[Wegener's granulomatosis (WG) presented with hearing loss and without positive serologic ANCA].
BACKGROUND
Wegener's granulomatosis (WG) is a granulomatous inflammation involving the upper and lower respiratory tract and necrotizing vasculitis affecting small to medium-sized vessels. In contrast to a generalised WG with glomerulonephritis initial or isolated forms of the upper respiratory tract may be a diagnostic challenge.


METHODS
We report the case of a 33 year old man with clinical signs of a limited WG exhibiting an imminent irreversible hearing loss, negative PR3-ANCA (anti neutrophil cytoplasmic antibodies with proteinase 3 as target) in serum and an ambiguous histology.


CONCLUSIONS
In case of a chronic otitis media and rhinitis as well as signs of a labyrinthine deafness a limited form of a WG has to be taken into account, even with an ambiguous histology and negative PR3-ANCA. This diagnosis is supported by high inflammation parameters, e. g. ESR and CRP, exclusion of infectious cause and response to corticosteroids. A quick therapeutic intervention with corticosteroids and cyclophosphamide is required in order to interrupt the vasculitis of the inner ear with consequential deafness.
Göktas
O
O
Klinik und Poliklinik für Hals-Nasen-Ohrenheilkunde der Charité, Universitätsklinikum der Humboldt-Universität zu Berlin. oegoektas@hotmail.com
Hiepe
F
F
Paschen
C
C
D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D001301:Audiometry, Pure-Tone; D001706:Biopsy; D003937:Diagnosis, Differential; D007758:Ear, Inner / Q000098:blood supply*; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D006311:Hearing Disorders / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D006801:Humans; D008297:Male; D008416:Mastoid / Q000000981:diagnostic imaging / Q000473:pathology; D009297:Nasal Mucosa / Q000473:pathology; D010488:Polyarteritis Nodosa / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D014057:Tomography, X-Ray Computed
D019268:Antibodies, Antineutrophil Cytoplasmic