COGANS-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. Experie nce 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 preesentation at age 10. The respo nse to treatment, as revealed by audiograms, is traced. The literature is reviewed with regard to treatments that have been proposed. In tho se cases where a sustained follow-up is available, the success of the therapy on the vestibuloauditory system is evaluated. Although some pa tients have a positive response to steroids, the prognosis for hearing remains poor. The ability of steroids to change the prognosis for hea ring loss is not yet established, but warrants further clinical trial.