Cochlear implantation in systemic autoimmune disease

Autoimmune 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.

Hearing 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.

Diagnosis of AIED is largely clinical, and a strong index of suspicion is required. Multidisciplinary care is crucial for optimal management. Cochlear implant outcomes are generally excellent, but the clinician needs to be cognizant of the pitfalls of encountering intracochlear fibrosis intraoperatively and likelihood of implant performance fluctuation related to ongoing inflammation in the cochlea.