Auditory neuropathy in mice and humans with Friedreich ataxia

Rance, G., Carew, P., Winata, L., Sale, P., Delatycki, M. and Sly, D. (2023), Auditory neuropathy in mice and humans with Friedreich ataxia. Ann Clin Transl Neurol. https://doi.org/10.1002/acn3.51777 

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.