction (NR) systems are commonplace in modern digital hearing aids. Though not improving speech intelligibility, NR helps the hearing-aid user in terms of lowering noise annoyance, reducing cognitive load and improving ease of listening. Previous psychophysical work has shown that NR does in fact improve the ability of normal-hearing (NH) listeners to discriminate the slow amplitude-modulation (AM) cues representative of those found in speech. The goal of this study was to assess whether this improvement of AM discrimination with NR can also be observed for hearing-impaired (HI) listeners. AM discrimination was measured at two audio frequencies of 500 Hz and 2 kHz in a background noise with a signal-to-noise ratio of 12 dB. Discrimination was measured for ten HI and ten NH listeners with and without NR processing. The HI listeners had a moderate sensorineural hearing loss of about 50 dB HL at 2 kHz and normal hearing (≤ 20 dB HL) at 500 Hz. The results showed that most of the HI listeners tended to benefit from NR at 500 Hz but not at 2 kHz. However, statistical analyses showed that HI listeners did not benefit significantly from NR at any frequency region. In comparison, the NH listeners showed a significant benefit from NR at both frequencies. For each condition, the fidelity of AM transmission was quantified by a computational model of early auditory processing. The parameters of the model were adjusted separately for the two groups (NH and HI) of listeners. The AM discrimination performance of the HI group (with and without NR) was best captured by a model simulating the loss of the fast-acting amplitude compression applied by the normal cochlea. This suggests that the lack of benefit from NR for HI listeners results from loudness recruitment.

  8. Effects of noise reduction on AM perception for hearing-impaired listeners.

    PubMed

    Ives, D Timothy; Kalluri, Sridhar; Strelcyk, Olaf; Sheft, Stanley; Miermont, Franck; Coez, Arnaud; Bizaguet, Eric; Lorenzi, Christian

    2014-10-01

    Noise reduction (NR) systems are commonplace in modern digital hearing aids. Though not improving speech intelligibility, NR helps the hearing-aid user in terms of lowering noise annoyance, reducing cognitive load and improving ease of listening. Previous psychophysical work has shown that NR does in fact improve the ability of normal-hearing (NH) listeners to discriminate the slow amplitude-modulation (AM) cues representative of those found in speech. The goal of this study was to assess whether this improvement of AM discrimination with NR can also be observed for hearing-impaired (HI) listeners. AM discrimination was measured at two audio frequencies of 500 Hz and 2 kHz in a background noise with a signal-to-noise ratio of 12 dB. Discrimination was measured for ten HI and ten NH listeners with and without NR processing. The HI listeners had a moderate sensorineural hearing loss of about 50 dB HL at 2 kHz and normal hearing (≤ 20 dB HL) at 500 Hz. The results showed that most of the HI listeners tended to benefit from NR at 500 Hz but not at 2 kHz. However, statistical analyses showed that HI listeners did not benefit significantly from NR at any frequency region. In comparison, the NH listeners showed a significant benefit from NR at both frequencies. For each condition, the fidelity of AM transmission was quantified by a computational model of early auditory processing. The parameters of the model were adjusted separately for the two groups (NH and HI) of listeners. The AM discrimination performance of the HI group (with and without NR) was best captured by a model simulating the loss of the fast-acting amplitude compression applied by the normal cochlea. This suggests that the lack of benefit from NR for HI listeners results from loudness recruitment. PMID:24899379

  9. Restoration of loudness summation and differential loudness growth in hearing-impaired listeners.

    PubMed

    Strelcyk, Olaf; Nooraei, Nazanin; Kalluri, Sridhar; Edwards, Brent

    2012-10-01

    When normal-hearing (NH) listeners compare the loudness of narrowband and wideband sounds presented at identical sound pressure levels, the wideband sound will most often be perceived as louder than the narrowband sound, a phenomenon referred to as loudness summation. Hearing-impaired (HI) listeners typically show less-than-normal loudness summation, due to reduced cochlear compressive gain and degraded frequency selectivity. In the present study, loudness summation at 1 and 3 kHz was estimated monaurally for five NH and eight HI listeners by matching the loudness of narrowband and wideband noise stimuli. The loudness summation was measured as a function both of noise bandwidth and level. The HI listeners were tested unaided and aided using three different compression systems to investigate the possibility of restoring loudness summation in these listeners. A compression system employing level-dependent compression channels yielded the most promising outcome. The present results inform the development of future loudness models and advanced compensation strategies for the hearing impaired. PMID:23039449

  10. Association of Age Related Macular Degeneration and Age Related Hearing Impairment

    PubMed Central

    Ghasemi, Hassan; Pourakbari, Malihe Shahidi; Entezari, Morteza; Yarmohammadi, Mohammad Ebrahim

    2016-01-01

    Purpose: To evaluate the association between age-related macular degeneration (ARMD) and sensory neural hearing impairment (SHI). Methods: In this case-control study, hearing status of 46 consecutive patients with ARMD were compared with 46 age-matched cases without clinical ARMD as a control group. In all patients, retinal involvements were confirmed by clinical examination, fluorescein angiography (FA) and optical coherence tomography (OCT). All participants were examined with an otoscope and underwent audiological tests including pure tone audiometry (PTA), speech reception threshold (SRT), speech discrimination score (SDS), tympanometry, reflex tests and auditory brainstem response (ABR). Results: A significant (P = 0.009) association was present between ARMD, especially with exudative and choroidal neovascularization (CNV) components, and age-related hearing impairment primarily involving high frequencies. Patients had higher SRT and lower SDS against anticipated presbycusis than control subjects. Similar results were detected in exudative, CNV and scar patterns supporting an association between late ARMD with SRT and SDS abnormalities. ABR showed significantly prolonged wave I and IV latency times in ARMD (P = 0.034 and 0.022, respectively). Average latency periods for wave I in geographic atrophy (GA) and CNV, and that for wave IV in drusen patterns of ARMD were significantly higher than controls (P = 0.030, 0.007 and 0.050, respectively). Conclusion: The association between ARMD and age-related SHI may be attributed to common anatomical components such as melanin in these two sensory organs. PMID:27195086

  11. Vowel formant discrimination in high-fidelity speech by hearing-impaired listeners

    NASA Astrophysics Data System (ADS)

    Kewley-Port, Diane; Liu, Chang; Burkle, T. Zachary

    2003-10-01

    The ability to discriminate differences in vowel formant frequency under a more ordinary listening condition has been reported recently by Kewley-Port and Zheng (1999) for synthetic speech and Liu and Kewley-Port (2003) for high-fidelity speech. Results for normal-hearing (YNH) listeners showed that a longer phonetic context (sentences versus words) degraded formant discrimination while adding a sentence identification task did not. The present study used the same high-fidelity stimuli but employed listeners with moderate hearing impairment (YHI). Experimental factors manipulated included phonetic context (isolated vowels, words and sentences), level [70-dB SPL (partially audible) and 95-dB SPL (fully audible)], and task (discrimination with and without the sentence identification task). Thresholds for F1 and F2 of the vowels /I, ɛ, æ invv/ were estimated using adaptive tracking for 71% correct discrimination. The anticipated degrading effects of higher formant frequency and longer phonetic context were obtained. Unexpectedly formant thresholds for high-fidelity vowels at the higher (95-dB SPL) were slightly elevated compared to the 70-dB SPL level, the opposite of results for synthetic vowels. No effect of the added sentence task was seen, similar to the YNH listeners. Details of the differences in vowel processing attributable to moderate hearing impairment will be discussed. [Research supported by NIH-NIDCD.

