Cochlear implantation in patients with bilateral deafness caused by otitis media with ANCA-associated vasculitis (OMAAV): A report of four cases

Abstract Objective Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) without systemic symptoms but with initial symptoms related to the ear, such as hearing loss, otalgia, and dizziness, has recently been reported. We have categorized this condition as otitis media with AAV (OMAAV), and have recently proposed its diagnostic criteria. Methods To determine the effectiveness of cochlear implantation (CI) in patients with profound hearing loss due to OMAAV. We examined the language understanding ability of four patients with bilateral profound or total deafness due to OMAAV, who underwent CI. Results In three of the four patients, the language understanding ability with CI was poor. These three patients with poor performance had characteristic features, including a short interval from the onset of ear symptoms to total deafness and clear enhancement of the cochlea on magnetic resonance imaging (MRI). Conclusion The poor results observed in patients with a rapidly progressive history of hearing loss were attributed to possible severe and profuse intracochlear bleeding and/or destruction of structures, including the spiral ganglion. All the three patients showed contrast enhancement in the inner ear on MRI. We believe that preoperative evaluation of the history of hearing loss as well as the findings of contrast-enhanced MRI is important for predicting the prognosis after CI.