(A821) First Report of Permanent Hearing Loss Due to Wernicke-Korsakoff Syndrome Treated with Bilateral Cochlear Implants

Introduction: Sensorineural hearing loss (SNHL) is a rare complication of Wernicke’s encephalopathy with only a handful of reports in the literature. In all cases hearing improved with thiamine replacement. We report the 1st case of permanent bilateral SNHL that did not improve with thiamine replacement due to Wernicke-Korsakoff syndrome in a patient with alcohol use disorder

Methods: A case report and review of the literature are presented.

Results: A 49 year-old female presented six months after hospitalization for Wernicke-Korsakoff Syndrome complaining of progressive hearing loss that began just prior to her hospitalization. Audiometry demonstrated profound bilateral SNHL. She was treated with bilateral cochlear implantation. AzBio scores in quite improved from 0% bilaterally to 58% in the right ear and 59% in the left ear at initial activation. Most patients presenting with SNHL in the setting of Wernicke's encephalopathy or thiamine deficiency will regain some degree hearing with appropriate medical treatment. We demonstrate that cochlear implantation is an appropriate treatment option in the rare instance of permanent SNHL resulting from Wernicke’s encephalopathy. Proposed mechanisms of SNHL related to Wernicke's encephalopathy and thiamine deficiency are discussed.

Conclusions: Though very rare, Wernicke-Korsakoff syndrome and Wernicke's encephalopathy can be associated with permanent hearing loss despite appropriate medical treatment. These patients may be excellent candidates for cochlear implantation.