  12. Development of a School Adaptation Program for Elementary School Students with Hearing Impairment

    PubMed Central

    Kim, Shin-Jeong; Kwon, Myung Soon

    2015-01-01

    Background and Objectives Although new technology of assistive listening device leads many hard of hearing children to be mainstreamed in public school programs, many clinicians and teachers still wonder whether the children are able to understand all instruction, access educational materials, and have social skills in the school. The purpose of this study is to develop a school adaptation program (SAP) for the hearing-impaired children who attend public elementary school. Subjects and Methods The theoretical framework of the SAP was a system model including microsystem, mesosystem, and macrosystem merged with Keller's ARCS theory. Results The SAP consisted of 10 sessions based on five categories (i.e., school life, activity in the class, relationship with friends, relationship with teacher, and school environments). For preliminary validity testing, the developed SAP was reviewed by sixteen elementary school teachers, using the evaluation questionnaire. The results of evaluation showed high average 3.60 (±0.52) points out of 4 while proving a reliable and valid school-based program. Conclusions The SAP indicated that it may serve as a practical and substantive program for hearing-impaired children in the public school in order to help them achieve better academic support and social integrations. PMID:26185788

  13. MEHIDA: an intelligent multimedia tutoring system for the hearing-impaired

    NASA Astrophysics Data System (ADS)

    Alonso, Fernando; de Antonio, Angelica; Fuertes, Jose L.; Montes, Cesar

    1995-03-01

    MEHIDA is a multimedia system offering hearing-impaired children an easy and attractive method to communicate with their hearing and deaf peers. It is a TOTAL COMMUNICATION method whose objective is the acquisition of various forms of communication available to the hearing impaired simultaneously: gesture, speech, dactylology, formal signing, lip reading, reading and writing. Didactic activities and games are used to teach the different means of communication. The approach gives the child the chance to practice the different types of communication. A character has been created in the shape of a pear to assist and guide the child. The pupil identifies with the character at all times, as it explains what the child is being asked to do during each activity. The MEHIDA learning process is divided into six stages: basic learning, prereading and prewriting, syllable, word, simple and complex sentence reading and writing. Each phase establishes a hierarchy of didactic objectives which are the expression of the skills and knowledge to be acquired by the child during the learning process (e.g., learning concepts of similarity) broken down into a series of lower level operational objectives (e.g., select figures of the same shape, size and color).

  14. Contribution of consonant versus vowel information to sentence intelligibility by normal and hearing-impaired listeners

    NASA Astrophysics Data System (ADS)

    Burkle, T. Zachary; Kewley-Port, Diane; Humes, Larry; Lee, Jae Hee

    2001-05-01

    The purpose of this study was to examine the contribution of information provided by vowels versus consonants to sentence intelligibility in young normal-hearing (YNH) and elderly hearing-impaired (EHI) listeners. Sentences were presented in three conditions, with either the vowels or the consonants replaced with speech shaped noise, or unaltered. Sentences from male and female talkers in the TIMIT database were selected. EHI subjects listened at 95 dB SPL, and YNH subjects at both 95 and 70 dB SPL. Subjects listened to each sentence twice and were asked to repeat the entire sentence after each presentation. Words were scored correct if identified exactly. Average performance for unaltered sentences was greater than 94%. Vowel-present conditions were always significantly more intelligible than consonant-present conditions, similar to data reported by Cole and colleagues [Proceedings of ICASSP, 1996]. Across groups, performance in the vowel-present conditions exceeded that in the consonant-present conditions by 14% to 40%, although EHI subjects performed more poorly than YNH subjects. In contrast to written English, vowels in spoken language carry more information about sentences than consonants for both normal and hearing-impaired listeners. [Work supported by NIDCD-02229.

  15. The effect of hearing impairment on localization dominance for single-word stimuli.

    PubMed

    Akeroyd, Michael A; Guy, Fiona H

    2011-07-01

    Localization dominance (one of the phenomena of the "precedence effect") was measured in a large number of normal-hearing and hearing-impaired individuals and related to self-reported difficulties in everyday listening. The stimuli (single words) were made-up of a "lead" followed 4 ms later by a equal-level "lag" from a different direction. The stimuli were presented from a circular ring of loudspeakers, either in quiet or in a background of spatially diffuse babble. Listeners were required to identify the loudspeaker from which they heard the sound. Localization dominance was quantified by the weighting factor c [B.G. Shinn-Cunningham et al., J. Acoust. Soc. Am. 93, 2923-2932 (1993)]. The results demonstrated large individual differences: Some listeners showed near-perfect localization dominance (c near 1) but many showed a much reduced effect. Two-thirds (64/93) of the listeners gave a value of c of at least 0.75. There was a significant correlation with hearing loss, such that better hearing listeners showed better localization dominance. One of the items of the self-report questionnaire ("Do you have the impression of sounds being exactly where you would expect them to be?") showed a significant correlation with the experimental results. This suggests that reductions in localization dominance may affect everyday auditory perception. PMID:21786901

  16. Function and expression pattern of nonsyndromic deafness genes

    PubMed Central

    Hilgert, Nele; Smith, Richard J.H.; Van Camp, Guy

    2010-01-01

    Hearing loss is the most common sensory disorder, present in 1 of every 500 newborns. To date, 46 genes have been identified that cause nonsyndromic hearing loss, making it an extremely heterogeneous trait. This review provides a comprehensive overview of the inner ear function and expression pattern of these genes. In general, they are involved in hair bundle morphogenesis, form constituents of the extracellular matrix, play a role in cochlear ion homeostasis or serve as transcription factors. During the past few years, our knowledge of genes involved in hair bundle morphogenesis has increased substantially. We give an up-to-date overview of both the nonsyndromic and Usher syndrome genes involved in this process, highlighting proteins that interact to form macromolecular complexes. For every gene, we also summarize its expression pattern and impact on hearing at the functional level. Gene-specific cochlear expression is summarized in a unique table by structure/cell type and is illustrated on a cochlear cross-section, which is available online via the Hereditary Hearing Loss Homepage. This review should provide auditory scientists the most relevant information for all identified nonsyndromic deafness genes. PMID:19601806

  17. Emotion Recognition/Understanding Ability in Hearing or Vision-Impaired Children: Do Sounds, Sights, or Words Make the Difference?

    ERIC Educational Resources Information Center

    Dyck, Murray J.; Farrugia, Charles; Shochet, Ian M.; Holmes-Brown, Martez

    2004-01-01

    Background: This study was designed to assess whether children with a sensory disability have consistent delays in acquiring emotion recognition and emotion understanding abilities. Method: Younger (6-11 years) and older (12-18 years) hearing-impaired children (HI; n = 49), vision-impaired children (VI; n = 42), and children with no sensory…

  18. Acoustic characteristics of vowels and plosives/affricates of Mandarin-speaking hearing-impaired children.

    PubMed

    Tseng, Shu-Chuan; Kuei, Ko; Tsou, Pei-Chen

    2011-09-01

    This article presents the results of an acoustic analysis of vowels and plosives/affricates produced by 45 Mandarin-speaking children with hearing impairment. Vowel production is represented and categorized into three groups by vowel space size calculated with normalized F1 and F2 values of corner vowels. The correlation between speech intelligibility and language abilities assessed by the level of word comprehension and the complexity of sentence structure is statistically significant. Vowel space grouping is correlated with speech intelligibility and spike percentage of plosives/affricates production. The generalized linear model analysis also shows that the level of word comprehension and the degree of hearing loss are the two most significant factors in predicting speech intelligibility. The statistical results suggest that the interplay of acoustic characteristic and speech ability is complex.

  19. Phonological development in 4-6-year-old moderately hearing impaired children.

    PubMed

    Huttunen, K H

    2001-01-01

    The acquisition of clear phonological rules is of utmost importance for a child when he/she is learning to decode and convey meanings using spoken language. Fifteen Finnish-speaking children, five normally hearing (NH) 3-year-olds and 10 moderately hearing impaired (HId) 4-6-year-olds were studied here. Speech samples consisting of 62 words were collected from each child during a picture naming task. Frequent phonetic (articulation) errors together with normal (but delayed) and deviant phonological processes of HId children were found in this study. The frequency and quality of the processes seemed to be related more to the grade of HI than, for example, the age of the child. Implications for speech therapy are briefly discussed.

