Objective: To investigate the effectiveness of a digitized intensive home-based speech rehabilitation, SpeechATAX, in people with progressive hereditary ataxia.

Background: The loss of the ability to speak is a devastating and inevitable outcome of many hereditary ataxias and can lead to significant declines in quality of life through social isolation, underemployment and reduced ability to complete daily tasks (Gibilisco and Vogel, 2013). Evidence based therapies are available for however, the value of treatments for dysarthria in ataxia remains largely unknow (Vogel et al., 2014).

Method: The study employed a rater blinded randomised controlled trial design across 3 countries. Participants completed a 4-week intensive home-based rehabilitation for dysarthria (SpeechATAX), using a handheld tablet. SpeechATAX was based on principles of motor learning and neuroplasticity and targeted to improve intelligibility, vocal control and prosody. During the 4-week rehabilitation period, patients trained for 45 min per day, 5 days per week (20 days of active treatment). Subjects were assessed at 4 timepoints: 4 weeks prior to speech rehabilitation (baseline), immediately prior to the 4-week home-based speech rehabilitation (pre), immediately after the 4-week home-based speech rehabilitation (post), and 4 weeks after to speech rehabilitation (retention). Speech and voice samples were recorded with listener-based assessment performed to determine direct magnitude estimations (DME) of intelligibility. Objective acoustic analysis was also performed.

Results: 165 patients with progressive ataxia commenced treatment. Intelligibility increased post rehabilitation (p=0.035) when compared to baseline and delayed entry controls. Perceptually, changes driven by improvements in voice quality (p=0.0032), articulation of vowels (p=0.03), vowel duration (p=0.026), asthenia (p=0.0095). SARA speech item (p = 0·0004) and patient self report (p < 0·0001) also improved in the active treatment arm post therapy.

Conclusion: Intensive speech treatment improves intelligibilty in a progressive neurological disease. This was the largest RCT for dysarthria run to date.

References: Gibilisco, P., and Vogel, A. P. (2013). “Friedreich ataxia,” BMJ 347.

Vogel, A. P., Folker, J. E., and Poole, M. L. (2014). “Treatment for speech disorder in Friedreich ataxia and other hereditary ataxia syndromes,” Cochrane Database of Systematic Reviews 10.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/speechatax-a-rater-blinded-randomized-controlled-trial-of-intensive-home-based-biofeedback-therapy-for-dysarthria-in-progressive-ataxia/