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

The importance of neural processing of temporal cues by the auditory system has been stressed by the discovery of auditory neuropathy spectrum disorders (ANSD) (Starr et al., 1996; Rance and Starr, 2015), in which disrupted auditory-nerve conduction leads to a characteristic pattern of hearing disorder. One striking complaint of ANSD patients is the presence of impaired speech understanding out of proportion with what the auditory sensitivity would predict, particularly in the presence of noise (Kraus et al., 2000; Zeng and Liu, 2006).

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