Journal
CORTEX
Volume 36, Issue 1, Pages 71-79Publisher
MASSON DIVISIONE PERIODICI
DOI: 10.1016/S0010-9452(08)70837-2
Keywords
deaf hearing; cortical deafness; cerebral deafness; auditory awareness; implicit awareness; auditory agnosia
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We describe a patient with the rare disorder of total deafness caused by a bilateral lesion in the temporal lobes and lesions in the central pontine area. Although she displayed no voluntary ability to detect or localize or identify sounds and denied hearing them when asked in writing, she retained some ability to respond reflexively to sounds. When attempts were made to restore awareness of sounds and/or voluntary responses to sounds by drawing her attention to her appropriate orienting head movements her performance improved and she began to respond successfully in a forced-choice paradigm. However, even when she became confident at detecting and localizing sounds she remained densely agnosic to their meaning. Her condition of deaf-hearing bears many similarities to that of blindsight.
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