Cochlear Implant in Hypoacusis With Alteration of Connexin 26

Objective The objective of this paper is to assess the benefits of cochlear implantation in a population of profound prelingual congenital deaf children with mutation of Connexin 26 (DFNB1 phenotype), compared with a population of profound congenital deaf children without mutation of this gene. Patients and method This retrospective study was carried out in 36 children with cochlear implants under the age of 6. All had profound congenital bilateral sensorineural hearing impairment, without cochlear malformation. Fifteen children were diagnosed as having DFNB1 and homozygous 30–35delG mutation, and 21 had no mutation of Connexin 26 (C × 26). All of them used Nucleus 24 K or ST cochlear implants, with complete non-traumatic insertion of the electrodes, and follow-up was 12 months. Results There is no significant difference in pure tone audiometry and logoaudiometric tests between the 2 groups, children diagnosed as having DFNB1 (homozygous 30–35delG mutation) and children without mutation of C × 26. However, the population with DFNB1 shows a tendency to achieve better results more quickly in vowels and bisyllabic word tests 12 months after implantation. Conclusions The cochlear implant is an effective therapy for children with profound prelingual congenital hearing loss with mutation of C × 26.