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Updated: Feb 3, 2026

Author Spotlight: Deciphering the Role of ATM in Ataxia-Telangiectasia and the Associated Cerebellar Degeneration
Published on: December 27, 2024
Cerebellar Degeneration Increases Visual Influence on Dynamic Estimates of Verticality
Christopher J Dakin1, Amy Peters2, Paola Giunti3
1Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, London, UK; Department of Kinesiology and Health Sciences, Utah State University, Logan, UT, USA.
Abstract:
Our perception of verticality relies on combining sensory information from multiple sources. Neuronal recordings in animals implicate the cerebellum in the process, yet disease of the human cerebellum was not found to affect this perception. Here we show that a perceptual disturbance of verticality is indeed present in people with a genetically determined and pure form of cerebellar degeneration (spinocerebellar ataxia type 6; SCA 6), but is only revealed under dynamic visual conditions. Participants were required to continuously orient a visually displayed bar to vertical while the bar angle was perturbed by a low-frequency random signal and a random dot pattern rotated in their visual periphery. The random dot pattern was rotated at one of two velocities (4°/s and 16°/s), traveling with either coherent or noisy motion. Perceived vertical was biased by visual rotation in healthy participants, particularly in a more elderly group, but SCA 6 participants were biased more than both groups. The bias was reduced by visual noise, but more so for SCA 6 participants than young controls. Distortion of verticality by visual rotation stems from the stimulus creating an illusion of self-rotation. We modeled this process using a maximum-likelihood sensory cue-combination model operating on noisy visual- and vestibular-rotation signals. The observed effects of visual rotation and visual noise could be compellingly explained by cerebellar degeneration, and to a lesser extent aging, causing an increase in central vestibular noise. This is consistent with the human cerebellum operating on dynamic vestibular signals to inform the process that estimates which way is up.
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