INTRODUCTION: Wegener's granulomatosis (WG) is a vasculitis that effects the upper and lower part of the respiratory tract and the kidneys. Untreated the disease results in death within weeks or months. The diagnosis is based on clinical criteria, level of antineutrophil cytoplasmatic antibodies (ANCA) and signs of granulomatous necrotizing vasculitis in histology. METHODS: A case of an 18-year-old woman with initially symptoms of bilateral "mastoiditis" and weakness of her facial nerve is described. In this case ANCA levels remained normal for 3 months and persistent otological symptoms were predominant. The further clinical course was characterized by neurological problems (Palsy Nn. VII, IX, XII and thrombosis of the right sigmoid sinus). The initial therapy consisted of Prednisone 100 mg and Cyclophosphamide 100 mg daily. The patient has been treated successfully with Methotrexate 20 mg 1 x/week and Prednisone 15 mg/die for 4 months now. CONCLUSION: A common clinical presentation of WG involves the upper respiratory tract. Therefore ENT-specialists should be familiar with the disease. Especially in cases of persistent signs of bilateral "Mastoiditis" and neurological symptoms WG should be ruled out as differential diagnosis. A close interdisciplinary cooperation is essential for therapy and follow-up, because systemic involvement is the limiting prognostic factor.
