Irish Child Health Database - Peer Reviewed Papers
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Connexin 26 (GJB2) gene-related deafness and speech intelligibility after cochlear implantation
Published in:
Otol Neurotol, Vol: 25, Page: 935-42
Publication Date:
November 2004
Aims & Objectives:
The aim of this study was to examine the hypothesis that speech intelligibility in children after cochlear implantation may depend on their deafness cause, including connexin 26 (GJB2) gene-related deafness
Abstract:
HYPOTHESIS: Speech intelligibility in children after cochlear implantation may depend on their deafness cause, including connexin 26 (GJB2) gene-related deafness. BACKGROUND: There is significant variability in the degree of intelligibility, or clarity, of children's speech after cochlear implantation. GJB2 gene-related deafness may be a factor, as preliminary data suggest that pathologic changes do not affect the spiral ganglion cells, which are the neural elements stimulated by the implant, thus favoring better results. METHODS: In an observational retrospective cohort study of pediatric cochlear implantees, 38 patients with nonsyndromic deafness of unknown cause and 1 with keratitisichthyosis-deafness syndrome underwent GJB2 mutation analysis using polymerase chain reaction amplification and direct sequencing. The primary outcome measure assessed was Speech Intelligibility Rating score from postoperative Year 1 (n = 39) to Year 5 (n = 17). Educational setting was considered as a secondary outcome measure. Statistical analysis was double-blinded, with patients and assessors of outcome unaware of GJB2 status. RESULTS: Fourteen patients had GJB2-related deafness and 25 had GJB2-unrelated deafness. Comparisons at Year 3 (n = 31) revealed intelligible speech achieved by 9 of 11 with GJB2-related deafness, compared with only 6 of 20 with GJB2-unrelated deafness (p = 0.017). Ordinal logistic regression analysis on Speech Intelligibility Rating scores found statistically significantly better scores in children with GJB2-related deafness (p < 0.05) both before and after adjustment for confounding variables. A larger proportion with GJB2-related deafness also attended mainstream school (p = 0.01). CONCLUSION: In pediatric cochlear implantees, GJB2-related deafness is a predictor of good speech intelligibility.
Authors:
Study Type:
Study Papers » Case Study » Descriptive Studies - Without a comparision
Categories:
deafness; cochlear implant
International Classification:
Diseases of the ear and mastoid process - Other disorders of ear - deafness
Keywords:
- Child
- Child, Preschool
- Cochlear Implantation
- Cohort Studies
- Connexins/ genetics
- Deafness/ genetics/physiopathology/therapy
- Female
- Hearing Impaired Persons/education
- Humans
- Logistic Models
- Male
- Mutation/genetics
- Observer Variation
- Retrospective Studies
- Speech Intelligibility
- Speech Perception
- Treatment Outcome
- Videotape Recording
Geography:
Northern Ireland (Northern Ireland)