  20. Impaired binaural hearing in children produced by a threshold level of middle ear disease.

    PubMed

    Hogan, Sarah C M; Moore, David R

    2003-06-01

    Otitis media with effusion (OME), a form of middle ear disease, is the most common reason for young children both to visit their family doctor and to have surgery. Almost all children have at least a single episode of OME before their first birthday and annual incidence rates exceed 50% in each of the first five years. For most children, OME occurs infrequently, but about 10-15% of children have OME during more than half of their first six years. Middle ear effusions attenuate and delay sound, causing conductive sound distortion during the crucial years for language acquisition. The many studies of OME effects on language and other indices of development have produced mixed results. However, a consensus is emerging of mild language impairment in the preschool years, with subsequent performance, emotional, and behavioral difficulties. In addition to the peripheral hearing loss produced directly by the disease, binaural and other central auditory deficits can outlive the OME. It has been unclear which children are at risk of central impairment following OME, since the children studied have generally been recruited from otolaryngology clinics. Consequently, a detailed prospective history of the middle ear status of participants has not been available. By studying six-year-old children with a lifetime known history of OME, we show in this study that only those children with a cumulative OME experience of more than about half the time during the first five years consistently have residual impaired binaural hearing. PMID:12943367

  1. Impaired binaural hearing in children produced by a threshold level of middle ear disease.

    PubMed

    Hogan, Sarah C M; Moore, David R

    2003-06-01

    Otitis media with effusion (OME), a form of middle ear disease, is the most common reason for young children both to visit their family doctor and to have surgery. Almost all children have at least a single episode of OME before their first birthday and annual incidence rates exceed 50% in each of the first five years. For most children, OME occurs infrequently, but about 10-15% of children have OME during more than half of their first six years. Middle ear effusions attenuate and delay sound, causing conductive sound distortion during the crucial years for language acquisition. The many studies of OME effects on language and other indices of development have produced mixed results. However, a consensus is emerging of mild language impairment in the preschool years, with subsequent performance, emotional, and behavioral difficulties. In addition to the peripheral hearing loss produced directly by the disease, binaural and other central auditory deficits can outlive the OME. It has been unclear which children are at risk of central impairment following OME, since the children studied have generally been recruited from otolaryngology clinics. Consequently, a detailed prospective history of the middle ear status of participants has not been available. By studying six-year-old children with a lifetime known history of OME, we show in this study that only those children with a cumulative OME experience of more than about half the time during the first five years consistently have residual impaired binaural hearing.

  2. The perception of apparent auditory source width in hearing-impaired adults

    PubMed Central

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

    2014-01-01

    In a previous study [W. Whitmer, B. Seeber and M. Akeroyd, J. Acoust. Soc. Am. 132, 369-379 (2012)], it was demonstrated that older hearing-impaired (HI) listeners produced visual sketches of headphone-presented noises that were insensitive to changes in interaural coherence. The current study further explores this insensitivity by comparing (a) binaural temporal fine-stucture (TFS) resolution and (b) sound localization precision to (c) auditory source width judgments. Thirty-five participants aged 26-81 years with normal to moderately impaired hearing (a) discriminated interaurally phase-shifted tones from diotic tones presented over headphones, (b) located 500-ms speech-spectrum filtered click trains presented over loudspeakers between ±30° in quiet, and (c) sketched the perceived width of low-pass, high-pass and speech-spectrum noise stimuli presented over loudspeakers from 0° and simultaneously from ±45° at attenuations of 0-20 dB to generate partially coherent stimuli. The results showed a decreasing sensitivity to width with age and impairment which was related to binaural TFS threshold: the worse one’s threshold – which was correlated with age – the less the perceived width increased with decreasing interaural coherence. These results suggest that senescent changes to the auditory system do not necessarily lead to perceptions of broader, more diffuse sound images based on interaural coherence. PMID:24907818

  3. Association of Hearing Impairment with Declines in Physical Functioning and the Risk of Disability in Older Adults

    PubMed Central

    Chen, David S.; Betz, Joshua; Yaffe, Kristine; Ayonayon, Hilsa N.; Kritchevsky, Stephen; Martin, Kathryn R.; Harris, Tamara B.; Purchase-Helzner, Elizabeth; Satterfield, Suzanne; Xue, Qian-Li; Pratt, Sheila; Simonsick, Eleanor M.

    2015-01-01

    Background: Identifying factors associated with functional declines in older adults is important given the aging of the population. We investigated if hearing impairment is independently associated with objectively measured declines in physical functioning in a community-based sample of older adults. Methods: Prospective observational study of 2,190 individuals from the Health, Aging, and Body Composition study. Participants were followed annually for up to 11 visits. Hearing was measured with pure-tone audiometry. Physical functioning and gait speed were measured with the Short Physical Performance Battery (SPPB). Incident disability and requirement for nursing care were assessed semiannually through self-report. Results: In a mixed-effects model, greater hearing impairment was associated with poorer physical functioning. At both Visit 1 and Visit 11, SPPB scores were lower in individuals with mild (10.14 [95% CI 10.04–10.25], p < .01; 7.35 [95% CI 7.12–7.58], p < .05) and moderate or greater hearing impairment (10.04 [95% CI 9.90–10.19], p < .01; 7.00 [95% CI 6.69–7.32], p < .01) than scores in normal hearing individuals (10.36 [95% CI 10.26–10.46]; 7.71 [95% CI 7.49–7.92]). We observed that women with moderate or greater hearing impairment had a 31% increased risk of incident disability (Hazard ratio [HR] =1.31 [95% CI 1.08–1.60], p < .01) and a 31% increased risk of incident nursing care requirement (HR = 1.31 [95% CI 1.05–1.62], p = .02) compared to women with normal hearing. Conclusions: Hearing impairment is independently associated with poorer objective physical functioning in older adults, and a 31% increased risk for incident disability and need for nursing care in women. PMID:25477427

  4. Smoking, Central Adiposity, and Poor Glycemic Control Increase Risk of Hearing Impairment

    PubMed Central

    Cruickshanks, Karen J.; Nondahl, David M.; Dalton, Dayna S.; Fischer, Mary E.; Klein, Barbara E.K.; Klein, Ronald; Nieto, F. Javier; Schubert, Carla R.; Tweed, Ted S.

    2015-01-01

    Objectives To determine associations between smoking, adiposity, diabetes, and other cardiovascular disease (CVD) risk factors and the 15-yr incidence of hearing impairment (HI). Design The Epidemiology of Hearing Loss Study (EHLS) is a longitudinal population-based cohort study (1993–95 to 2009–2010). Setting Beaver Dam, WI. Participants Participants in the Beaver Dam Eye Study (1988–90; residents of Beaver Dam, WI ages 43–84 years in 1987–88) were eligible for the EHLS. There were 1925 participants with normal hearing at baseline. Measurements 15-year cumulative incidence of HI (pure-tone average (PTA) of hearing thresholds at 0.5, 1, 2 and 4 kHz > 25 decibels Hearing Level (dB HL) in either ear). Cigarette smoking, exercise, and other factors were ascertained by questionnaire. Blood pressure, waist circumference, body mass index and glycosylated hemoglobin were measured. Results Follow-up examinations (≥1) were obtained from 87.2% (n=1678; mean baseline age 61 years). The 15-year cumulative incidence of HI was 56.8%. Adjusting for age and sex, current smoking (Hazard Ratio (HR) =1.31, p=0.048), education (<16 yrs; HR=1.35, p=0.01), waist circumference (HR=1.08 per 10 cm, p=0.017), and poorly controlled diabetes (HR=2.03, p=0.048) were associated with increased risk of HI. Former smokers and people with better controlled diabetes were not at increased risk. Conclusion Smoking, central adiposity and poorly controlled diabetes predicted incident HI. These well-known CVD risk factors, suggest vascular changes may contribute to HI in aging. Interventions targeting reductions in smoking and adiposity, and improved glycemic control in people with diabetes, may help to prevent or delay the onset of HI. PMID:25953199

