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Progressive neural hearing impairment: case report

J E Widen1, J A Ferraro, S E Trouba

  • 1Department of Hearing and Speech, University of Kansas Medical Center, Kansas City 66160-7605, USA.

Journal of the American Academy of Audiology
|May 1, 1995
PubMed
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A patient with severe speech understanding loss, despite normal hearing sensitivity, showed absent auditory brainstem responses but present otoacoustic emissions. This case highlights how auditory evoked potentials (AEPs) can reveal specific auditory pathway pathologies.

Area of Science:

  • Neuroscience
  • Audiology
  • Otoacoustic Emissions

Background:

  • Auditory pathway disorders can significantly impact speech comprehension.
  • Otoacoustic emissions (OAEs) and auditory evoked potentials (AEPs) are key diagnostic tools in audiology.
  • Differentiating pathologies within the auditory system requires comprehensive electrophysiological testing.

Observation:

  • A patient presented with a 20-year history of progressive speech understanding decline, nearing cochlear implant candidacy.
  • Pure-tone hearing sensitivity loss did not correlate with the severity of speech comprehension deficits.
  • Otoacoustic emissions (OAEs) were present and normal, while auditory brainstem and middle latency responses were absent.

Findings:

  • The auditory N1-P2 complex of long-latency AEPs was present despite the absence of earlier AEP components.

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  • This dissociation in AEPs suggests a specific site of lesion within the auditory pathway.
  • Otoacoustic emissions provided crucial information about cochlear function, independent of central auditory processing deficits.

    • Implications:

    • Otoacoustic emissions are valuable for localizing auditory lesions, particularly when central auditory pathways are affected.
    • Pathologies can manifest selectively in specific epochs of auditory evoked potentials, affecting some components while sparing others.
    • This case underscores the importance of utilizing a multi-test approach for accurate diagnosis of complex auditory processing disorders.