Abstract
Objectives: Cochlear implants stimulate spiral ganglion cells survival which varies with deafness etiology. These cells appear unaffected in patients with Connexin 26 gene deafness, possibly reflecting better postimplantation outcomes. Methods: Thirty-nine children underwent cochlear implantation by a single surgeon at the same center. Year 3 outcomes assessed blind to Connexin 26 status were: speech discrimination via Glendonald Auditory Screening Procedure (GASP) and IOWA Matrix Sentences test, speech intelligibility via Speech Intelligibility Rating (SIR), and educational setting. Connexin 26 screening involved polymerase chain reaction amplification and direct sequencing of both exons. Twelve patients had Connexin 26 deafness, 20 had Connexin 26-unrelated unknown causes of deafness, 7 had other known causes of deafness. Results: GASP scores were not significantly different, but median IOWA Matrix scores were 88.8% in patients with Connexin 26 deafness, 80.4% in Connexin 26-unrelated unknown causes of deafness, and 0% in other known causes of deafness (N = 36, P = 0.046). Intelligible speech was achieved by 9/11 with Connexin 26 deafness, 6/20 with Connexin 26-unrelated unknown causes of deafness, and 1/7 with other known causes of deafness (N = 38, P = 0.005). Ordinal logistic regression analysis on IOWA Matrix and SIR scores found best results in Connexin 26 deafness ( P < 0.05, P < 0.05). Most children attending mainstream school had Connexin 26 deafness (N = 39, P = 0.02). Conclusions: Implanted children with Connexin 26 deafness are more likely to achieve good speech discrimination, speech intelligibility, and mainstream schooling compared to other deafness etiologies.
Published Version
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