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. 2018 Mar 23;9:85. doi: 10.3389/fgene.2018.00085

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Copyright © 2018 Ahmad, Requena, Frejo, Cobo, Gallego-Martinez, Martin, Lopez-Escamez and Bronstein.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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Head and eye horizontal movements in the CANVAS proband. The patient fixates a visual target on the wall while the examiner manually oscillates his head from behind in a quasi-sinusoidal fashion [visually assisted vestibulo-ocular reflex or visually enhanced vestibulo-ocular reflex (VVOR)]. The compensatory eye movement elicited is severely broken-up or cog-wheeled due to the presence of multiple eye saccades (best seen as ‘spikes’ in the eye velocity trace). Upward deflections correspond to rightward head or eye movements.