Vestibular findings in autosomal recessive ataxia

Abstract

The most evident neurotological symptoms were dizziness, lack of coordination of movement, and imbalance when walking. Alterations in vestibular examinations occurred in 89.5% of patients, mostly in the caloric test, with a predominance of deficient central vestibular system dysfunction. This underscores the importance of the contribution of topodiagnostic labyrinthine evaluations for neurodegenerative diseases.

Otoneurological findings prevalent in hereditary ataxias

“…These findings agree with other studies 25 that showed involvement of the central vestibular system, and stress the importance of vestibular evaluations in diseases affecting the posterior fossa. Some authors 26 stressed that the range of changes in eye movements suggested changes in the brain stem, as well as the cortical and vestibular pathways.…”

“…The most common alterations in other studies 16,25 were the presence of positional nystagmus, irregular eye movement calibration, spontaneous rebound nystagmus, bidirectional and multiple semi-spontaneous nystagmus, abolition of optokinetic nystagmus, pendular tracking type IV, vestibular hyperfunction, absence of inhibitory effect of eye fixation, and Aubry signs in the Barany test. In the damaged neuronal structures, it is known that there is vestibular hypofunction, but little is known about when and why it occurs.…”