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A case of cortical deafness and anarthria
Kimitaka Kaga1, Masako Nakamura, Yoshihiro Takayama
1Department of Otolaryngology, Graduate School of Medicine, University of Tokyo, Japan. kimikaga-tky@umin.ac.jp
Acta Oto-Laryngologica
|April 10, 2004
Summary
This case study details a rare instance of simultaneous progressive cortical deafness and anarthria. These symptoms may indicate early signs of frontotemporal dementia or progressive aphasia.
Area of Science:
- Neurology
- Audiology
- Neuroimaging
Background:
- Cortical deafness and anarthria typically occur independently.
- This case presents a unique co-occurrence of both conditions.
Observation:
- A 70-year-old woman presented with rapidly progressing hearing loss and articulation difficulties.
- Auditory tests showed elevated thresholds and poor speech discrimination, but normal otoacoustic emissions and auditory brainstem responses.
- Neurological examination revealed no other deficits, but brain imaging showed atrophy in auditory and language centers.
Findings:
- The patient experienced simultaneous progressive cortical deafness and anarthria, with preserved reading and writing abilities.
- Brain MRI revealed atrophy of the auditory cortex and Wernicke's area.
- PET scans indicated reduced glucose metabolism around the Sylvian fissures, suggesting neuronal dysfunction.
Implications:
- This case suggests that simultaneous cortical deafness and anarthria can be early indicators of neurodegenerative diseases.
- The findings challenge the traditional understanding of these conditions as independent entities.
- Further research is needed to explore the link between these symptoms and progressive aphasia or frontotemporal dementia.