Abstract 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.