  5. Noise Exposure and Hearing Impairment among Chinese Restaurant Workers and Entertainment Employees in Hong Kong

    PubMed Central

    Lao, Xiang Qian; Yu, Ignatius Tak Sun; Au, Dennis Kin Kwok; Chiu, Yuk Lan; Wong, Claudie Chiu Yi; Wong, Tze Wai

    2013-01-01

    Background Noise-induced hearing loss (NIHL) is a major concern in the non-manufacturing industries. This study aimed to investigate the occupational noise exposure and the NIHL among Chinese restaurant workers and entertainment employees working in the service industry in Hong Kong. Methods This cross-sectional survey involved a total of 1,670 participants. Among them, 937 were randomly selected from the workers of Chinese restaurants and 733 were selected from workers in three entertainment sectors: radio and television stations; cultural performance halls or auditoria of the Leisure and Cultural Services Department (LCSD); and karaoke bars. Noise exposure levels were measured in the sampled restaurants and entertainment sectors. Each participant received an audiometric screening test. Those who were found to have abnormalities were required to take another diagnostic test in the health center. The “Klockhoff digit” method was used to classify NIHL in the present study. Results The main source of noise inside restaurants was the stoves. The mean hearing thresholds showed a typical dip at 3 to 6 KHz and a substantial proportion (23.7%) of the workers fulfilled the criteria for presumptive NIHL. For entertainment sectors, employees in radio and television stations generally had higher exposure levels than those in the halls or auditoria of the LCSD and karaoke bars. The mean hearing thresholds showed a typical dip at 6 KHz and a substantial proportion of the employees fulfilled the criteria for presumptive NIHL (38.6%, 95%CI: 35.1–42.1%). Being male, older, and having longer service and daily alcohol consumption were associated with noise-induced hearing impairment both in restaurant workers and entertainment employees. Conclusion Excessive noise exposure is common in the Chinese restaurant and entertainment industries and a substantial proportion of restaurant workers and entertainment employees suffer from NIHL. Comprehensive hearing conservation programs

  6. Tone decay for hearing-impaired listeners with and without dead regions in the cochlea

    NASA Astrophysics Data System (ADS)

    Huss, Martina; Moore, Brian C. J.

    2003-12-01

    For people with normal hearing, a sustained tone with a frequency within the standard audiometric range remains audible when presented at a level well above threshold. However, for a pure tone with frequency close to the upper limit of hearing (well above 8 kHz), the loudness may decrease within seconds and the tone may decay to inaudibility, even when presented at a level between 20 and 40 dB SL. Scharf [in Hearing Research and Theory, edited by J. V. Tobias and E. D. Schubert (Academic, New York, 1983), Vol. 2, pp. 1-53] suggested that marked loudness adaptation only occurs when the excitation pattern evoked by a tone is spatially limited. The upper limit of hearing may be comparable to the boundary of a ``dead region,'' which is a region with a complete loss of inner hair cell (IHC) and/or neural function. The present study investigated the perceived decay of pure tones for 9 normal-hearing subjects and 12 subjects with moderate to severe sensorineural hearing loss, using a wide range of frequencies (0.125-12 kHz). A dead region was diagnosed for 8 of the 12 subjects. No consistent association was found between the degree of tone decay and the presence of a dead region. Subjects with dead regions did not experience significantly more tone decay than subjects with comparable absolute thresholds but without a dead region, even when the frequency of the tone fell within or close to the edge of a dead region. For severely hearing-impaired subjects, spatial restriction of the excitation pattern was neither necessary nor sufficient to lead to tone decay. The prevalence of tone decay was not well predicted by the audiometric threshold at the test frequency. It is proposed that tone decay depends on the physiological condition of the place in the cochlea where the tone is detected, which, in a case involving a dead region, is the place adjacent to the dead region. The prevalence of tone decay increased when the audiometric threshold was above 50 dB HL in the frequency

  7. [The algorithm for the medical maintenance of the aircraft personnel suffering from chronic sensorineural impairment of hearing].

    PubMed

    Pankova, V B; Skryabina, L Yu; Barkhatova, O A

    2016-01-01

    The present study was designed to systematize the causes underlying the development of chronic sensorineural impairment of hearing in the aircraft personnel engaged in commercial aviation of the Russian Federation. A detailed clinical and audiological picture of chronic sensorineural loss of hearing in the aircraft personnel is presented with special reference to the criteria accepted in the civil aviationfor the evaluation of professional suitability and occupational selection in terms of hearing conditions. The study has demonstrated the paramount importance of the aviation medical expertise for the flight safety control in civil aviation. We analyzed the results of the audiological examination of the aircraft personnel suffering from chronic sensorineural impairment of hearing and proposed the algorithm for the rehabilitation of such subjects taking into consideration the stage of the chronic process. PMID:27213653

  8. Unraveling of Enigmatic Hearing-Impaired GJB2 Single Heterozygotes by Massive Parallel Sequencing: DFNB1 or Not?

    PubMed Central

    Kim, So Young; Kim, Ah Reum; Kim, Nayoung K. D.; Lee, Chung; Kim, Min Young; Jeon, Eun-Hee; Park, Woong-Yang; Choi, Byung Yoon

    2016-01-01

    Abstract The molecular etiology of nonsyndromic sensorineural hearing loss (SNHL) in subjects with only one detectable autosomal recessive GJB2 mutation is unclear. Here, we report GJB2 single heterozygotes with various final genetic diagnoses and suggest appropriate diagnostic strategies. A total of 160 subjects with SNHL without phenotypic markers were screened for GJB2 mutations. Single-nucleotide variants or structural variations within the DFNB1 locus or in other deafness genes were examined by Sanger sequencing, breakpoint PCR, and targeted exome sequencing (TES) of 129 deafness genes. We identified 27 subjects with two mutations and 10 subjects with only one detectable mutation in GJB2. The detection rate of the single GJB2 mutation among the 160 SNHL subjects in the present study (6.25%) was higher than 2.58% in normal hearing controls in Korean. The DFNB1 was clearly excluded as a molecular etiology in four (40%) subjects: other recessive deafness genes (N = 3) accounted for SNHL and the causative gene for the other non-DFNB1 subject (N = 1) was not identified. The etiology of additional two subjects was potentially explained by digenic etiology (N = 2) of GJB2 with MITF and GJB3, respectively. The contribution of the single GJB2 mutation in the four remaining subjects is unclear. Comprehensive diagnostic testing including TES is prerequisite for understanding GJB2 single heterozygotes. PMID:27057829

  9. Large-scale training to increase speech intelligibility for hearing-impaired listeners in novel noises.

    PubMed

    Chen, Jitong; Wang, Yuxuan; Yoho, Sarah E; Wang, DeLiang; Healy, Eric W

    2016-05-01

    Supervised speech segregation has been recently shown to improve human speech intelligibility in noise, when trained and tested on similar noises. However, a major challenge involves the ability to generalize to entirely novel noises. Such generalization would enable hearing aid and cochlear implant users to improve speech intelligibility in unknown noisy environments. This challenge is addressed in the current study through large-scale training. Specifically, a deep neural network (DNN) was trained on 10 000 noises to estimate the ideal ratio mask, and then employed to separate sentences from completely new noises (cafeteria and babble) at several signal-to-noise ratios (SNRs). Although the DNN was trained at the fixed SNR of - 2 dB, testing using hearing-impaired listeners demonstrated that speech intelligibility increased substantially following speech segregation using the novel noises and unmatched SNR conditions of 0 dB and 5 dB. Sentence intelligibility benefit was also observed for normal-hearing listeners in most noisy conditions. The results indicate that DNN-based supervised speech segregation with large-scale training is a very promising approach for generalization to new acoustic environments. PMID:27250154

  10. High prevalence of vaterite in sagittal otoliths causes hearing impairment in farmed fish

    PubMed Central

    Reimer, T.; Dempster, T.; Warren-Myers, F.; Jensen, A. J.; Swearer, S. E.

