A 72-year-old female patient presented with otitis of her left ear with inner ear depletion. Despite administration of intravenous antibiotics according to the resistogram and mastoidectomy the patient developed a similar pathological condition in the right ear and facial nerve palsy on the left side. Treatment with trimethoprim/sulfamethoxazole under the working diagnosis of a localised seronegative Wegener's granulomatosis was initiated without achieving remission. Cyclophosphamide pulse therapy in combination with high-dose methylprednisolone was initiated with rapid cure of the bilateral otitis. Sensorineural hearing loss and facial nerve palsy remained irreversible.