title: Cortical deafness: a longitudinal study.
abstract: We have studied a patient with MRI-confirmed bilateral absence of considerable portions of her temporal lobes resulting in cortical deafness. Although physiologic measures demonstrate normal peripheral hearing sensitivity, this patient's speech has the inflection and prosodic characteristics associated with profound peripheral hearing loss, and she is unable to understand spoken communication. Behaviorally obtained pure-tone thresholds taken over nearly 20 years range from normal to moderate hearing loss with normal middle ear muscle reflexes and normal ABRs; however, we consistently found abnormal middle latency and cortical evoked potentials. Because of her total inability to communicate auditorily, this patient was ultimately taught American Sign Language and educated at the Louisiana School for the Deaf. This rare case highlights the importance of using multiple audiologic measures sensitive to abnormalities at various levels of the auditory system.
authors.affiliation: Kresge Hearing Research Laboratory of the South, Department of Otorhinolaryngology and Biocommunication, Louisiana State University Medical Center (LSUMC), New Orleans 70112.
authors.forename: L J
authors.initials: LJ
authors.lastname: Hood
authors.forename: C I
authors.initials: CI
authors.lastname: Berlin
authors.forename: P
authors.initials: P
authors.lastname: Allen
mesh_terms: D000328:Adult; D017668:Age of Onset; D001300:Audiometry, Evoked Response; D001301:Audiometry, Pure-Tone; D001302:Audiometry, Speech; D001303:Auditory Cortex / Q000000981:diagnostic imaging / Q000503:physiopathology*; D003051:Cochlea / Q000502:physiology; D003638:Deafness / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology*; D004558:Electric Stimulation; D016057:Evoked Potentials, Auditory, Brain Stem; D005260:Female; D006801:Humans; D007806:Language Disorders / Q000175:diagnosis / Q000209:etiology; D007808:Language Therapy; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D011859:Radiography; D012022:Reflex, Acoustic; D012813:Sign Language; D013067:Speech Perception; D013069:Speech Reception Threshold Test