Cogan's Syndrome

Cogan's syndrome (CS) is characterized by interstitial keratitis, which rarely produces blindness, and recurrent Meniere's-like vestibulo-auditory events with frequent progression to hearing loss and deafness. It is a rare disease with a mean age of onset at 29 years. The mortality rate is unknown. Approximately half of the patients report an antecedent upper respiratory illness. Inflammatory cardiovascular disease develops in 15% of patients. The diagnosis of CS requires the presence of ocular inflammation and inner ear dysfunction not explained by another illness. Because the differential diagnostic considerations in CS cross a broad spectrum of illnesses and toxicities, the proper assessment of CS and its management often requires the collaboration of numerous specialties. The exact etiology of CS remains a mystery, although immune mechanisms likely have a pivotal role in its pathogenesis. Treatment consists typically of corticosteroids and the judicious use of other immunosuppressive therapies.