Friedreich's ataxia, other hereditary ataxias Nervous System Diseases Early-onset cerebellar ataxia
Conditions
Interventions
Sponsors
Eligibility
Inclusion criteria
Inclusion criteria: 1. Confirmed diagnosis of hereditary ataxia, primarily Friedreich's ataxia 2. Presence of a speech impairment 3. Ability to perform assessment and treatment tasks 4. No uncorrected hearing loss 5. Availability of appropriate technology to record voice and operate Skype
Exclusion criteria
Exclusion criteria: 1. Ataxia due to non-hereditary reasons 2. Lack of speech impairment 3. Inability to perform assessment or treatment tasks 4. Significant hearing loss that would impact on speech performance 5. Unable to participate in Skype conversations
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Participants will be assessed before and after treatment. Multiple baseline assessments will be taken (assessments 1 & 2, approximately 2 weeks apart). Performance will then be re-assessed immediately following (assessment 3) and 6-8 weeks after the end of treatment (assessment 4). 1. Maximum loudness on prolonged /a/ and mean loudness of a reading passage measured acoustically in decibel at assessments 1, 2, 3 & 4 2. Voice quality of prolonged /a/ evaluated acoustically (shimmer, jitter, harmonics to noise ratio) at assessments 1, 2, 3 & 4 3. Articulation and speech rate of a reading passage assessed acoustically in syllables per minute at assessments 1, 2, 3 & 4 4. Intelligibility of a reading passage measured perceptually on a 9-point scale according to Dobinson (2007) at assessments 1, 2, 3 & 4 | — |
Secondary
| Measure | Time frame |
|---|---|
| Participants will be assessed before and after treatment. Multiple baseline assessments will be taken (assessments 1 & 2, approximately 2 weeks apart). Performance will then be re-assessed immediately following (assessment 3) and 6-8 weeks after the end of treatment (assessment 4). 1. Communication participation assessed with the Communication Participation Item Bank short form (CPIB, Baylor et al. 2012) at assessments 1 & 3 2. Impact of the voice problem assessed with the Voice Handicap Index ( VHI, Jacobson et al. 1997) at assessments 1 & 3 3. Levels of communication impact, confidence and engagement evaluated with a self-constructed, unpublished questionnaire on a Visual Analogue Scale, and baseline and exit semi-structured interviews at assessments 1 & 3 4. Participants’ evaluation of therapy programme and Skype delivery established during exit interview at assessment 3 5. Fatigue measured with the Fatigue Severity Scale (FSS, Krupp et al, 1989 ) at assessments 1 & 3 | — |
Countries
Switzerland, United Kingdom