抄録
Wegener's granulomatosis is a distinct clinicopathologic entity characterized by granulomatosis vasculitis of the respiratory tract accompanied by glomerulonephritis. We report a case of a 56-year-old female with localized Wegener's granulomatosis presenting as otitis media. The patient complained of right ear pain. Antibiotic treatment was ineffectual and right sensorineural deafness and right facial palsy became apparent. Perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) was positive, but cytoplasmic anti-neutrophil cytoplasmic antibody (c-ANCA) was negative. The histopathologic appearance of granulation tissue in her tympanic and nasal cavity indicated inflammatory granulation involving multinucleated giant cells and epithelioid cells. The disease did not involve her lung or kidney, so it was diagnosed as localized Wegener's granulomatosis. Glucocorticoid treatment with cyclophosphamide improved the right hearing loss and facial palsy. CRP and p-ANCA became negative.
Early diagnosis of Wegener's granulomatosis is difficult because of atypical manifestations of the disease, but ANCA measurements may be effective in the diagnosis. The present case suggests that inner ear blood flow impairment due to p-ANCA-associated small vessel vasculitis induces hearing loss.
