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Rance G et al. – The results suggest that auditory pathway abnormality is a relatively common consequence of FRDA. Regular auditory evaluation should therefore be part of the management regime for all affected individuals. This assessment should include both ABR testing, which can provide insights into the degree to which auditory neural activity is disrupted, and some functional measure of hearing capacity such as speech perception assessment, which can quantify the disorder and provide a basis for intervention.

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