Cochlear implantation in patients with Cogan's syndrome: a review of four cases.

Cogan's syndrome is a rare disorder that is characterized by a rapid onset of bilateral severe sensorineural hearing loss associated with vertigo and tinnitus, ocular involvement (most commonly an interstitial keratitis) and variable systemic symptoms. We review our experience with four cases treated in our department. We describe their symptoms, the evolution of their disease, the diagnosis, and the radiological investigations with computed tomography and/or magnetic resonance imaging. When medical treatment with corticosteroids failed and no benefit could be obtained with hearing aids at each patient's end stage, all patients received a cochlear implant with excellent results. The considerations for implantation in these patients are discussed.