Early diagnosis of Wegener's granulomatosis presenting with facial nerve palsy

Wegener's granulomatosis is a multi-system disease characterized by granuloma formation and a necrotizing vasculitis. It classically presents with involvement of the upper and lower respiratory and renal systems. However locoregional disease is common and may include otological manifestations....

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Bibliographic Details
Published in:Journal of laryngology and otology 1996-05, Vol.110 (5), p.459-461
Main Authors: Hern, J. D., Hollis, L. J., Mochloulis, G., Montgomery, P. Q., Tolley, N. S.
Format: Article
Language:English
Subjects:
Aged
Antibodies, Antineutrophil Cytoplasmic
Autoantibodies - blood
Biological and medical sciences
Blood Sedimentation
Clinical Records
Facial paralysis
Facial Paralysis - etiology
Facial Paralysis - pathology
Female
Granulomatosis with Polyangiitis - blood
Granulomatosis with Polyangiitis - complications
Granulomatosis with Polyangiitis - diagnosis
Granulomatosis with Polyangiitis - pathology
Humans
Medical sciences
Nasal Mucosa - pathology
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
Tomography, X-Ray Computed
Wegener's granulomatosis
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Summary:Wegener's granulomatosis is a multi-system disease characterized by granuloma formation and a necrotizing vasculitis. It classically presents with involvement of the upper and lower respiratory and renal systems. However locoregional disease is common and may include otological manifestations. Facial nerve palsy has been reported during the course of the disease process but it is extremely rare for it to be the presenting feature. Previously reported cases have involved a protracted diagnostic process including exploratory tympanotomy, mastoidectomy and facial nerve decompression. We report a case of Wegener's granulomatosis which presented with a facial nerve palsy. An early diagnosis was achieved by measurement of the erythrocyte sedimentation rate (ESR), followed by serological assay of cytoplasmic anti-neutrophil cytoplasmic antibody (cANCA). Histological examination of nasal mucosal biopsies confirmed granuloma formation consistent with a diagnosis of Wegener's granulomatosis. This allowed early treatment with immunosuppressive therapy and avoided unnecessary and potentially hazardous middle ear surgery.
ISSN:0022-2151
1748-5460
DOI:10.1017/S0022215100133973