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Published on: March 8, 2022
Hearing Loss in Leucine-Rich Glioma-Inactivated 1 Encephalitis: Cochlear Implantation Considerations
Nawfal Saleem1, Mackenzie Robbins2, Jacob Foster3
1Otolaryngology, Lake Erie College of Osteopathic Medicine - Bradenton (LECOM-Bradenton), Bradenton, USA.
None:
Leucine-rich glioma-inactivated 1 (LGI-1) antibody-associated autoimmune encephalitis is a rare neurologic disorder primarily presenting with memory impairment, behavioral changes, and seizures. Auditory symptoms, particularly sensorineural hearing loss (SNHL), are uncommon but may occur due to LGI-1 expression in auditory pathways. A 78-year-old male with a prior diagnosis of LGI-1 autoimmune encephalitis presented with progressive bilateral SNHL and frequent falls. His audiogram revealed significant deficits in high frequencies and impaired speech recognition without evidence of middle ear pathology, affecting quality of life. Neuroimaging showed chronic white matter lesions consistent with LGI-1 encephalitis. Cochlear implantation was discussed as a potential rehabilitative treatment. This case underscores SNHL as a potentially significant yet rare manifestation of LGI-1 antibody-associated autoimmune encephalitis. The patient's profound hearing loss suggests an autoimmune mechanism affecting both central auditory and peripheral cochlear pathways. Cochlear implantation, although not widely studied in autoimmune-mediated auditory disorders or trialed in this case, emerged as a promising option for rehabilitation, necessitating further investigation into its efficacy and safety.

