Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jun 26, 2022; 10(18): 6333-6337
Published online Jun 26, 2022. doi: 10.12998/wjcc.v10.i18.6333
Creutzfeldt-Jakob disease presenting with bilateral hearing loss: A case report
Seunghee Na, Se A Lee, Jong Dae Lee, Eek-Sung Lee, Tae-Kyeong Lee
Seunghee Na, Department of Neurology, Incheon St. Mary’s Hospital, The Catholic University of Korea, Incheon 21431, South Korea
Se A Lee, Jong Dae Lee, Department of Otorhinolaryngology-Head and Neck Surgery, Soonchunhyang University Bucheon Hospital, Bucheon 14584, South Korea
Eek-Sung Lee, Tae-Kyeong Lee, Department of Neurology, Soonchunhyang University Bucheon Hospital, Bucheon 14584, South Korea
Author contributions: Lee ES conceived and designed the study; Lee TK provided supervision; Lee ES and Lee collected the data; Lee SN wrote the first draft of the manuscript; Lee SA and Lee JD edited and contributed to critical revision; and All authors read and approved the final version of the manuscript for submission.
Supported by National Research Foundation of Korea, No. 2019R1C1C1006539.
Informed consent statement: The authors declare that this single case report has been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Eek-Sung Lee, MD, PhD, Assistant Professor, Department of Neurology, Soonchunhyang University Bucheon Hospital, 170, Jomaru-ro, Bucheon 14584, South Korea.
Received: February 9, 2022
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 presenting symptom of sCJD, but hearing loss as an early manifestation is very rare.


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.

Keywords: Case report, Creutzfeldt-Jakob disease, Bilateral hearing loss, Diffusion-weighted imaging, Real-time quaking-induced conversion assay

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.