Cogan's syndrome: a review of otologic management and 10-year follow-up of a pediatric case.

Although first characterized over 45 years ago Cogan's syndrome still remains a rare disease, with a high incidence of poor outcome. Experience with a case of Cogan's syndrome over a 10-year period is presented. The fluctuating clinical evolution and laboratory findings over this period are depicted, starting from presentation at age 10. The response to treatment, as revealed by audiograms, is traced. The literature is reviewed with regard to treatments that have been proposed. In those cases where a sustained follow-up is available, the success of the therapy on the vestibuloauditory system is evaluated. Although some patients have a positive response to steroids, the prognosis for hearing remains poor. The ability of steroids to change the prognosis for hearing loss is not yet established, but warrants further clinical trial.