Cortical deafness is a rare form of sensorineural hearing loss caused by damage to the primary auditory cortex.

[1] Although patients appear and feel completely deaf, they can still exhibit some reflex responses such as turning their head towards a loud sound.

[10] Patients presenting with cortical hearing loss and no other associated symptoms recover to a variable degree, depending on the size and type of the cerebral lesion.

Patients whose symptoms include both motor deficits and aphasias often have larger lesions with an associated poorer prognosis in regard to functional status and recovery.

Any auditory signal, regardless if has been amplified to normal or high intensities, is useless to a system unable to complete its processing.

[4] Ideally, patients should be directed toward resources to aid them in lip-reading, learning American Sign Language, as well as speech and occupational therapy.

Advances in imaging techniques, such as MRI, greatly improved the diagnosis and localization of cerebral infarcts that coincide with primary or secondary auditory centers.

[10] Neurological and cognitive testing help to distinguish between total cortical deafness and auditory agnosia, resulting in the inability to perceive words, music, or specific environmental sounds.