Mutational spectrum of the WFS1 gene in Wolfram syndrome, nonsyndromic hearing impairment, diabetes mellitus, and psychiatric disease.

NARCIS (Netherlands)

Cryns, K.; Sivakumaran, T.A.; Ouweland, J.M.W. van den; Pennings, R.J.E.; Cremers, C.W.R.J.; Flothmann, K.; Young, T.L.; Smith, R.J.H.; Lesperance, M.M.; Camp, G. van

2003-01-01

WFS1 is a novel gene and encodes an 890 amino-acid glycoprotein (wolframin), predominantly localized in the endoplasmic reticulum. Mutations in WFS1 underlie autosomal recessive Wolfram syndrome and autosomal dominant low frequency sensorineural hearing impairment (LFSNHI) DFNA6/14. In addition,

Clinical evaluation and mitochondrial DNA sequence analysis in two Chinese families with aminoglycoside-induced and non-syndromic hearing loss

International Nuclear Information System (INIS)

Zhao Lidong; Wang Qiuju; Qian Yaping; Li Ronghua; Cao Juayng; Hart, Laura Christine; Zhai Suoqiang; Han Dongyi; Young Wieyen; Guan Minxin

2005-01-01

We report here the clinical, genetic, and molecular characterization of two Chinese pedigrees with aminoglycoside-induced and non-syndromic hearing impairment. Clinical evaluation revealed the variable phenotype of hearing impairment including audiometric configuration in these subjects. Penetrances of hearing loss in BJ105 and BJ106 pedigrees are 67% and 33%, respectively. In particular, three of 10 affected matrilineal relatives of BJ105 pedigree had aminoglycoside-induced hearing loss, while seven affected matrilineal relatives in BJ105 pedigree and six affected matrilineal relatives in BJ106 pedigree did not have a history of exposure to aminoglycosides. Sequence analysis of the complete mitochondrial genomes in these pedigrees showed the identical homoplasmic A1555G mutation and distinct sets of mtDNA variants belonging to haplogroups F3 and M7b. These variants showed no evolutionary conservation, implying that mitochondrial haplotype may not play a significant role in the phenotypic expression of the A1555G mutation in these Chinese pedigrees. However, aminoglycosides and nuclear backgrounds appear to be major modifier factors for the phenotypic manifestation of the A1555G mutation in these Chinese families

Audiometric Characteristics of a Dutch DFNA10 Family With Mid-Frequency Hearing Impairment

NARCIS (Netherlands)

Beelen, E. van; Oonk, A.M.M.; Leijendeckers, J.M.; Hoefsloot, E.H.; Pennings, R.J.E.; Feenstra, I.; Dieker, H.J.; Huygen, P.L.M.; Snik, A.F.M.; Kremer, H.; Kunst, H.P.M.

2016-01-01

OBJECTIVES: Mutations in EYA4 can cause nonsyndromic autosomal dominant sensorineural hearing impairment (DFNA10) or a syndromic variant with hearing impairment and dilated cardiomyopathy. A mutation in EYA4 was found in a Dutch family, causing DFNA10. This study is focused on characterizing the

Hearing loss among patients with Turner's syndrome: literature review

Directory of Open Access Journals (Sweden)

Cresio Alves

2014-06-01

Full Text Available INTRODUCTION: Turner's syndrome (TS is caused by a partial or total deletion of an X chromosome, occurring in 1:2,000 to 1:5,000 live born females. Hearing loss is one of its major clinical manifestations. However, there are few studies investigating this problem. OBJECTIVES: To review the current knowledge regarding the epidemiology, etiology, clinical manifestations and diagnosis of hearing impairment in patients with TS. METHODS: A bibliographic search was performed in the Medline and Lilacs databanks (1980-2012 to identify the main papers associating Turner's syndrome, hearing impairment and its clinical outcomes. CONCLUSIONS: Recurrent otitis media, dysfunction of the Eustachian tube, conductive hearing loss during infancy and sensorineural hearing loss in adolescence are the audiologic disorders more common in ST. The karyotype appears to be important in the hearing loss, with studies demonstrating an increased prevalence in patients with monosomy 45,X or isochromosome 46,i(Xq. Morphologic studies of the cochlea are necessary to help out in the clarifying the etiology of the sensorineural hearing loss.

A combination of two truncating mutations in USH2A causes more severe and progressive hearing impairment in Usher syndrome type IIa

NARCIS (Netherlands)

Hartel, B.P.; Lofgren, M.; Huygen, P.L.; Guchelaar, I.; Lo, A.N.K.N.; Sadeghi, A.M.; van Wijk, E.; Tranebjaerg, L.; Kremer, H.; Kimberling, W.J.; Cremers, C.W.R.J.; Moller, C.; Pennings, R.J.

2016-01-01

OBJECTIVES: Usher syndrome is an inherited disorder that is characterized by hearing impairment (HI), retinitis pigmentosa, and in some cases vestibular dysfunction. Usher syndrome type IIa is caused by mutations in USH2A. HI in these patients is highly heterogeneous and the present study evaluates

Aminoglycoside-induced and non-syndromic hearing loss is associated with the G7444A mutation in the mitochondrial COI/tRNASer(UCN) genes in two Chinese families

International Nuclear Information System (INIS)

Zhu Yi; Qian Yaping; Tang Xiaowen; Wang Jindan; Yang Li; Liao Zhisu; Li Ronghua; Ji Jinzhang; Li Zhiyuan; Chen Jianfu; Choo, Daniel I.; Lu Jianxin; Guan Minxin

2006-01-01

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 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

Assessment of Hearing Impaired Youth.

Science.gov (United States)

Hicks, Doin E., Ed.; And Others

1980-01-01

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

PSYCHOSOCIAL INFLUENCE OF HEARING IMPAIRMENT ON THE INTERPERSONAL BEHAVIOR OF YOUTHS WITH HEARING IMPAIRMENT IN OYO STATE, NIGERIA

Directory of Open Access Journals (Sweden)

Osisanya AYO

2013-03-01

Full Text Available Individuals with hearing impairment are confronted with a lot of problems due to the condition of their disability. This has a negative impact on their social and psychological well-being with multiplying effect on their interpersonal relationship. Therefore, this study investigated the psycho-social influence of hearing impairment on interpersonal behavior of youths with hearing loss.MethodologyThe study adopted a survey research design. A sample consisting of 211 participants with hearing loss were purposively selected from the Federal College of Education (Special Oyo, Nigeria. A questionnaire, part of Psycho-social Competence Scale (PCS, was used for data collection with reliability coefficient of 0.72.ResultsThe findings revealed that hearing impairement affects social interaction of youths with hearing impairment, hearing loss affects emotional well-being of youths with hearing impairment and youths with hearing impairment feel inferior in company of persons without hearing impairment. Based on this, it was recommended that a friendly home environment should be made and youths with hearing impairment should be advised to accept their loss and take it as a challenge that can be used to achieve a better end and the society should have right attitude and beliefs toward youths with hearing impairment.

Auditory hallucinations in adults with hearing impairment: a large prevalence study.

Science.gov (United States)

Linszen, M M J; van Zanten, G A; Teunisse, R J; Brouwer, R M; Scheltens, P; Sommer, I E

2018-03-20

Similar to visual hallucinations in visually impaired patients, auditory hallucinations are often suggested to occur in adults with hearing impairment. However, research on this association is limited. This observational, cross-sectional study tested whether auditory hallucinations are associated with hearing impairment, by assessing their prevalence in an adult population with various degrees of objectified hearing impairment. Hallucination presence was determined in 1007 subjects aged 18-92, who were referred for audiometric testing to the Department of ENT-Audiology, University Medical Center Utrecht, the Netherlands. The presence and severity of hearing impairment were calculated using mean air conduction thresholds from the most recent pure tone audiometry. Out of 829 participants with hearing impairment, 16.2% (n = 134) had experienced auditory hallucinations in the past 4 weeks; significantly more than the non-impaired group [5.8%; n = 10/173; p impairment, with rates up to 24% in the most profoundly impaired group (p impairment in the best ear. Auditory hallucinations mostly consisted of voices (51%), music (36%), and doorbells or telephones (24%). Our findings reveal that auditory hallucinations are common among patients with hearing impairment, and increase with impairment severity. Although more research on potential confounding factors is necessary, clinicians should be aware of this phenomenon, by inquiring after hallucinations in hearing-impaired patients and, conversely, assessing hearing impairment in patients with auditory hallucinations, since it may be a treatable factor.

The combination of vestibular impairment and congenital sensorineural hearing loss predisposes patients to ocular anomalies, including Usher syndrome.

Science.gov (United States)

Kletke, S; Batmanabane, V; Dai, T; Vincent, A; Li, S; Gordon, K A; Papsin, B C; Cushing, S L; Héon, E

2017-07-01

The co-occurrence of hearing impairment and visual dysfunction is devastating. Most deaf-blind etiologies are genetically determined, the commonest being Usher syndrome (USH). While studies of the congenitally deaf population reveal a variable degree of visual problems, there are no effective ophthalmic screening guidelines. We hypothesized that children with congenital sensorineural hearing loss (SNHL) and vestibular impairment were at an increased risk of having USH. A retrospective chart review of 33 cochlear implants recipients for severe to profound SNHL and measured vestibular dysfunction was performed to determine the ocular phenotype. All the cases had undergone ocular examination and electroretinogram (ERG). Patients with an abnormal ERG underwent genetic testing for USH. We found an underlying ocular abnormality in 81.81% (27/33) of cases; of which 75% had refractive errors, and 50% of those patients showed visual improvement with refractive correction. A total of 14 cases (42.42%; 14/33) had generalized rod-cone dysfunction on ERG suggestive of Usher syndrome type 1, confirmed by mutational analysis. This work shows that adding vestibular impairment as a criterion for requesting an eye exam and adding the ERG to detect USH increases the chances of detecting ocular anomalies, when compared with previous literature focusing only on congenital SNHL. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Does hearing aid use affect audiovisual integration in mild hearing impairment?

Science.gov (United States)

Gieseler, Anja; Tahden, Maike A S; Thiel, Christiane M; Colonius, Hans

2018-04-01

There is converging evidence for altered audiovisual integration abilities in hearing-impaired individuals and those with profound hearing loss who are provided with cochlear implants, compared to normal-hearing adults. Still, little is known on the effects of hearing aid use on audiovisual integration in mild hearing loss, although this constitutes one of the most prevalent conditions in the elderly and, yet, often remains untreated in its early stages. This study investigated differences in the strength of audiovisual integration between elderly hearing aid users and those with the same degree of mild hearing loss who were not using hearing aids, the non-users, by measuring their susceptibility to the sound-induced flash illusion. We also explored the corresponding window of integration by varying the stimulus onset asynchronies. To examine general group differences that are not attributable to specific hearing aid settings but rather reflect overall changes associated with habitual hearing aid use, the group of hearing aid users was tested unaided while individually controlling for audibility. We found greater audiovisual integration together with a wider window of integration in hearing aid users compared to their age-matched untreated peers. Signal detection analyses indicate that a change in perceptual sensitivity as well as in bias may underlie the observed effects. Our results and comparisons with other studies in normal-hearing older adults suggest that both mild hearing impairment and hearing aid use seem to affect audiovisual integration, possibly in the sense that hearing aid use may reverse the effects of hearing loss on audiovisual integration. We suggest that these findings may be particularly important for auditory rehabilitation and call for a longitudinal study.

Early Hearing Loss and Language Abilities in Children with Down Syndrome

Science.gov (United States)

Laws, Glynis; Hall, Amanda

2014-01-01

Background: Although many children with Down syndrome experience hearing loss, there has been little research to investigate its impact on speech and language development. Studies that have investigated the association give inconsistent results. These have often been based on samples where children with the most severe hearing impairments have…

Comparison of Reading Literacy in Hearing Impaired and Normal Hearing Students

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Dr. Ali Asghar Kakojoibari

2011-06-01

Full Text Available Background and Aim: listening, speaking, reading and writing are considered the lingual skills. These skills are in direct relation with each other. Listening is the first skill learnt by the individual through development. If damaged by hearing impairment, listening can cause serious defect to lingual skills. The goal of our research was to study the effect of hearing loss on reading literacy in hearing impairment students in comparison with normal hearing students.Methods: Study was performed using the examination booklets of Progress in International Reading Literacy Study (PIRLS 2001. 119 hearing impairment students of 4th grade primary school, last year guidance school, and last year high school levels in schools providing exceptional student education were included. These individuals were compared to 46 normal hearing students of 4th grade primary school of ordinary schools. Comparative statistical analysis was performed using t-test.Results: Reading literacy and literal contents understanding was shown to have a significant difference between normal hearing and whole hearing impaired student (p<0.05, except the ones in high school level with moderate hearing loss. There was also seen a significant difference between normal hearing and hearing impairment students in understanding of information contents (p=0.03.Conclusion: Hearing loss has a negative effect on reading literacy. Consequently, curriculum change and evolution of educational programs in exceptional centers is needed, in order to promote reading literacy and to enhance rest hearing

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

DEFF Research Database (Denmark)

Santurette, Sébastien; Dau, Torsten

2007-01-01

The effects of hearing impairment on the perception of binaural-pitch stimuli were investigated. Several experiments were performed with normal-hearing and hearing-impaired listeners, including detection and discrimination of binaural pitch, and melody recognition using different types of binaural...... pitches. For the normal-hearing listeners, all types of binaural pitches could be perceived immediately and were musical. The hearing-impaired listeners could be divided into three groups based on their results: (a) some perceived all types of binaural pitches, but with decreased salience or musicality...... compared to normal-hearing listeners; (b) some could only perceive the strongest pitch types; (c) some were unable to perceive any binaural pitch at all. The performance of the listeners was not correlated with audibility. Additional experiments investigated the correlation between performance in binaural...

Hearing, speech, language, and vestibular disorders in the fetal alcohol syndrome: a literature review.

Science.gov (United States)

Church, M W; Kaltenbach, J A

1997-05-01

Fetal alcohol syndrome (FAS) is characterized in part by mental impairment, as well as craniofacial and ocular anomalies. These conditions are traditionally associated with childhood hearing disorders, because they all have a common embryonic origin in malformations of the first and second branchial arches, and have similar critical periods of vulnerability to toxic insult. A review of human and animal research indicates that there are four types of hearing disorders associated with FAS. These are: (1) a developmental delay in auditory maturation, (2) sensorineural hearing loss, (3) intermittent conductive hearing loss due to recurrent serous otitis media, and (4) central hearing loss. The auditory and vestibular systems share the same peripheral apparatuses (the inner ear and eighth cranial nerve) and are embryologically and structurally similar. Consequently, vestibular disorders in FAS children might be expected. The evidence for vestibular dysfunction in FAS is ambiguous, however. Like other syndromes associated with craniofacial anomalies, hearing disorders, and mental impairment, FAS is also characterized by a high prevalence of speech and language pathology. Hearing disorders are a form of sensory deprivation. If present during early childhood, they can result in permanent hearing, language, and mental impairment. Early identification and intervention to treat hearing, language, and speech disorders could therefore result in improved outcome for the FAS child. Specific recommendations are made for intervention and future research.

Is Hearing Impairment Associated with Rheumatoid Arthritis?

DEFF Research Database (Denmark)

Emamifar, Amir; Bjoerndal, Kristine; Jensen Hansen, Inger Marie

2016-01-01

BACKGROUND: Rheumatoid arthritis (RA) is a systemic, inflammatory disease that affects 1% of the population. The auditory system may be involved during the course of disease; however the association of RA and hearing impairment has not been clearly defined. OBJECTIVE: The objective of this review...... is to evaluate published clinical reports related to hearing impairment in patients with RA. Furthermore, we discuss possible pathologies and associated factors as well as new treatment modalities. METHOD: A thorough literature search was performed using available databases including Pubmed, Embase, Cochrane...... and ComDisDome to cover all relative reports. The following keywords were used: hearing loss, hearing difficulties, hearing disorders, hearing impairment, sensorineural hearing loss, conductive hearing loss, mixed hearing loss, autoimmune hearing loss, drug ototoxicity, drug-induced hearing loss, hearing...

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

Science.gov (United States)

Kang, Seok Hui; Jung, Da Jung; Lee, Kyu Yup; Choi, Eun Woo; Do, Jun Young

2015-01-01

The objective of the present study was to examine the associations between various anthropometric measures and metabolic syndrome and hearing impairment in Asian women. 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. 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. Our results demonstrated that WHR may be a surrogate marker for predicting the risk of hearing loss resulting from metabolic syndrome.

Localization of A Novel Autosomal Recessive Non-Syndromic Hearing Impairment Locus (DFNB38) to 6q26–q27 in a Consanguineous Kindred from Pakistan

OpenAIRE

Ansar, Muhammad; Ramzan, Mohammad; Pham, Thanh L.; Yan, Kai; Jamal, Syed Muhammad; Haque, Sayedul; Ahmad, Wasim; Leal, Suzanne M.

2003-01-01

For autosomal recessive nonsyndromic hearing impairment over 30 loci have been mapped and 19 genes have been identified. DFNB38, a novel locus for autosomal recessive nonsyndromic hearing impairment, was localized in a consanguineous Pakistani kindred to 6q26–q27. The affected family members present with profound prelingual sensorineural hearing impairment and use sign language for communications. Linkage was established to microsatellite markers located on chromosome 6q26–q27 (Multipoint lod...

Comparison of general health status in mothers of hearing and hearing-impaired children

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Movallali

2013-05-01

Full Text Available Background and Aim: The birth of a hearing-impaired child and raising him/her often brings special psychological feelings for parents, especially mothers who spend more time with the child. This study aimed to compare the general health status in mothers of hearing-impaired and hearing children. Methods: This was a descriptive-analytic study. General Health Questionnaire was used to identify general health status; and data were analyzed with independent-t test. Results: The general health level of mothers of hearing-impaired children was lower than mothers of normal hearing children (p=0.01 . The average scores of anxiety (p=0.01, depression (p= 0.01 and physical (p=0.02 symptoms and social function (p=0.01 of mothers of hearing-impaired children was higher than mothers of normal hearing ones (p=0.01. Conclusion: Having a child with hearing impairment affects mothers’ general health status. Our findings show that it’s necessary to provide psychological and social support for mothers of hearing-impaired children.

Non-syndromic hearing impairment in India: high allelic heterogeneity among mutations in TMPRSS3, TMC1, USHIC, CDH23 and TMIE.

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Aparna Ganapathy

Full Text Available Mutations in the autosomal genes TMPRSS3, TMC1, USHIC, CDH23 and TMIE are known to cause hereditary hearing loss. To study the contribution of these genes to autosomal recessive, non-syndromic hearing loss (ARNSHL in India, we examined 374 families with the disorder to identify potential mutations. We found four mutations in TMPRSS3, eight in TMC1, ten in USHIC, eight in CDH23 and three in TMIE. Of the 33 potentially pathogenic variants identified in these genes, 23 were new and the remaining have been previously reported. Collectively, mutations in these five genes contribute to about one-tenth of ARNSHL among the families examined. New mutations detected in this study extend the allelic heterogeneity of the genes and provide several additional variants for structure-function correlation studies. These findings have implications for early DNA-based detection of deafness and genetic counseling of affected families in the Indian subcontinent.

Permanent Childhood Hearing Impairment: Aetiological Evaluation of Infants identified through the Irish Newborn Hearing Screening Programme

LENUS (Irish Health Repository)

Smith, A

2017-11-01

The Newborn Hearing Screening Programme (NHSP) was established in Cork University Maternity Hospital (CUMH) in April 2011. Between April 2011 and July 2014, 42 infants were identified with a Permanent Childhood Hearing Impairment (PCHI). Following this diagnosis, infants underwent a paediatric assessment according to recognised guidelines with the intention of identifying the underlying aetiology of the PCHI. The aim of this study was to assess the findings of this aetiological workup via retrospective chart review. PCHI data was obtained from the eSP database. This is a web based information system (eSP) used to track each baby through the screening and referral process A retrospective chart review of these patients was performed. Sixteen (38%) infants were diagnosed with a bilateral sensorineural hearing loss. Two infants had congenital CMV infection. A Connexin 26 gene mutation was detected in one infant. Two infants were diagnosed with Waardenburg syndrome, One with Pendred syndrome and one with Pfeiffer syndrome. Five babies underwent cochlear implantation. Through adherence to the recommended protocol a possible cause of PCHI may be determined. This study has identified areas of future improvement for this service in Ireland.

Hearing impairment, social networks, and coping: the need for families with hearing-impaired children to relate to other parents and to hearing-impaired adults.

Science.gov (United States)

Hintermair, M

2000-03-01

For a report on the stress experiences of parents with hearing-impaired children in Germany, 317 parents completed a survey on how their families communicate and socialize, among other issues. The report focuses on how contacts with other parents and with hearing-impaired adults affect stress experiences, in the context of the child's hearing status and the means of communication. Parents who frequently meet with other parents show evidence of a warm, accepting, trusting relationship with their child. Parents who have many contacts with hearing-impaired adults show evidence of a strong sense of competence in regard to their child's upbringing. The findings confirm the implication found in most reports describing empirical studies. Social support is to be regarded as a cornerstone of psychosocial intervention and has to play as great a role as possible in institutional programs.

Pendred syndrome (goitre and sensorineural hearing loss) maps to chromosome 7 in the region containing the nonsyndromic deafness gene DFNB4.

Science.gov (United States)

Coyle, B; Coffey, R; Armour, J A; Gausden, E; Hochberg, Z; Grossman, A; Britton, K; Pembrey, M; Reardon, W; Trembath, R

1996-04-01

Inherited causes account for about 50% of individuals presenting with childhood (prelingual) hearing loss, of which 70% are due to mutation in numerous single genes which impair auditory function alone (non-syndromic). The remainder are associated with other developmental anomalies termed syndromic deafness. Genes responsible for syndromic forms of hearing loss include the COL4A5 gene in Alport syndrome and the PAX3 and MITF genes in Waardenburg syndrome. Pendred syndrome is an autosomal recessive disorder associated with developmental abnormalities of the cochlea, sensorineural hearing loss and diffuse thyroid enlargement (goitre). Pendred syndrome is the most common syndromal form of deafness, yet the primary defect remains unknown. We have established a panel of 12 families with two or more affected individuals and used them to search for the location of the Pendred gene by linkage analysis. We excluded localization to four previously mapped nonsyndromic deafness loci but obtained conclusive evidence for linkage of the Pendred syndrome gene to microsatellite markers on chromosome 7q31 (D7S495 Zmax 7.32, Qmax = 0). This region contains a gene, DFNBL, for autosomal recessive non-syndromic sensorineural hearing loss. Multipoint analysis indicates that DFNB4 and Pendred syndrome co-localize to the same 5.5 centiMorgan (cM) interval flanked by D7S501 and D7S523. These data raise the possibility that Pendred syndrome is either allelic with DFNB4 or may represent an inherited contiguous gene disorder, not clinically manifest in the heterozygote.

Homozygous disruption of PDZD7 by reciprocal translocation in a consanguineous family: a new member of the Usher syndrome protein interactome causing congenital hearing impairment.

Science.gov (United States)

Schneider, Eberhard; Märker, Tina; Daser, Angelika; Frey-Mahn, Gabriele; Beyer, Vera; Farcas, Ruxandra; Schneider-Rätzke, Brigitte; Kohlschmidt, Nicolai; Grossmann, Bärbel; Bauss, Katharina; Napiontek, Ulrike; Keilmann, Annerose; Bartsch, Oliver; Zechner, Ulrich; Wolfrum, Uwe; Haaf, Thomas

2009-02-15

A homozygous reciprocal translocation, 46,XY,t(10;11),t(10;11), was detected in a boy with non-syndromic congenital sensorineural hearing impairment. Both parents and their four other children were heterozygous translocation carriers, 46,XX,t(10;11) and 46,XY,t(10;11), respectively. Fluorescence in situ hybridization of region-specific clones to patient chromosomes was used to localize the breakpoints within bacterial artificial chromosome (BAC) RP11-108L7 on chromosome 10q24.3 and within BAC CTD-2527F12 on chromosome 11q23.3. Junction fragments were cloned by vector ligation and sequenced. The chromosome 10 breakpoint was identified within the PDZ domain containing 7 (PDZD7) gene, disrupting the open reading frame of transcript PDZD7-C (without PDZ domain) and the 5'-untranslated region of transcript PDZD7-D (with one PDZ and two prolin-rich domains). The chromosome 11 breakpoint was localized in an intergenic segment. Reverse transcriptase-polymerase chain reaction analysis revealed PDZD7 expression in the human inner ear. A murine Pdzd7 transcript that is most similar in structure to human PDZD7-D is known to be expressed in the adult inner ear and retina. PDZD7 shares sequence homology with the PDZ domain-containing genes, USH1C (harmonin) and DFNB31 (whirlin). Allelic mutations in harmonin and whirlin can cause both Usher syndrome (USH1C and USH2D, respectively) and congenital hearing impairment (DFNB18 and DFNB31, respectively). Protein-protein interaction assays revealed the integration of PDZD7 in the protein network related to the human Usher syndrome. Collectively, our data provide strong evidence that PDZD7 is a new autosomal-recessive deafness-causing gene and also a prime candidate gene for Usher syndrome.

What factors influence help-seeking for hearing impairment and hearing aid adoption in older adults?

Science.gov (United States)

Meyer, Carly; Hickson, Louise

2012-02-01

The purpose of this review paper was to identify factors that influence older adults' decisions to seek help for hearing impairment and to adopt hearing aids. A literature search of abstracts within peer-reviewed journal articles published in English between 1990 and 2010 was conducted in online bibliographic databases using the keywords: hearing; impair* or loss; seek* or candidate* or applicant* or acquir*; and hearing aid* or help or advice. Twenty-two articles that were directly relevant to this topic were included in this review. The evidence suggests that individuals are more likely to seek help for hearing impairment and/or adopt hearing aids if they: (1) have a moderate to severe hearing impairment and self-reported hearing-related activity limitations or participation restrictions; (2) are older; (3) perceive their hearing as poor; (4) consider there to be more benefits than barriers to amplification; and (5) perceive their significant other/s as supportive of hearing rehabilitation. A barrier to help-seeking for hearing impairment and hearing aid adoption appears to be general practitioners' management of age-related hearing impairment.

Dimensions for hearing-impaired mobile application usability model

Science.gov (United States)

Nathan, Shelena Soosay; Hussain, Azham; Hashim, Nor Laily; Omar, Mohd Adan

2017-10-01

This paper discuss on the dimensions that has been derived for the hearing-impaired mobile applications usability model. General usability model consist of general dimension for evaluating mobile application however requirements for the hearing-impaired are overlooked and often scanted. This led towards mobile application developed for the hearing-impaired are left unused. It is also apparent that these usability models do not consider accessibility dimensions according to the requirement of the special users. This complicates the work of usability practitioners as well as academician that practices research usability when application are developed for the specific user needs. To overcome this issue, dimension chosen for the hearing-impaired are ensured to be align with the real need of the hearing-impaired mobile application. Besides literature studies, requirements for the hearing-impaired mobile application have been identified through interview conducted with hearing-impaired mobile application users that were recorded as video outputs and analyzed using Nvivo. Finally total of 6 out of 15 dimensions gathered are chosen for the proposed model and presented.

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

Science.gov (United States)

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

Korsakoff Syndrome in Non-alcoholic Psychiatric Patients. Variable Cognitive Presentation and Impaired Frontotemporal Connectivity

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Georgios Nikolakaros

2018-05-01

Full Text Available Background: Non-alcoholic Wernicke's encephalopathy and Korsakoff syndrome are greatly underdiagnosed. There are very few reported cases of neuropsychologically documented non-alcoholic Korsakoff syndrome, and diffusion tensor imaging (DTI data are scarce.Methods: We report clinical characteristics and neuropsychological as well as radiological findings from three psychiatric patients (one woman and two men with a history of probable undiagnosed non-alcoholic Wernicke's encephalopathy and subsequent chronic memory problems.Results: All patients had abnormal neuropsychological test results, predominantly in memory. Thus, the neuropsychological findings were compatible with Korsakoff syndrome. However, the neuropsychological findings were not uniform. The impairment of delayed verbal memory of the first patient was evident only when the results of the memory tests were compared to her general cognitive level. In addition, the logical memory test and the verbal working memory test were abnormal, but the word list memory test was normal. The second patient had impaired attention and psychomotor speed in addition to impaired memory. In the third patient, the word list memory test was abnormal, but the logical memory test was normal. All patients had intrusions in the neuropsychological examination. Executive functions were preserved, except for planning and foresight, which were impaired in two patients. Conventional MRI examination was normal. DTI showed reduced fractional anisotropy values in the uncinate fasciculus in two patients, and in the corpus callosum and in the subgenual cingulum in one patient.Conclusions: Non-alcoholic Korsakoff syndrome can have diverse neuropsychological findings. This may partly explain its marked underdiagnosis. Therefore, a strong index of suspicion is needed. The presence of intrusions in the neuropsychological examination supports the diagnosis. Damage in frontotemporal white matter tracts, particularly in the

Korsakoff Syndrome in Non-alcoholic Psychiatric Patients. Variable Cognitive Presentation and Impaired Frontotemporal Connectivity.

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Nikolakaros, Georgios; Kurki, Timo; Paju, Janina; Papageorgiou, Sokratis G; Vataja, Risto; Ilonen, Tuula

2018-01-01

Background: Non-alcoholic Wernicke's encephalopathy and Korsakoff syndrome are greatly underdiagnosed. There are very few reported cases of neuropsychologically documented non-alcoholic Korsakoff syndrome, and diffusion tensor imaging (DTI) data are scarce. Methods: We report clinical characteristics and neuropsychological as well as radiological findings from three psychiatric patients (one woman and two men) with a history of probable undiagnosed non-alcoholic Wernicke's encephalopathy and subsequent chronic memory problems. Results: All patients had abnormal neuropsychological test results, predominantly in memory. Thus, the neuropsychological findings were compatible with Korsakoff syndrome. However, the neuropsychological findings were not uniform. The impairment of delayed verbal memory of the first patient was evident only when the results of the memory tests were compared to her general cognitive level. In addition, the logical memory test and the verbal working memory test were abnormal, but the word list memory test was normal. The second patient had impaired attention and psychomotor speed in addition to impaired memory. In the third patient, the word list memory test was abnormal, but the logical memory test was normal. All patients had intrusions in the neuropsychological examination. Executive functions were preserved, except for planning and foresight, which were impaired in two patients. Conventional MRI examination was normal. DTI showed reduced fractional anisotropy values in the uncinate fasciculus in two patients, and in the corpus callosum and in the subgenual cingulum in one patient. Conclusions: Non-alcoholic Korsakoff syndrome can have diverse neuropsychological findings. This may partly explain its marked underdiagnosis. Therefore, a strong index of suspicion is needed. The presence of intrusions in the neuropsychological examination supports the diagnosis. Damage in frontotemporal white matter tracts, particularly in the uncinate

Sound localization in noise in hearing-impaired listeners.

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Lorenzi, C; Gatehouse, S; Lever, C

1999-06-01

The present study assesses the ability of four listeners with high-frequency, bilateral symmetrical sensorineural hearing loss to localize and detect a broadband click train in the frontal-horizontal plane, in quiet and in the presence of a white noise. The speaker array and stimuli are identical to those described by Lorenzi et al. (in press). The results show that: (1) localization performance is only slightly poorer in hearing-impaired listeners than in normal-hearing listeners when noise is at 0 deg azimuth, (2) localization performance begins to decrease at higher signal-to-noise ratios for hearing-impaired listeners than for normal-hearing listeners when noise is at +/- 90 deg azimuth, and (3) the performance of hearing-impaired listeners is less consistent when noise is at +/- 90 deg azimuth than at 0 deg azimuth. The effects of a high-frequency hearing loss were also studied by measuring the ability of normal-hearing listeners to localize the low-pass filtered version of the clicks. The data reproduce the effects of noise on three out of the four hearing-impaired listeners when noise is at 0 deg azimuth. They reproduce the effects of noise on only two out of the four hearing-impaired listeners when noise is at +/- 90 deg azimuth. The additional effects of a low-frequency hearing loss were investigated by attenuating the low-pass filtered clicks and the noise by 20 dB. The results show that attenuation does not strongly affect localization accuracy for normal-hearing listeners. Measurements of the clicks' detectability indicate that the hearing-impaired listeners who show the poorest localization accuracy also show the poorest ability to detect the clicks. The inaudibility of high frequencies, "distortions," and reduced detectability of the signal are assumed to have caused the poorer-than-normal localization accuracy for hearing-impaired listeners.

COMPARISON OF ABILITY OF OPPOSITION WORDS PROCESSING BETWEEN HEARING AND HEARING IMPAIRED STUDENT IN FIFTH GRADE IN TEHRAN

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B SHAFIEI

2003-12-01

Full Text Available Introduction: Hearing impaired Persons have disorders in Communication. Theu are not able to learning many aspects of language Structures in Paroper time; quantity and quality. They can not Process these factors as same as Partners. In this research we going to assess and comparison opposite word in hearing and hearing impaired Student in fifth grade. This is a semantic research.
Methods: Subjects of this research were hearing impaired students in fifth grade in tehramwohad + 70dbheaing loss in Best Binaural Average and in order to comparison with them, we selected hearing students in fifth grade. In this research four non linguistic factors were investigated (age, sex, words of Farsi language. The subjects must read these words and write an opposite Word in front of it. In this examination the quantity of types: right, false and without answers.
Findings: The sequence of right answers had significant different in two group. The Sequence of learned words had significant different two groups. The time of processing in hearing students was shorter than hearing impaired students. The female subjects gave right answers more than male subjects. Discussion: The differences between bearing and hearing impaired students were in the quantity of answers specially in right answers and time of doing the test. probably these differences were due to lack of proper lexicon words and/or poor of it. The hearing students had more right answers and were shorter in time of processing.

Attitudes hearing impaired children face from hearing people : a case study from Wollega, Ethiopia

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Olika, Ruth Erin Liselott

2009-01-01

The aim of this research is to find out what kind of attitudes that hearing impaired children face from hearing people, and how these attitudes influence the hearing impaired child’s life. This is a qualitative research project with interview as the instrument of collecting data. The interviews were conducted in Wollega, Ethiopia with four different informant groups: Children with hearing impairment (CWHI), their parents (Parents CWHI), Children with hearing (CWH), and their parents (Parents ...

Prevalence and Parental Awareness of Hearing Loss in Children with Down Syndrome

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Wai-Ling Lau

2015-01-01

Full Text Available Background: To establish the prevalence of hearing deficit in children with Down syndrome (DS in Hong Kong as measured by brainstem auditory evoked potentials (BAEP. The secondary objective is to examine the agreement between BAEP and clinical questioning in detecting hearing deficit in DS. Methods: Consecutive DS patients attending the Down′s Clinic in a regional pediatric referral center were recruited into this cross-sectional study. BAEP data performed within 12 months were retrieved. The care-taker was interviewed with a structured questionnaire to detect any symptom of hearing impairment. BAEP findings and clinical questionings were compared in an agreement analysis using quadratic weighted kappa statistics. Results: Fifty DS patients (35 male, 15 female, mean age 11.70 years ± 5.74 standard deviation were recruited. Eighteen patients (36.0% were identified having hearing deficit by BAEP. Among patients with hearing impairment, 13 patients (72.2% had a conductive deficit, and most have mild to moderate hearing loss. Five patients (27.8% had sensorineural deficit and most have moderate to severe degree. Eight (44.4% had bilateral hearing deficit. Care-takers of 13 patients (26.0% reported symptoms of hearing impairment, with 9 (69.2% having mild symptoms, 3 (23.1% had moderate symptoms and 1 (7.7% had severe symptoms. The weighted kappa was 0.045 (95.0% confidence interval − 0.138-0.229, indicating very poor strength of agreement between BAEP and clinical questioning. For patients with conductive hearing impairment, only 1 patients (7.7% recalled history of otitis media. Conclusions: The estimated point prevalence of hearing impairment in Chinese DS children in Hong Kong is 36%. Our finding of poor strength of agreement between objective testing and symptom questioning reflects significant underestimation of hearing impairment by history taking alone. In view of the high prevalence and low parental awareness, continuous surveillance of

Sentence Writing and Perception of Written Sentences in Hearing-Impaired and Normal-Hearing Primary School Students in Hamadan, Western Iran

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Afsaneh Yaghobi

2011-06-01

Full Text Available Background and Aim: Learning language is acquired in early childhood and gradually developed by new words and new structures. Hearing sense is the most important acquisition for learning this skill. Hearing disorders are barriers for natural language learning. The purpose of this study was to investigate the relationship between writing sentences and perception of written sentences in hearing-impaired and normal-hearing students.Methods: A cross-sectional study was conducted among thirty hearing-impaired students with hearing loss of 70-90 dB and thirty normal hearing students. They were selected from 3rd grade primary school students in Hamadan, a large city in Western Iran. The language skills and non language information was assessed by questionnaire, Action Picture Test, and Sentence Perception Test.Results: Results showed that there was a significant relation between writing sentences and perception of written sentences in hearing impaired students (p<0.001, (r=0.8. This significant relation was seen in normal-hearing students as well (p<0.001, (r=0.7.Conclusion: Disability of hearing-impaired students in verbal communication is not only related to articulation and voice disorders but also is related to their disability to explore and use of language rules. They suffer lack of perception of written sentences, and they are not skilled to convey their feelings and thoughts in order to presenting themselves by using language structures.

Localization of A Novel Autosomal Recessive Non-Syndromic Hearing Impairment Locus (DFNB38) to 6q26–q27 in a Consanguineous Kindred from Pakistan

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Ansar, Muhammad; Ramzan, Mohammad; Pham, Thanh L.; Yan, Kai; Jamal, Syed Muhammad; Haque, Sayedul; Ahmad, Wasim; Leal, Suzanne M.

2010-01-01

For autosomal recessive nonsyndromic hearing impairment over 30 loci have been mapped and 19 genes have been identified. DFNB38, a novel locus for autosomal recessive nonsyndromic hearing impairment, was localized in a consanguineous Pakistani kindred to 6q26–q27. The affected family members present with profound prelingual sensorineural hearing impairment and use sign language for communications. Linkage was established to microsatellite markers located on chromosome 6q26–q27 (Multipoint lod score 3.6). The genetic region for DFNB38 spans 10.1 cM according to the Marshfield genetic map and is bounded by markers D6S980 and D6S1719. This genetic region corresponds to 3.4 MB on the sequence-based physical map. PMID:12890929

38 CFR 4.85 - Evaluation of hearing impairment.

Science.gov (United States)

2010-07-01

... impairment. 4.85 Section 4.85 Pensions, Bonuses, and Veterans' Relief DEPARTMENT OF VETERANS AFFAIRS SCHEDULE FOR RATING DISABILITIES Disability Ratings Impairment of Auditory Acuity § 4.85 Evaluation of hearing impairment. (a) An examination for hearing impairment for VA purposes must be conducted by a state-licensed...

Story retelling skills in Persian speaking hearing-impaired children.

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

2017-05-01

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

Cigarette smoking causes hearing impairment among Bangladeshi population.

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Ahmed Faisal Sumit

Full Text Available Lifestyle including smoking, noise exposure with MP3 player and drinking alcohol are considered as risk factors for affecting hearing synergistically. However, little is known about the association of cigarette smoking with hearing impairment among subjects who carry a lifestyle without using MP3 player and drinking alcohol. We showed here the influence of smoking on hearing among Bangladeshi subjects who maintain a lifestyle devoid of using MP3 player and drinking alcohol. A total of 184 subjects (smokers: 90; non-smokers: 94 were included considering their duration and frequency of smoking for conducting this study. The mean hearing thresholds of non-smoker subjects at 1, 4, 8 and 12 kHz frequencies were 5.63 ± 2.10, 8.56±5.75, 21.06 ± 11.06, 40.79 ± 20.36 decibel (dB, respectively and that of the smokers were 7 ± 3.8, 13.27 ± 8.4, 30.66 ± 12.50 and 56.88 ± 21.58 dB, respectively. The hearing thresholds of the smokers at 4, 8 and 12 kHz frequencies were significantly (p5 years showed higher level of auditory threshold (62.16 ± 19.87 dB at 12 kHz frequency compared with that (41.52 ± 19.21 dB of the subjects smoked for 1-5 years and the difference in auditory thresholds was statistically significant (p<0.0002. In this study, the Brinkman Index (BI of smokers was from 6 to 440 and the adjusted odds ratio showed a positive correlation between hearing loss and smoking when adjusted for age and body mass index (BMI. In addition, age, but not BMI, also played positive role on hearing impairment at all frequencies. Thus, these findings suggested that cigarette smoking affects hearing level at all the frequencies tested but most significantly at extra higher frequencies.

Prevalence and causes of hearing impairment in Africa.

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

2016-02-01

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

Comparison of reading comprehension and working memory in hearing-impaired and normal-hearing children

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Mohammad Rezaei

2013-03-01

Full Text Available Background and Aim: Reading is the most important human need for learning. In normal-hearing people working memory is a predictor of reading comprehension. In this study the relationship between working memory and reading comprehension skills was studied in hearing-impaired children, and then compared with the normal-hearing group.Methods: This was a descriptive-analytic study. The working memory and reading comprehension skills of 18 (8 male, 10 female sever hearing-impaired children in year five of exceptional schools were compared by means of a reading test with 18 hearing children as control group. The subjects in the control group were of the same gender and educational level of the sample group.Results: The children with hearing loss performed similarly to the normal-hearing children in tasks related to auditory-verbal memory of sounds (reverse, visual-verbal memory of letters, and visual-verbal memory of pictures. However, they showed lower levels of performance in reading comprehension (p<0.001. Moreover, no significant relationship was observed between working memory and reading comprehension skills.Conclusion: Findings indicated that children with hearing loss have a significant impairment in the reading comprehension skill. Impairment in language knowledge and vocabulary may be the main cause of poor reading comprehension in these children. In hearing-impaired children working memory is not a strong predictor of reading comprehension.

