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.