Speech rehabilitation in Recessive Spastic Ataxia CharlevoixSaguenay ARSACS

Speech rehabilitation in Recessive Spastic Ataxia Charlevoix-Saguenay (ARSACS) Adam P Vogel 1, 2, 3, (Ph. D), Natalie Rommel (MSc) 4, Matthis Synofzik (MD) 1, 3 1 Department of Neurodegeneration, Hertie Institute for Clinical Brain Research, University of Tübingen, Germany. 2 Centre for Neuroscience of Speech, The University of Melbourne, Victoria, Australia, 3 German Research Center for Neurodegenerative Diseases (DZNE), University of Tübingen, Germany. 4 Therapie. Zentrum, Universitätsklinikum Tübingen, Germany vogela@unimelb. edu. au Objective: To improve speech intelligibility (ability to be understood) in people with dysarthria resulting from Autsomal Recessive Spastic Ataxia Charlevoix-Saguenay (ARSACS) using a biofeedback driven intensive home based speech rehabilitation program compared with existing care. Background The loss of the ability to speak is a devastating and inevitable outcome of many neurodegenerative diseases. It results in daily disadvantage, stigmatisation, social marginalisation and underemployment. Disordered speech is an inevitable consequence of hereditary ataxias. Methods We have designed a home-based, intensive four-week speech exercise programme designed to improve speech in people with hereditary ataxia. The treatment protocol is based on principles of motor learning and neuroplasticity with a focus on improving speech intelligibility and vocal control. Exercises and feedback were created to enhance self-monitoring and include computer based aural, visual and results feedback and self-management (see Figure 1 for example of stimuli and feedback delivery). Eight people with Autosomal Recessive Spastic Ataxia of Charlevoix. Saguenay [ARSACS] have completed the study. Efficacy was measured by an expert listener blinded to time-point rating intelligibility of speech samples from the study participants using Direct Magnitude Estimation. (DME). DME dictates that samples are compared to a pre-identified anchor. Figure 1: Template of visual, aural and performance feedback Results In these 8 participants, mean (SD) intelligibility was 117. 1 (19. 3) points at baseline and 128. 8 (14. 6) 4 -weeks postbaseline with Pearson’s correlation coefficient of 0. 69. The relative (%) increase in speech intelligibility from baseline to post-treatment was statistically significant (p=0. 014) using a one-sided paired t-test on base-2 log-transformed outcome data. See Figure 2). Relative improvements from baseline in intelligibility of 510% represent clinically meaningful change in participants’ speech including significant change in voice quality and naturalness in a variety of tasks (eg. conversation). We saw individual relative changes in intelligibility between -7 -33% in our data. On an individual level, 4/8 (50%) patients responded to treatment, defined as baseline to posttreatment intelligibility improvement of ≥ 10%. Conclusions Our preliminary speech rehabilitation data has yielded promising results. Pilot data suggests that even mild dysarthria improves with our intervention in most patients. The relative improvement is typically larger for those individuals with more severe dysarthria. Figure 2: Intelligibility at baseline & post treatment FUNDING ACKNOWLEDGMENT Fondation de l'Ataxie Charlevoix-Saguenay (Canada) | Alexander von Humboldt Fellowship (Germany) | National Health & Medical Research Council, Australia 160 140 120 100 80 60 40 20 0 ARSACS Baseline ARSACS Post tx ARSACS