Waardenburg Syndrome: An Unusual Indication of Cochlear Implantation, Experienced on 11 Patients
Katip Çelebi University, Atatürk Education and Research Hospital, Department of Otorhinolaryngology, İzmir, Turkey
SB University, Bozyaka Education and Research Hospital, , Department of Otorhinolaryngology, İzmir, Turkey
J Int Adv Otol ; : -
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Key Words: Waardenburg syndrome, cochlear, implantation
Objective: The aim of this study is to present the surgical findings in children with Waardenburg syndrome (WS) and to investigate the speech development after cochlear implantation in this unique group of patients.
Material and Methods: A retrospective chart review of the patients diagnosed with WS and implanted between 1998 and 2015 has been analyzed Categories of auditory performance (CAP) test have been used to assess the auditory skills of these patients. The CAP is a nonlinear hierarchical scale used to rate a child’s developing auditory abilities. Preoperative test results and intra-operative surgical findings of these patients have been also presented.
Results: 1835 cases have been implanted in our institution and 1210 of them have been children. Among these implantees, 11 have been diagnosed as having WS (0,59). 4 of the 11 patients have had bony labyrinth abnormality (BLA) as incomplete partition type 2 (Mondini deformity) and all patients have had intraoperative gusher during cochleostomy, which had been subsided with routine interventions. No other complications have occurred during surgery and all Waardenburg patients have had the satisfactory CAP results in the late postoperative period.
Conclusion: Our experiences with CI on WS have shown that the procedure is safe and effective in this group of patients. As other syndromes, surgeons should be aware of possible labyrinth malformations and intraoperative problems such as gusher in WS patients. In long term, auditory performances may exhibit satisfactory results with optimal postoperative educational and supportive measures.