A Case of Atypical Cogan Syndrome with Late-Onset Corneal Involvement

ABSTRACT

PURPOSE: To report a case of atypical Cogan syndrome with late-onset corneal involvement. METHODS: Bilateral conjunctival injection with severe headache developed in a 38-years old woman who had suffered from tinnitus, vertigo and hearing difficulty. Inflammation in anterior chamber and vitreous cavity, macular edema, disc edema and retinal hemorrhages were found on ocular examination. RESULTS: After initiation of systemic steroid therapy, ocular inflammatory reaction and visual acuity improved, but hearing difficulty did not respond. After 7 years of lost follow-up, corneal stromal opacity occurred and she was diagnosed as atypical Cogan syndrome. Elevated ESR, multiple stenosis in renal and lower extremity arteries in angiography were found. CONCLUSIONS: Atypical Cogan syndrome with late-onset corneal involvement is rare and difficult to diagnose, which may delay in initiation of adequate treatment.