Cochlear implantation in a patient with atypical Cogan's syndrome complicated with hypertrophic cranial pachymeningitis

A 55-year-old woman had bilateral sensorineural hearing loss (SNHL), vertigo, uveitis, and aortitis associated with Cogan's syndrome (CS). She had a history of listeria meningitis and hypertrophic cranial pachymeningitis (HCP), both of which were considered to be related to SNHL progression. She developed bilateral profound deafness within 1 year, despite medical treatment with corticosteroids and methotrexate (MTX). She underwent cochlear implantation (CI) of the left ear. Although the left and right basal turns of the cochleae were ossified, all electrodes were successfully inserted, and subsequently the inferior segment of the basal turn was drilled out. The patient did not have any postoperative complications and showed good speech perception.