A Case of Severe Wernicke Encephalopathy with Bilateral Sensorineural Hearing Loss (P2.139)

Objective: NA Background: Wernicke encephalopathy (WE) is a feared neurological complication of thiamine deficiency. Delayed treatment can cause significant morbidity. The syndrome is associated with poor nutrition and classically presents with the triad of encephalopathy, oculomotor dysfunction, and gait ataxia. We report a patient who presented with bilateral sensorineural hearing loss in addition to the typical triad of WE. Design/Methods: NA Results: A 43-year-old woman with past a medical history of alcohol abuse, ulcerative colitis, and colectomy presented with altered mental status after being found sleeping on the floor of her home. The patient was found to have mild encephalopathy, severe ophthalmoplegia with bilateral involvement of cranial nerves 3, 4, and 6, marked gait ataxia, and bilateral sensorineural hearing loss with tinnitus. MRI brain demonstrated symmetric T2 and FLAIR hyperintensities without contrast enhancement in the bilateral dorsal median nuclei of the thalamus, periaqueductal gray matter and the floor of 4th ventricle, all of which are typically observed in WE. These were interval developments when compared to a MRI obtained five months prior for an episode of altered mental status. WE was promptly diagnosed and the patient was given aggressive thiamine supplementation. Within hours, the patient demonstrated striking, although incomplete, improvement of her ophthalmoplegia. Her bilateral hearing loss demonstrated incomplete improvement based on repeat audiograms at three month follow-up. Conclusions: Wernicke encephalopathy is a neurological emergency, requiring emergent treatment. Prompt recognition is essential, however, the entire triad is absent in two thirds of patients and is often missed clinically. Sensorineural hearing loss is not associated with WE, however this case illustrates that it can be part of the presenting clinical picture. We hypothesize that the bilateral sensorineural hearing loss is secondary to involvement of the ascending afferent auditory fibers located in the midbrain and pons, and may respond to thiamine therapy. Disclosure: Dr. Hansen has nothing to disclose. Dr. Bozorgi has nothing to disclose. Dr. Richards has nothing to disclose. Dr. Thammongkolchai has nothing to disclose. Dr. Devereaux has nothing to disclose.