Cortical Deafness
A central auditory processing disorder resulting in profound hearing loss despite intact peripheral auditory structures.
Full Definition
Cortical deafness is a rare condition where patients experience severe to profound hearing impairment due to bilateral damage to the auditory cortex, despite having normal peripheral hearing structures and brainstem auditory pathways. Unlike peripheral deafness, brainstem auditory evoked responses remain normal, but patients cannot perceive or process auditory information meaningfully. This condition typically results from bilateral temporal lobe strokes, carbon monoxide poisoning, or other causes of bilateral cortical damage. Patients may retain some automatic auditory responses (such as startle reflexes) but lose conscious sound perception. Rehabilitation focuses on alternative communication methods and environmental safety modifications rather than traditional auditory rehabilitation approaches.
Usage
Usage note: Distinguish from peripheral hearing loss through audiological testing patterns.
In Context
- "The patient developed cortical deafness following bilateral temporal lobe infarctions, requiring alternative communication strategies." — Audiological evaluation
- "Cortical deafness was confirmed by normal brainstem auditory evoked responses despite profound hearing impairment." — Neurological consultation