Rapid exhaustion of auditory neural conduction in a prototypical mitochondrial disease, Friedreich ataxia

Fabrice Giraudet, Perrine Charles, Thierry Mom, Odile Boespflug-Tanguy, Alexandra Dürr, Paul Deltenre, Paul Avan, Clinical Neurophysiology, Available online 27 March 2018, ISSN 1388-2457, doi:10.1016/j.clinph.2018.03.005.

In FRDA, the assumption of stationarity used for extracting standard auditory brainstem-evoked responsesis invalid. The preservation of early split-ABRs indicates no short-term dyssynchrony of action potentials. A large decrease in conduction velocity along auditory neurons occurs within seconds, attributed to fast energetic failure.

 Rapid exhaustion of auditory neural conduction in a prototypical mitochondrial disease, Friedreich ataxia