Objective: To identify a subset of acoustic markers of speech that objectively describe listener perceptions, disease severity and speech related quality of life in Friedreich ataxia.

Background: Dysarthria is a key aspect of Friedreich ataxia (FA). Speech changes as disease progresses and in response to both behavioral and pharmacological treatment. Current assessment practices rely on clinical impressions and listener based examinations of speech which are subjective and have poor inter/intra-rater reliability. Objective evaluation of speech (acoustic analysis) can yield valuable insights into how disease progresses over time, and how individuals respond to therapy.

Method: We applied a wide range of known and novel speech signal processing algorithms to a large database (1000+ recordings from >130 individuals with FA over a 10-year period). We used principled variable selection methods to determine a subset of the extracted markers, which was presented into a classifier using a standard supervised learning setup to map onto (a) listener perceptions of intelligibility and naturalness, (b) a disease severity scale (Friedreich Ataxia Rating Scale) and (c) speech related quality of life scale (sub-domain of the Friedreich Ataxia Impact Scale).

Results: We have assessed the resulting models using 10-fold cross-validation with 100 iterations for statistical confidence. Findings suggest there are specific objective markers of speech that change over time and reflect to some degree other clinical features of the disease.

Conclusion: Our ability to develop objective features of dysarthria require large longitudinal datasets combined with a variety of clinical endpoints. Here we describe an important advance in our ability to quantify this meaningful outcome measure. Wholistic speech descriptors will better serve investigations seeking to capture meaningful metrics of change resulting from progression or treatment.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/alignment-of-objective-markers-of-speech-with-listener-based-judgements-and-disease-severity-in-friedreich-ataxia/