Cochlear Implantation Outcomes in Patients With Autoimmune and Immune-Mediated Inner Ear Disease

Abstract

Cochlear implantation is a safe and viable option for hearing rehabilitation in patients deafened by progressive AIED. Hearing outcomes in AIED patients are excellent and support transition to implantation when hearing is lost or long-term steroid therapy becomes undesirable. Cochlear fibrosis or ossification seems to affect nearly 50% of implanted ears (41.6% of patients) in this study and implies that the cochlea is at risk for ossification changes long term. In appropriate candidates, earlier implantation may be indicated before postinflammatory obliterative changes in the cochlea.

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

“…Speech perception results reported in this study are comparable to other reports in IMIED patients, which include open‐set word recognition and sentence scores of 61% and 69%, respectively, at 6 months, with subsequent scores of 84% and 100% at 1 year 19. In a similar study, Aftab et al5 reported no difference in hearing outcomes between 10 patients with IMIED compared to 12 randomly selected CI patients with other diagnoses. Unlike studies in diverse patient populations, the present study did not reveal a significant effect of baseline speech understanding or duration of deafness on post‐CI speech perception 20–22.…”

“…Eventually, patients with IMIED, after being placed on several trials of immunosuppressive agents, reach a level of profound deafness and are deemed candidates for cochlear implantation (CI). Although favorable CI benefits have been reported, prognostic indicators unique to this population are lacking 5…”

Systematic Review of Treatments for Autoimmune Inner Ear Disease

“…Although there may be higher rates of fibrosis and ossification within the cochlea, patients with AIED perform at least as well as matched control patients, with other etiologies of hearing loss, after implantation (52,53).…”

Successful Cochlear Implantation in a Case of Vogt–Koyanagi–Harada Disease

“…Autoimmune inner ear disease has been shown to cause inflammation that may result in cochlear fibrosis and calcification in animal models (11) leading to osteoneogenesis similar to that observed in meningitis (6). Intracochlear changes in VKH have not been reported, but could be possible.…”