[Wegener's granulomatosis: otologic and clinico-immunologic aspects].

Clinical, audiometric and immunological data of 26 patients with histological proven Wegener's granulomatosis are presented. In 21 patients the middle ear was involved with serous otitis media, adhesive process or subacute otitis media. 28 of 36 audiometric examinations showed a mild to moderate sensorineural hearing loss. One ear did not recover from a sudden deafness in the early stage of the disease despite of immunosuppressive therapy. Beside clinical and morphological findings in Wegener's granulomatosis the detection of anticytoplasmic antibodies against neutrophil granulocytes (ANCA) is a reliable method in diagnosing the disease. In the present study, the serum of 19 out of 26 patients with histologically proven Wegener's granulomatosis was examined for ANCA by immunofluorescence test. In high activity stage of Wegener's granulomatosis all sera showed a positive ANCA-test. 39 patients with systemic vasculitis served as controls. Only two sera from patients with leucocytoclastic vasculitis and two with panarteriitis nodosa were ANCA positive. The follow-up (35 serum probes) of 8 patients showed no ANCA after immunosuppressive induced remission. In four histologically detected cases of recurrence ANCAs were positive again. ANCAs are very useful in primary diagnosis of Wegener's granulomatosis as well as in the follow-up. In addition, the analysis reveals, that immunosuppressive therapy with cyclophosphamide and prednisone facilitates complete remission and a long survival rate of patients with Wegener's granulomatosis.