Long-Term Hearing, Language, and Educational Outcomes After Cochlear Implantation in Children With Waardenburg Syndrome

Objective The objective of this study was to evaluate hearing and language outcomes in children with Waardenburg syndrome (WS) who underwent cochlear implantation (CI) at a single center in Japan. Methods A retrospective review was performed on 12 children with WS who received CI between 1995 and 2020. Preoperative and postoperative auditory thresholds, speech recognition and language comprehension scores, and school placement were analyzed. Outcomes were compared according to the timing of intervention and the presence of cochlear malformations. Results The mean age at CI was 2.5 years. Early intervention was correlated with higher 67S and CI-2004 word/sentence scores. Children without cochlear malformations or delayed treatment achieved mean speech recognition scores exceeding 80%. All participants advanced to regular junior high school classes. Conclusion Early CI in children with WS contributes to substantial improvements in hearing, language comprehension, and educational integration.