Dysarthria in children and adults with ataxia telangiectasia.
Stefanie J G Veenhuis, Nienke J H van Os, Marjo H J C van Gerven and 4 others
PMID 33521952WHAT IT FOUND
All participants with ataxia telangiectasia had dysarthria.
Most had mild to mild/severe speech impairment, and all could communicate orally. Children needed to repeat words more often than adults.
Key findings
01Dysarthria was present in all 22 participants, and oral communication remained possible despite it.
02Most participants needed to repeat their words to be understood, and children needed to repeat words more often than adults (12 out of 15 children; 3 out of 7 adults).
03All children showed predominantly uncontrolled dysarthric components, producing monotonous, unstable, slow, hypernasal, and chanted speech. All adults showed ataxic dysarthria with some strained components.
STILL TO COME
How it was doneWhat they foundWhat it means for SLPs
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What it does not show
The analysed sample was only 22 participants, and the disease is rare, so findings may not generalise widely. Classic and variant ataxia telangiectasia were unevenly distributed between children and adults, which complicates child-adult comparisons. Some maximum performance tasks were not completed because of coughing, laughing, breathing, or limited cooperation, especially in young children. Only 20 Intelligibility in Context Scale questionnaires were completed. Intelligibility was rated by parents or partners, who may be more familiar with the participant's speech than unfamiliar listeners. The study required Dutch or French understanding and excluded children under 5 years, so it does not describe very young children or other language groups. It is a baseline descriptive cohort, not a treatment trial, so it cannot show that any therapy improves speech.
Declared interests
Funding was provided by the A-T Children's Project.
The easy way to misread this
Do not read these speech features as proof that a treatment works. This study described baseline dysarthria and did not test the therapy advice.