Late failure of cochlear implantation resulting from advanced cochlear otosclerosis: surgical and programming challenges.

Objective: The objective of this study was to discuss cochlear implantation in the setting of severe cochlear otosclerosis and review programming challenges in a patient whose performance is deteriorating as a result of advancing disease.

Study Design: We conducted a case report and literature review.

Setting: Tertiary care medical center. PATIENTS, INTERVENTION, AND RESULTS: A case is presented of a 66-year-old man with otosclerosis who initially had good benefit from a cochlear implant but gradually lost benefit even with reimplantation. Imaging studies demonstrated severely distorted otic capsule anatomy from cochlear otosclerosis.

Conclusions: Advancing cochlear otosclerosis can result in a severely thinned and distorted otic capsule. Although cochlear implantation is generally beneficial in cochlear otosclerosis, implantation in certain severe cases may be complicated as a result of difficulties with cerebrospinal fluid leak, programming challenges, and other potential hazards that can occur with a distorted anatomy.