Cochlear-Implantation in X-linked-Deafness – a case report

We report the case of a three-year old boy who was diagnosed with congenital sensorineural hearing loss. MRI and CT-scans showed an inner ear malformation with corkscrew-like cochlea and a missing modiolus as well as an incomplete separation between inner auditory canal and cochlea. These findings suggested the presence of a rare inner ear malformation, an incomplete partition type III (or x-linked deafness) after Sennaroğlu und Saatci. We implanted a CI622-slim-straight electrode from Cochlear​ unilaterally on the left side via an approach anterior to the round window membrane to make sure the electrode was inserted in the vestibule scala and not accidently misplaced into the inner auditory canal. Cerebral fluid leakage (Gusher) was observed during the surgery. A CT-scan on the following way verified the correct placement of the electrode. This case shows the importance of complete preoperative diagnostic with CT to plan the best surgical approach and to prevent peri- and intraoperative complications.

Publikationsverlauf

Artikel online veröffentlicht:
12. Mai 2023