Effects of GJB2 (Connexin 26) gene mutations on cochlear implant performance in genetic non-syndromic hearing loss

The aim of this study was to analyse the effects of GJB2 (Connexin 26) gene mutations on the cochlear implant performance genetic non-syndromic hearing loss.Sixty-five children who underwent cochlear implantation due to genetic non-syndromic bilateral senseurineural hearing loss in Gazi University Faculty of Medicine ORL & HNS Department between 2006 and 2008 were screened for Cx-26 gene mutations. MAIS, MUSS and LittlEARS tests were performed for the analysis of the auditory performance after cochlear implantation.Twenty-two of 65 patients had Cx-26 mutations. All the patients have had a comprehensive evaluation of development in auditory performance (p<0.01). However, no significant differences were found between the mutation positive and negative groups in the term of auditory performance.The patients with associated disabilities that can effect educational and developmental performance had poor outcomes of auditory performance after cochlear implantation. Patients who used hearing aids at least 3 months preoperatively had better auditory outcomes. This study also showed that higher family enthusiasm resulted in better outcomes.