The objective was to identify a sensitive marker of disease progression in Friedreich's ataxia. This study prospectively evaluated speech, voice, and oromotor function in 40 patients at two time points. The mean disease duration was 20.8 ± 9.8 years and mean SARA score 23.7 ± 8.6 at baseline. Oral motor mobility, assessed by a combination of movements of the face, eyes, cheeks, lips, and tongue, decreased significantly after 1 year (P < 0.0001). The standardized response mean over 12 months was considered as large for oral mobility (1.26) but small for SARA (0.12). Oral mobility could therefore be a sensitive marker in therapeutic trials, especially in later stage, non-ambulatory individuals.

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