Clinical records: a case report of Wegener's granulomatosis limited to the ear.

A 12-year-old girl had a 1-week history of left otalgia with hearing loss in May, 1989. Audiometry showed a conductive hearing loss of 60 dB. The conventional medical treatment for acute otitis media was initiated, but the symptoms and signs failed to respond this treatment. Four weeks after initial presentation, a diagnostic mastoidectomy was performed. The histological findings of the granulomatous tissue in the mastoid cavity showed nonspecific granulomatous inflammation, infiltrated with multinucleated giant cells. Initial laboratory studies revealed elevated ESR, positive CRP and RA, inverted A/G ratio, and increased Ig level. She had no other sites of involvement, such as the nose, paranasal sinus, pharynx, and larynx, and also no pulmonary or renal involvement was indicated. The diagnosis of a limited form of Wegener's granulomatosis was made on the above clinical and histological findings and laboratory examinations. The patient was then treated with cyclophosphamide, 80 mg/day (2 mg/kg) and prednisolone, 60 mg/day. After 1 week her symptoms and general condition were dramatically improved. Out-patient follow-up 1 year later showed her hearing level was improved to 28 dB, and she continues to do well without evidence of recurrence of the disease.