[Cogan-I-syndrome. A rare differential diagnosis in progressive sensorineural hearing loss]

We report on two patients affected by Cogan's syndrome since infancy.Cogan's syndrome is described as the association of abrupt or intermittent worsening of vestibulo-auditory function and non-syphilic interstitial keratitis or, in patients with "atypical" Cogan's syndrome, various severe, inflammatory eye diseases. The first patient was affected with different inflammatory eye diseases from her fifth year and became blind in one eye when she was 7 years old. At that time, a deterioration of her hearing ability was also diagnosed. The hearing loss progressed to a severe hearing disorder. The second patient suffered from different inflammatory eye diseases from his second year. When he was 3 years old,he was diagnosed with a severe hearing disorder that progressed.Cogan's syndrome was diagnosed when he was 13 years old. The recognition of Cogan's syndrome is particularly complex in young patients. The difficulty of the diagnosis requires a search for ophthalmic diseases in young children with acute and progressive vestibulo-auditory symptoms.