Auditory neuropathy in mice and humans with Friedreich ataxia.

Gary RancePeter CarewLeon WinataPhillip SaleMartin DelatyckiDavid Sly

Published in: Annals of clinical and translational neurology (2023)

This study found degenerative changes in auditory structure and function in YG8Pook/J mice, indicating that auditory measures in these animals may provide a model for testing Friedreich ataxia treatments. In addition, auditory steady-state response findings in a clinical population suggested that these scalp-recorded potentials may serve as an objective biomarker for disease progress in affected individuals.

Keyphrases

working memory
hearing loss
high fat diet induced
early onset
type diabetes
insulin resistance
adipose tissue