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Updated: Sep 24, 2025

Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
Bilateral hearing loss as an initial presentation of Creutzfeldt-Jakob disease
Janaina Mariana de Araujo Miranda Brito-Marques1, Eduardo Sousa de Melo1,2, Fabíola Lys de Medeiros1
1Department of Neurology, Hospital Universitário Oswaldo Cruz, Universidade de Pernambuco - Recife, PE, Brazil.
Abstract:
We reported a case of a 61-year-old male patient with anacusis, cerebellar syndrome, myoclonus, and frontal signs. The brain magnetic resonance imaging showed bilateral striated hyperintensity of the fluid-attenuated inversion recovery and restricted diffusion in the diffusion-weighted imaging and hypointense areas corresponding to the apparent diffusion coefficient in the cerebral cortex. The autopsy revealed positive immunohistochemistry for the PrPSc protein. Creutzfeldt-Jakob disease presenting with hearing loss is unusual.
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