[A case of Cogan's syndrome associated with multiple cranial neuropathy]

A 52-year-old woman was admitted to our hospital because of disturbance of right visual acuity and double vision. At 38-year-old she became deaf bilaterally and experienced many vertigo attacks. She was diagnosed as Ménière disease. At 45-year-old vertigo attacks disappeared. At 47-year-old right peripheral facial nerve palsy developed transiently with interstitial keratitis and episcleritis of the both eyes. Oral adrenocorticosteroid therapy produced an improvement of interstitial keratitis and episcleritis. On admission, ophthalmological examination revealed bilateral interstitial keratitis and episcleritis, right retrobulbar optic neuritis and she was proven to have bilateral sensorineural deafness by otologist. Neurological examination revealed right abducens nerve paresis. Laboratory examinations revealed slightly increased erythrocyte sedimentation rate. CRP was positive. Serological tests for syphilis were negative. CSF showed mildly elevated protein level. Orbital CT scans revealed the swelling of right optic nerve. Cerebral MRI showed multiple high spotty areas in left thalamus, bilateral basal ganglia and deep white matter in T2 weighted images. After treatment with adrenocorticosteroid, right optic neuritis and abducens nerve paresis improved together with bilateral interstitial keratitis and episcleritis. Multiple cranial neuropathy may develop with Cogan's syndrome.