[Application of MALDI-TOF-MS in gene testing for non-syndromic hearing loss].

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Zeng, Yun; Jiang, Dan; Feng, Da-fei; Jin, Dong-dong; Wu, Xiao-hui; Ding, Yan-li; Zou, Jing

2013-12-01

To investigate the feasibility of Matrix-Assisted Laser Desorption-Ionization Time of Flight Mass Spectrometry (MALDI-TOF-MS) , according to the genetic test of non-syndromic hearing loss (NSHL), and check using the direct sequencing. Peripheral blood was collected from 454 NSHL patients. DNA samples were extracted and 20 loci of the four common disease-causing genes were analysed by MALDI-TOF-MS, including GJB2 (35delG, 167delT, 176_191del16, 235delC, 299_300delAT ), GJB3 (538C→T, 547G→A), SLC26A4 (281C→T, 589G→A, IVS7-2A→G, 1174A→T, 1226G→A, 1229C→T, IVS15+5G→A, 1975G→C, 2027T→A, 2162C→T, 2168A→G), and mitochondrial 12S rRNA (1494C→T, 1555A→G). Direct sequencing was also used to analyse the aforementioned 20 loci in order to validate the accuracy of MALDI-TOF-MS. Among the 454 patients, 166 cases (36.56%) of disease-causing mutations were detected, which included 69 cases (21.15%) of GJB2 gene mutation, four cases (0.88%) of GJB3 gene mutation, 64 cases (14.10%) of SLC26A4 gene mutation, and three cases (0.66%) of mitochondrial 12S rRNA gene mutation. Moreover, the results obtained from direct sequencing and MALDI-TOF-MS were consistent, and the results showed that the two methods were consistent. The MALDI-TOF-MS detection method was designed based on the hearing loss-related mutation hotspots seen in the Chinese population, and it has a high detection rate for NSHL related mutations. In comparison to the conventional detection methods, MALDI-TOF-MS has the following advantages: more detection sites, greater coverage, accurate, high throughput and low cost. Therefore, this method is capable of satisfying the needs of clinical detection for hearing impairment and it is suitable for large-scale implementation.

Effect of simultaneous exposure to occupational noise and cigarette smoke on binaural hearing impairment

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Saber Mohammadi

2010-01-01

Full Text Available In recent years, it has been postulated that cigarette smoking can aggravate noise-induced hearing loss. In this study, we aimed to assess the effect of concurrent exposure to cigarette smoke and occupational noise on binaural hearing impairment (BHI. In an analytic study on the workers of a large wagon manufacturing company in 2007, 622 male workers (252 smokers and 370 non-smokers, matched for other variables participated and their BHI was compared. BHI was significantly higher in smokers than in non-smokers (odds ratio= 5.6, P < 0.001, 95% CI =3.4-9.4. Logistic regression confirmed this significant difference as well, and showed a direct relationship between the amount of BHI and pack/years of smoking. Cigarette smoking accompanied by exposure to workplace noise may play a role in causing binaural hearing impairment, so giving up or decreasing the amount of smoking may prevent or at least delay binaural hearing impairment, and eventually reduce its compensation costs.

A combination of two truncating mutations in USH2A causes more severe and progressive hearing impairment in Usher syndrome type IIa

DEFF Research Database (Denmark)

Hartel, Bas P.; Lofgren, Maria; Huygen, Patrick L. M.

2016-01-01

Objectives Usher syndrome is an inherited disorder that is characterized by hearing impairment (HI), retinitis pigmentosa, and in some cases vestibular dysfunction. Usher syndrome type IIa is caused by mutations in USH2A. HI in these patients is highly heterogeneous and the present study evaluates...... the effects of different types of USH2A mutations on the audiometric phenotype. Data from two large centres of expertise on Usher Syndrome in the Netherlands and Sweden were combined in order to create a large combined sample of patients to identify possible genotype-phenotype correlations. Design...... A retrospective study on HI in 110 patients (65 Dutch and 45 Swedish) genetically diagnosed with Usher syndrome type IIa. We used methods especially designed for characterizing and testing differences in audiological phenotype between patient subgroups. These methods included Age Related Typical Audiograms (ARTA...

Image and Video for Hearing Impaired People

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Aran Oya

2007-01-01

Full Text Available We present a global overview of image- and video-processing-based methods to help the communication of hearing impaired people. Two directions of communication have to be considered: from a hearing person to a hearing impaired person and vice versa. In this paper, firstly, we describe sign language (SL and the cued speech (CS language which are two different languages used by the deaf community. Secondly, we present existing tools which employ SL and CS video processing and recognition for the automatic communication between deaf people and hearing people. Thirdly, we present the existing tools for reverse communication, from hearing people to deaf people that involve SL and CS video synthesis.

Audiological findings in Usher syndrome types IIa and II (non-IIa).

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Sadeghi, Mehdi; Cohn, Edward S; Kelly, William J; Kimberling, William J; Tranebjoerg, Lisbeth; Möller, Claes

2004-03-01

The aim was to define the natural history of hearing loss in Usher syndrome type IIa compared to non-IIa. People with Usher syndrome type II show moderate-to-severe hearing loss, normal balance and retinitis pigmentosa. Several genes cause Usher syndrome type II. Our subjects formed two genetic groups: (1) subjects with Usher syndrome type IIa with a mutation and/or linkage to the Usher IIa gene; (2) subjects with the Usher II phenotype with no mutation and/or linkage to the Usher IIa gene. Four hundred and two audiograms of 80 Usher IIa subjects were compared with 435 audiograms of 87 non-IIa subjects. Serial audiograms with intervals of > or = 5 years were examined for progression in 109 individuals Those with Usher syndrome type IIa had significantly worse hearing thresholds than those with non-IIa Usher syndrome after the second decade. The hearing loss in Usher syndrome type IIa was found to be more progressive, and the progression started earlier than in non-IIa Usher syndrome. This suggests an auditory phenotype for Usher syndrome type IIa that is different from that of other types of Usher syndrome II. Thus, this is to our knowledge one of the first studies showing a genotype-phenotype auditory correlation.

Concurrent Speech Segregation Problems in Hearing Impaired Children

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Hossein Talebi

2014-04-01

Full Text Available Objective: This study was a basic investigation of the ability of concurrent speech segregation in hearing impaired children. Concurrent segregation is one of the fundamental components of auditory scene analysis and plays an important role in speech perception. In the present study, we compared auditory late responses or ALRs between hearing impaired and normal children. Materials & Methods: Auditory late potentials in response to 12 double vowels were recorded in 10 children with moderate to severe sensory neural hearing loss and 10 normal children. Double vowels (pairs of synthetic vowels were presented concurrently and binaurally. Fundamental frequency (F0 of these vowels and the size of the difference in F0 between vowels was 100 Hz and 0.5 semitones respectively. Results: Comparing N1-P2 amplitude showed statistically significant difference in some stimuli between hearing impaired and normal children (P<0.05. This complex indexing the vowel change detection and reflecting central auditory speech representation without active client participation was decreased in hearing impaired children. Conclusion: This study showed problems in concurrent speech segregation in hearing impaired children evidenced by ALRs. This information indicated deficiencies in bottom-up processing of speech characteristics based on F0 and its differences in these children.

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

Science.gov (United States)

2016-01-01

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

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

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Diane Bell

2016-09-01

Conclusion: It is crucially important for academic, support and administrative staff to be aware of both the assumed ‘hearing’ identity and therefore subsequent non-disclosure practices of students with a hearing impairment using the oral method of communication. Universities need to put measures in place to encourage students to voluntarily disclose their hearing impairment in order to provide more targeted teaching and learning support. This could lead to improved educational outcomes for students.

Follow-up of permanent hearing impairment in childhood.

Science.gov (United States)

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

2016-02-01

Programmes for early childhood childhood hearing impairment identification allows to quickly start the appropriate hearing aid fitting and rehabilitation process; nevertheless, a large number of patients do not join the treatment program. The goal of this article is to present the results of a strategic review of the strengths, weaknesses, opportunities and threats connected with the audiologic/prosthetic/language follow-up process of children with bilateral permanent hearing impairment. Involving small children, the follow-up includes the involvement of specialised professionals of a multidisciplinary team and a complex and prolonged multi-faced management. Within the framework of the Italian Ministry of Health project CCM 2013 "Preventing Communication Disorders: a Regional Program for Early Identification, Intervention and Care of Hearing Impaired Children", the purpose of this analysis was to propose recommendations that can harmonise criteria for outcome evaluation and provide guidance on the most appropriate assessment methods to be used in the follow-up course of children with permanent hearing impairment. © Copyright by Società Italiana di Otorinolaringologia e Chirurgia Cervico-Facciale.

New polymorphic mtDNA restriction site in the 12S rRNA gene detected in Tunisian patients with non-syndromic hearing loss

International Nuclear Information System (INIS)

Mkaouar-Rebai, Emna; Tlili, Abdelaziz; Masmoudi, Saber; Charfeddine, Ilhem; Fakhfakh, Faiza

2008-01-01

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

The management of children with Down syndrome and profound hearing loss.

Science.gov (United States)

Phelan, E; Pal, R; Henderson, L; Green, K M J; Bruce, I A

2016-01-01

Although, the association between Down syndrome (DS) and conductive hearing loss is well recognized, the fact that a small proportion of these children may have a severe to profound sensorineural hearing loss that could benefit from cochlear implantation (CI) is less well understood. The management of significant co-morbidities in children with DS can delay initial diagnosis of hearing impairment and assessment of suitability for CI can likewise be challenging, due to difficulties conditioning to behavioural hearing tests. We performed a retrospective case note review of three children with DS referred to the Manchester Cochlear Implant Programme. Three illustrative cases are described including CI in a 4 years old. Using conventional outcome measurement instruments, the outcome could be considered to be suboptimal with a Categories of Auditory Performance score of 4 at 6 months post-op and at last follow up. In part, this is likely to reflect the delay in implantation, but the role of cognitive impairment must be considered. The cases described emphasize the importance of comprehensive radiological and audiological assessment in children with DS being considered for CI. The influence of cognitive impairment upon outcome of CI must be taken into account, but should not be considered a contra-indication to implantation in children with DS. Benefit that might be considered limited when quantified using existing general outcome measurement instruments, may have a significant impact upon psychosocial development and quality of life in children with significant cognitive impairment, or other additional needs.

A combination of two truncating mutations in USH2A causes more severe and progressive hearing impairment in Usher syndrome type IIa.

Science.gov (United States)

Hartel, Bas P; Löfgren, Maria; Huygen, Patrick L M; Guchelaar, Iris; Lo-A-Njoe Kort, Nicole; Sadeghi, Andre M; van Wijk, Erwin; Tranebjærg, Lisbeth; Kremer, Hannie; Kimberling, William J; Cremers, Cor W R J; Möller, Claes; Pennings, Ronald J E

2016-09-01

Usher syndrome is an inherited disorder that is characterized by hearing impairment (HI), retinitis pigmentosa, and in some cases vestibular dysfunction. Usher syndrome type IIa is caused by mutations in USH2A. HI in these patients is highly heterogeneous and the present study evaluates the effects of different types of USH2A mutations on the audiometric phenotype. Data from two large centres of expertise on Usher Syndrome in the Netherlands and Sweden were combined in order to create a large combined sample of patients to identify possible genotype-phenotype correlations. A retrospective study on HI in 110 patients (65 Dutch and 45 Swedish) genetically diagnosed with Usher syndrome type IIa. We used methods especially designed for characterizing and testing differences in audiological phenotype between patient subgroups. These methods included Age Related Typical Audiograms (ARTA) and a method to evaluate the difference in the degree of HI developed throughout life between subgroups. Cross-sectional linear regression analysis of last-visit audiograms for the best hearing ear demonstrated a gradual decline of hearing over decades. The congenital level of HI was in the range of 16-33 dB at 0.25-0.5 kHz, and in the range of 51-60 dB at 1-8 kHz. The annual threshold deterioration was in the range of 0.4-0.5 dB/year at 0.25-2 kHz and in the range of 0.7-0.8 dB/year at 4-8 kHz. Patients with two truncating mutations, including homozygotes for the common c.2299delG mutation, developed significantly more severe HI throughout life than patients with one truncating mutation combined with one nontruncating mutation, and patients with two nontruncating mutations. The results have direct implications for patient counselling in terms of prognosis of hearing and may serve as baseline measures for future (genetic) therapeutic interventions. Copyright © 2016 Elsevier B.V. All rights reserved.

Hearing impairment associated with oral terbinafine use: a case series and case/non-case analysis in the Netherlands Pharmacovigilance Centre Lareb database and VigiBase™.

Science.gov (United States)

Scholl, Joep H G; van Puijenbroek, Eugene P

2012-08-01

The Netherlands Pharmacovigilance Centre Lareb received reports of six cases of hearing impairment in association with oral terbinafine use. This study describes these cases and provides support for this association from the Lareb database for spontaneous adverse drug reaction (ADR) reporting and from Vigibase™, the ADR database of the WHO Collaborating Centre for International Drug Monitoring, the Uppsala Monitoring Centre. The objective of the current study was to identify whether the observed association between oral terbinafine use and hearing impairment, based on cases received by Lareb, constitutes a safety signal. Cases of hearing impairment in oral terbinafine users are described. In a case/non-case analysis, the strength of the association in Vigibase™ and the Lareb database was determined (date of analysis August 2011) by calculating the reporting odds ratios (RORs), adjusted for possible confounding by age, sex and ototoxic concomitant medication. For the purpose of this study, RORs were calculated for deafness, hypoacusis and the combination of both, defined as hearing impairment. In the Lareb database, six reports concerning individuals aged 31-82 years, who developed hearing impairment after starting oral terbinafine, were present. The use of oral terbinafine was disproportionally associated with hypoacusis in both the Lareb database (adjusted ROR 3.9; 95% CI 1.7, 9.0) and in Vigibase™ (adjusted ROR 1.7; 95% CI 1.0, 2.8). Deafness was not disproportionally present in either of the databases. Based on the described cases and the statistical analyses from both databases, a causal relationship between the use of oral terbinafine and hearing impairment is possible. The mechanism by which terbinafine could cause hearing impairment has not been elucidated yet. The pharmacological action of terbinafine is based on the inhibition of squalene epoxidase, an enzyme present in both fungal and human cells. This inhibition might result in a decrease in

38 CFR 4.86 - Exceptional patterns of hearing impairment.

Science.gov (United States)

2010-07-01

... hearing impairment. 4.86 Section 4.86 Pensions, Bonuses, and Veterans' Relief DEPARTMENT OF VETERANS AFFAIRS SCHEDULE FOR RATING DISABILITIES Disability Ratings Impairment of Auditory Acuity § 4.86 Exceptional patterns of hearing impairment. (a) When the puretone threshold at each of the four specified...

Psychopathology among a sample of hearing impaired adolescents.

Science.gov (United States)

Mosaku, Kolawole; Akinpelu, Victoria; Ogunniyi, Grace

2015-12-01

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

Objective Scaling of Sound Quality for Normal-Hearing and Hearing-Impaired Listeners

DEFF Research Database (Denmark)

Nielsen, Lars Bramsløw

) Subjective sound quality ratings of clean and distorted speech and music signals, by normal-hearing and hearing-impaired listeners, to provide reference data, 2) An auditory model of the ear, including the effects of hearing loss, based on existing psychoacoustic knowledge, coupled to 3) An artificial neural......A new method for the objective estimation of sound quality for both normal-hearing and hearing-impaired listeners has been presented: OSSQAR (Objective Scaling of Sound Quality and Reproduction). OSSQAR is based on three main parts, which have been carried out and documented separately: 1...... network, which was trained to predict the sound quality ratings. OSSQAR predicts the perceived sound quality on two independent perceptual rating scales: Clearness and Sharpness. These two scales were shown to be the most relevant for assessment of sound quality, and they were interpreted the same way...

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

Science.gov (United States)

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

2015-01-01

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

"Non-Vocalization": A Phonological Error Process in the Speech of Severely and Profoundly Hearing Impaired Adults, from the Point of View of the Theory of Phonology as Human Behaviour

Science.gov (United States)

Halpern, Orly; Tobin, Yishai

2008-01-01

"Non-vocalization" (N-V) is a newly described phonological error process in hearing impaired speakers. In N-V the hearing impaired person actually articulates the phoneme but without producing a voice. The result is an error process looking as if it is produced but sounding as if it is omitted. N-V was discovered by video recording the speech of…

Communication between hearing impaired and normal hearing students: a facilitative proposal of learning in higher education

Directory of Open Access Journals (Sweden)

Krysne Kelly de França Oliveira

2014-09-01

Full Text Available Introduction: There has been an increase in the number of hearing impaired people with access to higher education. Most of them are young people from a different culture who present difficulties in communication, inter-relationship, and learning in a culture of normal hearing people, because they use a different language, the Brazilian Sign Language - LIBRAS. Objective: The present study aimed to identify the forms of communication used between hearing impaired and normal hearing students, verifying how they can interfere with the learning process of the first. Methods: A qualitative study that used the space of a private university in the city of Fortaleza, Ceará state, Brazil, from February to April 2009. We carried out semi-structured interviews with three hearing impaired students, three teachers, three interpreters, and three normal hearing students. The content of the speeches was categorized and organized by the method of thematic analysis. Results: We verified that the forms of communication used ranged from mime and gestures to writing and drawing, but the most accepted by the hearing impaired students was LIBRAS. As a method of communication, it supports the learning of hearing impaired students, and with the mediation of interpreters, it gives them conditions to settle in their zones of development, according to the precepts of Vygotsky. Conclusion: Thus, we recognize the importance of LIBRAS as predominant language, essential to the full academic achievement of hearing impaired students; however, their efforts and dedication, as well as the interest of institutions and teachers on the deaf culture, are also important for preparing future professionals.

Attitude and help-seeking for hearing impairment

NARCIS (Netherlands)

vandenBrink, RHS; Wit, HP; Kempen, GIJM; vanHeuvelen, MJG

1996-01-01

The aim of the present study was to investigate help-seeking for hearing impairment in the elderly, and to compare groups showing dissimilar help-seeking on their attitude toward hearing loss and hearing aids. Attitude factors were based on a revised version of the Health Belief Model, and included

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

Science.gov (United States)

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

2016-04-22

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

HIV/AIDS among Adolescents with Hearing Impairment in Nigeria ...

African Journals Online (AJOL)

AIDS to be elusive; such as prejudice against individuals with hearing impairment, lack of adequate data, exclusion from programmes that talk about sexuality, cultural beliefs, poor knowledge and attitude of adolescents with hearing impairment ...

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

International Nuclear Information System (INIS)

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

2009-01-01

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.

[Connexin gene 26 (GJB2) mutations in patients with hereditary non-syndromic sensorineural loss of hearing in the Republic of Sakha (Yakutia)].

Science.gov (United States)

Barashkov, N A; Dzhemileva, L U; Fedorova, S A; Maksimova, N R; Khusnutdinova, E K

2008-01-01

The aim of the study was to elucidate the causes of hereditary non-syndromic loss of hearing, a frequent monogene pathology in the Republic of Sakha (Yakutia). A search for mutations in the coding sequence of the connexin 26 gene gap-junction B2 (GJB2) was undertaken in 79 members of 65 unrelated families with the diagnosis of grade III-IV non-syndromic bilateral sensorineural loss of hearing. Five recessive mutations (35delG, V371, 312-326del14, 333-334delAA, R127H) and three polymorphic variants (V271, M34T, E114G) were identified in Yakut patients. Mutations 35delG (41.7%), 312-326dell4 (4.2%), and 333-334delAA (4.2%) were found in Caucasian patients (Russians, Ukrainians, Inguish). Yakuts were carriers of mutations 35delG (2.1%), V371 (2.1%), R127H (1.0%) and sequence variants V271 (6.3%), M34T (1.0%), E114G (1.0%). GJB2 mutations were identified in 50.1% of the Caucasian patients and in 7.2% of the Yakut patients. The low frequency of GJB2 mutations in Yakuts with non-syndromic sensorineural loss of hearing testifies to the presence of mutations of other genes controlling sound perception in this population.

The Mass1frings mutation underlies early onset hearing impairment in BUB/BnJ mice, a model for the auditory pathology of Usher syndrome IIC

Science.gov (United States)

Johnson, K.R.; Zheng, Q.Y.; Weston, M.D.; Ptacek, L.J.; Noben-Trauth, K.

2010-01-01

The human ortholog of the gene responsible for audiogenic seizure susceptibility in Frings and BUB/BnJ mice (mouse gene symbol Mass1) recently was shown to underlie Usher syndrome type IIC (USH2C). Here we report that the Mass1frings mutation is responsible for the early onset hearing impairment of BUB/BnJ mice. We found highly significant linkage of Mass1 with ABR threshold variation among mice from two backcrosses involving BUB/BnJ mice with mice of strains CAST/EiJ and MOLD/RkJ. We also show an additive effect of the Cdh23 locus in modulating the progression of hearing loss in backcross mice. Together, these two loci account for more than 70% of the total ABR threshold variation among the backcross mice at all ages. The modifying effect of the strain-specific Cdh23ahl variant may account for the hearing and audiogenic seizure differences observed between Frings and BUB/BnJ mice, which share the Mass1frings mutation. During postnatal cochlear development in BUB/BnJ mice, stereocilia bundles develop abnormally and remain immature and splayed into adulthood, corresponding with the early onset hearing impairment associated with Mass1frings. Progressive base–apex hair cell degeneration occurs at older ages, corresponding with the age-related hearing loss associated with Cdh23ahl. The molecular basis and pathophysiology of hearing loss suggest BUB/BnJ and Frings mice as models to study cellular and molecular mechanisms underlying USH2C auditory pathology. PMID:15820310

Peripheral auditory processing and speech reception in impaired hearing

DEFF Research Database (Denmark)

Strelcyk, Olaf

One of the most common complaints of people with impaired hearing concerns their difficulty with understanding speech. Particularly in the presence of background noise, hearing-impaired people often encounter great difficulties with speech communication. In most cases, the problem persists even...... if reduced audibility has been compensated for by hearing aids. It has been hypothesized that part of the difficulty arises from changes in the perception of sounds that are well above hearing threshold, such as reduced frequency selectivity and deficits in the processing of temporal fine structure (TFS......) at the output of the inner-ear (cochlear) filters. The purpose of this work was to investigate these aspects in detail. One chapter studies relations between frequency selectivity, TFS processing, and speech reception in listeners with normal and impaired hearing, using behavioral listening experiments. While...

Preliminary evaluation of child self-rating using the Child Tourette Syndrome Impairment Scale.

Science.gov (United States)

Cloes, Kelly Isaacs; Barfell, Kara S Francis; Horn, Paul S; Wu, Steve W; Jacobson, Sarah E; Hart, Kathleen J; Gilbert, Donald L

2017-03-01

To evaluate and compare how children with Tourette syndrome and parents rate tic and non-tic behavioral related impairment in home, school, and social domains; to compare these with clinician tic ratings; and to identify factors that may predict greater impairment. In a sample of 85 Tourette syndrome and 92 healthy control families, the Child Tourette Syndrome Impairment Scale, designed for parent-report and which includes 37 items rated for tic and non-tic impairment, was administered to parents and, with the referent modified, to children ages 9 to 17 years. Tic severity was rated using the Yale Global Tic Severity Scale (YGTSS). Analyses utilized descriptive and multivariate statistics. Tourette syndrome children's and parents' impairment ratings were higher than HC (ptic impairment ratings correlated with YGTSS (r=0.36 to 0.37; ptic and all 37 non-tic impairment items. For 29 items, children self-rated impairment higher for tics than non-tics. Diagnoses of attention-deficit-hyperactivity disorder and obsessive-compulsive disorder had larger effects on parent impairment ratings. The Child Tourette Syndrome Impairment Scale appears informative for child self-rating in Tourette syndrome. © 2016 Mac Keith Press.

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

Science.gov (United States)

2010-10-01

... OF HUMAN DEVELOPMENT SERVICES, DEPARTMENT OF HEALTH AND HUMAN SERVICES THE ADMINISTRATION FOR... impairment including deafness. (a) A child is classified as deaf if a hearing impairment exists which is so... hearing loss can include impaired listening skills, delayed language development, and articulation...

Estrogen and hearing from a clinical point of view; characteristics of auditory function in women with Turner syndrome.

Science.gov (United States)

Hederstierna, Christina; Hultcrantz, Malou; Rosenhall, Ulf

2009-06-01

Turner syndrome is a chromosomal aberration affecting 1:2000 newborn girls, in which all or part of one X chromosome is absent. This leads to ovarial dysgenesis and little or no endogenous estrogen production. These women have, among many other syndromal features, a high occurrence of ear and hearing problems, and neurocognitive dysfunctions, including reduced visual-spatial abilities; it is assumed that estrogen deficiency is at least partially responsible for these problems. In this, study 30 Turner women aged 40-67, with mild to moderate hearing loss, performed a battery of hearing tests aimed at localizing the lesion causing the sensorineural hearing impairment and assessing central auditory function, primarily sound localization. The results of TEOAE, ABR and speech recognition scores in noise were all indicative of cochlear dysfunction as the cause of the sensorineural impairment. Phase audiometry, a test for sound localization, showed mild disturbances in the Turner women compared to the reference group, suggesting that auditory-spatial dysfunction is another facet of the recognized neurocognitive phenotype in Turner women.

Etiology and prevalence rate of bilateral sensorineural hearing impairment in children born in Kobe city over a 10 year period

International Nuclear Information System (INIS)

Yoshioka, Mieko; Naito, Yashushi

2008-01-01

The study was carried out on children born over a 10 year period from 1997 to 2006 in Kobe city and referred to our center for specialist audiological assessment. A total of 107 cases had a bilateral sensorineural hearing impairment averaging 25 dB or over in the better hearing ear during the study period. To ascertain causes of sensorineural hearing impairment, full medical histories were obtained with detailed family history relevant to hearing impairment and perinatal course for adverse etiological factors. The children were investigated for possible congenital infection and chromosomal anomalies. Children with positive family history of deafness in parents or siblings constituted 11.2% of cases (genetic group). Other etiological groups showed the following distribution: syndromal group 5.6%; inner ear anomalies 5.6%; perinatal group 13.1%; congenital infection 11.2%; chromosomal anomalies 16.8%; multiple congenital anomalies 5.6%; causes unknown 30.9%. The high incidence of causes unknown indicates that steps should be taken to yield a diagnosis. The total number of children born in Kobe city was 117,896 during the period from 1997 to 2005, which gave a prevalence rate of hearing impairment of 0.87/1,000 births. Newborn hearing screening identified many children earlier and also provide the opportunity to finetune the evaluation. (author)

Syllabic compression and speech intelligibility in hearing impaired listeners

NARCIS (Netherlands)

Verschuure, J.; Dreschler, W. A.; de Haan, E. H.; van Cappellen, M.; Hammerschlag, R.; Maré, M. J.; Maas, A. J.; Hijmans, A. C.

1993-01-01

Syllabic compression has not been shown unequivocally to improve speech intelligibility in hearing-impaired listeners. This paper attempts to explain the poor results by introducing the concept of minimum overshoots. The concept was tested with a digital signal processor on hearing-impaired

Reading comprehension of pupils with hearing impairment

OpenAIRE

Pinkasová, Lucie

2010-01-01

Presented diploma thesis is focused on reading problems of people with hearing impairment. The theoretical part deals a hearing impairment, process of acquisition of reading skills of healthy people and deaf people as well. The practical part efforts to clarify preparations, process and results of the research. I am presenting my own reading comprehension test, which was compiled on purpose due to the research. The goal of my diploma thesis is to determine when the level of reading acquiremen...

Binaural pitch perception in hearing-impaired listeners

DEFF Research Database (Denmark)

Dau, Torsten; Santurette, Sébastien; Strelcyk, Olaf

2007-01-01

When two white noises differing only in phase in a particular frequency range are presented simultaneously each to one of our ears, a pitch sensation may be perceived inside the head. This phenomenon, called ’binaural pitch’ or ’dichotic pitch’, can be produced by frequency-dependent interaural...... phasedifference patterns. The evaluation of these interaural phase differences depends on the functionality of the binaural auditory system and the spectro-temporal information at its input. A melody recognition task was performed in the present study using pure-tone stimuli and six different types of noises...... that can generate a binaural pitch sensation. Normal-hearing listeners and hearing-impaired listeners with different kinds of hearing impairment participated in the experiment....

Usher syndrome type III can mimic other types of Usher syndrome.

Science.gov (United States)

Pennings, Ronald J E; Fields, Randall R; Huygen, Patrick L M; Deutman, August F; Kimberling, William J; Cremers, Cor W R J

2003-06-01

Clinical and genetic characteristics are presented of 2 patients from a Dutch Usher syndrome type III family who have a new homozygous USH3 gene mutation: 149-152delCAGG + insTGTCCAAT. One individual (IV:1) is profoundly hearing impaired and has normal vestibular function and retinitis punctata albescens (RPA). The other individual is also profoundly hearing impaired, but has well-developed speech, vestibular areflexia, and retinitis pigmentosa sine pigmento (RPSP). These findings suggest that Usher syndrome type III can be clinically misdiagnosed as either Usher type I or II; that Usher syndrome patients who are profoundly hearing impaired and have normal vestibular function should be tested for USH3 mutations; and that RPA and RPSP can occur as fundoscopic manifestations of pigmentary retinopathy in Usher syndrome.

Comparison of Different Levels of Reading Comprehension between Hearing-Impaired Loss and Normal-Hearing Students

Directory of Open Access Journals (Sweden)

Azam Sharifi

2011-12-01

Full Text Available Background and Aim: Reading skill is one of the most important necessities of students' learning in everyday life. This skill is referred to the ability of comprehension, comment and conclusion from texts and receiving the meaning of the massage which is composed. Educational development in any student has a direct relation with the ability of the comprehension. This study is designed to investigate the effects of hearing loss on reading comprehension in hearing-impaired students compared to normal-hearing ones.Methods: Seventeen hearing-impaired students in 4th year of primary exceptional schools in Karaj, Robatkarim and Shahriyar, Iran, were enrolled in this cross-sectional study. Seventeen normal-hearing students were randomly selected from ordinary schools next to exceptional ones as control group. They were compared for different levels of reading comprehension using the international standard booklet (PIRLS 2001. Results: There was a significant difference in performance between hearing-impaired and normal- hearing students in different levels of reading comprehension (p<0.05.Conclusion: Hearing loss has negative effects on different levels of reading comprehension, so in exceptional centers, reconsideration in educational planning in order to direct education from memorizing to comprehension and deeper layers of learning seems necessary.

Prevalence of hearing impairment in a rural midwestern cohort: estimates from the Keokuk county rural health study, 1994 to 1998.

Science.gov (United States)

Flamme, Gregory A; Mudipalli, V Ranjit; Reynolds, Stephen J; Kelly, Kevin M; Stromquist, Ann M; Zwerling, Craig; Burmeister, Leon F; Peng, Shu-Chen; Merchant, James A

2005-06-01

The current paper estimates the prevalence of hearing problems in a rural population, analyzes the prevalence of hearing problems across age groups, and compares the prevalence of hearing problems in this population with results obtained in other populations. Data were obtained from a random sample of the residents of a rural county, stratified by farm, rural non-farm, and town residence. Hearing test results were obtained from participants between the ages of 8 and 92 years (N = 1972; 47% male). Pure-tone thresholds were classified as normal or impaired using a number of metrics, including speech intelligibility index values. Selected comparisons of crude rates were made with previous population-based studies of hearing loss prevalence. Nearly all (99%) of the participants in this study had significant hearing impairment. Atypical hearing impairment is most prevalent at 6 kHz. In males, this excess impairment shifts to lower frequencies with age but monotonically decreases in females. Notched configurations were most common among those between 30 and 59 years old. In females, the prevalence of hearing impairments sufficient to interfere with speech understanding begins to rapidly increase in the 6th decade (4th decade in males). Comparisons with prior population-based studies in the United States and Great Britain identified few significant differences. Significant hearing impairment is common in rural populations. The high prevalence in this population is similar to that found in other population-based studies. Future studies are needed to examine (1) the risk factors for hearing impairment, (2) the natural course of hearing problems across the life span, and (3) the effect of programs for the prevention of hearing impairment and rehabilitation for persons with existing hearing impairments.

Rhyme and Syllable Recognition in Severe to Profound Hearing-Impaired Children

Directory of Open Access Journals (Sweden)

MohammadReza Keihani

2003-09-01

Full Text Available Objective: A research was conducted to assess the phonological awareness in hearing-impaired children in comparison to normal children. In this context, we discussed about the ability of these children in identification of rhyme and word segmentation to syllables. Method and Material: The sample of this study is composed of 320 children, 160 normal & 160 hearing-impaired with a hearing-loss. Of over 70 dB, studying in the 1st t 4th grade of the primary schools in Tehran. They are divided into two groups at each level (20 girls & 20 boys. Results: 1 Hearing-impaired children's scores on test related to rhyme and syllable is lower as compared with normal children. 2 Hearing-impaired children are more potent on test of word segmentation to syllables in comparison to that related to rhyme, while normal children are more successful on test of word segmentation to syllables as compared with that related to rhyme. Discussion: Hearing is an important factor in phonological awareness. Formal education at special schools doesn't compensate for the hearing impairment as to development of the phonological awareness.

Modeling auditory processing and speech perception in hearing-impaired listeners

DEFF Research Database (Denmark)

Jepsen, Morten Løve

in a diagnostic rhyme test. The framework was constructed such that discrimination errors originating from the front-end and the back-end were separated. The front-end was fitted to individual listeners with cochlear hearing loss according to non-speech data, and speech data were obtained in the same listeners......A better understanding of how the human auditory system represents and analyzes sounds and how hearing impairment affects such processing is of great interest for researchers in the fields of auditory neuroscience, audiology, and speech communication as well as for applications in hearing......-instrument and speech technology. In this thesis, the primary focus was on the development and evaluation of a computational model of human auditory signal-processing and perception. The model was initially designed to simulate the normal-hearing auditory system with particular focus on the nonlinear processing...

Validation of the Korean Version of the Spatial Hearing Questionnaire for Assessing the Severity and Symmetry of Hearing Impairment.

Science.gov (United States)

Kong, Tae Hoon; Park, Yoon Ah; Bong, Jeong Pyo; Park, Sang Yoo

2017-07-01

Spatial hearing refers to the ability to understand speech and identify sounds in various environments. We assessed the validity of the Korean version of the Spatial Hearing Questionnaire (K-SHQ). We performed forward translation of the original English SHQ to Korean and backward translation from the Korean to English. Forty-eight patients who were able to read and understand Korean and received a score of 24 or higher on the Mini-Mental Status Examination were included in the study. Patients underwent pure tone audiometry (PTA) using a standard protocol and completed the K-SHQ. Internal consistency was evaluated using Cronbach's alpha, and factor analysis was performed to prove reliability. Construct validity was tested by comparing K-SHQ scores from patients with normal hearing to those with hearing impairment. Scores were compared between subjects with unilateral or bilateral hearing loss and between symmetrical and asymmetrical hearing impairment. Cronbach's alpha showed good internal consistency (0.982). Two factors were identified by factor analysis: There was a significant difference in K-SHQ scores for patients with normal hearing compared to those with hearing impairment. Patients with asymmetric hearing impairment had higher K-SHQ scores than those with symmetric hearing impairment. This is related to a lower threshold of PTA in the better ear of subjects. The hearing ability of the better ear is correlated with K-SHQ score. The K-SHQ is a reliable and valid tool with which to assess spatial hearing in patients who speak and read Korean. K-SHQ score reflects the severity and symmetry of hearing impairment. © Copyright: Yonsei University College of Medicine 2017

Ear malformations, hearing loss and hearing rehabilitation in children with Treacher Collins syndrome.

Science.gov (United States)

Rosa, Francisco; Coutinho, Miguel Bebiano; Ferreira, João Pinto; Sousa, Cecilia Almeida

2016-01-01

The aim of this study was to assess the main ear malformations, hearing loss and auditory rehabilitation in children with Treacher Collins syndrome. We performed a retrospective study of 9 children with Treacher Collins syndrome treated in a central hospital between January 2003 and January 2013. This study showed a high incidence of malformations of the outer and middle ear, such as microtia, atresia or stenosis of the external auditory canal, hypoplastic middle ear cavity, dysmorphic or missing ossicular chain. Most patients had bilateral hearing loss of moderate or high degree. In the individuals studied, there was functional improvement in patients with bone-anchored hearing aids in relation to conventional hearing aids by bone conduction. Treacher Collins syndrome is characterized by bilateral malformations of the outer and middle ear. Hearing rehabilitation in these children is of utmost importance, and bone-anchored hearing aids is the method of choice. Copyright © 2014 Elsevier España, S.L.U. y Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

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

Science.gov (United States)

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.

Genes and Syndromic Hearing Loss.

Science.gov (United States)

Keats, Bronya J. B.

2002-01-01

This article provides a description of the human genome and patterns of inheritance and discusses genes that are associated with some of the syndromes for which hearing loss is a common finding, including: Waardenburg, Stickler, Jervell and Lange-Neilsen, Usher, Alport, mitochondrial encephalomyopathy, and sensorineural hearing loss. (Contains…

Narrative competence among hearing-impaired and normal-hearing children: analytical cross-sectional study

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Alexandra Dezani Soares

Full Text Available CONTEXT AND OBJECTIVE: Oral narrative is a means of language development assessment. However, standardized data for deaf patients are scarce. The aim here was to compare the use of narrative competence between hearing-impaired and normal-hearing children. DESIGN AND SETTING: Analytical cross-sectional study at the Department of Speech-Language and Hearing Sciences, Universidade Federal de São Paulo. METHODS: Twenty-one moderately to profoundly bilaterally hearing-impaired children (cases and 21 normal-hearing children without language abnormalities (controls, matched according to sex, age, schooling level and school type, were studied. A board showing pictures in a temporally logical sequence was presented to each child, to elicit a narrative, and the child's performance relating to narrative structure and cohesion was measured. The frequencies of variables, their associations (Mann-Whitney test and their 95% confidence intervals was analyzed. RESULTS: The deaf subjects showed poorer performance regarding narrative structure, use of connectives, cohesion measurements and general punctuation (P < 0.05. There were no differences in the number of propositions elaborated or in referent specification between the two groups. The deaf children produced a higher proportion of orientation-related propositions (P = 0.001 and lower proportions of propositions relating to complicating actions (P = 0.015 and character reactions (P = 0.005. CONCLUSION: Hearing-impaired children have abnormalities in different aspects of language, involving form, content and use, in relation to their normal-hearing peers. Narrative competence was also associated with the children's ages and the school type.

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

Science.gov (United States)

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

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

Hearing loss in Usher syndrome type II is nonprogressive.

Science.gov (United States)

Reisser, Christoph F V; Kimberling, William J; Otterstedde, Christian R

2002-12-01

Usher syndrome is an autosomal recessive disorder characterized by sensorineural hearing loss and progressive visual loss secondary to retinitis pigmentosa. In the literature, a possible progression of the moderate to severe hearing loss in Usher syndrome type II (Usher II) is controversial. We studied the development of the hearing loss of 125 patients with a clinical diagnosis of Usher syndrome type II intraindividually and interindividually by repeatedly performing complete audiological and neuro-otologic examinations. Our data show a very characteristic slope of the hearing curve in all Usher II patients and no clinically relevant progression of the hearing loss over up to 17 years. The subjective impression of a deterioration of the communicative abilities of Usher II patients must therefore be attributed to the progressive visual loss. The patients should be reassured that changes in their hearing abilities are unlikely and should be provided with optimally fitted modern hearing aids.

Speech intelligibility of normal listeners and persons with impaired hearing in traffic noise

Science.gov (United States)

Aniansson, G.; Peterson, Y.

1983-10-01

Speech intelligibility (PB words) in traffic-like noise was investigated in a laboratory situation simulating three common listening situations, indoors at 1 and 4 m and outdoors at 1 m. The maximum noise levels still permitting 75% intelligibility of PB words in these three listening situations were also defined. A total of 269 persons were examined. Forty-six had normal hearing, 90 a presbycusis-type hearing loss, 95 a noise-induced hearing loss and 38 a conductive hearing loss. In the indoor situation the majority of the groups with impaired hearing retained good speech intelligibility in 40 dB(A) masking noise. Lowering the noise level to less than 40 dB(A) resulted in a minor, usually insignificant, improvement in speech intelligibility. Listeners with normal hearing maintained good speech intelligibility in the outdoor listening situation at noise levels up to 60 dB(A), without lip-reading (i.e., using non-auditory information). For groups with impaired hearing due to age and/or noise, representing 8% of the population in Sweden, the noise level outdoors had to be lowered to less than 50 dB(A), in order to achieve good speech intelligibility at 1 m without lip-reading.

Hearing Impairment Among Children Referred to a Public Audiology Clinic in Gaborone, Botswana.

