Initially described in the late 19th century by Wernicke and Friedlander, cortical deafness is a rare form of sensorineural hearing loss resulting from bilateral insults to Heschl’s gyrus (transverse temporal gyrus).  The similarity of the clinical presentation with other forms of communication disorders like auditory and verbal agnosia makes the diagnosis challenging. To our knowledge, fewer than fifteen cases of cortical deafness have been reported in the literature.  We report an older gentleman who presented with sudden onset complete hearing loss resulting from acute infracts in the bilateral Primary Auditory Cortex (PAC)

76-year-old Caucasian male with a history of mild bilateral hearing loss, prior right occipital ischemic cerebrovascular accident, myocardial infarction, and hypertension presented to the emergency with sudden onset complete hearing loss and difficulty communicating after waking up. The family reported some confusion in answering questions but thought it could be secondary to hearing impairment.

Initial examination demonstrated complete hearing loss and agraphia without alexia or anomia. Co-ordination and sensory examination were unremarkable. Left upper and lower extremity had chronic weakness secondary to a neck injury in his late twenties. Computed Tomography (CT) of the head without contrast was unremarkable for any acute intracranial abnormality. CT Angiography of the head and neck showed mild atherosclerotic changes without significant hemodynamic stenosis or occlusion. Subsequent MRI brain demonstrated diffusion restricted lesions in the bilateral temporal lobes, bilateral frontal lobes, right lateral parietal lobe, left parietotemporal junction. A 2D Echocardiogram was positive for left ventricular apical aneurysm, which was assumed to be the source of the embolic infarcts. 

Cortical deafness is an exceedingly rare form of complete sensory-neural hearing loss. Acute ischemic stroke secondary to an embolic cause should be considered for acute presentations of bilateral hearing loss and should be evaluated for acute stroke management including tPA .