Cochlear Implantation in a Patient with Superficial Siderosis of the Central Nervous System

Objective To perform an assessment of the viability of cochlear implantation in a patient with superficial siderosis of the brain. Study Design Case review. Setting Tertiary referral center. Patients Patient with superficial siderosis of the brain. Intervention Cochlear implantation. Main Outcome Measures Electrically evoked auditory brainstem responses and fitting strategy for cochlear implant based on patient's subjective responses. Results After some time, C levels and T levels showed a pattern comparable with the evoked auditory brainstem responses. Conclusions Bilateral profound hearing loss after superficial siderosis of the brain is no absolute contraindication for implantation. Furthermore, evoked auditory brainstem measurements can potentially guide the fitting process of the implant in difficult cases.