Cochlear Implantation in a Patient With Noonan Syndrome and Enlarged Cochlear Apertures.

Yang, Kai-Shan; Tsai, Meng-Han; Hwang, Chung-Feng; Lin, Li-Han; Yang, Chao-Hui · Laryngoscope · 2026

case_report · Level V

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Abstract

We report the first documented case of bilateral cochlear implantation (CI) in a patient with Noonan Syndrome (NS) and enlarged cochlear apertures (ECA). Despite the potential risk of cerebrospinal fluid (CSF) gusher, both surgeries were uneventful, and the patient demonstrated significant improvement in auditory thresholds and speech development. This case highlights that ECA should not be considered a contraindication for CI in NS patients.

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