Cochlear implantation in children with large vestibular aqueduct syndrome

Objective: This study describes the effectiveness of a multielectrode cochl ear implant prosthesis (Cochlear; Cochlear Pty., Lane Cove, Australia) for providing hearing to children with deafness caused by large vestibular aque duct syndrome (LVAS). Study Design: The study design was a retrospective study. Setting: All the children attended The Children's Cochlear Implant Center ( NSW), which is a specialist center that provides audiologic testing, speech therapy, habilitation, and medical assistance for children with cochlear i mplants. Patients: Ten children were studied who had profound hearing loss and radio logic evidence of a vestibular aqueduct larger than 2 mm in width in its in traosseous portion. Intervention: The children received a multielectrode (Cochlear) cochlear im plant prosthesis, and the associated programming of the device and habitati on were performed postoperatively. No significant problems were encountered at any of the surgeries, although there was an initial gush of perilymph w hen the otic capsule was opened in 7 ears. Main Outcome Measures: Postoperative audiologic performance at six monthly intervals and school performance were assessed. Results: The postoperative auditory performance was improved in all childre n. At 6 months, their average BKB score had increased from 31% to 79%; aver age word score, from 8% to 43%; and average phoneme score, from 38% to 70%. The older children were able to continue their education in their usual se tting with less reliance on hearing support staff. Conclusion: Children with a deteriorating hearing loss caused by LVAS can d erive considerable benefit from a cochlear implant.