Science.gov (United States)

Banda, Francis M; Powis, Kathleen M; Mokoka, Agnes B; Mmapetla, Moalosi; Westmoreland, Katherine D; David, Thuso; Steenhoff, Andrew P

2018-01-01

Objective . To describe and quantify hearing impairment among children referred to the audiology clinic in Princess Marina Hospital, a public referral hospital in Botswana. Methods . In a retrospective case series, we reviewed medical records of children aged 10 years and younger whose hearing was assessed between January 2006 and December 2015 at the audiology clinic of Princess Marina Hospital in Gaborone, Botswana. Results . Of 622 children, 50% were male, and median age was 6.7 years (interquartile range = 5.0-8.3). Hearing impairment was diagnosed in 32% of clinic attendees, comprising sensorineural (23%), conductive (25%), and mixed (11%) hearing loss, while 41% of children with diagnosed hearing impairment did not have a classification type. Hearing impairment was mild in 22.9%, moderate in 22.4%, severe in 19.4%, profound in 16.9%, and of undocumented severity in 18.4%. Children younger than 5 years were 2.7 times (95% confidence interval = 1.29-5.49; P = .008) more likely to be diagnosed with sensorineural hearing impairment compared with those older than 5 years. By contrast, children older than 5 years were 9.6 times (95% confidence interval = 2.22-41.0; P = .002) more likely to be diagnosed with conductive hearing loss compared with those under 5 years. Conclusion . Hearing impairment was common among children referred to this audiology clinic in Botswana. Of those with hearing impairment, more than a third had moderate or severe deficits, suggesting that referrals for hearing assessments are not occurring early enough. Hearing awareness programs individually tailored to parents, educators, and health care workers are needed. Neonatal and school hearing screening programs would also be beneficial.

Newborn hearing screening vs later hearing screening and developmental outcomes in children with permanent childhood hearing impairment

NARCIS (Netherlands)

Korver, Anna M. H.; Konings, Saskia; Dekker, Friedo W.; Beers, Mieke; Wever, Capi C.; Frijns, Johan H. M.; Oudesluys-Murphy, Anne M.; de Vries, Jutte; Vossen, Ann; Kant, Sarina; van den Akker-van Marle, Elske; le Cessie, Saskia; Rieffe, Carolien; Ens-Dokkum, Martina; van Straaten, Irma; Uilenburg, Noelle; Elvers, Bert; Loeber, Gerard; Meuwese-Jongejeugd, Anneke; Maré, Marcel; van Zanten, Bert; Goedegebure, André; Coster, Francien; van Dijk, Pim; Goverts, Theo; Admiraal, Ronald; Cremers, Cor; Kunst, Dirk; de Leeuw, Marina; Dijkhuizen, Janette; Scharloo, Marleen; Hoeben, Dirk; Rijpma, Gerti; Graef, Wim; Linschoten, Dik; Kuijper, Jessica; Hof, Nanda; Koldewijn, Reinoud; Pans, Donné; Jorritsma, Frank; van Beurden, Maarten; ter Huurne, Christien; Brienesse, Patrick; Seekles, Lisanne; de Jong, Jantine; Thijssen, Andrea; Lievense, Andrea; van Egdom-van der Wind, Marina; Theunissen, Stephanie; Mooij, Sophie

2010-01-01

Newborn hearing screening programs have been implemented in many countries because it was thought that the earlier permanent childhood hearing impairment is detected, the less developmentally disadvantaged children would become. To date, however, no strong evidence exists for universal introduction

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

Science.gov (United States)

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.

Teaching Strategies for Economics to the Hearing Impaired | Adu ...

African Journals Online (AJOL)

The study sought to look at the teaching strategies for economics to the hearing impaired. Economics is said to be a living and dynamic subject, which is a vehicle of strict intellectual discipline, that involves looking at the world in a way which is for most, if not all of us quite new. Hearing impairment in a generic term covering ...

Age, duration of work, noise and vibration in inducing hearing and balance impairments

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Jenny Bashiruddin

2005-06-01

Full Text Available Noisy and vibrating bajaj, a public transportation in Jakarta, is a potential risk in inducing hearing and balance problems. Bajaj drivers in the Jakarta area were chosen by consecutive sampling and examined medically in the Neurotological Subdivision of the ENT Department of the Cipto Mangunkusumo Hospital, Jakarta. Hearing and balance impairments were then diagnosed from audiometric and posturographic tests. The study was carried out from March 2000 until October 2001. A number of 350 bajaj drivers participated in this study. There were 97 subjects without hearing and balance impairments, and 96 subjects suffered from hearing and balance impairments. Compared to drivers aged less than 40 years, those aged 41 years or more had a four-fold increased risk of developing hearing and balance impairments [adjusted odds ratio (OR = 3.90; 95% confidence interval (CI = 1.67-9.01. Drivers working 9 hours or more a day had an increased risk 2.3 times of developing hearing and balance impairments compared those working less than 9 hours a day (adjusted OR = 2.32; 95% CI=I.22-4.41. Furthermore, when compared to those who had been working for 1-5 years, those who had been working for 5 years had an increased tendency of developing hearing and balance impairments. Those who had been working 21-30 years had a seven-fold increased risk of developing hearing and balance impairments (adjusted OR = 7.11; 95% CI = 1.88-26.92. To minimize hearing and balance impairments bajaj drivers are recommended to work less than 8 hours a day. (Med J Indones 2005; 14: 101-6Keywords: noise, vibration, hearing, balance impairments, driver

Modeling Speech Intelligibility in Hearing Impaired Listeners

DEFF Research Database (Denmark)

Scheidiger, Christoph; Jørgensen, Søren; Dau, Torsten

2014-01-01

speech, e.g. phase jitter or spectral subtraction. Recent studies predict SI for normal-hearing (NH) listeners based on a signal-to-noise ratio measure in the envelope domain (SNRenv), in the framework of the speech-based envelope power spectrum model (sEPSM, [20, 21]). These models have shown good...... agreement with measured data under a broad range of conditions, including stationary and modulated interferers, reverberation, and spectral subtraction. Despite the advances in modeling intelligibility in NH listeners, a broadly applicable model that can predict SI in hearing-impaired (HI) listeners...... is not yet available. As a firrst step towards such a model, this study investigates to what extent eects of hearing impairment on SI can be modeled in the sEPSM framework. Preliminary results show that, by only modeling the loss of audibility, the model cannot account for the higher speech reception...

The Hearing Outcomes of Cochlear Implantation in Waardenburg Syndrome.

Science.gov (United States)

Koyama, Hajime; Kashio, Akinori; Sakata, Aki; Tsutsumiuchi, Katsuhiro; Matsumoto, Yu; Karino, Shotaro; Kakigi, Akinobu; Iwasaki, Shinichi; Yamasoba, Tatsuya

2016-01-01

Objectives. This study aimed to determine the feasibility of cochlear implantation for sensorineural hearing loss in patients with Waardenburg syndrome. Method. A retrospective chart review was performed on patients who underwent cochlear implantation at the University of Tokyo Hospital. Clinical classification, genetic mutation, clinical course, preoperative hearing threshold, high-resolution computed tomography of the temporal bone, and postoperative hearing outcome were assessed. Result. Five children with Waardenburg syndrome underwent cochlear implantation. The average age at implantation was 2 years 11 months (ranging from 1 year 9 months to 6 years 3 months). Four patients had congenital profound hearing loss and one patient had progressive hearing loss. Two patients had an inner ear malformation of cochlear incomplete partition type 2. No surgical complication or difficulty was seen in any patient. All patients showed good hearing outcome postoperatively. Conclusion. Cochlear implantation could be a good treatment option for Waardenburg syndrome.

The Hearing Outcomes of Cochlear Implantation in Waardenburg Syndrome

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Hajime Koyama

2016-01-01

Full Text Available Objectives. This study aimed to determine the feasibility of cochlear implantation for sensorineural hearing loss in patients with Waardenburg syndrome. Method. A retrospective chart review was performed on patients who underwent cochlear implantation at the University of Tokyo Hospital. Clinical classification, genetic mutation, clinical course, preoperative hearing threshold, high-resolution computed tomography of the temporal bone, and postoperative hearing outcome were assessed. Result. Five children with Waardenburg syndrome underwent cochlear implantation. The average age at implantation was 2 years 11 months (ranging from 1 year 9 months to 6 years 3 months. Four patients had congenital profound hearing loss and one patient had progressive hearing loss. Two patients had an inner ear malformation of cochlear incomplete partition type 2. No surgical complication or difficulty was seen in any patient. All patients showed good hearing outcome postoperatively. Conclusion. Cochlear implantation could be a good treatment option for Waardenburg syndrome.

Incidence of risk factors for hearing impairment in premature babies

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Nikolić Mina

2016-01-01

Full Text Available According to the World Health Organization, the incidence of hearing impairment in newborn population is 1-3 per 1000 (WHO, 2012. Apart from that, many authors have found that the incidence of hearing impairment is twenty times higher, 2-4%, in neonatal intensive care unit (NICU. Thus, a congenital hearing loss is the most frequent sensory or motor deficit that could be diagnosed immediately upon birth. The objective of this study was to determine the incidence of risk factors for hearing impairment in the population of preterm babies. We were especially interested in the impact of gestational age at birth on the incidence of risk factors for hearing loss. A cohort of 150 preterm babies was enrolled in the study. They were hospitalized in the Institute for Neonatology in Belgrade during 2014 and 2015 and the data were obtained from their medical files. The results of this study indicate high incidence of risk factors for hearing impairment in this population of babies. Gestational age at birth had a strong, statistically significant, correlation with risk factor incidence in lower gestational age and vice versa. High incidence of risk factors and their interaction could account for twenty times higher occurrence of congenital and early acquired hearing loss in population of preterm babies compared to term neonates. These results imply the need for systematic audiological surveillance of prematurely born babies at least until 12 months of corrected age.

'Non-vocalization': a phonological error process in the speech of severely and profoundly hearing impaired adults, from the point of view of the theory of phonology as human behaviour.

Science.gov (United States)

Halpern, Orly; Tobin, Yishai

2008-01-01

'Non-vocalization' (N-V) is a newly described phonological error process in hearing impaired speakers. In N-V the hearing impaired person actually articulates the phoneme but without producing a voice. The result is an error process looking as if it is produced but sounding as if it is omitted. N-V was discovered by video recording the speech of two groups, profoundly and severely hearing impaired adults in four elicitation tasks of varying difficulty, and analysing 2065 phonological error processes (substitutions, omissions, and N-V) according to 24 criteria resulting in 49,560 data points. Results, which are discussed in view of the theory 'Phonology as Human Behaviour' (PHB), indicate that: (a) The more communicative the error process was; the more effort was made for its production and the more frequent its distribution; (b) The easier the elicitation task was, the more frequent the use of communicative error processes; c) The more difficult the elicitation task was, the more frequent the use of the relatively less communicative and easier to produce error processes; and d) The process of N-V functioned like a communicative error process for the group of profoundly hearing impaired adults.

Perception Development of Complex Syntactic Construction in Children with Hearing Impairment

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Robab Teymouri

2014-12-01

Full Text Available Objectives: Auditory perception or hearing ability is critical for children in acquisition of language and speech hence hearing loss has different effects on individuals’ linguistic perception, and also on their functions. It seems that deaf people suffer from language and speech impairments such as in perception of complex linguistic constructions. This research was aimed to study the perception of complex syntactic constructions in children with hearing-impairment. Methods: The study design was case-control. According to the inclusion and exclusion criteria, twenty children with severe to profound hearing impairment, aged 8-12 years and twenty normal-hearing children, aged 6-7 years were selected in a simple random sampling from exceptional schools for deaf people and from normal kindergartens and schools for normal cases. The perception of sentences was tested by using a researcher-made task called sentence-picture matching task. At first the content validity was determined and then the reliability was confirmed with Cronbach Alpha Test. Data were analyzed by statistical tests such as Independent Samples T-Test and Mann-Whitney U Test using SPSS. Results: Perception of the group with hearing-impairment was significantly lower than the normal control group. The hearing-impaired children failed to perceive complex syntactic structures. Linguistic function of the group with hearing-impairment on perception of sentences with simple word order was better than on complex sentences. Discussion: If rich linguistic inputs are not available for children during the critical period of the first language acquisition, the syntactic skill, especially in complex syntactic constructions, will not normally develop. In order to establish a foundation for a healthy perfect development of syntax, at the early years of life, children should be exposed to a natural language.

Genetic mutation susceptibility of hearing loss in child with severe neonatal jaundice

International Nuclear Information System (INIS)

Zahedi, F.D.; Rahman, R.A.; Abdullah, A.

2015-01-01

This case report demonstrates a case of 5-year-old non-syndromic Malay boy who passed the hearing screening test however he was confirmed has bilateral profound sensorineural hearing loss diagnosed at 3 months of age by brain stem evoked response (BSER). He has background history of severe neonatal jaundice and male siblings of hearing impairment. The antenatal and birth history was uneventful apart from maternal hypothyroidism. His other two elder brothers have bilateral sensorineural hearing loss and history of severe neonatal jaundice as well. The ear examinations, computed tomography scan and magnetic resonance imaging revealed normal findings. Right sided cochlear implantation was done at the age of 3 years old and he is still under audiology follow-up. Conclusion: Genetic studies are important to determine the cause of genetic mutation in susceptibility to hearing impairment that run in his family after severe neonatal jaundice. Those baby with risk of developing hearing loss required diagnostic hearing assessment. (author)

Performance, fatigue and stress in open-plan offices: The effects of noise and restoration on hearing impaired and normal hearing individuals

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Helena Jahncke

2012-01-01

Full Text Available Hearing impaired and normal hearing individuals were compared in two within-participant office noise conditions (high noise: 60 L Aeq and low noise: 30 L Aeq . Performance, subjective fatigue, and physiological stress were tested during working on a simulated open-plan office. We also tested two between-participants restoration conditions following the work period with high noise (nature movie or continued office noise. Participants with a hearing impairment (N = 20 were matched with normal hearing participants (N = 18 and undertook one practice session and two counterbalanced experimental sessions. In each experimental session they worked for two hours with basic memory and attention tasks. We also measured physiological stress indicators (cortisol and catecholamines and self-reports of mood and fatigue. The hearing impaired participants were more affected by high noise than the normal hearing participants, as shown by impaired performance for tasks that involve recall of semantic information. The hearing impaired participants were also more fatigued by high noise exposure than participants with normal hearing, and they tended to have higher stress hormone levels during the high noise compared to the low noise condition. Restoration with a movie increased performance and motivation for the normal hearing participants, while rest with continued noise did not. For the hearing impaired participants, continued noise during rest increased motivation and performance, while the movie did not. In summary, the impact of noise and restorative conditions varied with the hearing characteristics of the participants. The small sample size does however encourage caution when interpreting the results.

The usefulness of science knowledge for parents of hearing-impaired children.

Science.gov (United States)

Shauli, Sophie; Baram-Tsabari, Ayelet

2018-04-01

Hearing-impaired children's chances of integrating into hearing society largely depend on their parents, who need to learn vast amounts of science knowledge in the field of hearing. This study characterized the role played by science knowledge in the lives of nonscientists faced with science-related decisions by examining the interactions between general science knowledge, contextual science knowledge in the field of hearing, and parents' advocacy knowledge and attitudes. Based on six semi-structured interviews and 115 questionnaires completed by parents of hearing-impaired children, contextual science knowledge emerged as the only predictor for having slightly better advocacy attitudes and knowledge (5.5% explained variance). Although general science knowledge was the best predictor of contextual knowledge (14% of explained variance), it was not a direct predictor of advocacy knowledge and attitudes. Science knowledge plays some role in the lives of hearing-impaired families, even if they do not list it as a resource for successful rehabilitation.

The effectiveness of linguistic plays on the grammatical skills of hearing-impaired children with hearing aids

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Sahar Mohammad Esmaeilzadeh

2014-12-01

Full Text Available Background and Aim: Grammatical skills development of hearing-impaired children depends on using appropriate educational rehabilitation programs. This study aims to investigate the effectiveness of linguistic plays on the grammatical skills in hearing-impaired children with hearing aids.Methods: Ten hearing-impaired children with hearing aids, aged between 5 and 7, were randomly assigned to two groups (5 children in each group. Each treatment group received 12 sessions on linguistic plays. The grammatical skills of these children were evaluated via the TOLD-P: 3 (Persian version; in addition, their level of intelligence was assessed by the Raven test.Results: The difference between the scores of both control and treatment groups revealed a statistically significant difference in grammatical skills (t=7.61, p=0.001 and three subskills of the children who participated in the linguistic plays. These subskills include syntactic understanding (t=3.16, p=0.013, sentence imitation (t=1.71, p=0.006, and morphological completion (t=6.55, p=0.001. In other words, the findings suggest that linguistic plays have a significant impact on the improvement of the aforementioned skills in hearing-impaired children.Conclusion: Results suggest that it would be beneficial to include linguistic plays as part of routine rehabilitation programs as a means of improving the grammatical difficulties of children. After partaking in linguistic plays, children significantly improved their ability to comprehend the meaning of sentences and also to recognize, understand, and use common Persian morphological forms.

Childhood Hearing Impairment: How Do Parents Feel About It ...

African Journals Online (AJOL)

-Briggs. Abstract. Background Hearing impairment or deafness is a major disabling condition worldwide. Etiology of the hearing loss range from congenital to acquired, and includes common and preventable childhood infections like otitis ...

Early Detection of Hearing Impairment Among High Risk Neonates in a Tertiary Care Hospital

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Gurudutt Joshi

2014-07-01

Full Text Available Background: Hearing impairment has a devastating, detrimental and an invariably adverse impact on the development of the newborns and the psychological well-being of their families. It also adversely affects development of the central auditory nervous system, and can affect speech perception that interferes with growth in social, emotional, behavioural and cognitive spheres, academic achievement, vocational options, employment opportunities and economic selfsufficiency. Objectives: To find out incidence of hearing impairment in high risk neonates in Neonatal Intensive Care Unit (NICU, prevalence of hearing impairment with and without high risk factors in newborns and to correlate the risk factors with hearing impairment. Material and Methods: A cohort study was carried out at a tertiary care hospital of Surat, Gujarat, India consisting of 190 normal newborns and 163 newborns with high risk factors. These newborns underwent a systematized Transient Otoacoustic Emissions Examination (TOAE and Brain Stem Evoked Audiometry (BERA examination according to designed protocol and were followed up with repeated ear examinations. Data were recorded and analyzed statistically. Results: The incidence of hearing impairment in NICU, newborns were 3.6% and the prevalence of hearing impairment was 13%. Hearing impairment was statistically significant in newborns with high risk factors such as low birth weight, preterms 5 days when compared to normal newborns. Conclusion: Presence of risk factors in newborns predisposes them to hearing impairment more as compared to normal newborns and the more the number of risk factors they are exposed to, the more will be the chances of hearing impairment.

Externalization versus Internalization of Sound in Normal-hearing and Hearing-impaired Listeners

DEFF Research Database (Denmark)

Ohl, Björn; Laugesen, Søren; Buchholz, Jörg

2010-01-01

The externalization of sound, i. e. the perception of auditory events as being located outside of the head, is a natural phenomenon for normalhearing listeners, when perceiving sound coming from a distant physical sound source. It is potentially useful for hearing in background noise......, but the relevant cues might be distorted by a hearing impairment and also by the processing of the incoming sound through hearing aids. In this project, two intuitive tests in natural real-life surroundings were developed, which capture the limits of the perception of externalization. For this purpose...

Characteristics of reading and understanding of hearing impaired students in classes VI-VIII

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Mustaf Morina

2015-03-01

Full Text Available Good reading has a very important role in the development of children with hearing impaired; also reading in explicit way is one of the crucial factors which affect the oral language development of children with hearing impaired. The best form and possibility of improvement, development of oral language, development of communicating, receipt of information, knowledge, and ideas over the world, is reading. When the auditory perception is damaged reading is poor. Hearing impairment causes a lot of problems in the development of personality of children with hearing impairment in these fields: poor development of vocabulary, poor quality of lexica, poor quality of sentences, and disorder in articulation. The purpose of this research is to verify the following: 1-Speed of reading of hearing impaired children, 2-The number of errors, 3-The kind of errors, 4-To understand the text in the context of the degree of hearing impairment, age (class, success in school and gender. This theoretical-experimental study was made with students from two schools; special school “Mother Teresa” in Prizren and Primary School “Elena Gjika” in Prishtina (class attached. The research included a total of 32 students (respondent 27 students (respondent from special schools “Mother Teresa” in Prizren and 5 elementary school students “Elena Gjika” Prishtina, all these students are with hearing impairment. From 32 students involved in the research, 23 were male and 9 female. The research was done by applying a text fables “The fox and the raven” watched and analyzed in terms of three dimensions. The research results have shown that students with hearing impairments have considerable problems in many aspects; in terms of speed of reading, students with hearing impairment have stagnated compared with their peers in the ratio 8/1. In terms of reading errors have stagnated considered being incomparable. In terms of understanding the text students with hearing

Modeling auditory perception of individual hearing-impaired listeners

DEFF Research Database (Denmark)

Jepsen, Morten Løve; Dau, Torsten

showed that, in most cases, the reduced or absent cochlear compression, associated with outer hair-cell loss, quantitatively accounts for broadened auditory filters, while a combination of reduced compression and reduced inner hair-cell function accounts for decreased sensitivity and slower recovery from...... selectivity. Three groups of listeners were considered: (a) normal hearing listeners; (b) listeners with a mild-to-moderate sensorineural hearing loss; and (c) listeners with a severe sensorineural hearing loss. A fixed set of model parameters were derived for each hearing-impaired listener. The simulations...

Working memory and novel word learning in children with hearing impairment and children with specific language impairment.

Science.gov (United States)

Hansson, K; Forsberg, J; Löfqvist, A; Mäki-Torkko, E; Sahlén, B

2004-01-01

Working memory is considered to influence a range of linguistic skills, i.e. vocabulary acquisition, sentence comprehension and reading. Several studies have pointed to limitations of working memory in children with specific language impairment. Few studies, however, have explored the role of working memory for language deficits in children with hearing impairment. The first aim was to compare children with mild-to-moderate bilateral sensorineural hearing impairment, children with a preschool diagnosis of specific language impairment and children with normal language development, aged 9-12 years, for language and working memory. The special focus was on the role of working memory in learning new words for primary school age children. The assessment of working memory included tests of phonological short-term memory and complex working memory. Novel word learning was assessed according to the methods of. In addition, a range of language tests was used to assess language comprehension, output phonology and reading. Children with hearing impairment performed significantly better than children with a preschool diagnosis of specific language impairment on tasks assessing novel word learning, complex working memory, sentence comprehension and reading accuracy. No significant correlation was found between phonological short-term memory and novel word learning in any group. The best predictor of novel word learning in children with specific language impairment and in children with hearing impairment was complex working memory. Furthermore, there was a close relationship between complex working memory and language in children with a preschool diagnosis of specific language impairment but not in children with hearing impairment. Complex working memory seems to play a significant role in vocabulary acquisition in primary school age children. The interpretation is that the results support theories suggesting a weakened influence of phonological short-term memory on novel word

Biochemical alteration in children with idiopathic nephrotic syndrome associated with an increased risk of sensorineural hearing loss; additional insights in cochlear renal relationship.

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El Mashad, Ghada Mohamed; Abo El Fotoh, Wafaa Moustafa M; Zein El Abedein, Ahmed Mahmoud; Abd El Sadek, Fatma Abd El Raoof

2017-06-01

Children with Idiopathic Nephrotic Syndrome (INS) are at risk of hearing loss due to the adverse impact of medications and related immunological and genetic factors on both cochlea and kidney. So this work was planned to evaluate hearing status in children with INS and to clarify the possible associated risk factors by interpreting the clinical and laboratory profiles of those children. Ninety children with INS aged 5-14 years [30 patients with steroid-sensitive nephrotic syndrome (SSNS), 30 patients with steroid dependent/frequently relapsing nephrotic syndrome (SDNS/FRNS), and 30 patients with steroid-resistant nephrotic syndrome (SRNS)], and 90 age and sex matched normal controls were enrolled into this study. Laboratory measurements of serum calcium, creatinine, cholesterol, blood urea and other relevant investigations were done. Pure tone audiometry was done with the sensory-neural hearing loss (SNHL) diagnosed when the level bone conduction was >20 dB and the difference in air to the bone gap was children with INS had SNHL, mostly of mild degree HL and primarily occurred at the lower frequencies. A highly significant statistical difference between controls and various types of nephrotic syndrome regarding pure tone audiometry measurements at frequencies 250, 500, 1000 Hz, whereas insignificant difference interpreting pure tone audiometry measurements in 2000, 4000 and 8000 Hz. Children with different phenotypes of nephrotic syndrome are at risk of sensorineural hearing impairment. The hazards associated with this impairment were higher blood pressure, hypercholesterolemia, hypoalbuminemia, and hypocalcemia. Copyright © 2017 Elsevier B.V. All rights reserved.

Age-related Hearing Impairment and the Triad of Acquired Hearing Loss

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Chao-Hui eYang

2015-07-01

Full Text Available 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 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.

Severe hearing impairment among military veterans--United States, 2010.

Science.gov (United States)

2011-07-22

A substantial proportion of hearing loss in the United States is attributable to employment-related exposure to noise. Among military veterans, the most common service-connected disabilities are hearing impairments, suggesting that occupational noise exposure during military service might cause more veterans to have hearing loss than nonveterans. However, a recent analysis of data from the 1993-1995 Epidemiology of Hearing Loss Study did not find significant differences between the two groups. To further investigate hearing loss among veterans, specifically the prevalence of severe hearing impairment (SHI), data from the 2010 Annual Social and Economic Supplement (ASEC) to the Current Population Survey (CPS) were analyzed. This report describes the results of those analyses, which indicated that the prevalence of SHI among veterans was significantly greater than among nonveterans. Veterans were 30% more likely to have SHI than nonveterans after adjusting for age and current occupation, and veterans who served in the United States or overseas during September 2001-March 2010, the era of overseas contingency operations (including Operations Enduring Freedom and Iraqi Freedom), were four times more likely than nonveterans to have SHI. These findings suggest a need for increased emphasis on improving military hearing conservation programs (HCPs) and on hearing loss surveillance in military and veterans' health systems.

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

Science.gov (United States)

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.

Hearing Impairment Caused by Occupational Noise | Mets | South ...

African Journals Online (AJOL)

Occupational noise-induced hearing impairment is an insidiously developing injury which only becomes apparent when it affects the hearing of conversational speech. As no remedy is possible, prevention is the only answer. In view of the impending legislation in South Africa a review of the literature is presented. This is ...

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

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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…

Study of the knowledge of Pediatricians and Senior Residents Relating to the Importance of Hearing Impairment and Deafness Screening Among Newborns in Isfahan city in 2012

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Mehrdad Rogha

2014-04-01

Full Text Available Introduction: Newborn hearing screening leads to the early detection of hearing impairment. The aim of screening is to decrease or remove the effect of hearing impairment on development of speech and language by timely diagnosis and effective treatment. A number of risk factors lead to delayed start of decreased hearing ability including: 1. Congenital infection with cytomegalovirus  (CMV virus, 2. Meningitis, 3. Mumps, 4. Positive family history, 5. Head trauma, 6. Chemotherapy, 7. Syndrome pertaining to delayed start of decreased hearing. Unfortunately, lack of attention to early diagnosis of hearing impairment is becoming a general health problem. No research has yet been carried out relating to the knowledge of pediatricians on this issue, particularly the importance of hearing impairment and hearing screening. The aim of this study was to determine the attitude to newborn hearing screening among pediatricians.   Materials and Methods: This cross-sectional, descriptive-analytic study was conducted in Isfahan in 2012 among 300 pediatricians and final-year pediatric residents. An adjusted 22-question version of the Early Hearing Detection and Intervention (EHDI questionnaire was used to collect data. The validity and reliability of the EHDI questionnaire was previously demonstrated by Boys Town National Research Hospital and its Farsi translated version was validated by the EDC Center at the Isfahan University of Medical Sciences.   Results: In our study, 83% of pediatricians agreed on the importance of hearing impairment screening for all infants. However 65% were not aware of special needs for hearing-impaired patients.   Conclusion:  Newborn hearing impairment and deafness screening is important, irrespective of the costs, and lack of timely diagnosis results in both individual and social consequences. The majority of physicians use textbooks to gain information about hearing screening, but recognize that this is insufficient. Although

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

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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, pimpairment 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

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

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Chunfeng Yun

2017-01-01

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

Molecular Investigation of Pediatric Portuguese Patients with Sensorineural Hearing Loss

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Célia Nogueira

2011-01-01

Full Text Available The understanding of the molecular genetics in sensorineural hearing loss (SNHL has advanced rapidly during the last decade, but the molecular etiology of hearing impairment in the Portuguese population has not been investigated thoroughly. To provide appropriate genetic testing and counseling to families, we analyzed the whole mitochondrial genome in 95 unrelated children with SNHL (53 nonsyndromic and 42 syndromic and searched for variations in two frequent genes, GJB2 and GJB6, in the non-syndromic patients. Mutations in mtDNA were detected in 4.2% of the cases, including a hitherto undescribed change in the mtDNA-tRNATrp gene (namely, m.5558A>G. We also identified mono- or biallelic GJB2 mutations in 20 of 53 non-syndromic cases and also detected two novel mutations (p.P70R and p.R127QfsX84. Our data further reinforce the notion that genetic heterogeneity is paramount in children with SNHL.

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

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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.

Hearing-impaired child at the pediatrician office from a phoniatrician‘s point of view

International Nuclear Information System (INIS)

Myska, P.

2014-01-01

Early diagnosis of hearing impairment is of crucial importance in a child’s life. For this reason, a pediatrician should perform a hearing exam, along with other necessary specialised tests, immediately after he/she had identified risks of hearing impairment in the newborn‘s anamnesis or had ben warned by the parents about a possible problem. Both the diagnosis of congenital or acquired hearing impairment after birth, and the installment of hearing aids, should take place by the end of the first year in a child’s life at the latest. (author)

Speech understanding and directional hearing for hearing-impaired subjects with in-the-ear and behind-the-ear hearing aids

NARCIS (Netherlands)

Leeuw, A. R.; Dreschler, W. A.

1987-01-01

With respect to acoustical properties, in-the-ear (ITE) aids should give better understanding and directional hearing than behind-the-ear (BTE) aids. Also hearing-impaired subjects often prefer ITEs. A study was performed to assess objectively the improvement in speech understanding and directional

Genetic Linkage Analysis of DFNB2 Locus with Autosomal Recessive Hearing Loss in Families Negative for GJB2 Mutations in Khuzestan Province

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Parisa Tahmasebi

2016-09-01

Full Text Available Abstract Background: Hearing loss is a common sensory impairment in humans which half of its causes are genetic reasons. Genetic hearing loss can be divided into the two types of syndromic and non-syndromic, which 80% of non-syndromic cases is Autosomal Recessive Non-Syndromic Hearing Loss. The aim of the present research is to determine the contribution of DFNB2 locus (MYO7A gene in causing an autosomal recessive hearing loss in the one group of the deaf families of Khuzestan province. Materials and Methods: This study was conducted on 26 families with autosomal recessive hearing loss (with 4 patients and negative for GJB2 mutations in Khuzestan province. 22 families suffered from ARNSHL and 4 families suffered from Usher syndrome. Linkage analysis was performed by using STR (Short Tandem Repeat markers related to DFNB2 locus. Each family’s genotype was determined by PCR-PAGE method. Furthermore, haplotypes drawing and LOD score calculations were performed. Results: From 26 families with hearing loss participating in this research, following genetic linkage analysis and haplotypes drawing, two families (7.7% of the families showed linkage to DFNB2 locus. One family (4.5% suffered from ARNSHL and another family suffered from Usher syndrome. Conclusion: The results of the present research show that the contribution of DFNB2 locus in causing hearing loss in the population of Khuzestan province was similar to other studies conducted in Iran and this locus with other important loci should be considered to check in the hearing loss panel.

Exploring the Correlates of Impaired Non-Word Repetition in Down Syndrome

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Cairns, Peter; Jarrold, Christopher

2005-01-01

Non-word repetition, in which participants hear and repeat unfamiliar verbal stimuli, is thought to provide a particularly sensitive measure of verbal short-term memory capacity. However, performance on this task can also be constrained by hearing and speech production skills, and by an individuals' linguistic knowledge. This study examined real…

TELEGRAM: contribution in assistive technology indication for individuals with hearing impairment.

Science.gov (United States)

Jacob, Regina Tangerino de Souza; Lopes, Natália Barreto Frederigue; Cruz, Aline Duarte da; Alves, Tacianne Kriscia Machado; Santos, Larissa Germiniani Dos; Angelo, Thais Corina Said de; Mondelli, Maria Fernanda Capoani Garcia; Moret, Adriane Lima Mortari

2017-02-23

The objective of the study was to translate and culturally adapt to Portuguese the TELEGRAM instrument and to evaluate its effectiveness in adults with hearing impairment using hearing aids. The TELEGRAM was translated into the Portuguese language, reviewed for grammatical and idiomatic equivalences (reverse translations) and linguistic and cultural adaptations. After translation, the TELEGRAM was applied to 20 individuals with hearing impairment. A descriptive analysis of the results was performed. After the grammatical and idiomatic equivalence, the replacement of one term/item was suggested, which was modified and adapted to the Brazilian context. In general, the questions of the instrument were considered easy to understand. Among the categories assessed, individuals with hearing loss had greater difficulty using the telephone and in activities such as attending church gatherings, parties, or in situations of noisy environments, distance and reverberation. The TELEGRAM translated into Brazilian Portuguese proved to be an easily applicable tool in population studies and effective to assess which are the main situations where individuals with hearing impairment have greater difficulty in communication, reinforcing the importance of hearing rehabilitation and assistive technology to minimize these difficulties.

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

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

2015-11-01

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

Overview of a public health approach to pediatric hearing impairment in the Pacific Islands.

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Kaspar, Annette; Kei, Joseph; Driscoll, Carlie; Swanepoel, De Wet; Goulios, Helen

2016-07-01

Childhood hearing impairment is a significant cause of disability in developing countries. Otitis media and meningitis are leading infectious causes of preventable hearing loss in children. It is estimated that the Pacific Islands have among the greatest global burden of childhood hearing impairment due to infectious causes, and yet there is currently very little in the research literature on pediatric hearing disorders in this region. (1) To review existing research literature on pediatric hearing impairment in the Pacific Islands, and (2) to present a public health approach to the development and improvement of childhood hearing services in the Pacific Islands. The primary tool was a comprehensive literature review. MEDLINE and ScienceDirect databases were searched for relevant journal articles. There was no limit on the date of publication. Any article reporting on hearing impairment in the Pacific Region was included. A total of 23 journal articles were found that satisfied the above inclusion criteria. The limited information available in the literature suggests that otitis media and vaccine-preventable infections are a significant cause of avoidable childhood hearing impairment in the Pacific Islands. Pediatric audiology services are limited in this region. Further research is required to develop effective public health programs that should reduce the burden of preventable childhood hearing loss in the Pacific Islands. There is limited information in the research literature on pediatric hearing impairment and audiology services in the Pacific Islands. Epidemiological data based on the WHO Ear and Hearing Disorders Survey Protocol are urgently needed, and the development of audiology services within the existing public and primary health care framework should reduce the burden of preventable hearing loss in the Pacific Islands. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Perceived competence and school adjustment of hearing impaired children in mainstream primary school settings.

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Hatamizadeh, N; Ghasemi, M; Saeedi, A; Kazemnejad, A

2008-11-01

Although educational main streaming of children with special needs formally began in Iran since 1992 there is little information whether hearing impaired children feel competent in regular schools. To determine the perceived competence and school adjustment of hearing impaired children in mainstream primary school settings, the self-perception profile was administered to 60 mainstreamed hard of hearing children and 60 classmates with normal hearing matched for gender by a single interviewer. The instrument comprised 28 items, 23 of which were similar to those of 'adapted test Image for children with cochlear implants' asking children about their feelings about their own cognitive, physical, socio-emotional and communication competence and school adjustment. The Cronbach alpha coefficient for the instrument was 0.93. Hard of hearing children rated their competence significantly poorer than their hearing classmates for all domains. Mean differences for the five domains ranged from 0.48 (for physical competence) to 0.90 (for school adjustment) on a scale of 1-4. There were no significant differences between girls' and boys' competence, in either the hearing or the hearing impaired groups. Classifying overall scores for perceived competence into four groups ('poor competence', 'low competence', 'moderate competence' and 'high competence'), 23.4% of hearing impaired children but none of the hearing classmates rated themselves as having low or poor competence. On the other hand 85% of hearing children and only 18.3% of hearing impaired children rated themselves as highly competent. We suggest that periodical assessments of mainstreamed children might help to identify those children who are having difficulty adapting to their environment.

Elements of museum mobile augmented reality for engaging hearing impaired visitors

Science.gov (United States)

Baker, Esraa Jaffar; Bakar, Juliana Aida Abu; Zulkifli, Abdul Nasir

2017-10-01

Nowadays, designers are more concern with the issue of engagement and informal learning at museum and gallery sites. This has made studies to focus more on the use of Mobile Augmented Reality (MAR) at museum and gallery sites. However, most of the MAR applications for museum visitors are largely tailored to normal hearing visitors while the hearing-impaired (HI) visitors are not supported. The hearing impaired (HI) community account for over 5% of the world's populace which is about 360 million people. Thus, this paper explores the design elements of mobile augmented reality for engaging hearing impaired visitors at the museum site. The findings of this paper argues that there are eleven major elements of engagement of MAR needed for the design of an efficient museum MAR app for hearing impaired visitors. These eleven elements include Aesthetics, Curiosity, Usability, Interaction, Motivation, Satisfaction, Self-Efficacy, Perceived Control, Enjoyment, Focused Attention and Interest. This study pointed out that for an efficient and engaged MAR app for the HI community especially HI visitors to museum sites, these eleven elements are critical. This finding will help MAR designers and developers on how to design an efficient and engaged MAR app for the HI community at large and museum HI visitors specifically.

The Perception and Decoding of Expressive Emotional Information by Hearing and Hearing-Impaired Children.

Science.gov (United States)

Sanders, Gina

1985-01-01

Hearing and hearing-impaired children between ages 4.5 to 15.5 years in England and Belgium were invited to abstract the concept of emotion from photographs and line drawings of facial expressions and body postures. A further experiment isloated the element of context in the task of decoding expression of emotion, resulting in comparatively…

Rotatory and collic vestibular evoked myogenic potential testing in normal-hearing and hearing-impaired children.

Science.gov (United States)

Maes, Leen; De Kegel, Alexandra; Van Waelvelde, Hilde; Dhooge, Ingeborg

2014-01-01

Vertigo and imbalance are often underestimated in the pediatric population, due to limited communication abilities, atypical symptoms, and relatively quick adaptation and compensation in children. Moreover, examination and interpretation of vestibular tests are very challenging, because of difficulties with cooperation and maintenance of alertness, and because of the sometimes nauseatic reactions. Therefore, it is of great importance for each vestibular laboratory to implement a child-friendly test protocol with age-appropriate normative data. Because of the often masked appearance of vestibular problems in young children, the vestibular organ should be routinely examined in high-risk pediatric groups, such as children with a hearing impairment. Purposes of the present study were (1) to determine age-appropriate normative data for two child-friendly vestibular laboratory techniques (rotatory and collic vestibular evoked myogenic potential [cVEMP] test) in a group of children without auditory or vestibular complaints, and (2) to examine vestibular function in a group of children presenting with bilateral hearing impairment. Forty-eight typically developing children (mean age 8 years 0 months; range: 4 years 1 month to 12 years 11 months) without any auditory or vestibular complaints as well as 39 children (mean age 7 years 8 months; range: 3 years 8 months to 12 years 10 months) with a bilateral sensorineural hearing loss were included in this study. All children underwent three sinusoidal rotations (0.01, 0.05, and 0.1 Hz at 50 degrees/s) and bilateral cVEMP testing. No significant age differences were found for the rotatory test, whereas a significant increase of N1 latency and a significant threshold decrease was noticeable for the cVEMP, resulting in age-appropriate normative data. Hearing-impaired children demonstrated significantly lower gain values at the 0.01 Hz rotation and a larger percentage of absent cVEMP responses compared with normal-hearing children

Effects of self-reported hearing or vision impairment on depressive symptoms: a population-based longitudinal study.

Science.gov (United States)

Han, J H; Lee, H J; Jung, J; Park, E-C

2018-02-08

The aims of this study were to investigate the effects of either hearing, vision or dual sensory impairment on depressive symptoms and to identify subgroups that are vulnerable and significantly affected. Data from the 2006-2014 Korean Longitudinal Study of Aging (KLoSA) were used and a total of 5832 individuals were included in this study. Depressive symptoms were assessed using the Center for Epidemiologic Studies Depression (CES-D10) scale. Sensory impairment was assessed according to the levels of self-reported hearing or vision, which were categorised as either good (excellent, very good or good) or poor (fair or poor). The changes in hearing or vision from records of previous survey were investigated. Changes from good to poor, which indicates new onset, were defined as hearing impairment or vision impairment. Interactions of changes in hearing and vision were considered in the analysis. Dual sensory impairment was indicated when hearing impairment and vision impairment both developed at the same time. Demographic, socioeconomic and health-related factors were considered as potential confounders and were adjusted for in the generalised estimating equation model. Individuals with hearing impairment demonstrated significantly more severe depressive symptoms [β = 0.434, standard errors (s.e.) = 0.097, p impairment also showed significantly elevated depressive symptoms (β = 0.253, s.e. = 0.058, p impairment showed significantly more severe depressive symptoms (β = 0.768, s.e. = 0.197, p impairment on depressive symptoms was significant in both sexes and across age groups, except for vision impairment in male participants. Hearing, vision and dual sensory impairment are significantly associated with depressive symptoms. Our results suggest that treatment or rehabilitation of either hearing or vision impairment would help prevent depression.

