Rapidly progressive sensorineural hearing loss due to sporadic Creutzfeldt-Jakob disease.

A patient developed several weeks of progressive bilateral sensorineural hearing loss, initially suspected to be an ischaemic stroke due to right parietal diffusion restriction on MRI. However, the persistence and progression of symptoms, including left upper extremity weakness, word deafness and cognitive decline, were inconsistent with a vascular event. Repeat imaging revealed expanding cortical ribboning without infarct evolution. Extensive workup ruled out stroke, autoimmune encephalitis, infection and seizure-related processes. Cerebrospinal fluid analysis demonstrated significantly elevated 14-3-3 and T-tau proteins, making sporadic Creutzfeldt-Jakob disease probable. This case illustrates how early prion disease can mimic stroke radiographically and clinically, underscoring the importance of reconsidering the diagnosis when neurological deficits progress rapidly and atypically.