耳症状を主訴とした Wegener 肉芽腫症の1剖検例

A 41-year-old woman had a sudden attack of vertigo in June, 1981. Her audiogram showed severe left sensorineural hearing loss and mild right mixed hearing loss. She had nystagmus and absent left caloric response.Otolaryngological examination revealed granulation tissue in both nasal cavities, epipharynx, left middle ear and glottic regions. Laboratory data revealed elevated ESR, positive CRP and RA, inverted A/G ratio and leucocytosis. A biopsy specimen was obtained from the granulation tissue in the right nasal septum. Clinically and histologically, the diagnosis was a limited form of Wegener's granulomatosis.She was treated with cyclophosphamide and prednisolone. The granulation tissue decreased and hearing improved in the right ear, but severe sensorineural hearing loss in the left ear was not improved.She died of respiratory insufficiency about two years after the onset of the disease. Autopsy revealed necrotizing granulomatous lesions in the pharynx, larynx, trachea, main bronchi and lungs, and necrotizing angiitis in the main bronchi, lungs, kidneys and spleen.Wegener's granulomatosis is a connective-tissue disease with varying manifestations in numerous organ systems, especially the respiratory tract, but often involves the ear. The literature on hearing disturbance associated with Wegener's granulomatosis is reviewed, and the cause of sensorineural hearing loss is discussed.