Association of GJB2 gene mutation with cochlear implant performance in genetic non-syndromic hearing loss.

Objective: To analyze the association of GJB2 gene mutations with cochlear implant performance in children.

Methods: Sixty-five consecutive children who underwent cochlear implantation due to congenital profound senseurineural hearing between 2006 and 2008 were included in the study. In children, GJB2 gene mutation analysis was performed. Their auditory performance was assessed using MAIS, MUSS and LittlEARS tests.

Results: Twenty-two of sixty-five patients GJB2 mutations, and 35delG was the most frequent mutation. No significant difference was found between the auditory performance of mutation positive and negative children after one year follow up (p>0.05).

Conclusion: GJB2 gene mutations do not impact on the outcome of cochlear implantation.