Cochlear Implantation in Cogan's Syndrome

Cogan's syndrome is a rare multi-systemic disease which is thought to be autoimmune in origin. It targets various organs, but all patients will suffer some degree of audiovestibular involvement at some point through the disease process, with many sustaining a profound sensorineural hearing loss. This article describes three cases of bilateral profound sensorineural hearing loss due to Cogan's syndrome treated at the Manchester Cochlear Implantation Centre with a focus on surgical and audiological outcome from cochlear implantation. The symptomatology, aetiology, diagnosis, treatment and prognosis of Cogan's syndrome are also discussed with evidence from the world literature to highlight the key points of this uncommon syndrome.