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Updated: Jan 27, 2026
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Published on: July 2, 2013
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Prominent auditory deficits in primary progressive aphasia: A case study
Rene L Utianski1, Joseph R Duffy1, Heather M Clark1
1Department of Neurology, Mayo Clinic, Rochester, MN, USA.
Summary
This case study details a rare instance of auditory agnosia within primary progressive aphasia (PPA), a condition typically linked to stroke. The findings highlight corticobasal degeneration as a potential cause.
Area of Science:
- Neurology
- Neuroscience
- Linguistics
Background:
- Aphasia involves language deficits, while auditory verbal agnosia presents disproportionate spoken language comprehension issues.
- Auditory agnosia, distinct from pure word deafness, involves impaired processing of both speech and non-speech sounds.
Observation:
- A 65-year-old woman exhibited progressive aphasia and apraxia of speech with a prominent auditory deficit.
- Her symptoms included disproportionate difficulty understanding spoken versus written language.
- Clinical presentation suggested auditory agnosia within a mixed primary progressive aphasia.
Findings:
- Neuroimaging revealed progressive bilateral hypometabolism in language and motor areas.
- Autopsy confirmed pathological lesions consistent with corticobasal degeneration.
- This case links auditory agnosia to corticobasal degeneration in a primary progressive aphasia context.
Implications:
- Auditory agnosia, though rare, can manifest in progressive neurological disorders.
- Corticobasal degeneration should be considered in cases of primary progressive aphasia with prominent auditory processing deficits.
- Further research is needed to understand the neurobiological underpinnings of auditory agnosia in PPA.
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