Learning Objectives: 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. 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 session, the current understanding of this newly proposed concept of OMAAV is discussed.

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. By reviewed previous case reports, such disease was proposed to be categorized as “otitis media with AAV (OMAAV).”

Nationwide survey (total 235 cases) performed between December 2013 and February 2014 by the Japan otological society presented the following features: 1) otitis media following sudden progressive hearing loss almost in one month; 2) intractable otitis media not effected by antibiotics and tympanic tube insertion; 3) mostly PR3- and/or MPO-ANCA positive but 16% showed both ANCAs-negative; 4) occasionally clinical complications such as facial palsy (36%) or hypertrophic pachymeningitis (28%); 5) tympanic membrane showing a dull appearance similar to OME and vessel dilatation of tympanic membrane ‘OME type’, otitis media with granulation ‘OMG type’, or normal appearance only with sensorineural hearing loss; 6) effectiveness of corticosteroid and immunosuppressive therapy using cyclophosphamide or methotrexate. By analysis of these clinical features, four factors such as facial palsy, hypertrophic pachymeningitis, both ANCAs-negative phenotype, and disease relapse were related to an unfavorable clinical course for patient's hearing and prognosis. 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 session, the current understanding of this newly proposed concept of OMAAV is discussed.