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Published on: September 7, 2022
Auditory perception in individuals with Friedreich's ataxia
Gary Rance1, Louise Corben, Elizabeth Barker
1Department of Otolaryngology, The University of Melbourne, Parkville, Vic., Australia. grance@unimelb.edu.au
Audiology & Neuro-Otology
|November 7, 2009
Summary
Friedreich's ataxia (FRDA) often causes auditory processing disorders, impacting temporal and spectral cue perception. Regular auditory evaluations, including ABR and speech tests, are recommended for FRDA management.
Area of Science:
- Neuroscience
- Genetics
- Audiology
Background:
- Friedreich's ataxia (FRDA) is a genetic disorder characterized by progressive axonal degeneration, particularly affecting sensory nerves.
- Auditory system involvement is a recognized, yet often under-investigated, feature of FRDA.
Purpose of the Study:
- To investigate auditory perception and processing abilities in individuals diagnosed with Friedreich's ataxia.
- To correlate auditory processing deficits with speech understanding in FRDA patients.
Main Methods:
- Evaluated auditory processing using tasks assessing temporal and spectral cue perception in 14 FRDA subjects.
- Included control groups of healthy individuals and those with sensorineural hearing loss.
- Correlated auditory findings with open-set speech understanding and utilized auditory brainstem response (ABR) testing.
Main Results:
- Nine out of 14 FRDA individuals (64%) exhibited auditory processing disorder.
- Impaired detection of rapid signal changes (gap, amplitude modulation) and disrupted neural activity (ABR) were observed.
- Speech understanding deficits were linked to temporal processing impairments, specifically in consonant voice onset time and vowel duration.
Conclusions:
- Auditory pathway abnormalities are common in Friedreich's ataxia.
- Routine auditory assessment, incorporating ABR and speech perception tests, is crucial for FRDA management and intervention planning.
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