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Genetic Hearing Loss Affects Cochlear Processing
Cris Lanting1, Ad Snik1, Joop Leijendeckers1
1Department of Otorhinolaryngology, Radboud University Medical Centre, Donders Institute for Brain, Cognition and Behaviour, Postbus 9101, 6500 HB Nijmegen, The Netherlands.
Genes
|November 11, 2022
Summary
Genetic hearing loss impairs cochlear processing, affecting speech recognition in noise. Loudness growth and spectral resolution are key biomarkers for understanding these genetic-related cochlear malfunctions.
Area of Science:
- Audiology
- Genetics
- Otoacoustic Emissions
Background:
- Hereditary hearing loss involves genetic defects impacting cochlear sound processing.
- Speech recognition in noise is a critical measure of auditory function.
Purpose of the Study:
- To investigate the relationship between genetic hearing loss and speech recognition in noise.
- To identify cochlear processing aspects that correlate with speech understanding in genetically hearing-impaired individuals.
Main Methods:
- Data from nine patient groups with specific genetic hearing loss types were analyzed.
- Cochlear site-of-lesion was inferred from animal studies.
- Speech recognition in noise scores were correlated with psychophysical measurements of supra-threshold cochlear processing.
Main Results:
- Differences in speech perception in noise among patient groups were linked to cochlear processing factors.
- Genetic-related cochlear malfunctioning was associated with impaired speech recognition in noise.
- Loudness growth and spectral resolution emerged as significant predictors of speech understanding in noise.
Conclusions:
- Speech recognition in noise is demonstrably affected by genetics-related cochlear dysfunction.
- Loudness growth and spectral resolution serve as valuable biomarkers for assessing speech understanding deficits in genetic hearing loss.
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