Spinocerebellar ataxia type 3 (SCA3 or Machado-Joseph disease) is the second most common autosomal-dominant hereditary ataxia and often presents with ocular motor and vestibular impairment. The value of ocular motor testing for early disease detection, monitoring disease progression and as a potential treatment biomarker has been acknowledged in several studies....
For audiologists and vestibular specialists managing SCA3 patients, this review provides a useful reference for selecting quantitative ocular motor and vestibular assessment tools, but does not generate new evidence to change existing assessment protocols.
Standardising quantitative vestibular and ocular motor assessment in SCA3 is essential for tracking disease progression and evaluating future therapies in clinical trials.
- 01Systematic review of quantitative ocular motor and vestibular assessments in SCA3/Machado-Joseph Disease.
- 02Published in Cerebellum, a specialised peer-reviewed journal.
- 03SCA3 is the most common dominantly inherited ataxia worldwide.
- 04Findings could guide outcome measure selection for future SCA3 clinical trials.
- 05Highlights gaps in standardisation of vestibular measurement in this population.
Quantitative ocular motor and vestibular assessments can detect and characterise dysfunction in SCA3/Machado-Joseph Disease patients.
studypartially supported- PMID
- 42115447
- DOI
- 10.1007/s12311-026-02021-2.
- Journal
- Cerebellum
- Publication type
- review
- Evidence level
- 1a
- Population
- Patients with Spinocerebellar Ataxia Type 3 (SCA3 / Machado-Joseph Disease)
- Intervention
- Quantitative ocular motor and vestibular assessment methods
Primary outcomes
Characterisation and comparison of quantitative ocular motor assessment tools in SCA3; Characterisation and comparison of quantitative vestibular assessment tools in SCA3