The hearing prognosis of otitis media with ANCA-associated vasculitis

The concept of otitis media with ANCA-associated vasculitis (OMAAV) was recently proposed by the study group of the Japan Otological Society. However, information remains limited regarding the hearing outcome of OMAAV. Thus, we investigated this issue in this study. We retrospectively examined 50 ears from 32 patients diagnosed with OMAAV at our hospital between 2010 and 2019. We collected the results of pure tone audiometry (PTA) at diagnosis and changes in PTA threshold after treatment, serological findings including ANCA type, titer, soluble interleukin-2 receptor (sIL2R), and C-reactive protein, organs involved at initial diagnosis, treatment, and disease relapse from medical records. According to the hearing outcome, patients were divided into two groups: good prognosis and poor prognosis groups. We investigated the clinical features, treatment, and changes in PTA between the groups. Age, sex, ANCA negativity, and the use of intravenous cyclophosphamide (IVCY) were significantly related to hearing prognosis of OMAAV, while other organs involved at diagnosis, serological findings, and relapse rate were not significantly associated with hearing outcome. Hearing level at diagnosis was significantly better in good prognosis group, while air-bone gap (ABG) was not significantly different between the groups. The air conduction (AC), bone conduction (BC), and ABG were significantly improved in the good prognosis group. However, ABG was not improved in the poor prognosis group, while AC and BC were significantly improved. The AC hearing level at diagnosis (58.5 dB) and hearing gain at 2 weeks after treatment (12.5 dB) were suggested as good indicators for predicting the hearing outcome of OMAAV. Younger age, male sex, shorter period from onset to diagnosis, the use of IVCY, and better hearing threshold at diagnosis were the good prognostic factors of the hearing outcome of OMAAV. These results suggest that earlier diagnosis of OMAAV might be needed for better hearing outcome, and the use of IVCY may be recommended for the treatment of OMAAV patients.