Spectrum of immune-mediated inner ear disease and cochlear implant results

Objectives/Hypothesis: To characterize the progression of hearing loss in patients with immune-mediated inner ear disease (IMIED), and to identify disease- and patient-specific factors associated with cochlear implant (CI) performance. Study Design: Retrospective cohort study. Methods: Subjects consisted of CI patients suspected to have lost their hearing due to IMIED. The primary dependent variable for functional decline was time to deafness, whereas for CI benefit it was post-CI speech perception scores. Independent variables included presence or absence of systemic autoimmune disease, age at CI, and insertion depth of the cochlear electrode. Results: A transient favorable response to immunosuppressive therapy was reported in 16 of 26 patients (66.67%). The time to deafness differed between an organ (ear)-specific immune-mediated group, a systemic immune-mediated group including Cogan syndrome and relapsing polychondritis (subgroup A), and a systemic immune-mediated group associated with other autoimmune diseases (subgroup B; P =.001). Disease group (-15.52; P =.04), insertion depth of the CI electrode (40.71; P =.01), and the age at CI (-0.48, P =.05) were associated with speech perception results. Conclusions: Triaging IMIED cases based on presence and type of systemic autoimmune disease may aid in selecting a management strategy. Knowledge about the predictors of CI outcome will help clinicians select appropriate patients for CIs. In the setting of significant and irreversible hearing deficit, the restoration of hearing using a cochlear prosthesis may be appropriate earlier rather than later.