Creutzfeldt-Jakob disease is a rare type of spongiform encephalopathy. Affected patients present with constitutional symptoms, which progress to severe mental deterioration and movement disorders. Dizziness is the most common early otologic symptom. Few reports in the literature describe patients with Creutzfeldt-Jakob disease who present with sudden-onset hearing loss as their primary symptom for seeking treatment. This paper discusses one such patient and reviews the clinical presentation, treatment options, and relevant literature.