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

Science.gov (United States)

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…

The effect of hearing aid noise reduction on listening effort in hearing-impaired adults.

Science.gov (United States)

Desjardins, Jamie L; Doherty, Karen A

2014-01-01

The purpose of the present study was to evaluate the effect of a noise-reduction (NR) algorithm on the listening effort hearing-impaired participants expend on a speech in noise task. Twelve hearing-impaired listeners fitted with behind-the-ear hearing aids with a fast-acting modulation-based NR algorithm participated in this study. A dual-task paradigm was used to measure listening effort with and without the NR enabled in the hearing aid. The primary task was a sentence-in-noise task presented at fixed overall speech performance levels of 76% (moderate listening condition) and 50% (difficult listening condition) correct performance, and the secondary task was a visual-tracking test. Participants also completed measures of working memory (Reading Span test), and processing speed (Digit Symbol Substitution Test) ability. Participants' speech recognition in noise scores did not significantly change with the NR algorithm activated in the hearing aid in either listening condition. The NR algorithm significantly decreased listening effort, but only in the more difficult listening condition. Last, there was a tendency for participants with faster processing speeds to expend less listening effort with the NR algorithm when listening to speech in background noise in the difficult listening condition. The NR algorithm reduced the listening effort adults with hearing loss must expend to understand speech in noise.

Health related quality of life in parents of children with speech and hearing impairment.

Science.gov (United States)

Aras, Ivana; Stevanović, Ranko; Vlahović, Sanja; Stevanović, Siniša; Kolarić, Branko; Kondić, Ljiljana

2014-02-01

Hearing impairment and specific language disorder are two entities that seriously affect language acquisition in children and reduce their communication skills. These children require specific treatment and higher levels of care than healthy children. Their language abilities also strongly influence parent-child interactions. The purpose of our study was to evaluate the health-related quality of life (HRQOL) of the parents of hearing-impaired children and the parents of children with speech difficulties (specific language disorder). Our study subjects included 349 parents (182 mothers and 167 fathers) of preschool-aged children with receptive expressive language disorder and 131 parents (71 mothers and 60 fathers) of children with severe hearing impairment. A control group was composed of 146 parents (82 mothers and 64 fathers) of healthy children of the same age. HRQOL was assessed using the SF-36 questionnaire. For all groups of parents, the mothers had poorer scores compared with the fathers, but large differences were apparent depending on the child's impairment. In the control group, the scores of the mothers were significantly lower than the fathers' scores in only two (of eight) health domains. In contrast, the scores were lower in three domains for the mothers of speech-impaired children and in six domains for the mothers of hearing-impaired children, representing the greatest difference between the parents. When compared with the control group, both the mothers and fathers of speech-impaired children scored significantly worse in five health domains. Fathers of hearing-impaired children scored significantly worse than controls in three health domains. The lowest scores, indicating the poorest HRQOL, were observed for mothers of hearing-impaired children, who obtained significantly lower scores than the control mothers in all health domains except the emotional role. The parents of preschool-aged speech-and hearing-impaired children experience poorer HRQOL

Causes of permanent childhood hearing impairment

NARCIS (Netherlands)

Korver, Anna M. H.; Admiraal, Ronald J. C.; Kant, Sarina G.; Dekker, Friedo W.; Wever, Capi C.; Kunst, Henricus P. M.; Frijns, Johan H. M.; Oudesluys-Murphy, Anne Marie; Oudesluys-Murphy, A. M.; Korver, A. M. H.; Frijns, J. H. M.; Wever, C. C.; Konings, S.; Beers, W.; Dekker, F. W.; de Vries, J. J. C.; Vossen, A. C. T. M.; Kant, S. G.; van den Akker-van Marle, M. E.; Rieffe, C.; Ens-Dokkum, M. H.; van Straaten, H. L. M.; Uilenburg, N. N.; Elvers, B.; Loeber, G.; Meuwese-Jongejeugd, J.; Maré, M. J.; Van Zanten, G. A.; Goedegebure, A.; Coster, F.; Goverts, S. T.; Admiraal, R. J. C.; Cremers, C. W. R. J.; Kunst, H. P. M.; de Leeuw, M.; Dijkhuizen, J.; Scharloo, M.; Hoeben, D.; Rijpma, G.; Graef, W.; Linschoten, D.; Kuijper, J.; Hof, N. J.; Pans, D.; Jorritsma, F.; van Beurden, M.; ter Huurne, C. T.; Brienesse, P.; Koldewijn, G. J.; Letourneur, K. G.

2011-01-01

The causes of Permanent Childhood Hearing Impairment (PCHI) are often quoted as being hereditary in 50%, acquired in 25%, and unknown in 25% of cases. Interest in the causes of PCHI has grown recently due to increasing diagnostic possibilities. We investigated the evidence for the reported

Causes of permanent childhood hearing impairment

NARCIS (Netherlands)

Korver, A.M.; Admiraal, R.J.C.; Kant, S.G.; Dekker, F.W.; Wever, C.; Kunst, H.P.M.; Frijns, J.H.; Oudesluys-Murphy, A.M.

2011-01-01

INTRODUCTION: The causes of Permanent Childhood Hearing Impairment (PCHI) are often quoted as being hereditary in 50%, acquired in 25%, and unknown in 25% of cases. Interest in the causes of PCHI has grown recently due to increasing diagnostic possibilities. We investigated the evidence for the

The comparison of stress and marital satisfaction status of parents of hearing-impaired and normal children

Directory of Open Access Journals (Sweden)

Karim Gharashi

2013-03-01

Full Text Available Background and Aim: Stress is the source of many problems in human-kind lives and threatens people's life constantly. Having hearing-impaired child, not only causes stress in parents, but also affects their marital satisfaction. The purpose of this study was comparing the stress and marital satisfaction status between the normal and hearing-impaired children's parents.Methods: This was a causal-comparative study. Eighty parents of normal children and 80 parents of hearing-impaired children were chosen from rehabilitation centers and kindergartens in city of Tabriz, Iran by available and clustering sampling method. All parents were asked to complete the Friedrich's source of stress and Enrich marital satisfaction questionnaires.Results: Parents of hearing-impaired children endure more stress than the normal hearing ones (p<0.001. The marital satisfaction of hearing-impaired children's parents was lower than the parents of normal hearing children, too (p<0.001.Conclusion: Having a hearing-impaired child causes stress and threatens the levels of marital satisfaction. This requires much more attention and a distinct planning for parents of handicap children to reduce their stress.

Childhood Hearing Impairment: How do Parents Feel about it?

African Journals Online (AJOL)

DATONYE ALASIA

burden of hearing impairment on the children and their ... The Nigerian Health Journal, Vol. 12, No 4 ... certain cultural misconception and. 9,10 ... school. Information collected included parents age, sex and questions related to the perception ... routine care needed to avoid hearing loss due to. 15 ... Mental health: New.

Noise-Induced Hearing Impairment As An Occupational Risk Factor ...

African Journals Online (AJOL)

Noise pollution in workplaces poses serious health risks including that of cardiovascular disturbances and impairment of hearing. The objective of this study was to assess the effects of occupational noise on hearing among selected industrial workers in Benin City, Nigeria. Male and female workers (n=150) in sawmills, ...

Group Communication Training for Young People with Combined Visual and Hearing Impairments

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Khokhlova A. Yu.

2018-04-01

Full Text Available The article presents the experience of the communication training for young people with visual and hearing impairments. Boys and girls aged 16–25 with simultaneous hearing and visual impairments of varying severity took part in the group trainings. The variety of means of communication used by them described, conditions of effective training work outlined. The results showed that young people with visual and hearing impairments demonstrate a fairly high level of possession of various means of communication without pronounced additional violations. Communicative needs and preferences in young people with visual and hearing impairments are age-appropriate. Communication training allows the following: to eliminate some of the objective communicative difficulties which are exists in deaf-blind people, to motivate participants to show initiative in communication, to learn new about each other. Also communicative training creates a positive experience of communication with a wider range of people. The most important result is the opportunity to talk about ones feelings in a supportive atmosphere.

The communicative performance of a severely hearing-impaired adolescent

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Ann Russel

1981-11-01

Full Text Available This study describes the communicative performance of a severely hearing-impaired adolescent.The experimenter taught the subject how to play Russian Backgammon. The subject conversed with, and afterwards taught his mother, speech therapist, and a peer how to play the game. Each dyad played the game once. Videotape recordings were made of each dyadic situation. The channels of communication, both verbal and nonverbal, used by each speaker, were determined. A relational communication coding scheme, involving the analysis of requests and subsequent responses, was applied to the data. Results indicate that the hearing-impaired adolescent, though not always able to hold a dominant position in a dyadic situation, was capable of expressing the same types of control as normal adults. Moreover, the types of control expressed varied as a function of each contextual setting. Whenever the subject did hold a dominant position, the combined verbal plus nonverbal channel was his predominant mode of  communication. These findings  suggest that a sociolinguistic approach provides important information regarding a hearing-impaired adolescent's communicative performance.

Stress coping strategies in hearing-impaired students

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Bahman Akbari

2013-03-01

Full Text Available Background and Aim: The majority of people experience problems and stressors, such as job layoffs and illnesses during their lives. However, the way people cope with stress varies. According to previous research, use of effective coping strategies can significantly reduce stress and tension. The aim of the present study was to investigate the effectiveness of stress coping strategies on hearing-impaired students.Methods: This is a quasi-experimental study with pre-test, post-test, and control group. The sample consisted of 40 hearing-impaired male students of guidance and high schools of the city of Sari, Iran, in the years 2010 and 2011. They were randomly selected and assigned to two experimental and control groups (n=20. The data collection tool was the General Health Questionnaire-28. The experimental group was administered eight sessions of stress coping strategies based on cognitive-behavioral techniques two hours weekly. Data were analyzed using statistical indices including mean, standard deviation, and two-way analysis of variance.Results: There was a significant difference between the pre-test and post-test scores in the subscales of somatic symptoms, anxiety and insomnia, depression, and social function (p<0.05. Moreover, after the eight sessions the rate of general health of the experimental group was higher than the control group.Conclusion: Stress coping strategies based on cognitive-behavioral techniques increased the general health of hearing-impaired people. Therefore, stress management training should be developed and expanded as an appropriate intervention.

Comparison of Reading Comprehension Skill of Students with Severe to Profound Hearing Impairment from Second up to Fifth Grade of Exceptional Schools with Normal Hearing Students

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Maryam Jalalipour

2016-03-01

Full Text Available Background: Reading is known as one of the most important learning tools. Research results consistently have shown that even a mild hearing impairment could affect the reading skills. Due to the reported differences in reading comprehension skills between hearing impaired students and their normal hearing peers, this research was conducted to compare the differences between the two groups. The other aim was to find any changes in the reading ability of hearing impaired group during elementary school. Methods: This study is a cross-sectional (descriptive–analytic one in which reading comprehension ability of 91 students with severe and profound hearing impairment (33 girls and 58 boys from 2nd up to 5th grade of exceptional schools were compared with 50 2nd grade normal hearing students in Ahvaz, Iran. The first section of Diagnostic Reading Test (Shirazi – Nilipour, 2004 was used in this study. Then the mean reading scores of hearing impaired students in each grade was compared with control group using SPSS 13 with Mann Whitney test. Results: There was a significant difference between average scores of hearing impaired students (boys and girls in 2nd to 5th grade with normal hearing students of 2nd grade (P<0.001. Reading comprehension scores of students with hearing impairment in higher grades had improved slightly, but it was still lower than that of the normal hearing students in the 2nd grade. Conclusion: It appears that reading comprehension skill of students with significant hearing impairment near the end of elementary school years becomes weaker than normal hearing students in the second grade. Therefore, it is essential to find and resolve the underlying reasons of this condition by all professionals who work in the field of education and rehabilitation of these students.

Hearing Loss Associated with Retinitis Pigmentosa. Short Reports.

Science.gov (United States)

Karp, Adrienne

1985-01-01

The article describes a variation of Usher's Syndrome, a genetic condition characterized by visual and auditory impairments, in which moderate, postlingual, and sometimes progressive hearing impairments may go undetected. Identification guidelines are offered. (Author/CL)

Assessment of narrative writing by Persian-speaking students with hearing impairments.

Science.gov (United States)

Zamani, P; Soleymani, Z; Mousavi, S M; Akbari, N

2018-02-16

Previous studies have highlighted that narrative skill is critical to the development of the literacy skills by children. Children with cochlear implants (CI) and hearing aids (HA) may have problems in narrative development compared to peers with healthy hearing (HH). There is no exact data about the narrative writing ability of Persian-speaking students who are hearing-impaired. This study was undertaken to compare the microstructure and macrostructure scores for narrative writing of Persian-speaking students who are hearing-impaired and peers with HH. This was a cross-sectional descriptive-analytical study. The subjects were recruited from elementary schools in the city of Tehran. A total of 144 elementary school students were participated. The written narratives were elicited using a wordless pictorial storybook story. Three-way ANOVA with post hoc adjusted Bonferroni test was applied to determine the main effects and interactions of grounded variables on the microstructure and macrostructure components of narrative writing. No significant differences were observed in the macrostructure components of narrative writing between hearing-impaired and HH students. Factors analysis showed that the 4th grade HH students had significantly the highest scores, and the 3rd grade HA students had significantly the lowest scores in microstructure components of narrative writing. The findings revealed that hearing-impaired students similarly to their HH peers can transmit the main idea (macrostructure) of narrative writing, but show critical difficulties when using complete grammatical elements (microstructures) to form sentences to convey the idea in the narrative. © 2018 John Wiley & Sons Ltd.

[Examination of relationship between level of hearing and written language skills in 10-14-year-old hearing impaired children].

Science.gov (United States)

Turğut, Nedim; Karlıdağ, Turgut; Başar, Figen; Yalçın, Şinasi; Kaygusuz, İrfan; Keleş, Erol; Birkent, Ömer Faruk

2015-01-01

This study aims to review the relationship between written language skills and factors which are thought to affect this skill such as mean hearing loss, duration of auditory deprivation, speech discrimination score, and pre-school education attendance and socioeconomic status of hearing impaired children who attend 4th-7th grades in primary school in inclusive environment. The study included 25 hearing impaired children (14 males, 11 females; mean age 11.4±1.4 years; range 10 to 14 years) (study group) and 20 children (9 males, 11 females; mean age 11.5±1.3 years; range 10 to 14 years) (control group) with normal hearing in the same age group and studying in the same class. Study group was separated into two subgroups as group 1a and group 1b since some of the children with hearing disability used hearing aid while some used cochlear implant. Intragroup comparisons and relational screening were performed for those who use hearing aids and cochlear implants. Intergroup comparisons were performed to evaluate the effect of the parameters on written language skills. Written expression skill level of children with hearing disability was significantly lower than their normal hearing peers (p=0.001). A significant relationship was detected between written language skills and mean hearing loss (p=0.048), duration of auditory deprivation (p=0.021), speech discrimination score (p=0.014), and preschool attendance (p=0.005), when it comes to socioeconomic status we were not able to find any significant relationship (p=0.636). It can be said that hearing loss affects written language skills negatively and hearing impaired individuals develop low-level written language skills compared to their normal hearing peers.

Association between diabetes mellitus and hearing impairment in American and Korean populations.

Science.gov (United States)

Moon, Shinje; Park, Jung Hwan; Yu, Jae Myung; Choi, Moon-Ki; Yoo, Hyung Joon

2018-04-20

The aim of this study was to evaluate ethnic- and sex-specific associations between DM and hearing impairment. For this cross-sectional study using National Health and Nutrition Examination Survey in the U.S. and Korea, the total number of eligible participants included was 7081 in the U.S. and 15,704 in Korea. Hearing impairment was defined as a pure tone threshold level ≥ 25 dB. Multivariate logistic regression analysis was conducted, adjusting for age, sex, race/ethnicity, socioeconomic status, body mass index, noise exposure, smoking, hypertension, and dyslipidemia. The association between DM and hearing impairment was found to be sex-specific. The multivariate adjusted ORs of high-frequency impairment were 0.843 (95% CI, 0.524-1.356) in American men, and 1.073 (95% CI, 0.835-1.379) in Korean men, while the ORs in women from U.S. and Korea were 1.911 (95% CI, 1.244-2.935) and 1.421 (95% CI, 1.103-1.830), respectively. A subgroup analysis of each race/ethnicity among the U.S. adults showed similar results. In contrast to high-frequency impairment, there was no significant association between low-frequency impairment and DM in both men and women. Our results suggest that DM is associated with hearing impairment in only women, irrespective of race/ethnicity groups. Copyright © 2018. Published by Elsevier Inc.

The specifics of reading to students with hearing and speech impairment in classes VI-VIII

Directory of Open Access Journals (Sweden)

Mustaf Morina

2016-03-01

Full Text Available Good reading has a very important role in the development of children with hearing impaired; also reading in explicit way is one of the crucial factors which affect the oral language development of children with hearing impaired. The best form and possibility of improvement, development of oral language, development of communicating, receipt of information, knowledge, and ideas over the world, is reading. When the auditory perception is damaged reading is poor. Hearing impairment causes a lot of problems in the development of personality of children with hearing impairment in these fields: poor development of vocabulary, poor quality of lexica, poor quality of sentences, and disorder in articulation. The purpose of this research is to verify the following: 1-Speed of reading of hearing impaired children, 2-The number of errors, 3-The kind of errors, 4-To understand the text in the context of the degree of hearing impairment, age (class, success in school and gender. This theoretical-experimental study was made with students from two schools; special school “Mother Teresa” in Prizren and Primary School “Elena Gjika” in Prishtina (class attached. The research included a total of 32 students (respondent 27 students (respondent from special schools “Mother Teresa” in Prizren and 5 elementary school students “Elena Gjika” Prishtina, all these students are with hearing impairment. From 32 students involved in the research, 23 were male and 9 female. The research was done by applying a text fables “The fox and the raven” watched and analyzed in terms of three dimensions. The research results have shown that students with hearing impairments have considerable problems in many aspects; in terms of speed of reading, students with hearing impairment have stagnated compared with their peers in the ratio 8/1. In terms of reading errors have stagnated considered being incomparable. In terms of understanding the text students with hearing

Processing Mechanisms in Hearing-Impaired Listeners: Evidence from Reaction Times and Sentence Interpretation.

Science.gov (United States)

Carroll, Rebecca; Uslar, Verena; Brand, Thomas; Ruigendijk, Esther

The authors aimed to determine whether hearing impairment affects sentence comprehension beyond phoneme or word recognition (i.e., on the sentence level), and to distinguish grammatically induced processing difficulties in structurally complex sentences from perceptual difficulties associated with listening to degraded speech. Effects of hearing impairment or speech in noise were expected to reflect hearer-specific speech recognition difficulties. Any additional processing time caused by the sustained perceptual challenges across the sentence may either be independent of or interact with top-down processing mechanisms associated with grammatical sentence structure. Forty-nine participants listened to canonical subject-initial or noncanonical object-initial sentences that were presented either in quiet or in noise. Twenty-four participants had mild-to-moderate hearing impairment and received hearing-loss-specific amplification. Twenty-five participants were age-matched peers with normal hearing status. Reaction times were measured on-line at syntactically critical processing points as well as two control points to capture differences in processing mechanisms. An off-line comprehension task served as an additional indicator of sentence (mis)interpretation, and enforced syntactic processing. The authors found general effects of hearing impairment and speech in noise that negatively affected perceptual processing, and an effect of word order, where complex grammar locally caused processing difficulties for the noncanonical sentence structure. Listeners with hearing impairment were hardly affected by noise at the beginning of the sentence, but were affected markedly toward the end of the sentence, indicating a sustained perceptual effect of speech recognition. Comprehension of sentences with noncanonical word order was negatively affected by degraded signals even after sentence presentation. Hearing impairment adds perceptual processing load during sentence processing

Effect of musical training on pitch discrimination performance in older normal-hearing and hearing-impaired listeners

DEFF Research Database (Denmark)

Bianchi, Federica; Dau, Torsten; Santurette, Sébastien

2017-01-01

-discrimination performance for NH listeners. It is unclear whether a comparable effect of musical training occurs for listeners whose sensory encoding of F0 is degraded. To address this question, F0 discrimination was investigated for three groups of listeners (14 young NH, 9 older NH and 10 HI listeners), each......Hearing-impaired (HI) listeners, as well as elderly listeners, typically have a reduced ability to discriminate the fundamental frequency (F0) of complex tones compared to young normal-hearing (NH) listeners. Several studies have shown that musical training, on the other hand, leads to improved F0...... including musicians and non-musicians, using complex tones that differed in harmonic content. Musical training significantly improved F0 discrimination for all groups of listeners, especially for complex tones containing low-numbered harmonics. In a second experiment, the sensitivity to temporal fine...

Analysis of Undergraduate Training Given to Students with Hearing Impairment in their Acquisition of Professional Competences

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Mariona DALMAU MONTALÀ

2015-12-01

Full Text Available The aim of this study was to understand how university graduates with a hearing impairment in Spain perceived their studies. The method used was quantitativequalitative and descriptive. The participating sample consisted of 84 Spanish university graduates with a hearing impairment. The results allow us to confirm that: university graduates with a hearing impairment, in general, are not provided with appropriate/ sufficient support at university to develop their professional competence; these graduates believe that employers consider good command of professional competences to be pivotal; professionalizing practices during university studies are an opportunity for the employment of graduates with hear impairment; finally, it is highlighted that university lecturers lack knowledge of the needs and difficulties of students with hearing impairment.

Hearing impairment associated with oral terbinafine use

NARCIS (Netherlands)

Scholl, Joep; Van Grootheest, Kees; Van Puijenbroek, Eugene

Background: The Netherlands Pharmacovigilance Centre Lareb received six reports of hearing impairment in association with oral terbinafine use. This study describes these cases and provides support for this association from the Lareb database of spontaneous ADR reporting and from Vigibase, the

Association between dipstick proteinuria and hearing impairment in health check-ups among Japanese workers: a cross-sectional study.

Science.gov (United States)

Umesawa, Mitsumasa; Hara, Mikako; Sairenchi, Toshimi; Haruyama, Yasuo; Nagao, Masanori; Matsushita, Munehiro; Kobashi, Gen

2018-05-14

Prevention of hearing impairment is important because it is difficult to recover from it. Epidemiological studies have examined the risk factors for hearing impairment; however, the association between dipstick proteinuria and hearing impairment has not been previously examined. This study aimed to clarify the association between dipstick proteinuria and hearing impairment. Cross-sectional study. Office and factory workers from all over Japan. The total number of subjects was 7005. All were employees of the same company. Of these, we recruited 6192 subjects who underwent dipstick urine test and hearing test by audiometry in annual health check-ups (mean age 44.9 years, men 88.3%). Hearing tests were performed at two frequencies (1 kHz, 4 kHz) as prescribed by law in Japan. We defined the inability of subjects to respond to 30 dB at 1 kHz and/or 40 dB at 4 kHz as overall moderate hearing impairment. In addition, we defined moderate hearing impairment at 1 kHz (4 kHz) as an abnormal finding at 1 kHz (4 kHz). We examined the associations between degree of dipstick proteinuria and hearing impairment after adjustment for age, sex, body mass index, hypertension, diabetes mellitus, serum creatinine level and history of noisy work environment. Overall moderate hearing impairment was noted in 324 subjects (5.2%). Of these, 107 subjects (1.7%) had moderate hearing impairment at 1 kHz and 278 subjects (4.5%) at 4 kHz. Dipstick proteinuria was significantly associated with overall moderate hearing impairment, as well as moderate hearing impairment at both 1 kHz and 4 kHz. The prevalence of overall moderate hearing impairment among subjects with proteinuria ≥2+ was 23.5%, while that among subjects without proteinuria was 5.2% (pimpairment in Japanese workers. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

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

Science.gov (United States)

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

2016-02-01

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

Using Modality Replacement to Facilitate Communication between Visually and Hearing-Impaired People

DEFF Research Database (Denmark)

Moustakas, K.; Tzovaras, D.; Dybkjaer, L.

2011-01-01

Using sign language, speech, and haptics as communication modalities, a virtual treasure-hunting game serves as an entertainment and educational tool for visually-and hearing-impaired users.......Using sign language, speech, and haptics as communication modalities, a virtual treasure-hunting game serves as an entertainment and educational tool for visually-and hearing-impaired users....

Investigation of Psychophysiological and Subjective Effects of Long Working Hours - Do Age and Hearing Impairment Matter?

Science.gov (United States)

Wagner-Hartl, Verena; Kallus, K Wolfgang

2017-01-01

Following current prognosis, demographic development raises expectations of an aging of the working population. Therefore, keeping employees healthy and strengthening their ability to work, becomes more and more important. When employees become older, dealing with age-related impairments of sensory functions, such as hearing impairment, is a central issue. Recent evidence suggests that negative effects that are associated with reduced hearing can have a strong impact at work. Especially under exhausting working situations such as working overtime hours, age and hearing impairment might influence employees' well-being. Until now, neither the problem of aged workers and long working hours, nor the problem of hearing impairment and prolonged working time has been addressed explicitly. Therefore, a laboratory study was examined to answer the research question: Do age and hearing impairment have an impact on psychophysiological and subjective effects of long working hours. In total, 51 white-collar workers, aged between 24 and 63 years, participated in the laboratory study. The results show no significant effects for age and hearing impairment on the intensity of subjective consequences (perceived recovery and fatigue, subjective emotional well-being and physical symptoms) of long working hours. However, the psychophysiological response (the saliva cortisol level) to long working hours differs significantly between hearing impaired and normal hearing employees. Interestingly, the results suggest that from a psychophysiological point of view long working hours were more demanding for normal hearing employees.

Self-Concept of Severely to Profoundly Hearing-Impaired Children.

Science.gov (United States)

Warren, Charlotte; Hasenstab, Suzanne

1986-01-01

A study examined demographic, impairment-related, and parental variables that best predicted self-concept among 49 severely to profoundly hearing-impaired 5- to 11-year-olds. A strong relationship was observed between self-concept and parental indulgence, parental rejection, parental protection, parental discipline, and extent of language…

The Recreational Activities of Hearing-impaired Children of a Young School Age

OpenAIRE

Šrámková, Markéta

2007-01-01

In my diploma work I would like to focus on theme of spending free time in school for hearing impaired children (age 6-12) . The work has two parts. In the first one - theoretical- literary - I will describe types of spending free time of deaf and hard of hearing children different enviroments : family, school, out of school. In the second one -practical research - I will focus on the profile of school for hearing impaired children. I would like to touch the problem of activities (during the ...

The strengths and weaknesses in verbal short-term memory and visual working memory in children with hearing impairment and additional language learning difficulties.

Science.gov (United States)

Willis, Suzi; Goldbart, Juliet; Stansfield, Jois

2014-07-01

To compare verbal short-term memory and visual working memory abilities of six children with congenital hearing-impairment identified as having significant language learning difficulties with normative data from typically hearing children using standardized memory assessments. Six children with hearing loss aged 8-15 years were assessed on measures of verbal short-term memory (Non-word and word recall) and visual working memory annually over a two year period. All children had cognitive abilities within normal limits and used spoken language as the primary mode of communication. The language assessment scores at the beginning of the study revealed that all six participants exhibited delays of two years or more on standardized assessments of receptive and expressive vocabulary and spoken language. The children with hearing-impairment scores were significantly higher on the non-word recall task than the "real" word recall task. They also exhibited significantly higher scores on visual working memory than those of the age-matched sample from the standardized memory assessment. Each of the six participants in this study displayed the same pattern of strengths and weaknesses in verbal short-term memory and visual working memory despite their very different chronological ages. The children's poor ability to recall single syllable words in relation to non-words is a clinical indicator of their difficulties in verbal short-term memory. However, the children with hearing-impairment do not display generalized processing difficulties and indeed demonstrate strengths in visual working memory. The poor ability to recall words, in combination with difficulties with early word learning may be indicators of children with hearing-impairment who will struggle to develop spoken language equal to that of their normally hearing peers. This early identification has the potential to allow for target specific intervention that may remediate their difficulties. Copyright © 2014. Published

Speech perception benefits of internet versus conventional telephony for hearing-impaired individuals.

Science.gov (United States)

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

2012-07-16

Telephone communication is a challenge for many hearing-impaired individuals. One important technical reason for this difficulty is the restricted frequency range (0.3-3.4 kHz) of conventional landline telephones. Internet telephony (voice over Internet protocol [VoIP]) is transmitted with a larger frequency range (0.1-8 kHz) and therefore includes more frequencies relevant to speech perception. According to a recently published, laboratory-based study, the theoretical advantage of ideal VoIP conditions over conventional telephone quality has translated into improved speech perception by hearing-impaired individuals. However, the speech perception benefits of nonideal VoIP network conditions, which may occur in daily life, have not been explored. VoIP use cannot be recommended to hearing-impaired individuals before its potential under more realistic conditions has been examined. To compare realistic VoIP network conditions, under which digital data packets may be lost, with ideal conventional telephone quality with respect to their impact on speech perception by hearing-impaired individuals. We assessed speech perception using standardized test material presented under simulated VoIP conditions with increasing digital data packet loss (from 0% to 20%) and compared with simulated ideal conventional telephone quality. We monaurally tested 10 adult users of cochlear implants, 10 adult users of hearing aids, and 10 normal-hearing adults in the free sound field, both in quiet and with background noise. Across all participant groups, mean speech perception scores using VoIP with 0%, 5%, and 10% packet loss were 15.2% (range 0%-53%), 10.6% (4%-46%), and 8.8% (7%-33%) higher, respectively, than with ideal conventional telephone quality. Speech perception did not differ between VoIP with 20% packet loss and conventional telephone quality. The maximum benefits were observed under ideal VoIP conditions without packet loss and were 36% (P = .001) for cochlear implant users, 18

Primary Sjogren’s Syndrome Presented with Sensory Ataxia Associated with Bilateral Hearing Loss and Dementia

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Madjdinasab Nastaran

2009-10-01

Full Text Available Primary Sjorgen syndrome is one of the commonest autoimmune diseases with characteristic of involvement of lachrymal and salivary glands, but other organ involvements as peripheral and central nervous system are also possible. The reported case is a 23 year old lady presented with progressive sensory ataxia and weakness of four limbs, bilateral sensory hearing loss and cognitive impairment with minimental score equal to 15/30 since one year prior to admission with associated bilateral central corneal opacity, dry mouth and dry eyes. Electro physiologic studies showed sensory motor axonal polyneuropathy . A biopsy of sural nerve and salivary glands of lower lip showed lymphocytic infiltration. Serologic evidence showed positive Anti Ro (SS-B, negative HCV and HIV antibody, thereafter the diagnosis was confirmed and according to this diagnosis she received high dose of intravenous methyl prednisolon then both hearing loss and cognitive impairment improved partially (minimental score 21/30 . At last, she underwent plasmapheresis and her sensory ataxia improved greatly.

Hearing loss in a mouse model of 22q11.2 Deletion Syndrome.

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Jennifer C Fuchs

Full Text Available 22q11.2 Deletion Syndrome (22q11DS arises from an interstitial chromosomal microdeletion encompassing at least 30 genes. This disorder is one of the most significant known cytogenetic risk factors for schizophrenia, and can also cause heart abnormalities, cognitive deficits, hearing difficulties, and a variety of other medical problems. The Df1/+ hemizygous knockout mouse, a model for human 22q11DS, recapitulates many of the deficits observed in the human syndrome including heart defects, impaired memory, and abnormal auditory sensorimotor gating. Here we show that Df1/+ mice, like human 22q11DS patients, have substantial rates of hearing loss arising from chronic middle ear infection. Auditory brainstem response (ABR measurements revealed significant elevation of click-response thresholds in 48% of Df1/+ mice, often in only one ear. Anatomical and histological analysis of the middle ear demonstrated no gross structural abnormalities, but frequent signs of otitis media (OM, chronic inflammation of the middle ear, including excessive effusion and thickened mucosa. In mice for which both in vivo ABR thresholds and post mortem middle-ear histology were obtained, the severity of signs of OM correlated directly with the level of hearing impairment. These results suggest that abnormal auditory sensorimotor gating previously reported in mouse models of 22q11DS could arise from abnormalities in auditory processing. Furthermore, the findings indicate that Df1/+ mice are an excellent model for increased risk of OM in human 22q11DS patients. Given the frequently monaural nature of OM in Df1/+ mice, these animals could also be a powerful tool for investigating the interplay between genetic and environmental causes of OM.

A framework for communication between visually impaired, hearing impaired and speech impaired using arduino

Science.gov (United States)

Sujatha, R.; Khandelwa, Prakhar; Gupta, Anusha; Anand, Nayan

2017-11-01

A long time ago our society accepted the notion of treating people with disabilities not as unviable and disabled but as differently-abled, recognizing their skills beyond their disabilities. The next step has to be taken by our scientific community, that is, to normalize lives of the people with disabilities and make it so as if they are no different to us. The primary step in this direction would be to normalize communication between people. People with an impaired speech or impaired vision or impaired hearing face difficulties while having a casual conversation with others. Any form of communication feels so strenuous that the impaired end up communicating just the important information and avoid a casual conversation. To normalize conversation between the impaired we need a simple and compact device which facilitates the conversation by providing the information in the desired form.

Prevalence and causes of hearing impairment in Fundong Health District, North-West Cameroon.

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Ferrite, Silvia; Mactaggart, Islay; Kuper, Hannah; Oye, Joseph; Polack, Sarah

2017-04-01

To estimate the prevalence and causes of hearing impairment in Fundong Health District, North-West Cameroon. We selected 51 clusters of 80 people (all ages) through probability proportionate to size sampling. Initial hearing screening was undertaken through an otoacoustic emission (OAE) test. Participants aged 4+ years who failed this test in both ears or for whom an OAE reading could not be taken underwent a manual pure-tone audiometry (PTA) screening. Cases of hearing impairment were defined as those with pure-tone average ≥41 dBHL in adults and ≥35 dBHL in children in the better ear, or children under age 4 who failed the OAE test in both ears. Each case with hearing loss was examined by an ear, nose and throat nurse who indicated the main likely cause. We examined 3567 (86.9%) of 4104 eligible people. The overall prevalence of hearing impairment was 3.6% (95% confidence interval [CI]: 2.8-4.6). The prevalence was low in people aged 0-17 (1.1%, 0.7-1.8%) and 18-49 (1.1%, 0.5-2.6%) and then rose sharply in people aged 50+ (14.8%, 11.7-19.1%). Among cases, the majority were classified as moderate (76%), followed by severe (15%) and profound (9%). More than one-third of cases of hearing impairment were classified as unknown (37%) or conductive (37%) causes, while sensorineural causes were less common (26%). Prevalence of hearing impairment in North-West Cameroon is in line with the WHO estimate for sub-Saharan Africa. The majority of cases with known causes are treatable, with impacted wax playing a major role. © 2017 John Wiley & Sons Ltd.

The benefit of gestures during communication: evidence from hearing and hearing-impaired individuals.

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Obermeier, Christian; Dolk, Thomas; Gunter, Thomas C

2012-07-01

There is no doubt that gestures are communicative and can be integrated online with speech. Little is known, however, about the nature of this process, for example, its automaticity and how our own communicative abilities and also our environment influence the integration of gesture and speech. In two Event Related Potential (ERP) experiments, the effects of gestures during speech comprehension were explored. In both experiments, participants performed a shallow task thereby avoiding explicit gesture-speech integration. In the first experiment, participants with normal hearing viewed videos in which a gesturing actress uttered sentences which were either embedded in multi-speaker babble noise or not. The sentences contained a homonym which was disambiguated by the information in a gesture, which was presented asynchronous to speech (1000 msec earlier). Downstream, the sentence contained a target word that was either related to the dominant or subordinate meaning of the homonym and was used to indicate the success of the disambiguation. Both the homonym and the target word position showed clear ERP evidence of gesture-speech integration and disambiguation only under babble noise. Thus, during noise, gestures were taken into account as an important communicative cue. In Experiment 2, the same asynchronous stimuli were presented to a group of hearing-impaired students and age-matched controls. Only the hearing-impaired individuals showed significant speech-gesture integration and successful disambiguation at the target word. The age-matched controls did not show any effect. Thus, individuals who chronically experience suboptimal communicative situations in daily life automatically take gestures into account. The data from both experiments indicate that gestures are beneficial in countering difficult communication conditions independent of whether the difficulties are due to external (babble noise) or internal (hearing impairment) factors. Copyright © 2011 Elsevier

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

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Yasue Uchida

2014-01-01

Full Text Available 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.

[From gene to disease; genetic causes of hearing loss and visual impairment sometimes accompanied by vestibular problems (Usher syndrome)

NARCIS (Netherlands)

Pennings, R.J.E.; Kremer, J.M.J.; Deutman, A.F.; Kimberling, W.J.; Cremers, C.W.R.J.

2002-01-01

Usher syndrome is an autosomal recessively inherited disease, characterised by sensorineural hearing loss, tapetoretinal degeneration and in some cases vestibular problems. Based on the clinical heterogeneity, the disease can be classified into three clinical types (I, II and III), which have their

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

Science.gov (United States)

Vernon, McCay

1982-01-01

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

Investigation of Psychophysiological and Subjective Effects of Long Working Hours – Do Age and Hearing Impairment Matter?

Science.gov (United States)

Wagner-Hartl, Verena; Kallus, K. Wolfgang

2018-01-01

Following current prognosis, demographic development raises expectations of an aging of the working population. Therefore, keeping employees healthy and strengthening their ability to work, becomes more and more important. When employees become older, dealing with age-related impairments of sensory functions, such as hearing impairment, is a central issue. Recent evidence suggests that negative effects that are associated with reduced hearing can have a strong impact at work. Especially under exhausting working situations such as working overtime hours, age and hearing impairment might influence employees’ well-being. Until now, neither the problem of aged workers and long working hours, nor the problem of hearing impairment and prolonged working time has been addressed explicitly. Therefore, a laboratory study was examined to answer the research question: Do age and hearing impairment have an impact on psychophysiological and subjective effects of long working hours. In total, 51 white-collar workers, aged between 24 and 63 years, participated in the laboratory study. The results show no significant effects for age and hearing impairment on the intensity of subjective consequences (perceived recovery and fatigue, subjective emotional well-being and physical symptoms) of long working hours. However, the psychophysiological response (the saliva cortisol level) to long working hours differs significantly between hearing impaired and normal hearing employees. Interestingly, the results suggest that from a psychophysiological point of view long working hours were more demanding for normal hearing employees. PMID:29379452

Investigation of Psychophysiological and Subjective Effects of Long Working Hours – Do Age and Hearing Impairment Matter?

Directory of Open Access Journals (Sweden)

Verena Wagner-Hartl

2018-01-01

Full Text Available Following current prognosis, demographic development raises expectations of an aging of the working population. Therefore, keeping employees healthy and strengthening their ability to work, becomes more and more important. When employees become older, dealing with age-related impairments of sensory functions, such as hearing impairment, is a central issue. Recent evidence suggests that negative effects that are associated with reduced hearing can have a strong impact at work. Especially under exhausting working situations such as working overtime hours, age and hearing impairment might influence employees’ well-being. Until now, neither the problem of aged workers and long working hours, nor the problem of hearing impairment and prolonged working time has been addressed explicitly. Therefore, a laboratory study was examined to answer the research question: Do age and hearing impairment have an impact on psychophysiological and subjective effects of long working hours. In total, 51 white-collar workers, aged between 24 and 63 years, participated in the laboratory study. The results show no significant effects for age and hearing impairment on the intensity of subjective consequences (perceived recovery and fatigue, subjective emotional well-being and physical symptoms of long working hours. However, the psychophysiological response (the saliva cortisol level to long working hours differs significantly between hearing impaired and normal hearing employees. Interestingly, the results suggest that from a psychophysiological point of view long working hours were more demanding for normal hearing employees.

A Review of literature on the clinical psychological study on the hearing impaired in Japan

OpenAIRE

Fujitomo, Masakazu

2002-01-01

The purpose of this paper was to review literatures about past clinical psychological studies on the hearing impaired in japan, in order to get suggestions about subjects on psychological supports and clinical psychological studies for the hearing impaired in Japan. First, in the topic of the history and subjects on the clinical psychological study and psycological supports for the hearing impaired, it was showed that the pathological viewpoint was taken place for the cultural viewpoint in th...

Hearing Impaired Education of the Department of Education in Region X, Philippines: Its Approaches and Implication

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James Mirabeau B. Undalok

2015-11-01

Full Text Available One way to attain improvement of the educational programs of the hearing impaired is by conducting survey and assessment of the status of the hearing impaired education of the Department of Education in Region X, Philippines. The Special Education (SpEd teachers play a vital role for the pupils of the hearing impaired children as they are viewed as linchpins in the learning process of the children. This cannot be attained without the different approaches on hearing impaired education. Survey -questionnaires were used to gather information needed. Data were analyzed using the descriptive statistics such as weighted mean and standard deviation. The ANOVA test was used to determine the significance of the hearing impaired education of the Department of Education in Region X. Anchored on the findings, the following conclusions are made about different educational approaches should be given priority by the SPED teachers is further enhance the lifelong learning skills of the pupils. It helps them for their learning process and acquiring language skills. There should be an advocacy on the hearing impaired education program to the public and stakeholders.

