Speech treatment for spinocerebellar ataxia and Friedreich ataxia

INTERVENTION: Treatment involved a behavioural intervention designed to improve speech intelligibility and quality. Materials: Patients are provided with a laptop computer with treatment software installed, head mounted USB microphone, treatment manual. Procedures: The program specifically targets prosody, vocal control and over‐enunciation with the aim of improving overall speech intelligibility and confidence when communicating. It is based on principles of motor learning and neuroplasticity relating to practice conditions and bio‐feedback. Phase I: Teaching and Establishment of treatment components The clinician introduces the goals and content of the therapy. Patients learn the skills needed to undertake the program and implementation of the exercises. Using the computer program will be reviewed and practiced together on the first visit. Phase II: Instatement and Rehabilitation A: Visual feedback: Visual feedback is provided through the real time loudness display. Patients can monitor the stability or variability of their loudness while speaking. For example, if patients cannot see any colour in the visual display they need to increase the loudness of their speech. Similarly, a stable production is required for the long vowel task, meaning the visual display should remain at a constant level throughout the production. The feedback given by the interface supports the patient by training self‐awareness. Loudness is represented visually, providing an alternative mode of feedback other than listening. Demonstrate and encourage the use of these feedback options. B: Aural (listening) feedback: Patients are prompted to record parts of their speech each day. They are then required to listen to their recorded sample from the previous day. Listening feedback is designed to enhance self‐monitoring and help the speaker identify aspects of their speech that need improvement. Listening to speech can be upsetting for some patients but it is a vital component of therapy. Delayed aural feedback is important for the development of self‐monitoring skills by providing an opportunity to hear their performance, identify what worked and what went wrong and set some goals for the day. C: Outcome feedback: Patients are provided with immediate and objective feedback of their performance. Three pieces of information are derived from the recorded samples and compared against the previous days’ scores: 1. Duration, which is important for the long vowel task; 2‐3. Loudness and pitch variation, both of which are important for the vowel and connected speech tasks. This information is designed to enhance the patient’s understanding of their performance and to provide a benchmark on which to compare earlier productions. Summary figures are provided at the end of each day after tasks are completed. The clinician will be able to plot your patients’ progress – highlighting the gains made during therapy. Therapy is completed in the home by the patient over 20 sessions within one month. Each session consists of excercises aiming to improve vocal quality and control, articulation, prosody and intelligibility. Self‐evaluation skills are refined by the use of visual, aural and performance feedback and personalised problem‐solving strategies. Clinicians monitor patient progress and provide support during this stage through weekly skype or phone calls. CONDITION: Dysarthria Friedreich ataxia Spinocerebellar ataxia PRIMARY OUTCOME: Intelligibility as examined by blinded raters using direct magnitude estimation Naturalness as examined by blinded raters using direct magnitude estimation INCLUSION CRITERIA: Genetically confirmed diagnosis of spinocerebellar ataxia (any type) or genetically confirmed diagnosis of Friedreich ataxia Who: Treatment is coordinated by the treating clinician and completed in the home or clinic by the patient. Treating clinicians are trained speech‐language pathologists with a minimum of 5 years' experience; Mode of delivery: The first treatment session is conducted face to face. Each subsequent treatment day is conducted using the computer software. Adherence and progress is monitored weekly by the treating clinician via skype or telephone. Treatment is provided individually. Number of times: The intervention will be delivered 20 times over a 30‐day period. Patients are required to complete therapy 5 days out of every 7 for the period of the study. Treatment sessions take approximately 45 minutes to complete. 20 x 45 minute sessions 5 times a week for 4 weeks. SECONDARY OUTCOME: Acoustic analysis of speech: mean pause length Acoustic analysis of speech: speech rate calculated by dividing syllables per second Acoustic analysis of voice: harmonics to noise ratio using praat Overall disease severity using the Scale for the assessment and rating of ataxia (SARA) Speech‐related quality of life using the Dysarthria Impact Profile