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Cortical deafness: a longitudinal study

L J Hood1, C I Berlin, P Allen

  • 1Kresge Hearing Research Laboratory of the South, Department of Otorhinolaryngology and Biocommunication, Louisiana State University Medical Center (LSUMC), New Orleans 70112.

Journal of the American Academy of Audiology
|September 1, 1994
PubMed
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This study details a rare case of cortical deafness due to temporal lobe absence. Despite normal peripheral hearing, the patient experienced profound auditory processing deficits, emphasizing comprehensive audiological evaluation.

Area of Science:

  • Neuroscience
  • Audiology
  • Neurology

Background:

  • Bilateral temporal lobe absence can lead to cortical deafness.
  • Cortical deafness affects auditory processing despite intact peripheral hearing.

Observation:

  • A patient with MRI-confirmed bilateral temporal lobe absence presented with cortical deafness.
  • Physiologic measures showed normal peripheral hearing but abnormal middle latency and cortical evoked potentials.
  • The patient exhibited speech characteristics of profound hearing loss and could not understand spoken language.

Findings:

  • Pure-tone thresholds varied from normal to moderate hearing loss over 20 years.
  • Auditory Brainstem Responses (ABRs) were normal, but middle latency and cortical evoked potentials were consistently abnormal.

Related Experiment Videos

  • The patient was taught American Sign Language due to inability to communicate auditorily.

    • Implications:

    • This case underscores the necessity of employing diverse audiological assessments to detect auditory system abnormalities at multiple levels.
    • Comprehensive audiological testing is crucial for diagnosing and managing complex auditory processing disorders.
    • Understanding the auditory pathway's central processing is vital for effective communication strategies.