    2016-01-01

    The rapid growth of aquaculture raises questions about the welfare status of mass-produced species. Sagittal otoliths are primary hearing structures in the inner ear of all teleost (bony) fishes and are normally composed of aragonite, though abnormal vaterite replacement is sometimes seen in the wild. We provide the first widespread evaluation of the prevalence of vaterite in otoliths, showing that farmed fish have levels of vaterite replacement over 10 times higher than wild fish, regardless of species. We confirm this observation with extensive sampling of wild and farmed Atlantic salmon in Norway, the world’s largest producer, and verify that vateritic otoliths are common in farmed salmon worldwide. Using a mechanistic model of otolith oscillation in response to sound, we demonstrate that average levels of vaterite replacement result in a 28–50% loss of otolith functionality across most of a salmonid’s known hearing range and throughout its life cycle. The underlying cause(s) of vaterite formation remain unknown, but the prevalence of hearing impairment in farmed fish has important implications for animal welfare, the survival of escapees and their effects on wild populations, and the efficacy of restocking programs based on captive-bred fish. PMID:27121086

  11. High prevalence of vaterite in sagittal otoliths causes hearing impairment in farmed fish.

    PubMed

    Reimer, T; Dempster, T; Warren-Myers, F; Jensen, A J; Swearer, S E

    2016-01-01

    The rapid growth of aquaculture raises questions about the welfare status of mass-produced species. Sagittal otoliths are primary hearing structures in the inner ear of all teleost (bony) fishes and are normally composed of aragonite, though abnormal vaterite replacement is sometimes seen in the wild. We provide the first widespread evaluation of the prevalence of vaterite in otoliths, showing that farmed fish have levels of vaterite replacement over 10 times higher than wild fish, regardless of species. We confirm this observation with extensive sampling of wild and farmed Atlantic salmon in Norway, the world's largest producer, and verify that vateritic otoliths are common in farmed salmon worldwide. Using a mechanistic model of otolith oscillation in response to sound, we demonstrate that average levels of vaterite replacement result in a 28-50% loss of otolith functionality across most of a salmonid's known hearing range and throughout its life cycle. The underlying cause(s) of vaterite formation remain unknown, but the prevalence of hearing impairment in farmed fish has important implications for animal welfare, the survival of escapees and their effects on wild populations, and the efficacy of restocking programs based on captive-bred fish. PMID:27121086

  12. High prevalence of vaterite in sagittal otoliths causes hearing impairment in farmed fish.

    PubMed

    Reimer, T; Dempster, T; Warren-Myers, F; Jensen, A J; Swearer, S E

    2016-01-01

    The rapid growth of aquaculture raises questions about the welfare status of mass-produced species. Sagittal otoliths are primary hearing structures in the inner ear of all teleost (bony) fishes and are normally composed of aragonite, though abnormal vaterite replacement is sometimes seen in the wild. We provide the first widespread evaluation of the prevalence of vaterite in otoliths, showing that farmed fish have levels of vaterite replacement over 10 times higher than wild fish, regardless of species. We confirm this observation with extensive sampling of wild and farmed Atlantic salmon in Norway, the world's largest producer, and verify that vateritic otoliths are common in farmed salmon worldwide. Using a mechanistic model of otolith oscillation in response to sound, we demonstrate that average levels of vaterite replacement result in a 28-50% loss of otolith functionality across most of a salmonid's known hearing range and throughout its life cycle. The underlying cause(s) of vaterite formation remain unknown, but the prevalence of hearing impairment in farmed fish has important implications for animal welfare, the survival of escapees and their effects on wild populations, and the efficacy of restocking programs based on captive-bred fish.

  13. Prevalence of permanent childhood hearing impairment in the United Kingdom and implications for universal neonatal hearing screening: questionnaire based ascertainment study

    PubMed Central

    Fortnum, Heather M; Summerfield, A Quentin; Marshall, David H; Davis, Adrian C; Bamford, John M

    2001-01-01

    Objective To estimate the prevalence of confirmed permanent childhood hearing impairment and its profile across age and degree of impairment in the United Kingdom. Design Retrospective total ascertainment through sources in the health and education sectors by postal questionnaire. Setting Hospital based otology and audiology departments, community health clinics, education services for hearing impaired children. Participants Children born from 1980 to 1995, resident in United Kingdom in 1998, with severe permanent childhood hearing impairment (hearing level in the better ear >40 dB averaged over 0.5, 1, 2, and 4 kHz). Main outcome measures Numbers of cases with date of birth and severity of impairment converted to prevalences for each annual birth cohort (cases/1000 live births) and adjusted for underascertainment. Results 26 000 notifications ascertained 17 160 individual children. Prevalence rose from 0.91 (95% confidence interval 0.85 to 0.98) for 3 year olds to 1.65 (1.62 to 1.68) for children aged 9-16 years. Adjustment for underascertainment increased estimates to 1.07 (1.03 to 1.12) and 2.05 (2.02 to 2.08). Comparison with previous studies showed that prevalence increases with age, rather than declining with year of birth. Conclusions Prevalence of confirmed permanent childhood hearing impairment increases until the age of 9 years to a level higher than previously estimated. Relative to current yields of universal neonatal hearing screening in the United Kingdom, which are close to 1/1000 live births, 50-90% more children are diagnosed with permanent childhood hearing impairment by the age of 9 years. Paediatric audiology services must have the capacity to achieve early identification and confirmation of these additional cases. What is already known on this topicThe prevalence of confirmed permanent childhood hearing impairment (>40 dB HL) in the United Kingdom has been estimated to rise with age to 1.33/1000 live births among children aged 5 years and

  14. Empowering the family during the first months after identification of permanent hearing impairment in children.

    PubMed

    Ciciriello, E; Bolzonello, P; Marchi, R; Falzone, C; Muzzi, E; Orzan, E

    2016-02-01

    The latest international guidelines highlight the importance of involving the family in the diagnostic and rehabilitation process of children affected by permanent hearing impairment. This emphasises how meaningful this approach is for the development of the deaf child. So far, there is very little evidence about this approach in Italy, and there are still some barriers to its practical management. The aim of this paper is to report the results of a strategic analysis, which identifies the strengths, weaknesses, opportunities and threats of the family empowerment process during early auditory diagnosis and rehabilitation. The audiology programme should have the goal to offer information and support to families in order to achieve a conscious decision about the use and type of auditory prosthesis and rehabilitation choice within three months after audiologic diagnosis. Within the framework of the Ministry of Health project CCM 2013 "Preventing Communication Disorders: a Regional Program for Early Identification, Intervention and Care of Hearing Impaired Children", a group of professionals identified three main recommendations that can be useful to foster the natural communicative development of the child by strengthening the therapeutic alliance and empowerment of the family. The recommendations obtained with this analysis can help to develop new Italian guidelines with the aim to foster natural communicative development of the child by strengthening the therapeutic alliance and empowerment of the family. PMID:27054393

  15. Cochlear nonlinearity between 500 and 8000 Hz in listeners with impaired hearing

    NASA Astrophysics Data System (ADS)

    Lopez-Poveda, Enrique A.; Plack, Christopher J.; Meddis, Ray; Blanco, Jose L.

