Published online Jun 26, 2022. doi: 10.12998/wjcc.v10.i18.6333
Peer-review started: February 9, 2022
First decision: March 23, 2022
Revised: March 24, 2022
Accepted: April 22, 2022
Article in press: April 22, 2022
Published online: June 26, 2022
Sporadic Creutzfeldt-Jakob disease (sCJD) is a prion disease characterized as a fatal transmissible neurodegenerative disorder. Dizziness is often the first pre
A 76-year-old man presented with bilateral sudden hearing impairment and dizziness for 10 d. He was taking medications for hypertension and diabetes. He denied any difficulty with activities of daily living or hearing impairment before the onset of symptoms. Pure tone audiometry showed bilateral severe hearing impairment. Brain magnetic resonance imaging (MRI) and laboratory tests were within normal limits. Given his diagnosis of sudden sensory hearing loss, the patient received corticosteroid treatment but it was ineffective. Two weeks later, he complained of aggravated gait impairment, disorientation, and cognitive impairment. Repeat brain MRI showed diffuse cortical high signal intensities on diffusion-weighted imaging. In cerebrospinal fluid analysis, the real-time quaking-induced conversion assay was positive, and 14-3-3 protein was detected in the by western blotting. Considering all the data, we diagnosed probable sCJD, and the patient’s symptoms rapidly progressed into akinetic mutism.
For patients with abrupt bilateral hearing impairment, especially in the elderly, various differential diagnoses, including sCJD, should be considered.
Core Tip: Hearing impairment as an initial manifestation of Creutzfeldt-Jakob disease is rare. However, it should be regarded as a differential diagnosis in an elderly patient with bilateral hearing impairment unresponsive to standard corticosteroid treatment. Repeat brain imaging including diffusion-weighted imaging and cerebrospinal fluid analysis will be helpful for diagnosis.