Bilateral Cochlear Implantation in Children With Anomalous Cochleovestibular Anatomy

Objective: To outline clinical experience with bilateral cochlear implantation in children with cochleovestibular anomalies. Design: A prospective cohort study with a mean follow-up of 12 months. Setting: An academic, pediatric, tertiary referral center. Patients: All eligible children were prospectively recruited from January 1, 2007, through October 31, 2008. Ten children aged 9 to 33 months who had congenital inner ear malformations, including common cavity, incomplete partition, and cochleovestibular hypoplasia, participated. Interventions: Bilateral cochlear implantation was performed sequentially with an interimplantation delay greater than 2 years in 7 children and less than 1 year in 1 child. Bilateral simultaneous implantation was performed in 2 children. Main Outcome Measures: Complications, heating Outcomes, and balance outcomes. Results: All children underwent successful implantation. Five children had a perilymph "gusher" (on 1 side only), and there were no other complications. All children had 22 active electrodes bilaterally and achieved speech reception. All 8 children who underwent closed-set speech perception testing scored above 75%, and 5 of the 7 children who underwent open-set testing achieved scores above 75%. Despite variable vestibular function before bilateral implantation, no prolonged imbalance occurred, although 3 children (30%) had transient unsteadiness for up to 2 weeks after the second implantation. Conclusions: Bilateral cochlear implantation was performed safely and successfully in children with a spectrum of bilaterally anomalous cochleovestibular anatomy. Hearing Outcomes Suggest that these children should not be excluded from undergoing bilateral implantation. This study provides guidance on candidacy issues, surgical decision making, and surgical techniques in this group.