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Patient-Reported Visual Function in Ataxias and Association with Clinical Oculomotor Findings
Faye X Yang1, Rohin Manohar1, Anna C Luddy1
1Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
Cerebellum (London, England)
|July 20, 2026
Summary
Patients with ataxia experience significant visual impairment, especially in visual processing speed. Gaze-evoked nystagmus, an eye movement abnormality, is linked to this reduced visual function in spinocerebellar ataxias (SCAs).
Area of Science:
- Neurology
- Ophthalmology
- Genetics
Background:
- Oculomotor dysfunction is prevalent in ataxia patients, yet its effect on patient-reported visual function is not fully understood.
- Spinocerebellar ataxias (SCAs) are a group of inherited neurodegenerative disorders affecting motor control and potentially vision.
Purpose of the Study:
- To characterize patient-reported visual function across various ataxia types.
- To assess the functional significance of specific oculomotor abnormalities in spinocerebellar ataxias.
Main Methods:
- 117 participants (56 with genetically-defined ataxias, 61 controls) were recruited.
- Patient-reported visual function was assessed using the Visual Activities Questionnaire (VAQ).
- Clinical oculomotor assessments and ataxia severity scales were employed.
Main Results:
- All ataxia groups reported significantly worse visual function than controls, with the greatest deficits in visual processing speed.
- Individuals with SCA3 and SCA6 showed greater impairment across all visual subcategories compared to controls.
- Gaze-evoked nystagmus was the sole oculomotor sign independently associated with reduced visual function.
Conclusions:
- Ataxias are linked to substantial visual impairment, particularly affecting visual processing speed.
- Gaze-evoked nystagmus is a predictor of reduced visual function in ataxias, underscoring the impact of fixation instability.
- Patient-reported visual function and oculomotor assessments are crucial for evaluating visual disability in ataxia clinical care and trials.
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