Cochlear implantation in patients with Waardenburg syndrome type II

Objective:To describe the multi-channel cochlear implantation in patients with Waardenburg syndrome including surgeries,pre and postoperative hearing assessments as well as outcomes of speech recognition.Method:Multi-channel cochlear implantation surgeries have been performed in 12 cases with Waardenburg syndrome typeⅡin our department from 2000 to 2008.All the patients received multi-channel cochlear implantation through transmastoid facial recess approach.The postoperative outcomes of 12 cases were compared with 12 cases with no inner ear malformation as a control group.Result:The electrodes were totally inserted into the cochlears successfully,there was no facial paralysis and cerebrospinal fluid leakage occurred after operation.The hearing threshold in this series were similar to that of the normal cochlear implantation.After more than half a year of speech rehabilitation,the abilities of speech discrimination and spoken language of all the patients were improved compared with that of preoperation.Conclusion:Multi-channel cochlear implantation could be performed in the cases with Waardenburg syndrome,preoperative hearing and images assessments should be done.