Göktas O, Hiepe F, Paschen C
Klinik und Poliklinik für Hals-Nasen-Ohrenheilkunde der Charité, Universitätsklinikum der Humboldt-Universität zu Berlin.
Laryngorhinootologie. 2004 Mar;83(3):180-4. doi: 10.1055/s-2004-814308.
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.
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.
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.