    2003-10-01

    Cochlear nonlinearity was estimated in listeners with impaired hearing using a forward-masking method. For a fixed low-level probe, the masker level required to mask the probe was measured as a function of the masker-probe interval, to produce a temporal masking curve (TMC). TMCs were measured for probe frequencies from 500 to 8000 Hz, and for masker frequencies from 0.5 to 1.6 times the probe frequency. Unlike what happens for normal-hearing (NH) listeners [Lopez-Poveda et al., J. Acoust. Soc. Am. 113, 951-960 (2003)], TMCs for on-frequency maskers sometimes show a single slope, suggesting linear responses for tones at CF. Sometimes, however, two distinct slopes are visible, suggesting remaining compression. Both patterns are uncorrelated with the absolute threshold and are consistent with selective damage to outer or inner hair cells (IHC), respectively. Remarkably, the slope of the TMCs for very low-frequency maskers is shallower for the impaired ears than for normal ones. This result implies that for NH listeners, the slope of the TMCs for very low-frequency maskers reflects some kind of compression, even at high CFs. It is discussed that this compression is likely to occur at the IHC rather than at the basilar membrane. [Work supported by FIS PI020343, G03/203, and Oticon Spain.

  16. Sentence Recognition Prediction for Hearing-impaired Listeners in Stationary and Fluctuation Noise With FADE

    PubMed Central

    Schädler, Marc René; Warzybok, Anna; Meyer, Bernd T.; Brand, Thomas

    2016-01-01

    To characterize the individual patient’s hearing impairment as obtained with the matrix sentence recognition test, a simulation Framework for Auditory Discrimination Experiments (FADE) is extended here using the Attenuation and Distortion (A+D) approach by Plomp as a blueprint for setting the individual processing parameters. FADE has been shown to predict the outcome of both speech recognition tests and psychoacoustic experiments based on simulations using an automatic speech recognition system requiring only few assumptions. It builds on the closed-set matrix sentence recognition test which is advantageous for testing individual speech recognition in a way comparable across languages. Individual predictions of speech recognition thresholds in stationary and in fluctuating noise were derived using the audiogram and an estimate of the internal level uncertainty for modeling the individual Plomp curves fitted to the data with the Attenuation (A-) and Distortion (D-) parameters of the Plomp approach. The “typical” audiogram shapes from Bisgaard et al with or without a “typical” level uncertainty and the individual data were used for individual predictions. As a result, the individualization of the level uncertainty was found to be more important than the exact shape of the individual audiogram to accurately model the outcome of the German Matrix test in stationary or fluctuating noise for listeners with hearing impairment. The prediction accuracy of the individualized approach also outperforms the (modified) Speech Intelligibility Index approach which is based on the individual threshold data only. PMID:27604782

  17. Genome-wide association analysis demonstrates the highly polygenic character of age-related hearing impairment

    PubMed Central

    Fransen, Erik; Bonneux, Sarah; Corneveaux, Jason J; Schrauwen, Isabelle; Di Berardino, Federica; White, Cory H; Ohmen, Jeffrey D; Van de Heyning, Paul; Ambrosetti, Umberto; Huentelman, Matthew J; Van Camp, Guy; Friedman, Rick A

    2015-01-01

    We performed a genome-wide association study (GWAS) to identify the genes responsible for age-related hearing impairment (ARHI), the most common form of hearing impairment in the elderly. Analysis of common variants, with and without adjustment for stratification and environmental covariates, rare variants and interactions, as well as gene-set enrichment analysis, showed no variants with genome-wide significance. No evidence for replication of any previously reported genes was found. A study of the genetic architecture indicates for the first time that ARHI is highly polygenic in nature, with probably no major genes involved. The phenotype depends on the aggregated effect of a large number of SNPs, of which the individual effects are undetectable in a modestly powered GWAS. We estimated that 22% of the variance in our data set can be explained by the collective effect of all genotyped SNPs. A score analysis showed a modest enrichment in causative SNPs among the SNPs with a P-value below 0.01. PMID:24939585

  18. Empowering the family during the first months after identification of permanent hearing impairment in children.

    PubMed

    Ciciriello, E; Bolzonello, P; Marchi, R; Falzone, C; Muzzi, E; Orzan, E

    2016-02-01

    The latest international guidelines highlight the importance of involving the family in the diagnostic and rehabilitation process of children affected by permanent hearing impairment. This emphasises how meaningful this approach is for the development of the deaf child. So far, there is very little evidence about this approach in Italy, and there are still some barriers to its practical management. The aim of this paper is to report the results of a strategic analysis, which identifies the strengths, weaknesses, opportunities and threats of the family empowerment process during early auditory diagnosis and rehabilitation. The audiology programme should have the goal to offer information and support to families in order to achieve a conscious decision about the use and type of auditory prosthesis and rehabilitation choice within three months after audiologic diagnosis. Within the framework of the Ministry of Health project CCM 2013 "Preventing Communication Disorders: a Regional Program for Early Identification, Intervention and Care of Hearing Impaired Children", a group of professionals identified three main recommendations that can be useful to foster the natural communicative development of the child by strengthening the therapeutic alliance and empowerment of the family. The recommendations obtained with this analysis can help to develop new Italian guidelines with the aim to foster natural communicative development of the child by strengthening the therapeutic alliance and empowerment of the family.

  19. Effect of enhancement of spectral changes on speech intelligibility and clarity preferences for the hearing impaired.

    PubMed

    Chen, Jing; Baer, Thomas; Moore, Brian C J

    2012-04-01

    Most information in speech is carried in spectral changes over time, rather than in static spectral shape per se. A form of signal processing aimed at enhancing spectral changes over time was developed and evaluated using hearing-impaired listeners. The signal processing was based on the overlap-add method, and the degree and type of enhancement could be manipulated via four parameters. Two experiments were conducted to assess speech intelligibility and clarity preferences. Three sets of parameter values (one corresponding to a control condition), two types of masker (steady speech-spectrum noise and two-talker speech) and two signal-to-masker ratios (SMRs) were used for each masker type. Generally, the effects of the processing were small, although intelligibility was improved by about 8 percentage points relative to the control condition for one set of parameter values using the steady noise masker at -6 dB SMR. The processed signals were not preferred over those for the control condition, except for the steady noise masker at -6 dB SMR. Further work is needed to determine whether tailoring the processing to the characteristics of the individual hearing-impaired listener is beneficial. PMID:22501075

  20. Hearing impairment in Thais due to sport shooting: a preliminary report.

    PubMed

    Charakorn, C; Amatyakul, P

    1998-05-01

    Ninety one sportshooters from several sportshooting clubs ranging in age from 20-49 and 85 control subjects were carefully examined for hearing ability. The male and female ratio was 10.37:1. It was found that 9.34 per cent of the subjects were suffering from hearing impairment at the level of 3,000-8,000 Hertz. At most frequencies the mean decibel between the groups of shooters (1-5 years, 5-10 years, more than 10 years) was significantly different, with the right ear being significantly more impaired than the left ear (alpha = 0.1). At all frequencies the mean decibel level for the control group was significantly different from that for the sportshooters (alpha = 0.1). In conclusion, it is the responsibility of the National Environmental Board on Noise Pollution, the Royal College of Otolaryngologists of Thailand and other related organizations should be much more aggressive in educating sportshooters with regard to protecting their ears against acoustic trauma. PMID:9623034

  1. The ASSR: clinical application in normal-hearing and hearing-impaired infants and adults, comparison with the click-evoked ABR and pure-tone audiometry.

