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

Abstract

AbstractWegener'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.