Mastoiditis and facial paralysis as initial manifestations of Wegener's Granulomatosis

Wegener's Granulomatosis (WG) is characterized by necrotizing granulomas and vasculitis. If left untreated, the prognosis is poor – a 90% mortality rate within 2 years. Several authors have described the otologic manifestations of WG; these authors, however, have not mentioned the stage of the disease in which these findings present – whether as initial manifestations or subsequent to other findings. AIM: To describe three confirmed cases of WG with mastoiditis as the first manifestation, progressing to peripheral facial paralysis (PFP). MATERIAL AND METHOD: A clinical series study. Patients diagnosed with WG that initially presented with otologic findings are described. RESULTS: The three cases presented with unilateral otalgia, otorrhea, and hearing loss associated with ipsilateral PFP. None recovered in spite of the treatment; an investigation of associated diseases was therefore undertaken. Positive ANCA-C titers where detected in all patients, confirming the diagnosis of WG. Clinical improvement was seen after treatment of WG; the PFP regressed and hearing thresholds improved partially. CONCLUSION: Complications of otitis media (mastoiditis and PFP) that do not respond to the usual treatment require an investigation of associated diseases; WG should be included for an early diagnosis to change the prognosis in these patients.