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2 edition of Auditory responses in paediatric cochlear implant users with and without GJB2 deafness. found in the catalog.

Auditory responses in paediatric cochlear implant users with and without GJB2 deafness.

Evan Jon Propst

Auditory responses in paediatric cochlear implant users with and without GJB2 deafness.

by Evan Jon Propst

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  • 5 Currently reading

Published .
Written in English


About the Edition

In GJB2-related deafness, we hypothesize that because gap junction deficits are uniformly distributed along the cochlea, there will be a uniform pattern of cochlear damage as compared with subjects with non-GJB2 etiologies. Blood from 301 paediatric cochlear implant users was analyzed for mutations in GJB2. Thirty-nine children with biallelic GJB2 mutations and 58 without GJB2 mutations were evaluated. After cochlear implantation, neural activity at apical and basal ends of the implanted array was measured using electrically evoked compound action potentials of the auditory nerve and evoked stapedius reflexes. Children with GJB2 deafness had greater similarities between low and high frequency residual hearing and between neural activity evoked at apical and basal regions of the cochlea as compared with children with non-GJB2 deafness. The latter group demonstrated larger deficits in basal regions. Results suggest more uniform spiral ganglion survival along the cochlea in GJB2 deafness as compared with non-GJB2 deafness.

The Physical Object
Pagination50 leaves.
Number of Pages50
ID Numbers
Open LibraryOL19215200M
ISBN 109780494161784

Benefits and detriments of unilateral cochlear implant use on bilateral auditory development in children who are deaf. Front Psychol. 4: pp Abstract. Gordon KA, Papsin BC. From nucleus 24 to changing cochlear implant design affects auditory response thresholds. Otol Neurotol 34(3): pp Abstract. Auditory and speech performance after cochlear implantation. Categories of auditory performance (CAP) and speech intelligibility rating (SIR) scores were assessed at 3 months, 6 months, 1 year, 3 years and 5 years post‐implant, whereas speech perception tests were performed at 3 years post‐implant. 7 The duration of CI use in the

Implantable auditory technology has become the standard of care for children born with severe to profound sensorineural hearing loss. Today, multichannel cochlear implants (CI) provide access to speech, enabling many recipients to become proficient in spoken communication (Geers, Mitchell, Warner-Czyz, Wang, & Eisenberg, ; Niparko et al., ).   The results show that cochlear implantation is effective in the development of speech perception after cochlear implantation in GJB2-related deafness to a similar extent as in deafness due to unknown etiology. These findings are in agreement .

Search term. Advanced Search Citation Search. Search term. Clinical application of mutation screening and its effect on the outcome of cochlear implantation is widely debated. We investigated the effect of mutations in GJB2 gene on the outcome of cochlear implantation in a population with a high rate of consanguineous marriage and autosomal recessive nonsyndromic hearing loss. Two hundred and one children with profound prelingual sensorineural.


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Auditory responses in paediatric cochlear implant users with and without GJB2 deafness by Evan Jon Propst Download PDF EPUB FB2

Methods: Blood from pediatric cochlear implant users was analyzed for mutations in GJB2 by direct sequencing. After exclusion of patients with monoallelic GJB2 mutations, associated syndromes, or risk factors for HL that were not congenital, 39 children with biallelic GJB2 mutations and 58 without GJB2 mutations were by: Auditory responses in cochlear implant users with and without GJB2 deafness.

Propst EJ(1), Papsin BC, Stockley TL, Harrison RV, Gordon KA. Author information: (1)Department of Otolaryngology-Head and Neck Surgery and the Molecular Genetics Laboratory, The Hospital for Sick Children, Toronto, Ontario, Canada.

@d by: Request PDF | Auditory Responses in Cochlear Implant Users With and Without GJB2 Deafness | It is reasonable to suppose that the pattern of sensorineural damage along the length of the cochlea. BibTeX @ARTICLE{Propst06auditoryresponses, author = {Evan Jon Propst and Md Blake and C.

Papsin and Frcsc Tracy and L. Stockley and Robert V. Harrison and Dsc Karen and A. Gordon}, title = {Auditory responses in cochlear implant users with and without GJB2 deafness}, journal = {Laryngoscope}, year = {}, pages = {}}. Methods. Patients who received CIs before 18 years of age and had used CIs for more than 3 years were enrolled in this study.

All patients underwent mutation screening of three common deafness genes: GJB2, SLC26A4 and the mitochondrial 12S rRNA gene.