Screening an elderly hearing impaired population for mild cognitive impairment using Mini-Mental State Examination (MMSE) and Montreal Cognitive Assessment (MoCA).

Science.gov (United States)

Lim, Magdalene Yeok Leng; Loo, Jenny Hooi Yin

2018-07-01

To determine if there is an association between hearing loss and poorer cognitive scores on Mini-Mental State Examination (MMSE) and Montreal Cognitive Assessment (MoCA) and to determine if poor hearing acuity affects scoring on the cognitive screening tests of MMSE and MoCA. One hundred fourteen elderly patients (Singapore residents) aged between 55 and 86 years were sampled. Participants completed a brief history questionnaire, pure tone audiometry, and 2 cognitive screening tests-the MMSE and MoCA. Average hearing thresholds of the better ear in the frequencies of 0.5, 1, 2, and 4 kHz were used for data analysis. Hearing loss was significantly associated with poorer cognitive scores in Poisson regression models adjusted for age. Mini-Mental State Examination scores were shown to decrease by 2.8% (P = .029), and MoCA scores by 3.5% (P = .013) for every 10 dB of hearing loss. Analysis of hearing-sensitive components of "Registration" and "Recall" in MMSE and MoCA using chi-square tests showed significantly poorer performance in the hearing loss group as compared to the normal hearing group. Phonetic analysis of target words with high error rates shows that the poor performance was likely contributed by decreased hearing acuity, on top of a possible true deficit in cognition in the hearing impaired. Hearing loss is associated with poorer cognitive scores on MMSE and MoCA, and cognitive scoring is likely confounded by poor hearing ability. This highlights an important, often overlooked aspect of sensory impairment during cognitive screening. Provisions should be made when testing for cognition in the hearing-impaired population to avoid over-referral and subsequent misdiagnoses of cognitive impairment. Copyright © 2018 John Wiley & Sons, Ltd.

Mutations in the Wolfram syndrome 1 gene (WFS1) are a common cause of low frequency sensorineural hearing loss.

NARCIS (Netherlands)

Bespalova, I.N.; Camp, G. van; Bom, S.J.H.; Brown, D.J.; Cryns, K.; Wan, A.T. de; Erson, A.E.; Flothmann, K.; Kunst, H.P.M.; Kurnool, P.; Sivakumaran, T.A.; Cremers, C.W.R.J.; Leal, S.M.; Burmeister, M.; Lesperance, M.M.

2001-01-01

Non-syndromic low frequency sensorineural hearing loss (LFSNHL) affecting only 2000 Hz and below is an unusual type of hearing loss that worsens over time without progressing to profound deafness. This type of LFSNHL may be associated with mild tinnitus but is not associated with vertigo. We have

Difficulty understanding speech in noise by the hearing impaired: underlying causes and technological solutions.

Science.gov (United States)

Healy, Eric W; Yoho, Sarah E

2016-08-01

A primary complaint of hearing-impaired individuals involves poor speech understanding when background noise is present. Hearing aids and cochlear implants often allow good speech understanding in quiet backgrounds. But hearing-impaired individuals are highly noise intolerant, and existing devices are not very effective at combating background noise. As a result, speech understanding in noise is often quite poor. In accord with the significance of the problem, considerable effort has been expended toward understanding and remedying this issue. Fortunately, our understanding of the underlying issues is reasonably good. In sharp contrast, effective solutions have remained elusive. One solution that seems promising involves a single-microphone machine-learning algorithm to extract speech from background noise. Data from our group indicate that the algorithm is capable of producing vast increases in speech understanding by hearing-impaired individuals. This paper will first provide an overview of the speech-in-noise problem and outline why hearing-impaired individuals are so noise intolerant. An overview of our approach to solving this problem will follow.

Speech Perception Benefits of Internet Versus Conventional Telephony for Hearing-Impaired Individuals

Science.gov (United States)

Dubach, Patrick; Pfiffner, Flurin; Kompis, Martin; Caversaccio, Marco

2012-01-01

Background Telephone communication is a challenge for many hearing-impaired individuals. One important technical reason for this difficulty is the restricted frequency range (0.3–3.4 kHz) of conventional landline telephones. Internet telephony (voice over Internet protocol [VoIP]) is transmitted with a larger frequency range (0.1–8 kHz) and therefore includes more frequencies relevant to speech perception. According to a recently published, laboratory-based study, the theoretical advantage of ideal VoIP conditions over conventional telephone quality has translated into improved speech perception by hearing-impaired individuals. However, the speech perception benefits of nonideal VoIP network conditions, which may occur in daily life, have not been explored. VoIP use cannot be recommended to hearing-impaired individuals before its potential under more realistic conditions has been examined. Objective To compare realistic VoIP network conditions, under which digital data packets may be lost, with ideal conventional telephone quality with respect to their impact on speech perception by hearing-impaired individuals. Methods We assessed speech perception using standardized test material presented under simulated VoIP conditions with increasing digital data packet loss (from 0% to 20%) and compared with simulated ideal conventional telephone quality. We monaurally tested 10 adult users of cochlear implants, 10 adult users of hearing aids, and 10 normal-hearing adults in the free sound field, both in quiet and with background noise. Results Across all participant groups, mean speech perception scores using VoIP with 0%, 5%, and 10% packet loss were 15.2% (range 0%–53%), 10.6% (4%–46%), and 8.8% (7%–33%) higher, respectively, than with ideal conventional telephone quality. Speech perception did not differ between VoIP with 20% packet loss and conventional telephone quality. The maximum benefits were observed under ideal VoIP conditions without packet loss and

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

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Yang Wang

Full Text Available 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.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.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.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.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 relationship between parasympathetic activity and hearing

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

Science.gov (United States)

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

2016-01-01

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. 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. 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. 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. 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 relationship between parasympathetic activity and hearing impairment

Understanding Basic Temporal Relations in Primary School Pupils with Hearing Impairments.

Science.gov (United States)

Dulcić, Adinda; Bakota, Koraljka; Saler, Zrinka

2015-09-01

Time can be observed as a subjective, as well as an objective phenomenon which is a component of our life, and due to its communicational needs, it is standardized by temporal signs and symbols. The aim of this study was to determine the understanding of basic temporal relations of pupils with hearing impairments. We assumed that the knowledge of basic time relations is a precondition for the acquisition of knowledge that is connected with the understanding of the syllabus in regular school programs. Three groups of pupils have been examined: pupils with hearing impairments who attend the primary school of SUVAG Polyclinic under special condition, integrated hearing impaired pupils with minor additional difficulties who attend regular primary schools in Zagreb with a prolonged expert procedure and pupils of the control group. The subjects have been examined with a measuring instrument constructed by the expert team of the Polyclinic Suvag. Twenty nine subjects have been questioned, chronologically aged between 10 and 12.

Does the introduction of newborn hearing screening improve vocabulary development in hearing-impaired children? A population-based study in Japan.

Science.gov (United States)

Ohmori, Shuhei; Sugaya, Akiko; Toida, Naomi; Suzuki, Etsuji; Izutsu, Masato; Tsutsui, Tomoko; Kataoka, Yuko; Maeda, Yukihide; Fukushima, Kunihiro; Nishizaki, Kazunori

2015-02-01

Permanent hearing impairment has a life-long impact on children and its early identification is important for language development. A newborn hearing screening (NHS) program has started in Okayama Prefecture, Japan, in 1999 to detect hearing impairment immediately after birth. We aim to examine the effect of this screening program on vocabulary development in pre-school children in a before and after comparative study design. A total of 107 5-year-old children who graduated from Okayama Kanariya Gakuen (an auditory center for hearing-impaired children) between 1998 and 2011 were enrolled in this study. The pre-NHS group (n=40) was defined as those who graduated between 1998 and 2003, while the post-NHS group (n=67) was defined as those who graduated between 2004 and 2011. The primary outcome was receptive vocabulary, which was assessed by the Picture Vocabulary Test [score vocabulary, or the number of productive words, which was assessed by an original checklist [vocabulary development and compared both groups. The adjusted Picture Vocabulary Test score and number of productive words were significantly higher (pvocabulary and 4.17 (95% confidence interval: 1.69-10.29) for productive vocabulary. The introduction of NHS in Okayama Prefecture significantly improved both receptive and productive vocabulary development in hearing-impaired children. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

The Hearing Outcomes of Cochlear Implantation in Waardenburg Syndrome

OpenAIRE

Koyama, Hajime; Kashio, Akinori; Sakata, Aki; Tsutsumiuchi, Katsuhiro; Matsumoto, Yu; Karino, Shotaro; Kakigi, Akinobu; Iwasaki, Shinichi; Yamasoba, Tatsuya

2016-01-01

Objectives. This study aimed to determine the feasibility of cochlear implantation for sensorineural hearing loss in patients with Waardenburg syndrome. Method. A retrospective chart review was performed on patients who underwent cochlear implantation at the University of Tokyo Hospital. Clinical classification, genetic mutation, clinical course, preoperative hearing threshold, high-resolution computed tomography of the temporal bone, and postoperative hearing outcome were assessed. Result. F...

Speech intelligibility for normal hearing and hearing-impaired listeners in simulated room acoustic conditions

DEFF Research Database (Denmark)

Arweiler, Iris; Dau, Torsten; Poulsen, Torben

Speech intelligibility depends on many factors such as room acoustics, the acoustical properties and location of the signal and the interferers, and the ability of the (normal and impaired) auditory system to process monaural and binaural sounds. In the present study, the effect of reverberation...... on spatial release from masking was investigated in normal hearing and hearing impaired listeners using three types of interferers: speech shaped noise, an interfering female talker and speech-modulated noise. Speech reception thresholds (SRT) were obtained in three simulated environments: a listening room......, a classroom and a church. The data from the study provide constraints for existing models of speech intelligibility prediction (based on the speech intelligibility index, SII, or the speech transmission index, STI) which have shortcomings when reverberation and/or fluctuating noise affect speech...

Congenital stapes malformation: Rare conductive hearing loss in a patient with Waardenburg syndrome.

Science.gov (United States)

Melzer, Jonathan M; Eliason, Michael; Conley, George S

2016-04-01

Waardenburg syndrome is a known autosomal dominant cause of congenital hearing loss. It is characterized by a distinctive phenotypic appearance and often involves sensorineural hearing loss. Temporal bone abnormalities and inner ear dysmorphisms have been described in association with the disease. However, middle ear abnormalities as causes of conductive hearing loss are not typically seen in Waardenburg syndrome. We discuss a case of an 8-year-old female who meets diagnostic criteria for Waardenburg syndrome type 3 and who presented with a bilateral conductive hearing loss associated with congenital stapes fixation. We discuss management strategy in this previously unreported phenotype. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

Static and Dynamic Balance in Congenital Severe to Profound Hearing-Impaired Children

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Farideh HajiHeydari

2011-09-01

Full Text Available Background and Aim: Research conducted since the early 1900s has consistently identified differences between deaf and hearing children on performance of a wide variety of motor tasks, most notably balance. Our study was performed to test static and dynamic balance skills in congenital severe to profound hearing impaired children in comparison with normal age-matched children.Methods: This cross-sectional study was conducted on 30 severe to profound hearing impaired and 40 normal children with age 6 to 10 years old. Bruininks-Oseretsky test of motor proficiency 2, balance subset with 9 parts was used for evaluation of balance skills.Results: Hearing-impaired children showed 16.7 to 100% fail results in 7 parts of the balance subset. In normal children fail result was revealed just in 3 parts of the balance subset from 2.5 to 57.5%, and differences between two groups were significant (p<0.0001. There was a significant difference between two groups in two static balance skills of standing on one leg on a line and standing on one leg on a balance beam with eyes closed (p<0.0001.conclusion: It seems that development of static balance skills are longer than dynamic ones. Because severe to profound hearing-impaired children showed more weakness than normal children in both static and dynamic balance abilities, functional tests of balance proficiency can help to identify balance disorders in these children.

Development of a voice database to aid children with hearing impairments

International Nuclear Information System (INIS)

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

2011-01-01

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.

Can parenting practices predict externalizing behavior problems among children with hearing impairment?

Science.gov (United States)

Pino, María J; Castillo, Rosa A; Raya, Antonio; Herruzo, Javier

2017-11-09

To identify possible differences in the level of externalizing behavior problems among children with and without hearing impairment and determine whether any relationship exists between this type of problem and parenting practices. The Behavior Assessment System for Children was used to evaluate externalizing variables in a sample of 118 boys and girls divided into two matched groups: 59 with hearing disorders and 59 normal-hearing controls. Significant between-group differences were found in hyperactivity, behavioral problems, and externalizing problems, but not in aggression. Significant differences were also found in various aspects of parenting styles. A model for predicting externalizing behavior problems was constructed, achieving a predicted explained variance of 50%. Significant differences do exist between adaptation levels in children with and without hearing impairment. Parenting style also plays an important role.

GRM7 variants confer susceptibility to age-related hearing impairment

DEFF Research Database (Denmark)

Friedman, Rick A; Van Laer, Lut; Huentelman, Matthew J

2009-01-01

Age-related hearing impairment (ARHI), or presbycusis, is the most prevalent sensory impairment in the elderly. ARHI is a complex disease caused by an interaction between environmental and genetic factors. Here we describe the results of the first whole genome association study for ARHI. The stud...

Impact of Age and Hearing Impairment on Work Performance during Long Working Hours

Directory of Open Access Journals (Sweden)

Verena Wagner-Hartl

2018-01-01

Full Text Available Based on demographic prognoses, it must be assumed that a greater number of older workers will be found in the future labor market. How to deal with their possible age-related impairments of sensory functions, like hearing impairment and work performance during extended working time, has not been addressed explicitly until now. The study addresses this interplay. The study was performed on two consecutive days after normal working hours. The 55 participants had to “work” in the study at least three additional hours to simulate a situation of long working hours. The tested measures for (job performance were: general attention, long-term selective attention, concentration, and reaction time. All of the investigated variables were taken at both days of the study (2 × 2 × 2 repeated measurement design. The results show effects for age, the interaction of hearing impairment and time of measurement, and effects of the measurement time. Older participants reacted slower than younger participants did. Furthermore, younger participants reacted more frequently in a correct way. Hearing impairment seems to have a negative impact especially on measures of false reactions, and therefore especially on measurement time 1. The results can be interpreted in a way that hearing-impaired participants are able to compensate their deficits over time.

Impact of Age and Hearing Impairment on Work Performance during Long Working Hours.

Science.gov (United States)

Wagner-Hartl, Verena; Grossi, Nina R; Kallus, K Wolfgang

2018-01-09

Based on demographic prognoses, it must be assumed that a greater number of older workers will be found in the future labor market. How to deal with their possible age-related impairments of sensory functions, like hearing impairment and work performance during extended working time, has not been addressed explicitly until now. The study addresses this interplay. The study was performed on two consecutive days after normal working hours. The 55 participants had to "work" in the study at least three additional hours to simulate a situation of long working hours. The tested measures for (job) performance were: general attention, long-term selective attention, concentration, and reaction time. All of the investigated variables were taken at both days of the study (2 × 2 × 2 repeated measurement design). The results show effects for age, the interaction of hearing impairment and time of measurement, and effects of the measurement time. Older participants reacted slower than younger participants did. Furthermore, younger participants reacted more frequently in a correct way. Hearing impairment seems to have a negative impact especially on measures of false reactions, and therefore especially on measurement time 1. The results can be interpreted in a way that hearing-impaired participants are able to compensate their deficits over time.

Rapid word-learning in normal-hearing and hearing-impaired children: effects of age, receptive vocabulary, and high-frequency amplification.

Science.gov (United States)

Pittman, A L; Lewis, D E; Hoover, B M; Stelmachowicz, P G

2005-12-01

This study examined rapid word-learning in 5- to 14-year-old children with normal and impaired hearing. The effects of age and receptive vocabulary were examined as well as those of high-frequency amplification. Novel words were low-pass filtered at 4 kHz (typical of current amplification devices) and at 9 kHz. It was hypothesized that (1) the children with normal hearing would learn more words than the children with hearing loss, (2) word-learning would increase with age and receptive vocabulary for both groups, and (3) both groups would benefit from a broader frequency bandwidth. Sixty children with normal hearing and 37 children with moderate sensorineural hearing losses participated in this study. Each child viewed a 4-minute animated slideshow containing 8 nonsense words created using the 24 English consonant phonemes (3 consonants per word). Each word was repeated 3 times. Half of the 8 words were low-pass filtered at 4 kHz and half were filtered at 9 kHz. After viewing the story twice, each child was asked to identify the words from among pictures in the slide show. Before testing, a measure of current receptive vocabulary was obtained using the Peabody Picture Vocabulary Test (PPVT-III). The PPVT-III scores of the hearing-impaired children were consistently poorer than those of the normal-hearing children across the age range tested. A similar pattern of results was observed for word-learning in that the performance of the hearing-impaired children was significantly poorer than that of the normal-hearing children. Further analysis of the PPVT and word-learning scores suggested that although word-learning was reduced in the hearing-impaired children, their performance was consistent with their receptive vocabularies. Additionally, no correlation was found between overall performance and the age of identification, age of amplification, or years of amplification in the children with hearing loss. Results also revealed a small increase in performance for both

Language ability in children with permanent hearing impairment: the influence of early management and family participation.

Science.gov (United States)

Watkin, Peter; McCann, Donna; Law, Catherine; Mullee, Mark; Petrou, Stavros; Stevenson, Jim; Worsfold, Sarah; Yuen, Ho Ming; Kennedy, Colin

2007-09-01

The goal was to examine the relationships between management after confirmation, family participation, and speech and language outcomes in the same group of children with permanent childhood hearing impairment. Speech, oral language, and nonverbal abilities, expressed as z scores and adjusted in a regression model, and Family Participation Rating Scale scores were assessed at a mean age of 7.9 years for 120 children with bilateral permanent childhood hearing impairment from a 1992-1997 United Kingdom birth cohort. Ages at institution of management and hearing aid fitting were obtained retrospectively from case notes. Compared with children managed later (> 9 months), those managed early (early did not have significantly higher scores for these outcomes. Family Participation Rating Scale scores showed significant positive correlations with language and speech intelligibility scores only for those with confirmation after 9 months and were highest for those with late confirmed, severe/profound, permanent childhood hearing impairment. Early management of permanent childhood hearing impairment results in improved language. Family participation is also an important factor in cases that are confirmed late, especially for children with severe or profound permanent childhood hearing impairment.

Attitude of Regular and Itinerant Teachers Towards the Inclusion of Hearing Impairment Children

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Kamal Parhoon

2014-12-01

Full Text Available Objectives: Inclusive education is a process of enabling all children to learn and participate effectively within mainstream school systems. It does not segregate children who have different abilities or needs. This article explores the attitudes of regular and itinerant teachers about inclusion of hearing impairment children in their schools in general education. Methods: In a descriptive Survey research design, the sample included 100 teachers (50 regular and 50 itinerant who were selected randomly, according to a multistage sampling method. Data was collected by using questionnaire with 32 questions regarding their attitudes. One-way Analysis of Variance and t-test were performed to obtain between- group comparisons. Results: The results indicated that the teacher's positive attitudes towards inclusive educational system of students with hearing impairment. Significant difference in attitudes was observed, based on the teaching experience, gender, level of teaching. The results also indicate that most teachers are agreeable to the inclusion of students with hearing impairment in their classrooms. Discussion: successful inclusion for hearing impairment children in regular classrooms entails the positive attitudes of Regular and itinerant teachers through a systematic programming within the classroom.

Status of Perceived Social Support and Quality of Life among Hearing-Impaired Adolescents

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Tayebeh Reyhani

2016-02-01

Full Text Available Background Annual four to five thousand babies are born with hearing loss in the Iran. Hearing impairment is a disability that affects the quality of life of people with this problem. These individuals need to support from family and friends because of their specific conditions that this received support has impact on their quality of life. This study was conducted to assess the status of perceived social support and quality of life of hearing-impaired adolescent. Material and Methods A cross-correlation study was performed with cluster and multi stage random sampling method on 83 students with hearing impairment who met the inclusion criteria of the study in Mashhad. The data collection tools included Pediatric quality of life inventory (adolescent form and perceived social support inventory (from family and friends.The data obtained from the questionnaires were analyzed through SPSS software version 16. Results The results showed that the majority of the most of adolescents with hearing impairment were reported moderate total quality of life (%51.8. But the majority of them reported perceived social support from family was moderate (%61.5 and from friends was week (%45.8. Also there was a significant relationship between category of total quality of life of adolescent viewpoint with perceived social support from family (P=0.056. Conclusion Based on the obtained results, the majority of the most of adolescents with hearing impairment were reported moderate total quality of life. Disability and condition of these persons affects quality of life of them, so need for adequate support from family, friends and society. Nurses play an important role in identifying and introduce these needs and condition and how to deal with them.

Can parenting practices predict externalizing behavior problems among children with hearing impairment?

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María J. Pino

2017-11-01

Full Text Available Objective: To identify possible differences in the level of externalizing behavior problems among children with and without hearing impairment and determine whether any relationship exists between this type of problem and parenting practices. Methods: The Behavior Assessment System for Children was used to evaluate externalizing variables in a sample of 118 boys and girls divided into two matched groups: 59 with hearing disorders and 59 normal-hearing controls. Results: Significant between-group differences were found in hyperactivity, behavioral problems, and externalizing problems, but not in aggression. Significant differences were also found in various aspects of parenting styles. A model for predicting externalizing behavior problems was constructed, achieving a predicted explained variance of 50%. Conclusion: Significant differences do exist between adaptation levels in children with and without hearing impairment. Parenting style also plays an important role.

Hearing dysfunction in heterozygous Mitf(Mi-wh) /+ mice, a model for Waardenburg syndrome type 2 and Tietz syndrome.

Science.gov (United States)

Ni, Christina; Zhang, Deming; Beyer, Lisa A; Halsey, Karin E; Fukui, Hideto; Raphael, Yehoash; Dolan, David F; Hornyak, Thomas J

2013-01-01

The human deafness-pigmentation syndromes, Waardenburg syndrome (WS) type 2a, and Tietz syndrome are characterized by profound deafness but only partial cutaneous pigmentary abnormalities. Both syndromes are caused by mutations in MITF. To illuminate differences between cutaneous and otic melanocytes in these syndromes, their development and survival in heterozygous Microphthalmia-White (Mitf(Mi-wh) /+) mice were studied and hearing function of these mice characterized. Mitf(Mi-wh) /+ mice have a profound hearing deficit, characterized by elevated auditory brainstem response thresholds, reduced distortion product otoacoustic emissions, absent endocochlear potential, loss of outer hair cells, and stria vascularis abnormalities. Mitf(Mi-wh) /+ embryos have fewer melanoblasts during embryonic development than their wild-type littermates. Although cochlear melanocytes are present at birth, they disappear from the Mitf(Mi-wh) /+ cochlea between P1 and P7. These findings may provide insight into the mechanism of melanocyte and hearing loss in human deafness-pigmentation syndromes such as WS and Tietz syndrome and illustrate differences between otic and follicular melanocytes. © 2012 John Wiley & Sons A/S.

Cochlear Implantation in Patients With Usher Syndrome Type IIa Increases Performance and Quality of Life

NARCIS (Netherlands)

Hartel, B.P.; Nierop, J.W.I. van; Huinck, W.J.; Rotteveel, L.J.C.; Mylanus, E.A.M.; Snik, A.F.M.; Kunst, H.P.M.; Pennings, R.J.E.

2017-01-01

OBJECTIVES: Usher syndrome type IIa (USH2a) is characterized by congenital moderate to severe hearing impairment and retinitis pigmentosa. Hearing rehabilitation starts in early childhood with the application of hearing aids. In some patients with USH2a, severe progression of hearing impairment

Siblings of the Hearing Impaired: Perspectives for Parents.

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Atkins, Dale V.

1987-01-01

The author describes the concerns and needs of siblings of hearing-impaired children, offering practical suggestions for parents, educators, and mental health and community service workers. Examples of successful programs for siblings that provide emotional support, training and information about their siblings' disability, and effective…

Selective attention in normal and impaired hearing.

Science.gov (United States)

Shinn-Cunningham, Barbara G; Best, Virginia

2008-12-01

A common complaint among listeners with hearing loss (HL) is that they have difficulty communicating in common social settings. This article reviews how normal-hearing listeners cope in such settings, especially how they focus attention on a source of interest. Results of experiments with normal-hearing listeners suggest that the ability to selectively attend depends on the ability to analyze the acoustic scene and to form perceptual auditory objects properly. Unfortunately, sound features important for auditory object formation may not be robustly encoded in the auditory periphery of HL listeners. In turn, impaired auditory object formation may interfere with the ability to filter out competing sound sources. Peripheral degradations are also likely to reduce the salience of higher-order auditory cues such as location, pitch, and timbre, which enable normal-hearing listeners to select a desired sound source out of a sound mixture. Degraded peripheral processing is also likely to increase the time required to form auditory objects and focus selective attention so that listeners with HL lose the ability to switch attention rapidly (a skill that is particularly important when trying to participate in a lively conversation). Finally, peripheral deficits may interfere with strategies that normal-hearing listeners employ in complex acoustic settings, including the use of memory to fill in bits of the conversation that are missed. Thus, peripheral hearing deficits are likely to cause a number of interrelated problems that challenge the ability of HL listeners to communicate in social settings requiring selective attention.

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

DEFF Research Database (Denmark)

Oxenham, Andrew J.; Dau, Torsten

2004-01-01

curvature. Results from 12 listeners with sensorineural hearing loss showed reduced masker phase effects, when compared with data from normal-hearing listeners, at both 250- and 1000-Hz signal frequencies. The effects of hearing impairment on phase-related masking differences were not well simulated...... are affected by a common underlying mechanism, presumably related to cochlear outer hair cell function. The results also suggest that normal peripheral compression remains strong even at 250 Hz....

Newborn hearing screening and strategy for early detection of hearing loss in infants.

Science.gov (United States)

Jakubíková, Janka; Kabátová, Zuzana; Pavlovcinová, Gabriela; Profant, Milan

2009-04-01

More than 80% of permanent hearing losses (HL) in children are congenital. Newborn hearing screening (NHS) is the best method for early detection of suspected hearing loss. If the NHS is not universal more than 30% permanent hearing losses are not identified. There are various methods of NHS: otoacoustic emissions (TEOAE, DPOAE) and automatic auditory brainstem response (AABR). After hearing screening, and when hearing loss is suspected, tympanometry and audiological methods then used for determination of hearing threshold; these include ABR, ASSR or/and behavioral methods. The goal of this study is to evaluate the influence of UNHS on the early detection of hearing loss in children before and after the implementation of obligatory universal newborn hearing screening in Slovakia, and also on the etiologic evaluation of hearing impaired infants identified by screening. In Slovakia NHS started in 1998 and was provided in ENT departments. From May 1, 2006 UNHS has been mandatory in Slovakia, using two stages TEOAE in all newborn departments in Slovakia (64 newborn departments). In year 2005--42% of newborns in Slovakia were screened, in 2006--66% newborns and in 2007--94, 99% (three small newborn departments do not yet have equipment for OAE screening). For determination of hearing thresholds ASSR are used in two ENT departments and ABR in the other four ENT departments. Comparing the number of identified cases with bilateral severe permanent HL or deafness before and after UNHS, 22.8% more cases of PHL were identified in the first year of UNHS. Also the average age of diagnosis of PHL was lower. In the year 2007, 94% of newborns were screened. We found 0.947/1000 newborns with bilateral severe PHL (35.9%) more than before UNHS). After audiologic and etiologic assessment of the 76 infants who failed screening, 5 (6.58%) were found to have normal hearing, 16 (22.54%) had unilateral and 55 (77.46%) had bilateral SNHL. A non-syndromic genetic cause was present in 25

HIV/AIDS among Adolescents with Hearing Impairment in Nigeria ...

African Journals Online (AJOL)

Toshiba

low literacy level, cycle of poverty among people with hearing impairment ... some strategies, such as participatory approaches, deaf-friendly .... incapable of learning. ... that the greatest life challenge to any persons with disability is social.

Is hearing loss a feature of Joubert syndrome, a ciliopathy?

NARCIS (Netherlands)

Kroes, H.Y.; Van Zanten, B.G.A.; De Ru, S.A.; Boon, M.; Mancini, G.M.S.; van der Knaap, M.S.; Poll-The, B.; Lindhout, D.

2010-01-01

Objective: To assess if hearing loss is a feature of Joubert syndrome (JBS), one of the ciliopathies and therefore possibly associated with hearing loss. Design: Retrospective case series. Setting: University Children's Hospital. Patients: Dutch patients with JBS. Main outcome measures: Audiological

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

Science.gov (United States)

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…

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

Science.gov (United States)

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

2017-04-01

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

Zellweger Syndrome

Science.gov (United States)

... swallow. Some babies will be born with glaucoma, retinal degeneration, and impaired hearing. Jaundice and gastrointestinal bleeding also may occur. Treatment There is no cure for Zellweger syndrome, nor ...

The economics of screening infants at risk of hearing impairment: an international analysis.

Science.gov (United States)

Burke, Martyn J; Shenton, Ruth C; Taylor, Matthew J

2012-02-01

Hearing impairment in children across the world constitutes a particularly serious obstacle to their optimal development and education, including language acquisition. Around 0.5-6 in every 1000 neonates and infants have congenital or early childhood onset sensorineural deafness or severe-to-profound hearing impairment, with significant consequences. Therefore, early detection is a vitally important element in providing appropriate support for deaf and hearing-impaired babies that will help them enjoy equal opportunities in society alongside all other children. This analysis estimates the costs and effectiveness of various interventions to screen infants at risk of hearing impairment. The economic analysis used a decision tree approach to determine the cost-effectiveness of newborn hearing screening strategies. Two unique models were built to capture different strategic screening decisions. Firstly, the cost-effectiveness of universal newborn hearing screening (UNHS) was compared to selective screening of newborns with risk factors. Secondly, the cost-effectiveness of providing a one-stage screening process vs. a two-stage screening process was investigated. Two countries, the United Kingdom and India, were used as case studies to illustrate the likely cost outcomes associated with the various strategies to diagnose hearing loss in infants. In the UK, the universal strategy incurs a further cost of approximately £2.3 million but detected an extra 63 cases. An incremental cost per case detected of £36,181 was estimated. The estimated economic burden was substantially higher in India when adopting a universal strategy due to the higher baseline prevalence of hearing loss. The one-stage screening strategy accumulated an additional 13,480 and 13,432 extra cases of false-positives, in the UK and India respectively when compared to a two-stage screening strategy. This represented increased costs by approximately £1.3 million and INR 34.6 million. The cost

Is hearing loss a feature of Joubert syndrome, a ciliopathy?

NARCIS (Netherlands)

Kroes, Hester Y.; Van Zanten, Bert G. A.; De Ru, Sander A.; Boon, Maartje; Mancini, Grazia M. S.; Van der Knaap, Marjo S.; Poll-The, Bwee Tien; Lindhout, Dick

Objective To assess if hearing loss is a feature of Joubert syndrome (JBS). one of the ciliopathies and therefore possibly associated with hearing loss Design: Retrospective case series. Setting University Children's Hospital Patients Dutch patients with JBS. Main outcome measures Audiological data.

Is hearing loss a feature of Joubert syndrome, a ciliopathy?

NARCIS (Netherlands)

Kroes, Hester Y.; van Zanten, Bert G. A.; de Ru, Sander A.; Boon, Maartje; Mancini, Grazia M. S.; van der Knaap, Marjo S.; Poll-The, Bwee Tien; Lindhout, Dick

2010-01-01

Objective To assess if hearing loss is a feature of Joubert syndrome (JBS). one of the ciliopathies and therefore possibly associated with hearing loss Design: Retrospective case series. Setting University Children's Hospital Patients Dutch patients with JBS. Main outcome measures Audiological data.

Hearing loss in Waardenburg syndrome: a systematic review.

Science.gov (United States)

Song, J; Feng, Y; Acke, F R; Coucke, P; Vleminckx, K; Dhooge, I J

2015-06-22

Waardenburg syndrome (WS) is a rare genetic disorder characterized by hearing loss (HL) and pigment disturbances of hair, skin and iris. Classifications exist based on phenotype and genotype. The auditory phenotype is inconsistently reported among the different Waardenburg types and causal genes, urging the need for an up-to-date literature overview on this particular topic. We performed a systematic review in search for articles describing auditory features in WS patients along with the associated genotype. Prevalences of HL were calculated and correlated with the different types and genes of WS. Seventy-three articles were included, describing 417 individual patients. HL was found in 71.0% and was predominantly bilateral and sensorineural. Prevalence of HL among the different clinical types significantly differed (WS1: 52.3%, WS2: 91.6%, WS3: 57.1%, WS4: 83.5%). Mutations in SOX10 (96.5%), MITF (89.6%) and SNAI2 (100%) are more frequently associated with hearing impairment than other mutations. Of interest, the distinct disease-causing genes are able to better predict the auditory phenotype compared with different clinical types of WS. Consequently, it is important to confirm the clinical diagnosis of WS with molecular analysis in order to optimally inform patients about the risk of HL. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

The Effectiveness of a Group Counseling Program on the Mental Health of Parents of Hearing Impaired Children

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Dr. Mahshid Foroughan

2007-12-01

Full Text Available Background and Aim: Most of the studies indicates that the parents of the hearing impaired children show many mental health problems after the diagnosis of their children's hearing impairment. Counselling with the parents of the hearing impaired children is one of the most important goals of any early intervention program. This paper describes a study to determine the effectiveness of a group counselling programme for parents of hearing impaired children. Materials and Method: It was a semi-experimental study with a single group pretest-post test design. The participants were all the parents of hearing impaired children attending in an early intervention center. First the parents' mental health were assessed.Then the group counselling program was implemented. Program has involved six weekly 1.5 hour sessions. The format of each session included both lecture presentation and group discussion using cognitive behavioral procedure. Subjects were assessed before and immediately after group therapy by means of General Health Questionnaire(GHQ and Symptom Check List 90 (SCL-90 questionnaires. Resuts: The first part of the project had shown that over the half of the parents had considerable psychosocial morbidity. Comparisons showed a significant reduction from pretreatment to posttreatment in depression, anxiety and most of other psychological problems. Conclusion: The study supports the effectiveness of group therapy programs in the treatment of parents of hearing impaired children. Concerning the progress of early detection programs for the children's hearing impairment more studies should be done in the field of counseling with their parents.

The influence of Pilates exercises on body balance in the standing position of hearing impaired people.

Science.gov (United States)

Walowska, Jagoda; Bolach, Bartosz; Bolach, Eugeniusz

2017-11-13

Hearing impairment may affect the body posture maintenance. The aim of the study was to evaluate the effect of modified Pilates exercise program on the body posture maintenance in hearing impaired people. Eighty students (aged 13-24) were enrolled and randomly allocated into two groups: test group (n = 41) which attended an original program based on modified Pilates exercises and control group (n = 39) which attended standard physical education classes. Stabilographic tests were conducted at baseline and after 6-week training program. Both groups showed improved control of body balance in a standing position manifested in reductions of the length of path, surface area, and speed of deflection. Modified Pilates program was significantly more effective in improving body balance control in relaxed posture and with feet together than standard physical education classes. The greater efficiency of the modified Pilates program was expressed in a significant improvement in balance control parameters, i.e., path length, surface area, and speed of deflection. The modified Pilates program was more effective in improving body balance control in the hearing impaired people than standard physical education classes. Modification of physical activity recommendations for hearing impaired students may be considered; however, further research is required. Implications for Rehabilitation Hearing impairment impacts the mental, social and, physical spheres of life as well as deteriorates equivalent reactions and the way body posture is maintained. In hearing impaired people, control of body balance and muscle coordination is often disturbed, thus more attention should be paid to exercises associated with balance which may improve the ability to learn and develop motor skills. Modified Pilates program was significantly more effective in improving body balance control than standard physical education classes in hearing impaired people.

Personal Experiences of Hearing-impaired People in Higher ...

African Journals Online (AJOL)

This qualitative paper analyses the personal experiences of hearing-impaired people in accessing, participating and completing higher education in Zimbabwe, and the authors' experiences in the education of students with disabilities. A 6As' framework is informed by the 4As framework of Tomaševski (2001), namely ...

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

DEFF Research Database (Denmark)

Locsei, Gusztav; Pedersen, Julie Hefting; Laugesen, Søren

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...... 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...

Hearing impairment and deafness among HIV infected children and ...

African Journals Online (AJOL)

Hearing impairment and deafness among HIV infected children and adolescents in Harare, Zimbabwe. C Chidziva, J Matsekete, T Bandason, S Shamu, T Dzongodza, N Matinhira, HA Mujuru, C Kunzekwenyika, M Wellington, R Luthy, C Prescott, RA Ferrand ...

A portable digital speech-rate converter for hearing impairment.

Science.gov (United States)

Nejime, Y; Aritsuka, T; Imamura, T; Ifukube, T; Matsushima, J

1996-06-01

A real-time hand-sized portable device that slows speech speed without changing the pitch is proposed for hearing impairment. By using this device, people can listen to fast speech at a comfortable speed. A combination of solid-state memory recording and real-time digital signal processing with a single chip processor enables this unique function. A simplified pitchsynchronous, time-scale-modification algorithm is proposed to minimize the complexity of the DSP operation. Unlike the traditional algorithm, this dynamic-processing algorithm reduces distortion even when the expansion rate is only just above 1. Seven out of 10 elderly hearing-impaired listeners showed improvement in a sentence recognition test when using speech-rate conversion with the largest expansion rate, although no improvement was observed in a word recognition test. Some subjects who showed large improvement had limited auditory temporal resolution, but the correlation was not significant. The results suggest that, unlike conventional hearing aids, this device can be used to overcome the deterioration of auditory ability by improving the transfer of information from short-term (echoic) memory into a more stable memory trace in the human auditory system.

Use of nouns and verbs in the oral narrative of individuals with hearing impairment and normal hearing between 5 and 11 years of age

Directory of Open Access Journals (Sweden)

Erica Endo Amemiya

Full Text Available CONTEXT AND OBJECTIVE: Nouns and verbs indicate actions in oral communication. However, hearing impairment can compromise the acquisition of oral language to such an extent that appropriate use of these can be challenging. The objective of this study was to compare the use of nouns and verbs in the oral narrative of hearing-impaired and hearing children. DESIGN AND SETTING: Analytical cross-sectional study at the Department of Speech-Language and Hearing Sciences, Universidade Federal de São Paulo. METHODS: Twenty-one children with moderate to profound bilateral neurosensory hearing impairment and twenty-one with normal hearing (controls were matched according to sex, school year and school type. A board showing pictures was presented to each child, to elicit a narrative and measure their performance in producing nouns and verbs. RESULTS: Twenty-two (52.4% of the subjects were males. The mean age was 8 years (standard deviation, SD = 1.5. Comparing averages between the groups of boys and girls, we did not find any significant difference in their use of nouns, but among verbs, there was a significant difference regarding use of the imperative (P = 0.041: more frequent among boys (mean = 2.91. There was no significant difference in the use of nouns and verbs between deaf children and hearers, in relation to school type. Regarding use of the indicative, there was a nearly significant trend (P = 0.058. CONCLUSION: Among oralized hearing-impaired children who underwent speech therapy, their performance regarding verbs and noun use was similar to that of their hearing counterparts.

Processing of Binaural Pitch Stimuli in Hearing-Impaired Listeners

DEFF Research Database (Denmark)

Santurette, Sébastien; Dau, Torsten

2009-01-01

Binaural pitch is a tonal sensation produced by introducing a frequency-dependent interaural phase shift in binaurally presented white noise. As no spectral cues are present in the physical stimulus, binaural pitch perception is assumed to rely on accurate temporal fine structure coding and intact...... binaural integration mechanisms. This study investigated to what extent basic auditory measures of binaural processing as well as cognitive abilities are correlated with the ability of hearing-impaired listeners to perceive binaural pitch. Subjects from three groups (1: normal-hearing; 2: cochlear...... hearingloss; 3: retro-cochlear impairment) were asked to identify the pitch contour of series of five notes of equal duration, ranging from 523 to 784 Hz, played either with Huggins’ binaural pitch stimuli (BP) or perceptually similar, but monaurally detectable, pitches (MP). All subjects from groups 1 and 2...

Auditory and Non-Auditory Contributions for Unaided Speech Recognition in Noise as a Function of Hearing Aid Use.

