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Challenges in evaluating the oculomotor function in individuals with Rett syndrome using electronystagmography
Laurèl H M de Breet1, Gillian S Townend2, Leopold M G Curfs2
1Faculty of Medicine, Maastricht University, Maastricht, the Netherlands.
Summary
Electronystagmography (ENG) challenges in Rett syndrome (RTT) highlight the need for adapted oculomotor exams. Modifications can improve communication assessments for individuals with RTT.
Area of Science:
- Neurology
- Ophthalmology
- Biomedical Engineering
Background:
- Rett syndrome (RTT) presents significant communication challenges.
- Oculomotor function is crucial for eye-tracking communication systems.
- Electronystagmography (ENG) is a method to assess oculomotor function, but faces challenges in individuals with RTT.
Purpose of the Study:
- To identify and evaluate challenges during ENG examinations in individuals with RTT.
- To inform adaptations for improved oculomotor assessments in RTT.
Main Methods:
- Oculomotor function assessed using ENG in 17 individuals with RTT and 16 typically developing (TD) controls.
- Observational analysis of performance and results focused on attention and signal quality.
- Quantitative exploration of attention (looking time) and signal quality (drift).
Main Results:
- The RTT group exhibited significantly reduced attention and suboptimal electrode signals compared to the TD group.
- These deficits were observed across most ENG tests, with the exception of the torsion swing test.
Conclusions:
- Standard oculomotor examinations require modification for individuals with RTT.
- The torsion swing test may be less visually dependent, potentially explaining better attention in the RTT group.
- Suggested adaptations include fewer electrodes, modified visual stimuli, observational assessments, and virtual reality goggles.
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