The outcomes with CIs were assessed at post-implant years 3 and 5 using the Categories of Auditory Performance (CAP) scale, Speech Cited by:   Among children who received CIs before years of age, patients with GJB2 or SLC26A4 mutations showed significantly higher CAP/SIR scores than those without mutations at post-implant year 3 (p.

In a study of 4 Japanese children with homozygous delC of GJB2, a mutation associated with uniform profound bilateral non-progressive deafness at birth, post implant speech perception scores of all patients were found to be better after cochlear implantation than those with no.

ain method for treating severe and extremely severe bilateral sensorineural deafness and it is widely used in clinical treatment. As clinical cases of cochlea implantation accumulate, differences in the efficacy of implantation in individuals are emerging and attracting attention.

In addition to residual hearing level, implantation age, and other factors, gene mutation is an important factor. INTRODUCTION. Cochlear implantation has revolutionized the care for children and adults with deafness. The first surgery for implantation of a single channel cochlear implant (CI) occurred inby House and Doyle, and was followed by the first multichannel CI in 1 In the intervening six decades, the goals for CI recipients' sound perception have advanced and continue to.

Objective: To investigate the etiology of deafness in cochlear implanted children and to address the question whether there is a need for more thorough diagnostics, especially concerning : Systematic databases were searched for studies (year –) on cochlear implanted children (n > ).Studies were excluded if etiology had influenced their inclusion.

Propst EJ, Papsin BC, Stockley TL, Harrison RV, Gordon KA. Auditory responses in cochlear implant users with and without GJB2 deafness. Laryngoscope. ;(2)– PubMed CrossRef Google Scholar.

Some cochlear implant users have little or no benefit from using them, and as many as 7% of children stop using cochlear implants [6,7]. The development of auditory competence of cochlear implant.

Abstract. To analyze the treatment outcomes in pediatric cochlear implant patients with mutations in GJB2 or SLC26A4 and to determine these mutations’ impact on rehabilitative outcomes.

The study included 41 children who received unilateral cochlear implantations. 32 Daneshi et al. found GJB2 mutations in per cent of Iranian children with cochlear implants. 33 • The GJBdelG mutation is a major cause of congenital, non-syndromic hearing loss in.

The purpose of the study was to compare the speech perception performance of cochlear implant patients with GJB2-related deafness to patients without GJB2-related deafness. Rather, accompanying central nervous system problem which may coexist with GJB2 unrelated deafness may be the cause of controversial cochlear implant performance results in the literature.

Auditory experience before implantation is known as another factor that affects the performance of the cochlear implant [22], [23]. An evoked potential study of the developmental time course of the auditory nerve and brainstem in children using cochlear implants.

Audiology and Neurotology. () Propst E.J., Papsin B.C., Stockley T.L., Harrison R.V., Gordon K.A. Auditory responses in cochlear implant users with and without GJB2 deafness.

The laryngoscope. ( ods: Blood from pediatric cochlear implant users was analyzed for mutations in GJB2 by direct se-quencing. After exclusion of patients with monoal-lelic GJB2 mutations, associated syndromes, or risk factors for HL that were not congenital, 39 children with biallelic GJB2 mutations and 58 without GJB2 mutations were evaluated.

Hearing was. Propst EJ, Papsin BC, Stockley TL, Harrison RV, Gordon KA (a) Auditory responses in cochlear implant users with and without GJB2 deafness. Laryngoscope (2)– PubMed; Article; Google Scholar. Ethics of cochlear implantation in young children. Otolaryngology: Head and Neck Surgery,– 8.

Tucker, B. Deaf culture, cochlear implants and elective disability. Hastings Center Report. 28(4), 6– 9. Francis HW, Yeagle JD, Bowditch S, et al.

Cochlear implant outcome is not influenced by the choice of ear. Methods. Genetic testing was performed on 29 children with CI. The speech perception in 9 children with GJB2 gene-related deafness fitted with CI was compared with those in matched 10 children who were diagnosed as having no genetic loci.

The average follow-up period after CI was months and months, respectively.Behaviourally determined auditory response in pediatric cochlear implant users with and without GJB2 deafness.

60th Annual Meeting of the Canadian Society of Otolaryngology, Kelowna, BC, May Propst EJ, Gordon KA, Stockley TL, Harrison RV, Papsin BC.Its use in improving speech understanding in cochlear implant users is described, together with the specific problems of auditory training in children.

Music training and video gaming could be new tools that might be used in training certain aspects of hearing in noisy environments, enhanced use of hearing aids and cochlear implants.