Science.gov (United States)

Gieseler, Anja; Tahden, Maike A S; Thiel, Christiane M; Wagener, Kirsten C; Meis, Markus; Colonius, Hans

2017-01-01

Differences in understanding speech in noise among hearing-impaired individuals cannot be explained entirely by hearing thresholds alone, suggesting the contribution of other factors beyond standard auditory ones as derived from the audiogram. This paper reports two analyses addressing individual differences in the explanation of unaided speech-in-noise performance among n = 438 elderly hearing-impaired listeners ( mean = 71.1 ± 5.8 years). The main analysis was designed to identify clinically relevant auditory and non-auditory measures for speech-in-noise prediction using auditory (audiogram, categorical loudness scaling) and cognitive tests (verbal-intelligence test, screening test of dementia), as well as questionnaires assessing various self-reported measures (health status, socio-economic status, and subjective hearing problems). Using stepwise linear regression analysis, 62% of the variance in unaided speech-in-noise performance was explained, with measures Pure-tone average (PTA), Age , and Verbal intelligence emerging as the three most important predictors. In the complementary analysis, those individuals with the same hearing loss profile were separated into hearing aid users (HAU) and non-users (NU), and were then compared regarding potential differences in the test measures and in explaining unaided speech-in-noise recognition. The groupwise comparisons revealed significant differences in auditory measures and self-reported subjective hearing problems, while no differences in the cognitive domain were found. Furthermore, groupwise regression analyses revealed that Verbal intelligence had a predictive value in both groups, whereas Age and PTA only emerged significant in the group of hearing aid NU.

Reaching Out to Develop the Creative Reading Potential of the Hearing Impaired in Community Colleges.

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Norton, Audree L.

Hearing impaired students need the opportunity to develop their creative reading ability (the ability to acquire ideas and information through printed words, to apply them to personal problems, and to attempt to resolve those problems). Although many hearing impaired students have the intelligence to be successful readers, they have been…

Career Education of Hearing-Impaired Students: A Review.

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Bullis, Michael, Ed.; Watson, Douglas, Ed.

This monograph reviews the many efforts that have been made in the fields of education and vocational rehabilitation to develop and refine career preparation services for hearing-impaired individuals. The following papers are included in the volume: "Career Education: A Literature Review," by Charlene Dwyer; "A Current Profile of Career Education…

Risk behaviors and sexual abuse among men and woman with visual or hearing impairments

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Silvia Susana Robles Montijo

2017-06-01

Full Text Available The present study proposed a descriptive analysis of sociodemographic and family variables, as well as risky sexual behaviors and sexual abuse in a sample of young Mexicans with hearing or visual impairments, examining differences based on gender and type of disability of the participants. 128 young persons (64 hearing impaired and 64 visually impaired of whom, 53.2% were male and 86.6% were single, were included in the study. The instruments used were adapted linguistically to be answered by young people with hearing disabilities, and translated into Braille for visually impaired young people. The results show that participants maintain an active sex life with a risk profile that is characterized by the early onset of sexual intercourse, lack of planning their first sexual encounter, underuse of condoms and victimization of some form of sexual abuse. The risk was higher in women and among those with visual disabilities.

Metabolic Syndrome Increases the Risk of Sudden Sensorineural Hearing Loss in Taiwan: A Case-Control Study.

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Chien, Chen-Yu; Tai, Shu-Yu; Wang, Ling-Feng; Hsi, Edward; Chang, Ning-Chia; Wu, Ming-Tsang; Ho, Kuen-Yao

2015-07-01

Sudden sensorineural hearing loss has been reported to be associated with diabetes mellitus, hypertension, and hyperlipidemia in previous studies. The aim of this study was to examine whether metabolic syndrome increases the risk of sudden sensorineural hearing loss in Taiwan. A case-control study. Tertiary university hospital. We retrospectively investigated 181 cases of sudden sensorineural hearing loss and 181 controls from the Department of Otorhinolaryngology, Kaohsiung Medical University Hospital, in southern Taiwan from 2010 to 2012, comparing their clinical variables. We analyzed the relationship between metabolic syndrome and sudden sensorineural hearing loss. Metabolic syndrome was defined according to the National Cholesterol Education Program Adult Treatment Panel III with Asian modifications. The demographic and clinical characteristics, audiometry results, and outcome were reviewed. Subjects with metabolic syndrome had a 3.54-fold increased risk (95% confidence interval [CI] = 2.00-6.43, P diabetes mellitus, hypertension, and hyperlipidemia. With increases in the number of metabolic syndrome components, the risk of sudden sensorineural hearing loss increased (P for trend Vertigo was associated with a poor outcome (P = .02; 95% CI = 1.13~5.13, adjusted odds ratio = 2.39). The hearing loss pattern may influence the outcome of sudden sensorineural hearing loss (P Vertigo and total hearing loss were indicators of a poor outcome in sudden sensorineural hearing loss. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2015.

Cockayne syndrome group B (Csb) and group a (Csa) deficiencies predispose to hearing loss and cochlear hair cell degeneration in mice.

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Nagtegaal, A Paul; Rainey, Robert N; van der Pluijm, Ingrid; Brandt, Renata M C; van der Horst, Gijsbertus T J; Borst, J Gerard G; Segil, Neil

2015-03-11

Sensory hair cells in the cochlea, like most neuronal populations that are postmitotic, terminally differentiated, and non-regenerating, depend on robust mechanisms of self-renewal for lifelong survival. We report that hair cell homeostasis requires a specific sub-branch of the DNA damage nucleotide excision repair pathway, termed transcription-coupled repair (TCR). Cockayne syndrome (CS), caused by defects in TCR, is a rare DNA repair disorder with a broad clinical spectrum that includes sensorineural hearing loss. We tested hearing and analyzed the cellular integrity of the organ of Corti in two mouse models of this disease with mutations in the Csb gene (CSB(m/m) mice) and Csa gene (Csa(-/-) mice), respectively. Csb(m/m) and Csa(-/-) mice manifested progressive hearing loss, as measured by an increase in auditory brainstem response thresholds. In contrast to wild-type mice, mutant mice showed reduced or absent otoacoustic emissions, suggesting cochlear outer hair cell impairment. Hearing loss in Csb(m/m) and Csa(-/-) mice correlated with progressive hair cell loss in the base of the organ of Corti, starting between 6 and 13 weeks of age, which increased by 16 weeks of age in a basal-to-apical gradient, with outer hair cells more severely affected than inner hair cells. Our data indicate that the hearing loss observed in CS patients is reproduced in mouse models of this disease. We hypothesize that accumulating DNA damage, secondary to the loss of TCR, contributes to susceptibility to hearing loss. Copyright © 2015 the authors 0270-6474/15/354280-07$15.00/0.

Acoustics and sociolinguistics: Patterns of communication in hearing impairing classrooms

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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.

Neurocognitive impairment in deficit and non-deficit schizophrenia: a meta-analysis.

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Bora, E; Binnur Akdede, B; Alptekin, K

2017-10-01

Most studies suggested that patients with deficit schizophrenia have more severe impairment compared with patients with non-deficit schizophrenia. However, it is not clear whether deficit and non-deficit schizophrenia are associated with differential neurocognitive profiles. The aim of this meta-analytic review was to compare cognitive performances of deficit and non-deficit patients with each other and with healthy controls. In the current meta-analysis, differences in cognitive abilities between 897 deficit and 1636 non-deficit patients with schizophrenia were examined. Cognitive performances of 899 healthy controls were also compared with 350 patients with deficit and 592 non-deficit schizophrenia. Both deficit (d = 1.04-1.53) and non-deficit (d = 0.68-1.19) schizophrenia were associated with significant deficits in all cognitive domains. Deficit patients underperformed non-deficit patients in all cognitive domains (d = 0.24-0.84) and individual tasks (d = 0.39-0.93). The relationship between deficit syndrome and impairment in olfaction, social cognition, verbal fluency, and speed-based cognitive tasks were relatively stronger. Our findings suggest that there is consistent evidence for a significant relationship between deficit syndrome and more severe cognitive impairment in schizophrenia.

Hearing Impairment

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... Ear Hears Think about how you can feel speakers vibrate on your sound system or feel your throat vibrate when you speak. Sound, which is made up of invisible waves of energy, causes these vibrations. Hearing begins when sound waves that travel through ...

Assistive Technologies for Improving Communication of Hearing Impairment in the Higher Education in Panama

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Lineth Alain

2016-12-01

Full Text Available The ability to communicate, specifically the gift of hearing, is a necessity often taken for granted. A lack of sense of hearing affects the intellectual and emotional development of the human being who suffers from it. It prevents the fluid exchange of knowledge, thoughts and ideas that allow personal growth and development. This article emerges due to an interest in providing assistive technologies that can be considered to improve communication among hearing impaired and normal hearing listeners in the class-room of a higher education level in the Republic of Panama. Information has been compiled from various primary and secondary sources highlighting the communication problem facing this group of disabled people. Information about the situation of hearing impairment, laws, organizations, the reality with the higher education system, and finally, we will talk about Information and Communication Technologies (TICs that will work as technology support in order to improve communication in the classroom in higher education among normal-hearing and deaf people.

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

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

2016-06-01

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

CE: Original Research: Understanding the Hospital Experience of Older Adults with Hearing Impairment.

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Funk, Amy; Garcia, Christina; Mullen, Tiara

2018-06-01

: Background: Older hospitalized adults with hearing impairment are vulnerable to adverse outcomes. These patients are at risk for being labeled confused, experiencing a loss of control, experiencing heightened fear and anxiety, and misunderstanding the plan of care. This qualitative study sought to assess the hospital experience of older adults with hearing impairment in order to formulate suggestions for improving nursing care. Open-ended interviews were conducted with eight participants, ages 70 to 95 years, who were identified as having a hearing impairment and were admitted as inpatients to a midwestern medical center. Through data analysis, three common themes emerged: health care communication difficulties, passivity and vulnerability, and frustration with family. Nurses will benefit from having a deeper understanding of the hospital experience of this vulnerable population. Efforts to address their needs can be accomplished through the following nursing actions: assess, accommodate, educate, empower, and advocate.

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

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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…

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

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Lalwani, A.K.; Brister, J.R.; Fex, J.; Grundfast, K.M.; Pikus, A.T.; Ploplis, B.; San Agustin, T.; Skarka, H.; Wilcox, E.R. [National Institutes of Health, Bethesda, MD (United States)

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.

The Efficacy of Group Play Therapy on the Social Skills of Pre-School Hearing-Impaired Children

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Gita Movallali

2015-04-01

Full Text Available Objective: The purpose of the present study was to investigate of the efficacy of group play therapy on the social skills of pre-school hearing-impaired children. Materials & Methods: The present research was a semi-experimental study with pre-test, post-test design and control group. The participants were 30 male hearing-impaired children from pre-schools centers in Varamin and Gharchak provinces using an available method. Subjects were randomly divided into experimental and control groups, each group consisting of 15 children. The experimental group received 12 sessions of group play therapy and the control group did not. The instruments were done using the Raven coloure progressive matrices test and social skills rating scale. The data were recorded and statistically analyzed using MANCOVA. Results: The results of MANCOVA showed that group play therapy had a significant effect on the social skills of hearing-impaired children (P<0.001. The results also revealed that group play therapy had a positive and significant effect on all subscales of social skills in these children: cooperation, self-assertiveness and self-control (P<0.001. Conclusion: Group play therapy can improve the social skills of hearing-impaired children. It is recommended that planning play therapy for hearing-impaired children receives serious attention .

Cognitive skills and the effect of noise on perceived effort in employees with aided hearing impairment and normal hearing

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Håkan Hua

2014-01-01

Full Text Available The aim of the following study was to examine the relationship between working memory capacity (WMC, executive functions (EFs and perceived effort (PE after completing a work-related task in quiet and in noise in employees with aided hearing impairment (HI and normal hearing. The study sample consisted of 20 hearing-impaired and 20 normally hearing participants. Measures of hearing ability, WMC and EFs were tested prior to performing a work-related task in quiet and in simulated traffic noise. PE of the work-related task was also measured. Analysis of variance was used to analyze within- and between-group differences in cognitive skills, performance on the work-related task and PE. The presence of noise yielded a significantly higher PE for both groups. However, no significant group differences were observed in WMC, EFs, PE and performance in the work-related task. Interestingly, significant negative correlations were only found between PE in the noise condition and the ability to update information for both groups. In summary, noise generates a significantly higher PE and brings explicit processing capacity into play, irrespective of hearing. This suggest that increased PE involves other factors such as type of task that is to be performed, performance in the cognitive skill required solving the task at hand and whether noise is present. We therefore suggest that special consideration in hearing care should be made to the individual′s prerequisites on these factors in the labor market.

Identification of a novel mutation in WFS1 in a family affected by low-frequency hearing impairment

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Kunz, Juergen; Marquez-Klaka, Ben; Uebe, Steffen; Volz-Peters, Anja; Berger, Roswitha; Rausch, Peter

2003-04-09

Previously we confirmed linkage of autosomal dominantly inherited low-frequency sensorineural hearing impairment (LFSNHI) in a German family to the genetic locus DFNA6/DFNA14 on chromosome 4p16.3 close to the markers D4S432 and D4S431. Analysis of data from the Human Genome Project, showed that WFS1 is located in this region. Mutations in WFS1 are known to be responsible for Wolfram syndrome (DIDMOAD, MIM no. 606201), which follows an autosomal recessive trait. Studies in low-frequency hearing loss families showed that mutations in WFS1 were responsible for the phenotype. In all affected family members analysed, we detected a missense mutation in WFS1 (K705N) and therefore confirm the finding that the majority of mutations responsible for LFSNHI are missense mutations which localise to the C-terminal domain of the protein.

Identification of a novel mutation in WFS1 in a family affected by low-frequency hearing impairment

International Nuclear Information System (INIS)

Kunz, Juergen; Marquez-Klaka, Ben; Uebe, Steffen; Volz-Peters, Anja; Berger, Roswitha; Rausch, Peter

2003-01-01

Previously we confirmed linkage of autosomal dominantly inherited low-frequency sensorineural hearing impairment (LFSNHI) in a German family to the genetic locus DFNA6/DFNA14 on chromosome 4p16.3 close to the markers D4S432 and D4S431. Analysis of data from the Human Genome Project, showed that WFS1 is located in this region. Mutations in WFS1 are known to be responsible for Wolfram syndrome (DIDMOAD, MIM no. 606201), which follows an autosomal recessive trait. Studies in low-frequency hearing loss families showed that mutations in WFS1 were responsible for the phenotype. In all affected family members analysed, we detected a missense mutation in WFS1 (K705N) and therefore confirm the finding that the majority of mutations responsible for LFSNHI are missense mutations which localise to the C-terminal domain of the protein

Fine-structure processing, frequency selectivity and speech perception in hearing-impaired listeners

DEFF Research Database (Denmark)

Strelcyk, Olaf; Dau, Torsten

2008-01-01

Hearing-impaired people often experience great difficulty with speech communication when background noise is present, even if reduced audibility has been compensated for. Other impairment factors must be involved. In order to minimize confounding effects, the subjects participating in this study...... consisted of groups with homogeneous, symmetric audiograms. The perceptual listening experiments assessed the intelligibility of full-spectrum as well as low-pass filtered speech in the presence of stationary and fluctuating interferers, the individual's frequency selectivity and the integrity of temporal...... modulation were obtained. In addition, these binaural and monaural thresholds were measured in a stationary background noise in order to assess the persistence of the fine-structure processing to interfering noise. Apart from elevated speech reception thresholds, the hearing impaired listeners showed poorer...

Consanguineous Marriage Among the Parents of Hearing Impaired Students in Baghcheban Primary Schools

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Mansoureh Nikbakht

2005-06-01

Full Text Available Objective: Genetic studies show that consanguineous marriage can increase the probability of incidence of genetic impairments such as hearing impairments. The target of this study is to identify the prevalence of consanguinity among the parents of hearing impaired students in primary schools. Materials and Methods: We selected all of deaf students of Tehran (614 students. Their mothers answered to questionnaires. The questions were about Risk Factors of deafness in mother pregnancy or in neonatal period. Results: from 614 students, 389 parents of them (64% had consanguineous marriage and 223 person (36% didn’t have this factor. 2 person did not answer to this question. In this study we observed that there is 32.3% family history of hearing loss, 29.2%deaf sister and brother, 17% ear infection history. Other risk factors were studied too. Also there is significant correlation between consanguinity and more than one deaf children in the family (p<0.005. Conclusion: According to high incidence of consanguinity (64%that was observed in this study it may be one of most important causes of sensory neural hearing loss in children, so we should give enough information about this problem to the people.

The effectiveness of mental rehabilitation based on positive thinking skills training on increasing happiness in hearing impaired adolescents

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Mohammad Rostami

2014-08-01

Full Text Available Background and Aim: Hearing impairment can have an impact on adolescents’ quality of life and can lead to their isolation and depression. The main purpose of this research was to study the effectiveness of a mental rehabilitation program based on positive thinking skills training on increasing happiness in a group of hearing impaired girls and boys.Methods: In this experimental study, pre- and post-test plan with a control group was used. All of the hearing impaired students (girls and boys in all high schools of southern parts of Tehran, Iran, in year 2012-13 were our statistical research community. 48 hearing impaired girls and boys were selected by multistage sampling including stratified, purposive, and random sampling. They were randomly divided into two groups of experimental and control. Each group consists of 12 boys and 12 girls. Positive thinking skills were trained to experimental groups during eight 45-minutes sessions, twice a week. We used the Oxford happiness questionnaire to assess the level of happiness.Results: Using analysis of covariance showed that positive thinking skills training had meaningful and positive effect on increasing happiness of hearing impaired boys and girls in the experimental groups (p<0.01. Also, the mean happiness scores of boys and girls were meaningfully different (p<0.01.Conclusion: Positive thinking skills training increases the happiness scores of hearing impaired adolescents. So, the approach taken in this study can be considered as an appropriate method for psychological-education interventions, counseling and treatment in hearing impaired adolescent.

Non-syndromic hearing loss caused by the dominant cis mutation R75Q with the recessive mutation V37I of the GJB2 (Connexin 26) gene.

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Kim, Juwon; Jung, Jinsei; Lee, Min Goo; Choi, Jae Young; Lee, Kyung-A

2015-06-19

GJB2 alleles containing two cis mutations have been rarely found in non-syndromic hearing loss. Herein, we present a Korean patient with non-syndromic hearing loss caused by the R75Q cis mutation with V37I, which arose de novo in the father and was inherited by the patient. Biochemical coupling and hemichannel permeability assays were performed after molecular cloning and transfection of HEK293T cells. Student's t-tests or analysis of variance followed by Tukey's multiple comparison test was used as statistical analysis. Biochemical coupling was significantly reduced in connexin 26 (Cx26)-R75Q- and Cx26-V37I-transfected cells, with greater extent in Cx26-R75Q and Cx26-R75Q+V37I cells. Interestingly, our patient and his father with the mutations had more residual hearing compared with patients with the dominant mutation alone. Although the difference in hemichannel activity between R75Q alone and R75Q in combination with V37I failed to reach significance, it is of note that there is a possibility that V37I located upstream of R75Q might have the ability to ameliorate R75Q expression. Our study emphasizes the importance of cis mutations with R75Q, as the gene effect of R75Q can be modulated depending on the type of additional mutation.

Middle and inner ear malformations in mutation-proven branchio-oculo-facial (BOF) syndrome: case series and review of the literature.

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Carter, Melissa T; Blaser, Susan; Papsin, Blake; Meschino, Wendy; Reardon, Willie; Klatt, Regan; Babul-Hirji, Riyana; Milunsky, Jeff; Chitayat, David

2012-08-01

Hearing impairment is common in individuals with branchio-oculo-facial (BOF) syndrome. The majority of described individuals have conductive hearing impairment due to malformed ossicles and/or external canal stenosis or atresia, although a sensorineural component to the hearing impairment in BOF syndrome is increasingly being reported. Sophisticated computed tomography (CT) of the temporal bone has revealed middle and inner ear malformations in three previous reports. We present middle and inner ear abnormalities in three additional individuals with mutation-proven BOF syndrome. We suggest that temporal bone CT imaging be included in the medical workup of a child with BOF syndrome, in order to guide management. Copyright © 2012 Wiley Periodicals, Inc.

Writing Skills of Hearing-Impaired Students Who Benefit from Support Services at Public Schools in Turkey

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Karasu, H. Pelin

2017-01-01

Support services provide an essential role for hearing-impaired students attending public schools, in terms of improving their language and academic skills. In this study, the writing skills of hearing-impaired students enrolled in public schools were evaluated, and the relationship between the writing scores, audiological variables and…

Parent and Patient Perceptions of Functional Impairment Due to Tourette Syndrome: Development of a Shortened Version of the Child Tourette Syndrome Impairment Scale.

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Barfell, Kara S Francis; Snyder, Ryan R; Isaacs-Cloes, Kelly M; Garris, Jordan F; Roeckner, Alyssa R; Horn, Paul S; Guthrie, Michael D; Wu, Steve W; Gilbert, Donald L

2017-07-01

The Child Tourette Syndrome Impairment Scale (CTIM) rates 37 problems in school, social, and home domains separately for tics and for comorbid diagnoses. However, a shorter version would be easier to implement in busy clinics. Using published data from 85 children with Tourette syndrome, 92 controls, and parents, factor analysis was used to generate a "mini-CTIM" composed of 12 items applied to tic and comorbid diagnoses. Child- and parent-rated mini-CTIM scores were compared and correlated across raters and accounting for clinician-rated tic severity and presence of attention-deficit hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD). The mini-CTIM achieved domain Cronbach alphas ranging from 0.71 to 0.94 and intra-item correlation coefficients ranging from 0.84 to 0.96. The resulting scale correlated with clinician-rated tic severity and reflected the presence of ADHD and OCD. The mini-CTIM appears promising as a practical assessment tool for tic- and non-tic-related impairment in children with Tourette syndrome.

Binaural speech discrimination under noise in hearing-impaired listeners

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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.

Problems among childrens with impaired hearing. The impact of noise on the work of teachers

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Ewelina Karolina Kucharska

2017-09-01

Full Text Available Introduction and Aim In the life of every human senses, including the sense of hearing, they play an important role in the harmonious development. Each hearing loss in childhood influences, depending on the depth, nature and time of its inception, in a negative way on the development of many mental activities, intellectual, behavior, cognitive, causing significant impairment in the child's position the hearing impaired, deaf and especially in the world of the hearing. The aim of this study is to assess the parents' awareness of hearing loss in children with hearing loss as a result of a survey of parents of children from the first classes of primary schools in the Lublin province. Material and Methods Analysis was performed on 31 results of parents of children with hearing impairment made in children aged 6 and 7 years, pupils and primary schools in the Lublin province. Parents were asked the question: 'Do you think your child has hearing problems?' The analysis indicated the assessment of awareness of parents of children with hearing disorders.   Results In the study group, as many as 77% of parents stated that the child does not have problems with hearing, only 3% of parents noted the problem, while 20% of parents did not answer the question. Conclusions Based on the research it can be stated with a high percentage of parents still do not notice hearing loss in their children. Early notice of the problem, the correct procedure and the cooperation with the parent group of specialists are often the way to success. It is crucial to support the child in difficult situations, which often is exposed to contact with all sorts of obstacles. An important issue is the numerous health programs, shaping the attitude of parents and society as a whole.

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

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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…

Phrasing in the speech and reading of the hearing impaired.

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Gregory, J F

1986-08-01

The study reported here explored a partial explanation for the fourth-grade "bottleneck" in literacy advancement by hearing-impaired students. Speech samples from 21 deaf subjects were rated for degree of evident phrasal quality. Likewise, reading comprehension scores for each student were obtained under four reading conditions: reading in whole sentences, in phrases, in fragmented word groups, and in single words. Degree of rated speech phrasality was found to relate significantly and positively to correct recall answers to questions based upon silent reading of passages typed in meaningful word groups (but not when the passages were typed in whole sentences, fragmented word groups, or in single words). The results were taken to suggest that--whereas staccato-speaking deaf students may lack a sense of the phrase altogether--phrasal-speaking deaf youngsters fail to independently apply their phrase sense in the normal reading situation. Thus, both types of deaf youngsters have difficulty affecting the transition to phrase reading that is common for hearing students at or about the fourth-grade level. Finally, I argue that this phrase sense can be instilled in hearing-impaired students and that they can be trained to use it in reading.

[From gene to disease; genetic causes of hearing loss and visual impairment sometimes accompanied by vestibular problems (Usher syndrome)].

Science.gov (United States)

Pennings, R J E; Kremer, H; Deutman, A F; Kimberling, W J; Cremers, C W R J

2002-12-07

Usher syndrome is an autosomal recessively inherited disease, characterised by sensorineural hearing loss, tapetoretinal degeneration and in some cases vestibular problems. Based on the clinical heterogeneity, the disease can be classified into three clinical types (I, II and III), which have their own genetic subtypes (Usher 1A-Usher IG, Usher 2A-Usher 2C and Usher 3). The majority of the Usher type I cases are caused by mutations in the MYO7A gene (Usher 1B) while mutations in the USH2A gene (Usher 2A) are the cause of most cases of type II. Usher syndrome type III, caused by mutations in the USH3 gene, is frequently seen only in Finland.

The Learning Disabled, Hearing Impaired Students: Reality, Myth, or Overextension?

Science.gov (United States)

Laughton, Joan

1989-01-01

This paper focuses on definitions, incidence, and characteristics of the multihandicapping condition known as "learning disabled, hearing impaired," in order to provide a means of identifying these children and determining whether or not they require different teaching strategies. (JDD)

The lexical development of children with hearing impairment and associated factors.

Science.gov (United States)

Penna, Leticia Macedo; Lemos, Stela Maris Aguiar; Alves, Cláudia Regina Lindgren

2014-01-01

This study aimed at analyzing the association between the lexical development of children with hearing impairment and their psychosocial and socioeconomic characteristics and medical history. An analytic transversal study was conducted in an Auditive Health Attention Service. One hundred and ten children from 6 to 10 years old using hearing aids and presenting hearing loss that ranged from light to deep levels were evaluated. All children were subjected to oral, written language and auditory perception tests. Parents answered a structured questionnaire to collect data from their medical history and socioeconomic status, and questionnaires about the features of the family environment and psychosocial characteristics. Multivariate analysis was performed by logistic regression, being the initial model composed by variables with panalysis. In the final model, we adopted a significance level of 5%. The final model of the multivariate analysis showed an association between the performance on the vocabulary test and the results of phonemic discrimination test (OR=0.81; 95%CI 0.73-0.89). The results show the importance of stimulating the auditory processing, particularly the phonemic discrimination skill, throughout the rehabilitation process of children with hearing impairment. This stimulation can enhance lexical development and minimize the metalanguage and learning difficulties often observed in these children.

Auditory stream segregation with multi-tonal complexes in hearing-impaired listeners

Science.gov (United States)

Rogers, Deanna S.; Lentz, Jennifer J.

2004-05-01

The ability to segregate sounds into different streams was investigated in normally hearing and hearing-impaired listeners. Fusion and fission boundaries were measured using 6-tone complexes with tones equally spaced in log frequency. An ABA-ABA- sequence was used in which A represents a multitone complex ranging from either 250-1000 Hz (low-frequency region) or 1000-4000 Hz (high-frequency region). B also represents a multitone complex with same log spacing as A. Multitonal complexes were 100 ms in duration with 20-ms ramps, and- represents a silent interval of 100 ms. To measure the fusion boundary, the first tone of the B stimulus was either 375 Hz (low) or 1500 Hz (high) and shifted downward in frequency with each progressive ABA triplet until the listener pressed a button indicating that a ``galloping'' rhythm was heard. When measuring the fusion boundary, the first tone of the B stimulus was 252 or 1030 Hz and shifted upward with each triplet. Listeners then pressed a button when the ``galloping rhythm ended.'' Data suggest that hearing-impaired subjects have different fission and fusion boundaries than normal-hearing listeners. These data will be discussed in terms of both peripheral and central factors.

Screening tools for the identification of dementia for adults with age-related acquired hearing or vision impairment: a scoping review.

Science.gov (United States)

Pye, Annie; Charalambous, Anna Pavlina; Leroi, Iracema; Thodi, Chrysoulla; Dawes, Piers

2017-11-01

Cognitive screening tests frequently rely on items being correctly heard or seen. We aimed to identify, describe, and evaluate the adaptation, validity, and availability of cognitive screening and assessment tools for dementia which have been developed or adapted for adults with acquired hearing and/or vision impairment. Electronic databases were searched using subject terms "hearing disorders" OR "vision disorders" AND "cognitive assessment," supplemented by exploring reference lists of included papers and via consultation with health professionals to identify additional literature. 1,551 papers were identified, of which 13 met inclusion criteria. Four papers related to tests adapted for hearing impairment; 11 papers related to tests adapted for vision impairment. Frequently adapted tests were the Mini-Mental State Examination (MMSE) and the Montreal Cognitive Assessment (MOCA). Adaptations for hearing impairment involved deleting or creating written versions for hearing-dependent items. Adaptations for vision impairment involved deleting vision-dependent items or spoken/tactile versions of visual tasks. No study reported validity of the test in relation to detection of dementia in people with hearing/vision impairment. Item deletion had a negative impact on the psychometric properties of the test. While attempts have been made to adapt cognitive tests for people with acquired hearing and/or vision impairment, the primary limitation of these adaptations is that their validity in accurately detecting dementia among those with acquired hearing or vision impairment is yet to be established. It is likely that the sensitivity and specificity of the adapted versions are poorer than the original, especially if the adaptation involved item deletion. One solution would involve item substitution in an alternative sensory modality followed by re-validation of the adapted test.

THE DEVELOPMENT OF SCIENCE LEARNING MODULE FOR CHILDRENT WITH HEARING IMPAIRMENT

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Ahmad Marzuqi

2017-02-01

Full Text Available There are the absence of teaching materials in accordance with the characteristics and conditions of a hearing impairment children in terms of learning, especially science subjects. The characteristics of hearing impairment children is poor in their vocabularies, so that, the teaching materials emphasizing the visual aspect is necessary. This study used a Research and Development (R & D adapted by the Sugiyono model in order to produce teaching materials in the form of pictorial modules and to test their effectiveness. The result of the research showed that it was a very valid criteria with a score of 97% of the materials experts, 85% of media experts, and 93% of skilled practitioners. The score of the effectiveness of the modules was 75% with the effective criteria.

Speech Production and Speech Discrimination by Hearing-Impaired Children.

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Novelli-Olmstead, Tina; Ling, Daniel

1984-01-01

Seven hearing impaired children (five to seven years old) assigned to the Speakers group made highly significant gains in speech production and auditory discrimination of speech, while Listeners made only slight speech production gains and no gains in auditory discrimination. Combined speech and auditory training was more effective than auditory…

Hearing-impaired children in the United Kingdom, III : cochlear implantation and the economic costs incurred by families

OpenAIRE

Barton, GR; Fortnum, HM; Stacey, PC; Summerfield, AQ

2006-01-01

Objectives: This article addresses two questions. First, are there differences in the economic costs incurred by families of hearing-impaired children depending on whether or not children have cochlear implants? Second, are these differences important when assessed from the perspective of society? Methods: In a cross-sectional survey, parents of a representative sample of hearing-impaired children provided data about annual resources used by the family because of their child’s hearing impairm...

Visual impairment, but not hearing impairment, is independently associated with lower subjective well-being among individuals over 95 years of age: A population-based study.

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Liu, Zuyun; Wu, Di; Huang, Jiapin; Qian, Degui; Chen, Fei; Xu, Jun; Li, Shilin; Jin, Li; Wang, Xiaofeng

2016-01-01

Sensory impairment affects an increasing number of elderly adults, with a negative psychological impact. Our objective was to examine the associations of visual and hearing impairment with subjective well-being (SWB), an important psychological concept defined by life satisfaction [LS], positive affect [PA], negative affect [NA], and affect balance [AB] among long-lived individuals (LLIs) over 95 years of age. Data on 442 LLIs from the Rugao longevity cohort, a population-based study in Rugao, China, were analyzed. Graded classifications of visual and hearing impairment (none, mild, moderate, and severe) were constructed from self-reported items. Bivariate correlation and multiple regression analysis were performed to test the associations. Approximately 66.1% and 87.3% of the subjects reported varying degrees of visual and hearing impairment. Following the degree of vision impairment, LS, PA, and AB decreased linearly, whereas NA increased linearly (all p for trendimpairment with LS, NA, and AB, while diminished, still existed. Visual impairment, but not hearing impairment, was independently associated with low SWB among LLIs, and functional ability may play a mediating role in the observed relationship. The findings indicate that rehabilitation targeted for those with reduced vision and functioning in long-lived populations may be important for promoting well-being and quality of life. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

DEVELOPMENT OF MULTIMEDIA-BASED INSTRUCTIONAL OF NATURAL SCIENCE IN SOLAR SYSTEM MATERIAL IN THE GRADE IX FOR HEARING IMPAIRMENT STUDENTS

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Nurvita Dwi Andriani

2016-12-01

Full Text Available Hearing impairment students have limitation in obtaining information. Hearing impairment students needs a multimedia-based instructional to visualize a subject matter as a learning experience. This study was aimed at producing a theoretically and empirically valid multimedia-based instructional of Natural Science in Solar System material which is reviewed by experts and could be implemented in Segment B of SMPLB (Hearing impairment students. This study employed a research and development (R&D model by William W. Lee and Diana L. Owens. Overall, the multimedia-based instructional of Natural Science in Solar System material for Segment B of SMPLB (Hearing impairment students was valid

Stress coping strategies in hearing-impaired students

OpenAIRE

Bahman Akbari; Zohreh Teymori; Shahnam Abolghasemi; Hamidreza Khorshidiyan

2013-01-01

Background and Aim: The majority of people experience problems and stressors, such as job layoffs and illnesses during their lives. However, the way people cope with stress varies. According to previous research, use of effective coping strategies can significantly reduce stress and tension. The aim of the present study was to investigate the effectiveness of stress coping strategies on hearing-impaired students.Methods: This is a quasi-experimental study with pre-test, post-test, and control...

Usher syndrome with psychotic symptoms: two cases in the same family.

Science.gov (United States)

Wu, Chen-Ying; Chiu, Chih-Chiang

2006-10-01

Usher syndrome is a heterogeneous autosomal recessive disorder characterized by hearing and visual sensory impairment. Retinitis pigmentosa is essential for its diagnosis. There are only a few reports describing patients with Usher syndrome presenting with psychotic features and the etiology of its psychiatric manifestation is still unknown. Herein, the authors report variable congenital hearing impairment and progressive visual loss occurring in five of seven family members and two of them meeting the diagnostic criteria of Usher syndrome with psychotic features. Furthermore, the authors compare their psychiatric symptoms with other reports and the possible etiologies of psychotic symptoms are discussed.

[Assistive Services in the Workplace of People with Hearing Impairment in the State of North Rhine-Westphalia].

Science.gov (United States)

Weber, A; Menzel, F; Weber, U; Niehaus, M; Kaul, T; Schlenker-Schulte, C

2015-09-01

Assistive services in the workplace are an important aspect of the participation of people with hearing impairment in working life. This article presents the results of the GINKO study and an survey conducted by the University of Cologne on behalf of the MAIS in order to provide a comprehensive examination of the employment situation of hearing impaired people in North Rhine-Westphalia. The GINKO study examines the impact of laws on the integration of hard-of-hearing and deaf people as well as people who have become deaf as adults, focusing on communication and organizations; this project was funded by the German Federal Ministry for Labour and Social Affairs (BMAS). In the GINKO study, conducted in cooperation with the German Association of the Hard of Hearing and the German Association of the Deaf, a standardised questionnaire with questions about the workplace was administered to employed people with hearing impairments. The questionnaire was administered on paper and was also available online accompanied by sign language videos. The University of Cologne study in North Rhine-Westphalia examined the service situation of hard-of-hearing, deaf and deaf-blind people through face-to-face interviews and government statistics. The results of the nationwide GINKO study show that hearing-impaired people in North Rhine-Westphalia draw on assistive services in employment more often than hearing-impaired people in the rest of Germany. The study found statistically significant differences in the categories of "maintenance and development of professional knowledge and skills" and "psychosocial support in conflict situations resulting from disability". One reason for the more positive evaluations of the participants in North Rhine-Westphalia as compared to other regions in Germany could be the particular network of support services in that state. However, the overall positive results from North Rhine-Westphalia should not obscure the fact that a majority of participants in

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

Science.gov (United States)

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.

[The occupational risk of hearing impairment associated with cardiovascular pathologies in the subjects engaged in 'noisy' industries].

Science.gov (United States)

Pankova, V B; Preobrazhenskaya, E A; Fedina, I N

The objective of the present study was to analyze the structure of concomitant somatic pathology in the subjects experiencing the occupational hearing problems. The study demonstrated the increase in the frequency of concomitant chronic diseases and the prevalence of polynosological conditions with the increasing severity of hearing impairment. It was shown that cardiovascular pathologies tend to enhance the risk of hearing loss in the employees engaged in «noisy» occupations. The cause-and-effect relationships were elucidated as the contribution of the "vascular" factor to the formation of occupational pathology of the organs of hearing. The clinical audilogical features of co-morbid occupational hearing impairment associated with vascular pathology were characterized by the accelerated development and progression of the hearing disorders with the gradual disappearance of the audiological signs characteristic of noise-induced lesions.

Interaction Between Syndromic and Non-Syndromic Factors Affecting Speech and Language Development in Treacher-Collins Syndrome

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Marziyeh Poorjavad

2011-09-01

Full Text Available Background: Treacher-Collins syndrome is a congenital craniofacial disorder with multiple anomalies. This syndrome affects the maxilla, mandible, eyes, middle and outer ears, and soft palate. Conductive hearing loss due to the deformities of the middle and external ears is prevalent. The characteristics of this syndrome include multiple and serious threats to normal communication development in children. In this study, speech and language features of a Persian speaking child with this syndrome are presented.Case: The case was an 8-year old girl with Treacher-Collins syndrome and bilateral moderate conductive hearing loss due to atretic canal. In language and speech assessments, moderate hypernasality, numerous compensatory errors and morphosyntactic deficits were observed. There were 13 phonemes that were incorrectly produced at least in one position. Besides, she used 22 types of phonological processes that were abnormal and disappear before the age of three in normal Persian speaking children.Conclusion: Moderate hearing loss, velopharyngeal incompetency, malocclusion and dental anomalies, attention deficit/hyperactivity disorder (ADHD and environmental factors resulted in severe speech and language disorders in this case. These disorders affected her academic performance as well. Moderate hypernasality, numerous compensatory errors, and excessive and abnormal use of phonological processes were not presented as prevalent characteristics of Treacher-Collins syndrome in other resources.

Allelic mutations of KITLG, encoding KIT ligand, cause asymmetric and unilateral hearing loss and Waardenburg syndrome type 2

NARCIS (Netherlands)

Zazo Seco, C. (Celia); Serrão De Castro, L. (Luciana); J.W.I. van Nierop; Morín, M. (Matías); S.N. Jhangiani (Shalini N.); E.J.J. Verver (Eva J. J.); M. Schraders (Margit); Maiwald, N. (Nadine); Wesdorp, M. (Mieke); H. Venselaar (Hanka); L. Spruijt (Liesbeth); Oostrik, J. (Jaap); J. Schoots (Jeroen); J. van Reeuwijk (Jeroen); Lelieveld, S.H. (Stefan H.); P.L.M. Huygen (Patrick); Insenser, M. (María); R.J. Admiraal (Ronald); R.J.E. Pennings (Ronald J.E.); E.H. Hoefsloot (Lies); A. Arias-Vásquez (Alejandro); J. de Ligt (Joep); H.G. Yntema; Jansen, J.H. (Joop H.); D. Muzny (Donna); G. Huls (Gerwin); M.M. van Rossum (Michelle); J.R. Lupski (James R.); Moreno-Pelayo, M.A. (Miguel Angel); H.P.M. Kunst (Henricus P.M.); H. Kremer (Hannie)

2015-01-01

textabstractLinkage analysis combined with whole-exome sequencing in a large family with congenital and stable non-syndromic unilateral and asymmetric hearing loss (NS-UHL/AHL) revealed a heterozygous truncating mutation, c.286-303delinsT (p.Ser96Ter), in KITLG. This mutation co-segregated with

Exploring an educational assessment tool to measure registered nurses' knowledge of hearing impairment and effective communication strategies: A USA study.

Science.gov (United States)

Ruesch, Amy L

2018-01-01

Poor communication between the Registered Nurse and a hearing impaired patient can affect quality of care and health outcomes. Communication skills training programs for healthcare providers are needed to improve patient centered care. A descriptive research study, using a knowledge assessment tool developed and validated by the researcher, was conducted on 339 Registered Nurses to identify knowledge deficits to be addressed in a communication skills training program being designed. The educational tool measured the Registered Nurses' knowledge across four areas - hearing impairment, hearing aids, communication strategies, and regulations regarding access to care for a person with a hearing disability. Knowledge deficits were detected in all four areas. Using this educational assessment tool may enable nurse educators to tailor communication skills training programs to specifically address the gaps identified regarding hearing impairment and how to effectively communicate with the hearing impaired patient. Post training program, nurse educators can use the tool to evaluate effectiveness. Copyright © 2017 Elsevier Ltd. All rights reserved.

Hearing impairment, social support, and depressive symptoms among U.S. adults: A test of the stress process paradigm.

Science.gov (United States)

West, Jessica S

2017-11-01

Hearing impairment is a growing physical disability affecting older adults and is an important physical health stressor, but few studies have examined it in relation to mental health outcomes and even fewer have considered the role of social support in buffering this relationship. The current study builds on the stress process framework and uses longitudinal data from three waves of the Health and Retirement Study (2006, 2010, 2014) to examine the relationship between hearing impairment and depressive symptoms among U.S. adults aged 50 and older (n = 6075). The analysis uses fixed-effects models to assess this relationship and examine the extent to which social support mediates (buffers) or moderates (interaction) the association. The results found that worse self-rated hearing was associated with a significant increase in depressive symptoms, even after controlling for sociodemographic factors. Social support did not buffer this relationship. Instead, social support interacted with hearing impairment: low levels of social support were associated with more depressive symptoms but only among people with poor self-rated hearing. Among those with excellent self-rated hearing, low levels of social support did not increase depressive symptoms. Moreover, high levels of social support reduced depressive symptoms for those with poor hearing. These findings suggest that hearing impairment is a chronic stressor in individuals' lives, and that responses to this stressor vary by the availability of social resources. Copyright © 2017 Elsevier Ltd. All rights reserved.

