Wegener's Granulomatosis (WG) is a rare form of systemic necrotizing vasculitis characterized by inflammation of the walls of the blood vessels and affects the upper and lower respiratory tracts and kidneys. The nose and paranasal sinuses are often the first or main sites to be affected. Otologic symptoms as a complication of Wegener's granulomatosis (WG) isrelatively rare. The authors report a 50-year-old femal suffering from bilateral hearing loss. She had been diagnosed as having otitis media with effusion, and received medical treatment, but the symptoms and signs failed to respond the treatment. She had no other sitesof involvement, such as the nose, paranasal sinus, pharynx, and larynx. Audiometry showed bilateral mixed hearing impairment with a loss of 100 dB. The histological findings of theswollen and eczematous right external auditory canal revealed granulomatous inflammation accompanied by epithelioid cell aggregation around capillaries. Chest X-ray showed a small nodule in the right lower lung field and TBLB revealed leukocytoclastic vasculitis of capillary vessels. Laboratory studies revealed positive CRP and RA. Anti-neutrophil cytoplasmic autoantibody (cANCA) was negative. Thus, the diagnosis of a localized form of Wegener's granulomatosis was made. She was given prednisolone 60mg/day, cyclophosphamide 75mg/day and sulfamethoxazole trimethoprim 960mg/day, which greatly improved mixed hearing loss to 20-30 dB bilaterally. The patient had no complications' during the treatment and showed good recovery.
