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Abstract

Objectives: Waardenburg syndrome is known as a collagen and pigment disease which causes different phenotypes. One of the most important phenotypes is severe bilateral congenital hearing loss. It may be possible to improve their hearing and speech abilities with cochlear implantation and rehabilitation. Nevertheless, long-term outcome after surgery in these patients is obscured. In this study, we evaluated both auditory and speech abilities in order to examine to what extent they had improved three years after surgery.

Methods: We retrospectively gathered 51 deaf Waardenburg children without any other ear anomalies along with 210 congenitally deaf and age matched children without any other anomalies as control group. All children were at least 3 years post-operation. All of them underwent speech and auditory examinations. “Categories of Auditory Performance” for was used auditory and “Speech Intelligibility Rating” was employed for speech evaluations.

Results: Male-to-female ratio was 50% in the control group and 47% in Waardenburg. The difference between hearing and speech outcomes in both genders was insignificant. Speech Intelligibility Rating in the control group was 3.49 (S.D. ± 0.07) and in Waardenburg group was 3.09 (S.D. ± 0.09), which was insignificant. Similarly, Categories of Auditory Performance in the control and Waardenburg groups were 4.4 (S.D. ± 0.69) and 4.1 (S.D. ± 2.10) respectively, which is insignificant either. There was no significant correlation between age at operation and hearing and speech results. Finally, the correlation between hearing and speech was not significant either (p ≤ 0.81).

Conclusion: This study confirmed that there is not correlation between age at operation in cochlear implantation in Waardenburg patients and there would be no difference in hearing and speech results between them and other deaf implanted populations. The results were not sex dependent.

Keywords: Cochlear implantation; Waardenburg; Categories of auditory performance