To describe acute sensorineural hearing loss as a rare manifestation of Wernicke Encephalopathy (WE) and underscore the importance of early recognition and prompt thiamine repletion. 

Thiamine deficiency is a well-recognized etiological factor in neurological and cardiovascular pathologies, most commonly associated with chronic alcohol use, prior bariatric surgery, or diets predominantly composed of milled grains, which are low in thiamine. Wernicke Encephalopathy (WE), a severe neuropsychiatric disorder resulting from thiamine deficiency, classically presents with the triad of ophthalmoplegia, ataxia, and confusion. Sensorineural hearing loss, however, is an exceedingly rare complication of WE, with only a limited number of cases reported in the literature.

Single-patient Case Report. 

A 19-year-old female, 11 months postpartum, with a history of anxiety, bipolar disorder, bulimia nervosa, and postpartum depression presented with altered mental status, generalized weakness, lethargy, and acute bilateral hearing loss in the context of profound weight loss. The patient had discontinued her psychiatric medications during pregnancy and did not resume them postpartum due to persistent nausea. Five months after delivery, she developed a silicone allergy exacerbating her anxiety with resultant severe food aversion and recurrent emesis. Over the ensuing months, she experienced progressive weight loss of approximately 100 pounds. Communication was conducted through writing and lip reading due to hearing loss. Neurological examination revealed altered mentation, ophthalmoplegia and truncal ataxia. MRI brain demonstrated restricted diffusion and FLAIR signal abnormalities in the bilateral medial thalami and periaqueductal gray matter, consistent with the diagnosis of WE. High-dose intravenous thiamine and folate were initiated promptly, resulting in clinical improvement in hearing, mentation, and ataxia. 

WE is an acute neurological emergency that is often underdiagnosed in clinical practice. The presence of vestibular dysfunction is well-established, however evidence of hearing loss as a symptom of WE is rare.