診断が遅れたANCA関連血管炎性中耳炎（OMAAV）例

抄録

We encountered a difficult case of otitis media with ANCA-associated vasculitis (OMAAV), in which definitive diagnosis and treatment were delayed because serology for ANCA showed a negative result at the first visit.

The patient was a 71-year-old woman who visited us with the complaint of bilateral progressive combined hearing loss. Despite bilateral ventilation tube insertion to treat the otitis media with effusion, the hearing impairment only became worse. Therefore, we started the patient on oral prednisolone as the second treatment option, and the dramatic response to prednisolone led us to strongly consider the possibility of OMAAV. However, based on the initial negative result of serology for ANCA at that time, we rejected the possibility. Approximately a year later, the patient was diagnosed as having bilateral pneumonia, and the serum test for ANCA was positive this time. Based on these serial observations, we finally diagnosed the patient as having eosinophilic granulomatosis with polyangiitis (EGPA), possibly associated with OMAAV.

The main issue here was the long time (almost one year) it took to arrive at the correct diagnosis of OMAAV, which resulted in delayed treatment and unsatisfactory recovery of the hearing impairment. Precise comprehension of both the history of steroid therapy and modulation of hearing during the initial treatment are keys for correct diagnosis of OMAAV. We also briefly review the literature on OMAAV.