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.
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: Robert W Eppsteiner; A Eliot Shearer; Michael S Hildebrand; Adam P Deluca; Haihong Ji; Camille C Dunn; Elizabeth A Black-Ziegelbein; Thomas L Casavant; Terry A Braun; Todd E Scheetz; Steven E Scherer; Marlan R Hansen; Bruce J Gantz; Richard J H Smith Journal: Hear Res Date: 2012-08-28 Impact factor: 3.208
Authors: Richard J Vivero; Kenneth Fan; Simon Angeli; Thomas J Balkany; Xue Z Liu Journal: Int J Pediatr Otorhinolaryngol Date: 2010-07-22 Impact factor: 1.675
Authors: Lirong Tan; Scott K Holland; Aniruddha K Deshpande; Ye Chen; Daniel I Choo; Long J Lu Journal: Brain Behav Date: 2015-10-12 Impact factor: 2.708