Abstract Details

Title Rapid Bilateral Sensorineural Hearing Loss in a Patient with Creutzfeldt-Jakob Disease

Topic Infections/AIDS/Prion Disease

Presentation(s) P03 - (-)

Poster/Presentation
Number 251

Objective

Background BACKGROUND: CJD is a prion disease with uniformly fatal outcome. The classic symptoms include rapid cognitive decline, cerebellar dysfunction, myoclonus, seizures, motor deficits, extrapyramidal signs and visual disturbances. Hearing loss is not universally recognized as a feature of CJD and so far, only three cases in the literature have described it as the initial presenting symptom.

Design/Methods DESIGN/METHODS: A 67 year-old man with no significant medical history, presented with a two-month history of rapidly progressive bilateral hearing loss and tinnitus followed by cognitive decline. During his hospital stay, he displayed myoclonus and ataxia.

Results RESULTS: The patient's brain MRI revealed cortical ribbon hyperintensity along the left temporal, occipital, parietal lobes on diffusion weighted imaging, and hyperintensity of the bilateral basal ganglia and posterior thalami on FLAIR imaging. CSF analysis was positive for protein 14-3-3. Multiple EEGs were unremarkable. Otological evaluation revealed bilateral sensorineural hearing loss with preserved middle ear function. Patient's hearing never improved and he eventually passed a month from initial presentation.

Conclusions CONCLUSIONS: Sensorineural hearing loss can be a presenting complaint of patients with CJD. Involvement of brainstem auditory nuclei could be the likely underlying pathology. CJD and its workup should be considered in patients with rapidly progressive hearing loss in the appropriate clinical setting.

Authors/Disclosures
Ahmad Riad Ramadan, MD (Henry Ford Hospital) PRESENTER	Dr. Ramadan has received publishing royalties from a publication relating to health care. Dr. Ramadan has received publishing royalties from a publication relating to health care.
Keyur Patel, MD	No disclosure on file
Chandan B. Mehta, MD (Henry Ford Hospital)	Dr. Mehta has nothing to disclose.
	No disclosure on file
Nelson J. Maldonado, MD (Neuro Quito)	No disclosure on file
Panayiotis Mitsias, MD (Henry Ford Hospital, Neurology)	The institution of Dr. Mitsias has received research support from European Union.
S. A. Josephson, MD, FAAN (UCSF)	Dr. Josephson has received personal compensation in the range of $50,000-$99,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for JAMA Neurology. Dr. Josephson has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for McGraw Hill---Harrison's Principles of Internal Medicine. Dr. Josephson has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for various legal firms. The institution of Dr. Josephson has received research support from NINDS. Dr. Josephson has received publishing royalties from a publication relating to health care. Dr. Josephson has received publishing royalties from a publication relating to health care.
S. A. Josephson, MD, FAAN (UCSF)	Dr. Josephson has received personal compensation in the range of $50,000-$99,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for JAMA Neurology. Dr. Josephson has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for McGraw Hill---Harrison's Principles of Internal Medicine. Dr. Josephson has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for various legal firms. The institution of Dr. Josephson has received research support from NINDS. Dr. Josephson has received publishing royalties from a publication relating to health care. Dr. Josephson has received publishing royalties from a publication relating to health care.

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