    PubMed

    Scherf, Fanny; Brokx, Jan; Wuyts, Floris L; Van de Heyning, Paul H

    2006-05-01

    The objective of this study was to investigate the clinical application of the ASSR (GSI Audera). It was completed in two parts: Study 1. Correlation between the ASSR-based threshold estimations and the conventional pure-tone thresholds in adults; and Study 2. Correlation between the average of the 2-4 kHz ASSR-based threshold estimations and c-ABR thresholds in children. The ASSRs were recorded in awake adults and sleeping infants with a range of hearing loss at CFs of 0.5 to 4 kHz and MFs between 46 and 95 Hz. The results show that in hearing-impaired adults (thresholds > 40 dBHL) good correlations can be observed between the behavioural thresholds and the ASSR-based threshold estimations. For the normal- to near-normal-hearing adults, a significant correspondence exists between the ASSR-based threshold estimations and FPTA. In children, strong correlations were found between the c-ABR and the 2-4 kHz ASSR-based threshold estimation average. These studies illustrate that the GSI Audera ASSR can accurately predict the behavioural audiogram in hearing-impaired subjects. In subjects with normal hearing the individual ASSR-based threshold estimations scatter too much. Instead the average of the ASSR-based threshold estimations corresponds well with the FPTA. PMID:16717018

  2. Cognitive performance and perceived effort in speech processing tasks: effects of different noise backgrounds in normal-hearing and hearing-impaired subjects.

    PubMed

    Larsby, Birgitta; Hällgren, Mathias; Lyxell, Björn; Arlinger, Stig

    2005-03-01

    Cognitive tests of speech understanding were administered (presented as text, or in auditory or audiovisual modality) and perceived effort was rated. This was done in four background conditions: in silence, and in three types of noise (S/N=+10 dB) varying in temporal structure and meaningfulness. Four groups of 12 subjects each (young/elderly with normal hearing and young/elderly with hearing impairment) participated. The presence of noise had a negative effect on accuracy and speed of performance in the speech processing tasks, and resulted in higher scores of perceived effort, even when the stimuli were presented as text. Differences in performance between noise conditions existed. In the subjective scores, the noise with temporal variations, but without meaningful content, was the most disruptive of the three noise conditions. In the objective scores the hearing-impaired subjects showed poorer results in noise with temporal variations. The elderly subjects were more distracted by noise with temporal variations, and especially by noise with meaningful content. In noise, all subjects, particularly those with impaired hearing, were more dependent upon visual cues than in the quiet condition.

  3. Temporal Masking Contributions of Inherent Envelope Fluctuations for Listeners with Normal and Impaired Hearing

    NASA Astrophysics Data System (ADS)

    Svec, Adam

    Gaussian noise (GN) simultaneous maskers yield higher masked thresholds for pure tones than low-fluctuation noise (LFN) simultaneous maskers for listeners with normal hearing. This increased residual masking is thought to be due to inherent fluctuations in the temporal envelope of Gaussian noise, but these masking effects using forward maskers have been previously unexamined. Because differences in forward masking due to age and hearing loss are known, the first study measured forward-masked detection thresholds for younger and older adults with normal hearing (NH) and older adults with hearing loss (HI) for a 4000 Hz pure-tone probe at a single masker-probe delay in narrowband noises with maximal (GN) or minimal (LFN) inherent envelope fluctuations. As predicted, results suggested that no effect of age was observed. Surprisingly, forward-masked threshold differences between GN and LFN, an estimate of the magnitude of the effect of inherent masker envelope fluctuations, were not significantly different for older HI listeners compared to younger or older NH listeners. Due to the surprising similarities between listeners with normal and impaired hearing, the second study was designed to assess effects of hearing loss on the slopes and magnitudes of recovery from forward maskers that varied in inherent envelope fluctuations for masker-probe delays of 25, 50, and 75 ms. In addition to measuring these effects centered at 4000 Hz, forward-masked thresholds were also measured at 2000 Hz, a region of better hearing for the HI listeners. As hypothesized, regardless of masker fluctuations, slopes of recovery from forward masking were shallower for HI than NH listeners in all conditions. At 4000 Hz, additional residual masking was greater in HI than NH listeners at the longest masker-probe delays; whereas, no differences in additional residual masking between HI and NH listeners were observed for 2000 Hz. These results suggest that the masking effects from inherent envelope

  4. Symptoms of Autism in Males with Fragile X Syndrome: A Comparison to Nonsyndromic ASD Using Current ADI-R Scores.

    PubMed

    McDuffie, Andrea; Thurman, Angela John; Hagerman, Randi J; Abbeduto, Leonard

    2015-07-01

    Symptoms of autism are frequent in males with fragile X syndrome (FXS), but it is not clear whether symptom profiles differ from those of nonsyndromic ASD. Using individual item scores from the Autism Diagnostic Inventory-Revised, we examined which current symptoms of autism differed in boys with FXS relative to same-aged boys diagnosed with nonsyndromic ASD. In addition, different subsamples of participants were matched on autism diagnostic status and severity of autism symptoms. Between-group comparisons revealed that boys with FXS showed significantly less impairment in Social Smiling than did age-, diagnostic-, and severity-matched boys with nonsyndromic ASD. Severity-matched boys with FXS showed more impairment in complex mannerisms than did boys with nonsyndromic ASD. Behavioral differences between FXS and nonsyndromic ASD may be of theoretical importance in understanding the causes and correlates of ASD in FXS and in developing and implementing appropriate treatments.

  5. Consensus Development Conference on Early Identification of Hearing Impairment in Infants and Young Children (Bethesda, Maryland, March 1-3, 1993).

    ERIC Educational Resources Information Center

    National Inst. of Child Health and Human Development (NIH), Bethesda, MD.

    This document compiles abstracts of papers that were presented at a 3-day conference of experts which developed a consensus statement on early identification of hearing impairment in infants and young children. Papers addressed taxonomy; epidemiology; developmental consequences of early hearing impairment; methodology, instrumentation, and…

  6. Lexical Influences on Spoken Spondaic Word Recognition in Hearing-Impaired Patients

    PubMed Central

    Moulin, Annie; Richard, Céline

    2015-01-01

    Top-down contextual influences play a major part in speech understanding, especially in hearing-impaired patients with deteriorated auditory input. Those influences are most obvious in difficult listening situations, such as listening to sentences in noise but can also be observed at the word level under more favorable conditions, as in one of the most commonly used tasks in audiology, i.e., repeating isolated words in silence. This study aimed to explore the role of top-down contextual influences and their dependence on lexical factors and patient-specific factors using standard clinical linguistic material. Spondaic word perception was tested in 160 hearing-impaired patients aged 23–88 years with a four-frequency average pure-tone threshold ranging from 21 to 88 dB HL. Sixty spondaic words were randomly presented at a level adjusted to correspond to a speech perception score ranging between 40 and 70% of the performance intensity function obtained using monosyllabic words. Phoneme and whole-word recognition scores were used to calculate two context-influence indices (the j factor and the ratio of word scores to phonemic scores) and were correlated with linguistic factors, such as the phonological neighborhood density and several indices of word occurrence frequencies. Contextual influence was greater for spondaic words than in similar studies using monosyllabic words, with an overall j factor of 2.07 (SD = 0.5). For both indices, context use decreased with increasing hearing loss once the average hearing loss exceeded 55 dB HL. In right-handed patients, significantly greater context influence was observed for words presented in the right ears than for words presented in the left, especially in patients with many years of education. The correlations between raw word scores (and context influence indices) and word occurrence frequencies showed a significant age-dependent effect, with a stronger correlation between perception scores and word occurrence frequencies

  7. Aspects of a Communication Curriculum for Hearing-Impaired Pupils: Report of the Second National Workshop on Language Curriculum Development for Hearing-Impaired Pupils. Occasional Paper Number Four.

    ERIC Educational Resources Information Center

    Power, D. J., Ed.; Hollingshead, Anne, Ed.

