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Remediation of Perceptual Deficits in Progressive Auditory Neuropathy: A Case Study
Gary Rance1, Dani Tomlin1, Eppie M Yiu2,3,4
1Department of Audiology and Speech Pathology, The University of Melbourne, Carlton, VIC 3053, Australia.
Journal of Clinical Medicine
|April 13, 2024
Summary
Auditory neuropathy (AN) worsened over time, but a remote-microphone device improved listening skills and neuroplasticity in a child with Charcot-Marie-Tooth disease.
Area of Science:
- Neuroscience
- Audiology
- Genetics
Background:
- Auditory neuropathy (AN) is a hearing disorder impacting the VIIIth cranial nerve and central auditory pathways.
- Progressive AN can occur in neurodegenerative diseases, causing deafferentiation and desynchronization.
- Axonal neuropathy, like Charcot-Marie-Tooth disease, can be associated with progressive AN.
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01:01Neuroplasticity
Neuroplasticity reflects the brain's remarkable capacity to adapt and evolve, responding dynamically to learning, experiences, or injury by reorganizing its neural circuitry. This reorganization involves creating new neural connections and refining old ones through a series of biological processes that contribute to the brain's lifelong development and adaptability.
01:17Auditory Perception
The auditory system is essential for sound perception, utilizing various critical structures. When sound waves enter the outer ear, they travel through the ear canal and cause the eardrum to vibrate. These vibrations are then transmitted to the middle ear, where three tiny bones – the malleus, incus, and stapes – amplify the sound. This amplification is crucial, as it ensures that the sound vibrations are strong enough to be conveyed to the inner ear. These vibrations then reach the cochlea, a...