Abstract
Antineutrophil cytoplasmic antibody (ANCA) - associated vasculitis (AAV) is characterized by systemic necrotizing vasculitis. AAV comprise Granulomatosis with polyangitis (GPA), microscopic polyangitis (MPA) defined as necrotizing vasculitis of small vessels, including its renal limited subset designated as idiopathic necrotizing crescentic glomerulonephritis (iNCGN) and Churg-Strauss syndrome. The elevation of proteinase-ANCA (PRO-ANCA) and / or myeloperoxidase - ANCA (MPO-ANCA) titers are one of the criteria for diagnosis of ANCA associated vasculitis. AAV commonly manifests as a rapidly progressive glomerular nephritis (RPGN), with necrotizing glomerular tufts, alveolar hemorrhage, or interstitial pneumonia. The clinical features of PR3- positive and MPO-ANCA positive cases which diagnosed by the hearing loss as initial symptoms are presented.
Eight cases (six females, two males; age from 54 to 73 years; six MPO (myeloperoxidase)-ANCA positive, two PR3 (proteinase 3)-ANCA positive cases) were included in this study. Progressive hearing loss (100%) and hemi/bilateral facial palsy (62.5%) were present in the patients before diagnosis. The characteristic features of hearing were as follows: 1) Rapid hearing loss within less than 2 months 2) completely deaf could not be recovered after immunosuppression therapy 3) hearing levels better than 95 dB were improved with good speech discrimination even at the high frequency.
This study showed the diagnosis of localized ANCA associated vasculitis and the effectiveness of immunosuppressive therapy for hearing loss at an early stage. The immunosuppression therapy would be necessary for hearing disturbance as soon as detecting the elevation of PR3-ANCA/MPO-ANCA titer.