Otitis Media and Children with Hearing Impairments: A Sequela.

Science.gov (United States)

Beattie, R. G. John L.

1991-01-01

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

Non-alcoholic Korsakoff syndrome in psychiatric patients with a history of undiagnosed Wernicke's encephalopathy.

Science.gov (United States)

Nikolakaros, Georgios; Ilonen, Tuula; Kurki, Timo; Paju, Janina; Papageorgiou, Sokratis G; Vataja, Risto

2016-11-15

Wernicke's encephalopathy is often undiagnosed, particularly in non-alcoholics. There are very few reports of non-alcoholic patients diagnosed with Korsakoff syndrome in the absence of a prior diagnosis of Wernicke's encephalopathy and no studies of diffusion tensor imaging in non-alcoholic Korsakoff syndrome. We report on three non-alcoholic psychiatric patients (all women) with long-term non-progressive memory impairment that developed after malnutrition accompanied by at least one of the three Wernicke's encephalopathy manifestations: ocular abnormalities, ataxia or unsteadiness, and an altered mental state or mild memory impairment. In neuropsychological examination, all patients had memory impairment, including intrusions. One patient had mild cerebellar vermis atrophy in MRI taken after the second episode of Wernicke's encephalopathy. The same patient had mild hypometabolism in the lateral cortex of the temporal lobes. Another patient had mild symmetrical atrophy and hypometabolism of the superior frontal lobes. Two patients were examined with diffusion tensor imaging. Reduced fractional anisotropy values were found in the corona radiata in two patients, and the uncinate fasciculus and the inferior longitudinal fasciculus in one patient. Our results suggest that non-alcoholic Korsakoff syndrome is underdiagnosed. Psychiatric patients with long-term memory impairment may have Korsakoff syndrome and, therefore, they should be evaluated for a history of previously undiagnosed Wernicke's encephalopathy. Copyright © 2016 Elsevier B.V. All rights reserved.

An assistive technology for hearing-impaired persons: analysis, requirements and architecture.

Science.gov (United States)

Mielke, Matthias; Grunewald, Armin; Bruck, Rainer

2013-01-01

In this contribution, a concept of an assistive technology for hearing-impaired and deaf persons is presented. The concept applies pattern recognition algorithms and makes use of modern communication technology to analyze the acoustic environment around a user, identify critical acoustic signatures and give an alert to the user when an event of interest happened. A detailed analysis of the needs of deaf and hearing-impaired people has been performed. Requirements for an adequate assisting device have been derived from the results of the analysis, and have been turned into an architecture for its implementation that will be presented in this article. The presented concept is the basis for an assistive system which is now under development at the Institute of Microsystem Engineering at the University of Siegen.

Older Adults With a Combination of Vision and Hearing Impairment Experience Higher Rates of Cognitive Impairment, Functional Dependence, and Worse Outcomes Across a Set of Quality Indicators.

Science.gov (United States)

Davidson, Jacob G S; Guthrie, Dawn M

2017-08-01

Hearing and vision impairment were examined across several health-related outcomes and across a set of quality indicators (QIs) in home care clients with both vision and hearing loss (or dual sensory impairment [DSI]). Data collected using the Resident Assessment Instrument for Home Care (RAI-HC) were analyzed in a sample of older home care clients. The QIs represent the proportion of clients experiencing negative outcomes (e.g., falls, social isolation). The average age of clients was 82.8 years ( SD = 7.9), 20.5% had DSI and 8.5% had a diagnosis of Alzheimer's disease (AD). Clients with DSI were more likely to have a diagnosis of dementia (not AD), have functional impairments, report loneliness, and have higher rates across 20 of the 22 QIs, including communication difficulty and cognitive decline. Clients with highly impaired hearing, and any visual impairment, had the highest QI rates. Individuals with DSI experience higher rates of adverse events across many health-related outcomes and QIs. Understanding the unique contribution of hearing and vision in this group can promote optimal quality of care.

Treatment Efficacy: Hearing Aids in the Management of Hearing Loss in Adults.

Science.gov (United States)

Weinstein, Barbara E.

1996-01-01

This article reviews the efficacy of hearing aids in adults with hearing impairments. Information is provided on the prevalence of hearing impairments; the daily effects of a hearing impairment; and the role of the audiologist. The effectiveness and benefits of hearing aids are reviewed, and a case study is provided. (CR)

Usher syndrome: Hearing loss, retinal degeneration and associated abnormalities

OpenAIRE

Mathur, Pranav; Yang, Jun

2015-01-01

Usher syndrome (USH), clinically and genetically heterogeneous, is the leading genetic cause of combined hearing and vision loss. USH is classified into three types, based on the hearing and vestibular symptoms observed in patients. Sixteen loci have been reported to be involved in the occurrence of USH and atypical USH. Among them, twelve have been identified as causative genes and one as a modifier gene. Studies on the proteins encoded by these USH genes suggest that USH proteins interact a...

Clinical measurement of various aspects of hearing impairment and their relation to auditory functioning: the development of an Auditory Profile

NARCIS (Netherlands)

van Esch, T.E.M.

2013-01-01

In terms of disability and handicap, problems in auditory function involve much more than a reduced sensitivity to soft sounds, the most commonly used measure of hearing impairment. In daily life, many hearing-impaired (HI) listeners suffer more from impaired processing of audible sounds, than from

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

Science.gov (United States)

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.

Hearing aid use and long-term health outcomes: hearing handicap, mental health, social engagement, cognitive function, physical health and mortality

Science.gov (United States)

Dawes, Piers; Cruickshanks, Karen J.; Fischer, Mary E.; Klein, Barbara E.K.; Klein, Ronald; Nondahl, David M.

2016-01-01

Objective To clarify the impact of hearing aids on mental health, social engagement, cognitive function, and physical health outcomes in older adults with hearing impairment. Design We assessed hearing handicap (Hearing Handicap Inventory for the Elderly; HHIE-S), cognition (Mini Mental State Exam, Trail Making, Auditory Verbal Learning, Digit-Symbol Substitution, Verbal Fluency, incidence of cognitive impairment), physical health (SF-12 physical component, basic and instrumental activities of daily living, mortality), social engagement (hours per week spent in solitary activities) and mental health (SF-12 mental component) at baseline, 5 years prior to baseline, and 5 and 11 years after baseline. Study sample Community-dwelling older adults with hearing impairment (N=666) from the Epidemiology of Hearing Loss Study cohort. Results There were no significant differences between hearing aid users and non-users in cognitive, social engagement or mental health outcomes at any time point. Aided HHIE-S was significantly better than unaided HHIE-S. At 11 years hearing aid users had significantly better SF-12 physical health scores (46.2 versus 41.2; p=0.03). There was no difference in incidence of cognitive impairment or mortality. Conclusion There was no evidence that hearing aids promote cognitive function, mental health, or social engagement. Hearing aids may reduce hearing handicap and promote better physical health. PMID:26140300

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

Science.gov (United States)

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…

Allelic Mutations of KITLG, Encoding KIT Ligand, Cause Asymmetric and Unilateral Hearing Loss and Waardenburg Syndrome Type 2

NARCIS (Netherlands)

Zazo Seco, C.; Castro, L.S. de; Nierop, J.W. van; Morin, M.; Jhangiani, S.; Verver, E.J.; Schraders, M.; Maiwald, N.; Wesdorp, F.M.; Venselaar, H.; Spruijt, L.; Oostrik, J.; Schoots, J.; Reeuwijk, J. van; Lelieveld, S.H.; Huygen, P.L.M.; Insenser, M.; Admiraal, R.J.C.; Pennings, R.J.E.; Hoefsloot, L.H.; Arias Vasquez, A.; Ligt, J. de; Yntema, H.G.; Jansen, J.H.; Muzny, D.M.; Huls, G.A.; Rossum, M.M. van; Lupski, J.R.; Moreno-Pelayo, M.A.; Kunst, H.P.M.; Kremer, H.

2015-01-01

Linkage analysis combined with whole-exome sequencing in a large family with congenital and stable non-syndromic unilateral and asymmetric hearing loss (NS-UHL/AHL) revealed a heterozygous truncating mutation, c.286_303delinsT (p.Ser96Ter), in KITLG. This mutation co-segregated with NS-UHL/AHL as a

Does extracorporeal shock wave lithotripsy cause hearing impairment?

Science.gov (United States)

Tuncer, Murat; Erdogan, Banu A; Yazici, Ozgur; Sahin, Cahit; Altin, Gokhan; Faydaci, Gokhan; Eryildirim, Bilal; Sarica, Kemal

2014-07-01

To evaluate the possible effects of extracorporeal shock wave lithotripsy (ESWL) on the hearing status of the patients in this prospective controlled study. A total of 40 patients with normal hearing function were included to the study. We had 20 patients each in the study group and control group. The treatment parameters were standardized in all 3 sessions in which a total of 3000 shock waves with a rate of 90/min along with a total energy value of 126 J at the fourth energy level have been applied (Dornier Compact Sigma, Medtech, Germany). In addition to the testing of hearing functions and possible cochlear impairment by Transient Evoked Otoacoustic Emissions test at 1.0, 1.4, 2.0, 2.8, and 4.0 kHz frequencies, complications such as ear pain, tinnitus, and hearing loss have been well evaluated in each patient before the procedure and 2 hours and 1 month after the completion of the third session of ESWL in the study group. The same evaluation procedures were performed before the study and after 7-weeks in the control group. Regarding Transient Evoked Otoacoustic Emissions data obtained in study group and control group patients, there was no significant alteration in values obtained after ESWL when compared with the values before the procedure. A well-planned ESWL procedure is a safe and effective treatment in urinary stones and causes no detectable harmful effect on the hearing function of treated patients. Copyright © 2014 Elsevier Inc. All rights reserved.

Hearing impairment among workers exposed to excessive levels of noise in ginning industries

Directory of Open Access Journals (Sweden)

Kamalesh J Dube

2011-01-01

Full Text Available Cotton ginning workers have a risk of hearing loss due to excessive noise levels at the workplace environment. In this study, estimates of typical sound levels prevailing at the workplace environment and its effects on hearing ability of the exposed workers were made among cotton ginning workers. Data on self-reported health status was collected by a questionnaire survey at 10 cotton ginning industries located at Jalgaon district of Maharashtra state, India. The cotton ginning workers were exposed to continuous noise levels between 89 and 106 dBA. The hearing ability of the subjects was accessed by pure tone audiometry. The results of audiometry show mild, moderate and moderately severe degree of hearing impairment among the cotton ginning workers. The data generated during the study show that hearing loss was significantly associated with period of exposure to the workplace noise (P <0.0001. The prevalence of audiometric hearing impairment defined as a threshold average greater than 25 dB hearing level was 96% for binaural low-frequency average, 97% for binaural mid frequency average and 94% for binaural high-frequency average in the cotton ginning workers. We recommend the compulsory use of personal protective equipment like ear plug by the cotton ginning workers at the workplace environment. A regular maintenance of ginning and pressing machineries will avoid the emission of excessive noise at the workplace environment of cotton gins. A regular periodic medical examination is necessary to measure the impact of workplace noise on the health of cotton ginning workers.

Sign-Lingo : Feasibility of a Serious Game for Involving Parents in the Language Development of their Deaf or Hearing Impaired Child

NARCIS (Netherlands)

Schalk, I van der; Spruit, M.

2017-01-01

Family involvement plays a critical factor in the language development of a deaf or hearing impaired child. Hearing parents often have major difficulties in communicating with their child when it is deaf or hearing impaired. These difficulties often lead to issues in the language development of the

Impact of cognitive and linguistic ability on gaze behavior in children with hearing impairment

Directory of Open Access Journals (Sweden)

Olof eSandgren

2013-11-01

Full Text Available In order to explore verbal-nonverbal integration, we investigated the influence of cognitive and linguistic ability on gaze behavior during spoken language conversation between children with mild-to-moderate hearing impairment (HI and normal-hearing (NH peers. Ten HI-NH and ten NH-NH dyads performed a referential communication task requiring description of faces. During task performance, eye movements and speech were tracked. Cox proportional hazards regression was used to model associations between performance on cognitive and linguistic tasks and the probability of gaze to the conversational partner’s face. Analyses compare the listeners in each dyad (HI: n = 10, mean age = 12;6 years, SD = 2;0, mean better ear pure-tone average 33.0 dB HL, SD = 7.8; NH: n = 10, mean age = 13;7 years, SD = 1;11. Group differences in gaze behavior – with HI gazing more to the conversational partner than NH – remained significant despite adjustment for ability on receptive grammar, expressive vocabulary, and complex working memory. Adjustment for phonological short term memory, as measured by nonword repetition, removed group differences, revealing an interaction between group membership and nonword repetition ability. Stratified analysis showed a twofold increase of the probability of gaze-to-partner for HI with low phonological short term memory capacity, and a decreased probability for HI with high capacity, as compared to NH peers. The results revealed differences in gaze behavior attributable to performance on a phonological short term memory task. Participants with hearing impairment and low phonological short term memory capacity showed a doubled probability of gaze to the conversational partner, indicative of a visual bias. The results stress the need to look beyond the hearing impairment in diagnostics and intervention. Acknowledgment of the finding requires clinical assessment of children with hearing impairment to be supported by tasks tapping

The role of social frailty in explaining the association between hearing problems and mild cognitive impairment in older adults.

Science.gov (United States)

Bae, Seongryu; Lee, Sangyoon; Lee, Sungchul; Jung, Songee; Makino, Keitaro; Park, Hyuntae; Shimada, Hiroyuki

2018-06-01

We examined the role of social frailty in the association between hearing problems and mild cognitive impairment (MCI), and investigated which cognitive impairment domains are most strongly involved. Participants were 4251 older adults (mean age 72.5 ± 5.2 years, 46.1% male) who met the study inclusion criteria. Hearing problems were measured using the Hearing Handicap Inventory for the Elderly. Social frailty was identified using responses to five questions. Participants were divided into four groups depending on the presence of social frailty and hearing problems: control, social frailty, hearing problem, and co-occurrence. We assessed memory, attention, executive function, and processing speed using the National Center for Geriatrics and Gerontology-Functional Assessment Tool. Participants were categorized into normal cognition, single- and multiple-domain MCI, depending on the number of impaired cognitive domains. Participants with multiple-domain MCI exhibited the highest odds ratios (OR) of the co-occurrence group (OR: 3.89, 95% confidence intervals [CI]: 1.96-7.72), followed by the social frailty (OR: 2.65, 95% CI: 1.49-4.67), and hearing problem (OR: 1.90, 95% CI: 1.08-3.34) groups, compared with the control group. However, single-domain MCI was not significantly associated with any group. Cognitive domain analysis revealed that impaired executive function and processing speed were associated with the co-occurrence, hearing problem, and social frailty groups, respectively. Social frailty and hearing problems were independently associated with multiple-domain MCI. Comorbid conditions were more strongly associated with multiple-domain MCI. Longitudinal studies are needed to elucidate the causal role of social frailty in the association between hearing impairment and MCI. Copyright © 2018 Elsevier B.V. All rights reserved.

Negotiating hearing disability and hearing disabled identities

DEFF Research Database (Denmark)

Lykke Hindhede, Anette

2012-01-01

  　 Using disability theory as a framework and social science theories of identity to strengthen the arguments, this paper explores empirically how working-age adults confront the medical diagnosis of hearing impairment. For most participants hearing impairment threatens the stability of social...... interaction and the construction of hearing disabled identities is seen as shaped in the interaction with the hearing impaired person‟s surroundings. In order to overcome the potential stigmatisation the „passing‟ as normal becomes predominant. For many the diagnosis provokes radical redefinitions of the self....... The discursively produced categorisation and subjectivity of senescence mean that rehabilitation technologies such as hearing aids identify a particular life-style (disabled) which determines their social significance. Thus wearing a hearing aid works against the contemporary attempt to create socially ideal...

Does Differential Visual Exploration Contribute to Visual Memory Impairments in 22Q11.2 Microdeletion Syndrome?

Science.gov (United States)

Bostelmann, M.; Glaser, B.; Zaharia, A.; Eliez, S.; Schneider, M.

2017-01-01

Background: Chromosome 22q11.2 microdeletion syndrome (22q11.2DS) is a genetic syndrome characterised by a unique cognitive profile. Individuals with the syndrome present several non-verbal deficits, including visual memory impairments and atypical exploration of visual information. In this study, we seek to understand how visual attention may…

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

Science.gov (United States)

Byrne, Bronagh

2014-01-01

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

Retinal function in deaf-blind syndromes

OpenAIRE

Malm, Eva

2011-01-01

A variety of disorders can cause retinal degeneration and hearing impairment, and it is of great value to have an early diagnosis since there is a large variation in phenotype and prognosis both within and between the different disorders. The general aim of this thesis was to characterize the retinal function, to describe the phenotype, and – where appropriate – to relate the phenotype to genotype in patients with combined visual and hearing impairment. Alström syndrome is a rare auto...

Oral communication in individuals with hearing impairment – considerations regarding attentional, cognitive and social resources

Directory of Open Access Journals (Sweden)

Ulrike eLemke

2015-07-01

Full Text Available 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.

[Research progress of mutational spectrum and pathophysiology of WFS1 gene in Wolfram syndrome and nonsyndromic low frequency sensorineural hearing loss].

Science.gov (United States)

Shi, S M; Han, Y H; Wang, H B

2016-09-07

Compound homozygous or heterozygous mutations in WFS 1 can lead to autosomal recessive Wolfram syndrome (WS), and heterozygous mutations in WFS 1 can lead to autosomal dominant non-syndromic low frequency sensorineural hearing loss (LFSNHL). In addition, mutations in the WFS region has relationship with diabetes and psychiatric diseases. In this paper, we provide an overview of genetic research with different phenotypes, including WS and LFSNHL.

The effect of ice skating on psychological well-being and sleep quality of children with visual or hearing impairment.

Science.gov (United States)

Dursun, Onur Burak; Erhan, Süleyman Erim; Ibiş, Esra Özhan; Esin, Ibrahim Selcuk; Keleş, Sadullah; Şirinkan, Ahmet; Yörük, Özgür; Acar, Ethem; Beyhun, Nazim Ercument

2015-01-01

Physical exercise and sports have a key role in preventing physical and psychiatric problems in children. However, children with a disability often experience difficulty participating in physical activity due to a lack of suitable opportunities. Participation in an accessible sport is particularly important for these children, but studies examining which sports are beneficial for which disability groups are rare. In this study, we assessed the effects of ice skating on the psychological well-being, self-concept, and sleep quality of children with hearing or visual impairment. Forty students (20 visually impaired and 20 hearing impaired) aged 8-16 were included in a regular ice skating programme for three months. We examined the sleep quality, self-concept, and behavioural and emotional states of the children before and after participating in the programme. There was a significant improvement in self-concept, behavioural and emotional problems, and sleep quality (p sleep quality (p = 0.019) and emotional problem scores (p = 0.000) of the visually impaired children improved; self-concept, peer relations and hyperactivity scores of these children worsened (p sport alternatives that gives children the opportunity to exercise and have fun together. The results of this study revealed that regular ice skating programmes may have positive effects on the psychological well-being of children with hearing impairment. Despite some positive effects, caution must be use when including visually impaired children in ice skating programmes. Generalization of the study's outcomes is limited as the study group were residential students enrolled in special education institutions for children who are blind or deaf. Ice skating is a community-based sport and a popular leisure activity that can also have benefits for people with disabilities. Ice skating and children with hearing impairment: Self-concept, behavioural and emotional problems, and sleep quality of the children

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

Science.gov (United States)

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

2017-09-01

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

Development of a genotyping microarray for Usher syndrome

OpenAIRE

Cremers, Frans P M; Kimberling, William J; Külm, Maigi; de Brouwer, Arjan P; van Wijk, Erwin; te Brinke, Heleen; Cremers, Cor W R J; Hoefsloot, Lies H; Banfi, Sandro; Simonelli, Francesca; Fleischhauer, Johannes C; Berger, Wolfgang; Kelley, Phil M; Haralambous, Elene; Bitner‐Glindzicz, Maria

2006-01-01

Background: Usher syndrome, a combination of retinitis pigmentosa (RP) and sensorineural hearing loss with or without vestibular dysfunction, displays a high degree of clinical and genetic heterogeneity. Three clinical subtypes can be distinguished, based on the age of onset and severity of the hearing impairment, and the presence or absence of vestibular abnormalities. Thus far, eight genes have been implicated in the syndrome, together comprising 347 protein-coding exons. Methods: To im...

Development of a genotyping microarray for Usher syndrome

OpenAIRE

Cremers, F P M; et al; Fleischhauer, J C; Berger, W

2007-01-01

BACKGROUND: Usher syndrome, a combination of retinitis pigmentosa (RP) and sensorineural hearing loss with or without vestibular dysfunction, displays a high degree of clinical and genetic heterogeneity. Three clinical subtypes can be distinguished, based on the age of onset and severity of the hearing impairment, and the presence or absence of vestibular abnormalities. Thus far, eight genes have been implicated in the syndrome, together comprising 347 protein-coding exons. METHODS: To improv...

Effectiveness of rehabilitation interventions in improving emotional and functional status in hearing or visually impaired older adults : a systematic review with meta-analyses

NARCIS (Netherlands)

Roets-Merken, Lieve M.; Draskovic, Irena; Zuidema, Sytse U.; van Erp, Willemijn S.; Graff, Maud J. L.; Kempen, Gertrudis I. J. M.; Vernooij-Dassen, Myrra J. F. J.

Objective: To assess the effectiveness of non-equipment based rehabilitation interventions for older adults with an age-related hearing or visual impairment. Data sources: We searched PubMed, EMBASE, PsychInfo, CINAHL, and the Cochrane Central Register of Controlled Trials. Review methods: Two

Overview of Usher's Syndrome: Congenital Deafness and Progressive Loss of Vision

Science.gov (United States)

Vernon, McCay

1974-01-01

Usher's syndrome, a genetic condition causing congenital profound hearing loss and a progressive blindness due to retinitis pigmentosa, affects an estimated three to six percent of children in educational and rehabilitative programs for the hearing impaired. (Author)

Kallmann syndrome and deafness: an uncommon combination: A case report and a literature review

Directory of Open Access Journals (Sweden)

Nader Salama

2016-08-01

Full Text Available Background: Kallmann syndrome (Kal S is an isolated form of hypogonadotrophic hypogonadism in combination with a defect in smell sensation. Depending on the genetic form of the disease, a number of non-reproductive, non-olfactory abnormalities may also be existent. In the present report, we describe a male with Kal S associated with hearing loss, and the successful treatment of his sexual and reproductive defects. Case: A 23-year-old Caucasian man presented with a lifelong lack of erection and ejaculation. The patient reported also anosmia combined with loss of hearing ability. A diagnostic work-up identified the presence of Kal S associated with sensorineural hearing loss. Administration of gonadotrophins regained the erection and a viable-sperm containing ejaculation. Conclusion: Lack of erection and ejaculation are important components of delayed puberty which could lead to diagnosis of Kal S. The existence of a hearing impairment in the reported patient makes the recommendation to screen the hearing ability in Kal S of utmost importance

[Molecular updates on Usher syndrome].

Science.gov (United States)

Roux, A-F

2005-01-01

Usher syndrome (USH) is an autosomal recessive disorder characterized by the association of sensorineural hearing loss and retinitis pigmentosa (RP). Usher syndrome is both clinically and genetically heterogeneous. Three clinical subtypes are defined with respect to vestibular dysfunction and the degree of hearing loss. Type I (USH1) patients have profound hearing loss and vestibular dysfunction from birth. Type II (USH2) is the most frequent and patients tend to have less severe hearing impairment and normal vestibular response. Type III (USH3) is characterized by a progressive loss of hearing and is found more frequently among Finnish patients. Recently, major breakthroughs have been made in the molecular genetics of Usher syndrome as a number of chromosomal loci and causative genes have been identified in each clinical subtype. Twelve loci are known and the corresponding genes have been cloned for six of them. Although their functions are not always clearly established, a common role is emerging for the proteins identified within each subtype. As a result, each subtype could emanate from defects affecting distinct cellular mechanisms.

[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)].

Science.gov (United States)

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.

RELATIONSHIP BETWEEN LIFE BUILDING SKILLS AND SOCIAL ADJUSTMENT OF STUDENTS WITH HEARING IMPAIRMENT: IMPLICATIONS FOR COUNSELING

Directory of Open Access Journals (Sweden)

Samuel O. ADENIYI

2017-10-01

Full Text Available Introduction Hearing impairment contributes greatly to social and psychological deficits of the affected individuals, which can affect their interpersonal relation. The inability to hear and communicate effectively results in adjustment problem that leads to social isolation. Objectives: The objective of this study is to examine the relationship between life building skills and social adjustment of students with hearing impairment. Methods: The study employed descriptive survey research design. The samples consisted of 150 students with hearing impairment purposively selected from two inclusive schools in Lagos state, Nigeria. The samples comprised of 65 boys and 85 girls with age range between 15 and 18 years in the Senior Secondary School. The instruments used for data collection were Life building skills inventory (adapted with reliability of 0.80 and Social adjustment scale (Self developed. The instruments consisted of two sections namely: A&B. Section A of Life building skills contained bio- data of the respondents, while B contained 3 subscales: Self-efficacy inventory adapted from Schwarzer and Jerusalem 1995 with reliability of 0.85, Decision-making inventory adapted from Rowe 1997 with reliability of 0.75, Assertiveness inventory adapted from Aberti and Emmons 1995 with reliability of 0.80. The self-constructed Social Adjustment scale contained 10 items probing questions with reliability of 0.69. Data collected was analysed using Pearson Product Moment Correlation and Multiple Regression. Results: The results revealed relative contributions of some life building skills to social adjustment of students with hearing impairment. There were joint contributions of the independent variables to dependent variable, while decision-making contributed mostly. Conclusion: This study examined relationship between life building skills and social adjustment of students with hearing impairment with a bid to provide adequate counseling services. It was

The effect of positive parenting program on parenting stress of mothers with impaired hearing children

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Mahnaz Aliakbari Dehkordi

2015-02-01

Full Text Available Background and Aim: Research indicates that impaired hearing is one of the most stressful disabilities. The parenting stress involved could lead to family malfunction and improper parenting. Therefore, this study aimed to evaluate the effects of positive parenting programs on the parenting stress of mothers with impaired hearing children.Methods: The statistical population comprised mothers of all 7-12-year-old impaired hearing children in Tehran city. Thereafter, using the random sampling method, 24 individuals were shortlisted as research participants and were randomly assigned to two groups: control and experimental. The experimental group was trained with a positive parenting program based on the Sanders program (1993 over eight sessions. The measurement instrument was the Abidin parenting stress questionnaire.Results: The mean score for grades in the experimental groups’ parent and child domains at the pre- and post-test stages had reduced more than that in the control group. In addition, the results of a multivariate covariance analysis indicated that positive parenting training was effective in the reduction of parenting stress scores, reinforcement, and child mood components in the child domain, and in the feelings of competence, relationships with the spouse, and role limitation components (p<0.05 in the parent domain.Conclusion : Considering the benefits of training parents for the reduction of parenting stress of mothers with impaired hearing children, this method is recommended in all learning centers for the deaf.

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

Science.gov (United States)

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)

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

Science.gov (United States)

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

2017-01-01

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

Expressivity of hearing loss in cases with Usher syndrome type IIA.

Science.gov (United States)

Sadeghi, André M; Cohn, Edward S; Kimberling, William J; Halvarsson, Glenn; Möller, Claes

2013-12-01

The purpose of this study was to compare the genotype/phenotype relationship between siblings with identical USH2A pathologic mutations and the consequent audiologic phenotypes, in particular degree of hearing loss (HL). Decade audiograms were also compared among two groups of affected subjects with different mutations of USH2A. DNA samples from patients with Usher syndrome type II were analysed. The audiological features of patients and affected siblings with USH2A mutations were also examined to identify genotype-phenotype correlations. Genetic and audiometric examinations were performed in 18 subjects from nine families with Usher syndrome type IIA. Three different USH2A mutations were identified in the affected subjects. Both similarities and differences of the auditory phenotype were seen in families with several affected siblings. A variable degree of hearing loss, ranging from mild to profound, was observed among affected subjects. No significant differences in hearing thresholds were found the group of affected subjects with different pathological mutations. Our results indicate that mutations in the USH2A gene and the resulting phenotype are probably modulated by other variables, such as modifying genes, epigenetics or environmental factors which may be of importance for better understanding the etiology of Usher syndrome.

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

Science.gov (United States)

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.

Language Characteristics of Preschool Children with Hearing Loss in Tehran, Iran

Directory of Open Access Journals (Sweden)

Dr. Younes Lotfi

2010-06-01

Full Text Available Background and Aim: Hearing impairment affects all aspect of individual life, specially language and communication skills. When hearing impairment is congenital or occurs early in life, the child’s ability to learn optimally through audition, will be affected. The aim of this study was to evaluate linguistic skills of preschool hearing impaired children and compare these skills with normal peers.Methods: This descriptive cross-sectional study was performed on 38 preschool hearing impaired children that the main handicap was severe to profound hearing loss with ability to communicate orally and 28 normal children with the same cultural and social context. Twenty four non linguistic variables including age, gender, the age of entrance of preschool center, number of hearing aids, etc. were obtained by filling a questionnaire and fifteen linguistics variables including number of utterance, morphemes, correct utterance, noun phrase, ambiguous utterance, correct sentences, compound sentences, etc. were collected by some part of TOLD-P-3 test and three complementary questions. Then we compared the data from two groups.Results: There were significant differences between number of utterance, number of correct mean length utterance, number of well-formed sentences in normal and hearing impaired group (p0.05.Conclusion: This study showed a severe deficit in linguistic skills in preschool hearing impaired children.

Interventions to prevent occupational noise induced hearing loss

NARCIS (Netherlands)

Verbeek, Jos H.; Kateman, Erik; Morata, Thais C.; Dreschler, Wout; Sorgdrager, Bas

2009-01-01

BACKGROUND: Millions of workers worldwide are exposed to noise levels that increase their risk of hearing impairment. Little is known about the effectiveness of hearing loss prevention interventions. OBJECTIVES: To assess the effectiveness of non-pharmaceutical interventions for preventing

Interventions to prevent occupational noise-induced hearing loss

NARCIS (Netherlands)

Verbeek, Jos H.; Kateman, Erik; Morata, Thais C.; Dreschler, Wouter A.; Mischke, Christina

2012-01-01

Background Millions of workers worldwide are exposed to noise levels that increase their risk of hearing impairment. Little is known about the effectiveness of hearing loss prevention interventions. Objectives To assess the effectiveness of non-pharmaceutical interventions for preventing

In hospital with a hearing impaired child - How parents experience communication between nurses and their child

Science.gov (United States)

Brooks, Seraina; Eckerli-Wäspi, Irene; Händler Schuster, Daniela

2018-04-01

Background: In daily communication, children with hearing impairment are restricted and dependent on their parents’ help. In case of a hospitalisation, the risk of insufficient information and resulting traumatisation for those children is high. The aim of this study is the investigation of the communicative needs of the children concerned in order to avoid negative consequences of a hospitalisation and of inappropriate communication by nursing staff. Aim: This study explores how parents of a child with hearing impairment experience the communication between the nursing staff and their hospitalised child. Method: The study was conducted together with an advisory centre for hearing-impaired children, where most of the parents could be recruited. Narrative, semi-structured interviews were conducted. The transcribed interviews were analysed according to the method of interpretative phenomenology. Results: The parents expressed their wish for affectionate verbal and nonverbal love and care for their child. They often experienced the nursing staff having little time, that there was no continuity and that the communicative needs of the child were not recognised. Since the parents did not think the nursing staff were capable of communicating with the child and because they wanted to protect him or her, they adopted a mediating role. Conclusions: Besides the sensitisation of the nursing staff, time resources, continuity, professional knowledge and benevolence in the nursing care of a child with hearing impairment play a fundamental role.

Methods of technical and tactical training basketball players with hearing impairments using innovative technologies

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Zh.L Kozina

2014-06-01

Full Text Available Purpose: to develop information technology for technical and tactical training of skilled basketball players with hearing impairments. Material : the study involved 24 women's national team athletes Ukrainian Basketball hearing impaired (age - 25-30 years. Technical protocols were processed 12 games from the World Cup and Europe, XXII Summer Deaflympics. Fixed number of shots and hit free throws, steals, rebounds and on its offensive rebounds, fouls and errors. Results : the developed system of tactical training basketball players with hearing impairments. Accentuation was made of the prevalence of method visibility while developing tactical interactions. The main result was the development of the author's method of video tutorials with animated illustrations. Also, use the lamp LS Line-3-65-12-C to control the training process athletes. Conclusions : there was a significant improvement in competitive activity athletes. Athletes performed significantly more hits from the foul line and successful shots from long range compared to main rivals. Also increased the number of interceptions in games. Application of information technology had an impact on the competitive result: basketball team of Ukraine took the second place in the Deaflympics in Sofia in 2013.

Visual impairment in Finnish Usher syndrome type III.

NARCIS (Netherlands)

Plantinga, R.F.; Pennings, R.J.E.; Huygen, P.L.M.; Sankila, E.M.; Tuppurainen, K.; Kleemola, L.; Cremers, C.W.R.J.; Deutman, A.F.

2006-01-01

PURPOSE: To evaluate visual impairment in Finnish Usher syndrome type 3 (USH3) and compare this with visual impairment in Usher syndrome types 1b (USH1b) and 2a (USH2a). METHODS: We carried out a retrospective study of 28 Finnish USH3 patients, 24 Dutch USH2a patients and 17 Dutch USH1b patients.

Disease: H00604 [KEGG MEDICUS

Lifescience Database Archive (English)

Full Text Available H00604 Deafness, autosomal dominant Hereditary deafness is divided into syndromic forms (in which hearing... with 2-3% of the cases of non-syndromic hearing loss. Autosomal-dominant forms of deafness are usually post...-lingual and progressive. Dominant mutations may be consistent with initial function and subsequent hearin...Griffin A, Fernandez B, Lee MK, King MC ... TITLE ... Non-syndromic progressive hearing loss DFNA38 is caused ...2 deficiency impairs otic development and hearing ability in a zebrafish model of

Hearing rehabilitation with single-stage bilateral vibroplasty in a child with Franceschetti syndrome.

Science.gov (United States)

Sargsyan, Sona; Rahne, Torsten; Kösling, Sabrina; Eichler, Gerburg; Plontke, Stefan K

2014-05-01

Hearing is of utmost importance for normal speech and social development. Even children who have mild or unilateral permanent hearing loss may experience difficulties with understanding speech, as well as problems with educational and psycho-social development. The increasing advantages of middle-ear implant technologies are opening new perspectives for restoring hearing. Active middle-ear implants can be used in children and adolescents with hearing loss. In addition to the well-documented results for improving speech intelligibility and quality of hearing in sensorineural hearing loss active middle-ear implants are now successfully used in patients with conductive and mixed hearing loss. In this article we present a case of successful, single-stage vibroplasty, on the right side with the fixation of the FMT on the stapes and PORP CLiP vibroplasty on the left side in a 6-year-old girl with bilateral mixed hearing loss and multiple dyslalia associated with Franceschetti syndrome (mandibulofacial dysostosis). CT revealed bilateral middle-ear malformations as well as an atretic right and stenotic left external auditory canal. Due to craniofacial dysmorphia airway and (post)operative, management is significantly more difficult in patients with a Franceschetti syndrome which in this case favoured a single-stage bilateral procedure. No intra- or postoperative surgical complications were reported. The middle-ear implants were activated 4 weeks after surgery. In the audiological examination 6 months after surgery, the child showed 100% speech intelligibility with activated implants on each side.

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

Science.gov (United States)

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…

The preparation for the year main competition teams in basketball with hearing impairments with innovative technologies

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I.N. Sobko

2014-10-01

Full Text Available Purpose : develop and prove experimentally comprehensive training program on the Ukrainian national team basketball with hearing impairment in the annual cycle for the major competitions. Material : The study involved 12 basketball hearing impaired 20-25 years old - female players team of Ukraine on basketball. Also analyzed the test results and competitive activity 12 basketball players with hearing impairments - Lithuanian team players. Results : We showed the need for a qualitative change in the training process through the development and application of innovative technologies. This allows a greater level of communication between the coach and athletes to intensify training process. Developed and experimentally substantiated comprehensive training program for the Ukrainian national team. In technical training device used light. This increased mobility, agility, activity and intensity workouts. In tactical training improved situational and planned change tactical drawing game using copyright protection of video tutorials with animated illustrations. Conclusions : A positive impact of the developed system for basketball training result in major competitions.

Hereditary Hearing Loss.

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Tran, LenhAnh P.; Grundfast, Kenneth M.

1997-01-01

This article discusses inheritance patterns in hearing loss, epidemiology, clues to genetic causes, locating genes that cause hereditary disorders, genes related to hearing loss disorders in individuals with Usher syndrome, Waardenburg syndrome, Treacher-Collins syndrome, Branchio-oto-renal and Pendred syndromes, and the significance of finding…

Relations between psychophysical data and speech perception for hearing-impaired subjects. II

NARCIS (Netherlands)

Dreschler, W. A.; Plomp, R.

1985-01-01

Twenty-one sensorineurally hearing-impaired adolescents were studied with an extensive battery of tone-perception, phoneme-perception, and speech-perception tests. Tests on loudness perception, frequency selectivity, and temporal resolution at the test frequencies of 500, 1000, and 2000 Hz were

Causes of hearing impairment in the Norwegian paediatric cochlear implant program.

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Siem, Geir; Fagerheim, Toril; Jonsrud, Christoffer; Laurent, Claude; Teig, Erik; Harris, Sten; Leren, Trond P; Früh, Andreas; Heimdal, Ketil

2010-08-01

Severe to profound hearing impairment (HI) is estimated to affect around 1/2000 young children. Advances in genetics have made it possible to identify several genes related to HI. This information can cast light upon prognostic factors regarding the outcome in cochlear implantation, and provide information both for scientific and genetic counselling purposes. From 1992 to 2005, 273 children from 254 families (probands) were offered cochlear implants in Norway. An evaluation of the causes of HI, especially regarding the genes GJB2, GJB6, SLC26A4, KCNQ1, KCNE1, and the mutation A1555G in mitochondrial DNA was performed in 85% of the families. The number of probands with unknown cause of HI was thus reduced from 120 to 68 (43% reduction). Ninety-eight (46%) of the probands had an identified genetic etiology of their HI. A relatively high prevalence of Jervell and Lange-Nielsen syndrome was found. The main causes of severe and profound HI were similar to those found in other European countries. GJB2 mutations are a common cause of prelingual HI in Norwegian cochlear implanted children.

A mutation in synaptojanin 2 causes progressive hearing loss in the ENU-mutagenised mouse strain Mozart.

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Manji, Shehnaaz S M; Williams, Louise H; Miller, Kerry A; Ooms, Lisa M; Bahlo, Melanie; Mitchell, Christina A; Dahl, Hans-Henrik M

2011-03-15

Hearing impairment is the most common sensory impairment in humans, affecting 1:1,000 births. We have identified an ENU generated mouse mutant, Mozart, with recessively inherited, non-syndromic progressive hearing loss caused by a mutation in the synaptojanin 2 (Synj2), a central regulatory enzyme in the phosphoinositide-signaling cascade. The hearing loss in Mozart is caused by a p.Asn538Lys mutation in the catalytic domain of the inositol polyphosphate 5-phosphatase synaptojanin 2. Within the cochlea, Synj2 mRNA expression was detected in the inner and outer hair cells but not in the spiral ganglion. Synj2(N538K) mutant protein showed loss of lipid phosphatase activity, and was unable to degrade phosphoinositide signaling molecules. Mutant Mozart mice (Synj2(N538K/N538K)) exhibited progressive hearing loss and showed signs of hair cell degeneration as early as two weeks of age, with fusion of stereocilia followed by complete loss of hair bundles and ultimately loss of hair cells. No changes in vestibular or neurological function, or other clinical or behavioral manifestations were apparent. Phosphoinositides are membrane associated signaling molecules that regulate many cellular processes including cell death, proliferation, actin polymerization and ion channel activity. These results reveal Synj2 as a critical regulator of hair cell survival that is essential for hair cell maintenance and hearing function.

Hearing Status in Pediatric Renal Transplant Recipients.

