Objective:

To report a case of Wernicke Encephalopathy presenting as bilateral sensorineural hearing loss in the setting of acute pancreatitis.

Background:

Wernicke Encephalopathy (WE) is a severe neuropsychiatric disorder characterized by the clinical triad of ophthalmoplegia, ataxia and confusion. Despite our understanding of this condition, patients rarely present with this unambiguous triad. Per literature review, a few cases of thiamine deficiency with sensorineural hearing loss (SNHL) have been reported thus far.

Design/Methods:

50-year-old African American female admitted with sudden onset hearing loss and gait imbalance. She also had visual hallucinations of cats in her hospital room. She was recently treated for acute pancreatitis and had 50-pound weight loss over the last 3 months. Her neurologic exam demonstrated severe bilateral mixed hearing loss on Weber and Rinne tests. She had sustained nystagmus in all directions. Her speech remained intact. Thiamine level was low at 45nmol/L. Audiometry exam showed mixed SNHL loss with hearing levels of 95dB in right ear and 100dB in the left at 1000Hz. MRI head was unremarkable of typical Wernicke findings. Ancillary labs including CSF were unremarkable for any other inflammatory or autoimmune etiology. Thiamine was administered inpatient. Patient showed only mild improvement of her nystagmus. Audiometry after thiamine administration showed persistent severe to profound bilaterally hearing loss.

Results:

NA

Conclusions:

SNHL is an extremely rare complication of WE. Non-alcoholic causes of WE are less common, hence, a large portion of WE secondary to malnutrition is misdiagnosed in early stages. WE presents a diagnostic challenge because of its variability in presentation and radio-imaging. Prompt detection and treatment of WE is vital to prevent permanent injury to the central nervous system and inner ear hair cells.