[Inner ear autoimmune disorder. Cogan's syndrome].

We present a case of Cogan's syndrome (interstitial keratitis, rapidly evolving sensorineural hearing loss and vestibular dysfunction) in a 12-year-old girl. Diagnosis was made by clinical and audiologic examination as well as by nonspecific laboratory findings (increased erythrosedimentation rate and positive IgA and IgM rheumatoid factor titers and antineutrophil cytoplasmic autoantibodies). Detection of specific autoantibodies against cochlear antigenic epitopes with a molecular weight of 68 kDa by Western-blot assay confirmed an organ-specific autoimmune mechanism. Therapy with deflazacort 1 mg/kg per day 3 three weeks decreasing progressively thereafter was successful and the patient remains symptom-free.