Combined Electric acoustic stimulation in a patient with otitis media with antineutrophil cytoplasmic antibody-associated vasculitis

Abstract

There are few reports of the treatment for severe hearing loss due to otitis media with antineutrophil cytoplasmic antibody-associated vasculitis (OMAAV) achieved by cochlear implantation (CI). Here, we have reported the case of a patient with severe bilateral sensorineural hearing loss with low-frequency residual hearing by OMAAV. CI was performed in her right ear based on the results of contrast-enhanced magnetic resonance imaging (CE-MRI) and promontory stimulation test (PST). The residual hearing in her right ear was preserved after CI and utilized for combined electric acoustic stimulation (EAS). The combined EAS was used for 3 years until the residual hearing became stabilized. However, the usable hearing in low frequency worsened gradually, and the fitting strategy of cochlear implant was changed from combined EAS to CI alone 4 years after CI. Even when the speech discrimination score with CI no longer exceeds 50 %, the patient continued using CI because of its advantages in maintaining the quality of life of the patient. The combined EAS was found to be a feasible option even in an OMAAV patient with residual hearing. CE-MRI and PST may thus be helpful in deciding the side of CI surgery in a patient with OMAAV.

Keywords: Antineutrophil cytoplasmic antibody; Cochlear implant; Combined electric acoustic stimulation; Otitis media with antineutrophil cytoplasmic antibody-associated vasculitis.