    The document presents findings and discussion from a 1981 Australian workshop on language curriculum development for hearing impaired students. The first 11 papers, unattributed, address the following issues regarding development of a language curriculum: content, principles, levels of language, techniques and materials, features of conversational…

  8. [The nursing process in helping a family with foreign mother and hearing impaired child].

    PubMed

    Wu, Meei-Lian; Tang, Jing-Shia

    2004-12-01

    This case report aims to present a nursing experience involving a child with severe hearing impairment and delayed language development. The patient was discovered during a home visit. At the time she was two and a half years old, but still had not developed any language behavior. She only used eye contact, physical touch, and body language to communicate with her family. She also did not respond to sound stimulation. The results of a Denver Developmental Screening Test (DDST) showed delayed development, especially of language. The child's mother is from Vietnam. The culture, education, language, and environment of Vietnam are totally different from Taiwan. In addition, the mother did not know how to raise her child. So the author tried to follow up on the case. Data were collected by home visits, phone calls, interviews, and communication with members of a professional health care team during the nursing care period (about six months). Data were recorded and it was written a processing analyzed. They revealed five health problems, as follows: (1) hearing impairment causing delayed language development; (2) poor family recognition deviation understanding of delayed development; (3) insufficient community resources; (4) low self-protection, limited capacity for caused by hearing impairment; (5) foreign mother's sense of helplessness about raising the child. The author provided supportive care to the patient and her family, counseled them, and transferred the child quickly to a treatment center. She also coordinated resources and the professional care team in assisting the parents in facing and adapting to the child's developmental delay. As a result, the parents gained knowledge and the ability to make judgments about developmental delay. This fostered a positive attitude on their part and acceptance of the child's admission to the treatment center. The child and family could deal with their problems appropriately because the nurse intervened at the appropriate time

  9. Glomerular Filtration Rate and Urine Albumin to Creatinine Ratio Associated With Hearing Impairment Among Korean Adults With Diabetes: A Nationwide Population-Based Study.

    PubMed

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

    2016-04-01

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

  10. Qualitative analysis of dermatoglyphics of the digito-palmar complex in children with severe recessive perceptively impaired hearing.

    PubMed

    Sikić, Nada; Milicić, Jasna; Vrca, Andelko; Dulcić, Adinda; Runjić, Nada

    2009-06-01

    The possible hereditary indicated differences in the ascending family tree was investigated in children with severe recessive perceptively impaired hearing, their parents, and healthy controls, using qualitative analysis of dermatoglyphics of the digito-palmar complex. The chi2 test was performed and biological distance was investigated by means according to Hiernaux Ag methods, and presented using Ward's method for the examined groups. The results show that the healthy control group differs from the groups of boys and girls with impaired hearing and also from their fathers mostly in palmar variables. The mothers were biologically more distant from the examined groups of patients, and more similar to the control group of randomly selected healthy female controls. The results indicate polygenic inheritance of sporadic sensoneurial hearing loss.

  11. The Benefit of Oral English-Only as Compared with Signed Input to Hearing-Impaired Students.

    ERIC Educational Resources Information Center

    Luetke-Stahlman, Barbara

    1988-01-01

    The study compared scores on a literacy battery of hearing-impaired subjects exposed to either an instructional communication system that attempts to completely encode a language (e.g. oral English, Signing Exact English) or to signed systems that incompletely encode spoken English. Students using the completely encoded language tended to perform…

  12. Directory of Services for the Multiply Handicapped Deaf and/or Hearing Impaired. Resources for the Rubella Deaf Child.

    ERIC Educational Resources Information Center

    Gallaudet Coll., Washington, DC.

    The directory contains information on centers, facilities, and schools which provide some services or programs suitable to the needs of the deaf or hearing impaired who have additonal handicaps (adults as well as children). A brief description of the facility, the clients served, and the services offered accompanies the listing of each facility's…

  13. The Effectiveness and Usability of the Educational Software on Concept Education for Young Children with Impaired Hearing

    ERIC Educational Resources Information Center

    Goker, Hanife; Ozaydin, Latife; Tekedere, Hakan

    2016-01-01

    Early intervention and early education have a special place in educating the children with Impaired Hearing (IH). The advancements in information and communication technologies have led to adopting the view that such technologies could be applied in the educational process of the children with IH. Besides, the positive results acquired in the…

  14. Validating the Thinking Styles Inventory-Revised II among Chinese University Students with Hearing Impairment through Test Accommodations

    ERIC Educational Resources Information Center

    Cheng, Sanyin; Zhang, Li-Fang

    2014-01-01

    The present study pioneered in adopting test accommodations to validate the Thinking Styles Inventory-Revised II (TSI-R2; Sternberg, Wagner, & Zhang, 2007) among Chinese university students with hearing impairment. A series of three studies were conducted that drew their samples from the same two universities, in which accommodating test…

  15. Enhancing Literacy Skills of Students with Congenital and Profound Hearing Impairment in Nigeria Using Babudoh's Comprehension Therapy

    ERIC Educational Resources Information Center

    Babudoh, Gladys B.

    2014-01-01

    This study reports the effect of a treatment tool called "Babudoh's comprehension therapy" in enhancing the comprehension and writing skills of 10 junior secondary school students with congenital and profound hearing impairment in Plateau State, Nigeria. The study adopted the single group pretest-posttest quasi-experimental research…

  16. A Comparison Study of Gross Motor Development Skills of Normal, Hearing-Impaired and Down Syndrome Children.

    ERIC Educational Resources Information Center

    Bilir, Sule; And Others

    This study, conducted in Ankara, Turkey, compared motor development in 48 normal children (ages 3 to 6), 12 children (ages 5 to 7) with Down syndrome, and 33 children (ages 3 to 7) with hearing impairments. The Motor Development Section of the Portage Early Childhood Educational Program checklist was administered to all the children. Results…

  17. Efficacy of Information and Communication Technology in Enhancing Learning Outcomes of Students with Hearing Impairment in Ibadan

    ERIC Educational Resources Information Center

    Egaga, Patrick I.; Aderibigbe, S. Akinwumi

    2015-01-01

    The study aimed at examining the efficacy of Information and Communication Technology (ICT) in enhancing learning outcomes of students with hearing impairment in Ibadan. The study adopted a pretest, post-test, control group quasi-experimental research design. Purposive sampling techniques was used for the selection of thirty participants…

  18. A Comparison of the Leiter-International Performance Scale to WPPSI Performance with Preschool Deaf and Hearing Impaired Children.

    ERIC Educational Resources Information Center

    Boyd, John; Shapiro, Alvin H.

    1986-01-01

    The study compared the Leiter International Performance Scale (LIPS) and the Wechsler Preschool and Primary Scale of Intelligence measures with 18 hearing impaired preschool children. Results suggested that the LIPS, with some reservations, is a valid instrument for use with deaf preschoolers. (DB)

  19. The Use of Microcomputer Technology in Assessing and Training Communication Skills of Young Hearing-Impaired Children.

    ERIC Educational Resources Information Center

    Fulton, Robert T.; And Others

    1983-01-01

    An interactive computer controlled audiovisual system has been used to investigate the acquisition of simple reading tasks and the assessment of bimodal (auditory-speech reading) interactions/interferences with severely hearing impaired preschoolers. It is noted that the system's capabilities exceed the parameters of current investigations.…

  20. Examining the Training Process of a New Teacher Educator in the Field of the Education of the Hearing Impaired

    ERIC Educational Resources Information Center

    Gurgur, Hasan

    2012-01-01

    Training of beginning teacher educators has become a popular topic in the literature. This study aimed to investigate the training procedures of a new teacher educator who would be working in the division of the hearing impaired. The study was designed as action research and a new teacher educator, an experienced teacher educator and an academic…