Purpose of reviewAutoimmune inner ear disease (AIED) is a rare, but likely underrecognized cause of hearing loss. However, hearing loss is common in systemic autoimmune disease and it is important for the clinician to be familiar with the spectrum of disease. In this article, we will review the developments in diagnosis and management of AIED, with a focus on the outcomes and potential pitfalls of cochlear implantation.
Recent findingsHearing loss in AIED tends to be progressive and bilateral, but up to 40% can present as sudden hearing loss and one-third can present unilaterally. HSP-70 serology may help with diagnosis and may predict steroid response. Cochlear implantation provides excellent hearing and quality of life outcomes in patients deafened by AIED. Intracochlear fibrosis/ossification is found intraoperatively in the majority (54%) of patients undergoing cochlear implantation. A large percentage (32%) of patients has fluctuating impedances postimplantation, which may interfere with implant performance and mapping.