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Gulleroglu, Kaan; Baskin, Esra; Aydin, Erdinc; Ozluoglu, Levent; Moray, Gokhan; Haberal, Mehmet

2015-08-01

Renal transplant provides a long-term survival. Hearing impairment is a major factor in subjective health status. Status of hearing and the cause of hearing impairment in the pediatric renal transplant group have not been evaluated. Here, we studied to evaluate hearing status in pediatric renal transplant patients and to determine the factors that cause hearing impairment. Twenty-seven pediatric renal transplant recipients were investigated. All patients underwent audiologic assessment by means of pure-tone audiometry. The factors on hearing impairment were performed. Sensorineural hearing impairment was found in 17 patients. There was marked hearing impairment for the higher frequencies between 4000 and 8000 Hz. Sudden hearing loss developed in 2 patients, 1 of them had tinnitus. Decrease of speech understanding was found in 8 patients. The cyclosporine level was significantly high in patients with hearing impairment compared with group without hearing impairment. Cyclosporine levels also were found to be statistically significantly high when compared with the group with decrease of speech understanding and the group without decrease of speech understanding. Similar relations cannot be found between tacrolimus levels and hearing impairment and speech understanding. Sensorineural hearing impairment prevalence was high in pediatric renal transplant recipients when compared with the general population of children. Cyclosporine may be responsible for causing hearing impairment after renal transplant. We suggest that this effect is a dose-dependent toxicity.

Teaching Hearing-Impaired Children in Iraq Using a New Teaching Method.

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Harris, N. D. C.; Mustafa, N.

1986-01-01

Describes a field test and results of a new didactic teaching method involving resource-based learning to teach various aspects of mathematics and science (fractions, magnetism, planets) to elementary aged hearing impaired student in Iraq. The dramatic improvements in language for learners is described and implications of the methods are…

Stress in Mothers of Hearing Impaired Children Compared to Mothers of Normal and Other Disabled Children

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Mahnaz Aliakbari Dehkordi

2011-06-01

Full Text Available Background and Aim: Stress is associated with life satisfaction and also development of some physical diseases. Birth of a disabled child with mental or physical disability (especially deaf or blind children, impose an enormous load of stress on their parents especially the mothers. This study compared stress levels of mothers with hearing impaired children and mothers of normal children or with other disabilities.Methods: In this study, cluster random sampling was performed in Karaj city. 120 mothers in four groups of having a child with mental retardation, low vision, hearing impairment and with normal children were included. Family inventory of life events (FILE of Mc Cubbin et al. was used to determine stress level in four groups of mothers.Results: The results of this research indicated a significant difference (p<0.05 between stress levels of mothers with hearing impaired children and mothers of other disabled and normal children in subscales of intra-family stress, finance and business strains, stress of job transitions, stress of illness and family care and family members "in and out''. There was no difference between compared groups in other subscales.Conclusion: Since deafness is a hidden inability, the child with hearing impairment has a set of social and educational problems causing great stress for parents, especially to mother. In order to decrease mother’s stress, it is suggested to provide more family consultation, adequate social support and to run educational classes for parents to practice stress coping strategies.

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

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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.

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

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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)

A potential source of hearing impairment; headphones

International Nuclear Information System (INIS)

Tabraiz, S.; Asif, M.B.; Iftekhar, S.; Ishtiaq, T.

2014-01-01

Technological expansions have been observed in recent years for portable devices with hi-fi audio playback capability such as MP4 players, multimedia phones and hand-held game consoles. Usually these devices are used with headphones; therefore the noise levels of different headphones are of particular relevance. Despite of its several benefits, noise levels can be quite high and may cause hearing impairment. In this research, different headphones were selected to check noise levels at varying volumes. It was found in majority of cases that noise levels ranged from 75dB to 85 dB; surpassing the NEQ's and OSHA standard values. (author)

An Estimation of the Whole-of-Life Noise Exposure of Adolescent and Young Adult Australians with Hearing Impairment.

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Carter, Lyndal; Black, Deborah; Bundy, Anita; Williams, Warwick

2016-10-01

Since amplified music gained widespread popularity, there has been community concern that leisure-noise exposure may cause hearing loss in adolescents and young adults who would otherwise be free from hearing impairment. Repeated exposure to personal stereo players and music events (e.g., nightclubbing, rock concerts, and music festivals) are of particular concern. The same attention has not been paid to leisure-noise exposure risks for young people with hearing impairment (either present from birth or acquired before adulthood). This article reports on the analysis of a subset of data (leisure participation measures) collected during a large, two-phase study of the hearing health, attitudes, and behaviors of 11- to 35-yr-old Australians conducted by the National Acoustic Laboratories (n = 1,667 hearing threshold level datasets analyzed). The overall aim of the two-phase study was to determine whether a relationship between leisure-noise exposure and hearing loss exists. In the current study, the leisure activity profiles and accumulated ("whole-of-life") noise exposures of young people with (1) hearing impairment and (2) with normal hearing were compared. Cross-sectional cohort study. Hearing impaired (HI) group, n = 125; normal (nonimpaired) hearing (NH) group, n = 296, analyzed in two age-based subsets: adolescents (13- to 17-yr-olds) and young adults (18- to 24-yr-olds). Participant survey. The χ² test was used to identify systematic differences between the leisure profiles and exposure estimates of the HI and NH groups. Whole-of-life noise exposure was estimated by adapting techniques described in ISO 1999. For adolescents, leisure profiles were similar for the two groups and few individuals exceeded the stated risk criterion. For young adults, participation was significantly lower for the HI group for 7 out of 18 leisure activities surveyed. Activity diversity and whole-of-life exposure were also significantly lower for the HI group young adults. A

Mutation screening of USH3 gene (clarin-1) in Spanish patients with Usher syndrome: low prevalence and phenotypic variability.

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Aller, E; Jaijo, T; Oltra, S; Alió, J; Galán, F; Nájera, C; Beneyto, M; Millán, J M

2004-12-01

Usher syndrome type III is an autosomal recessive disorder clinically characterized by the association of retinitis pigmentosa (RP), variable presence of vestibular dysfunction and progressive hearing loss, being the progression of the hearing impairment the critical parameter classically used to distinguish this form from Usher syndrome type I and Usher syndrome type II. Usher syndrome type III clinical subtype is the rarest form of Usher syndrome in Spain, accounting only for 6% of all Usher syndrome Spanish cases. The gene responsible for Usher syndrome type III is named clarin-1 and it is thought to be involved in hair cell and photoreceptor cell synapses. Here, we report a screening for mutations in clarin-1 gene among our series of Usher syndrome Spanish patients. Clarin-1 has been found to be responsible for the disease in only two families: the first one is a previously reported family homozygous for Y63X mutation and the second one, described here, is homozygous for C40G. This accounts for 1.7% of Usher syndrome Spanish families. It is noticeable that, whereas C40G family is clinically compatible with Usher syndrome type III due to the progression of the hearing loss, Y63X family could be diagnosed as Usher syndrome type I because the hearing impairment is profound and stable. Thus, we consider that the progression of hearing loss is not the definitive key parameter to distinguish Usher syndrome type III from Usher syndrome type I and Usher syndrome type II.

Usher's syndrome--case report.

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Kwiecień, Sława; Sulak, Robert; Szaflik, Jerzy

2008-01-01

The aim of this study is to present a case of coincidence of sensorineural hearing loss with chronic recurrent bilateral cystoid macular oedema in a 32-year-old woman, who was admitted to the clinic for deterioration of visual acuity of four months' duration. The patient gave a history of hearing loss for 29 years. Visual field examination disclosed peripheral ring scotoma. Electrophysiological examination was performed: pattern visual evoked response was within normal limits and electroretinogram displayed diminished both photopic and scotopic response. As ophthalmoscopy demonstrated no pigment in the fundus of the eye, the findings were consisted with diagnosis of retinitis pigmentosis sine pigmento. The presence of loss of hearing indicated the necessity of performing the genetic examination for Usher's syndrome. In order to establish a final diagnosis of Usher's syndrome genetic examination must be performed, but family history is relevant. Early investigation for Usher's syndrome in children with sensorineural hearing impairment is of a great significance. The patient may develop symptoms of retinitis pigmentosa in second or even third decade of his life. The necessity of thorough investigation for detecting other systemic abnormalities should be emphasized. There is no effective treatment of this syndrome. A child with Usher's syndrome requires a comprehensive care of different medical specialties. Psychological, educational and sociological attitude is also of a great importance in the child development.

Tactile and bone-conduction auditory brain computer interface for vision and hearing impaired users.

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Rutkowski, Tomasz M; Mori, Hiromu

2015-04-15

The paper presents a report on the recently developed BCI alternative for users suffering from impaired vision (lack of focus or eye-movements) or from the so-called "ear-blocking-syndrome" (limited hearing). We report on our recent studies of the extents to which vibrotactile stimuli delivered to the head of a user can serve as a platform for a brain computer interface (BCI) paradigm. In the proposed tactile and bone-conduction auditory BCI novel multiple head positions are used to evoke combined somatosensory and auditory (via the bone conduction effect) P300 brain responses, in order to define a multimodal tactile and bone-conduction auditory brain computer interface (tbcaBCI). In order to further remove EEG interferences and to improve P300 response classification synchrosqueezing transform (SST) is applied. SST outperforms the classical time-frequency analysis methods of the non-linear and non-stationary signals such as EEG. The proposed method is also computationally more effective comparing to the empirical mode decomposition. The SST filtering allows for online EEG preprocessing application which is essential in the case of BCI. Experimental results with healthy BCI-naive users performing online tbcaBCI, validate the paradigm, while the feasibility of the concept is illuminated through information transfer rate case studies. We present a comparison of the proposed SST-based preprocessing method, combined with a logistic regression (LR) classifier, together with classical preprocessing and LDA-based classification BCI techniques. The proposed tbcaBCI paradigm together with data-driven preprocessing methods are a step forward in robust BCI applications research. Copyright © 2014 Elsevier B.V. All rights reserved.

Usher syndrome type III can mimic other types of Usher syndrome.

NARCIS (Netherlands)

Pennings, R.J.E.; Fields, R.R.; Huygen, P.L.M.; Deutman, A.F.; Kimberling, W.J.; Cremers, C.W.R.J.

2003-01-01

Clinical and genetic characteristics are presented of 2 patients from a Dutch Usher syndrome type III family who have a new homozygous USH3 gene mutation: 149-152delCAGG + insTGTCCAAT. One individual (IV:1) is profoundly hearing impaired and has normal vestibular function and retinitis punctata

Genetic inactivation of Trpml3 does not lead to hearing and vestibular impairment in mice.

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Simone Jörs

2010-12-01

Full Text Available TRPML3, a member of the transient receptor potential (TRP family, is an inwardly rectifying, non-selective Ca2+-permeable cation channel that is regulated by extracytosolic Na+ and H+ and can be activated by a variety of small molecules. The severe auditory and vestibular phenotype of the TRPML3(A419P varitint-waddler mutation made this protein particularly interesting for inner ear biology. To elucidate the physiological role of murine TRPML3, we conditionally inactivated Trpml3 in mice. Surprisingly, lack of functional TRPML3 did not lead to circling behavior, balance impairment or hearing loss.

Delayed diagnosis of a patient with Usher syndrome 1C in a Louisiana Acadian family highlights the necessity of timely genetic testing for the diagnosis and management of congenital hearing loss.

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Umrigar, Ayesha; Musso, Amanda; Mercer, Danielle; Hurley, Annette; Glausier, Cassondra; Bakeer, Mona; Marble, Michael; Hicks, Chindo; Tsien, Fern

2017-01-01

Advances in sequencing technologies and increased understanding of the contribution of genetics to congenital sensorineural hearing loss have led to vastly improved outcomes for patients and their families. Next-generation sequencing and diagnostic panels have become increasingly reliable and less expensive for clinical use. Despite these developments, the diagnosis of genetic sensorineural hearing loss still presents challenges for healthcare providers. Inherited sensorineural hearing loss has high levels of genetic heterogeneity and variable expressivity. Additionally, syndromic hearing loss (hearing loss and additional clinical abnormalities) should be distinguished from non-syndromic (hearing loss is the only clinical symptom). Although the diagnosis of genetic sensorineural hearing loss can be challenging, the patient's family history and ethnicity may provide critical information, as certain genetic mutations are more common in specific ethnic populations. The early identification of the cause of deafness can benefit patients and their families by estimating recurrence risks for future family planning and offering the proper interventions to improve their quality of life. Collaboration between pediatricians, audiologists, otolaryngologists, geneticists, and other specialists are essential in the diagnosis and management of patients with hearing disorders. An early diagnosis is vital for proper management and care, as some clinical manifestations of syndromic sensorineural hearing loss are not apparent at birth and have a delayed age of onset. We present a case of Usher syndrome (congenital deafness and childhood-onset blindness) illustrating the challenges encountered in the diagnosis and management of children presenting with congenital genetic sensorineural hearing loss, along with helpful resources for clinicians and families.

Ichthyosis Follicularis with Atrichia and Photophobia Syndrome: First Case Report in Thailand

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Rattanavalai Nitiyarom

2017-05-01

Full Text Available We herein describe the first reported case of Ichthyosis Follicularis with Atrichia and Photophobia (IFAP syndrome in Thailand. A 6-year-old boy presented with a history of photophobia since 1 month of age. Then he developed widespread follicular hyperkeratotic papules and subtotal non-scarring alopecia by the age of 10 months and 5 years, respectively. Sparse eyelashes and nail dystrophy were also noted. No neurological abnormalities, systemic involvement, and hearing impairment were observed. The clinical manifestations were consistent with IFAP syndrome, although genetic testing did not confirm the diagnosis of this rare disorder.

Oral Communication Development in Severe to Profound Hearing Impaired Children After Receiving Aural Habilitation

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Soleimani Farin

2009-10-01

Full Text Available Communication, cognition, language, and speech are interrelated and develop together. It should come as no surprise to us that the key to intervention with deaf children is to establish, as early as possible, a functional communication system for the child and the parents. Early intervention programs need to be multidisciplinary, technologically sound and most important, it should take cognizance of the specific context (community, country in which the child and family function. The main aim of this study was to obtain oral communication development regarding current status of the intervention (aural habilitation and speech therapyfor children with severe to profound hearing impairment in Iran. A prospective longitudinal study was undertaken on a consecutive group of children with severe to profound deafness. Nine severe to profound hearing-impaired children out of the primer 42 cases, who were detected below two years old, had been selected in the previous study to receive aural habilitation. The average of their speech intelligibility scores was near 70% at age 6, which was accounted as poor oral communication and only two of them were able to communicate by spoken language. An integrated intervention services continued again for one year and their oral communication skill was assessed by their speech intelligibility. The intelligibility test of children was recorded on audio-tape, when they read 10 questions such as where is your home. This can be answered only in one word. Each tape was presented to10 normal hearing listeners, and their task was to write down, the answers in Persian orthography. At the beginning (at age 6 the average speech intelligibility score of these children was 72% and only two of them had score of 90% and 100%. At age 7, all of the severe groups were over 90%, and only two profound ones achieved the score of 48% and 62%. All of severe groups develop oral communication, but profound ones had a semi-intelligible speech

Adaptive psychological structure in childhood hearing impairment: audiological correlations.

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

2017-06-01

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

Self-Esteem in Hearing-Impaired Children: The Influence of Communication, Education, and Audiological Characteristics

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Theunissen, Stephanie C. P. M.; Rieffe, Carolien; Netten, Anouk P.; Briaire, Jeroen J.; Soede, Wim; Kouwenberg, Maartje; Frijns, Johan H. M.

2014-01-01

OBJECTIVE: Sufficient self-esteem is extremely important for psychosocial functioning. It is hypothesized that hearing-impaired (HI) children have lower levels of self-esteem, because, among other things, they frequently experience lower language and communication skills. Therefore, the aim of this study was to compare HI children's self-esteem across different domains with those of normal hearing (NH) children and to investigate the influence of communication, type of education, and audiolog...

Frequency of Usher syndrome in two pediatric populations: Implications for genetic screening of deaf and hard of hearing children.

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Kimberling, William J; Hildebrand, Michael S; Shearer, A Eliot; Jensen, Maren L; Halder, Jennifer A; Trzupek, Karmen; Cohn, Edward S; Weleber, Richard G; Stone, Edwin M; Smith, Richard J H

2010-08-01

Usher syndrome is a major cause of genetic deafness and blindness. The hearing loss is usually congenital and the retinitis pigmentosa is progressive and first noticed in early childhood to the middle teenage years. Its frequency may be underestimated. Newly developed molecular technologies can detect the underlying gene mutation of this disorder early in life providing estimation of its prevalence in at risk pediatric populations and laying a foundation for its incorporation as an adjunct to newborn hearing screening programs. A total of 133 children from two deaf and hard of hearing pediatric populations were genotyped first for GJB2/6 and, if negative, then for Usher syndrome. Children were scored as positive if the test revealed > or =1 pathogenic mutations in any Usher gene. Fifteen children carried pathogenic mutations in one of the Usher genes; the number of deaf and hard of hearing children carrying Usher syndrome mutations was 15/133 (11.3%). The population prevalence was estimated to be 1/6000. Usher syndrome is more prevalent than has been reported before the genome project era. Early diagnosis of Usher syndrome has important positive implications for childhood safety, educational planning, genetic counseling, and treatment. The results demonstrate that DNA testing for Usher syndrome is feasible and may be a useful addition to newborn hearing screening programs.

Finding new genes for non-syndromic hearing loss through an in silico prioritization study.

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Matteo Accetturo

Full Text Available At present, 51 genes are already known to be responsible for Non-Syndromic hereditary Hearing Loss (NSHL, but the knowledge of 121 NSHL-linked chromosomal regions brings to the hypothesis that a number of disease genes have still to be uncovered. To help scientists to find new NSHL genes, we built a gene-scoring system, integrating Gene Ontology, NCBI Gene and Map Viewer databases, which prioritizes the candidate genes according to their probability to cause NSHL. We defined a set of candidates and measured their functional similarity with respect to the disease gene set, computing a score ( S S M avg that relies on the assumption that functionally related genes might contribute to the same (disease phenotype. A Kolmogorov-Smirnov test, comparing the pair-wise distribution on the disease gene set with the distribution on the remaining human genes, provided a statistical assessment of this assumption. We found at a p-value 0.99. The twenty top-scored genes were finally examined to evaluate their possible involvement in NSHL. We found that half of them are known to be expressed in human inner ear or cochlea and are mainly involved in remodeling and organization of actin formation and maintenance of the cilia and the endocochlear potential. These findings strongly indicate that our metric was able to suggest excellent NSHL candidates to be screened in patients and controls for causative mutations.

Encouraging Reading through an Enactive Method: Strategies for Hearing Impaired Children.

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Winzer, M. A.

The paper describes the enactive method, an alternative approach to introducing and teaching reading to young hearing impaired children. The method actively involves the child as a processor of the material rather than as a passive consumer. The approach is established for a short period of time each day until the student outgrows its original…

Evaluation of Audiometric Test Results to Determine Hearing Impairment in Patients with Rheumatoid Arthritis: Analysis of Data from the Korean National Health and Nutrition Examination Survey.

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Hyemin Jeong

Full Text Available This study aimed to evaluate the association between rheumatoid arthritis (RA and hearing impairment in the Korean adult population. Audiometric and laboratory test data from the 2010-2012 Korean National Health and Nutrition Examination Survey (KNHANES were used for analysis. The relationship between RA and hearing impairment was analyzed, adjusting for various known risk factors associated with hearing impairment. RA was defined in the questionnaire as "RA diagnosed by a physician (yes/no through a standardized interview." We defined hearing impairment according to 2 categories of frequency (low/mid and high as follows (average values in kHz: low/mid frequency, 0.5, 1.0, and 2.0, and high frequency, 3.0, 4.0, and 6.0. Of the subjects, 15,158 (weighted n = 32,035,996 completed the audiometric tests. The overall weighted prevalence of RA was 1.5%. The prevalence of hearing impairment was higher in the subjects with RA than in those without RA, in both, the low/mid- and high-frequency categories (21.1% vs 7.5%, p < 0.001 and 43.3% vs. 26.2%, p < 0.001, respectively. In the multivariable logistic analysis, RA (odds ratios [OR] 1.47, 95% confidence interval [CI] 1.05-2.06, p = 0.025 was an independent risk factor of low/mid-frequency hearing impairment along with age (OR 1.12, 95% CI 1.12-1.13, p < 0.001, current smoking (OR 1.27, 95% CI 1.03-1.56, p = 0.026, and college graduation (OR 0.53, 95% CI 0.39-0.72, p < 0.001. In the multivariable analysis of high-frequency hearing impairment, RA did not show any association with hearing impairment. This study suggests that RA is associated with low/mid-frequency hearing impairment after adjustment for various known risk factors. Further study is needed to verify the hearing impairment in RA.

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

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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…

Hearing impairment associated with oral terbinafine use : a case series and case/non-case analysis in the Netherlands Pharmacovigilance Centre Lareb database and VigiBase™

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Scholl, Joep H G; van Puijenbroek, Eugene P

2012-01-01

BACKGROUND: The Netherlands Pharmacovigilance Centre Lareb received reports of six cases of hearing impairment in association with oral terbinafine use. This study describes these cases and provides support for this association from the Lareb database for spontaneous adverse drug reaction (ADR)

Comprehensive molecular etiology analysis of nonsyndromic hearing impairment from typical areas in China

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Kang Dongyang

2009-09-01

Full Text Available Abstract Background Every year, 30,000 babies are born with congenital hearing impairment in China. The molecular etiology of hearing impairment in the Chinese population has not been investigated thoroughly. To provide appropriate genetic testing and counseling to families, we performed a comprehensive investigation of the molecular etiology of nonsyndromic deafness in two typical areas from northern and southern China. Methods A total of 284 unrelated school children with hearing loss who attended special education schools in China were enrolled in this study, 134 from Chifeng City in Inner Mongolia and the remaining 150 from Nangtong City in JiangSu Province. Screening was performed for GJB2, GJB3, GJB6, SLC26A4, 12S rRNA, and tRNAser(UCN genes in this population. All patients with SLC26A4 mutations or variants were subjected to high-resolution temporal bone CT scan to verify the enlarged vestibular aqueduct. Results Mutations in the GJB2 gene accounted for 18.31% of the patients with nonsyndromic hearing loss, 1555A>G mutation in mitochondrial DNA accounted for 1.76%, and SLC26A4 mutations accounted for 13.73%. Almost 50% of the patients with nonsyndromic hearing loss in these typical Chinese areas carried GJB2 or SLC26A4 mutations. No significant differences in mutation spectrum or prevalence of GJB2 and SLC26A4 were found between the two areas. Conclusion In this Chinese population, 54.93% of cases with hearing loss were related to genetic factors. The GJB2 gene accounted for the etiology in about 18.31% of the patients with hearing loss, SLC26A4 accounted for about 13.73%, and mtDNA 1555A>G mutation accounted for 1.76%. Mutations in GJB3, GJB6, and mtDNA tRNAser(UCN were not common in this Chinese cohort. Conventionally, screening is performed for GJB2, SLC26A4, and mitochondrial 12S rRNA in the Chinese deaf population.

A Deficit in Movement-Derived Sentences in German-Speaking Hearing-Impaired Children

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Esther Ruigendijk

2017-06-01

Full Text Available Children with hearing impairment (HI show disorders in syntax and morphology. The question is whether and how these disorders are connected to problems in the auditory domain. The aim of this paper is to examine whether moderate to severe hearing loss at a young age affects the ability of German-speaking orally trained children to understand and produce sentences. We focused on sentence structures that are derived by syntactic movement, which have been identified as a sensitive marker for syntactic impairment in other languages and in other populations with syntactic impairment. Therefore, our study tested subject and object relatives, subject and object Wh-questions, passive sentences, and topicalized sentences, as well as sentences with verb movement to second sentential position. We tested 19 HI children aged 9;5–13;6 and compared their performance with hearing children using comprehension tasks of sentence-picture matching and sentence repetition tasks. For the comprehension tasks, we included HI children who passed an auditory discrimination task; for the sentence repetition tasks, we selected children who passed a screening task of simple sentence repetition without lip-reading; this made sure that they could perceive the words in the tests, so that we could test their grammatical abilities. The results clearly showed that most of the participants with HI had considerable difficulties in the comprehension and repetition of sentences with syntactic movement: they had significant difficulties understanding object relatives, Wh-questions, and topicalized sentences, and in the repetition of object who and which questions and subject relatives, as well as in sentences with verb movement to second sentential position. Repetition of passives was only problematic for some children. Object relatives were still difficult at this age for both HI and hearing children. An additional important outcome of the study is that not all sentence structures

Association between vestibular function and motor performance in hearing-impaired children.

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Maes, Leen; De Kegel, Alexandra; Van Waelvelde, Hilde; Dhooge, Ingeborg

2014-12-01

The clinical balance performance of normal-hearing (NH) children was compared with the balance performance of hearing-impaired (HI) children with and without vestibular dysfunction to identify an association between vestibular function and motor performance. Prospective study. Tertiary referral center. Thirty-six children (mean age, 7 yr 5 mo; range, 3 yr 8 mo-12 yr 11 mo) divided into three groups: NH children with normal vestibular responses, HI children with normal vestibular responses, and HI children with abnormal vestibular function. A vestibular test protocol (rotatory and collic vestibular evoked myogenic potential testing) in combination with three clinical balance tests (balance beam walking, one-leg hopping, one-leg stance). Clinical balance performance. HI children with abnormal vestibular test results obtained the lowest quotients of motor performance, which were significantly lower compared with the NH group (p beam walking and one-leg stance; p = 0.003 for one-leg hopping). The balance performance of the HI group with normal vestibular responses was better in comparison with the vestibular impaired group but still significantly lower compared with the NH group (p = 0.020 for balance beam walking; p = 0.001 for one-leg stance; not significant for one-leg hopping). These results indicate an association between vestibular function and motor performance in HI children, with a more distinct motor deterioration if a vestibular impairment is superimposed to the auditory dysfunction.

SELF-REGULATION STRATEGIES OF ORAL COMMUNICATION IN CHILDREN WITH HEARING IMPAIRMENT

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Alois GHERGUT

2011-09-01

Full Text Available The study identifies some self-regulation strategies used by deaf children in order to make their speech more intelligible. To achieve self-control while speaking, the child with severe hearing loss needs not only a high level of intelligence, but also an effective lip-reading capability and a strong intrinsic motivation. This is the reason why there are many cases of children with a high level of intelligence, but with a mediocre lip-reading capability and others with a lower level of intelligence, but with a good lip-reading capability. These differences also depend on the degree of hearing loss. Among the self-regulation strategies used by the children that achieve an intelligible speech are: the cognitive and meta-cognitive strategies, the motivational strategies etc. These results are important while designing the therapeutic activities, and especially the speech intelligibility factor being crucial in the social integration of those children with hearing impairment.

Dynamic-range reduction by peak clipping or compression and its effects on phoneme perception in hearing-impaired listeners

NARCIS (Netherlands)

Dreschler, W. A.

1988-01-01

In this study, differences between dynamic-range reduction by peak clipping and single-channel compression for phoneme perception through conventional hearing aids have been investigated. The results from 16 hearing-impaired listeners show that compression limiting yields significantly better

Opinions of hearing parents about the causes of hearing impairment of their children with biallelic GJB2 mutations.

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Solovyev, Aisen V; Dzhemileva, Lilya U; Posukh, Olga L; Barashkov, Nikolay A; Bady-Khoo, Marita S; Lobov, Semen L; Popova, Natalya Yu; Romanov, Georgii P; Sazonov, Nikolay N; Bondar, Alexander A; Morozov, Igor V; Tomsky, Mikhail I; Fedorova, Sardana A; Khusnutdinova, Elza K

2017-07-01

Hereditary hearing impairment (HI) caused by recessive GJB2 mutations is a frequent sensory disorder. The results of the molecular-based studies of HI are widely used in various genetic test systems. However, the ethical aspects are less described than the genetic aspects. The concerns expressed by individuals from groups with genetic risks must be included in the counseling of patients and their families. For evaluation of subjective opinions of hearing parents about the presumed causes of HI of their children, we analyze the cohort of parents having children with confirmed hereditary HI caused by biallelic recessive GJB2 mutations (in a homozygous or a compound heterozygous state). This study included 70 deaf children with HI due to mutations in the GJB2 gene and 91 questionnaires about the presumed causes of their deafness filled by their parents. Most of the parents at 78% (CI 68.4-85.4%) attributed their children's HI to "non-hereditary" causes and 22% (CI 14.7-31.6%) to "hereditary" causes (p < 0.05). Therefore, the prior opinions of the parents did not correspond to positive GJB2 genetic testing results. The subjective opinions of parents are probably partly based on family history, since respondents with deaf relatives in their pedigree more likely supposed hereditary causes for HI in their children than the respondents without deaf relatives (p < 0.001).

The impact of hearing aids and age-related hearing loss on auditory plasticity across three months - An electrical neuroimaging study.

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Giroud, Nathalie; Lemke, Ulrike; Reich, Philip; Matthes, Katarina L; Meyer, Martin

2017-09-01

The present study investigates behavioral and electrophysiological auditory and cognitive-related plasticity in three groups of healthy older adults (60-77 years). Group 1 was moderately hearing-impaired, experienced hearing aid users, and fitted with new hearing aids using non-linear frequency compression (NLFC on); Group 2, also moderately hearing-impaired, used the same type of hearing aids but NLFC was switched off during the entire period of study duration (NLFC off); Group 3 represented individuals with age-appropriate hearing (NHO) as controls, who were not different in IQ, gender, or age from Group 1 and 2. At five measurement time points (M1-M5) across three months, a series of active oddball tasks were administered while EEG was recorded. The stimuli comprised syllables consisting of naturally high-pitched fricatives (/sh/, /s/, and /f/), which are hard to distinguish for individuals with presbycusis. By applying a data-driven microstate approach to obtain global field power (GFP) as a measure of processing effort, the modulations of perceptual (P50, N1, P2) and cognitive-related (N2b, P3b) auditory evoked potentials were calculated and subsequently related to behavioral changes (accuracy and reaction time) across time. All groups improved their performance across time, but NHO showed consistently higher accuracy and faster reaction times than the hearing-impaired groups, especially under difficult conditions. Electrophysiological results complemented this finding by demonstrating longer latencies in the P50 and the N1 peak in hearing aid users. Furthermore, the GFP of cognitive-related evoked potentials decreased from M1 to M2 in the NHO group, while a comparable decrease in the hearing-impaired group was only evident at M5. After twelve weeks of hearing aid use of eight hours each day, we found a significantly lower GFP in the P3b of the group with NLFC on as compared to the group with NLFC off. These findings suggest higher processing effort, as

Waardenburg syndrome

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Tagra Sunita

2006-01-01

Full Text Available Waardenburg syndrome is a rare inherited and genetically heterogenous disorder of neural crest cell development. Four distinct subtypes showing marked interfamilial and intrafamilial variability have been described. We report a girl showing constellation of congenital hearing impairment with 110 dB and 105 dB loss in right and left ear respectively, hypoplastic blue iridis, white forelock, dystopia canthorum and broad nasal root. Other affected relatives of the family, with variable features of the syndrome, have been depicted in the pedigree.

A novel gene for Usher syndrome type 2: mutations in the long isoform of whirlin are associated with retinitis pigmentosa and sensorineural hearing loss.

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Ebermann, Inga; Scholl, Hendrik P N; Charbel Issa, Peter; Becirovic, Elvir; Lamprecht, Jürgen; Jurklies, Bernhard; Millán, José M; Aller, Elena; Mitter, Diana; Bolz, Hanno

2007-04-01

Usher syndrome is an autosomal recessive condition characterized by sensorineural hearing loss, variable vestibular dysfunction, and visual impairment due to retinitis pigmentosa (RP). The seven proteins that have been identified for Usher syndrome type 1 (USH1) and type 2 (USH2) may interact in a large protein complex. In order to identify novel USH genes, we followed a candidate strategy, assuming that mutations in proteins interacting with this "USH network" may cause Usher syndrome as well. The DFNB31 gene encodes whirlin, a PDZ scaffold protein with expression in both hair cell stereocilia and retinal photoreceptor cells. Whirlin represents an excellent candidate for USH2 because it binds to Usherin (USH2A) and VLGR1b (USH2C). Genotyping of microsatellite markers specific for the DFNB31 gene locus on chromosome 9q32 was performed in a German USH2 family that had been excluded for all known USH loci. Patients showed common haplotypes. Sequence analysis of DFNB31 revealed compound heterozygosity for a nonsense mutation, p.Q103X, in exon 1, and a mutation in the splice donor site of exon 2, c.837+1G>A. DFNB31 mutations appear to be a rare cause of Usher syndrome, since no mutations were identified in an additional 96 USH2 patients. While mutations in the C-terminal half of whirlin have previously been reported in non-syndromic deafness (DFNB31), both alterations identified in our USH2 family affect the long protein isoform. We propose that mutations causing Usher syndrome are probably restricted to exons 1-6 that are specific for the long isoform and probably crucial for retinal function. We describe a novel genetic subtype for Usher syndrome, which we named USH2D and which is caused by mutations in whirlin. Moreover, this is the first case of USH2 that is allelic to non-syndromic deafness.

[Cognitive impairments accompanying the burnout syndrome - a review].

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Riedrich, Karin; Weiss, Elisabeth M; Dalkner, Nina; Reininghaus, Eva; Papousek, Ilona; Schwerdtfeger, Andreas; Lackner, Helmut K; Reininghaus, Bernd

2017-03-01

The rising prevalence of the burnout syndrome has increasingly moved it into the focus of scientific interest. In addition to emotional exhaustion and depersonalization, particularly reduced personal accomplishment has strong societal and economic effects. In recent years reduced personal accomplishment has increasingly been linked to cognitive impairment. However, up to now only a few studies have objectively assessed cognitive deficits in burnout patients. This article gives an overview of 16 studies which examined cognitive abilities in burnout patients. The findings are partly contradictory, probably due to methodical differences. Consensus has emerged concerning impairments of executive functions, i.a. vigilance, and memory updating and monitoring. Multifactorial causation may underlie the cognitive impairments. Targeted longitudinal studies are necessary in order to identify the affected cognitive functions and be able to make causal inferences on links between the burnout syndrome and specific cognitive impairments.

Comparison of visual working memory in deaf and hearing-impaired students with normal counterparts: A research in people without sign language

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Farideh Tangestani Zadeh

2015-02-01

Full Text Available Background and Aim: The hearing defects in deaf and hearing-impaired students also affect their cognitive skills such as memory in addition to communication skills. Hence, the aim of this study was to compare visual working memory in deaf and hearing-impaired students with that in normal counterparts.Method: In the present study, which was a causal-comparative study using the André Rey test, 30 deaf and 30 hearing-impaired students were compared with 30 students in a normal group, and they were matched based on gender, intelligence, educational grade, and socioeconomic status.Findings: Findings show that there is significant difference between the three groups’ subjects (p0.05.Conclusion: Function of deaf or hard-of-hearing students in the visual working memory task was weaker in comparison with the normal counterparts, while the two deaf and hard-of-hearing groups have similar functions. With a better identification and understanding of the factors that affect the development of this cognitive ability, we can offer new methods of teaching and reduce many of the disadvantages of this group of people in the different fields of cognitive science.

2q24 deletion in a 9-month old girl with anal atresia, hearing impairment, and hypotonia.

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Zhao, Peiwei; Mao, Bing; Cai, Xiaonan; Jiang, Jun; Liu, Zhisheng; Lin, Jun; He, Xuelian

2018-06-01

Deletion of 2q24.2 is a rare cytogenetic aberration in patients, exhibiting heterogeneous clinical features, and common phenotypes included developmental delay, intellectual disability, hypotonia, and mild dysmorphic features. Hearing impairment and anal atresia are rarely described. Here we described a 9-month-old female patient with hypotonia in all four limbs, developmental delay, and intellectual disability. In addition, congenital anal atresia was diagnosed and treated after birth, and hearing impairment was found in right ear. Single nucleotide polymorphisms (SNP) array detected a 5.2 Mb deletion on 2q24.2q24.3, including 19 genes (ITGB6; TBR1; SLC4A10; KCNH7 SCN3A; SCN2A et al.). Among these genes, it is affirmative that TBR1 is a causative gene for intellectual disability; however, the pathogenic genes of other phenotypes remain unclear. We briefly review the knowledge of genes likely involved in these clinical features, including hearing impairment, anal atresia, and developmental delay. Copyright © 2018 Elsevier B.V. All rights reserved.

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

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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…

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

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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…

Congenital non-syndromal autosomal recessive deafness in Bengkala, an isolated Balinese village.

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Winata, S; Arhya, I N; Moeljopawiro, S; Hinnant, J T; Liang, Y; Friedman, T B; Asher, J H

1995-01-01

Bengkala is an Indonesian village located on the north shore of Bali that has existed for over 700 years. Currently, 2.2% of the 2185 people in this village have profound congenital deafness. In response to the high incidence of deafness, the people of Bengkala have developed a village specific sign language which is used by many of the hearing and deaf people. Deafness in Bengkala is congenital, sensorineural, non-syndromal, and caused by a fully penetrant autosomal recessive mutation at the DFNB3 locus. The frequency of the DFNB3 mutation is estimated to be 9.4% among hearing people who have a 17.2% chance of being heterozygous for DFNB3. PMID:7616538

Auditory and Non-Auditory Contributions for Unaided Speech Recognition in Noise as a Function of Hearing Aid Use

OpenAIRE

Gieseler, Anja; Tahden, Maike A. S.; Thiel, Christiane M.; Wagener, Kirsten C.; Meis, Markus; Colonius, Hans

2017-01-01

Differences in understanding speech in noise among hearing-impaired individuals cannot be explained entirely by hearing thresholds alone, suggesting the contribution of other factors beyond standard auditory ones as derived from the audiogram. This paper reports two analyses addressing individual differences in the explanation of unaided speech-in-noise performance among n = 438 elderly hearing-impaired listeners (mean = 71.1 ± 5.8 years). The main analysis was designed to identify clinically...

A novel mutation of the EYA4 gene associated with post-lingual hearing loss in a proband is co-segregating with a novel PAX3 mutation in two congenitally deaf family members.

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Cesca, Federica; Bettella, Elisa; Polli, Roberta; Cama, Elona; Scimemi, Pietro; Santarelli, Rosamaria; Murgia, Alessandra

2018-01-01

This work was aimed at establishing the molecular etiology of hearing loss in a 9-year old girl with post-lingual non-syndromic mild sensorineural hearing loss with a complex family history of clinically heterogeneous deafness. The proband's DNA was subjected to NGS analysis of a 59-targeted gene panel, with the use of the Ion Torrent PGM platform. Conventional Sanger sequencing was used for segregation analysis in all the affected relatives. The proband and all the other hearing impaired members of the family underwent a thorough clinical and audiological evaluation. A new likely pathogenic mutation in the EYA4 gene (c.1154C > T; p.Ser385Leu) was identified in the proband and in her 42-year-old father with post-lingual non-syndromic profound sensorineural hearing loss. The EYA4 mutation was also found in the proband's grandfather and uncle, both showing clinical features of Waardenburg syndrome type 1. A novel pathogenic splice-site mutation (c.321+1G > A) of the PAX3 gene was found to co-segregate with the EYA4 mutation in these two subjects. The identified novel EYA4 mutation can be considered responsible of the hearing loss observed in the proband and her father, while a dual molecular diagnosis was reached in the relatives co-segregating the EYA4 and the PAX3 mutations. In these two subjects the DFNA10 phenotype was masked by Waardenburg syndrome. The use of NGS targeted gene-panel, in combination with an extensive clinical and audiological examination led us to identify the genetic cause of the hearing loss in members of a family in which different forms of autosomal dominant deafness segregate. These results provide precise and especially important prognostic and follow-up information for the future audiologic management in the youngest affected member. Copyright © 2017 Elsevier B.V. All rights reserved.

Centre-level variation in outcomes and treatment for otitis media with effusion and hearing loss and the association of hearing loss with developmental outcomes at ages 5 and 7 years in children with non-syndromic unilateral cleft lip and palate: The Cleft Care UK study. Part 2.

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Hall, A; Wills, A K; Mahmoud, O; Sell, D; Waylen, A; Grewal, S; Sandy, J R; Ness, A R

2017-06-01

To explore centre-level variation in otitis media with effusion (OME), hearing loss and treatments in children in Cleft Care UK (CCUK) and to examine the association between OME, hearing loss and developmental outcomes at 5 and 7 years. Two hundred and sixty-eight 5-year-old British children with non-syndromic unilateral cleft lip and palate (UCLP) recruited to CCUK. Children had air and bone conduction audiometry at age 5. Information on grommet and hearing aid treatment was obtained from parental questionnaire and medical notes. Hearing loss at age 5 was defined as >20 dB in the better ear and history of OME and hearing loss was determined from past treatment. Children with sensorineural hearing loss were excluded. Associations were examined with speech, behaviour and self-confidence at age 5 and educational attainment at age 7. Centre variation was examined using hierarchical models and associations between hearing variables and developmental outcomes were examined using logistic regression. There was centre-level variation in early grommet placement (variance partition coefficient (VPC) 18%, P=.001) and fitting of hearing aids (VPC 8%, P=.03). A history of OME and hearing loss was associated with poor intelligibility of speech (adjusted odds ratio=2.87, 95% CI 1.42-5.77) and aspects of educational attainment. Hearing loss is an important determinant of poor speech and treatment variation across centres suggest management of OME and hearing loss could be improved. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Understanding of the Alphabetic Principle through Invented Spelling among Hearing-Impaired Children Learning to Read and Write: Experimentation with a Pedagogical Approach

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Sirois, Pauline; Boisclair, Andree; Giasson, Jocelyne

2008-01-01

Given the problems experienced by hearing-impaired individuals in learning the written language, a pedagogical approach was tested. The study examined the links between the development of representations of alphabetic system and the results in reading and writing of first graders. In the study, there were 31 hearing-impaired children and 25…

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