OBJECTIVE: To present 8 cases with hearing loss as an initial symptom of antineural cytoplasmic antibody (ANCA)-associated vasculitis (AAV) involving granulomatosis with polyangiitis (GPA) and to discuss the treatment and mechanisms of hearing outcomes after immunosuppressive therapy. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Eight patients were referred to our university hospital between 2004 and 2012 for intractable otitis media with acute progressive mixed (conductive and sensorineural) hearing loss and facial palsy. INTERVENTION(S): Diagnostics and treatment. MAIN OUTCOME MEASURES: Otologic symptoms as initial manifestations of otitis media with AAV and cochlear function after treatment. RESULTS: Eight cases (6 female and 2 male subjects; aged 54-73 yr; 6 MPO [myeloperoxidase]-ANCA-positive and 2 PR3 [proteinase 3]-ANCA-positive cases) were included. Progressive hearing loss was present in all patients, and facial palsy was present in 5 of 8 patients total. Patients with hearing levels better than 95 dB improved with good speech discrimination after immunosuppressive therapy, but the completely deaf could not be recovered. All patients have been successfully controlled for 1 to 8 years without any systemic disorders. CONCLUSION: This study showed the difficulty of diagnosing localized AAV and the effectiveness of immunosuppressive therapy for hearing loss in the early stage. Based on these results, early-stage AAV would influence the stria vascularis in the cochlea. Otitis media with ANCA-associated vasculitis is a new entity among the causes of intractable otitis media and progressive hearing loss.
OBJECTIVE: To present 8 cases with hearing loss as an initial symptom of antineural cytoplasmic antibody (ANCA)-associated vasculitis (AAV) involving granulomatosis with polyangiitis (GPA) and to discuss the treatment and mechanisms of hearing outcomes after immunosuppressive therapy. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Eight patients were referred to our university hospital between 2004 and 2012 for intractable otitis media with acute progressive mixed (conductive and sensorineural) hearing loss and facial palsy. INTERVENTION(S): Diagnostics and treatment. MAIN OUTCOME MEASURES: Otologic symptoms as initial manifestations of otitis media with AAV and cochlear function after treatment. RESULTS: Eight cases (6 female and 2 male subjects; aged 54-73 yr; 6 MPO [myeloperoxidase]-ANCA-positive and 2 PR3 [proteinase 3]-ANCA-positive cases) were included. Progressive hearing loss was present in all patients, and facial palsy was present in 5 of 8 patients total. Patients with hearing levels better than 95 dB improved with good speech discrimination after immunosuppressive therapy, but the completely deaf could not be recovered. All patients have been successfully controlled for 1 to 8 years without any systemic disorders. CONCLUSION: This study showed the difficulty of diagnosing localized AAV and the effectiveness of immunosuppressive therapy for hearing loss in the early stage. Based on these results, early-stage AAV would influence the stria vascularis in the cochlea. Otitis media with ANCA-associated vasculitis is a new entity among the causes of intractable otitis media and progressive hearing loss.