چکیده (انگلیسی):

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is histologically characterized by systemic
necrotizing vasculitis and is clinically classified into two phases, systemic or localized. Recently, otological
symptoms such as otitis media and hearing loss, not previously often associated with AAV, have been reported
in AAV cases. In these cases we propose a diagnosis of otitis media with AAV (OMAAV). The ANCA
titer is important for the diagnosis of OMAAV, and in most cases rapid progressive hearing loss is observed as
localized AAV. Peripheral facial nerve palsy or hypertrophic pachymeningitis are coupled with 25% of cases
and 18% of cases respectively. Proteinase 3-ANCA (PR3-ANCA) positive otitis media causes granulomatous
formation or middle ear effusion in the middle ear, on the other hand myeloperoxidase-ANCA (MPO-ANCA)
positive otitis media predominantly presents as otitis media with effusion. The early diagnosed case and the
sensorineural hearing loss not progressed deaf could be recovered by the immunosuppressive therapy. Delayed
diagnosis of AAV occasionally leads to progression to the irreversible phase; therefore, diagnosis at the
early-localized stage is important for treating AAV. In this review, we discuss the current understanding of this
newly proposed concept of OMAAV.