OTOLARYNGOLOGICAL MANIFESTATIONS OF CREUTZFELDT-JAKOB-DISEASE

Creutzfeldt-Jakob disease (CJD) is one of the uniformly fatal spongifo rm encephalopathies that is characterized clinically by an unrelenting progression of myoclonus, dementia, and ataxia, Since many of these p atients will develop cerebellar abnormalities, some may present to the otolaryngologist with dizziness. Hearing loss, however, to our knowle dge, has not been reported. We describe a patient with CJD who present ed with hearing loss and vague symptoms of imbalance, and whose diseas e progressed rapidly and fatally despite an extensive initial workup t hat was otherwise unrevealing. A review and discussion of the otolaryn gological manifestations of CJD is presented. The otolaryngologist sho uld be aware that CJD can present with otolaryngological manifestation s, and with proper diagnosis extensive workups may be avoided.