Creutzfetdt-Jakob disease (CJD) is one of the uniformly fatal spongiform encephalopathies that is characterized clinically by an unrelenting progression of myoclonus, dementia, and ataxia. Since many of these patients will develop cerebellar abnormalities, some may present to the otolaryngologist with dizziness. Hearing loss, however, to our knowledge, has not been reported. We describe a patient with CJD who presented with hearing loss and vague symptoms of imbalance, and whose disease progressed rapidly and fatally despite an extensive initial workup that was otherwise unrevealing. A review and discussion of the otolaryngological manifestations of CJD is presented. The otolaryngologist should be aware that CJD can present with otolaryngological manifestations, and with proper diagnosis extensive workups may be avoided.
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