Cochlear Implantation in Autoimmune Inner-Ear Disease: Outcome and Patient-Reported Benefit

To investigate cochlear implant (CI) outcomes in patients with hearing loss related to secondary autoimmune inner ear disease (AIED) compared to a matched control cohort.

In this retrospective cohort study, we included seven patients with profound sensorineural hearing loss (SNHL) related to secondary AIED treated with a CI. Data on pre- and postoperative pure-tone audiometry, speech perception in quiet and noise (German Freiburg mono- and multisyllable test, Oldenburg sentence test), and patient-reported outcome measures (Speech, Spatial, and Qualities of Hearing Scale, SSQ12) were assessed and compared to a matched control cohort.

Postoperative recognition of monosyllables (65 dB_SPL) in AIED patients and controls showed significant improvement (p < 0.0001) after 12 months with gains of 54 and 58 percentage points, respectively. However, AIED patients required a substantially higher signal to achieve 50%-word recognition in sentence testing in noise compared to controls. Notably, subjective hearing abilities tested in the SSQ12 in both groups improved substantially, reaching 4.4 [2.4-6.5] in the AIED group and 5.1 [4.5-5.8] in controls at 12 months, showing a convergence of scores compared to the preoperative group difference.

AIED patients substantially benefit from cochlear implantation despite potentially ongoing chronic inner-ear inflammation. In contrast to poorer performance in classical audiological test settings such as monosyllable presentation and word recognition in noise, self-perceived hearing was reported nearly as favorable as in the control cohort. Timely diagnosis, tailored preoperative imaging, and individualized rehabilitation are key to improved CI performance in AIED patients.