Multichannel Cochlear Implantation in Children with Large Vestibular Aqueduct Syndrome

This study examined the postoperative performance of multichannel cochlear implantation in children with severe-to-profound sensorineural hearing loss secondary to large vestibular aqueduct syndrome. Five children between ages 4 and 13 years who had large vestibular aqueducts confirmed by computed tomography scans underwent implantation of a CLARION Multi-Strategy Cochlear Implant between January 1995 and June 1996 at Boys Town National Research Hospital. In addition to preoperative evaluations, they were examined postoperatively with their implants on a battery of open-set speech recognition tests at 3, 6, 12, and 18 months following initial stimulation. Results indicated a noticeable improvement in open-set speech recognition within the first 3 months of implant use compared to preimplant performance with hearing aids. All 5 patients demonstrated substantial benefit from their implants, but there was variation among the children in the rate and amount of improvement in speech recognition skills.