Systemic vasculitis includes a broad spectrum of disorders that may involve blood vessels of any size in any organ system. Systemic vasculitis is associated with immunopathogenic mechanisms. Sixteen temporal bones from eight persons were studied to determine histopathologic changes that occur in systemic vasculitis. Three persons had Wegener's granulomatosis, two had polyarteritis nodosa, and three had systemic lupus erythematosus. Otitis media was seen in 15 ears, with ten ears showing chronic middle ear changes and two showing fibrotic inner ear changes. In Wegener's granulomatosis, granulation tissue was observed around the eustachian tube and protympanum, and in polyarteritis nodosa, inflammatory cell infiltrate and thickened blood vessels were observed around the facial nerve. Although sensorineural hearing loss has been described clinically in systemic lupus erythematosus, the present report describes findings in temporal bones, including severe fibrosis and new bone formation throughout the inner ear.